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 patients with serologically-proven dengue virus infection and Morbitz type I second degree atrioventricular (AV) block are described. A 7 years old boy (patient 1) with grade 2 and a 7 years old girl (patient 2) with grade 3 illness were admitted to the hospital on the 3rd and the 5th day of the illness, respectively. Both had typical resentation for dengue hemorrhagic fever including fever, hepatomegaly, thrombocytopenia and signs of extravascular leakage. The 7 year old girl also had epistaxis and anemia (Hct 24%). Morbitz type I second degree and 2:1 AV block developed on day 7 (patient 1) and day 8 (patient 2) of the illness, both during recovery periods. Patient 1 also had occasional monomorphic premature ventricular contraction (PVC). There was no other abnormality in the 12-lead EKGS and echocardiograms showed normal ventricular systolic function in both. Other than mild hypokalemia (3.3 and 3.4 mgq/l), serum electrolytes were normal. Neither patients had elevation of serum creatine phosphokinase (CPK). In patient 1, exercise (on day 10) normalized AV conduction and abolished the PVC. Follow up EKG and physical examination at 10 months after the illness was normal. The rhythm in patient 2 resolved to 1st degree AV block (with occasional morbitz type I second degree at night) on day 12. In this patient, exercise resulted in shortening of the PR interval and Valsalva maneuver resulted in further PR prolongation. The patient was well at 1-month follow up with a mormal EKG. Morbitz type I second degree AV block during recovery from dengue hemorrhagic fever may be a transient functional impairment of the AV node, in which altered autonomic tone may play a role.
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PMID:Morbitz type I second degree AV block during recovery from dengue hemorrhagic fever. 1141 4

Childhood primary hepatocellular carcinoma is rare and accounts for less than 1% of all abdominal malignancies in children < or = 14 years of age. A review of the records of the Cancer Registry, Ibadan, Nigeria covering the period 1960-1995 was scrutinised and 19 cases of hepatocellular carcinoma (HCC) were registered, accounting for 0.49% of all abdominal malignancies over the period of review. The mean (SD) age at presentation was 10.4 (3.0) years and the duration of illness before presentation was short. All the children presented late with abdominal distension and hepatomegaly as the major clinical features. Weight loss was evident in 80% of cases, splenomegaly occurred in 50% and jaundice was present in a third of them. The prognosis was poor; all the cases died within 2 weeks of presentation in hospital. There was evidence to suggest an association between hepatitis B virus infection and HCC in all the liver tissue stained by Shikata-Orcein. This review shows that HCC, though uncommon, is important enough to be considered a possible cause of unexplained hepatomegaly in Nigerian children and that hepatitis B virus is an important aetiological factor. Though the number of cases under review is small, universal early vaccination against hepatitis B virus is necessary in Nigerian children in order to reduce the burden of chronic hepatitis B disease and hepatocellular carcinoma.
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PMID:Hepatocellular carcinoma in Nigerian children. 1147 Dec 62

Three infants with documented mitochondrial fatty acid oxidation disorders are described in this report. Case 1. Carnitine/acylcarnitine translocase deficiency. (CACT) (OMIM 212138) A two-day-old male developed sudden cardiac arrest 48 hours postpartum, with a previous history of early death (day 2) in siblings with a history of parental consanguinity; somnolence, inactivity, refusal to suck within 24 h, hepatomegaly, persistent hypoglycemia, hypocalcemia, hyperkalemia and severe metabolic acidosis prior to cardiac arrest. Dried blood spots by tandem mass spectrometry demonstrated 10 x elevation of palmitoylcarnitine, moderate elevation of oleylcarnitine, steroylcarnitine and myristoylcarnitine. Case 2. Medium chain acyl CoA dehydrogenase (MCAD) deficiency. (OMIM 212139) A six-week-old male infant, developed sudden cardiac arrest after contacting a viral illness, resuscitated successfully in the first episode, only to succumb during the second episode, 2 weeks apart. Plasma acylcarnitine via tandem mass spectrometry was reported normal; however, urine organic acids via gas liquid chromatography and mass spectrometry demonstrated characteristic metabolites consistent with MCADD. Case 3. Carnitine deficiency, systemic primary. (CDSP) (OMIM 212140) A one-year-old girl with progressive dyspnea since birth and a history of parental consanguinity. Severe dilated cardiomyopathy with episodes of cardiac decompensations, hepatomegaly, anemia, generalized hypotonia, but no hypoglycemia were demonstrated prior to cardiac arrest. Extremely low carnitine level noted in dried blood spots via tandem mass spectrometry.
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PMID:Mitochondrial fatty acid oxidation disorders in Thai infants: a report of 3 cases. 1240 51

This retrospective study included 18 pediatric cases (median age, 3 years) with pathologically proved hemophagocytic syndrome (HPS) from a single institution during 1992 and 2001. There were 9 males and 9 females. Prolonged fever, cytopenia, liver dysfunction and hepatomegaly were the most common features at presentation. Sixteen (88.9%) cases were previously healthy. The case fatality rate was 61.1%, and all fatal cases died within 2 months of disease onset. The infectious agents associated with HPS were identified in 11 cases (61.1%), and 8 (72.7%) of them had evidence of Epstein-Barr virus (EBV) infection or reactivation. Underlying immunologic disorder or neoplastic disease was identified in 11.1% of the cases. Children less than 3 years of age with HPS were more vulnerable to neutropenia-associated bloodstream infection (85.7% vs 27.3%; p=0.025). Pseudomonas aeruginosa (3) and Candida tropicalis (2) were the 2 most commonly isolated pathogens. Regarding specific management of HPS, intravenous immunoglobulin and steroids were the first-line agents and were administered in 16 cases and 11 cases, respectively, while etoposide was administered in 5 refractory cases during the late phase of disease. Most HPS occurred in previously healthy children, and a substantial proportion of cases rapidly progressed to death. Most cases were associated with viral infection, particularly EBV, and young children tended to develop neutropenia-associated bacteremia during the active phase of the disease.
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PMID:Hemophagocytic syndrome: a review of 18 pediatric cases. 1522 Oct 35

Clinical manifestations of chronic viral hepatitis B (CVH-B) in replicative phase in patients with superinvasive opisthorchosis comprise the asthenic-vegetative syndrome, pain syndrome, dyspepsia (53.3% of cases) and hepatomegaly (46.7% of cases). Superinvasive opisthorchosis, when combined with CVH-B, stimulates the cytolysis of hepatocytes and the intensity of inflammation; it also shapes the fibrosis of the liver, which complicates the course of viral infection and its outcome.
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PMID:[The clinical features and the structural and functional status of the liver in chronic viral hepatitis B concurrent with superinvasion opisthorchiasis]. 1610 23

In this case report, a young woman with gallbladder sludge and acute pancreatitis due to acute hepatitis A (HAV) is presented. She was admitted to our hospital with abnormal hepatic enzymes. Five days prior to her admission, an initial abdominal ultrasound was performed at another hospital and revealed no abnormality, while her serum aspartate aminotransferase (AST) level was at the upper limit of normal (ULN) x 8. A second ultrasound was performed at our hospital and revealed a gallbladder wall thickness (9.3 mm), gallbladder sludge in the gallbladder lumen, pancreatic edema, ascites, and hepatomegaly while AST was at the ULN x 50. Magnetic resonance imaging and magnetic resonance cholangiopancreatography revealed imaging features of an acute stage of pancreatitis and gallbladder wall thickness with coexisting sludge in the gallbladder lumen. HAV infection was diagnosed by the detection of immunoglobulin M against HAV in the serum. The patient underwent two repeated abdominal ultrasound examinations on the 5th (AST was at the ULN x 3) and the 20th days (AST was at the normal) after her discharge, and both revealed normal findings. In our case, we observed reversible changes in the hepatobiliary and pancreatic system which was related to the severity of hepatic necro-inflammation. HAV-associated pancreatitis may be due to the formation of biliary sludge during the acute phase of the viral illness, but this association needs further investigation.
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PMID:Gallbladder sludge and acute pancreatitis induced by acute hepatitis A. 1790 17

Hepatic sinusoidal obstruction syndrome is frequently linked to high-dose chemotherapy/total-body irradiation in recipients of haematopoietic stem cell transplantation, long-term use of azathioprine after organ transplantation and other chemotherapeutic agents. The incidence of hepatic sinusoidal obstruction syndrome varies from 0% to 70%, and is decreasing. Disease risk is higher in patients with malignancies, hepatitis C virus infection, those who present late, when norethisterone is used to prevent menstruation, and when broad-spectrum antibiotics and antifungals are used during and after the conditioning therapy. Hepatic sinusoidal obstruction syndrome presents with tender hepatomegaly, hyperbilirubinaemia and ascites, and diagnosis is mainly clinical (Seattle and Baltimore Criteria). Imaging excludes biliary obstruction and malignancy, but cannot establish accurate diagnosis. Hepatic sinusoidal obstruction syndrome may be prevented by avoiding the highest risk regimens, using non-myelo-ablative regimens, and reducing total-body irradiation dose. Treatment is largely symptomatic and supportive, because 70-80% of patients recover spontaneously. Tissue plasminogen activator plus heparin improves outcome in <30% of cases. Defibrotide, a polydeoxyribonucleotide, is showing encouraging results. Transjugular intrahepatic porto-systemic shunt relieves ascites, but does not improve outcome. Liver transplantation may be an option in the absence of malignancy. Prognosis is variable and depends on disease severity, aetiology and associated conditions. Death is most commonly caused by renal or cardiopulmonary failure.
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PMID:Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome. 1639 76

Viral infection is one of the postulated causes of neonatal cholestasis. In addition to earlier reports on the association of viral infection and intrahepatic cholestasis (IH), recent studies have suggested a similar link to extra hepatic biliary atresia (EHBA). The aim of this work was to evaluate the role of some viral infections in neonates presenting with cholestasis to the Neonatology Ward of Zagazig University Hospitals. Sixty-two neonates were included in the study (44 cholestatic neonates and 18 apparently healthy neonates as the control group). All neonates were subjected to full history taking and complete physical examination. Laboratory investigations included CBC, liver function tests, bleeding profile, blood cultures, abdominal ultrasound and detection of HBsAg and serum IgM antibodies against certain viruses (CMV, Reovirus III, HSV I, HSV II, Rubella virus) using ELISA. Radionuclide cholescintigraphy was performed for patients only. The study revealed that cholestatic neonates were significantly associated with dark urine, pale stool and hepatomegaly compared with the control (P<0.004, P<0.001, P<0.008, respectively). Quantitation of IgM antibody titre using ELISA revealed significantly higher levels of serum anti-CMV IgM and anti-Reovirus III IgM in cholestatic than in the control groups. No significant differences were found in levels of anti-HSV I, anti-HSV II or anti-rubella antibodies between cholestatic and control groups. HBsAg was negative for all neonates; cholestatic and control. Lastly, no significant differences were found between neonates with EHBA (7 cases) and Intrahepatic cholestasis (37 cases) regarding anti -CMV IgM or anti -Reo III IgM. It can be concluded that CMV and Reovirus type 3 infections of the neonates are associated with the development of cholestatic disorder, not only due to IH cholestasis but also due to the production of EHBA.
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PMID:Role of some viral infections in neonatal cholestasis. 1673 27

While the impact of the dengue viruses on liver function is prominent as shown by hepatomegaly, liver enzyme abnormality, occasional fulminant hepatic failure and histological changes including hepatocellular necrosis, significant debate exists as to the possible involvement of the predominant cell type in the liver, hepatocytes, in the disease process. To address this issue purified human primary hepatocytes were exposed to dengue virus serotype 2 and the production of de novo viral progeny was established by standard plaque assay, RT-PCR and immunocytochemistry. To investigate the response of the primary hepatocytes to infection, the expression of a panel of 9 cytokine genes (IFN-beta, TRAIL, MCP-1, IL-6, IL-1beta, IL-8, MIP-1alpha, MIP-1beta, and RANTES) was semi-quantitatively investigated by RT-PCR and up-regulation of TRAIL, MIP-1alpha, IFN-beta, MIP-1beta, IL-8, and RANTES was observed in response to infection. The induction of IL-8 in response to infection was accompanied by the secretion of IL-8 as verified by ELISA assay. The ability of hepatocytes to be infected with dengue virus serotype 2 in vitro support evidence implicating human hepatocytes as a target cell in cases of dengue virus infection, and provide the first experimental evidence to support the large number of clinical studies that implicate the liver as a critical target organ in severe cases of dengue infection.
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PMID:Infection of human primary hepatocytes with dengue virus serotype 2. 1724 28

Acute Q fever is a worldwide zoonosis caused by Coxiella burnetii infection. In Taiwan, cases of acute Q fever increased during 3 y of observation, especially at Kaohsiung County and City in southern Taiwan. From 15 April 2004 to 15 April 2007, a total of 67 cases of acute Q fever were identified at E-Da hospital located at Kaohsiung County. 19 (28.4%) patients had a history of travel in rural areas and only 1 had been outside southern Taiwan. 21 (31.3%) patients had a history of animal contact. 20 (30.8%) of the 65 examined patients had underlying chronic hepatitis B or hepatitis C virus infection. Fever (98.5%), chills (79.1%), headache (79.1%), relative bradycardia (44.8%), elevated aminotransferases (100%), and thrombocytopenia (74.6%) were common manifestations. 12 (19.0%) cases had abnormal findings on chest X-ray. Fatty liver (50.0%) and hepatomegaly and/or splenomegaly (41.9%) were found by abdominal image examinations. 42 (76.4%) of 55 cases had defervescence within 3 d after treatment, whereas 4 (7.3%) had spontaneous remission. Acute Q fever is an endemic infectious disease with hepatitis rather than pneumonia as the major presentation in southern Taiwan and the emergence of Q fever is due to increased alertness for the disease by physicians.
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PMID:Acute Q fever: an emerging and endemic disease in southern Taiwan. 1785 9


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