Gauchers disease presenting with portal hypertension.
نویسندگان
چکیده
Gauchers disease is a rare lysosomal storage disorder characterized by abnormal accumulation of lipid-laden macrophages in different organs. Though hepatosplenomegaly is commonly found, symptomatic presentation with portal hypertension is rare. We report a child with liver cirrhosis and bleeding esophageal varices who was diagnosed with Gaucher's disease.
منابع مشابه
SURGICAL EXPERIENCE WITH PORTAL HYPERTENSION DUE TO HEPATOPORTAL SCLEROSIS (NONCIRRHOTIC PORTAL FIBROSIS)
Hepatoportal sclerosis or idiopathic portal hypertension has a worldwide distribution with prevalence in developing and temperate countries. Of 64 patients with this disease seen during a twelve year period, 36 underwent splenectomy or a shunt procedure. The indications for surgical intervention were severe hypersplenism and persistent left upper abdominal pain andlorhistory of frequent ep...
متن کاملگزارش یک مورد توده کاذب مدیاستن در یک بیمار سیروتیک
We report a case of paraesophageal varices presenting as a posterior mediastinal mass in a patient with long-standing portal hypertension and cirrhosis. The case is unusual because the varices were considerably larger than those usually encountered in portal hypertension. This report is a reminder that portal hypertension with paraesophageal varices can present as a retrocardiac mass on...
متن کاملCongenital Absence of the Portal Vein Presenting as Pulmonary Hypertension
malformation in which intestinal and splenic venous flow bypasses the liver and drains directly into the systemic circulation via a congenital portosystemic shunt. Variable associated anomalies and clinical manifestations caused by the disrupted enterohepatic circulation in CAPV have been reported, including congenital cardiac anomalies, skeletal and visceral anomalies, hepatic neoplasms, liver...
متن کامل[A case of gastric adenocarcinoma presenting as portal hypertension].
Portal vein thrombus has been detected in patients with liver cirrhosis, pancreatitis, ulcerative colitis, septicemia, myeloproliferative disorder, and neoplasm. The formation of portal tumor thrombus by hepatocellular carcinoma is well recognized, because of its high incidence, and subsequent development of portal hypertension such as rupture of varices, ascites and liver failure indicates the...
متن کاملVACTERL (vertebral anomalies, anal atresia or imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal and limb defect) spectrum presenting with portal hypertension: a case report
INTRODUCTION We report for the first time a unique case of VACTERL (vertebral anomalies, anal atresia or imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal and limb defect) spectrum associated with portal hypertension. The occurrence of both VACTERL spectrum and extrahepatic portal hypertension in a patient has not been reported in the literature. We examined whether or not t...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Indian pediatrics
دوره 48 10 شماره
صفحات -
تاریخ انتشار 2011