Waldmann’s Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect
Waldmann’s disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia and lymphopenia. Secondary...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Private Limited
2017-04-01
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| Series: | Journal of Clinical and Diagnostic Research |
| Subjects: | |
| Online Access: | https://jcdr.net/articles/PDF/9742/24989_CE[Ra1]_F(DK)_PF1(PG_RK)_PFA(PG_SS).pdf |
| Summary: | Waldmann’s disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics
and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia
and lymphopenia. Secondary lymphangiectasia occurs secondary to an elevated lymphatic pressure as in lymphoma, systemic lupus
erythematosus, constrictive pericarditis, cardiac surgeries (Fontan’s procedure), inflammatory bowel disease and malignancies. We,
hereby present a five-year-old male child who presented with abdominal distension and poor weight gain. He had hypoalbuminemia,
lymphocytopenia and hypogammaglobulinemia. Upper gastrointestinal endoscopy showed normal gastric mucosa and punctate white
lesions in duodenal mucosa with biopsy confirming intestinal lymphangiectasia. Secondary causes of intestinal lymphangiectasia were
ruled out. Echocardiography revealed atrial septal defect which is an uncommon association with Waldmann’s disease. He was started
on low fat, high protein diet and medium chain triglyceride supplementation following which he improved symptomatically. High index
of suspicion, early diagnosis and appropriate dietary treatment are necessary to alleviate symptoms as well as to achieve a sustainable
growth and development in these children. |
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| ISSN: | 2249-782X 0973-709X |