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Khalil Z, Ali HN, Ibrahim H, Al-Abbedien EZ. Endoscopic Diagnosis of Primary Intestinal Lymphangiectasia in an Adult With Refractory Protein-Losing Enteropathy: A Case Report. Cureus 2024; 16:e66141. [PMID: 39233993 PMCID: PMC11372727 DOI: 10.7759/cureus.66141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/04/2024] [Indexed: 09/06/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated lymphatic vessels in the gastrointestinal tract, leading to protein-losing enteropathy (PLE). We report the case of a 43-year-old male presenting with refractory PLE, characterized by generalized edema, chronic diarrhea, and significant weight loss over four months. Despite multiple consultations and treatments for presumed inflammatory bowel disease, his symptoms persisted, and his condition worsened. An upper endoscopy was performed, revealing white villi in the duodenum. Histopathological examination of duodenal biopsies confirmed the presence of markedly dilated lymphatic vessels in the lamina propria, consistent with PIL. The patient was managed with a high-protein, low-fat diet supplemented with medium-chain triglycerides (MCTs) and octreotide therapy. This treatment regimen led to significant clinical improvement, including reduced edema, normalization of albumin levels, and resolution of gastrointestinal symptoms. This case underscores the importance of considering PIL in adults with refractory PLE.
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Affiliation(s)
- Zeyad Khalil
- College of Medicine, October 6 University, Cairo, EGY
| | - Hamza N Ali
- College of Medicine, October 6 University, Cairo, EGY
| | - Hosam Ibrahim
- College of Medicine, October 6 University, Cairo, EGY
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2
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Savoca ML, Brownell JN. Comprehensive nutrition guidelines and management strategies for enteropathy in children. Semin Pediatr Surg 2024; 33:151425. [PMID: 38849288 DOI: 10.1016/j.sempedsurg.2024.151425] [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] [Indexed: 06/09/2024]
Abstract
Protein-losing enteropathy (PLE) describes a syndrome of excessive protein loss into the gastrointestinal tract, which may be due to a wide variety of etiologies. For children in whom the protein loss is associated with lymphangiectasia, medical nutrition therapy focused on restricting enteral long-chain triglycerides and thus intestinal chyle production is an integral component of treatment. This approach is based on the principle that reducing intestinal chyle production will concurrently decrease enteric protein losses of lymphatic origin. In patients with ongoing active PLE or those who are on a fat-restricted diet, particularly in infants and young children, supplemental calories may be provided with medium-chain triglycerides (MCT). MCT are absorbed directly into the bloodstream, bypassing intestinal lymphatics and not contributing to intestinal chyle production. Patients with active PLE or who are on dietary fat restriction should be monitored for associated micronutrient deficiencies. In this paper, we seek to formally present recommended nutrition interventions, principles of dietary education and patient counseling, and monitoring parameters in pediatric populations with PLE based on our experience in a busy clinical referral practice focused on this population.
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Affiliation(s)
- Melanie L Savoca
- Children's Hospital of Philadelphia, Department of Clinical Nutrition, Jill and Mark Fishman Center for Lymphatic Disorders, Comprehensive Vascular Anomalies Program, Philadelphia, PA.
| | - Jefferson N Brownell
- Children's Hospital of Philadelphia, Division of Gastroenterology, Hepatology and Nutrition, Jill and Mark Fishman Center for Lymphatic Disorders, Comprehensive Vascular Anomalies Program, Philadelphia, PA
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Prajapati S, Bohara S, Mainali G, Karki S, Thapa S, Aryal N. Diagnostic dilemma in diagnosing rare cause of protein losing enteropathy: Waldmann's disease. Clin Case Rep 2022; 10:e5992. [PMID: 35769238 PMCID: PMC9211774 DOI: 10.1002/ccr3.5992] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2022] [Revised: 06/01/2022] [Accepted: 06/10/2022] [Indexed: 11/30/2022] Open
Abstract
Waldmann's disease, or primary intestinal lymphangiectasia, is an unusual cause of protein-losing enteropathy primarily characterized by lymphopenia, hypoalbuminemia, and hypogammaglobulinemia. However, variable clinical presentations result dilemmas in diagnosis and effective management. We present a toddler diagnosed with Waldmann's disease managed with a high-protein diet and medium-chain triglyceride supplementation.
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Affiliation(s)
- Sarita Prajapati
- Department of PediatricsBirendra Military HospitalKathmanduNepal
| | - Sujan Bohara
- Department of General and Gastrointestinal SurgeryNepal Mediciti HospitalLalitpurNepal
| | - Gaurab Mainali
- Nepalese Army Institute of Health SciencesKathmanduNepal
| | | | - Sharan Thapa
- Lekhnath City Hospital Private LimitedPokharaNepal
| | - Nirjala Aryal
- Department of PediatricsBirendra Military HospitalKathmanduNepal
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Thibault R, Abbasoglu O, Ioannou E, Meija L, Ottens-Oussoren K, Pichard C, Rothenberg E, Rubin D, Siljamäki-Ojansuu U, Vaillant MF, Bischoff SC. ESPEN guideline on hospital nutrition. Clin Nutr 2021; 40:5684-5709. [PMID: 34742138 DOI: 10.1016/j.clnu.2021.09.039] [Citation(s) in RCA: 86] [Impact Index Per Article: 21.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2021] [Accepted: 09/17/2021] [Indexed: 12/16/2022]
Abstract
In hospitals through Europe and worldwide, the practices regarding hospital diets are very heterogeneous. Hospital diets are rarely prescribed by physicians, and sometimes the choices of diets are based on arbitrary reasons. Often prescriptions are made independently from the evaluation of nutritional status, and without taking into account the nutritional status. Therapeutic diets (low salt, gluten-free, texture and consistency modified, …) are associated with decreased energy delivery (i.e. underfeeding) and increased risk of malnutrition. The European Society for Clinical Nutrition and Metabolism (ESPEN) proposes here evidence-based recommendations regarding the organization of food catering, the prescriptions and indications of diets, as well as monitoring of food intake at hospital, rehabilitation center, and nursing home, all of these by taking into account the patient perspectives. We propose a systematic approach to adapt the hospital food to the nutritional status and potential food allergy or intolerances. Particular conditions such as patients with dysphagia, older patients, gastrointestinal diseases, abdominal surgery, diabetes, and obesity, are discussed to guide the practitioner toward the best evidence based therapy. The terminology of the different useful diets is defined. The general objectives are to increase the awareness of physicians, dietitians, nurses, kitchen managers, and stakeholders towards the pivotal role of hospital food in hospital care, to contribute to patient safety within nutritional care, to improve coverage of nutritional needs by hospital food, and reduce the risk of malnutrition and its related complications.
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Affiliation(s)
- Ronan Thibault
- Unité de Nutrition, CHU Rennes, INRAE, INSERM, Univ Rennes, Nutrition Metabolisms and Cancer Institute, NuMeCan, Rennes, France.
| | - Osman Abbasoglu
- Department of Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey
| | - Elina Ioannou
- Department of Nutrition, Limassol General Hospital, Cyprus
| | - Laila Meija
- Riga Stradins University, Pauls Stradins Clinical University Hospital, Latvia
| | - Karen Ottens-Oussoren
- Department of Nutrition and Dietetics, Amsterdam University Medical Centers, Location VUmc, Amsterdam, the Netherlands
| | - Claude Pichard
- Unité de Nutrition, Hôpitaux Universitaires de Genève, Geneva, Switzerland
| | - Elisabet Rothenberg
- Faculty of Health Sciences Kristianstad University Kristianstad Sweden, Sweden
| | - Diana Rubin
- Vivantes Netzwerk für Gesundheit GmbH, Humboldt Klinikum und Klinikum Spandau, Berlin, Germany
| | | | | | - Stephan C Bischoff
- University of Hohenheim, Institute of Nutritional Medicine, Stuttgart, Germany
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Home parenteral nutrition a life-saving therapy in a primary intestinal lymphangiectasia patient affecting the entire GI tract - 3 year follow-up case report. Ann Med Surg (Lond) 2021; 67:102483. [PMID: 34168874 PMCID: PMC8209671 DOI: 10.1016/j.amsu.2021.102483] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2021] [Revised: 06/03/2021] [Accepted: 06/05/2021] [Indexed: 12/17/2022] Open
Abstract
Introduction and Importance Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy of unknown etiology, characterized by impaired lymphatic vessels drainage. The pathological changes in PIL result in usually localized or diffuse dilatation of intestinal lacteals, leading to leakage of lymphatic fluid rich of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. PIL may be asymptomatic or mildly symptomatic in moderate forms of the disease. In some patients, though, the outcome may be poor or even life-threatening. This case report demonstrates the severity of protein malnutrition, in some cases, and the extent of GI tract affected, requiring to start PN early and the need for its continuation as home parenteral nutrition (HPN). Case presentation We present a case of 39-year-old male with Factor V Leiden deficiency, who presented initially with symptoms of malnutrition and anasarca. The diagnosis was confirmed by histopathological findings pathognomonic for PIL from biopsies of the stomach, small intestine and colon. Clinical discussion The patient was started on low fat, high protein parenteral nutrition from the beginning of the treatment and required a long-term HPN for 3 years, because trials of tapering off and discontinuation of PN led to worsening of the biochemical results and recurrence of symptoms. Patient gradually improved and stabilized with persistent nutritional support. Conclusions The presented case report shows the magnitude of nutritional support (HPN) needed for severe PIL patients. HPN offers PIL patients with poor outcome and life-threatening complications a chance to improve and lead a normal life.
Primary intestinal lymphangiectasia is a rare protein-losing gastroenteropathy characterized by impaired lymphatic drainage. In patients with primary intestinal lymphangiectasia (PIL) the outcome may be life-threatening when anasarca occures. Therapy of low-fat, high medium-chain triglyceride, high protein content has to be implemented in PIL patients. Home parenteral nutrition offers severe primary intestinal lymphangiectasia patients a chance to lead a normal life. Aggressive management is necessary in patients with primary intestinal lymphangiectasia with chronic intestinal failure.
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Kwon Y, Kim ES, Choe YH, Kim MJ. Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature. BMC Pediatr 2021; 21:21. [PMID: 33407260 PMCID: PMC7789338 DOI: 10.1186/s12887-020-02447-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2020] [Accepted: 11/26/2020] [Indexed: 12/18/2022] Open
Abstract
Background Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia. Method Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved. Results Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for 3 months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3–4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after 1 month of sirolimus treatment. Conclusion Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.
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Affiliation(s)
- Yiyoung Kwon
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Eun Sil Kim
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea
| | - Yon Ho Choe
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
| | - Mi Jin Kim
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
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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] [MESH Headings] [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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von der Weid PY, Day AS. Pediatric Lymphatic Development and Intestinal Lymphangiectasia. ENCYCLOPEDIA OF GASTROENTEROLOGY 2020:158-169. [DOI: 10.1016/b978-0-12-801238-3.66051-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Surampalli V, Ramaswamy S, Surendran D, Bammigatti C, Swaminathan RP. Primary Intestinal Lymphangiectasia (Waldmann's Disease) Presenting with Chylous Effusions in a 15-Year-Old. J Clin Diagn Res 2017; 11:OD17-OD18. [PMID: 28969188 DOI: 10.7860/jcdr/2017/29055.10522] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/05/2017] [Accepted: 07/09/2017] [Indexed: 12/19/2022]
Abstract
Primary Intestinal Lymphangiectasia (PIL) is a rare disease of unknown aetiology which presents in the paediatric age group with anasarca, diarrhoea, hypoproteinaemia, lymphoedema and chylous effusions. Tuberculosis, filariasis, chest trauma, malignancies and haematological disorders usually contribute to most cases of secondary lymphangiectasia and chylous effusions. We hereby describe a case of PIL presenting with chylous effusions which remained undiagnosed for eight years.
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Affiliation(s)
- Vijay Surampalli
- Junior Resident, Department of Medicine, JIPMER, Puducherry, India
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Lee SJ, Song HJ, Boo SJ, Na SY, Kim HU, Hyun CL. Primary intestinal lymphangiectasia with generalized warts. World J Gastroenterol 2015; 21:8467-8472. [PMID: 26217101 PMCID: PMC4507119 DOI: 10.3748/wjg.v21.i27.8467] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2015] [Revised: 02/25/2015] [Accepted: 04/17/2015] [Indexed: 02/06/2023] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.
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11
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El-Etreby SA, Altonbary AY, Sorogy ME, Elkashef W, Mazroa JA, Bahgat MH. Anaemia in Waldmann’s disease: A rare presentation of a rare disease. World J Gastrointest Endosc 2015; 7:567-572. [PMID: 25992197 PMCID: PMC4436926 DOI: 10.4253/wjge.v7.i5.567] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/11/2014] [Revised: 11/29/2014] [Accepted: 02/11/2015] [Indexed: 02/05/2023] Open
Abstract
A 32-year-old female presented with 5-year history of iron deficiency anemia, marked pallor and edema of both lower limbs. Laboratory investigations including complete blood count, blood film, iron studies, lipid profile, ascitic fluid analysis, test of stool for occult blood and alpha 1 anti-trypsin. Upper, lower gastrointestinal (GIT) endoscopies, and enteroscopy were performed. Imaging techniques as abdominal ultrasonography and computed tomography were done. Echocardiography, lymph node biopsy and bone marrow examination were normal. The case was diagnosed as Waldmann’s disease with protein losing enteropathy and recurrent GIT bleeding. Management started with low fat diet with medium chain triglyceride, octreotide 200 μg twice a day, tranexamic acid and blood transfusion. Then, exploratory laparotomy with pathological examination of resected segment was done when recurrent GIT bleeding occurred and to excluded malignant transformation.
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12
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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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Xinias I, Mavroudi A, Sapountzi E, Thomaidou A, Fotoulaki M, Kalambakas A, Karypidou E, Kollios K, Pardalos G, Imvrios G. Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome. Case Rep Gastroenterol 2013; 7:153-63. [PMID: 23626516 PMCID: PMC3617964 DOI: 10.1159/000348763] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema, lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder.
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Affiliation(s)
- Ioannis Xinias
- Third Pediatric Department, Papageorgiou Hospital, Thessaloniki, Greece
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Maamer AB, Baazaoui J, Zaafouri H, Soualah W, Cherif A. Primary intestinal lymphangiectasia or Waldmann's disease: a rare cause of lower gastrointestinal bleeding. Arab J Gastroenterol 2012; 13:97-98. [PMID: 22980601 DOI: 10.1016/j.ajg.2012.03.001] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2010] [Revised: 04/10/2011] [Accepted: 03/04/2012] [Indexed: 02/07/2023]
Affiliation(s)
- Anis Ben Maamer
- Department of Surgery, Habib Thameur Hospital, Tunis, Tunisia
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15
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Steinemann DC, Dindo D, Clavien PA, Nocito A. Atraumatic chylous ascites: systematic review on symptoms and causes. J Am Coll Surg 2011; 212:899-905.e1-4. [PMID: 21398159 DOI: 10.1016/j.jamcollsurg.2011.01.010] [Citation(s) in RCA: 99] [Impact Index Per Article: 7.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2010] [Revised: 01/10/2011] [Accepted: 01/11/2011] [Indexed: 12/14/2022]
Affiliation(s)
- Daniel C Steinemann
- Department of Visceral and Transplantation Surgery, University Hospital Zurich, Zurich, Switzerland
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Popović DDJ, Spuran M, Alempijević T, Krstić M, Djuranović S, Kovacević N, Damnjanović S, Micev M. [Congenital intestinal lymphangiectasia]. VOJNOSANIT PREGL 2011; 68:270-273. [PMID: 21526555 DOI: 10.2298/vsp1103270p] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022] Open
Abstract
BACKGROUND Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortuous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. CASE REPORT A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and supportive therapy. CONCLUSION Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.
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Morresey PR. Intestinal lymphangiectasia and chyloperitoneum. EQUINE VET EDUC 2010. [DOI: 10.1111/j.2042-3292.2010.00054.x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
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Kim NR, Lee SK, Suh YL. Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. J Pediatr Surg 2009; 44:e13-7. [PMID: 19853733 DOI: 10.1016/j.jpedsurg.2009.06.034] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/15/2009] [Revised: 06/25/2009] [Accepted: 06/29/2009] [Indexed: 12/12/2022]
Abstract
Primary intestinal lymphangiectasia is a rare cause of protein-losing enteropathy and usually presents with intermittent diarrhea or malnutrition. Diagnosis depends largely on its pathologic condition demonstrating greatly dilated lymphatics mainly in the lamina propria of the mucosa. We report a case of primary intestinal lymphangiectasia, of the diffuse type, presenting with abdominal pain and voluminous diarrhea in a previously healthy 8-year-old boy. He had periumbilical pain for 3 months before presentation. He was managed by segmental bowel resections and end-to-end anastomoses. The histopathologic condition of the resected small intestine showed lymphatic dilation limited mainly to the subserosa and mesentery but was not prominent in the mucosa. Abdominal pain and diarrhea subsided postoperatively. The present case is the fourth report describing a response to operative resection.
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Affiliation(s)
- Na Rae Kim
- Department of Pathology, Gil Medical Center, Gachon University of Medicine and Science, Incheon 405-760, Korea
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20
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Melero JL, García-Eliz M, Yago M, Nevárez A, Ortuño J, Ponce J. [Medium-term management of protein-losing enteropathy of cardiac origin unresponsive to medical therapy in a patient awaiting heart transplantation]. GASTROENTEROLOGIA Y HEPATOLOGIA 2009; 32:279-82. [PMID: 19371966 DOI: 10.1016/j.gastrohep.2008.11.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/08/2008] [Accepted: 11/15/2008] [Indexed: 10/20/2022]
Abstract
Protein-losing enteropathy is characterized by excessive leaking of serum proteins into the gastrointestinal tract, as a result of disease progression in several diseases. We report the case of a 17-year-old-woman with hypoproteinemia, generalized edema and serosal effusions diagnosed as protein-losing enteropathy due to right ventricular failure secondary to previous surgical damage. All previously described therapies were ineffective in curing or relieving the disease or its symptoms, and the patient was listed for heart transplantation. During the 7-month period on the waiting list, the patient was managed as an outpatient, with fortnightly albumin infusions and intravenous furosemide administration, which allowed her a better quality of life during that period, avoiding further admissions.
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Affiliation(s)
- Josep L Melero
- Servei de Medicina Digestiva, Hospital Universitari La Fe, Valencia, España.
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Protein-losing enteropathy during highly active antiretroviral therapy in a patient with AIDS-related disseminated Mycobacterial avium complex infection. J Infect Chemother 2009; 15:252-6. [DOI: 10.1007/s10156-009-0698-4] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2009] [Accepted: 04/20/2009] [Indexed: 11/25/2022]
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22
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 DOI: 10.1186/1750-1172-3-5%' and 2*3*8=6*8 and '3d5x'!='3d5x%] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 01/29/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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23
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 DOI: 10.1186/1750-1172-3-5'"] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 01/29/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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24
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 DOI: 10.1186/1750-1172-3-5'||'] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 01/29/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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25
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 DOI: 10.1186/1750-1172-3-5����%2527%2522\'\"] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 01/29/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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26
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 DOI: 10.1186/1750-1172-3-5" and 2*3*8=6*8 and "lme6"="lme6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 01/29/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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27
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 DOI: 10.1186/1750-1172-3-58ixwp1ha] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 01/29/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 DOI: 10.1186/1750-1172-3-5' and 2*3*8=6*8 and '9soh'='9soh] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 01/29/2024] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann's disease). Orphanet J Rare Dis 2008; 3:5. [PMID: 18294365 PMCID: PMC2288596 DOI: 10.1186/1750-1172-3-5] [Citation(s) in RCA: 162] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2007] [Accepted: 02/22/2008] [Indexed: 12/13/2022] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.
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Affiliation(s)
- Stéphane Vignes
- Department of Lymphology, Centre de référence des maladies vasculaires rares, Hôpital Cognacq-Jay, 15, rue Eugène Millon, 75015 Paris, France.
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Cole SL, Ledford DK, Lockey RF, Daas A, Kooper J. Primary gastrointestinal lymphangiectasia presenting as cryptococcal meningitis. Ann Allergy Asthma Immunol 2007; 98:490-2. [PMID: 17521035 DOI: 10.1016/s1081-1206(10)60765-x] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND Opportunistic infections commonly occur in immunocompromised patients; however, it is unusual for an adult to present with a combined cellular and humoral immunodeficiency. Cryptococcal meningitis is a fatal condition if untreated and is usually found in patients with cellular immunodeficiency. OBJECTIVE To report the case of an adult patient with cryptococcal meningitis secondary to intestinal lymphangiectasia. METHODS A 59-year-old man was admitted to the hospital for disseminated cryptococcal meningitis and osteomyelitis. Laboratory evaluation, computed tomography, esophagogastroduodenoscopy, and biopsy were performed. RESULTS Laboratory evaluation revealed a lymphopenia, hypoalbuminemia, hypogammaglobulinemia, and negative human immunodeficiency virus test results by enzyme-linked immunosorbent assay and polymerase chain reaction. The complete blood cell count, urinalysis, serum and urine protein electrophoresis, and functional antibody responses to protein and polysaccharide antigens were normal. Results of computed tomography of the chest, abdomen, and pelvis were unremarkable. Multiple lymphangiectasias were visualized with esophagogastroduodenoscopy and confirmed by biopsy. The patient was treated with intravenous amphotericin B and flucytosine, and the meningitis resolved. CONCLUSIONS Based on a computerized search of the medical literature, this is the first description of cryptococcal meningitis secondary to intestinal lymphangiectasias. The combination of lymphopenia, hypogammaglobulinemia, and hypoalbuminemia should alert the clinician to the possibility of intestinal lymphangiectasias and the potential for immune dysfunction.
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Affiliation(s)
- Steven L Cole
- Department of Medicine, Division of Allergy and Immunology, University of South Florida College of Medicine, James A. Haley Veterans' Hospital, Tampa, Florida 33612, USA.
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Fang YH, Zhang BL, Wu JG, Chen CX. A primary intestinal lymphangiectasia patient diagnosed by capsule endoscopy and confirmed at surgery: A case report. World J Gastroenterol 2007; 13:2263-5. [PMID: 17465517 PMCID: PMC4146860 DOI: 10.3748/wjg.v13.i15.2263] [Citation(s) in RCA: 32] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Intestinal lymphangiectasia (IL) is a rare disease characterized by dilated lymphatic vessles in the intestinal wall and small bowel mesentery which induce loss of protein and lymphocytes into bowel lumen. Because it most often occurs in the intestine and cannot be detected by upper gastroendoscopy or colonoscopy, and the value of common image examinations such as X-ray and computerized tomography (CT) are limited, the diagnosis of IL is difficult, usually needing the help of surgery. Capsule endoscopy is useful in diagnosing intestinal diseases, such as IL. We here report a case of IL in a female patient who was admitted for the complaint of recurrent edema accompanied with diarrhea and abdominal pain over the last twenty years, and aggravated ten days ago. She was diagnosed by M2A capsule endoscopy as a primary IL and confirmed by surgical and pathological examination.
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Affiliation(s)
- You-Hong Fang
- Department of Gastroenterlogy, The First Affiliated Hospital of College of Medicine, Zhejiang University, #79 Qingchun Road, Hangzhou 310003, Zhejiang Province, China
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Vignes S, Bellanger J. [Videocapsule endoscopy as a useful tool to diagnose primary intestinal lymphangiectasia]. Rev Med Interne 2006; 28:173-5. [PMID: 17229491 DOI: 10.1016/j.revmed.2006.11.019] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2006] [Accepted: 11/21/2006] [Indexed: 12/13/2022]
Abstract
INTRODUCTION Primary intestinal lymphangiectasia (Waldmann's disease) lead to a protein-losing enteropathy due to lymph leak into intestinal tract. EXEGESIS A 28-year-old woman presented a bilateral lower limb lymphedema. Laboratory examination showing lymphopenia, hypoalbuminemia, hypogammaglobulinemia suggested the diagnosis of primary intestinal lymphangiectasia. Gastroscopy was normal and second duodenum biopsies were negative. Videocapsule endoscopy gave evidence of intestinal lymphangiectasia of the small bowel. CONCLUSION Videocapsule endoscopy may be proposed to confirm intestinal lymphangiectasia and to precise their localization when gastroscopy is not conclusive.
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Affiliation(s)
- S Vignes
- Unité de Lymphologie, Hôpital Cognacq-Jay, 15, rue Eugène-Millon, 75015 Paris, France.
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33
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Baricault S, Soubrane JC, Courville P, Young P, Joly P. [Necrolytic migratory erythema in Waldmann's disease]. Ann Dermatol Venereol 2006; 133:693-6. [PMID: 17053741 DOI: 10.1016/s0151-9638(06)70994-2] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
BACKGROUND We report a case of necrolytic migratory erythema in a patient with Waldmann's disease. PATIENTS AND METHODS A 55-year-old male patient with a history of Waldmann's disease was hospitalized for a rash on the trunk and limbs comprising annular polycyclic lesions with peripheral scaling evocative of necrolytic migratory erythema. High-protein and fatty-acid-supplemented parenteral feeding led to rapid improvement of the patient's cutaneous lesions. DISCUSSION Waldmann's disease is characterized by intestinal lymphatic abnormalities leading to exudative intestinal disease causing protein loss in the bowel lumen and deficient fatty acid absorption. The pathogenesis of necrolytic migratory erythema is not fully understood. Increased serum glucagon does not appear to be the only mechanism involved. The occurrence of necrolytic migratory erythema in a patient with Waldmann's disease supports the current physiopathological hypothesis of the role of decreased plasma protein and amino acid levels in necrolytic migratory erythema.
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Affiliation(s)
- S Baricault
- Clinique Dermatologique, CHU de Rouen, France
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Biselli M, Andreone P, Gramenzi A, Cursaro C, Lorenzini S, Bonvicini F, Bernardi M. Acquired intestinal lymphangiectasia successfully treated with a low-fat and medium-chain triacylglycerol-enriched diet in a patient with liver transplantation. Eur J Gastroenterol Hepatol 2006; 18:561-564. [PMID: 16607157 DOI: 10.1097/00042737-200605000-00020] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Intestinal lymphangiectasia is defined as a dilatation of small bowel lymphatic capillaries and a loss of lymph into the bowel lumen. Clinically it is characterized by hypoproteinaemia and oedema. We present here a case of protein-losing enteropathy due to intestinal lymphangiectasia after liver transplantation in a 57-year-old man who was transplanted for hepatitis C virus. Four years after liver transplantation, the patient developed hypoalbuminaemia and ascites associated with recurrence of cirrhosis. The sudden fall in serum albumin led us to look for a cause of reduction other than or in addition to cirrhosis. Duodenal biopsies showed tall villi with dilated lymphatic vessels and widening of the villi caused by oedema, demonstrating intestinal lymphangiectasia. In this case a low-fat diet supplemented with medium-chain triacylglycerols achieved an early clinical improvement with increased serum albumin levels and ascites disappearance. Intestinal lymphangiectasia should be suspected in liver-transplanted patients developing hypoproteinaemia and hypoalbuminaemia after the recurrence of cirrhosis.
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Affiliation(s)
- Maurizio Biselli
- Semeiotica Medica, Dipartimento Medicina Interna, Cardioangiologia ed Epatologia, Università di Bologna, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy
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Aoyagi K, Iida M, Matsumoto T, Sakisaka S. Enteral nutrition as a primary therapy for intestinal lymphangiectasia: value of elemental diet and polymeric diet compared with total parenteral nutrition. Dig Dis Sci 2005; 50:1467-70. [PMID: 16110837 DOI: 10.1007/s10620-005-2863-7] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Intestinal lymphangiectasia (IL) is a rare disease requiring oral fat restriction. The aim of this study was to evaluate the efficacy of enteral nutrition compared to that of total parenteral nutrition (TPN). We retrospectively reviewed nine patients with IL presenting with protein-losing enteropathy. Of these, seven patients not responding to a low-fat diet were treated with elemental diet (ED), polymeric diet (PD) containing medium-chain triglycerides, or TPN. Improvement in serum total protein was observed in two of three on ED and in one of two on PD, compared with three of three on TPN. Enteric protein loss was improved in two of two on ED, one of two on PD, and two of two on TPN. Outpatients who continued to receive enteral nutrition maintained a total protein level. Enteral nutirition appears to be as effective as TPN for patients with IL, and it may provide a valid and safe alternative therapy.
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Affiliation(s)
- Kunihiko Aoyagi
- Third Department of Medicine, School of Medicine, Fukuoka University, Nanakuma, Fukuoka, Japan.
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36
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Lynn J, Knight AK, Kamoun M, Levinson AI. A 55-year-old man with hypogammaglobulinemia, lymphopenia, and unrelenting cutaneous warts. J Allergy Clin Immunol 2004; 114:409-14. [PMID: 15316525 DOI: 10.1016/j.jaci.2004.02.033] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
A 55-year-old white man with a history of hypertension, fibromyalgia, and colonic polyps presented with unrelenting plantar warts on his hands and feet for the past 4 years. He was otherwise healthy and without a history of recurrent infections. Physical examination was unremarkable except for extensive warts on his hands and feet. Pertinent laboratory findings included hypoalbuminemia, hypogammaglobulinemia, and lymphopenia most severely affecting CD4(+) T cells. Testing for HIV infection was negative. This clinical and laboratory presentation suggested a combined humoral and cellular immunodeficiency syndrome that could be best explained by loss of lymphocytes, immunoglobulins, and other serum proteins. Additional immunologic testing revealed a marked reduction in peripheral blood naive (CD4(+)CD45RA(+)) T cells. A 24-hour stool collection showed a markedly elevated alpha(1)-antitrypsin level. These findings were most consistent with the diagnosis of intestinal lymphangiectasia, a type of protein-losing enteropathy associated with hypoalbuminemia, hypogammaglobulinemia, and lymphopenia, characterized by a preferential loss of naive CD4(+) T cells into the gastrointestinal tract. This case illustrates the importance of considering intestinal loss of immunoglobulins and lymphocytes in the differential diagnosis of the adult patient who presents with laboratory evidence of a combined humoral and cellular immunodeficiency. It also underscores the diagnostic utility of the clinical immunology laboratory and how flow cytometry, in particular, can contribute to an understanding of pathogenic mechanisms.
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Affiliation(s)
- Jerry Lynn
- Pulmonary Allergy and Critical Care Division, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
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Abstract
A high-protein, low-fat diet supplemented with medium chain triglycerides (MCT) is the simplest, most effective, and most widely prescribed treatment with the fewest side effects. Octreotide has been helpful in cases in which treatment with MCT has failed, but it is costly and requires parenteral administration. Antiplasmin therapy may have some role when evidence of increased fibrinolysis is present. Surgery is reserved for palliation of large ascites or resection of isolated lesions.
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Affiliation(s)
- Charles M. Bliss
- Section of Gastroenterology, Department of Medicine, Boston Medical Center, D408, 88 East Newton Street, Boston, MA 02118, USA.
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38
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Klingenberg RD, Homann N, Ludwig D. Type I intestinal lymphangiectasia treated successfully with slow-release octreotide. Dig Dis Sci 2003; 48:1506-9. [PMID: 12924644 DOI: 10.1023/a:1024707605493] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Affiliation(s)
- R D Klingenberg
- The Division of Gastroenterology, Medical Clinic I, University of Lübeck, Lübeck, Germany
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39
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Holzknecht N, Helmberger T, Beuers U, Rust C, Wiebecke B, Reiser M. Cross-sectional imaging findings in congenital intestinal lymphangiectasia. J Comput Assist Tomogr 2002; 26:526-8. [PMID: 12218814 DOI: 10.1097/00004728-200207000-00009] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
A 37-year-old woman presented with an 18 month history of recurrent severe abdominal pain, recurrent diarrhea since her second year of life, and mild peripheral edema. CT and more clearly MRI revealed signs of small bowel and mesenterial edema with partial tubular appearance, which correlated well with the histopathologic findings shown in duodenal biopsy. This is the first report of MR findings in congenital intestinal lymphangiectasia with correlation with other imaging modalities.
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Affiliation(s)
- Nicolaus Holzknecht
- Department of Clinical Radiology, University Hospital of Munich, Munich, Germany.
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40
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MacLean JE, Cohen E, Weinstein M. Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy. Pediatrics 2002; 109:1177-80. [PMID: 12042562 DOI: 10.1542/peds.109.6.1177] [Citation(s) in RCA: 47] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/27/2022] Open
Abstract
Lymphangiectasia is a congenital or acquired disorder characterized by abnormal, dilated lymphatics with a variable age of presentation. We describe a case of lymphangiectasia with intestinal and pulmonary involvement in an adolescent female, who presented with many of the classic features including chylous pleural effusions, lymphopenia, hypogammaglobinemia, and a protein-losing enteropathy. She also presented with recurrent lower gastrointestinal bleeding, which is infrequently described. The patient did not improve with bowel rest and a low-fat medium-chain triglyceride diet and had little improvement with octreotide acetate therapy. However, she had a clinical response to antiplasmin therapy, trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid) in terms of serum albumin and gastrointestinal bleeding. She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions.
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Affiliation(s)
- Joanna E MacLean
- Division of Paediatric Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada M5G 1X8
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41
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Munck A, Sosa Valencia G, Faure C, Besnard M, Ferkdadji L, Cézard JP, Mougenot JF, Navarro J. [Long-term followup of primary intestinal lymphangiectasia in the child. Six case reports]. Arch Pediatr 2002; 9:388-91. [PMID: 11998426 DOI: 10.1016/s0929-693x(01)00799-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
UNLABELLED Primary intestinal lymphangiectasia induce symptoms of protein-losing gastroenteropathy. Only very few studies evaluate the long term follow up of such patients. We reviewed six children diagnosed at 17 +/- 12 months and followed for 11 +/- 4.9 years. CASE REPORTS As soon as the diagnosis was made the patients were submitted to a strict low fat diet with added medium chain triglycerides and intermittent liposoluble vitamins perfusions. The diet allowed the disappearance of symptoms for all the patients but laboratory findings indicated continuing chyle leak for most of the children. Only one child who had normal biological parameters tolerates a normal diet since four years. Relaxation of the diet by two patients who had moderate hypoalbuminemia and lymphopenia led to severe clinical relapses 14 and 17 years after the diagnosis period with therapeutic difficulties. Three patients with long term strict low fat diet remain asymptomatic. CONCLUSION In most asymptomatic patients, the underlying lymphatic defect remains with permanent biological abnormalities. Clinical relapses may be severe and difficult to treat; thus the need for dietary treatment appears to be permanent.
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Affiliation(s)
- A Munck
- Service de gastroentérologie et nutrition pédiatriques, hôpital Robert-Debré, 48, boulevard Sérurier, 75019 Paris, France.
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Uğuralp S, Mutus M, Kutlu O, Cetin S, Baysal T, Mizrak B. Primary intestinal lymphangiectasia: a rare disease in the differential diagnosis of acute abdomen. J Pediatr Gastroenterol Nutr 2001; 33:508-10. [PMID: 11698774 DOI: 10.1097/00005176-200110000-00019] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Affiliation(s)
- S Uğuralp
- Department of Pediatric Surgery, Turgut Ozal Medical Center, Malatya, Turkey.
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Antonetti M, Manuck TA, Schramm C, Hight D. Congenital pulmonary lymphangiectasia: a case report of thoracic duct agenesis. Pediatr Pulmonol 2001; 32:184-6. [PMID: 11477737 DOI: 10.1002/ppul.1106] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
We present a 17-year-old Caucasian male with congenital pulmonary lymphangiectasia and an absent thoracic duct. This patient is unique as he did not present with the disorder until age 9.5 years. Since his initial presentation he has had recurrent chylothoraces and has been treated symptomatically. We discuss the possible implications of his disorder as well as some of the limited treatment that is available.
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Affiliation(s)
- M Antonetti
- Department of Pediatric Surgery and Urology, Connecticut Children's Medical Center, University of Connecticut School of Medicine, Farmington, Connecticut 06106, USA
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Landzberg BR, Pochapin MB. Protein-Losing Enteropathy and Gastropathy. CURRENT TREATMENT OPTIONS IN GASTROENTEROLOGY 2001; 4:39-49. [PMID: 11177680 DOI: 10.1007/s11938-001-0045-z] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
The diagnosis of protein-losing enteropathy (PLE) should be considered in all patients with hypoalbuminemia and edema without other known causes, and established by plasma alpha(1)-antitrypsin (alpha(1)-AT) clearance or nuclear studies. The therapy for PLE should focus principally on the treatment of the underlying disease after it has been identified. Therapeutic goals should include improvement of hypoalbuminemia, edema, and lymphopenia. The existing primary literature for therapy of PLE syndromes consists mainly of case reports and expert opinions, subject to substantial reporting bias and unknown rates of spontaneous remission; the rarity of and the diversity among this set of diseases make future large randomized trials unlikely. Therapeutic choices, therefore, must involve clinical acumen, empiricism, and understanding of the pathophysiology of the underlying disease process, and must be tailored to each individual patient's syndrome. Dietary interventions including hypolipidic, high-protein regimens, supplemented by medium-chain triglycerides (MCTs), are extremely useful, particularly in protein loss due to increased lymphatic pressure. Corticosteroids can be very useful in certain cases of PLE (though not without substantial long-term toxicity) when clinical serologic or histologic markers of inflammatory disease are present. Octreotide is a well tolerated drug that has been demonstrated to improve PLE in some patients, and is worth consideration. Octreotide is a well tolerated drug that has been demonstrated to improve PLE in some patients, and is worth consideration. Surgery finds its best role in treating gastrointestinal protein loss from neoplasia, inflammatory bowel disease, and hypertrophic gastritis. Most other PLEs are distributed too widely for surgical intervention. Protein-losing gastropathy (PLG) behaves somewhat differently from the general group of PLE, marked by excellent responses to elimination of Helicobacter pylori, antisecretory therapy, and surgical resection. Protein-losing enteropathy stemming from cardiovascular disease is best treated by medical or surgical cardiovascular interventions; however, some patients may respond to mucosa-directed therapy.
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Affiliation(s)
- Brian R. Landzberg
- Division of Gastroenterology and Hepatology, Department of Medicine, Weill Medical College of Cornell University and New York-Presbyterian Hospital, 525 East 68th Street, F-231, New York, NY 10021, USA
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45
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Rust C, Pratschke E, Hartl W, Kessler M, Weibecke B, Sauerbruch T, Paumgartner G, Beuers U. Fibrotic entrapment of the small bowel in congenital intestinal lymphangiectasia. Am J Gastroenterol 1998; 93:1980-3. [PMID: 9772070 DOI: 10.1111/j.1572-0241.1998.00477.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Congenital intestinal lymphangiectasia is a rare protein-losing enteropathy that is characterized by diarrhea and peripheral edema. This report presents a 37-yr-old woman who had suffered from recurrent diarrhea and peripheral edema since her early childhood and who was admitted for severe attacks of abdominal pain. A diagnosis of intestinal lymphangiectasia was made endoscopically, histologically, and radiographically. Laparotomy revealed complete fibrotic entrapment of the small bowel, which caused partial mechanical bowel obstruction. Surgical decortication led to recovery. To the best of our knowledge, this is the first report on fibrotic entrapment of the small bowel in a patient with long lasting intestinal lymphangiectasia.
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Affiliation(s)
- C Rust
- Department of Medicine II, Klinikum Grosshadern, University of Munich, Germany
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46
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Craig GB, Darnell BE, Weinsier RL, Saag MS, Epps L, Mullins L, Lapidus WI, Ennis DM, Akrabawi SS, Cornwell PE, Sauberlich HE. Decreased fat and nitrogen losses in patients with AIDS receiving medium-chain-triglyceride-enriched formula vs those receiving long-chain-triglyceride-containing formula. JOURNAL OF THE AMERICAN DIETETIC ASSOCIATION 1997; 97:605-11. [PMID: 9183320 DOI: 10.1016/s0002-8223(97)00155-7] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
OBJECTIVE The purpose of this study was to compare two enteral formulas, differing only in fat source, for product acceptance, tolerance, and effect on fat malabsorption and nutritional status in subjects with acquired immune deficiency syndrome (AIDS). DESIGN The double-blind, randomized 15-day trial was divided into a 3-day period in which solid food was consumed followed by a 12-day experimental period in which liquid formulas were consumed. SETTING/SUBJECTS Twenty-three men and one woman with AIDS and fat malabsorption completed the study. The study was conducted in the General Clinical Research Center, University of Alabama Hospital, University of Alabama at Birmingham. Laboratory assays were performed in the Department of Nutrition Sciences. INTERVENTIONS After 3 days of consuming a controlled, solid food diet containing 100 g fat per day from mixed sources to document fat malabsorption, subjects were randomly assigned to one of two groups. Each group received a liquid formula containing 35% of energy as fat for 12 days. One group received a formula containing 85% medium-chain triglycerides (MCTs) and the control group received a formula containing 100% long-chain triglycerides. MAIN OUTCOME MEASURES Determinations included stool number, consistency, weight, and fat and nitrogen content; urine nitrogen and creatinine levels; and body weight. STATISTICAL ANALYSIS PERFORMED Subject demographic and other baseline characteristics were compared using two-sample t tests; stool and urine assessments were compared between groups at the initial experimental period using two-sample t tests; changes from initial to final experimental periods were assessed by means of analysis of covariance; changes in pooled intake, body weight, and the number and consistency of bowel movements were also assessed using analysis of covariance. All statistical tests were two-tailed and considered significant at P < .05. RESULTS Within-group comparisons indicated that subjects fed the MCT formula showed significantly decreased stool fat and stool nitrogen content (P = .01 and P = .03, respectively) and increased fat absorption (P = .03), whereas those fed the control formula did not. Differences in stool fat between the groups were not statistically significant. However, the difference in fat absorption from the initial to final formula period was significant (P = .04). Subjects consuming the MCT formula also tended to have a decreased number of bowel movements and abdominal symptoms, whereas subjects fed the control formula showed no improvement. All subjects maintained their body weights. APPLICATIONS There may be advantages to using an MCT-based formula in the treatment of AIDS-associated malabsorption.
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Affiliation(s)
- G B Craig
- Department of Nutrition Sciences, School of Health Related Professions, University of Alabama at Birmingham 35294, USA
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47
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Feldt RH, Driscoll DJ, Offord KP, Cha RH, Perrault J, Schaff HV, Puga FJ, Danielson GK. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 1996; 112:672-80. [PMID: 8800155 DOI: 10.1016/s0022-5223(96)70051-x] [Citation(s) in RCA: 213] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%. Hemodynamic studies done coincident with the diagnosis of protein-losing enteropathy have shown increased systemic venous pressure, decreased cardiac index, increased pulmonary vascular resistance, and increased ventricular end-diastolic pressure. Medical management of protein-losing enteropathy was only partially successful. Statistical analysis has shown that factors related to protein-losing enteropathy were ventricular anatomy, increased preoperative ventricular end-diastolic pressure, longer operative bypass time, increased length of hospital stay, and postoperative renal failure. This study suggests that scrupulous selection of cases for the Fontan operation is mandatory and that certain perioperative factors may predispose to this serious complication of the Fontan procedure.
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Affiliation(s)
- R H Feldt
- Section of Pediatric Cardiology, Mayo Clinic, Rochester, Minn 55905, USA
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Abstract
BACKGROUND Nutrient modules are commonly used by pediatricians and dietitians. There is no readily available current literature to which those using nutrient modules can refer. Therefore, we review nutrient module composition, module use in pediatrics, and complications associated with the use of nutrient modules. METHODS Using an online database, Meduline, we searched the literature from 1996 through 1995. RESULTS Nutrient modules are single or multiple nutrients that can be combined with a diet to add nutrients or to change the composition of the diet. These nutrients exist as a food or as medically compounded elements of a diet but alone are not complete foods. DISCUSSION The addition of modules to a diet can alter the composition of a diet so that a single nutrient may become deficient and the diet cannot support normal growth and development. With use of nutrient modules, diets can be prepared to meet the specific needs of children with common or rare nutritional problems. CONCLUSION Health care providers who use nutrient modules must understand the importance of diet composition and the careful monitoring of pediatric patients.
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Affiliation(s)
- A Davis
- Department of Pediatrics, Medical University of South Carolina, Charleston 29403, USA
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49
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Lenzhofer R, Lindner M, Moser A, Berger J, Schuschnigg C, Thurner J. Acute jejunal ileus in intestinal lymphangiectasia. THE CLINICAL INVESTIGATOR 1993; 71:568-71. [PMID: 8374252 DOI: 10.1007/bf00208483] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
A 26-year-old patient presented with epigastric pain of sudden onset and severe puffy swelling of both legs and forearms. An irregularly shaped nodular filling defect on selective jejunal films, severe hypoproteinemia, low IgG concentration, and lymphopenia were suggestive of primary intestinal lymphangiectasia with protein-losing enteropathy, and the patient was placed on a low-fat diet with medium-chain triglycerides. This initially improved his condition, but some weeks later he developed obstructive ileus of the small intestine. On laparotomy yellowish to whitish deposits were found to be present in some segments of the small intestine and a fist-sized mass 100 cm distal to the duodenojejunal flexure was resected without complications. Histologically, the submucosal lymphatics were dilated, and the jejunal wall showed extensive pseudocystic, intramural submucosal lymph edema with secondary bleeding and tight stenosis of the jejunal lumen. During the 14-month follow-up time after discharge the patient has been asymptomatic and working, on no treatment other than a low-fat diet with medium-chain triglycerides.
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Affiliation(s)
- R Lenzhofer
- Interne Abteilung, Kardinal Schwarzenberg'sches Krankenhaus Schwarzach
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50
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Abdullah AM, Abdullah MA, Yohannan MD, Al Herbish AS, Al Rabeeah A, Cheriyan M. Infantile intestinal lymphangiectasia and chylous ascites in Saudi Arabia. Ann Saudi Med 1993; 13:306-9. [PMID: 17590685 DOI: 10.5144/0256-4947.1993.306] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Affiliation(s)
- A M Abdullah
- Department of Pediatrics, College of Medicine, and King Khalid University Hospital and Suleimania Children's Hospital, Riyadh, Saudi Arabia
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