Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-one of 30 patients with essential mixed cryoglobulinemia (EMC) had evidence of liver involvement. The liver disease was characterized by the absence of clinical symptoms, hepatosplenomegaly, mild elevation of enzymes, abnormal BSP retention and low albumin levels. Histology, available in 12 patients, showed either chronic persistent or chronic active hepatitis or liver cirrhosis; 44% of the patients had HBsAg or HBsAb in sera and/or cryoglobulins, confirming the high frequency of exposure to hepatitis B virus (HBV) infection in EMC. However, liver lesions were similar in all patients, regardless of HBV exposure. Since other factors usually associated with chronic liver diseases were absent or apparently irrelevant, it is temptative to speculate that a 'cryoglobulinemic hepatitis' may exist as a distinct syndrome. The characteristic complement profile of the patients with EMC (low CH50 and C4, normal C3PA), not related to albumin levels, can help to differentiate this disease from chronic liver disease without cryoglobulins.
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PMID:Liver involvement in essential mixed cryoglobulinemia. 54 44

Two Chinese boys, aged 3.5 and 5 years, developed nephrotic syndrome and were chronic carriers of hepatitis B virus surface antigen (HBsAg) and hepatitis B virus e antigen (HBeAg). Renal biopsy showed membranous glomerulonephritis and liver biopsy showed chronic persistent hepatitis. They were given interferon-alpha-2a at a dose of 5 MU/m2 on alternate days for 12 and 16 weeks after 2 years of persistent nephrotic syndrome. Patient 1 showed complete remission and resolution of hepatosplenomegaly, but his serum remained positive for HBsAg, HBeAg and hepatitis B virus DNA. Patient 2 showed only a transient clinical response and seroconversion from HBeAg to anti-HBe status. Although not always successful, interferon treatment should be considered in severe persistent nephrotic states, since there is at present no satisfactory treatment for this form of glomerulonephropathy.
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PMID:Interferon treatment for hepatitis B-associated membranous glomerulonephritis in two Chinese children. 851 10

This retrospective case review sought to report the findings of a health screening of 100 international adoptees. Included were the 1st 100 children who attended the Outpatient Inter-Country Adoption Clinic at the Royal Children's Hospital in Melbourne. A medical history was taken and a thorough physical examination and series of screening investigations were performed on all children. The children range in age from 2 months-16 years (median=5 months; mean=2.8 years). The countries most represented were Korea with 36 children and India with 21. 30 children fell below the 3rd Australian centile for both height and weight. Abnormalities on physical examination included hepatosplenomegaly (13 children), scabies (6), and severe dental caries (6). Parasitic infestation of the stools was found in 23 children, a positive Mantoux test result in 9 (3 with active tuberculosis), anemia in 12, elevated hepatic transaminase levels in 9, and positive hepatitis B surface antigen and e antigen in 2. I child had acquired syphilis. Screening for human immunodeficiency virus antibody gave negative results in all children. These international adoptees constitute a special pediatric subgroup which require assessment and screening as soon as possible after arrival in their adoptive country.
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PMID:Health screening of international adoptees. Evaluation of a hospital based clinic. 154 38

Diphenylhydantoin-induced hepatitis and mononucleosis are uncommon in children. The occurrence of these two diseases in the same individual, with progression to hepatic failure is rare and has not been reported in infants. This report represents a 6-month-old male infant who developed an infectious mononucleosis-like syndrome and hepatic failure 16 days after diphenylhydantoin administration. He took this anticonvulsant for controlling seizures after a head injury. Fever, skin rash, hepatosplenomegaly, lymphadenopathy, and atypical lymphocytosis led to the initial diagnosis of infectious mononucleosis. However, negative heterophil antibody did not support the diagnosis. Jaundice ensued in the following course and became more and more profound. Meanwhile, physical examination showed shrinking in liver size. Negative virology studies, including Epstein-Barr virus, cytomegalovirus, and hepatitis B virus, excluded them as causative agents. The patient lapsed into a stage I hepatic coma, but gradually recovered clinically and biochemically after eight successive exchange transfusions and supportive care. Two liver biopsies were performed 20 and 50 days after the onset of disease, respectively. Remarkable hepatic parenchymal loss, cholestasis, and fatty change were found on histologic examination of the first biopsy specimen, and portal fibrosis was noted on the second.
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PMID:Mononucleosis and hepatic failure associated with diphenylhydantoin treatment in an infant. 167 17

A case of polymyositis associated with chronic active hepatitis was reported. A 53-year-old man, who had no previous history of blood transfusion nor hepatitis, noticed proximal dominant muscle weakness on January 29, 1985. He was admitted to Kyoto National Hospital on February 7, and laboratory studies disclosed the elevation of serum enzyme levels; creatine kinase (CK) 9845 IU/L (normal 54-263), glutamate oxaloacetate transaminase (GOT) 834 IU/L (9-31), glutamate pyruvate transaminase (GPT) 491 IU/L (4-34), lactate dehydrogenase (LDH) 2135 IU/L (248-464). Also serum gamma globulin was high (1.8 g/dl) and LE-like cell was found. The diagnosis of polymyositis was made and prednisolone therapy (60 mg/day) was started on February 23. The elevated serum enzymes decreased gradually, but severe muscle weakness persisted for about one month. On April 3, he was admitted to our hospital. Physical examination revealed moderate proximal dominant muscle weakness without skin eruption, jaundice or hepatosplenomegaly. The serum enzymes were still high; CK 1826, GOT 173, GPT 232 (GOT less than GPT), LDH 1548. However, alkaline phosphatase (ALP) and bilirubin were normal. Hepatitis B surface antigen (HBsAg) was not detected. Antinuclear antibody was positive. The electromyogram study showed myopathic change, and the muscle biopsy demonstrated myopathic change and cell infiltration, compatible with polymyositis. These results suggested liver dysfunction associated with polymyositis. Prednisolone therapy was continued and muscle weakness decreased. From December, 1985, serum enzymes (CK, GOT, GPT, LDH) elevated again and muscle weakness also slightly increased. Anti-smooth muscle antibody was positive. It was suggested that both polymyositis and liver dysfunction deteriorated.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of polymyositis associated with chronic active hepatitis]. 218 64

A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinical remission to recurrent relapses. Hepatitis B viral infection suddenly evolving to hepatic failure was the cause of death. From a rapid survey of the present knowledge, the nosology, immunological features, and therapy of AILD are discussed and a possible presumptive pathogenetic pathway is proposed.
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PMID:Angioimmunoblastic lymphadenopathy with dysproteinemia: report of a case in infancy with review of literature. 270

Because dual infection with Schistosoma mansoni and hepatitis B may lead to severe liver disease, populations living in schistosomiasis-endemic areas might benefit if effectively immunized against hepatitis B. To determine whether a plasma-derived hepatitis B vaccine is immunogenic in patients with schistosomiasis, 32 individuals infected with S. mansoni were given three 20-micrograms doses of Heptavax-B vaccine and treated with praziquantel. Antibody to hepatitis B surface antigen developed in 90.6% of the study subjects after three doses of vaccine. Five patients (15.6%) had a weak response to the vaccine, and three patients (9.4%) failed to develop antibody. A weak or failed response to the vaccine was significantly associated with the presence of hepatosplenomegaly. A plasma-derived vaccine is immunogenic for persons infected with S. mansoni; however, vaccine response is diminished in hepatosplenic schistosomiasis.
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PMID:Immunogenicity of hepatitis B vaccine in patients infected with Schistosoma mansoni. 295 63

To study the association between hepatitis B and schistosomiasis, 1,234 Egyptian males, ages 18 to 24, were interviewed, examined, and tested for Schistosoma mansoni infection and HBsAg. Sera from 91 (7.4%) of the study subjects were positive for HBsAg, and S. mansoni was found in the stools of 26.3%. There was no correlation between S. mansoni infection, with or without hepatosplenomegaly, or a history of schistosomiasis, and HBsAg. An association was found between HBsAg positivity and a previous history of parenteral antischistosomal therapy (P less than 0.01). The results of the study indicate that parenteral therapy for schistosomiasis may be a risk factor for hepatitis B antigenemia. Further studies are indicated to determine the importance of parenteral therapy in the transmission of hepatitis B.
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PMID:Parenteral antischistosomal therapy: a potential risk factor for hepatitis B infection. 311 70

S. mansoni and S. japonicum complex schistosomes cause hepatosplenic and hepatointestinal schistosomiasis. The prevalence and incidence of this disease is increasing in all the endemic areas. Hepatosplenic schistosomiasis is seen in a small subset of clinically infected patients and represents a good model of intrahepatic portal hypertension characterised by a presinusoidal portal block and a well preserved liver parenchyma. Symmers' fibrosis is seen in a significant proportion of patients with high worm load. While the pathogenesis of Symmers' pipe stem fibrosis has not been well established, experimental and clinical data point to egg induced granulomata. The main consequences are presinusoidal portal hypertension, oesophageal varices and hepatosplenomegaly. The most striking symptoms are haematemesis or melena secondary to variceal and gastrointestinal bleeding. Cofactors associated with the pathogenesis include aflatoxins, malnutrition, alcoholism, hepatitis B and C virus. While stool examination is the best technique for diagnosis, a number of immunological tests though sensitive are not specific. Ultrasonography is sensitive for detection of Symmer's fibrosis. Praziquantel and oxaminiquine are drugs found to be effective in the treatment of hepatosplenic schistosomiasis. Recently beta-blockers have been found to be effective in the treatment of gastrointestinal rebleeding. Endoscopic sclerotherapy has been found to be effective for treatment of bleeding oesophageal varices. The treatment of choice for portal hypertension is oesophagogastric devascularization with splenectomy (EGDS).
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PMID:Tropical gastrointestinal disease: hepatosplenic schistosomiasis--pathological, clinical and treatment review. 776 89

Indirect enzyme immunoassay, performed with Labsystems (Helsinki) kits, in 30 mothers and their newborns, revealed that 100% of parturient women present IgG antibody to hepatitis A (HAV), herpes simplex 1, and measles viruses, constantly transferring these antibodies to their newborns. 78.6% of the women had IgG to rubella (German measles) virus, passively transmitting them to their offsprings. Serological markers (HBsAg and anti-HBc) of hepatitis B virus infection were present in 42% of the investigated women, anti-HBc being also present in the serum of the newborns. Between the identified risk factors (in the past obstetrical history, current pregnancy, labor and early postnatal period) and the spectrum of IgG antibodies present in mothers no significant correlations were revealed. The same obvious lack of correlation between IgG antibodies and risk factors in the neonate (prematurity, low birth weight, malformations) was also found. However, the presence of IgG anti-measles (2 case), IgM anti-rubella (1 case) and IgM and HAV (4 cases) was associated, in the same order, with interstitial pneumonia, hepatosplenomegaly and death, icterus neonatorum, cardiovascular and neurologic malformations. Neither mothers nor their offsprings presented anti-HIV antibodies, suggesting that in the investigated patients no perinatal transmission had occurred.
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PMID:[The presence in pregnant women of the risk factor of serum antibodies against 9 viruses with significance in materno-infantile pathology and the transfer of these antibodies to the newborns]. 799 65


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