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)

The clinico-pathological features of 42 children with autoimmune haemolysis are described. Over 65% of cases were seen before their 5th birthday. In this group males predominated by the ratio of 2.5:1, but in the older children both sexes were equally affected. The incidence decreased from 1 in 188 X 10(3) in young males to 1 in 1,780 X 10(3) in children over 10. Cases were classified serologically. Of particular note was the frequency of Donath-Landsteiner haemolysis which equalled that due to warm autoantibodies; together these groups made up 79% of the total cases. Most haemolytic episodes followed an acute infection. This was frequently mild and often involved the upper respiratory tract; in only 2 patients was haemolysis associated with underlying collagenosis. Typically there was a sudden onset of pallor and malaise; jaundice, splenomegaly and hepatomegaly were found in about half the subjects. Haemoglobinuria was characteristic of Donath-Landsteiner haemolysis. The illness was severe, with Hb levels falling below 6.0 g/dl in 28 patients. Prednisolone, blood transfusion and, where indicated, antibiotics were usually effective in treating the illness, with splenectomy reserved for cases where this treatment was unsatisfactory. In several individuals no treatment was required. Recovery was rapid, and complete recovery occurred in 83% of patients, usually within 6 months. Although 2 patients died, a generally optimistic prognosis can be given, particularly in the absence of an underlying chronic disorder.
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PMID:Autoimmune haemolysis in childhood and adolescence. 643 92

A case of single ventricle with pulmonary stenosis operated at 20 years of age by direct atrio-pulmonary anastamosis is presented. This procedure in contrast to classical techniques does not use an intraventricular patch or a valved ventriculo-pulmonary conduit. It is an adaptation of the Fontan technique initially proposed for tricuspid atresia. The operation consisted of closing the right atrioventricular orifice with a piece of Dacron, suturing the pulmonary valves (which were stenosed) and connecting the right atrium and pulmonary artery by a direct anastamosis using the auricle. The clinical result remains satisfactory one year after surgery: the cyanosis has regressed, the functional tolerance is perfect, there is no hepatomegaly and the patient is in sinus rhythm. The technique is very simple and involves less risk to the His bundle than intraventricular septalisation. It should be reserved to cases of single ventricle with low pulmonary pressures and resistance without cardiac failure. The long-term prognosis depends on the tolerance of the right atrium and is, at present, unknown.
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PMID:[Treatment of single ventricle by direct atriopulmonary anastomosis without tube or valve. Apropos of a case operated on]. 681 Jul 87

Of 16 infants with infantile hepatic hemangioendothelioma, 14 (88%) presented before age 3 months. For seven cases (44%), the diagnosis was suspected from antenatal ultrasonographic findings. Two (13%) presented with asymptomatic hepatomegaly. The most common presenting features were high-output cardiac failure in 11 (69%), consumptive coagulopathy in 12 (75%), and anemia in 12(75%). Sixty-three percent of the children had associated cutaneous hemangiomata, and disseminated hemangiomatosis was noted in two (13%). Medical measures were effective in stabilizing seven (44%) cases with high-output congestive cardiac failure and/or consumptive coagulopathy. Partial right hepatectomy was successful for four patients; the only death occurred in a newborn, after intraperitoneal rupture of the hepatic hemangioma. Embolization was used in two children to induce involution. Spontaneous involution occurred in two patients. Initially, hepatic hemangiomas should be treated conservatively, with surgery reserved for intractable cardiac failure and/or refractory consumptive coagulopathy.
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PMID:Infantile hepatic hemangioendothelioma: the role of surgery. 878 79

Amebic liver abscess should be suspected in travelers returning from endemic areas or in immunocompromised patients who present with fever, right upper quadrant pain, hepatomegaly, and a liver lesion on an imaging study. Rapid initiation of therapy without serologic confirmation of infection, if necessary, is important to minimize complications. Metronidazole is given orally or intravenously for 14 days. The drug is generally well tolerated and leads to resolution of symptoms in most patients within 2 to 3 days. It is effective against luminal cysts in only 50% of patients and, therefore, must be followed by a course of treatment with paromomycin (Humatin; Parke-Davis, Morris Plains, NJ) or another luminal antiamebic agent to eradicate the parasite. Image-guided drainage of an amebic liver abscess is indicated in patients who do not respond to antimicrobial therapy or who are at risk of abscess rupture. Surgery is reserved for patients with a ruptured abscess. Although medical therapy is generally successful in the treatment of infection caused by Entamoeba histolytica, the development of potent vaccines will be needed for worldwide eradication of disease attributable to E. histolytica.
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PMID:Amebic Liver Abscess. 1240 81

Visceral leishmaniasis, or kala-azar, is a chronic disease caused by Leishmania donovani, Leishmania chagasi or Leishmania infantum. The disease is transmitted through the bite of a species of sandfly of the genus Phlebotomus, releasing amastigote parasites that invade various organs of the body and eventually result in such conditions as anemia, splenomegaly and hepatomegaly. Although no vaccine exists for the disease, diagnostic techniques based not only on pathological tests, but more sophisticated detectors such as polymerase chain reaction, enzyme-linked immunosorbent assay, latex agglutination and immunochromatographic strip testing have been developed. Traditional treatment for the disease consists of two pentavalent antimonial drugs, sodium stibogluconate and meglumine antimoniate, but the growing resistance to these drugs has compelled scientists to search for new efficient compounds. (c) 2002 Prous Science. All rights reserved.
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PMID:Visceral Leishmaniasis: Clinical Features, Pathology, Diagnosis and Chemotherapeutic Developments. 1267 77

Amebiasis can be considered the most aggressive disease of the human intestine, responsible in its invasive form for clinical syndromes, ranging from the classic dysentery of acute colitis to extra-intestinal disease, with emphasis on hepatic amebiasis, unsuitably named amebic liver abscess. Found worldwide, with a high incidence in India, tropical regions of Africa, Mexico and other areas of Central America, it has been frequently reported in Amazonia. The trophozoite reaches the liver through the portal system, provoking enzymatic focal necrosis of hepatocytes and multiple micro-abscesses that coalesce to develop a single lesion whose central cavity contains a homogeneous thick liquid, with typically reddish brown and yellow color similar to "anchovy paste". Right upper quadrant pain, fever and hepatomegaly are the predominant symptoms of hepatic amebiasis. Jaundice is reported in cases with multiple lesions or a very large abscess, and it affects the prognosis adversely. Besides chest radiography, ultrasonography and computerized tomography have brought remarkable contributions to the diagnosis of hepatic abscesses. The conclusive diagnosis is made however by the finding of Entamoeba histolytica trophozoites in the pus and by the detection of serum antibodies to the amoeba. During the evolution of hepatic amebiasis, in spite of the availability of highly effective drugs, some important complications may occur with regularity and are a result of local perforation with extension into the pleural and pericardium cavities, causing pulmonary abscesses and purulent pericarditis, respectively The ruptures into the abdominal cavity may lead to subphrenic abscesses and peritonitis. The treatment of hepatic amebiasis is made by medical therapy, with metronidazole as the initial drug, followed by a luminal amebicide. In patients with large abscesses, showing signs of imminent rupture, and especially those who do not respond to medical treatment, a percutaneous drainage must be performed with either ultrasound or computerized tomography guidance. Surgical drainage by laparotomy is reserved to patients with secondary infections.
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PMID:Hepatic amebiasis. 1295 80

The presence of granulomas in the liver raises consideration of a wide differential diagnosis, but in most Western series, sarcoidosis accounts for a majority of cases. This review will focus specifically on the diagnosis of and therapy for hepatic sarcoidosis. Sarcoidosis is a systemic granulomatous disease of unknown etiology. Hepatic involvement of sarcoidosis was described in 11.5% of 736 patients enrolled in the ACCESS study. However, presence alone of granulomas in an organ in sarcoidosis does not dictate treatment. The decision to treat should be based on symptoms and severity of disease. Although hepatic involvement usually is asymptomatic, a minority of patients progress to chronic cholestatic disease, portal hypertension, and cirrhosis that may require liver transplantation. Treatment of hepatic sarcoidosis should be reserved for patients who manifest this spectrum of disease. Glucocorticoid treatment is first-line therapy for hepatic sarcoidosis, improving symptoms and abnormal laboratory values but generally having no effect on progression of disease. In addition to glucocorticoids, immunomodulators such as azathioprine, methotrexate, hydroxychloroquine, and infliximab have been used with some positive effects on symptoms, liver enzyme abnormalities, and hepatomegaly, but none has been shown to prevent progression of disease. Ultimately, in cases of overt liver failure, liver transplantation is the definitive treatment. Overall, treatment for hepatic sarcoidosis is targeted toward alleviation of symptoms but has no curative potential at this time. Focus should be on discovering the etiology of the disease to target therapy at prevention, not cure.
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PMID:Diagnosis and treatment of hepatic sarcoidosis. 1708 81

Hepatic venous outflow tract obstruction (HVOTO) comprises of constellation of disorders causing obstruction of hepatic venous outflow or suprahepatic inferior vena cava (IVC) or both and leading to increased hepatic sinusoidal pressure and portal hypertension. Clinical presentation in HVOTO includes both acute onset or chronic insidious onset of the disease and predominant clinical manifestations consist of ascites, hepatomegaly, and portal hypertension. IVC/hepatic vein (HV) web or thrombosed hepatic veins replaced by fibrotic constriction or thrombus in suprahepatic IVC is encountered as the pathogenic process at such obstructions. Due to advances in radiologic techniques there has been a changes in the management protocol of HVOTO with surgery or liver transplantation reserved for patients not suitable for radiological interventions or requiring liver transplantation. The present article reviews the techniques of various radiological interventions in HVOTO and their efficacy.
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PMID:Radiological interventions in HVOTO--practical tips. 2192 50