Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present our experience with 18 pediatric patients with alpha 1-antitrypsin deficiency of the PiZZ phenotype. Fifteen patients (83%) presented with neonatal cholestatic jaundice at a mean age of 2 +/- 0.6 months (+/- S.D.). The male:female ratio was 15:3, indicating a male predominance. All metabolic, infectious and obstructive causes of jaundice were ruled out by appropriate tests in the patients with neonatal cholestasis. Liver biopsy in 14 patients with neonatal cholestasis showed a histological picture of cholestasis in all biopsies; neonatal giant cell hepatitis appeared in seven, increased fibrosis in appeared five and established liver cirrhosis appeared in two biopsies. Patients were followed for a mean of 3.7 +/- 2.4 years (+/- S.D.). Of the 15 patients with neonatal cholestasis, 3 under went liver transplantation because of decompensated liver cirrhosis at 3, 3 1/2 and 7 years. Two patients died at 4 months and 3 years from complications of liver cirrhosis. Of the remaining 10 patients, 3 had histological evidence of liver cirrhosis, and the remaining 7 patients continue to have enlarged liver and spleen with abnormal liver function tests. Of the three patients without history of neonatal cholestasis, only one had enlarged liver and spleen, and the remaining two are healthy with normal liver function tests. Our experience indicates serious liver disease is highly likely to develop in patients with PiZZ alpha 1-antitrypsin deficiency who present with neonatal cholestatic jaundice. Our experience differs from more recent reports on such patients.
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PMID:Liver disease in children with PiZZ alpha 1-antitrypsin deficiency. 328 87

The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
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PMID:Paediatric liver disorders in Singapore. 346 38

A case of secondary syphilitic hepatitis is reported. A 49-year old woman was admitted for weight-loss, fever, hepatomegaly and splenomegaly. Diagnosis of syphilitic hepatitis was based on cholestasis associated with positive serologic tests for syphilis without other immunological disturbances. Biopsy of the liver showed a moderate infiltration in and around the portal tracts. Immunofluorescence study for treponema was negative. Rapid improvement was obtained with penicillin initially associated with steroid therapy. Liver involvement in secondary syphilis is characterized by anicteric cholestasis, an inflammatory syndrome, and periportal infiltrate inconstantly associated with centrilobular necrosis, granulomatous reaction and presence of treponemas in the lesions. Due to the increasing frequency of sexually transmitted diseases, this diagnosis could become more frequent.
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PMID:[Hepatic involvement in secondary syphilis]. 355 64

Eleven patients of Chinese origin experienced spontaneous reactivation of chronic active hepatitis B. Eight HBsAg-positive patients were followed for an average of 15 months prior to, while three others presented during reactivation. Fatigue, hepatomegaly and jaundice were frequent findings. Elevation of both serum ALT (average = 1,212 units per liter) and hepatitis B virus DNA levels were noted in all patients, and reactivation lasted an average of 4.4 months. During resolution, clinical symptoms abated, serum ALT levels reverted toward normal, and in nine patients, the hepatitis B virus DNA values became undetectable. All patients lacked evidence for acute hepatitis A, Epstein-Barr Virus, cytomegalovirus or hepatitis delta virus infection. Histologic findings of liver tissue from eight patients showed piecemeal necrosis and fibrosis. Within the parenchyma, varying degrees of hepatocytolysis with cuffing, perivenular necrosis and acidophilic bodies were noted. Ground-glass cells and regenerative changes also were observed. Cirrhosis was not present in any of the liver biopsies. These findings suggest that spontaneous reactivation of hepatitis B occurs in heterosexual patients with chronic active hepatitis B and contributes to chronic inflammation and to the progression of their liver disease.
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PMID:Spontaneous reactivation of hepatitis B in Chinese patients with HBsAg-positive chronic active hepatitis. 361 49

Patients with chronic heart failure (CHF) can often develop such diseases as hepatitis of viral etiology, alcoholic hepatitis, drug affection of the liver and other diseases masked as congestive liver. In most cases CHF concomitant liver diseases have an atypical course with a tendency to a chronic course. CHF is one of the important pathogenetic mechanisms lying in the basis of chronicity of concomitant liver diseases. Refractory CHF, inconsistency of the hemodynamic indices of persistent hepatomegaly must lead a physician to the detection of probable independent liver diseases complicating the syndrome of heart failure. CHF is a factor causing an enhanced fibrosing liver reaction. An important diagnostic test of fibrinogenesis lying in the basis of chronicity of liver diseases, is the determination of enzymatic markers reflecting synthesis and catabolism of the main substance of connective tissue. Change in the levels of haptoglobin, ceruloplasmin and glutamic acid dehydrogenase is an indirect sign of damage of the liver parenchymal endoplasmic reticulum. These indices can serve as differential criteria of the prevalence of cardiovascular disorders in the liver or concomitant independent liver diseases.
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PMID:[Pathogenetic mechanisms of chronicity in liver diseases in patients with circulatory failure]. 361 41

A case of phenytoin-induced hepatitis with mononucleosis is reported, and syndromes associated with phenytoin hypersensitivity reactions are discussed. A 23-year-old black woman with a two-month history of seizure disorder was admitted to a hospital with nausea, vomiting, fever, lymphadenopathy, diffuse maculopapular rash, left-upper-quadrant tenderness, and hepatomegaly. She was receiving phenytoin sodium 300 mg/day; carbamazepine 200 mg four times daily had been discontinued four days before admission because of leukopenia. Phenytoin was discontinued after admission; however, phenytoin 1 g i.v. was given for a tonic-clonic seizure two days after admission, after which swelling of the face and legs and pruritus developed. Over the next few days, signs and symptoms of hepatotoxicity progressed, and she became comatose. Seizures were treated with diazepam. She began to recover after 10 days of supportive therapy and was discharged several weeks later on primidone therapy. Serious phenytoin hypersensitivity reactions may appear as dermatologic, lymphoid, or hepatic syndromes. Fever, rash, and lymphadenopathy often accompany hepatic injury. Encephalopathy and death may occur. Proposed mechanisms for phenytoin hypersensitivity include antigen-antibody reactions, alteration of lymphocyte function, and an enzyme abnormality causing the production of toxic metabolites. Treatment is supportive; phenobarbital and carbamazepine may be used with caution as alternate anticonvulsant therapy. The possibility of phenytoin hypersensitivity reactions should be considered when patients receiving phenytoin have unusual symptoms, particularly fever, rash, and lymphadenopathy.
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PMID:Phenytoin-induced hypersensitivity reactions. 367 71

We report 3 cases of rickettsial hepatitis observed in the Lyon area which were secondary to boutonneuse fever. The temperature was elevated and clinical signs were an often painful hepatomegaly and digestive disorders. These signs are only indicative when accompanied by atypical pneumonopathy or exantherma. Indeed, if the inflammatory syndrome is clear, hepatic function is little perturbed. Diagnosis is sometimes influenced by the epidemiological situation, and depends on serologic tests and histological evidence of granulomatous hepatitis (2 of the 3 cases here). The successful antibiotic treatment shows that patients presenting febrile granulomatous hepatitis should be tested for rickettsiosis.
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PMID:[Hepatic anomalies in Mediterranean boutonneuse fever]. 371 90

Abnormal liver chemistries, unexplained fevers, or hepatomegaly prompted 36 liver biopsies on 34 patients with the acquired immunodeficiency syndrome. The most common finding was the presence of hepatic granulomas, seen in 13 of the biopsy specimens. Eight of these granulomas were ill-defined, and 5 were more clearly associated with mycobacterial disease. Portal fibrosis and fatty infiltration were common, but a paucity of significant inflammatory activity was seen despite elevated aspartate aminotransferase levels, perhaps related to the underlying immunoincompetent status. Other noteworthy histopathologic findings included 1 patient each with peliosis hepatis and cryptococcal hepatitis. Electron-microscopic evidence of cytoplasmic tubular structures or viral particles were seen within the hepatocytes of 2 patients. It is concluded that a broad spectrum of hepatic histopathology may be seen in the acquired immunodeficiency syndrome, and that liver biopsy may be diagnostically valuable in the clinical investigation of such patients.
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PMID:The spectrum of liver disease in the acquired immunodeficiency syndrome. 372 95

The authors report the case of a 63 year-old woman who developed high-grade fever with chills, nausea, diarrhea, severe pain in the right hypochondrium, and jaundice after one month's treatment with 300 mg of hydroquinidine hydrochloride daily. Serum bilirubin and aminotransferases were slightly increased, while alkaline phosphatases and gamma-glutamyl-transpeptidase serum activities were markedly raised. Histological examination of a liver specimen obtained by the transvenous route showed numerous epithelioid granulomas with giant cell formation and eosinophils in hepatic lobules and portal tracts. Symptoms disappeared three days after withdrawal of the drug, but hepatomegaly and a mild increase in serum gamma-glutamyl-transpeptidase persisted more than eighteen months. Quinidine-induced hepatitis is almost always associated with fever, and, in one-third of the cases, with a pseudo-cholangitis picture. Extrahepatic hypersensitivity manifestations are often present. Histological examination of the liver shows granulomatous or cytolytic hepatitis. Withdrawal of the drug is rapidly followed by a favorable outcome; readministration causes immediate relapse; progression to chronic liver disease has never been reported previously.
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PMID:[Hepatitis caused by quinidine. Study of a case and review of the literature]. 373 35

Rhabdoid sarcoma is a tumor of unknown etiology that usually occurs in the kidneys of infants and small children. We report an adolescent with a rhabdoid sarcoma of the chest wall. In addition to the patient's age and the site of the tumor, other unusual features of this case were as follows: positive staining of tumor cells with neuron-specific enolase, the presence of chronic, active, hepatitis that apparently developed coincident with the sarcoma, and the presence of widespread hemosiderosis. Two of the patient's siblings died in infancy with degenerative neurologic disease, hepatomegaly, and multiple congenital anomalies. The histochemical findings and family history lend support to previous suggestions that some rhabdoid sarcomas may be of neural crest origin and may be heritable lesions.
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PMID:Extrarenal rhabdoid sarcoma. 374 41


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