Chylous ascites and lymphangiectasia in focal segmental glomerulosclerosis – a rare coexistence: a case report
نویسندگان
چکیده
INTRODUCTION Nephrotic syndrome is considered a rare cause of chylous ascites. Intestinal lymphangiectasia in a background of chylous ascites and without any lymphatic obstruction has been reported in association with yellow nail syndrome, which is a rare clinical occurrence in itself. The existence of chylous ascites, duodenal and splenic lymphangiectasia (without any lymphatic obstruction) and nephrotic syndrome in the form of focal segmental glomerulosclerosis in the same patient makes this case the first of its kind to be reported in the literature. CASE PRESENTATION Here we report the case of a 54-year-old Asian man who presented with recurrent episodes of anasarca for approximately 25 years. He was subsequently found to have chylous ascites, lymphangiectasia and persistent proteinuria. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified. A lymphangiogram, which was performed with the purpose of addressing the intestinal lymphangiectasia, failed to demonstrate any abnormality of lymphatic channels. He was put on oral steroids with consequent remission of his oedema and proteinuria. CONCLUSIONS This case highlights the fact that duodenal and splenic lymphangiectasia can exist in a scenario of chylous ascites without any obvious obstruction of lymphatic channels and in the absence of yellow nail syndrome. This case also signifies that chylous ascites may be a rare presenting feature of nephrotic syndrome and hence this aspect should be considered while in diagnostic dilemma regarding such a clinical presentation.
منابع مشابه
A rare case of chylous ascites and chyluria in an adult nephrotic syndrome with focal segmental glomerulosclerosis.
The authors report the first case of chylous ascites and chyluria in a 65-year-old Thai women with nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS), tip variant. She presented with generalized edema and abdominal discomfort. Abdominal paracentesis revealed milky white fluid. Chylous ascites was confirmed. Abdominal and thoracic computed tomography did not show any cause of ch...
متن کاملA Case Report of Schimke Immuno-Osseous Dysplasia: A Rare Autosomal Recessive Disorder
Schimke immune-osseous dysplasia (SIOD) is a rare autosomal recessive disorder presented with specific facial features, skeletal dysplasia, steroid resistance nephrotic syndrome (SRNS) and cellular immune insufficiency. This is a SIOD case reported from Iran. He was 5 years old boy when evaluated for proteinuria and short stature. In appearance, we detected hyperpigmented macules, kyphosc...
متن کاملSuccessful resection of localized intestinal lymphangiectasia post-Fontan: role of (99m)technetium-dextran scintigraphy.
Intestinal lymphangiectasia is a well-recognized complication of the Fontan procedure, occurring in up to 24% of patients. Because of the loss of chylous fluid into the gut lumen, protein-losing enteropathy results as well as lymphopenia and hypogammaglobulinaemia. In some cases, dilated lymphatics in the intestinal serosa or mesentery also rupture, causing chylous ascites. Standard medical and...
متن کاملIntestinal lymphangiectasia: a forgotten cause of chronic diarrhea.
Intestinal lymphangiectasia is a rare autosomal dominant disorder or acquired condition that leads to lymph obstruction, poor chyle transport and concomitant problems. We describe the cases of two women with chronic diarrhea in whom the common signs of lymphagiectasia-hypoalbuminemia, lymphopenia and distal edema- were found. One of them also had pleural effusion and chylous ascites. The diagno...
متن کاملCollapsing glomerulopathy in sickle cell disease: a case report
INTRODUCTION Sickle cell disease has been associated with many renal structural and functional abnormalities. Collapsing glomerulopathy or the collapsing variant of focal segmental glomerulosclerosis is a rare clinicopathologic entity in patients with sickle cell disease that requires timely diagnosis and aggressive management. CASE PRESENTATION In this case report we describe a 21-year-old A...
متن کامل