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A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl

Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion,...

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Detalles Bibliográficos
Autores principales: Najjar, Afaf Mohammad Maher, Babensi, Nour Khaled, Ghazal, Ahmad, Brimo Alsaman, Muhamad Zakaria, Ismail, Alaa aldin, Alnaeb, Hasnaa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10558647/
https://www.ncbi.nlm.nih.gov/pubmed/37808573
http://dx.doi.org/10.1002/ccr3.7980
Descripción
Sumario:Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion, or chylous ascites. The goal of the treatment is to restrict the consumption of long‐chain fats and administer a formula that includes protein and medium‐chain triglycerides. Here, we report 11‐year‐old Syrian girl presented to the hospital with 2 years history of diarrhea and generalized edema. Furthermore, investigation showed ascites and pleural effusion. The disease was detected by several biopsies from duodenum that showed dilation of the lymph vessels within the lamina propria without any evidence for inflammation. She was managed by modifying her diet and albumin transfusion, which caused significant improvement in the child's condition.