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Jablonski SA, Mazepa ASW, Tolbert MK. Use of octreotide for the treatment of protein-losing enteropathy in dogs: Retrospective study of 18 cases. J Vet Intern Med 2024; 38:145-151. [PMID: 38038236 PMCID: PMC10800202 DOI: 10.1111/jvim.16966] [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: 06/12/2023] [Accepted: 11/17/2023] [Indexed: 12/02/2023] Open
Abstract
BACKGROUND More than 50% of dogs with protein-losing enteropathy (PLE) fail to respond to standard therapies. Octreotide, a somatostatin analogue, is used in cases of intestinal lymphangiectasia (IL) in humans with some success. OBJECTIVES Describe the use of octreotide in dogs with PLE including reason for and details of prescription, adverse effects, and apparent response. ANIMALS Eighteen dogs with PLE, 13 with histopathology available. Ninety-two percent (12/13) had IL diagnosed on biopsy. All 13 dogs had intestinal inflammatory infiltrates noted. METHODS Multicenter, retrospective, descriptive study. Cases were volunteered for inclusion by individual attending veterinarians who reported the use of octreotide in cases of PLE. RESULTS In 16/18 (89%) cases octreotide was prescribed to PLE dogs with a clinical suspicion or confirmed diagnosis of IL that were refractory to standard therapies. Median serum albumin at the time of octreotide prescription was 1.7 g/dL (range, 1.0-3.1 g/dL). The median dose of octreotide prescribed was 20 μg/kg, SQ, daily with a range of 4-39 μg/kg, SQ, daily. Adverse effects were noted in 3/18 (17%, 95% CI [4%, 41%]) of dogs; discontinuation of the drug was necessary in 1 dog. Improvement in clinical signs was noted in 6/12 (50%, 95% CI [21%, 79%]). CONCLUSIONS AND CLINICAL IMPORTANCE Octreotide was most commonly prescribed to dogs with PLE and suspected or confirmed IL that had failed to respond to standard therapies. Though a benefit to PLE dogs cannot be confirmed, octreotide was well tolerated by the majority of dogs at the doses prescribed in this study.
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Affiliation(s)
- Sara A. Jablonski
- Department of Small Animal Clinical Sciences, College of Veterinary MedicineMichigan State UniversityEast LansingMichiganUSA
| | | | - M. Katherine Tolbert
- Gastrointestinal Laboratory, Department of Small Animal Clinical Sciences, College of Veterinary Medicine and Biomedical SciencesTexas A&M UniversityCollege StationTexasUSA
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2
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Safari Vejin T, Zepeda ME, Yglesias BS, Devito P. Newfound features associated with Hennekam Syndrome ( Intestinal Lymphangiectasia-Lymphedema-Intellectual-Disability Syndrome) complicated with comorbid Waldmann's Disease resulting in Celiac Disease. Clin Case Rep 2023; 11:e7891. [PMID: 38028107 PMCID: PMC10651965 DOI: 10.1002/ccr3.7891] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2023] [Revised: 08/17/2023] [Accepted: 08/28/2023] [Indexed: 12/01/2023] Open
Abstract
Key Clinical Message Adequate evaluation of patients with Hennekam Syndrome (HS) is challenging for physicians, because of multi-organ involvement and complex pathophysiology. We report the first case in an African American with lymphedema, who developed protein-losing enteropathy (PLE) and was successfully diagnosed with HS from cause-and-effect complications by Waldmann's Disease (WD) and comorbid Celiac Disease (CD). Abstract As far as we know, this is the 51st case of HS worldwide and the first one in an African American. The examined patient met all diagnostic criteria for HS, suggesting a dysfunction in the development of the lymphatic system, with associated comorbidities including developmental delay, gastrointestinal pathologies, facial and hearing abnormalities, and cardiac defects. Primary intestinal lymphangiectasia (WD) is a consequence of HS, which ultimately results in PLE and worsening interstitial lymph buildup. Based on our findings, CD, a complication not yet reported in HS, may arise from WD. Other autoimmune diseases may be seen in HS: a previous report demonstrated positive anti-thyroid stimulating hormone antibodies in HS patients. We propose that in HS, increased interstitial lymph (WD, if intestinal) with protein loss induces TNF-α- and IL-6-mediated immune reactions in the affected visceral organs, causing autoimmune pathologies. The interstitial lymph fluid-induced TNF-α and IL-6-mediated immunopathogenic reactions lead to inflammation and subsequent destruction of the intestinal mucosa. The chronic inflammatory increase in TGF-β causes gastric mucosa hypertrophy, which results in gastric fold thickening. Eventually, wider tight junctions develop, increasing gastric mucosa permeability, and leading to gastropathy. Considering the examined patient's history of gastroenteritis and the literature stating that CD is a non-mucosal cause of gastropathy and PLE, it is suggested that sequelae of GI complications occur in a cause-and-effect chain in HS. HS results in WD, which causes CD, resulting in hypertrophic gastropathy and loss of parietal and chief cells, eventually leading to malabsorption and PLE (Figure 1). HS primarily affects various organs due to inflammatory-mediated damage and accumulation of lymph fluid. Other findings for HS include keratoconjunctivitis sicca (dry eye disease), fibrous lymphedema exhibiting lymphorrhea, chylous ascites, anemia, and parathyroid abnormalities. Immune impairment in HS predisposes patients to autoimmune disorders, therefore autoimmunity (CD) and WD are concomitant comorbidities of HS. HS-associated comorbidities are primarily due to inflammation and damage to immune cell transport or underlying health conditions affecting proper lymphatic function. However, it is suggested that HS mutations may disrupt the development of the lymphatic system leading to further complication. complications can be compound heterozygous, and there is a need for further research to identify nearby genes that can cause concomitant co-morbidity.
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Affiliation(s)
- Tannaz Safari Vejin
- Department of SurgeryTrumbull Regional Medical CenterWarrenOhioUSA
- AUA College and MedicineAntigua and Barbuda
| | | | | | - Peter Devito
- Department of SurgeryTrumbull Regional Medical CenterWarrenOhioUSA
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Shinwari K, Rehman HM, Xiao N, Guojun L, Khan MA, Bolkov MA, Tuzankina IA, Chereshnev VA. Novel high-risk missense mutations identification in FAT4 gene causing Hennekam syndrome and Van Maldergem syndrome 2 through molecular dynamics simulation. INFORMATICS IN MEDICINE UNLOCKED 2023. [DOI: 10.1016/j.imu.2023.101160] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023] Open
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Jablonski SA. Pathophysiology, Diagnosis, and Management of Canine Intestinal Lymphangiectasia: A Comparative Review. Animals (Basel) 2022; 12:ani12202791. [PMID: 36290177 PMCID: PMC9597800 DOI: 10.3390/ani12202791] [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: 09/01/2022] [Revised: 10/05/2022] [Accepted: 10/13/2022] [Indexed: 11/06/2022] Open
Abstract
Intestinal lymphangiectasia was first described in the dog over 50 years ago. Despite this, canine IL remains poorly understood and challenging to manage. Intestinal lymphangiectasia is characterized by variable intestinal lymphatic dilation, lymphatic obstruction, and/or lymphangitis, and is a common cause of protein-losing enteropathy in the dog. Breed predispositions are suggestive of a genetic cause, but IL can also occur as a secondary process. Similarly, both primary and secondary IL have been described in humans. Intestinal lymphangiectasia is definitively diagnosed via intestinal histopathology, but other diagnostic results can be suggestive of IL. Advanced imaging techniques are frequently utilized to aid in the diagnosis of IL in humans but have not been thoroughly investigated in the dog. Management strategies differ between humans and dogs. Dietary modification is the mainstay of therapy in humans with additional pharmacological therapies occasionally employed, and immunosuppressives are rarely used due to the lack of a recognized immune pathogenesis. In contrast, corticosteroid and immunosuppressive therapies are more commonly utilized in canine IL. This review aims toward a better understanding of canine IL with an emphasis on recent discoveries, comparative aspects, and necessary future investigations.
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Affiliation(s)
- Sara A Jablonski
- Department of Small Animal Clinical Sciences, College of Veterinary Medicine, Michigan State University, East Lansing, MI 48824, USA
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5
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Mas E, Borrelli O, Broekaert I, de-Carpi JM, Dolinsek J, Miele E, Pienar C, Koninckx CR, Thomassen RA, Thomson M, Tzivinikos C, Benninga MA. Drugs in Focus: Octreotide Use in Children With Gastrointestinal Disorders. J Pediatr Gastroenterol Nutr 2022; 74:1-6. [PMID: 34508049 DOI: 10.1097/mpg.0000000000003294] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
ABSTRACT Octreotide, a somatostatin analogue, has been used for more than 20 years in children with gastrointestinal bleeding, chylothorax or chylous ascites, intestinal lymphangiectasia, pancreatitis, intestinal dysmotility, and severe diarrhoea; however, until now, there is a lack of randomised clinical trials evaluating the efficacy of this compound in childhood. Hence, we aimed to review the literature in order to determine the evidence of its use and safety in children, using PubMed from 2000 to 2021 with the search terms "octreotide" and "children" and "bleeding or chylous ascites or chylothorax or acute pancreatitis or lymphangiectasia or diarrhoea or intestinal dysmotility".
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Affiliation(s)
- Emmanuel Mas
- Unité de Gastroentérologie, Hépatologie, Nutrition, Diabétologie et Maladies Héréditaires du Métabolisme, Hôpital des Enfants, CHU de Toulouse; IRSD, Université de Toulouse, INSERM, INRAE, ENVT, UPS, Toulouse, France
| | - Osvaldo Borrelli
- Department of Paediatric Gastroenterology, Neurogastroenterology and Motility Unit, Great Ormond Street Hospital, London, UK
| | - Ilse Broekaert
- Department of Paediatrics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany
| | - J Martin de-Carpi
- Department of Paediatric Gastroenterology, Hepatology and Nutrition, Hospital Sant Joan de Déu, Barcelona, Spain
| | - Jernej Dolinsek
- Department of Paediatrics, University Medical Centre Maribor, Maribor, Slovenia
| | - Erasmo Miele
- Department of Translational Medical Science, Section of Paediatrics, University of Naples "Federico II", Naples Italy
| | - Corina Pienar
- Department of Paediatrics, 2 Paediatric Clinic, "Victor Babes" University of Medicine and Pharmacy, Timisoara, Romania
| | - C Ribes Koninckx
- Paediatric Gastroenterology, La Fe University Hospital, Valencia, Spain
| | - Ruth-Anne Thomassen
- Department of Paediatric Medicine, Division of Paediatric and Adolescent Medicine, Oslo University Hospital, Oslo, Norway
| | - Mike Thomson
- Centre for Paediatric Gastroenterology, Sheffield Children's Hospital NHS Foundation Trust, Sheffield, UK
| | - Christo Tzivinikos
- Department of Paediatric Gastroenterology, Al Jalila Children's Specialty Hospital, Dubai, UAE
| | - Marc A Benninga
- Department of Paediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
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Diamanti A, Calvitti G, Martinelli D, Santariga E, Capriati T, Bolasco G, Iughetti L, Pujia A, Knafelz D, Maggiore G. Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes. Nutrients 2021; 13:nu13030786. [PMID: 33673586 PMCID: PMC7997222 DOI: 10.3390/nu13030786] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2021] [Accepted: 02/24/2021] [Indexed: 12/20/2022] Open
Abstract
Background: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops. Methods: We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: “intestinal failure” OR “home parenteral nutrition” OR “short bowel syndrome” OR “chronic pseudo-obstruction” OR “chronic intestinal pseudo-obstruction” OR “autoimmune enteropathy” OR “long-term parenteral nutrition”. Results: We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion. Conclusions: The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management.
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Affiliation(s)
- Antonella Diamanti
- Hepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, Italy; (T.C.); (G.B.); (D.K.); (G.M.)
- Correspondence: ; Tel.: +39-0668592189
| | - Giacomo Calvitti
- Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, 41121 Modena, Italy; (G.C.); (L.I.)
| | - Diego Martinelli
- Metabolic Diseases Unit, “Bambino Gesù” Children Hospital, 00165 Rome, Italy;
| | - Emma Santariga
- Clinical Nutrition Unit, Department of Medical and Surgical Sciences, University Magna Graecia, 88100 Catanzaro, Italy; (E.S.); (A.P.)
| | - Teresa Capriati
- Hepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, Italy; (T.C.); (G.B.); (D.K.); (G.M.)
| | - Giulia Bolasco
- Hepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, Italy; (T.C.); (G.B.); (D.K.); (G.M.)
| | - Lorenzo Iughetti
- Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, 41121 Modena, Italy; (G.C.); (L.I.)
| | - Arturo Pujia
- Clinical Nutrition Unit, Department of Medical and Surgical Sciences, University Magna Graecia, 88100 Catanzaro, Italy; (E.S.); (A.P.)
| | - Daniela Knafelz
- Hepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, Italy; (T.C.); (G.B.); (D.K.); (G.M.)
| | - Giuseppe Maggiore
- Hepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, Italy; (T.C.); (G.B.); (D.K.); (G.M.)
- Medical Sciences Department Ferrara University, 44121 Ferrara, Italy
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7
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Lopez RN, Day AS. Primary intestinal lymphangiectasia in children: A review. J Paediatr Child Health 2020; 56:1719-1723. [PMID: 32463559 DOI: 10.1111/jpc.14837] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/06/2020] [Revised: 02/15/2020] [Accepted: 02/17/2020] [Indexed: 02/06/2023]
Abstract
Primary intestinal lymphangiectasia is an uncommon condition that usually presents early in childhood. This incurable condition is consequent to underlying lymphatic abnormalities that lead to loss of lymphatic contents into the intestinal lumen. This article outlines an approach to the assessment of children presenting with characteristic features and consideration of other conditions that could lead to enteric protein loss. An overview of the management of primary intestinal lymphangiectasia is outlined.
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Affiliation(s)
- Robert N Lopez
- Department of Gastroenterology, Hepatology and Liver Transplantation, Queensland Children's Hospital, Brisbane, Queensland, Australia
| | - Andrew S Day
- Department of Paediatrics, University of Otago, Christchurch, New Zealand.,Department of Paediatrics, Christchurch Hospital, Christchurch, New Zealand
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Peter HH, Ochs HD, Cunningham-Rundles C, Vinh DC, Kiessling P, Greve B, Jolles S. Targeting FcRn for immunomodulation: Benefits, risks, and practical considerations. J Allergy Clin Immunol 2020; 146:479-491.e5. [PMID: 32896308 PMCID: PMC7471860 DOI: 10.1016/j.jaci.2020.07.016] [Citation(s) in RCA: 62] [Impact Index Per Article: 12.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/09/2020] [Revised: 07/21/2020] [Accepted: 07/23/2020] [Indexed: 02/08/2023]
Abstract
The neonatal fragment crystallizable (Fc) receptor (FcRn) functions as a recycling mechanism to prevent degradation and extend the half-life of IgG and albumin in the circulation. Several FcRn inhibitors selectively targeting IgG recycling are now moving rapidly toward clinical practice in neurology and hematology. These molecules accelerate the destruction of IgG, reducing pathogenic IgG and IgG immune complexes, with no anticipated effects on IgA, IgM, IgE, complement, plasma cells, B cells, or other cells of the innate or adaptive immune systems. FcRn inhibitors have potential for future use in a much wider variety of antibody-mediated autoimmune diseases. Given the imminent clinical use, potential for broader utility, and novel mechanism of action of FcRn inhibitors, here we review data from 4 main sources: (a) currently available activity, safety, and mechanism-of-action data from clinical trials of FcRn inhibitors; (b) other procedures and treatments that also remove IgG (plasma donation, plasma exchange, immunoadsorption); (c) diseases resulting in loss of IgG; and (d) primary immunodeficiencies with potential mechanistic similarities to those induced by FcRn inhibitors. These data have been evaluated to provide practical considerations for the assessment, monitoring, and reduction of any potential infection risk associated with FcRn inhibition, in addition to highlighting areas for future research.
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Affiliation(s)
- Hans-Hartmut Peter
- Freiburg University Hospital, Centre for Chronic Immunodeficiency, Freiburg, Germany
| | - Hans D Ochs
- Seattle Children's Research Institute, Seattle, Wash; Department of Pediatrics, University of Washington, Seattle, Wash
| | | | - Donald C Vinh
- Division of Infectious Diseases, Department of Medicine and Department of Medical Microbiology, McGill University Health Centre, Montreal, Quebec, Canada; Infectious Diseases & Immunity in Global Health Program, Research Institute-McGill University Health Centre, Montreal, Quebec, Canada
| | | | | | - Stephen Jolles
- Immunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, United Kingdom.
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Ozen A. CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease. Immunol Rev 2019; 287:20-32. [PMID: 30565236 DOI: 10.1111/imr.12715] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2018] [Accepted: 08/31/2018] [Indexed: 12/17/2022]
Abstract
Primary intestinal lymphangiectasia (PIL) or Waldmann's disease was described in 1961 as an important cause of protein-losing enteropathy (PLE). PIL can be the sole finding in rare individuals or occur as part of a multisystemic genetic syndrome. Although genetic etiologies of many lymphatic dysplasia syndromes associated with PIL have been identified, the pathogenesis of isolated PIL (with no associated syndromic features) remains unknown. Familial cases and occurrence at birth suggest genetic etiologies in certain cases. Recently, CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and PLE (the CHAPLE syndrome) has been identified as a monogenic form of PIL. Surprisingly, loss of CD55, a key regulator of complement system leads to a predominantly gut condition. Similarly to other complement disorders, namely paroxysmal nocturnal and hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), CHAPLE disease involves pathogenic cross-activation of the coagulation system, predisposing individuals to severe thrombosis. The observation that complement system is overly active in CHAPLE disease introduced a novel concept into the management of PLE; anti-complement therapy. While CD55 deficiency constitutes a treatable subgroup in the larger pool of patients with isolated PIL, the etiology remains to be identified in the remaining patients with intact CD55.
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Affiliation(s)
- Ahmet Ozen
- Division of Allergy and Immunology, Marmara University School of Medicine, Istanbul, Turkey.,The Istanbul Jeffrey Modell Diagnostic Center for Primary Immunodeficiency Diseases, Istanbul, Turkey
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Craven MD, Washabau RJ. Comparative pathophysiology and management of protein-losing enteropathy. J Vet Intern Med 2019; 33:383-402. [PMID: 30762910 PMCID: PMC6430879 DOI: 10.1111/jvim.15406] [Citation(s) in RCA: 64] [Impact Index Per Article: 10.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2018] [Accepted: 11/30/2018] [Indexed: 12/17/2022] Open
Abstract
Protein‐losing enteropathy, or PLE, is not a disease but a syndrome that develops in numerous disease states of differing etiologies and often involving the lymphatic system, such as lymphangiectasia and lymphangitis in dogs. The pathophysiology of lymphatic disease is incompletely understood, and the disease is challenging to manage. Understanding of PLE mechanisms requires knowledge of lymphatic system structure and function, which are reviewed here. The mechanisms of enteric protein loss in PLE are identical in dogs and people, irrespective of the underlying cause. In people, PLE is usually associated with primary intestinal lymphangiectasia, suspected to arise from genetic susceptibility, or “idiopathic” lymphatic vascular obstruction. In dogs, PLE is most often a feature of inflammatory bowel disease (IBD), and less frequently intestinal lymphangiectasia, although it is not proven which process is the true driving defect. In cats, PLE is relatively rare. Review of the veterinary literature (1977‐2018) reveals that PLE was life‐ending in 54.2% of dogs compared to published disease‐associated deaths in IBD of <20%, implying that PLE is not merely a continuum of IBD spectrum pathophysiology. In people, diet is the cornerstone of management, whereas dogs are often treated with immunosuppression for causes of PLE including lymphangiectasia, lymphangitis, and crypt disease. Currently, however, there is no scientific, extrapolated, or evidence‐based support for an autoimmune or immune‐mediated mechanism. Moreover, people with PLE have disease‐associated loss of immune function, including lymphopenia, severe CD4+ T‐cell depletion, and negative vaccinal titers. Comparison of PLE in people and dogs is undertaken here, and theories in treatment of PLE are presented.
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Affiliation(s)
- Melanie D Craven
- Small Animal Clinical Sciences, Western College of Veterinary Medicine, University of Saskatchewan, Saskatoon, Canada
| | - Robert J Washabau
- Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, Minnesota
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Dong J, Xin J, Shen W, Wen T, Chen X, Sun Y, Wang R. CT Lymphangiography (CTL) in Primary Intestinal Lymphangiectasia (PIL): A Comparative Study with Intraoperative Enteroscopy (IOE). Acad Radiol 2019; 26:275-281. [PMID: 29885759 DOI: 10.1016/j.acra.2018.04.023] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2018] [Revised: 04/28/2018] [Accepted: 04/29/2018] [Indexed: 12/13/2022]
Abstract
RATIONALE AND OBJECTIVES To investigate the clinical feasibility of CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL) by comparison with intraoperative enteroscopy (IOE) during exploratory laparotomy. MATERIALS AND METHODS Eleven PIL patients (F/M, two/nine, age range 10-37 years) were recruited in this study, and they were performed IOE during exploratory laparotomy for suspected serious lymphatic-intestinal leakages. All the patients were performed CTL before surgery, and the imaging data were reviewed by two radiologists separately. CTL assessments included intestinal lesions, edematous lesions, intestinal and mesenteric lymphangiectasia, lymphaticabdominal leakages, lymph fluid reflux, lymphangioma and abnormal lymphatics in other area. The intestinal lymphangiectasia and lymphaticintestinal leakages were confirmed by histology and IOE. RESULTS For CTL, (1) nine intestinal wall thickening; (2) eight ascites, complicated with four pleural effusions, (3) eight intestinal and mesenteric lymphangiectasia, (4) six lymph fluid reflux (5) one lymphatic-abdominal leakage, (6) two lymphangioma. While for IOE, intestinal lymphangiectasia has been confirmed in all patients, including five segemental and six diffusive lesions in intestinal mucosa. Besides, one lymphatic-intestinal fistula, one lymphatic-abdominal leakage was confirmed. Compared to IOE and histology, the accuracy of CTL was 72.7% in detecting intestinal lymphangiectasia. CONCLUSION Compared to IOE, CTL demonstrates feasibility in detection of intestinal lymphangiectasia and other abnormalities in whole lymphatic circulation for PIL. Combination of CTL with IOE accommodates guidance for preoperative evaluation and therapeutic management for PIL.
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Affiliation(s)
- Jian Dong
- Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China
| | - Jianfeng Xin
- Department of Lymphatic surgery, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China
| | - Wenbin Shen
- Department of Lymphatic surgery, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China
| | - Tingguo Wen
- Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China
| | - Xiaobai Chen
- Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China
| | - Yuguang Sun
- Department of Lymphatic surgery, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China
| | - Rengui Wang
- Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi St, Haidian District, Beijing 100038, China.
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Abstract
Background and Objective Octreotide is a somatostatin analogue and has been used off-label for a variety of conditions. There are no specific guidelines for the use of octreotide in neonates and its safety and efficacy have not been systematically evaluated. The objective of this study is to present our experience of using octreotide therapy in neonates. Methods This is a retrospective study of neonates who received octreotide therapy during their hospital stay over a 15 years period (2003–2017) in a tertiary neonatal centre. The demographic details and indications of octreotide therapy including time of initiation, route, dose, duration and adverse effects of therapy were noted. The clinical course following octreotide administration was also analysed. Results Eleven neonates received octreotide therapy during the study period, of which nine had chylothorax and two had chylous ascites. Resolution of the chylous effusion with octreotide therapy was achieved in 4 out of 11 (36.3%) of the cases. The median duration of octreotide therapy in cases with successful resolution was 17.5 days. With the exception of minor side effects such as hyperglycaemia, none of the patients had any significant side effects that required discontinuation of therapy. Conclusion Octreotide was used safely as an adjunctive therapy for the treatment of chylothorax and chylous ascites in neonates. However, larger prospective controlled trials are required to establish the optimal dose, time of initiation, duration and efficacy of octreotide therapy in neonates.
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Affiliation(s)
- Syed Ahmed Zaki
- Monash Newborn, Monash Children's Hospital, Clayton, VIC, Australia
| | | | - Atul Malhotra
- Monash Newborn, Monash Children's Hospital, & Department of Paediatrics, Monash University, 246 Clayton Road, Clayton, VIC, Australia.
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13
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Vignes S, Bellanger J. Lymphangiectasies intestinales primitives (maladie de Waldmann). Rev Med Interne 2018; 39:580-585. [DOI: 10.1016/j.revmed.2017.07.009] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/20/2017] [Accepted: 07/24/2017] [Indexed: 12/17/2022]
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Lee YG, Kim SC, Park SB, Kim MJ. Hennekam Syndrome: A Case Report. Ann Rehabil Med 2018; 42:184-188. [PMID: 29560340 PMCID: PMC5852224 DOI: 10.5535/arm.2018.42.1.184] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/02/2017] [Accepted: 08/01/2017] [Indexed: 11/05/2022] Open
Affiliation(s)
- Yeong Guk Lee
- Department of Rehabilitation Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Seung Chan Kim
- Department of Rehabilitation Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Si-Bog Park
- Department of Rehabilitation Medicine, Hanyang University College of Medicine, Seoul, Korea
| | - Mi Jung Kim
- Department of Rehabilitation Medicine, Hanyang University College of Medicine, Seoul, Korea
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15
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Alshikho MJ, Talas JM, Noureldine SI, Zazou S, Addas A, Kurabi H, Nasser M. Intestinal Lymphangiectasia: Insights on Management and Literature Review. AMERICAN JOURNAL OF CASE REPORTS 2016; 17:512-22. [PMID: 27440277 PMCID: PMC4957630 DOI: 10.12659/ajcr.899636] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Patient: Male, 24 Final Diagnosis: Intestinal lymphangiectasia Symptoms: Frequent episodes of diarrhea • recurrent infections • swelling in the lower limbs Medication: Octreotide • MCT oils Clinical Procedure: Endoscopic exam • Doppler ultrasound study • abdominal CT scan Specialty: Gastroenterology and Hepatology
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Affiliation(s)
- Mohamad J Alshikho
- Department of Neurology, Massachusetts General Hospital, Harvard University, Boston, MA, USA
| | - Joud M Talas
- Department of Dermatology, Aleppo University, Aleppo, Syrian Arab Republic
| | - Salem I Noureldine
- Department of Otolaryngology - Head and Neck Surgery, Johns Hopkins University, Baltimore, MD, USA
| | - Saf Zazou
- Department of General Internal Medicine, Aleppo University, Aleppo, Syrian Arab Republic
| | - Aladdin Addas
- Department of General Internal Medicine, Aleppo University, Aleppo, Syrian Arab Republic
| | - Haitham Kurabi
- Department of General Internal Medicine, Aleppo University, Aleppo, Syrian Arab Republic
| | - Mahmoud Nasser
- Department of Gastroenterology, Aleppo University, Aleppo, Syrian Arab Republic
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16
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Berges-Raso I, Capel I, Caixàs A, Trallero R, Rigla M. Hypothyroidism and protein-losing enteropathy: A case report. ENDOCRINOLOGIA Y NUTRICION : ORGANO DE LA SOCIEDAD ESPANOLA DE ENDOCRINOLOGIA Y NUTRICION 2016; 63:95-96. [PMID: 26705734 DOI: 10.1016/j.endonu.2015.11.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/12/2015] [Revised: 11/04/2015] [Accepted: 11/09/2015] [Indexed: 06/05/2023]
Affiliation(s)
- Irene Berges-Raso
- Servicio de Endocrinología y Nutrición, Hospital Universitario Parc Taulí, Sabadell, Barcelona, España.
| | - Ismael Capel
- Servicio de Endocrinología y Nutrición, Hospital Universitario Parc Taulí, Sabadell, Barcelona, España
| | - Assumpta Caixàs
- Servicio de Endocrinología y Nutrición, Hospital Universitario Parc Taulí, Sabadell, Barcelona, España
| | - Roser Trallero
- Servicio de Endocrinología y Nutrición, Hospital Universitario Parc Taulí, Sabadell, Barcelona, España
| | - Mercedes Rigla
- Servicio de Endocrinología y Nutrición, Hospital Universitario Parc Taulí, Sabadell, Barcelona, España
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17
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Troskot R, Jurčić D, Bilić A, Gomerčić Palčić M, Težak S, Brajković I. How to treat an extensive form of primary intestinal lymphangiectasia? World J Gastroenterol 2015; 21:7320-7325. [PMID: 26109821 PMCID: PMC4476896 DOI: 10.3748/wjg.v21.i23.7320] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/10/2015] [Revised: 02/27/2015] [Accepted: 03/31/2015] [Indexed: 02/07/2023] Open
Abstract
We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.
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18
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Ingle SB, Hinge (Ingle) CR. Primary intestinal lymphangiectasia: Minireview. World J Clin Cases 2014; 2:528-533. [PMID: 25325063 PMCID: PMC4198405 DOI: 10.12998/wjcc.v2.i10.528] [Citation(s) in RCA: 37] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/18/2014] [Revised: 06/28/2014] [Accepted: 07/29/2014] [Indexed: 02/05/2023] Open
Abstract
Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.
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19
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Update March 2013. Lymphat Res Biol 2013. [DOI: 10.1089/lrb.2013.1113] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
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