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Goh L, Ho JMD, Chiou FK. Disease characteristics and long-term outcomes of cyclic vomiting syndrome in Singaporean children. World J Gastrointest Pharmacol Ther 2025; 16:103971. [DOI: 10.4292/wjgpt.v16.i2.103971] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/11/2024] [Revised: 03/13/2025] [Accepted: 03/26/2025] [Indexed: 06/03/2025] Open
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) and its effect on nutritional status has not been well described.
AIM To describe the clinical characteristics, treatment and outcomes of children with CVS in Singapore.
METHODS Retrospective cohort study of pediatric patients aged 1 to 18 years old with CVS diagnosed at KK Women’s and Children’s Hospital in Singapore from 2011 to 2021.
RESULTS Thirty-two children (69% female) with CVS were included in the study, with mean age of onset of symptoms at 7 (± 4) years and mean follow up duration of 5 years. Forty percent (12/32) of patients were underweight at diagnosis with no other identifiable organic cause, with a median body mass index (BMI) z score -3.2 (range -2 to -7.5). The incidence of systemic hypertension was 10% (3/32). The overall mean frequency of exacerbations in this cohort of patients was 4 (± 4) episodes per year. In total, 16 (50%) patients, who had mean baseline frequency of 6 (± 5) attacks per year, were commenced on prophylactic treatment. Twelve patients (75%) responded to first-line therapy, whereas 4 (25%) required escalation to second-line treatment. With prophylactic treatment, there was an overall improvement in the frequency of attacks with a mean reduction of 5 (± 3) attacks per year. Also, there was improvement in the BMI z score of these patients from a median of -2.9 to -0.9.
CONCLUSION Prophylactic treatment is effective in improving nutritional status as well as reducing symptom frequency and should be considered for patients with complications such as growth failure and significant hypertension.
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Affiliation(s)
- Lynette Goh
- Department of Gastroenterology, Hepatology and Nutrition, KK Women's and Children's Hospital, Singapore 229899, Singapore
| | - Jeremy Meng Dao Ho
- Department of Paediatrics, KK Women's and Children's Hospital, Singapore 229899, Singapore
| | - Fang Kuan Chiou
- Department of Gastroenterology, Hepatology and Nutrition, KK Women's and Children's Hospital, Singapore 229899, Singapore
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2
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Hasler WL, Li BUK, Levinthal DJ, Venkatesan T. Cyclic vomiting syndrome: Future clinical and research priorities for: Special supplement/proceedings of 3rd international symposium. Neurogastroenterol Motil 2025; 37:e14825. [PMID: 38775195 DOI: 10.1111/nmo.14825] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2024] [Revised: 04/29/2024] [Accepted: 05/13/2024] [Indexed: 09/21/2024]
Abstract
BACKGROUND An increasing number of studies have explored the clinical features, epidemiology, pathophysiology, and management of cyclic vomiting syndrome (CVS). CVS is common in adults and children and negatively impacts patients, families, and the healthcare system. A related condition, cannabinoid hyperemesis syndrome (CHS), has been a focus of interest in the lay press and published literature. PURPOSE Clinical presentations of CVS have been defined by small series and expert opinion, but recent prospective studies are refining our understanding of the spectrum of emetic episodes and the breadth of comorbid conditions. Large cross-sectional population analyses are clarifying CVS prevalence and factors related to age, ethnicity, and geographic region. CVS pathophysiology is multifactorial with contributions from migraines, dysautonomia, endogenous cannabinoids, mitochondrial dysfunction, genetic abnormalities, and rapid gastric emptying. CVS treatment relies on antiemetics and antimigraine therapies to abort acute episodes coupled with prophylactic regimens employing neuromodulators and antiepileptics. CHS represents a challenge partly because of difficulties in achieving sustained cannabis abstinence. Benefits of other therapies in CHS remain poorly defined. Several areas warrant further scrutiny including better identification of CVS triggers and characterization of different CVS subsets including those with frequent severe episodes, refined description of epidemiology to allow targeting of populations predisposed to CVS development, rigorous definition of pathogenic factors to provide a foundation for exploratory studies of novel therapies, and conduct of controlled trials by multicenter collaborations to confirm benefits of existing and new therapies in development. Progress in these areas will be facilitated by generous governmental and industry support.
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Affiliation(s)
| | - B U K Li
- Medical College of Wisconsin, Milwaukee, Wisconsin, USA
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Bujarska M, Bora G, Li BUK, Banda J, Thomas D, Simpson P, Karrento K. Diagnostic characteristics of pediatric cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr 2025; 80:417-425. [PMID: 39737559 DOI: 10.1002/jpn3.12448] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2024] [Revised: 08/29/2024] [Accepted: 10/11/2024] [Indexed: 01/01/2025]
Abstract
OBJECTIVES Cyclic vomiting syndrome (CVS) remains a diagnostic challenge due to its nonspecific presentation despite consensus-based diagnostic criteria. There is a need for improved, evidence-based diagnostic criteria. We hypothesized that symptoms differ quantitatively between children with CVS versus other vomiting conditions and that current diagnostic criteria are not sufficiently sensitive for diagnosing CVS. METHODS Observational, prospective study of children ages 3-18 years with unexplained vomiting episodes evaluated in the outpatient gastroenterology clinic, emergency department, and inpatient units at Children's Wisconsin. Parents completed symptom surveys at 0, 3, and 6 months. Diagnostic workup and treatment response were monitored by chart review. A final diagnosis (CVS vs. non-CVS) was assigned for group comparisons and receiver operating characteristics (ROC) analysis of diagnostic cutoffs. RESULTS Of 108 subjects enrolled, 46 CVS and 54 non-CVS patients were analyzed. The groups reported overall different episode frequencies with more CVS versus non-CVS (81% vs. 55%) having ≥4 episodes/preceding 12 months (p = 0.013). CVS patients also had longer vomiting episodes (p = 0.03). To distinguish CVS from non-CVS, ROC analyses demonstrated the highest sensitivity for a frequency of 4-10 episodes/12 months (p = 0.002) and a duration threshold of >2 h (p < 0.001). CVS patients reported specific episode characteristics: photophobia (p = 0.003), diaphoresis (p = 0.002), multiple emeses/hour (p = 0.001), stereotypical episodes (p = 0.008), and continued retching after gastric emptying (p = 0.008). Less than half of CVS patients met Rome IV and North American Society for Pediatric Gastroenterology, Hepatology & Nutrition diagnostic criteria. CONCLUSIONS Children with CVS display a distinctive vomiting pattern and clinical features compared to other vomiting conditions. Our findings will help improve current diagnostic criteria.
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Affiliation(s)
- Malgorzata Bujarska
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Geetanjali Bora
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Children's National Hospital, Washington, DC, USA
| | - B U K Li
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Julie Banda
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Danny Thomas
- Division of Emergency Medicine, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Pippa Simpson
- Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
| | - Katja Karrento
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
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Pizzol A, Adams KA, de Vries BBA, Curry CJ, Calvo PL. Cyclic Vomiting Syndrome in Patients Affected by Jansen-de Vries Syndrome: Results From an International Survey. Am J Med Genet A 2025; 197:e63918. [PMID: 39494522 DOI: 10.1002/ajmg.a.63918] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2024] [Revised: 10/02/2024] [Accepted: 10/14/2024] [Indexed: 11/05/2024]
Abstract
Jansen-de Vries syndrome (JdVS) is an autosomal dominant neurodevelopmental disorder with intellectual disability and gastrointestinal (GI) abnormalities, including recurrent vomiting. This study aimed to understand the frequency and severity of GI symptoms in JdVS patients and to investigate the potential association with cyclic vomiting syndrome (CVS), which has not been previously reported. An international online survey assessed the prevalence and features of CVS and GI disorders in JdVS patients using Rome IV Criteria. The anonymous survey was conducted via Google Forms in April 2021. A total of 21 patients/guardians responded to the survey. The average age at JdVS diagnosis was 8.22 years (range: 1-42). Of the respondents, 6 (28.5%) had a CVS diagnosis, 5 (23.8%) had migraine, and 2 (9.5%) had abdominal migraine. Additionally, 8 (38%) had gastroesophageal reflux disease (GERD) and 8 (38%) had functional constipation. An analysis targeted questions showed that 7 (33%) met the Rome IV Criteria for CVS but were undiagnosed, leading to a CVS prevalence of 61% in this cohort. This study highlights a high prevalence of CVS in JdVS patients and underscores the need for increased awareness and accurate diagnosis to address misdiagnosis.
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Affiliation(s)
- A Pizzol
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città Della Salute e Della Scienza, Turin, Italy
| | - K A Adams
- Cyclic Vomiting Syndrome Association, Milwaukee, Wisconsin, USA
| | - B B A de Vries
- Department of Human Genetics, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands
| | - C J Curry
- Genetic Medicine, Department of Pediatrics, University of California, San Francisco, California, USA
| | - P L Calvo
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città Della Salute e Della Scienza, Turin, Italy
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Frazier R, Ghoshal UC, Remes-Troche J, Robin D, Shun E, Aravind T. Exploring cyclic vomiting syndrome (CVS) worldwide: Current epidemiological insights and recent developments. Neurogastroenterol Motil 2025; 37:e14932. [PMID: 39370604 DOI: 10.1111/nmo.14932] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/14/2024] [Revised: 08/22/2024] [Accepted: 09/15/2024] [Indexed: 10/08/2024]
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS), a disorder of gut-brain interaction, presents with recurrent episodes of severe nausea and vomiting. It is often associated with missed or delayed diagnoses and substantial healthcare utilization. Despite historical recognition dating back to the 19th century, epidemiological insights remain limited, with research predominantly originating from specific regions, such as the US. CVS prevalence and incidence rates vary widely and are hindered by inconsistent methodologies and disease recognition. PURPOSE This review aims to provide a comprehensive overview of CVS prevalence and incidence rates. It reviews the currently available data and identifies gaps in knowledge. Understanding the global epidemiology of CVS, increasing awareness of the disease, and fostering global collaboration are crucial. Other pertinent issues include disparities in outcomes, particularly among African Americans and Hispanics in the United States, underscoring the need to understand the social determinants of health that drive disease outcomes. This understanding can inform targeted interventions to address these barriers and achieve equitable healthcare both in the United States and globally.
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Affiliation(s)
- Rosita Frazier
- Division of Gastroenterology and Hepatology, Mayo Clinic Arizona, Scottsdale, Arizona, USA
| | - Uday C Ghoshal
- Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Jose Remes-Troche
- Digestive Physiology and Motility Lab, Veracruzana University, Xalapa, Mexico
| | - Dover Robin
- Cyclical Vomiting Syndrome Association, Letchworth, UK
| | - Emoto Shun
- Department of Family Nursing, Division of Health Sciences and Nursing, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Thavamani Aravind
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, UH Rainbow Babies and Children's Hospital, Case Western Reserve University, Cleveland, Ohio, USA
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Greene KA, Gelfand AA, Larry Charleston. Evidence-based review and frontiers of migraine therapy. Neurogastroenterol Motil 2025; 37:e14899. [PMID: 39133210 PMCID: PMC11911302 DOI: 10.1111/nmo.14899] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/29/2024] [Revised: 07/25/2024] [Accepted: 07/31/2024] [Indexed: 08/13/2024]
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) is identified as one of the "episodic syndromes that may be associated with migraine," along with benign paroxysmal torticollis, benign paroxysmal vertigo, and abdominal migraine. It has been proposed that CVS and migraine may share pathophysiologic mechanisms of hypothalamic activation and altered dopaminergic signaling, and impaired sensorimotor intrinsic connectivity. The past decade has brought groundbreaking advances in the treatment of migraine and other headache disorders. While many of these therapies have yet to be studied in episodic syndromes associated with migraine including CVS and abdominal migraine, the potential shared pathophysiology among these conditions suggests that use of migraine-specific treatments may have a beneficial role even in those for whom headache is not the primary symptom. PURPOSE This manuscript highlights newer therapies in migraine. Calcitonin gene-related peptide (CGRP) and its relation to migraine pathophysiology and the therapies that target the CGRP pathway, as well as a 5HT1F receptor agonist and neuromodulation devices used to treat migraine are briefly discussed as they may potentially prove to be useful in the future treatment of CVS.
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Affiliation(s)
- Kaitlin A. Greene
- Division of Pediatric NeurologyOregon Health and Science UniversityPortlandOregonUSA
| | - Amy A. Gelfand
- Child and Adolescent Headache ProgramUniversity of CaliforniaSan FranciscoCaliforniaUSA
| | - Larry Charleston
- Department of Neurology and OphthalmologyMichigan State University College of Human MedicineGrand RapidsMichiganUSA
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Chen YJ, Princic N, Winer I, Richmond C, Williams J, Thavamani A, Levinthal DJ, Venkatesan T. Epidemiology, Comorbidities, and Treatment of Cyclic Vomiting Syndrome in the United States. Am J Gastroenterol 2024; 119:965-976. [PMID: 38088366 PMCID: PMC11062608 DOI: 10.14309/ajg.0000000000002628] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/22/2023] [Accepted: 12/06/2023] [Indexed: 01/17/2024]
Abstract
INTRODUCTION Cyclic vomiting syndrome (CVS) imposes a substantial burden, but epidemiological data are scarce. This study aimed to estimate the incidence and prevalence of CVS, comorbid conditions, and treatment patterns, using administrative databases in the United States. METHODS This cross-sectional study used claims data from Merative MarketScan Commercial/Medicare Supplemental and Medicaid databases in all health care settings. Incidence and prevalence rates for 2019 were calculated and stratified by age, sex, region, and race/ethnicity. Patient characteristics were reported among newly diagnosed patients with CVS (i.e., no documented claims for CVS before 2019). CVS was defined as having 1+ inpatient and/or 2+ outpatient CVS claims that were 7+ days apart. RESULTS The estimated prevalence of CVS was 16.7 (Commercial/Medicare) and 42.9 (Medicaid) per 100,000 individuals. The incidence of CVS was estimated to be 10.6 (Commercial/Medicare) and 26.6 (Medicaid) per 100,000 individuals. Both prevalence and incidence rates were higher among female individuals (for both Commercial/Medicare and Medicaid). Comorbid conditions were common and included abdominal pain (56%-64%), anxiety (32%-39%), depression (26%-34%), cardiac conditions (39%-42%), and gastroesophageal reflux disease (30%-40%). Despite a diagnosis of CVS, only 32%-35% had prescriptions for prophylactic treatment and 47%-55% for acute treatment within the first 30-day period following diagnosis. DISCUSSION This study provides the first population-level estimates of CVS incidence and prevalence in the United States. Comorbid conditions are common, and most patients with CVS do not receive adequate treatment. These findings underscore the need for improving disease awareness and developing better screening strategies and effective treatments.
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Affiliation(s)
| | | | | | - Camilla Richmond
- Takeda Development Center of Americas, Cambridge, Massachusetts, USA
| | - James Williams
- Takeda Development Center of Americas, Cambridge, Massachusetts, USA
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Khalil C, Almario CV, Dupuy T, Arnold C, Chen YJ, Spiegel BMR. Perspectives, experiences, and concerns with cyclical vomiting syndrome: Insights from online targeted-disease forums. Neurogastroenterol Motil 2024; 36:e14712. [PMID: 38191754 DOI: 10.1111/nmo.14712] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/09/2023] [Revised: 10/10/2023] [Accepted: 11/07/2023] [Indexed: 01/10/2024]
Abstract
INTRODUCTION There is limited research examining the biopsychosocial impact of cyclic vomiting syndrome (CVS) on patients. This study aims to assess individuals' experiences, fears, and concerns associated with CVS and the impact of CVS on their daily lives. METHODS We employed social netnography to analyze publicly available posts related to CVS that were identified from six US online forums and Twitter. A randomly selected sub-cohort of posts per pre-defined criteria was first qualitatively analyzed using an inductive thematic approach. Then, machine learning topic modeling was applied to explore themes in an unsupervised manner for the entire corpus of posts. Afterward, findings from the qualitative and quantitative approaches were integrated to generate a thematic network. RESULTS Based on the 39,179 collected posts, seven domain themes were identified. Overall, 41.4% of the posts were related to "biopsychosocial burden" of CVS, including physical impact, psychological impact, and social impact. In 22.3% of posts, individuals shared their experience of "interactions with the healthcare system", and 14.2% of posts were related to "perceived CVS triggers." Individuals also shared "solutions to alleviate their symptoms" and "mental health needs" in 10.2% and 8.8% of posts, respectively. Finally, 6.1% of the posts were about "seeking/sharing support" with others. DISCUSSION This is the first social netnography study to describe the in-depth experiences of individuals living with CVS and the marked impact on their physical, mental, and social health. The study also highlights the unmet need for effective therapies, both pharmacological and non-pharmacological, to alleviate the biopsychosocial impact of CVS.
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Affiliation(s)
- Carine Khalil
- Division of Health Services Research, Cedars-Sinai Medical Center, Center for Outcomes Research and Education (CS-CORE), Los Angeles, California, USA
| | - Christopher V Almario
- Division of Health Services Research, Cedars-Sinai Medical Center, Center for Outcomes Research and Education (CS-CORE), Los Angeles, California, USA
| | - Taylor Dupuy
- Division of Health Services Research, Cedars-Sinai Medical Center, Center for Outcomes Research and Education (CS-CORE), Los Angeles, California, USA
| | - Corey Arnold
- Department of Radiology, Medical Imaging Informatics, UCLA, Los Angeles, California, USA
| | - Yaozhu J Chen
- Takeda Development Center Americas, Cambridge, Massachusetts, USA
| | - Brennan M R Spiegel
- Division of Health Services Research, Cedars-Sinai Medical Center, Center for Outcomes Research and Education (CS-CORE), Los Angeles, California, USA
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Togha M, Babaei M, Jameie M. Good response to rectal diazepam in refractory cases of cyclic vomiting: A case-series and review of the literature. Clin Case Rep 2023; 11:e8109. [PMID: 38028097 PMCID: PMC10661318 DOI: 10.1002/ccr3.8109] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/29/2023] [Revised: 07/19/2023] [Accepted: 08/28/2023] [Indexed: 12/01/2023] Open
Abstract
Key Clinical Message Although increasing in number, cases of CVS are being frequently misdiagnosed and many are refractory to the available treatments. This paper draws attention to a timely consideration of this disorder upon suspicion and proposes rectal diazepam and cinnarizine as highly effective treatments in refractory cases of CVS. Abstract Cyclic vomiting syndrome (CVS) is a set of recurrent episodic attacks of nausea and vomiting. This is a migraine-related disorder that mostly affects children. Several medications have been recommended for abortive and prophylactic treatment. Unfortunately, in some cases, the treatment is not completely effective and affects the quality of life of the sufferer. In this paper, we report on two cases of children experiencing refractory CVS attacks who were not responsive to recommended medications for acute phase and prophylaxis. This account highlights the efficacy of rectal diazepam for the acute phase of CVS and cinnarizine, an anti-migraine and anti-histamine agent, for prophylaxis of further attacks.
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Affiliation(s)
- Mansoureh Togha
- Neurology department, Sina Hospital, School of MedicineTehran University of Medical SciencesTehranIran
- Headache department, Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
| | - Mahsa Babaei
- Headache department, Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
| | - Melika Jameie
- Headache department, Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
- Iranian Center of Neurological Research, Neuroscience InstituteTehran University of Medical SciencesTehranIran
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Thavamani A, Velayuthan S, Patel D, Al-Hammadi N, Sferra TJ, Sankararaman S. Association of Anxiety and Gastrointestinal Comorbidities in Repeat Hospital Admissions in Pediatric Cyclic Vomiting Syndrome. Am J Gastroenterol 2023; 118:1439-1445. [PMID: 37052354 DOI: 10.14309/ajg.0000000000002292] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/15/2022] [Accepted: 03/28/2023] [Indexed: 04/14/2023]
Abstract
INTRODUCTION Cyclic vomiting syndrome (CVS) is a functional gastrointestinal disorder with recurrent episodes of intense nausea and vomiting and thus may require frequent hospitalizations. There is paucity of data exploring the association of psychiatric and gastrointestinal comorbidities in repeat hospitalizations among pediatric patients with CVS. METHODS We analyzed the Pediatric Health Information System database and included all patients up to 18 years of age with a diagnosis of CVS between 2016 and 2020. We excluded patients with chronic conditions, which mimic CVS. The primary outcome variable was 90-day admission rate, which was defined as a visit to emergency department or admission to observation/inpatient unit with a primary diagnosis of CVS within 90 days after an index CVS hospitalization. RESULTS We evaluated a total of 2,604 hospitalizations represented by 1,370 unique individuals. The overall 90-day admission rate was 28.5%, which steadily decreased from 35.7% in 2016 to 23% in 2019 ( P < 0.001). Patients in the repeat hospitalization cohort were slightly older and more often men. Patients with repeat admissions had an increased proportion of anxiety and other gastrointestinal disorders. Multivariable logistic regression showed that anxiety, gastroesophageal reflux disease, functional dyspepsia, and abdominal migraine were associated with increased odds of repeat admissions. DISCUSSION Ninety-day admission rates in pediatric CVS are decreasing overall, although still contributing to significant healthcare expenditure. Anxiety and gastrointestinal comorbidities were associated with increased risk of repeat admissions. Further prospective studies are needed to better understand the complex interactions of these comorbidities and their management affecting the natural course of CVS.
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Affiliation(s)
- Aravind Thavamani
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
| | - Sujithra Velayuthan
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
| | - Dhiren Patel
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, SSM Health Cardinal Glennon Children's Medical Center, Saint Louis University School of Medicine, St. Louis, Missouri, USA
| | - Noor Al-Hammadi
- The Advanced HEAlth Data (AHEAD) Research Institute, Saint Louis University School of Medicine, St. Louis, Missouri, USA
| | - Thomas J Sferra
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
| | - Senthilkumar Sankararaman
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, UH Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
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11
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Gosalvez-Tejada A, Li BUK, Simpson P, Zhang L, Kovacic K. Natural History of Pediatric Cyclic Vomiting Syndrome: Progression to Dysautonomia. J Pediatr Gastroenterol Nutr 2023; 76:737-742. [PMID: 36800281 DOI: 10.1097/mpg.0000000000003738] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/18/2023]
Abstract
BACKGROUND The clinical features of pediatric cyclic vomiting syndrome (CVS) often evolve over time. Many patients develop a constellation of chronic symptoms that suggest autonomic nervous system (ANS) dysfunction during adolescence. We aimed to determine the proportion of children with CVS who develop chronic rather than episodic symptoms consistent with ANS dysfunction. METHODS Retrospective chart review of children ages 0-18 years followed in an outpatient tertiary care CVS center. Patients completed standardized questionnaires at intake and follow-up visits, documenting clinical symptom pattern. Continuous variables are summarized as median [interquartile range (IQR)]. A Mann-Whitney test was used for group comparisons. RESULTS One hundred subjects were included. A total of 40% developed symptoms of ANS dysfunction (ANS+); 20% were confirmed by comprehensive ANS testing, 11% by orthostatic vital sign abnormalities, and 9% by clinical symptoms. The median (IQR) age at onset of chronic symptoms was 14 (10.02, 15) years. The presence of another disorder of gut-brain interaction ( P = 0.018) and a greater number of comorbidities ( P = 0.031) were more common in the ANS+ group. ANS+ subjects missed more school days ( P = 0.047) and were seen less frequently in the emergency department ( P = 0.023). CONCLUSIONS Many children with CVS (40%) develop symptoms consistent with clinical dysautonomia in adolescence. These patients experience more comorbid conditions and a greater impact on school attendance, possibly representing a worsened quality of life as their disease course transitions to daily symptoms. When symptoms of CVS change over time, therapeutic interventions may need to be adjusted and targeted accordingly.
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Affiliation(s)
- Andrea Gosalvez-Tejada
- From the Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - B U K Li
- From the Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - Pippa Simpson
- the Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - Liyun Zhang
- the Division of Quantitative Health Sciences, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
| | - Katja Kovacic
- From the Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
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Haghighat M, Gholami Shahrebabak M, Dehghani SM, Ataollahi M, Amin Farzaneh N, Hamzeloo Hoseinabadi S, Javaherizadeh H. Relapse Rate of Clinical Symptoms After Stopping Treatment in Children with Cyclic Vomiting Syndrome. Middle East J Dig Dis 2023; 15:32-36. [PMID: 37547164 PMCID: PMC10404072 DOI: 10.34172/mejdd.2023.317] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/10/2022] [Accepted: 11/15/2022] [Indexed: 08/08/2023] Open
Abstract
Background: Cyclic vomiting syndrome (CVS) is a chronic functional gastrointestinal disorder. It is characterized by recurrent episodes of vomiting typically separated by periods of symptom-free or baseline health. The present study aimed at evaluating the effectiveness of propranolol and the relapse rate of clinical symptoms after stopping treatment in children suffering from CVS. Methods: Records of 504 patients below the age of 18 years with CVS who were treated with propranolol from March 2008 to March 2018 were reviewed. The duration of follow-up was 10 years. Results: The average age of CVS affliction was 4.3 years and the average age at the diagnosis was 5.8 years. All subjects were treated with propranolol (for an average of 10 months). 92% of treated subjects were cured, causing a dramatic decrease in the rate of vomiting (P < 0.001). Only an average of 10.5% of the studied subjects (53 people) showed a relapse of symptoms after stopping the treatment. The results of a 10-year follow-up period of the patients showed that 24 had abdominal migraine and 6 had migraine headaches, all of whom lacked the symptoms of disease relapse (prognostic evaluation). Conclusion: The findings of this investigation show that the duration of treating CVS with propranolol could be shortened to 10 months with a low percent of symptoms relapse and this shortening may be effective in preventing the undesirable side effects of the drug. The presence of abdominal migraine and migraine headaches in patients after treatment accomplishment and the lack of disease relapse can be prognostic measures for this disease, which require intensive attention.
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Affiliation(s)
- Mahmoud Haghighat
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Maryam Gholami Shahrebabak
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Seyed Mohsen Dehghani
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Maryam Ataollahi
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Nazanin Amin Farzaneh
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Samaneh Hamzeloo Hoseinabadi
- Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Hazhir Javaherizadeh
- Clinical Sciences Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
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Cheema S, Matharu M. Abdominal migraine and cyclical vomiting syndrome. HANDBOOK OF CLINICAL NEUROLOGY 2023; 198:209-219. [PMID: 38043963 DOI: 10.1016/b978-0-12-823356-6.00006-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/05/2023]
Abstract
Abdominal migraine and cyclical vomiting syndrome (CVS) are characteristic syndromes which have overlapping characteristics with migraine but lack the cardinal symptom of headache. Both abdominal migraine and CVS are characterized by recurrent attacks of nausea, vomiting, and/or abdominal pain lasting hours to a few days, with symptom freedom between attacks. Both abdominal migraine and CVS typically occur in children and adolescents, who often go on to develop more typical migraine headaches when older, but may also present for the first time in adults. Due to their shared characteristics and association with migraine headaches, abdominal migraine and CVS are sometimes called "migraine equivalents," and their pathophysiology is assumed to overlap with migraine headache. This chapter describes what is known about the clinical characteristics, epidemiology, pathophysiology, and prognosis of abdominal migraine and CVS, and explores their relationship to migraine. We also review the existing evidence for the nonpharmacological management, acute treatment of attacks, and preventive treatments for both abdominal migraine and CVS.
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Affiliation(s)
- Sanjay Cheema
- Headache and Facial Pain Group, University College London (UCL) Queen Square Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom
| | - Manjit Matharu
- Headache and Facial Pain Group, University College London (UCL) Queen Square Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.
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Frazier R, Venkatesan T. Current understanding of the etiology of cyclic vomiting syndrome and therapeutic strategies in its management. Expert Rev Clin Pharmacol 2022; 15:1305-1316. [PMID: 36259482 DOI: 10.1080/17512433.2022.2138341] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
INTRODUCTION Cyclic vomiting syndrome is a chronic debilitating disorder of the gut-brain interaction and is characterized by recurrent episodes of nausea and vomiting.Recent studies indicate that it is common and affects 2% of the US population. Unfortunately, there is significant heterogeneity in the management of these patients in the medical community. This review article aims to bridge this gap and will review the epidemiology and etiology with a focus on management of CVS. AREAS COVERED This article reviews the epidemiology, and pathophysiology of CVS and impact on patients. It also discusses management based on recent guidelines based on which is intended for the busy clinician. A literature search was done using PubMed and key words "cyclic vomiting", "management", "etiology", and pathophysiology were used to identify articles of importance. EXPERT OPINION CVS is a complex, poorly understood disorder of gut-brain interaction (DGBI) and has a significant negative impact on patients, families and the healthcare system. Recent guidelines recommend a multidisciplinary approach to management using prophylactic therapy in moderate-severe CVS and abortive medication for acute flares. However more research is needed to better understand the pathophysiology and develop targeted therapies for CVS.
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Affiliation(s)
- Rosita Frazier
- Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, Arizona
| | - Thangam Venkatesan
- Division of Gastroenterology, Hepatology and Nutrition, The Ohio State University, Columbus, OH
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15
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Isoldi S, Di Nardo G, Mallardo S, Parisi P, Raucci U, Tambucci R, Quitadamo P, Salvatore S, Felici E, Cisarò F, Pensabene L, Banzato C, Strisciuglio C, Romano C, Fusco P, Rigotti F, Sansotta N, Caimmi S, Savasta S, Zuin G, Di Stefano M, Provera S, Campanozzi A, Rossi P, Gatti S, Corpino M, Alvisi P, Martelossi S, Suppiej A, Gandullia P, Verrotti A, Terrin G, Pacenza C, Fornaroli F, Comito D, D'Arrigo S, Striano P, Raviglione F, Carotenuto M, Orsini A, Belcastro V, Di Corcia G, Raieli V, Ferilli MAN, Ruscitto C, Spadoni E, Grosso S, D'Alonzo R, Papa A, Pavone P, Meli M, Velardita M, Mainetti M, Vanacore N, Borrelli O. Cyclic vomiting syndrome in children: a nationwide survey of current practice on behalf of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP). Ital J Pediatr 2022; 48:156. [PMID: 36042474 PMCID: PMC9429644 DOI: 10.1186/s13052-022-01346-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2022] [Accepted: 08/10/2022] [Indexed: 11/23/2022] Open
Abstract
BACKGROUND Cyclic Vomiting Syndrome (CVS) is a rare functional gastrointestinal disorder, which has a considerable burden on quality of life of both children and their family. Aim of the study was to evaluate the diagnostic modalities and therapeutic approach to CVS among Italian tertiary care centers and the differences according to subspecialties, as well as to explore whether potential predictive factors associated with either a poor outcome or a response to a specific treatment. METHODS Cross-sectional multicenter web-based survey involving members of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP). RESULTS A total of 67 responses were received and analyzed. Most of the respondent units cared for less than 20 patients. More than half of the patients were referred after 3 to 5 episodes, and a quarter after 5 attacks. We report different diagnostic approaches among Italian clinicians, which was particularly evident when comparing gastroenterologists and neurologists. Moreover, our survey demonstrated a predilection of certain drugs during emetic phase according to specific clinic, which reflects the cultural background of physicians. CONCLUSION In conclusion, our survey highlights poor consensus amongst clinicians in our country in the diagnosis and the management of children with CVS, raising the need for a national consensus guideline in order to standardize the practice.
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Affiliation(s)
- Sara Isoldi
- Maternal and Child Health Department, Santa Maria Goretti Hospital, Sapienza-University of Rome, Latina, Italy
| | - Giovanni Di Nardo
- Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, 00189, Rome, Lazio, Italy
| | - Saverio Mallardo
- Maternal and Child Health Department, Santa Maria Goretti Hospital, Sapienza-University of Rome, Latina, Italy
| | - Pasquale Parisi
- Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, 00189, Rome, Lazio, Italy.
| | - Umberto Raucci
- Pediatric Emergency Department, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Renato Tambucci
- Digestive Endoscopy and Surgery Unit, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Paolo Quitadamo
- Department of Pediatrics, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Silvia Salvatore
- Pediatric Department, Ospedale "F. Del Ponte", University of Insubria, Varese, Italy
| | - Enrico Felici
- Unit of Pediatrics, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
| | - Fabio Cisarò
- Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria, Città della Salute e della Scienza di Torino, Turin, Italy
| | - Licia Pensabene
- Pediatric Unit, Department of Medical and Surgical Sciences, University "Magna Graecia" of Catanzaro, Catanzaro, Italy
| | - Claudia Banzato
- Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Pediatric Division, University of Verona, Verona, Italy
| | - Caterina Strisciuglio
- Department of Woman, Child, General and Specialistic Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy
| | - Claudio Romano
- Pediatric Gastroenterology Unit, Department of Human Pathology in Adulthood and Childhood "G. Barresi", University of Messina, Messina, Italy
| | - Patrizia Fusco
- Pediatric Unit, Montis Regalis Hospital, Mondovì, Cuneo, Italy
| | | | - Naire Sansotta
- Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy
| | - Silvia Caimmi
- Pediatric Clinic, Foundation IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
| | - Salvatore Savasta
- Pediatric Clinic, Foundation IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
| | - Giovanna Zuin
- Pediatric Department, University of Milano Bicocca, FMBBM, San Gerardo Hospital, Monza, Italy
| | - Marina Di Stefano
- Department of Pediatrics, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Silvia Provera
- Department of Pediatrics, IRCCS Sacro Cuore Don Calabria Hospital, Negrar, Verona, Italy
| | - Angelo Campanozzi
- Department of Medical and Surgical Sciences, Pediatric Unit, University of Foggia, Foggia, Italy
| | - Paolo Rossi
- Department of Pediatrics, Pediatric Gastroenterology and Liver Unit, Sapienza University of Rome, Rome, Italy
| | - Simona Gatti
- Department of Pediatrics, Università Politecnica delle Marche, Ancona, Italy
| | - Mara Corpino
- Gastroenterologia Pediatrica, Clinica Pediatrica e Malattie Rare, Ospedale Pediatrico Microcitemico A. Cao, ARNAS Brotzu, Cagliari, Italy
| | - Patrizia Alvisi
- Department of Paediatrics, Ospedale Maggiore, Azienda USL, Bologna, Italy
| | | | - Agnese Suppiej
- Pediatric Section, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
| | - Paolo Gandullia
- UOC Gastroenterologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | | | - Gianluca Terrin
- Department of Maternal and Child Health, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy
| | - Caterina Pacenza
- Department of Pediatrics, San Giovanni di Dio Hospital, Crotone, Italy
| | - Fabiola Fornaroli
- Gastroenterology and Endoscopy Unit, Department of Medicine and Surgery, University of Parma, Parma, Italy
| | | | - Stefano D'Arrigo
- Developmental Neurology Division, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Pasquale Striano
- Pediatric Neurology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy
| | | | - Marco Carotenuto
- Clinic of Child and Adolescent Neuropsychiatry, Department of Mental Health and Physical and Preventive Medicine, Università degli Studi della Campania "Luigi Vanvitelli", Caserta, Italy
| | - Alessandro Orsini
- Paediatric Neurology Section, Paediatric Department, Pisa University Hospital, University of Pisa, Pisa, Italy
| | | | | | - Vincenzo Raieli
- Child Neuropsychiatry Unit - ISMEP- ARNAS CIVICO, Palermo, Italy
| | - Michela Ada Noris Ferilli
- Department of Neuroscience, Paediatric Headache Center, Bambino Gesù Children Hospital, IRCCS, Rome, Italy
| | - Claudia Ruscitto
- Child Neurology and Psychiatry Unit, Systems Medicine Department, Tor Vergata University Hospital of Rome, Rome, Italy
| | - Elisabetta Spadoni
- Pediatric Department, Azienda Ospedaliera Nord-Ovest of Tuscany, San Luca Hospital of Lucca, Lucca, Italy
| | - Salvatore Grosso
- Molecular Medicine and Development, University of Siena, Siena, Italy
| | - Renato D'Alonzo
- Pediatric and Neonatological Unit, Maternal and Child Department, Nuovo Ospedale San Giovanni Battista, Foligno, Italy
| | - Amanda Papa
- Infantile Neuropsychiatry Departement Maggiore della Carità University Hospital, Novara, Italy
| | - Piero Pavone
- Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
| | - Mariaclaudia Meli
- Azienda Policlinico, "Rodolico-San Marco Hospital", University of Catania, Catania, Italy
| | - Mario Velardita
- Department of Pediatrics, Hospital "Salvatore e Saverio Gravina", Caltagirone, Italy
| | - Martina Mainetti
- Department of Pediatrics, Santa Maria delle Croci Hospital, Ravenna, Italy
| | - Nicola Vanacore
- National Centre for Epidemiology, Surveillance and Health Promotion, National Institute of Health, Rome, Italy
| | - Osvaldo Borrelli
- Division of Neurogastroenterology and Motility, Department of Pediatric Gastroenterology, University College London (UCL) Institute of Child Health and Great Ormond Street Hospital, London, UK
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Dipasquale V, Falsaperla R, Bongiovanni A, Ruggieri M, Romano C. Clinical features and long-term outcomes in pediatric cyclic vomiting syndrome: A 9-year experience at three tertiary academic centers. Neurogastroenterol Motil 2022; 34:e14224. [PMID: 34431167 DOI: 10.1111/nmo.14224] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2021] [Revised: 06/08/2021] [Accepted: 07/07/2021] [Indexed: 02/08/2023]
Abstract
BACKGROUND Pediatric cyclic vomiting syndrome (CVS) is a little-known clinical condition, frequently diagnosed with delay. This study aims to describe the clinical presentation and management and to define possible predictive factors of the disease outcome. METHODS In this retrospective study, all children who were diagnosed with CVS during the period 2010-2019 in three tertiary academic centers were included. The association between clinical variables and outcomes was investigated. KEY RESULTS Fifty-seven children were included (male/female ratio 1.3:1; mean age at diagnosis 8.2 years). At the time of diagnosis, 63% of children had at least one episode every month. One or more prodromes were reported by 75% of patients. Family history of migraine was reported for 47% of children. Nearly, all of the children were started on prophylactic treatment. The median follow-up period was 29 months ± 15. Overall, 56% of children had resolution of vomiting. Twenty-six percent of children developed migraine. There were no differences in gender, age at onset, duration of follow-up, severity, medication, family history, or trigger factors between children who underwent resolution of vomiting and those who did not, or between children who suffered from migraine or not at follow-up. Four types of disease outcome were recognized, that is, resolution of vomiting with or without associated symptoms; persistence of vomiting with or without associated symptoms. CONCLUSIONS AND INFERENCES Cyclic vomiting syndrome clinical presentation has inter-individual variability. Most children recover at follow-up, but often develop further somatic symptoms. In this study, no clinical variable clearly predicted the evolution of the syndrome toward one or the other outcome.
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Affiliation(s)
- Valeria Dipasquale
- Unit of Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood ''G. Barresi'', University of Messina, Messina, Italy
| | - Raffaele Falsaperla
- Neonatal Intensive Care Unit [NICU], Pediatrics and Pediatric Emergency Unit, AOU "Policlinico", PO "San Marco", University of Catania, Catania, Italy
| | - Annarita Bongiovanni
- Neonatal Intensive Care Unit [NICU], Pediatrics and Pediatric Emergency Unit, AOU "Policlinico", PO "San Marco", University of Catania, Catania, Italy
| | - Martino Ruggieri
- Unit of Rare Diseases of the Nervous System in Childhood, Department of Clinical and Experimental Medicine, Section of Pediatrics and Child Neuropsychiatry, University of Catania, Catania, Italy
| | - Claudio Romano
- Unit of Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood ''G. Barresi'', University of Messina, Messina, Italy
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Thavamani A, Umapathi KK, Khatana J, Bhandari S, Kovacic K, Venkatesan T. Cyclic Vomiting Syndrome-Related Hospitalizations Trends, Comorbidities & Health Care Costs in Children: A Population Based Study. CHILDREN (BASEL, SWITZERLAND) 2022; 9:55. [PMID: 35053680 PMCID: PMC8774277 DOI: 10.3390/children9010055] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/26/2021] [Revised: 12/20/2021] [Accepted: 12/27/2021] [Indexed: 11/29/2022]
Abstract
AIM To analyze the clinical characteristics, trends in hospitalization and health care resource utilization of pediatric patients with cyclical vomiting syndrome (CVS). METHODS We analyzed the latest 5 Healthcare Cost and Utilization Project-Kids Inpatient Database (HCUP-KID) datasets including years 2003, 2006, 2009, 2012 and 2016 for patients aged 1-20 years with a primary diagnosis of CVS and were compared with Age/gender-matched controls for comorbidities, clinical outcomes, and healthcare resource utilization. RESULTS A total of 12,396 CVS-related hospitalizations were analyzed. The mean age of CVS patients was 10.4 ± 6.7 years. CVS was associated with dysautonomia (OR: 12.1; CI: 7.0 to 20.8), dyspepsia (OR: 11.9; CI: 8.8 to 16.03), gastroesophageal reflux disease (OR: 6.9; Confidence Interval (CI): 6.4 to 7.5), migraine headaches (OR: 6.8; CI: 5.9 to 7.7) and irritable bowel syndrome (OR: 2.08; CI: 1.2 to 4.3) (all p < 0.001). CVS was also associated with increased cannabis use (OR: 5.26, 4.6 to 5.9; p < 0.001), anxiety disorder (OR: 3.9; CI: 3.5 to 4.4) and stress reaction (OR: 3.6; CI: 2.06 to 6.3), p < 0.001. Mean CVS-related hospitalization costs (inflation adjusted) more than doubled from $3199 in 2003 to $6721 in 2016, incurring $84 million/year in total costs. CONCLUSION Hospitalized CVS patients have increased prevalence of DGBIs, dysautonomia, psychiatric conditions and cannabis use compared to non-CVS controls. CVS-related hospitalizations in U.S. is associated with increasing health care costs. Better management of CVS and comorbid conditions is warranted to reduce health care costs and improve outcomes.
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Affiliation(s)
- Aravind Thavamani
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, UH Rainbow Babies and Children’s Hospital, Case Western Reserve University, Cleveland, OH 44106, USA
| | | | - Jasmine Khatana
- Department of Pediatrics, Metrohealth Medical Center, Cleveland, OH 44106, USA;
| | - Sanjay Bhandari
- Department of Medicine, Medical College of Wisconsin, Milwaukee, WI 53226, USA;
| | - Katja Kovacic
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI 53226, USA;
| | - Thangam Venkatesan
- Division of Gastroenterology and Hepatology, Medical College of Wisconsin, Milwaukee, WI 53226, USA;
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18
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Redon S. Episodic syndromes that may be associated with migraine: What adult neurologists need to know. Rev Neurol (Paris) 2021; 177:773-778. [PMID: 34366172 DOI: 10.1016/j.neurol.2021.07.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Revised: 07/02/2021] [Accepted: 07/06/2021] [Indexed: 11/24/2022]
Abstract
Four episodic syndromes are recognized in the current version of the International Classification of Headache Disorders (ICHD): cyclic vomiting syndrome (CVS); abdominal migraine; benign paroxysmal vertigo; benign paroxysmal torticollis. Previously called childhood periodic syndromes, they are thought to be early expressions of a migrainous spectrum. They usually occur in childhood or adolescence and some of these patients will develop migraine later in childhood or in adulthood. More rarely, some of these disorders, in particular CVS and abdominal migraine, can persist or even begin at adulthood. The concept of episodic syndromes associated with migraine in adults is relatively recent, recognized for the first time in the version III-beta of the ICHD (2013). It is important for the adult neurologist to recognize the clinical pattern of these disorders. Lack of knowledge of these disorders often leads to delayed diagnosis, and a large number of complementary tests. Treatments are often borrowed from migraine abortive and preventive treatments. This review summarizes the characteristics of episodic syndromes and is particularly focused on data in adults.
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Affiliation(s)
- S Redon
- Department of Evaluation and Treatment of Pain, FHU INOVPAIN, la Timone Hospital, AP-HM, Marseille, France.
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19
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Abstract
Migraine is a complex genetic brain disorder with an intricate pathogenesis and polymorphous clinical presentations, particularly in children. In this Perspective, we describe the different phenotypes of migraine in children, including conditions that have been referred to in the International Classification of Headache Disorders as "syndromes that may be related to migraine''. Evidence is presented for the integration of abdominal migraine, cyclical vomiting syndrome, benign paroxysmal vertigo, benign paroxysmal torticollis and infantile colic into the unified diagnosis of 'childhood migraine syndrome' on the basis of clinical and epidemiological characteristics, and shared inheritance. In our opinion, such integration will guide clinicians from specialities other than neurology to consider migraine in the assessment of children with these disorders, as well as stimulate research into the genetics, pathophysiology and clinical features of all disorders within the syndrome. A diagnosis of childhood migraine syndrome would also enable patients to benefit from inclusion in clinical trials of old and new migraine treatments, thus potentially increasing the number of treatment options available.
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20
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Nivatsi M, Aslanidou I, Mantadakis E. Highly effective use of aprepitant in an adolescent girl with severe cyclic vomiting syndrome. BMJ Case Rep 2021; 14:e241132. [PMID: 33664041 PMCID: PMC7934711 DOI: 10.1136/bcr-2020-241132] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/21/2021] [Indexed: 01/16/2023] Open
Abstract
Cyclic vomiting syndrome (CVS) is a debilitating functional gastrointestinal disorder. Diagnosis is based on the Rome III criteria. There are no evidence-based guidelines for the management of paediatric CVS, although ondansetron and antimigraine medications are frequently tried. We describe a 13-year-old adolescent girl with severe CVS and numerous hospital admissions for dehydration because of cyclic vomiting. She had failed oral ondansetron therapy. Oral aprepitant (125 mg in the first, and 85 mg on the second and third days), a neurokinin 1 receptor antagonist that has been approved for preventing chemotherapy-induced vomiting or postoperative emesis, was tried in our patient at home during the first prodromal signs of an upset stomach. She had a dramatic response to it, with no further episodes of vomiting since its start. There is an urgent need for randomised clinical studies to assess the efficacy of available treatment options, including aprepitant in patients with severe CVS.
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Affiliation(s)
- Maria Nivatsi
- Department of Paediatrics, University General Hospital of Alexandroupolis, Alexandroupolis, Thrace, Greece
| | - Ilona Aslanidou
- Department of Paediatrics, University General Hospital of Alexandroupolis, Alexandroupolis, Thrace, Greece
| | - Elpis Mantadakis
- Department of Paediatrics, Democritus University of Thrace Faculty of Medicine, Alexandroupolis, Thrace, Greece
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21
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Kovacic K, Li BUK. Cyclic vomiting syndrome: A narrative review and guide to management. Headache 2021; 61:231-243. [PMID: 33619730 DOI: 10.1111/head.14073] [Citation(s) in RCA: 16] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2020] [Revised: 01/03/2021] [Accepted: 01/06/2021] [Indexed: 12/18/2022]
Abstract
OBJECTIVES/BACKGROUND Cyclic vomiting syndrome (CVS) is a disabling disorder of gut-brain interaction manifested by stereotypical and severe episodes of nausea and vomiting. Prevalence data indicate that CVS affects 1-2% of children and there has been a recent dramatic rise in diagnosed adults. METHODS This narrative review summarizes relevant literature pertaining to pediatric and adult CVS and provides a guide to management based on extensive clinical experience. RESULTS More timely diagnosis is facilitated by an expert consensus diagnostic approach and limited testing. Some diagnostic tests of exclusion remain essential. These include an upper gastrointestinal (GI) contrast study to exclude intestinal malrotation and basic laboratory screening. An abdominal ultrasound is recommended to exclude renal hydronephrosis in children and biliary disease in adults. Exclusion of metabolic/genetic conditions is warranted in those with specific warning signs, presentation in infants/toddler age, and in those with refractory disease. In the absence of chronic GI symptoms, referral to a GI specialist for upper endoscopy is generally not necessary in children but recommended in adults. A large subset termed migraine-equivalent CVS display strong clinical and genetic features of migraine. A unifying pathophysiologic core concept involves neuronal hyperexcitability and aberrant central modulation of autonomic signals. This is coupled with multiple susceptibility factors including mitochondrial dysfunction/cellular energy deficits, a hyper-responsive hypothalamic-pituitary-adrenal axis and many comorbidities that increase vulnerability to triggering events. CVS episodes are frequently triggered by stressors and intercurrent illnesses. Lifestyle and non-pharmacological interventions thus play a pivotal role in successful management. Pharmacological therapies are categorized into abortive, supportive/rescue, and prophylactic treatments. The majority respond particularly well to migraine-focused treatment strategies. CONCLUSION Despite improved characterization and understanding, CVS remains classified as a functional disorder of brain-gut interaction that is often disjointly managed by generalists and subspecialists. Early recognition, evaluation, and management will facilitate care and improve outcomes. Further research into its natural history with common progression to migraine headaches, neuroendocrine mechanisms, and the pathophysiologic relation to migraine diathesis is much needed.
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Affiliation(s)
- Katja Kovacic
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI, USA
| | - B U K Li
- Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin, Milwaukee, WI, USA
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Raucci U, Borrelli O, Di Nardo G, Tambucci R, Pavone P, Salvatore S, Baldassarre ME, Cordelli DM, Falsaperla R, Felici E, Ferilli MAN, Grosso S, Mallardo S, Martinelli D, Quitadamo P, Pensabene L, Romano C, Savasta S, Spalice A, Strisciuglio C, Suppiej A, Valeriani M, Zenzeri L, Verrotti A, Staiano A, Villa MP, Ruggieri M, Striano P, Parisi P. Cyclic Vomiting Syndrome in Children. Front Neurol 2020; 11:583425. [PMID: 33224097 PMCID: PMC7667239 DOI: 10.3389/fneur.2020.583425] [Citation(s) in RCA: 28] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/14/2020] [Accepted: 09/14/2020] [Indexed: 12/13/2022] Open
Abstract
Cyclic Vomiting Syndrome (CVS) is an underdiagnosed episodic syndrome characterized by frequent hospitalizations, multiple comorbidities, and poor quality of life. It is often misdiagnosed due to the unappreciated pattern of recurrence and lack of confirmatory testing. CVS mainly occurs in pre-school or early school-age, but infants and elderly onset have been also described. The etiopathogenesis is largely unknown, but it is likely to be multifactorial. Recent evidence suggests that aberrant brain-gut pathways, mitochondrial enzymopathies, gastrointestinal motility disorders, calcium channel abnormalities, and hyperactivity of the hypothalamic-pituitary-adrenal axis in response to a triggering environmental stimulus are involved. CVS is characterized by acute, stereotyped and recurrent episodes of intense nausea and incoercible vomiting with predictable periodicity and return to baseline health between episodes. A distinction with other differential diagnoses is a challenge for clinicians. Although extensive and invasive investigations should be avoided, baseline testing toward identifying organic causes is recommended in all children with CVS. The management of CVS requires an individually tailored therapy. Management of acute phase is mainly based on supportive and symptomatic care. Early intervention with abortive agents during the brief prodromal phase can be used to attempt to terminate the attack. During the interictal period, non-pharmacologic measures as lifestyle changes and the use of reassurance and anticipatory guidance seem to be effective as a preventive treatment. The indication for prophylactic pharmacotherapy depends on attack intensity and severity, the impairment of the QoL and if attack treatments are ineffective or cause side effects. When children remain refractory to acute or prophylactic treatment, or the episode differs from previous ones, the clinician should consider the possibility of an underlying disease and further mono- or combination therapy and psychotherapy can be guided by accompanying comorbidities and specific sub-phenotype. This review was developed by a joint task force of the Italian Society of Pediatric Gastroenterology Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP) to identify relevant current issues and to propose future research directions on pediatric CVS.
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Affiliation(s)
- Umberto Raucci
- Pediatric Emergency Department, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Osvaldo Borrelli
- Division of Neurogastroenterology and Motility, Department of Pediatric Gastroenterology, University College London (UCL) Institute of Child Health and Great Ormond Street Hospital, London, United Kingdom
| | - Giovanni Di Nardo
- Chair of Pediatrics, Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy
| | - Renato Tambucci
- Digestive Endoscopy and Surgery Unit, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Piero Pavone
- Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
| | - Silvia Salvatore
- Pediatric Department, Ospedale “F. Del Ponte,” University of Insubria, Varese, Italy
| | | | | | - Raffaele Falsaperla
- Neonatal Intensive Care and Pediatric Units, S. Marco Hospital, Vittorio Emanuele Hospital, Catania, Italy
| | - Enrico Felici
- Unit of Pediatrics, The Children Hospital, Azienda Ospedaliera SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
| | - Michela Ada Noris Ferilli
- Division of Neurology, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Salvatore Grosso
- Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy
| | - Saverio Mallardo
- Pediatric Department, Santa Maria Goretti Hospital, Sapienza University of Rome, Latina, Italy
| | - Diego Martinelli
- Division of Metabolism, Department of Pediatric Specialties, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Paolo Quitadamo
- Department of Pediatrics, A.O.R.N. Santobono-Pausilipon, Naples, Italy
| | - Licia Pensabene
- Pediatric Unit, Department of Medical and Surgical Sciences, University “Magna Graecia” of Catanzaro, Catanzaro, Italy
| | - Claudio Romano
- Pediatric Gastroenterology Unit, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of Messina, Messina, Italy
| | | | - Alberto Spalice
- Child Neurology Division, Department of Pediatrics, “Sapienza,” University of Rome, Rome, Italy
| | - Caterina Strisciuglio
- Department of Woman, Child, General and Specialistic Surgery, University of Campania “Luigi Vanvitelli,” Naples, Italy
| | - Agnese Suppiej
- Pediatric Section, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
| | - Massimiliano Valeriani
- Division of Neurology, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy
| | - Letizia Zenzeri
- Emergency Pediatric Department, Santobono-Pausilipon Children's Hospital, Naples, Italy
| | - Alberto Verrotti
- Department of Pediatrics, University of L'Aquila, L'Aquila, Italy
| | - Annamaria Staiano
- Section of Pediatrics, Department of Translational Medical Science, “Federico II” University of Naples, Naples, Italy
| | - Maria Pia Villa
- Chair of Pediatrics, Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy
| | - Martino Ruggieri
- Unit of Rare Diseases of the Nervous System in Childhood, Section of Pediatrics and Child Neuropsychiatry, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
| | - Pasquale Striano
- Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genova, Italy
- Institute for Research, Hospitalization and Health Care (IRCCS) “G. Gaslini” Institute, Genova, Italy
| | - Pasquale Parisi
- Chair of Pediatrics, Department of Neuroscience, Mental Health and Sense Organs (NESMOS), Faculty of Medicine & Psychology, Sant'Andrea Hospital, Sapienza University of Rome, Rome, Italy
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Cyclic Vomiting Syndrome in a Medically Complex Patient: A Case Report. J Pediatr Health Care 2019; 33:711-717. [PMID: 31395300 DOI: 10.1016/j.pedhc.2019.06.009] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/12/2019] [Revised: 05/24/2019] [Accepted: 06/08/2019] [Indexed: 01/03/2023]
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Yu ES, Priyadharsini S S Y, Venkatesan T. Migraine, Cyclic Vomiting Syndrome, and Other Gastrointestinal Disorders. ACTA ACUST UNITED AC 2018; 16:511-527. [PMID: 30361855 DOI: 10.1007/s11938-018-0202-2] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
PURPOSE OF REVIEW Cyclic vomiting syndrome (CVS) is a chronic functional gastrointestinal disorder characterized by episodic nausea and vomiting and is diagnosed using Rome IV criteria. CVS is being recognized more frequently in adults with a prevalence of 2%. It is associated with several functional disorders like autonomic dysfunction, anxiety, and depression, but the strongest association is with migraine. We will elucidate the close relationship between migraine and CVS and briefly discuss its association with other gastrointestinal disorders. RECENT FINDINGS We highlight similarities in pathophysiology, clinical presentation, and response to medications between CVS and migraine (tricyclic antidepressants, triptans, antiepileptics). We also discuss novel therapies like CGRP inhibitors which are effective in migraine and have potential for adaptation in patients with CVS. Using migraine as a template should enable investigators to elucidate the mechanisms underlying this disorder, develop novel therapies, and direct future research in CVS.
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Affiliation(s)
- Elliot S Yu
- Department of Internal Medicine, The Hub for Collaborative Medicine, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA
| | - Yasodara Priyadharsini S S
- Division of Gastroenterology and Hepatology, The Hub for Collaborative Medicine, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA
| | - Thangam Venkatesan
- Division of Gastroenterology and Hepatology, The Hub for Collaborative Medicine, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA.
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Romano C, Dipasquale V, Rybak A, Comito D, Borrelli O. An overview of the clinical management of cyclic vomiting syndrome in childhood. Curr Med Res Opin 2018; 34:1785-1791. [PMID: 29484898 DOI: 10.1080/03007995.2018.1445983] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2017] [Revised: 02/21/2018] [Accepted: 02/23/2018] [Indexed: 01/03/2023]
Abstract
This narrative review provides an update on cyclic vomiting syndrome pathogenesis, diagnosis and management, based upon studies published after the 2008 North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) official recommendations. The review began with a comprehensive PubMed/Medline search for "cyclic vomiting syndrome", "periodic syndromes" and "pediatrics" from 2000 up to October 2017. Additional papers were identified by reviewing the re-ference lists of retrieved publications. Cyclic vomiting syndrome is a severe, debilitating disorder of the brain-gut axis with unclear pathogenesis, that significantly affects long-term quality of life of affected children and their families. The 2008 NASPGHAN recommendations defined the major clinical, diagnostic and therapeutic peculiarities. Over the last 10 years, advancements in pathogenesis and diagnostic criteria have been made, and new prophylactic and therapeutic strategies have been proposed. These aspects are discussed in this manuscript. For the pediatrician, the major aim is to have early clinical suspicion to avoid diagnostic delay and to start adequate, phase-related, symptom-tailored management.
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Affiliation(s)
- Claudio Romano
- a Division of Childhood Gastroenterology and Cystic Fibrosis, Department of Human Pathology in Adulthood and Childhood "G. Barresi" , University of Messina , Italy
| | - Valeria Dipasquale
- a Division of Childhood Gastroenterology and Cystic Fibrosis, Department of Human Pathology in Adulthood and Childhood "G. Barresi" , University of Messina , Italy
| | - Anna Rybak
- b Division of Neurogastroenterology and Motility, Department of Gastroenterology , Great Ormond Street Hospital and UCL , London , UK
| | - Donatella Comito
- a Division of Childhood Gastroenterology and Cystic Fibrosis, Department of Human Pathology in Adulthood and Childhood "G. Barresi" , University of Messina , Italy
| | - Osvaldo Borrelli
- b Division of Neurogastroenterology and Motility, Department of Gastroenterology , Great Ormond Street Hospital and UCL , London , UK
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26
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Singh P, Arora A, Strand TA, Leffler DA, Catassi C, Green PH, Kelly CP, Ahuja V, Makharia GK. Global Prevalence of Celiac Disease: Systematic Review and Meta-analysis. Clin Gastroenterol Hepatol 2018; 16:823-836.e2. [PMID: 29551598 DOI: 10.1016/j.cgh.2017.06.037] [Citation(s) in RCA: 923] [Impact Index Per Article: 131.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/01/2017] [Revised: 06/05/2017] [Accepted: 06/21/2017] [Indexed: 02/06/2023]
Abstract
BACKGROUND & AIMS Celiac disease is a major public health problem worldwide. Although initially it was reported from countries with predominant Caucasian populations, it now has been reported from other parts of the world. The exact global prevalence of celiac disease is not known. We conducted a systematic review and meta-analysis to estimate the global prevalence of celiac disease. METHODS We searched Medline, PubMed, and EMBASE for the keywords celiac disease, celiac, celiac disease, tissue transglutaminase antibody, anti-endomysium antibody, endomysial antibody, and prevalence for studies published from January 1991 through March 2016. Each article was cross-referenced with the words Asia, Europe, Africa, South America, North America, and Australia. The diagnosis of celiac disease was based on European Society of Pediatric Gastroenterology, Hepatology, and Nutrition guidelines. Of 3843 articles, 96 articles were included in the final analysis. RESULTS The pooled global prevalence of celiac disease was 1.4% (95% confidence interval, 1.1%-1.7%) in 275,818 individuals, based on positive results from tests for anti-tissue transglutaminase and/or anti-endomysial antibodies (called seroprevalence). The pooled global prevalence of biopsy-confirmed celiac disease was 0.7% (95% confidence interval, 0.5%-0.9%) in 138,792 individuals. The prevalence values for celiac disease were 0.4% in South America, 0.5% in Africa and North America, 0.6% in Asia, and 0.8% in Europe and Oceania; the prevalence was higher in female vs male individuals (0.6% vs 0.4%; P < .001). The prevalence of celiac disease was significantly greater in children than adults (0.9% vs 0.5%; P < .001). CONCLUSIONS In a systematic review and meta-analysis, we found celiac disease to be reported worldwide. The prevalence of celiac disease based on serologic test results is 1.4% and based on biopsy results is 0.7%. The prevalence of celiac disease varies with sex, age, and location. There is a need for population-based prevalence studies in many countries.
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Affiliation(s)
- Prashant Singh
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Boston, Massachusetts
| | | | - Tor A Strand
- Innlandet Hospital Trust, Lillehammer, Norway; Centre for International Health, University of Bergen, Bergen, Norway
| | - Daniel A Leffler
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Boston, Massachusetts; Gastroenterology Research and Development, Takeda Pharmaceuticals Inc, Cambridge, MA
| | - Carlo Catassi
- Department of Pediatrics, Università Politecnica delle Marche, Ancona, Italy
| | - Peter H Green
- Department of Medicine, Columbia University Medical Center, New York, New York; USA Celiac Disease Center, Columbia University Medical Center, New York, New York
| | - Ciaran P Kelly
- Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Boston, Massachusetts
| | - Vineet Ahuja
- Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi, India
| | - Govind K Makharia
- Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi, India.
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27
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Sagar RC, Sood R, Gracie DJ, Gold MJ, To N, Law GR, Ford AC. Cyclic vomiting syndrome is a prevalent and under-recognized condition in the gastroenterology outpatient clinic. Neurogastroenterol Motil 2018; 30. [PMID: 28745840 DOI: 10.1111/nmo.13174] [Citation(s) in RCA: 37] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2017] [Accepted: 06/30/2017] [Indexed: 02/08/2023]
Abstract
BACKGROUND Cyclic vomiting syndrome (CVS) is a functional gastrointestinal disorder (FGID) characterized by intermittent episodes of nausea and vomiting. Our aim was to report its prevalence and associated features. METHODS Data concerning demographics, symptoms, and psychiatric comorbidity were collected. Symptoms compatible with CVS were classified as per Rome III criteria. We recorded whether a diagnosis of CVS was considered in patients after negative investigation. We compared demographics and association with other FGIDs in patients with and without CVS. KEY RESULTS 920 of 1002 patients provided data. Of the 920 patients, 112 (12.2%) had symptoms compatible with CVS. Thirteen (11.6%) of these had an organic cause for their symptoms, but 99 patients (88.4%) were deemed to have CVS (prevalence=10.8%). Organic causes for symptoms compatible with CVS included gastroparesis, large hiatus hernia, achalasia, and small bowel obstruction. Only 39.4% of patients with CVS were asked about vomiting symptoms at their initial consultation, and a diagnosis of CVS was considered in only four (4.0%) of the 99 patients. CVS was associated with younger age, tobacco smoking, never having married, psychiatric comorbidity, and presence of symptoms compatible with other FGIDs (P≤.01). CONCLUSIONS AND INFERENCES Prevalence of CVS in this outpatient gastroenterology adult population was 10.8%. Identified associations included younger age, tobacco smoking, psychiatric comorbidity, and symptoms compatible with other FGIDs. The condition was considered as a possible diagnosis in <5% of patients who met the diagnostic criteria.
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Affiliation(s)
- R C Sagar
- Leeds Gastroenterology Institute, St James's University Hospital, Leeds, UK.,Leeds Institute of Biomedical and Clinical Sciences, University of Leeds, Leeds, UK
| | - R Sood
- Leeds Gastroenterology Institute, St James's University Hospital, Leeds, UK.,Leeds Institute of Biomedical and Clinical Sciences, University of Leeds, Leeds, UK
| | - D J Gracie
- Leeds Gastroenterology Institute, St James's University Hospital, Leeds, UK.,Leeds Institute of Biomedical and Clinical Sciences, University of Leeds, Leeds, UK
| | - M J Gold
- School of Medicine, University of Leeds, Leeds, UK
| | - N To
- Leeds Gastroenterology Institute, St James's University Hospital, Leeds, UK.,Leeds Institute of Biomedical and Clinical Sciences, University of Leeds, Leeds, UK
| | - G R Law
- School of Health and Social Care, University of Lincoln, Lincoln, UK
| | - A C Ford
- Leeds Gastroenterology Institute, St James's University Hospital, Leeds, UK.,Leeds Institute of Biomedical and Clinical Sciences, University of Leeds, Leeds, UK
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28
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Badihian N, Saneian H, Badihian S, Yaghini O. Prophylactic Therapy of Cyclic Vomiting Syndrome in Children: Comparison of Amitriptyline and Cyproheptadine: A Randomized Clinical Trial. Am J Gastroenterol 2018; 113:135-140. [PMID: 28719594 DOI: 10.1038/ajg.2017.194] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2016] [Accepted: 05/21/2017] [Indexed: 12/11/2022]
Abstract
OBJECTIVES Cyclic vomiting syndrome (CVS) is a common functional gastrointestinal disorder characterized by recurrent episodes of nausea and vomiting. There is no definite treatment for the condition, although some medications are recommended. We aimed to compare the efficacy of amitriptyline and cyproheptadine in prophylactic therapy of CVS. METHODS This is a single-blinded randomized clinical trial conducted during 2015-2016 in Isfahan, Iran. Sixty-four children who were 3-15 years old, with a diagnosis of CVS (based on Rome III criteria), were included in the study and were randomly divided into two groups of amitriptyline and cyproheptadine. They were followed for 6 months, looking for frequency and duration of attacks as the primary outcome. RESULTS The mean monthly frequency of attacks in the last 2 months of the study in the amitriptyline and cyproheptadine group were 0.38±0.55 and 0.59±0.71, respectively (P-value=0.197), after intervention. The mean duration of attacks between amitriptyline and cyproheptadine group were 1.41±2.86 and 1.81±2.22 h, respectively (P-value=0.212). In the amitriptyline group 65.6% of patients reported 100% remission, whereas in the cyproheptadine group 50% reported full remission (P-value=0.206). CONCLUSIONS There was no superiority of one of the medications over the other. We did not find any age-related effect on the efficacy of these medications as well.
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Affiliation(s)
- Negin Badihian
- Faculty of Medicine, Najafabad Branch, Islamic Azad University, Najafabad, Iran
| | - Hossein Saneian
- Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran
| | - Shervin Badihian
- Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran.,Students' Research Center, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
| | - Omid Yaghini
- Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran
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Bouzios I, Chouliaras G, Chrousos GP, Roma E, Gemou-Engesaeth V. Functional gastrointestinal disorders in Greek Children based on ROME III criteria: identifying the child at risk. Neurogastroenterol Motil 2017; 29. [PMID: 27679978 DOI: 10.1111/nmo.12951] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2016] [Accepted: 08/25/2016] [Indexed: 12/12/2022]
Abstract
BACKGROUND Functional gastrointestinal disorders (FGIDs) are a common, diverse group of disorders of unknown etiology, resulting in significant socieconomic burden. In this study, we aimed to assess the prevalence of FGIDs in children aged 6-18 years and examine their association with various demographic and socioeconomic parameters. METHODS This was a school-based, cross-sectional study approved by the relevant government authorities. Informed consent was obtained by the legal representatives of all children who participated. Diagnoses of FGIDs were based on the Greek official translation of the ROME-III questionnaire. Demographic and socioeconomic information were also collected. KEY RESULTS A total of 1588 children (51.8% females, mean age: 12.9±2.8 years) were included. The overall prevalence of any-FGID was 23.1% (95% CI: 21.1-25.2). The most common FGIDs were functional constipation, n=231 (13.9%), abdominal migraine, n=84 (5.6%), aerophagia, n=58 (3.5%), and irritable bowel syndrome, n=48 (3.0%). Multiple logistic regression analysis on the probability of any-FGID identified physical exercise, TV-exposure, victimization, gender, parental educational level, number of children at home and number of adults at home as significant covariates for any-FGID in the final model. CONCLUSIONS AND INFERENCES FGIDs affect approximately 1 in 4 school-aged children in Greece. The following characteristics are associated with a higher probability of any-FGID: female gender, living in a non-nuclear household, victimization, lower parental education level, infrequent physical activity, and high television exposure.
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Affiliation(s)
- I Bouzios
- 1st Department of Pediatrics, University of Athens, "Aghia Sophia" Children's Hospital, Athens, Greece
| | - G Chouliaras
- 1st Department of Pediatrics, University of Athens, "Aghia Sophia" Children's Hospital, Athens, Greece
| | - G P Chrousos
- 1st Department of Pediatrics, University of Athens, "Aghia Sophia" Children's Hospital, Athens, Greece
| | - E Roma
- 1st Department of Pediatrics, University of Athens, "Aghia Sophia" Children's Hospital, Athens, Greece
| | - V Gemou-Engesaeth
- 1st Department of Pediatrics, University of Athens, "Aghia Sophia" Children's Hospital, Athens, Greece
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30
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Sezer OB, Sezer T. A New Approach to the Prophylaxis of Cyclic Vomiting: Topiramate. J Neurogastroenterol Motil 2016; 22:656-660. [PMID: 27302967 PMCID: PMC5056575 DOI: 10.5056/jnm16035] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/07/2016] [Revised: 04/23/2016] [Accepted: 04/27/2016] [Indexed: 11/20/2022] Open
Abstract
BACKGROUND/AIMS The aim of this study was to compare the efficacy and tolerability of topiramate and propranolol in preventing pediatric cyclic vomiting syndrome. METHODS A retrospective medical-record review of patients who underwent prophylaxis after receiving a diagnosis of cyclic vomiting syndrome was performed. Patients who completed at least 12 months of treatment were included in the analysis. Responder rate, and adverseevent rates were also calculated from all patients. Response to treatment was assessed as the total number of vomiting attacks per year. Patients in whom the frequency of vomiting attack reduced greater or equal to 50% were defined as responders, and the remaining patients were classified as nonresponders. RESULTS A total of 38 patients who were treated prophylactically with either topiramate (16 patients) or propranolol (22 patients) were identified. Fifty-nine percent of the patients in the propranolol group and 81% of the patients in the topiramate group reported freedom from attacks. A decrease of more than 50% in attacks per year occurred in 23% of patients in the propranolol group and 13% of patients in the topiramate group. The responder rates were 81% for propranolol group and 94% for topiramate group (P = 0.001). Despite minor adverse effects (drowsiness, nervousness, and dizziness) observed in a few patients, the adverse event rates were not significantly different between the 2 groups (P = 0.240). CONCLUSIONS The efficacy of topiramate was superior to propranolol for the prophylaxis of pediatric cyclic vomiting syndrome.
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Affiliation(s)
- Oya B Sezer
- Department of Pediatric Gastroenterology and Nutrition, Kecioren Training and Research Hospital, Ankara,
Turkey
| | - Taner Sezer
- Department of Pediatric Neurology, Baskent University Faculty of Medicine, Ankara,
Turkey
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31
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Anxiety and physiological responses to the Trier Social Stress Test for Children in adolescents with cyclic vomiting syndrome. Auton Neurosci 2016; 202:79-85. [PMID: 27554769 DOI: 10.1016/j.autneu.2016.08.010] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2016] [Revised: 07/13/2016] [Accepted: 08/14/2016] [Indexed: 01/01/2023]
Abstract
This study compared anxiety and physiological responses during the Trier Social Stress Test for Children (TSST-C) in adolescents. 38 subjects (26 females) were enrolled: 11 cyclic vomiting syndrome (CVS), 11 anxiety, and 16 controls. Salivary cortisol, α-amylase and heart rate variability (HRV) were assessed during the TSST-C. Anxiety was measured by the Screen for Childhood Anxiety Related Emotional Disorders (SCARED), Anxiety Disorders Interview Schedule, and State-Trait Anxiety Inventory for Children (STAI-C). 11 anxiety and 7 CVS subjects had ≥1 anxiety disorder. 82% in the anxiety and CVS groups met criteria for an anxiety disorder on the SCARED. Combining groups, cortisol increased from baseline to recovery during the TSST-C (p=0.0004) and the stressor to recovery (p=0.005). α-amylase did not differ during the TSST-C for the total sample, but increased for anxiety compared to controls from baseline to recovery (p=0.01). HRV decreased during the stressor (p=0.0001) and increased at recovery (p=0.004). No associations were found between biomarkers and trait anxiety. Associations were found between baseline HRV and pre-test state anxiety (r=-0.406, p=0.012) and between recovery HRV and post-test state anxiety (r=-0.501, p=0.002) for the total sample. Anxiety is prevalent in CVS warranting screening. HRV may serve as a biomarker for evaluating stress as a potential trigger for CVS episodes. State but not trait anxiety was associated with changes in HRV, suggesting acute anxiety may be more relevant in linking stress and CVS episodes.
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32
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Nagarwala J, Dev S, Markin A. The Vomiting Patient: Small Bowel Obstruction, Cyclic Vomiting, and Gastroparesis. Emerg Med Clin North Am 2016; 34:271-91. [PMID: 27133244 DOI: 10.1016/j.emc.2015.12.005] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/20/2023]
Abstract
Vomiting and abdominal pain are common in patients in the emergency department. This article focuses on small bowel obstruction (SBO), cyclic vomiting, and gastroparesis. Through early diagnosis and appropriate management, the morbidity and mortality associated with SBOs can be significantly reduced. Management of SBOs involves correction of physiologic and electrolyte disturbances, bowel rest and removing the source of the obstruction. Treatment of acute cyclic vomiting is primarily directed at symptom control, volume and electrolyte repletion, and appropriate specialist follow-up. The mainstay of therapy for gastroparesis is metoclopramide.
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Affiliation(s)
- Jumana Nagarwala
- Department of Emergency Medicine, Henry Ford Hospital, 2799 West Grand Boulevard, CFP-258, Detroit, MI 48202, USA; Department of Emergency Medicine, Wayne State University School of Medicine, Detroit, MI, USA.
| | - Sharmistha Dev
- Departments of Emergency Medicine and Internal Medicine, University of Michigan, Ann Arbor, MI, USA
| | - Abraham Markin
- Department of Emergency Medicine, Henry Ford Hospital, 2799 West Grand Boulevard, CFP-258, Detroit, MI 48202, USA; Department of Internal Medicine, Henry Ford Hospital, 2799 West Grand Boulevard, Detroit, MI 48202, USA
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Lebron D, Vasconcellos E. The Episodic Syndromes That Maybe Associated with Migraines. Semin Pediatr Neurol 2016; 23:6-10. [PMID: 27017014 DOI: 10.1016/j.spen.2016.01.003] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
The "childhood periodic syndromes" have been renamed "the episodic syndromes that maybe associated with migraines". These syndromes were initially considered precursors of migraines that only occurred in childhood; however recent literature suggests that the episodic syndromes can occur in adults with known migraine and does not necessarily present as a precursor. This review article discusses the recent literature regarding the episodic syndromes and potential treatments. These disorders are seen by multiple subspecialists, therefore it is important to recognize and use the same definitions, criteria and nomenclature. A collaborative and multidisciplinary approach is critical to characterize, manage and potentially improve outcomes.
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Affiliation(s)
- Diana Lebron
- Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN.
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Tan MLN, Liwanag MJ, Quak SH. Cyclical vomiting syndrome: Recognition, assessment and management. World J Clin Pediatr 2014; 3:54-58. [PMID: 25254185 PMCID: PMC4162439 DOI: 10.5409/wjcp.v3.i3.54] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2014] [Revised: 06/16/2014] [Accepted: 07/14/2014] [Indexed: 02/06/2023] Open
Abstract
Cyclical vomiting syndrome (CVS) is a functional, debilitating disorder of childhood frequently leading to hospitalization. Affected children usually experience a stereotypical pattern of vomiting though it may vary between different individuals. The vomiting is intense often bilious, and accompanied by disabling nausea. Identifiable precipitating factors for CVS include psychosocial stressors, infections, lack of sleep and occasionally even food triggers. Often, it may be difficult to distinguish episodes of CVS from other causes of acute abdomen and altered consciousness. Thus, the diagnosis of CVS remains largely one of exclusion. Investigations routinely done during the work-up of a child with suspected CVS include both blood and imaging modalities. Plasma lactate, ammonia, amino acid and acylcarnitine profiles as well as urine organic acid profile are indicated to exclude inborn errors of metabolism. The treatment remains challenging and targeted at prevention or shortening of the attacks and can be considered as abortive, supportive and prophylactic. Use of non-pharmacological therapy is also part of the management of CVS. The prognosis of CVS is variable. More insight into the pathogenesis of this disorder as well as role of non-pharmacological therapy is needed.
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Approach to the diagnosis and treatment of cyclic vomiting syndrome: a large single-center experience with 106 patients. Pediatr Neurol 2014; 50:569-73. [PMID: 24842256 DOI: 10.1016/j.pediatrneurol.2014.02.009] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/18/2013] [Revised: 02/05/2014] [Accepted: 02/12/2014] [Indexed: 12/27/2022]
Abstract
BACKGROUND Cyclic vomiting syndrome is characterized by repeated, stereotypical vomiting episodes. The diagnosis is made by exclusion of other organic diseases, which can lead to extensive testing. It has been suggested that these patients can have mitochondrial dysfunction. The aim of the study was to examine the evaluation of our cyclic vomiting patients and to determine whether they had associated, undiagnosed metabolic abnormalities. METHODS This retrospective study included 106 patients aged <21 years at diagnosis. Information regarding medical history, laboratory, and imaging studies were collected. Metabolic studies in plasma and urine were obtained when patients were well and when patients were in a vomiting cycle, including plasma amino acids, acylcarnitines, and urine organic acids. RESULTS The mean age at diagnosis was 8.9 ± 5.0 years. Neuroimaging revealed previously unknown intracranial abnormalities in <10% of patients, none of whom explained the vomiting signs. Abdominal ultrasounds revealed abnormalities in 15% of patients during an acute episode and 7% of patients when well. Sixty-one patients had an upper gastrointestinal series, all of which were normal. A total of 92% of patients had laboratory testing with 38% indicating abnormalities possibly suggesting mitochondrial dysfunction. CONCLUSIONS This large, single-center study further evaluated the need for more focused evaluation in patients with suspected cyclic vomiting syndrome. Thirty-eight percent of our patients had abnormalities in blood and/or urine suggesting mitochondrial dysfunction, which requires more detailed investigation in the future.
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Pacheva IH, Ivanov IS. Migraine variants--occurrence in pediatric neurology practice. Clin Neurol Neurosurg 2013; 115:1775-83. [PMID: 23688445 DOI: 10.1016/j.clineuro.2013.04.012] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2013] [Revised: 04/01/2013] [Accepted: 04/15/2013] [Indexed: 12/22/2022]
Abstract
UNLABELLED Migraine is common in pediatric neurology practice, while migraine variants are rare and pose diagnostic problems. OBJECTIVE The aim was to establish the occurrence of migraine variants in pediatric neurology practice and among migraine, and to discuss their presentation. PATIENTS AND METHODS The files of 2509 newly diagnosed patients, aged 0-18 years, treated as in- and out-patients in the Neuropediatric Ward at the Plovdiv Medical University Hospital between 2002 and 2006 were examined retrospectively. Migraine forms were diagnosed according to ICHD-II. Benign paroxysmal torticolis and alternating hemiplegia of childhood were also accepted as migraine variants according to proposed diagnostic criteria in the appendix of ICHD-II. Some specific forms like acute confusional migraine (ACM), Alice in wonderland syndrome (AWS), ophthalmoplegic migraine were also diagnosed although not included as migraine variants in the ICHD-II classification. RESULTS 111 patients met diagnostic criteria for migraine. Patients with migraine variants comprised 24.3% of migrainous cases. Basilar type migraine was the most common (6.3% of all migrainous patients), followed by benign paroxysmal vertigo (5.4%), hemiplegic migraine (3.6%), ACM (2.7%), benign paroxysmal torticolis (2.7%), typical aura without headache (1.8%), abdominal migraine (1.8%), AWS (0.9%), ophthalmoplegic migraine (0.9%) and cyclical vomiting (0.9%). Alternating hemiplegia of childhood and retinal migraine was not found. Some patients either presented or were classified as different migraine variants. CONCLUSION Basilar type migraine was the most common migraine variant. ACM and AWS should be regarded as distinct entities in the ICHD as migraine with complex aura. Benign paroxysmal torticollis also deserves its place as a migraine variant. Cases of ophthalmoplegic migraine with spontaneous remission and no cranial nerve enhancement on MRI should be considered as migraine form. Analyzing migraine variants will contribute to better awareness and adequate diagnosis.
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Affiliation(s)
- Iliyana H Pacheva
- Department of Pediatrics and Medical Genetics, Medical University, Plovdiv, Bulgaria.
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Chew S, Balasubramanian R, Chan WM, Kang PB, Andrews C, Webb BD, MacKinnon SE, Oystreck DT, Rankin J, Crawford TO, Geraghty M, Pomeroy SL, Crowley WF, Jabs EW, Hunter DG, Grant PE, Engle EC. A novel syndrome caused by the E410K amino acid substitution in the neuronal β-tubulin isotype 3. Brain 2013; 136:522-35. [PMID: 23378218 PMCID: PMC3572929 DOI: 10.1093/brain/aws345] [Citation(s) in RCA: 93] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2012] [Revised: 10/29/2012] [Accepted: 11/09/2012] [Indexed: 01/31/2023] Open
Abstract
Missense mutations in TUBB3, the gene that encodes the neuronal-specific protein β-tubulin isotype 3, can cause isolated or syndromic congenital fibrosis of the extraocular muscles, a form of complex congenital strabismus characterized by cranial nerve misguidance. One of the eight TUBB3 mutations reported to cause congenital fibrosis of the extraocular muscles, c.1228G>A results in a TUBB3 E410K amino acid substitution that directly alters a kinesin motor protein binding site. We report the detailed phenotypes of eight unrelated individuals who harbour this de novo mutation, and thus define the 'TUBB3 E410K syndrome'. Individuals harbouring this mutation were previously reported to have congenital fibrosis of the extraocular muscles, facial weakness, developmental delay and possible peripheral neuropathy. We now confirm by electrophysiology that a progressive sensorimotor polyneuropathy does indeed segregate with the mutation, and expand the TUBB3 E410K phenotype to include Kallmann syndrome (hypogonadotropic hypogonadism and anosmia), stereotyped midface hypoplasia, intellectual disabilities and, in some cases, vocal cord paralysis, tracheomalacia and cyclic vomiting. Neuroimaging reveals a thin corpus callosum and anterior commissure, and hypoplastic to absent olfactory sulci, olfactory bulbs and oculomotor and facial nerves, which support underlying abnormalities in axon guidance and maintenance. Thus, the E410K substitution defines a new genetic aetiology for Moebius syndrome, Kallmann syndrome and cyclic vomiting. Moreover, the c.1228G>A mutation was absent in DNA from ∼600 individuals who had either Kallmann syndrome or isolated or syndromic ocular and/or facial dysmotility disorders, but who did not have the combined features of the TUBB3 E410K syndrome, highlighting the specificity of this phenotype-genotype correlation. The definition of the TUBB3 E410K syndrome will allow clinicians to identify affected individuals and predict the mutation based on clinical features alone.
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Affiliation(s)
- Sheena Chew
- 1 Department of Neurology, Boston Children’s Hospital, Boston, MA 02115, USA
- 2 FM Kirby Neurobiology Centre, Boston Children’s Hospital, Boston, MA 02115, USA
- 3 Department of Neurology, Harvard Medical School, Boston, MA 02115, USA
- 4 Harvard Medical School, Boston, MA 02115, USA
- 5 Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA
| | - Ravikumar Balasubramanian
- 6 Harvard Reproductive Endocrine Sciences Centre, Massachusetts General Hospital, Boston, MA 02114, USA
- 7 The Reproductive Endocrine Unit of the Department of Medicine, Massachusetts General Hospital, Boston, MA 02114, USA
| | - Wai-Man Chan
- 1 Department of Neurology, Boston Children’s Hospital, Boston, MA 02115, USA
- 2 FM Kirby Neurobiology Centre, Boston Children’s Hospital, Boston, MA 02115, USA
- 5 Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA
- 8 Program in Genomics, Boston Children’s Hospital, Boston, MA 02115, USA
| | - Peter B. Kang
- 1 Department of Neurology, Boston Children’s Hospital, Boston, MA 02115, USA
- 3 Department of Neurology, Harvard Medical School, Boston, MA 02115, USA
| | - Caroline Andrews
- 1 Department of Neurology, Boston Children’s Hospital, Boston, MA 02115, USA
- 2 FM Kirby Neurobiology Centre, Boston Children’s Hospital, Boston, MA 02115, USA
- 3 Department of Neurology, Harvard Medical School, Boston, MA 02115, USA
- 5 Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA
- 8 Program in Genomics, Boston Children’s Hospital, Boston, MA 02115, USA
| | - Bryn D. Webb
- 9 Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine, New York, NY, 10029, USA
- 10 Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, 10029, USA
| | - Sarah E. MacKinnon
- 11 Department of Ophthalmology, Boston Children’s Hospital, Boston, MA 02115, USA
| | - Darren T. Oystreck
- 11 Department of Ophthalmology, Boston Children’s Hospital, Boston, MA 02115, USA
| | - Jessica Rankin
- 1 Department of Neurology, Boston Children’s Hospital, Boston, MA 02115, USA
- 4 Harvard Medical School, Boston, MA 02115, USA
| | - Thomas O. Crawford
- 12 Department of Neurology, Johns Hopkins Hospital, Baltimore, MA 21287, USA
| | - Michael Geraghty
- 13 Department of Paediatrics, Children’s Hospital Eastern Ontario, University of Ottawa, Canada
| | - Scott L. Pomeroy
- 1 Department of Neurology, Boston Children’s Hospital, Boston, MA 02115, USA
- 2 FM Kirby Neurobiology Centre, Boston Children’s Hospital, Boston, MA 02115, USA
- 3 Department of Neurology, Harvard Medical School, Boston, MA 02115, USA
- 14 Broad Institute of MIT and Harvard, Cambridge, MA, 02142, USA
| | - William F. Crowley
- 6 Harvard Reproductive Endocrine Sciences Centre, Massachusetts General Hospital, Boston, MA 02114, USA
- 7 The Reproductive Endocrine Unit of the Department of Medicine, Massachusetts General Hospital, Boston, MA 02114, USA
| | - Ethylin Wang Jabs
- 9 Department of Genetics and Genomic Sciences, Mount Sinai School of Medicine, New York, NY, 10029, USA
- 10 Department of Pediatrics, Mount Sinai School of Medicine, New York, NY, 10029, USA
- 15 Department of Developmental and Regenerative Biology, Mount Sinai School of Medicine, New York, NY, 10029, USA
| | - David G. Hunter
- 11 Department of Ophthalmology, Boston Children’s Hospital, Boston, MA 02115, USA
| | - Patricia E. Grant
- 16 Department of Radiology, Boston Children’s Hospital, Boston, MA 02115, USA
| | - Elizabeth C. Engle
- 1 Department of Neurology, Boston Children’s Hospital, Boston, MA 02115, USA
- 2 FM Kirby Neurobiology Centre, Boston Children’s Hospital, Boston, MA 02115, USA
- 3 Department of Neurology, Harvard Medical School, Boston, MA 02115, USA
- 5 Howard Hughes Medical Institute, Chevy Chase, MD 20815, USA
- 8 Program in Genomics, Boston Children’s Hospital, Boston, MA 02115, USA
- 11 Department of Ophthalmology, Boston Children’s Hospital, Boston, MA 02115, USA
- 14 Broad Institute of MIT and Harvard, Cambridge, MA, 02142, USA
- 17 The Manton Centre for Orphan Disease Research, Boston Children’s Hospital, Boston, MA 02115, USA
- 18 Department of Medicine (Genetics), Boston Children’s Hospital, Boston, MA 02115, USA
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Lee LYW, Abbott L, Mahlangu B, Moodie SJ, Anderson S. The management of cyclic vomiting syndrome: a systematic review. Eur J Gastroenterol Hepatol 2012; 24:1001-6. [PMID: 22634989 DOI: 10.1097/meg.0b013e328355638f] [Citation(s) in RCA: 71] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Cyclic vomiting syndrome (CVS) is a rare abnormality of the neuroendocrine system that affects 2% of children. It is a frequently missed diagnosis in the emergency department and may require a number of emergency department visits before the diagnosis is made. The objectives of this review are to identify the clinical features that suggest a diagnosis of CVS and to review the literature on its management. The MEDLINE and EMBASE databases were searched from January 1948 to October 2011 using the keywords 'Cyclic' or 'Vomiting'. Papers were excluded if they did not follow the consensus guidelines or if they were case reports. This review analysed 1093 cases of cyclic vomiting in 25 papers that fulfilled the inclusion criteria. All except one paper were retrospective studies. The size of these cohort studies ranged from three to 181 patients, with a mean patient size of 29. This review found that over 40% of patients have headaches/migraines, with associated anxiety and depression in ≈ 30% of cases. There is a family history of headaches/migraines in 38.9%, and this association was much stronger in the adult CVS cohort compared with the paediatric cohort. Compared with paediatric CVS, adults have a longer duration of attacks and they occur more frequently (5.9 vs. 3.4 days, 14.4 vs. 9.6 episodes/year). Limited data are available on the treatment of the acute phase of CVS, but in adults, sumatriptan has been shown to be effective. For prophylactic treatment, tricyclics are effective in both adult and paediatric CVS, with a clinical response in 75.5 and 67.6% of patients, respectively, in nonplacebo-controlled cohort studies. Furthermore, propranolol has been shown to be useful in children. CVS is an intractable illness with a major impact on the patient's quality of life. There is a long duration between the onset of symptoms and the diagnosis of the condition. There is a high association with headaches/migraines and anxiety/depression. The symptoms are more severe in adult-onset CVS. Tricyclic antidepressants have good efficacy in reducing the frequency/duration or the intensity of attacks. There is limited evidence on the acute management of CVS.
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Affiliation(s)
- Lennard Y W Lee
- Epsom and St Helier University Hospitals NHS Trust, London, UK.
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Cyclic Vomiting Syndrome in Taiwanese Children. J Formos Med Assoc 2011; 110:14-8. [DOI: 10.1016/s0929-6646(11)60003-x] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2009] [Revised: 12/01/2009] [Accepted: 02/01/2010] [Indexed: 01/10/2023] Open
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Yang HR. Recent concepts on cyclic vomiting syndrome in children. J Neurogastroenterol Motil 2010; 16:139-47. [PMID: 20535344 PMCID: PMC2879837 DOI: 10.5056/jnm.2010.16.2.139] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2010] [Accepted: 03/26/2010] [Indexed: 11/20/2022] Open
Abstract
Cyclic vomiting syndrome (CVS) is a functional gastrointestinal disorder that can occur in both children and adults. Clinical courses of CVS manifesting recurrent severe vomiting episodes and interval illness may affect the long-term quality of life in children with CVS. Therefore, we should be careful in accessing a patient suggestive of CVS. Accurate diagnosis based on diagnostic criteria for CVS and the exclusion from other organic diseases mimicking clinical manifestations of cyclic vomiting is absolutely required. In patients diagnosed as CVS, optimal therapy should be performed to improve symptoms and to reduce complications in prodromal phase and emetic phase, and long-term prophylactic therapy should be tried to prevent the development of vomiting episodes. The identification of triggering factors which induce vomiting episodes might be helpful in preventing vomiting attacks. Systematic approach should be recommended to improve clinical outcome of CVS.
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Affiliation(s)
- Hye Ran Yang
- Department of Pediatrics, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seoul, Korea
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A comparison of dysautonomias comorbid with cyclic vomiting syndrome and with migraine. Gastroenterol Res Pract 2010; 2009:701019. [PMID: 20111731 PMCID: PMC2810453 DOI: 10.1155/2009/701019] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/17/2009] [Revised: 10/16/2009] [Accepted: 10/18/2009] [Indexed: 11/17/2022] Open
Abstract
Cyclic vomiting syndrome (CVS) shares many features with migraine headache, including auras, photophobia, and antimigrainous treatment response being traditionally viewed as a migraine variant. Aims. To determine whether CVS is associated with the same disorders as migraine headache, and compare these associations to those in healthy control subjects. Methods. Cross-sectional study of patients utilizing the ODYSA instrument, evaluating the probability of 12 functional/autonomic diagnoses, CVS, migraine, orthostatic intolerance (OI), reflex syncope, interstitial cystitis, Raynaud's syndrome, complex regional pain syndrome (CRPS), irritable bowel syndrome, functional dyspepsia, functional abdominal pain, fibromyalgia, and chronic fatigue syndrome. Control subjects were age-matched gender-matched friends. Patients had to fulfill criteria for CVS or migraine, while control subjects could not. Results. 103 subjects were studied, 21 with CVS, 46 with migraine and 36 healthy controls. CVS and migraine did not differ in the relative frequencies of fibromyalgia, OI, syncope, and functional dyspepsia. However, CVS patients did demonstrate a significantly elevated frequency of CRPS. Conclusions. Although CVS and migraine clearly share many of the same comorbidities, they do differ in one important association, suggesting that they may not be identical in pathophysiology. Since OI is common in CVS, treatment strategies could also target this abnormality.
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