Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty infants with obstructive jaundice were reviewed. Other than jaundice, hepatomegaly and splenomegaly were the other common presentations. The mean age of referral was 9.4 weeks. This was unsatisfactory as the prognosis depends on the age of corrective surgery. Majority of the non surgical cholestasis were due to idiopathic hepatitis. These patients did well as all of them were asymptomic and had no evidence of liver disease at 2 years of age.
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PMID:A clinical study of obstructive jaundice among Singapore infants. 130 62

Some clinical clues should alert paediatricians to the possibility of metabolic liver diseases. They can be classified into three categories: (i) Manifestations due to hepatocellular necrosis, acute or subacute, which can reveal galactosaemia, hereditary fructose intolerance, tyrosinaemia type I, Wilson disease and alpha 1-antitrypsin deficiency. Symptoms and signs suggestive of Reye syndrome should lead to a study of fatty acid oxidation and urea cycle enzymes. All these manifestations may necessitate a rapid diagnosis and treatment when liver dysfunction is severe. (ii) Cholestatic jaundice can reveal alpha 1-antitrypsin deficiency, Byler's disease, cystic fibrosis, Niemann-Pick disease and some disorders of peroxisome biogenesis. (iii) Hepatomegaly can reveal disorders with liver damage but also storage diseases such as glycogen storage diseases, cholesteryl ester storage disease and, when associated with splenomegaly, lysosomal storage diseases. Appropriate investigations for recognizing all these entities are proposed.
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PMID:Clinical presentation of metabolic liver disease. 174 17

Biliary mucinous cystadenocarcinoma is an extremely rare tumour. Less than 50 cases have been reported. It is usually a multilocular cystic tumour covered with mucous producing epithelium, with papillary excrescences containing mucinous mass arising from bile ducts. The size of the tumour varies from 3.5 to 25 cm in diameter. It is more frequent in women. The majority of patients belong to the middle age population. We present a 63-year-old man who had been suffering from an epigastric and right subcostal pain of unknown aetiology for over 35 years. During the last 10 years he suffered from multiple attacks of cholangitis with high temperature, rigor, chills, pain and obstructive jaundice. Five years ago he had the attack of pancreatitis with retroperitoneal fatty necrosis for which he was operated on in another institution and cholecystectomy and pancreatic necrectomy were carried out. The attacks of cholangitis continued they were more serious and more frequent until June 1987, when the "cyst" in the left lobe of the liver, dilated bile ducts and "polyps" in the common bile duct were diagnosed by ultrasonography. During the operation advanced biliary cirrhosis, portal hypertension, splenomegaly, very dilated common bile duct full of jelly and the "cyst" in the liver filled with jelly, were found. The removal of the jelly and choledochojejunostomy resulted in temporary relief. Two months later he was reoperated for recurrent obstructive jaundice during which left lobectomy, partial excision of the cyst and cystojejunostomy between the rest of the cyst and another Roux-en-Y jejunal limb, were carried out.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Biliary mucinous cystadenocarcinoma of the liver]. 221 37

Massive hematemesis followed by 48 hours the onset of obstructive jaundice in a previously asymptomatic 38-year-old male patient. Splenomegaly was the only abnormal physical finding. Esophagoscopy visualized bleeding varices. The liver scan was normal. Dilated intra- and extrahepatic bile ducts and a possible retroperitoneal mass were demonstrated by ultrasonography. Laparotomy disclosed the presence of a carcinoma of the head and body of the pancreas that obstructed the common bile duct and the portal vein. This case report should draw attention to the vast spectrum of rare presentations of pancreas carcinoma.
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PMID:Portal hypertension as an early manifestation of carcinoma of the pancreas. 407 11

To observe clinical results of continuous therapy with albendazole for hepatic alveolar echinococcosis associated with obstructive jaundice, we treated 6 patients continuously with albendazole at a dosage of 20 mg.kg-1.d-1. Jaundice disappeared and serum bilirubin level returned to normal within 1-2.5 months in the patients. Ultrasound and/or CT scanning of liver before treatment displayed irregular heterogenous lesions, obscure hilar region with marked intrahepatic biliary dilatation. In one severe jaundiced patient, the common bile duct and the pancreatic duct were dilated and the portal and splenic veins were also distended with marked splenomegaly. Two patients were followed up for 3-9 year after cessation of treatment, their hepatic lesions were almost completely calcified and considered cured. The other four patients were still subjected to treatment one year later. CT and ultrasound scanning showed marked improvement of intrahepatic lesions and disappearance of intrahepatic biliary dilatation. Liver function tests returned to normal except that serum globulin remained elevated in 2 cases. Clinical observation and CT scanning of liver indicated that continuous therapy with albendazole is effective in the treatment of hepatic alveolar echinococcosis associated with obstructive jaundice. No adverse side reactions were seen.
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PMID:[Continuous therapy with albendazole for hepatic alveolar echinococcosis associated with obstructive jaundice]. 938 44

Malignant lymphoma rarely presents with jaundice. We describe a patient who had a unique etiology for painless jaundice, dilated ducts, and a normal ampulla of Vater. A Whipple's procedure was performed for the suspicion of pancreatic cancer, and initial pathological review detected only mild focal chronic pancreatitis. Seven months later, the patient developed ascites, retroperitoneal mass, and splenomegaly caused by a T-cell lymphoma. Reevaluation of the Whipple's specimen revealed previously unrecognized microscopic infiltration and fibrosis of the sphincter of Oddi by atypical T-lymphocytes. Obstructive jaundice caused by a clinically undetectable primary duodenal T-cell lymphoma has not been previously reported and is contrasted with other causes of jaundice associated with malignant lymphoma and ampullary lesions.
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PMID:Jaundice caused by a clinically undetectable T-cell lymphoma infiltrating the sphincter of Oddi. 1172 70

Two cases of Polycystic hydatid disease (PH) are reported from the same municipal district of the Brazilian Amazon region (Sena Madureira, Acre). Both had a similar clinical presentation and course over two years of follow-up. Initially the patients complained of pain in the right hypochondrium or upper abdomen and presented obstructive jaundice, fever, increased abdominal volume and weight loss. By image analysis, in addition to splenomegaly, multiple and coalescent cysts were detected in the liver. Serum samples were reactive by counterimmunoelectrophoresis. Treatment with albendazole resulted in partial improvement, with symptomatic relief and reduction in size of the lesions. This report stresses the importance of performing clinical-epidemiological studies of polycystic hydatid disease in the Brazilian Amazon and especially in the municipality of Sena Madureira where many other cases of PH may remain undiagnosed.
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PMID:[Polycystic hydatid disease: report of two cases from the city of Sena Madureira, Acre, in Brazilian Amazon]. 1271 68

Imaging findings of secondary hepatic lymphoma have been reported as variable, ranging from single or multiple small nodules to diffuse infiltrative tumor patterns. We hear present a rare case report concerning aggressive B cells, secondary Burkitt's lymphoma in non-AIDS demonstrating a surprising periportal lymphoma infiltration, without upper abdominal lymphadenopathy or splenomegaly on the sonography and CT scans. Clinically, the case was characterized by atypical and highly aggressive course, with the patient presenting an abruptly developed obstructive jaundice with rapidly deteriorating hepatic function that could be indicative of cholestatic hepatitis, which differs in its clinical manifestations from hepatic lymphoma without functional deterioration in respect of its non-tissue destructive growth pattern. We suggest that hepatic lymphoma can sometimes be consistent with periportal infiltrating homogeneous mass, with no lymphadenopathy or splenomegaly on the imaging examination, with a predictable aggressive clinical course of the disease and poor prognosis.
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PMID:Burkitt's lymphoma representing periportal infiltrating mass on CT. 1534 16

We treated a young man with obstructive jaundice, in whom multiple hepatic peribiliary cysts were detected at autopsy. He had been diagnosed with von Recklinghausen's disease in early childhood and had undergone surgery for a ventricular septal defect with massive blood transfusion at the age of 4 years. Examination at the age of 21 revealed prominent splenomegaly and a low platelet count. He underwent splenectomy and liver cirrhosis was confirmed by open biopsy of the liver. He was followed up at a local hospital and was admitted several times. When he was 41 years old, he was transferred to our hospital with severe jaundice and hepatic encephalopathy. Computed tomography showed marked dilation of the intrahepatic bile ducts and liver function tests showed that jaundice was mainly due to an increase of direct bilirubin. Despite endoscopic nasobiliary drainage, plasma exchange, and continuous hemodiafiltration, he died of hepatic failure after 5 days. At autopsy, multiple hepatic peribiliary cysts were found. Although peribiliary cysts have generally been considered to cause no symptoms, this report describes a patient with multiple hepatic peribiliary cysts which appear to have been responsible for the progression of obstructive jaundice.
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PMID:A case of multiple hepatic peribiliary cysts which contributed to the obstructive jaundice and led to liver failure at the young man with von Recklinghausen's disease. 1673 Feb 25

Autoimmune pancreatitis is a recently characterized disease that still constitutes a diagnostic challenge, especially regarding differential diagnosis from neoplasia. Long-term outcome is poorly known. We herein report a case of a patient with autoimmune pancreatitis and 14 years of follow-up, and show its clinical, biochemical, and morphological characteristics. A 54-year-old female presented with obstructive jaundice and abdominal tenderness, as well as a mass at the pancreatic head on a CT scan, suggestive of pancreatic neoplasia. Surgery showed an increase of the whole pancreas, malignancy was intraoperatively ruled out, and a cholecystectomy and choledochoduodenostomy were carried out. The diagnosis was chronic pancreatitis. Over the following years different autoimmune complications developed, including asthma, salivary gland swelling, and sclerosing cholangitis, as well as recurrent episodes of jaundice, and exocrine and endocrine pancreatic failure. The development of these complications combined with the demonstration of high serum levels of IgG4 and carbonic anhydrase II led to a re-evaluation of the initial histology of the pancreas, leading to a final diagnosis of autoimmune pancreatitis: IgG4+ lymphoplasmacytic infiltrates, fibrosis, and obliterative phlebitis. New complications developed during the last few years: retroperitoneal fibrosis with portal hypertension, esophageal varices, and splenomegaly.
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PMID:[Autoimmune pancreatitis: inflammatory pseudotumor, multifocal fibrosclerosis, portal hypertension, and long-term outcome]. 1911 94


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