The authors report the observation of a primary intestinal lymphangiectasy diagnosed on a young girl sent for isolated edema of her inferior members, recently appeared. Clinical examination was normal. Biology found a low protein rate at 33 g/l and a low lymph rate : 183 L/mm3. Hepatic and renal records were normal. Test to marked albumin asserted the exsudative enteropathy with a fecal radio-activity of 3.6% (N 1%). Biopsy of the small intestines set out lymphangiectasies of the intestinal mucosa. Referring to this observation and to literature date, the authors realise a clinical and physiopathological analysis of Waldmann's disease or primary intestinal lymphangiectasy.
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Key Clinical Message: Rare yet significant, this case sheds light on the uncommon presentation of Waldmann's disease in adults, showcasing the diagnostic challenges it poses. A multidisciplinary approach, integrating clinical, endoscopic, histological, and radiological evaluations, is crucial for accurate diagnosis and management. Further research is needed to deepen our understanding of this complex disorder.
View Article and Find Full Text PDFKey Clinical Message: Adequate evaluation of patients with Hennekam Syndrome (HS) is challenging for physicians, because of multi-organ involvement and complex pathophysiology. We report the first case in an African American with lymphedema, who developed protein-losing enteropathy (PLE) and was successfully diagnosed with HS from cause-and-effect complications by Waldmann's Disease (WD) and comorbid Celiac Disease (CD).
Abstract: As far as we know, this is the 51st case of HS worldwide and the first one in an African American.
Cryptococcal meningitis and cerebral vasculitis in a patient with primary intestinal lymphangiectasia: a case report.
Eur J Clin Microbiol Infect Dis
October 2023
Department of Infectious Diseases, Hôpital Lariboisière, Saint-Louis-Lariboisière-Fernand Widal Hospitals, AP-HP, 2 Rue Ambroise Paré, 75010, Paris, France.
Primary intestinal lymphangiectasia (Waldmann's disease) is a rare exudative enteropathy without precisely assessed infectious risk. We report the case of a 49-year-old male patient with meningitis and cerebral vasculitis due to Cryptococcus neoformans complicating Waldmann's disease diagnosed 12 years ago. The treatment combined liposomal amphotericin B, 3 mg/kg daily plus flucytosine 25 mg/kg/6 h, both intravenously during 15 days, then fluconazole 800 mg daily during 8 weeks, and finally 200 mg daily indefinitely.
View Article and Find Full Text PDFWaldmann'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.
View Article and Find Full Text PDFHypomagnesemia in intestinal lymphangiectasia: a case report and review of the literature.
BMC Gastroenterol
May 2022
Department of Gastroenterology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.
Background: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed.
Case Presentation: A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL.
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