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)

Systemic mastocytosis is an uncommon disorder due to multiorgan infiltration by mast cells. The authors report the case of a man whose mastocytosis was revealed in an unusual way by hepatomegaly and portal hypertension of the sinusoidal type. This case was also characterized by the absence of urticaria pigmentosa, the presence of seborrheic warts in which mast cell infiltration was noted and the absence of digestive symptoms. The peculiarities of this case are compared to the published data.
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PMID:[Hepatomegaly with portal hypertension indicative of systemic mastocytosis]. 376 29

Massive compensatory hepatic myeloid metaplasia occurs infrequently after splenectomy for myeloid metaplasia, and it usually develops gradually over several years if it does occur. In this report, a patient is described in whom massive hepatomegaly and evidence of portal hypertension developed within three months of splenectomy. Early evidence for rapid hepatic growth was present at a second laparotomy three weeks after the first surgery, and probably should have triggered initiation of chemotherapy. Indications for splenectomy in these patients, surgical morbidity, and response to splenectomy are discussed.
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PMID:Massive hepatomegaly following splenectomy for myeloid metaplasia. Case report and review of the literature. 381 39

Alpha-1-antitrypsin is a blood glycoprotein synthesized in the liver. It is a protease inhibitor of the serpine group and has a specific action for elastase. Alpha-1-antitrypsin electrophoresis shows about 20 phenotypes, the normal one being PiM. The allele PiZ is usually responsible for liver or lung disease in children or adults, respectively. Eleven per cent of PiZZ infants present with prolonged neonatal cholestasis. Twenty-five of 45 PiZZ infants with prolonged neonatal cholestasis presented with later cirrhosis. Persistence of jaundice beyond the sixth month of age, early development of splenomegaly, persistence of hard hepatomegaly and liver function abnormalities, and early portal fibrosis have a poor prognostic significance. The most severe course occurs in infants with an early histologic pattern of paucity of interlobular bile ducts. Portal hypertension was present in 19 of 25 children presenting with cirrhosis; 8 of 25 PiZZ children with cirrhosis died during childhood.
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PMID:[Alpha 1-antitrypsin deficiency in childhood]. 387 73

This prospective study was carried out in order to assess the accuracy of ultrasound in the diagnosis of cirrhosis. One hundred and twenty eight alcoholic patients were included. A careful ultrasonographic examination of the liver was performed before liver biopsy (100 patients). In 15 cases, liver histology was normal, steatosis and/or fibrosis, cirrhosis were diagnosed in 13 and 72 cases respectively. Ultrasonic patterns were classified by the same examiner, according to several criteria: volume, irregular outline, coarse and fine bright echo pattern, attenuation of the ultrasound beam, splenomegaly, ascite, portal hypertension. Cirrhosis was diagnosed in 58 out of 72 patients (80.5%). Specificity was 78.5%, positive and negative predictive values were 90.6% and 61% respectively, and global efficacy was 80%. Irregular outline (0.66), hepatomegaly (0.66) and attenuation of the ultrasound beam (0.64) were the best signs. In case of fine bright echo pattern, the diagnosis of cirrhosis would be missed. The results suggest that ultrasonography is a good test for screening alcoholics for cirrhosis. Therefore, it is useful when liver biopsy is contra indicated or refused or when liver is not detected at the clinical examination.
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PMID:[Value of ultrasonography in the diagnosis of cirrhoses. Prospective study of 128 patients]. 391 96

Three patients with the Budd-Chiari syndrome are presented. This is a rare condition characterized by hepatomegaly, progressive and refractory ascites, distension of the abdominal wall veins, abdominal pain and leg oedema. These features are attributed to congestion of the liver and portal hypertension. The condition has been notoriously difficult to treat medically. Surgical measures are directed towards relieving the liver congestion and lowering pressure in the portal system by portal-systemic shunting operations. In some cases refractory ascites may be treated by peritoneovenous shunting with a Le Veen shunt. In a select group of patients orthotopic liver transplantation has proved to be worth while.
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PMID:Budd-Chiari syndrome. A report of 3 cases. 396 23

Four young Chinese women took daily doses of an unidentified 'Indian' herbal tea as treatment for psoriasis. Three (one of whom died), developed ascites, hepatomegaly and biochemical abnormalities within 19-45 days. The fourth patient discontinued herbal tea after 21 days when she developed a skin rash. Two patients had portal hypertension, while all had liver histology showing features of veno-occlusive disease. Pyrrolizidine alkaloids were identified spectrophotometrically in the brewed tea, and in the chopped leaves of the herbal mixture; the mean dose in the tea prepared for consumption being 12 mg/day of alkaloid base and 18 mg/day of N-oxide. The mean cumulative dose of alkaloids (base + N-oxide) before onset of symptoms (three patients), was estimated to be 18 mg/kg. In the asymptomatic patient with histological liver disease only, the corresponding dose was 15 mg/kg. These cases thus provide some measure of pyrrolizidine alkaloid toxicity in adults.
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PMID:Herbal tea induced hepatic veno-occlusive disease: quantification of toxic alkaloid exposure in adults. 396 60

One hundred twenty-eight records of patients with multiple myeloma were reviewed to assess the incidence and manifestations of liver involvement. Histologic study of the liver was available in 21 patients. Diffuse infiltration of the liver by plasma cells was observed in 10 patients, myeloid metaplasia in four, amyloidosis in two, toxic hepatitis in two, and extrahepatic cholestasis secondary to infiltration of the peripancreatic tissue by plasma cells in one. The clinical signs of plasma cell infiltration of the liver consisted of hepatomegaly in seven patients, mild elevation of liver enzymes in five, and portal hypertension in two. Jaundice was only observed in patients with hepatitis or extrahepatic cholestasis. Liver infiltration by plasma cells did not appear to have a major prognostic significance.
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PMID:Liver involvement in multiple myeloma. 402 19

An unusual disorder of liver development, which is to our knowledge not described in the literature, was observed in a female infant who died, at the age of 25 weeks, of the sequelae of portal hypertension. At the time of autopsy, the enlarged liver revealed multiple small nodules histologically composed of trabecular hepatic tissue, whereas the remaining liver parenchyma consisted of hepatocytes arranged as tubular structures. Based on comparisons of these morphologic features with the different steps of normal hepatogenesis, we suggest that the disorder is the result of a partial arrest of liver differentiation with focal progression of maturation in the hyperplastic nodules. This assumption is supported by the observation of hepatocytes reacting with anti-alpha-fetoprotein antibodies in the tubular part of the liver, this being absent in the trabecular nodules.
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PMID:Immature tubular liver with focal nodular trabecular hyperplasia. 617 10

Primary tumors of the liver infrequently develop in patients with a normal liver or in those who have not been exposed to one of several tumor-producing compounds. Hepatocellular adenoma was one of the rarest liver tumors prior to the use of oral contraceptives (OCs). Now the annual incidence in longterm users is estimated at 3-4/100,000. An adenoma that follows OC use is one that often regresses with discontinuation. Focal nodular hyperplasia is a nonencapsulated solitary lesion that has a fibrotic stellate center in which large thick-walled arteries are the source of the blood supply, and occurs most often in women during the menstrual age, and there is no evidence that OCs have increased their frequency. Adenomatous hyperplasia occurs occasionally in patients with submassive necrosis and also in those with cirrhosis. Liver cysts present most often in middle aged women and the ratio of females to males is 4:1. In the US, metastatic carcinoma of the liver is some 18-20 times more frequent and about 85% of these arise in a cirrhotic or precirrhotic liver. Malignant mesenchymal tumors have been associated with exposure to vinyl chloride of injection of Thorotrast. Signs and symptoms of liver disease occur in about 50% of patients with hepatic metastases with hepatomegaly being the most common physical sign. Metastatic carcinoma most often produces multiple umbilicated nodules that involve the liver uniformly. Portal hypertension may be associated with a hepatic neoplasm.
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PMID:Tumors of the liver: pathologic features. 630 41

Clinical, biochemical and histological features of chronic hepatitis type B were studied in 29 children aged 8 months to 13 years. On entry into the study, all were known to have had hepatitis B surface antigen (HBsAg) with elevated serum transaminase levels for at least six months. A possible source of infection was found in 15 children. When they entered the study, all patients were anicteric and all but one asymptomatic. Hepatomegaly was detected in 15 patients and was associated with splenomegaly in two. Hypergammaglobulinemia was present in 4 children. Serological evaluation of hepatitis B virus markers showed evidence of complete viral replication (HBeAg positivity) in 24 cases and incomplete replication (anti-HBeAg positivity) in 5. Liver histology showed chronic persistent hepatitis (CPH) in 18 children, and chronic aggressive hepatitis (CAH) in 10 (3 moderately active and 7 with major signs of aggressivity ) associated with cirrhosis in 5. One patient had only minimal histological changes. Evaluation of clinical, biochemical and virological parameters did not strictly parallel the histological diagnosis in terms of "activity" of the disease. Follow-up for a mean period of 13 months showed good clinical tolerance to the disease in both CPH and CAH patients. Only 2 children with CAH were given corticosteroids and/or azathioprine for a short period. During follow-up no children with active disease developed liver insufficiency or evidence of portal hypertension. No significant difference in the percentage of children who had seroconversion to antiHBe was found between CPH and CAH groups. Only one child with CAH became HBsAg negative.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Chronic HB virus hepatitis in children. A study of 29 cases]. 632 26


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