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)

Two young human immunodeficiency virus (HIV)-infected patients, a 25-year-old woman and a 26-year-old man, consumed large amounts of germanium lactate citrate 18% as an "immunostimulant" for 9 months. The woman, who had stage II HIV infection, developed severe renal dysfunction (creatinine clearance, 7 mL/min/1.73 m2) and slight proteinuria (0.28 g/d) after ingesting 260 g germanium lactate citrate 18%. Hepatomegaly with liver dysfunction (SGOT, 102 U/L; gamma-glutamyl transferase (GT), 159 U/L) and lactic acidosis (plasma lactate, 7.3 mmol/L) developed simultaneously. Renal biopsy revealed tubulointerstitial nephropathy with vacuolar cell degeneration and periodic acid-Schiff-positive intracellular deposits mainly in distal tubules. Liver biopsy disclosed severe hepatic steatosis; liver function tests returned to normal within 5 weeks. Since renal failure persisted for 2 years after ingestion of germanium (creatinine clearance, 14 mL/min/1.73 m2; proteinuria, 0.84 g/d), a second renal biopsy was performed, which showed marked but focal distal tubular atrophy and slight interstitial fibrosis. The male patient, who had stage III HIV infection, had ingested the same compound; he presented with a creatinine clearance of 43 mL/min/m2 and proteinuria of 0.36 g/d. Renal biopsy disclosed tubulointerstitial changes similar to those found in the female patient. After 9 months off germanium, creatinine clearance remained unchanged. Neutron activation analysis of all biopsy specimens in both cases documented germanium concentrations 10 to 70 times normal in renal tissue and 140 times normal in liver tissue.
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PMID:Tubulointerstitial nephropathy persisting 20 months after discontinuation of chronic intake of germanium lactate citrate. 848 24

Hyperlipidemia has been implicated in the pathogenesis of experimental progressive glomerulosclerosis, but its role in human renal injury is controversial. This report describes a 12-yr-old boy presenting with massive proteinuria, hepatomegaly, anemia, severe mixed hyperlipidemia, and progressive renal failure. The initial renal biopsy disclosed large numbers of foam cells that were shown to be monocytes. Evidence is presented suggesting that apoprotein-E2 homozygosity in our patient, together with an 88% reduction in plasma lipoprotein lipase activity associated with severe nephrotic syndrome, is responsible for the atypical clinical features, lipoprotein phenotype III with chylomicronemia, and renal lipidosis. A regimen of dietary lipid restriction, gemfibrozil, and niacin resulted in significant but partial improvement of the dyslipidemia and resolution of the hepatomegaly and ascites. This report stresses the importance of characterizing unique lipid disorders in patients with nephrotic syndrome in order to prescribe effective lipid-lowering strategies. Moreover, the striking resemblance of the clinical and nephrohistologic features of this patient to those occurring in experimental models of coexisting glomerular injury and hyperlipidemia led to the speculation that, in this setting, the hyperlipidemia may contribute to the development of progressive glomerulosclerosis.
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PMID:Atypical hyperlipidemia and nephropathy associated with apolipoprotein E homozygosity. 858 83

Amanita phalloides is responsible for about 90 per cent of all fatal cases of mushroom intoxication. The amatoxins, the main toxic component of these fungi, are responsible for gastro-intestinal symptoms as well as hepatic and renal failure. Three brothers with Amanita phalloides poisoning were admitted with gastro-intestinal symptoms beginning 12 h after ingestion. Jaundice, hepatomegaly and neurological symptoms were not present, but liver enzymes were moderately increased. Alfa-amanitin was detected in sera of all patients. All patients underwent charcoal hemoperfusion and two of them had additional hemodialysis along with conservative therapy. Liver enzymes that showed a marked increase on the second day of therapy decreased to normal levels on the 28th day. All of our patients survived. This life saving role of early haemoperfusion in Amanita phalloides poisoning is emphasized.
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PMID:Haemoperfusion in Amanita phalloides poisoning. 860 49

Systemic lupus erythematosus is an uncommon disease in men. The clinical and pathological features and prognosis in 50 male patients with lupus nephritis (LN) and 50 age-matched female patients with LN were analysed. The age at which the disease began and the diagnosis was made was generally older in the male. The incidence of LN was higher in the male than in female (P < 0.01). The incidence of types IV and V LN was more common in the male than in female. Type II LN was not found in male patients. Proteinuria over 3.5g/24h and renal failure were more common in the male than in female (P < 0.05). The three most common clinical manifestations in male patients were irregular fever, skin rashes and painful joints. Rashes, hepatomegaly and neuropsychiatric dysfunction were more common in the male than in female. The patients were followed up one to twelve years. The rate of recovery and improvement was lower, but that of relapse and mortality higher in the male than in female.
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PMID:[Lupus nephritis in male adults, an analysis of the clinical and pathological features]. 873 62

Polycystic liver disease (PLD) is an autosomal-dominant hereditary disease which usually presents together with polycystic kidney degeneration (ADPKD). The renal problems determine the course of this disease. Due to the development of dialysis an increasing number of patients present with symptoms from their liver cysts: These range from compression caused by hepatomegaly, which can severely limit the quality of life, to chronic liver failure. Ten patients with advanced symptoms of PLD underwent orthotopic liver transplantation, five of them with combined renal transplantation. Postoperative complications occurred in three patients. One patient died postoperatively from multiorgan failure after experiencing coagulopathy of unknown origin. After follow-up of 6-60 months, all patients had better quality of life after transplantation. There was a complete relief of symptoms; liver or renal failure did not occur. Liver transplantation should be considered in patients with highly symptomatic PLD. In the case of severe hepatomegaly or liver and renal failure the combined liver and renal transplantation are able to cure the PLD and ADPKD without rising the disadvantage of immunosuppression incurred with single organ transplantation.
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PMID:[Orthotopic liver transplantation in therapy of advanced polycystic liver disease]. 877 81

We prospectively studied 50 Vietnamese patients with blackwater fever (BWF). All patients had fever and hemoglobinuria, 40 (80%) were jaundiced, 25 (50%) had hepatomegaly, 15 (34%) had splenomegaly, and 9 (18%) had hepatosplenomegaly. Twenty-one patients (42%) had impaired renal function, with creatinine clearances of < 50 mL/min/m2; however, only four (8%) developed oliguric renal failure, three (6%) of whom required dialysis. Forty-four patients (88%) developed anemia, which was severe (hematocrit, < 20% in 32 (64%). One patient died, representing a death rate for this once-feared disease that is considerably lower than that reported by earlier investigators. BWF was associated with quinine ingestion in 28 patients (56%), glucose-6-phosphate dehydrogenase (G6PD) deficiency in 27 (54%), and concurrent malaria infection in 16 (32%). There was no statistically significant difference in the severity of BWF associated with each of these three factors, as assessed by creatinine clearance and the hematocrit value on admission and by the number of units of blood transfused. There was considerable overlap in the occurrence of G6PD deficiency, quinine ingestion, and malaria, suggesting that these factors may interact and that it may not be justifiable to regard hemoglobinuria caused by G6PD deficiency as a separate syndrome.
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PMID:Blackwater fever in southern Vietnam: a prospective descriptive study of 50 cases. 940 15

The symptoms of amyloidosis depend on the type of precursor, the amount of deposits and their location. In systemic amyloidosis almost every organ may be involved. Cardiac involvement is severe, especially in AL amyloidosis, responsible for restrictive cardiomyopathy with right ventricular failure, leading rapidly to death. Renal amyloid deposition causes nephrotic syndrome with hypertension and renal failure. Neurological complications include peripheral neuropathy with dysautonomia cerebral involvement (dementia, cerebral haemorrhages). Arterial deposits are common in systemic senile amyloidosis, and may cause ischaemia. Osteo-articular damage is mainly seen in patients on long-term haemodialysis. Liver enlargement is often the only manifestation of hepatic amyloidosis. Digestive tract involvement includes macroglossia deposits in salivary glands and disturbances in gastrointestinal motility. Pulmonary amyloidosis causes nodular or interstitial infiltrates. Cutaneous lesions are various. Localized amyloidoses include goiter, breast and vesical involvement which can be difficult to differentiate from neoplasm, as well as ocular amyloidosis mimicking posterior uveitis.
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PMID:[Localizations and consequences]. 945

Coronary arteries are frequently involved in systemic arteritis. The inflammatory infiltrate damages the intima and may trigger the occurrence of coronary thrombosis. We report an extreme example of how intimal inflammation in multiple sites of a coronary tree with and without atherosclerosis may trigger coronary thrombosis, in an elderly female patient who died of a clinically unrecognized systemic autoimmune-inflammatory disorder with necrotizing arteritis. The clinical picture was dominated by abdominal symptoms (peritonitis and possible chronic hepatic disease), renal failure and pulmonary X-ray opacities. A precise clinical diagnosis was not formulated, and the patient died of cardiac arrest 15 days after admission. Autopsy showed findings typical of Wegener's granulomatosis and of systemic arteritis with fibrinoid necrosis and multiorgan infarctions. Wegener's granulomatosis-polyarteritis nodosa overlap syndrome was pathologically diagnosed. Although there were no clinical signs of heart involvement, the coronary tree showed inflammation associated with multiple mural and occlusive thrombi. The atypical severe clinical presentation, the short course of the disease and the age of the patient probably contributed to the non proper clinical diagnosis. Old age does not preclude the occurrence of autoimmune disorders, whose course may be dramatically fatal. The abrupt occurrence of a systemic disease with renal failure, hepatomegaly, lung opacities and serositis should prompt analysis to consider these disorders. If properly diagnosed, cardiac involvement should be suspected in autoimmune disorders, even when clinically silent or masked by the systemic clinical picture. In our patient, the role that heart involvement played in the outcome, if any, remains unknown, even though the postmortem pathological identification of coronary mural and occlusive thrombi is generally sufficient to attribute the final cause of death to coronary thrombosis itself.
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PMID:Coronary artery inflammation and thrombosis in Wegener's granulomatosis-polyarteritis nodosa overlap syndrome. 961 52

Four cases of typhoid fever complicated by both acute oliguric renal failure and hepatitis are presented. Two patients had type II hepatitis according to criteria proposed by Khosla et al. (30) with hepatomegaly, hyperbilirubinaemia and markedly elevated asparate transaminase (AST); the others had type III hepatitis, characterized clinically and biochemically by profound jaundice, hepatomegaly, hepatic encephalopathy (one case only), hyperbilirubinemia and markedly elevated serum AST. Renal biopsy was not performed in any of our patients. However, a combination of proteinuria and abnormal urinary sediments containing red cell casts and granular casts, as noted in these patients, is considered highly suggestive of glomerulonephritis. Although isolated renal failure and hepatitis with hepatomegaly and deranged liver enzyme values have been reported previously in typhoid fever, their occurrence simultaneously in the same patient in distinctly rare, having been reported only twice in the English language literature.
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PMID:Typhoid fever complicated by acute renal failure and hepatitis: case reports and review. 964 38

Psittacosis, also referred to as ornithosis, is a disease primarily of birds, which may be transmitted to humans. Psittacosis is caused by Chlamydia psittaci, an obligate intracellular parasite found worldwide. Humans are infected with C. psittaci when the organism enters the blood stream, usually through inhalation of dried excrement from diseased birds or through wound contamination with infected avian secretions. C. psittaci replicates in the liver and spleen and infects the lung and other organs hematogenously.1 The clinical manifestations of human psittacosis range from a mild respiratory infection to a severe systemic illness.1,2 Symptoms are frequently described as flu-like with fever, headache, body aches, and dry or productive cough. Sore throat, chest pain, abdominal pain, vomiting, and diarrhea are variably present. Physical findings may include a pulse-temperature dissociation, localized lung crackles, hepatomegaly, splenomegaly, and a pale macular skin rash. Chest radiographs may demonstrate lesions that are atelectatic, patchy, miliary, nodular, or consolidated in one or both lungs. White cell counts, erythrocyte sedimentation rates, and liver function tests are usually normal. In severe illness, signs and symptoms of liver dysfunction, neurological impairment, and respiratory and renal failure may be present. Since 1879 when psittacosis was recognized as a disease entity, cases have been reported in North and South America, Europe, Asia, and Australia. However, reports of psittacosis in Africa have been rare. An Ethiopian group, studying community-acquired pneumonia, published what they claimed to be the first report of psittacosis in Africa in 1994.3 The report published here is believed to be the first documented case of human psittacosis in Egypt.
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PMID:Psittacosis in Egypt: A Case Study. 981 79


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