Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ultrasonography is the first line imaging investigation in patients with jaundice, right upper quadrant pain, or hepatomegaly. Most patients with jaundice have parenchymal or obstructive jaundice. A 56-year old man was admitted to the Institute of Digestive Diseases, Belgrade, with severe pains in the epigastrium, vomiting, icteric coloration of sclera, white stools and dark urine. The symptoms had developed 5 days after a Billroth II resection. The functional liver test indicated a pronounced cholestasis. Real-time ultrasonography showed in the projection of the pancreas head, a clear bordered unechogenic oval formation, with a size of 40 x 35 mm. This finding indicated a possibility of an afferent loop obstruction, accompanied by consecutive obstructive icterus due to compression on the common bile duct. Ultrasonographic examination was followed by surgical reintervention. With the development of noninvasive imaging, transcutaneous ultrasonography, computed tomography and magnetic resonance imaging, the diagnosis of pancreatic and other cysts in upper abdomen is usually straightforward. The important ultrasonographic characteristic of empty organs, compared to cysts and pseudocyts, is a change in the morphology and size of organs after a specific time interval. Air bubbles in the organs lumen are like mobile reflectors, and they are an important sign in identifying segments of the gastrointestinal tract.
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PMID:Jaundice after Billroth II resection. A case report. 1236 42

Amiodarone is a commonly used anti-arrhythmic in elderly patients. Abnormal liver function is frequently reported with its use but clinically symptomatic disease is rare. Hepatomegaly, cholestasis, acute hepatitis and rarely fulminant liver failure have been recorded [1, 2], however amiodarone toxicity presenting with cirrhosis is exceedingly rare. Toxic effects of amiodarone are well described with higher dosage but severe hepatic toxicity and cirrhosis with low dose amiodarone has not been reported in the English language literature. We present a report on a patient with pseudo-alcoholic cirrhosis with low dose amiodarone.
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PMID:Low dose amiodarone causing pseudo-alcoholic cirrhosis. 1496 Apr 46

Ten children (4.6%) among a cohort of 219 with Kawasaki disease (KD) had their onset with severe abdominal complaints. Incomplete KD presentation at the time of acute abdomen was present in nine of 10 patients. Acute abdominal pain and distension, vomiting, hepatomegaly, and jaundice were the most common symptoms at onset. Hematemesis was present in one; toxic shock syndrome requiring care in the intensive care unit occurred in four. Five patients had laparotomy, three had percutaneous transhepatic biliary drainage, and one had a gastrointestinal endoscopy. Postoperative diagnosis was gallbladder hydrops with cholestasis in five, paralytic ileus in three, appendicular vasculitis in one, and hemorrhagic duodenitis in one. All patients completely recovered, but 50% developed coronary aneurysms despite early intravenous gammaglobulin treatment. Acute surgical abdomen can be the presenting manifestation of KD. In older children with fever, rash, and acute abdominal pain or hematemesis, KD should be considered in the differential diagnosis.
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PMID:Acute surgical abdomen as presenting manifestation of Kawasaki disease. 1283 7

We present the case of a 70-year-old woman who had been suffering from constitutional syndrome for several months, abdominal distension, and yellowish coloration of the skin for the previous few days with a rapidly fatal course. Examination revealed hepatomegaly and ascites. Laboratory investigations revealed hyperbilirubinemia with cholestasis. The remaining investigations (abdominal ultrasound, barium transit evaluation, bone marrow study, analysis of ascitic fluid and laparoscopy) did not establish the diagnosis. This was established by liver and subcutaneous fatty tissue biopsies, which revealed type AL amyloid deposits. Autopsy confirmed that the patient had primary systemic amyloidosis. This infrequent form of presentation of systemic amyloidosis and its poor prognosis are discussed.
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PMID:[Cholestatic jaundice and constitutional syndrome as early manifestations of primary systemic amyloidosis]. 1288 57

We studied the histopathological changes of liver in four patients who developed hepatomegaly and abnormal liver chemistry tests 2 to 20 days following administration of L-asparaginase as a part of a combination chemotherapy regimen for treatment of acute lymphoblastic leukemia. The severity of the liver disease due to L-asparaginase was unpredictable. One patient developed acute fulminant hepatic failure and required liver transplantation. The most consistent pathological change, observed in all four cases, was diffuse steatosis. Other changes included patchy hepatocyte necrosis, mixed inflammatory cell infiltrates in the portal tracts, and variable degrees of hepatocellular, or canalicular cholestasis, or a combination of these.
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PMID:Histopathological features of L-asparaginase-induced liver disease. 1452 82

OBJECTIVE: To report the unusual association between neonatal hepatitis and polysplenia syndrome.METHODS: Clinical, biochemical, histopathological, surgical and image analysis methods were used.RESULTS: The 36 days old patient presented hyperbilirubinemia with increase of direct reacting bilirubin and high alkaline phosphatase. The physical examination evidenced jaundice and hepatomegaly. The chest X-ray showed situs inversus and dextrocardia. Abdominal ultrasonography presented an increase in liver volume, mainly at the right lobe. There were two cystic lesions at the liver with absence of intrahepatic biliary tract dilatation. Absence of splenomegaly. Biliary scintigraphy didn't show any excretion of radioisotope to the duodenum while transoperative cholangiography presented contrast medium flowing to the duodenum lumen. During laparotomy it was possible to observe polysplenia, abdominal aorta to the right of the lower vena cava, gallbladder at the left lobe of the liver, appendix on the left side of the abdomen and sigmoid colon on the right side. The hepatic wedge biopsy was compatible with idiopathic neonatal hepatitis.CONCLUSION: The finding of polysplenia syndrome in patients with neonatal cholestasis doesn't necessarily indicate the recurrent presence of extrahepatic biliary atresia.
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PMID:[Polysplenia syndrome associated to neonatal hepatitis] 1468 63

A twenty year old, foreign-born sportsman visited the Out-patient Clinic of our Hospital with complaints of progressive arthralgia, hepatomegaly and increasingly abnormal liver function tests of six months duration. Tests for virus hepatitis were negative, alcohol abuse or drug addiction could be excluded. An open needle biopsy of the liver was performed and the tissue was examined with the light and electron microscope. On routine light microscopy no abnormality was recognized. Electron microscopic examination revealed changes characteristic of vitamin A toxicity: hyperplasia of the perisinusoidal (Ito) cells with evidence of their activation and transformation, increased storage of lipids and vitamin A, perisinusoidal fibrosis, damage of the sinusoidal wall, partial necrosis in hepatocytes and an increased number of lysosomes, megalysosomes and smooth endoplasmic reticulum (SER), the signs of cholestasis as well as an increased number of Kupffer cells in the lobules etc. Histochemical examination showed a high content of vitamin A in the transitional (Ito) cells and in hepatocytes. These data led to further questioning of the patient who disclosed that he had acne conglobata which had been treated with Isotretionin, 20 mg/day, for more than half a year. After the therapy was stopped, the symptoms of polyarthralgia improved and after a few months they ceased entirely, however, the laboratory data returned to normal only after a long period of time. This case indicates that electron microscopic examination of the liver biopsy may play an important role in the recognition of vitamin A intoxication. It also illustrates that symptoms of joint disease may be caused by long-term retinoid treatment. The authors have presented the latest clinical and experimental data concerning the changes in the liver, joints and skeleton caused by retinoid intoxication.
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PMID:[Ultrastructural findings in the liver due to long-term retinol (isotretinoin) treatment. Significance of the perisinusoidal (Ito) cells]. 1497 83

We retrospectively studied the records of 6 Malaysian children who were diagnosed with Alagille Syndrome (AGS) according to this criteria from January 1999 to January 2001, at the Institute of Paediatrics, Kuala Lumpur Hospital. Four patients (66%) had a positive family history. Thirteen individuals (6 patients and 7 relatives) were diagnosed with AGS in these 5 families. Only 6/13 (46%) of them presented with liver involvement. All 6 patients presented with typical facies and cholestasis (100%). Three (50%) presented with portal hypertension (PHT) with synthetic liver dysfunction (1 died), 1/6 (17%) have PHT and normal synthetic liver function. Two have cleared their jaundice but have biochemical evidence of hepatitis and hepatomegaly, four have congenital heart disease 5/6 posterior embryotoxon, 2/6 butterfly vertebrae, 4/6 hyperlipidaemia and 4/6 failure to thrive. One patient has a Jagged-1 gene disruption at the translocation breakpoint locus 20p12.3 2n = 46,XX,t(12.20) (q22, p12.3). 5/6 (83%) are still alive. Two-thirds of our patients developed chronic liver disease by 3 years of age. Two-thirds of the index patients have a family history. Only 46% of individuals in these families have clinical evidence of liver involvement. Mortality depends on cardiac/renal disease, end-stage liver failure and intercurrent infection.
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PMID:Morbidity in Alagille syndrome in 6 Malaysian children. 1519 Jun 47

This is the second reported patient with systemic juvenile xanthogranuloma (JXG) to die with liver failure. The infant was born with multiple skin lesions and mild hepatomegaly. Direct hyperbilirubinemia was noted on the 2nd day of life, followed by progressive hepatomegaly, cholestasis, and death at 29 days of age. At autopsy, nodular tumor infiltrates of JXG were present throughout the liver, as well as in skin, abdominal lymph nodes, spleen, and pancreas.
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PMID:Fatal congenital systemic juvenile xanthogranuloma with liver failure. 1525 37

Hepatomegaly is a common finding at autopsy in severely burned children surviving less than 6 months. This study validates a reliable ultrasound method which can be used to identify changes in liver size in severely burned children during acute hospitalization. Thirty-eight children, age 0.5-17 years with burns covering over 40% of their total surface area were studied at autopsy. Liver weight was measured at autopsy and compared to predicted liver weight for age and height. Eighteen had liver size measured by ultrasound within 10 days of death while five had ultrasound liver measures after death just prior to autopsy. All burned children who survived 7 days or more (n = 33) had liver weights at autopsy that were greater than predicted for age and height while all 23 livers measured by ultrasound were greater than predicted. Autopsy weights correlated well with weights estimated by ultrasound, R = 0.824. At autopsy, those who survived 7 days or more had enlarged livers ranging from 142 to 406% of their predicted normal age and height. Common histologic findings include large and small-droplet fat deposits, and cholestasis. The degree of these histologic abnormalities correlated with the increase in liver weight, R = 0.652. Ultrasound is a valid, noninvasive method for measuring liver weight changes in severely burned children during acute hospitalization. Ninety-five percent of the severely burned children from this institute had significant hepatomegaly identified at autopsy.
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PMID:Increased liver weights in severely burned children: comparison of ultrasound and autopsy measurements. 1530 22


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