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)

Hepatoportal sclerosis is the term used to name a clinicopathological condition responsible for non-chirrotic portal hypertension. A three cases report of children with hepatoportal sclerosis is presented associated with portal vein thrombosis. The first two patients presented as main complaint upper digestive hemorrhage and the third one was admitted for investigation of hepatosplenomegaly. The ultrasonographic exam revealed alterations indicative of extrahepatic portal vein thrombosis in the three cases. The patients underwent liver biopsy for they had presented altered liver enzymes. The main histological findings were: subintimal sclerosis, portal fibrosis and telangiectases of the intrahepatic venous branches, consistent with the diagnosis of hepatoportal sclerosis. The three patients showed good evolution, being the hemorrhage controlled in the first two cases through esclerotherapy of esophageal varices.
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PMID:[Hepatoportal sclerosis associated with portal vein thrombosis in children: report of 3 cases]. 949 28

In 1992 a foci of Schistosomiasis mekongi was rediscovered in the province of Kracheh in Northeast Cambodia. Severe clinical signs due to portal hypertension, which were frequently observed in this population, allowed the discovery of this 'forgotten' focus. Elements of the perception of the population and clinical observations of 20 severe cases due to S. mekongi infections are presented. Interviews with patients and villagers of the area of Kracheh showed severe psychosocial impact including fear from death, infirmity and invalidity. The symptoms of schistosomiasis were well known by the population and were reported to have increased in frequency in the last two decades. They have received traditional names and specific traditional treatment. The description of the clinical cases illustrates the severe pathology, which was observed in the hospital of Sambour, in the north of the province of Kracheh. It shows the pathogenic potential of S. mekongi in all age groups (from 7 to 58 years old): cachexia, hepatosplenomegaly, stunting and retardation of puberty, decompensation of portal hypertension with ascites and rupture of oesophagual varicies. The efficacy of the treatment in the severe stages varies. A follow-up after 30 months showed that 5 patients died, 5 initially improved but then relapsed, 3 remained unchanged and only 5 patients clearly improved. Two patients could not be followed-up. The clinical observations and interviews show severe pathology with impact at both individual and community level. The infection with S. mekongi is the main factor but additional concomitant factors are responsible for this fact. At a certain stage of the disease the prognosis for successful treatment is very low. These observations show the importance of the foci in the Province of Kracheh, Cambodia and underline the need for a long-term global intervention.
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PMID:[A foci of Schistosomiasis mekongi rediscovered in Northeast Cambodia: cultural perception of the illness; description and clinical observation of 20 severe cases]. 1058

People infected with schistosomes may present with a variety of clinical manifestations ranging from the relatively asymptomatic intestinal (INT) form to the hepatointestinal (HI) or hepatosplenic (HS) forms characterized by hepatomegaly and hepatosplenomegaly with severe portal hypertension, respectively. Flow cytometry analyses were used to evaluate the contribution of apoptosis in specific cell populations from schistosomiasis patients to the development of the different clinical forms of the disease. The results showed that cell death induced by combinations of specific antigen and cytokines corresponds with specific clinical presentations. It was shown that soluble egg antigen (SEA) increased the level of apoptosis only in T cells from INT patients. Stimulation with soluble lung worm antigen preparation (SLAP) did not induce significant differences in the levels of apoptosis in T cells from the patients with the different clinical forms of schistosomiasis. These results suggest for the first time that apoptosis plays an important role in the modulation of the anti-SEA response in INT patients.
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PMID:Apoptosis: a mechanism of immunoregulation during human Schistosomiasis mansoni. 1084 6

Schistosomiasis japonica is a serious communicable disease and a major disease risk for more than 30 million people living in the tropical and subtropical zones of China. Infection remains a major public health concern despite 45 years of intensive control efforts. It is estimated that 865,000 people and 100,250 bovines are today infected in the provinces where the disease is endemic, and its transmission continues. Unlike the other schistosome species known to infect humans, the oriental schistosome, Schistosoma japonicum, is a true zoonotic organism, with a range of mammalian reservoirs, making control efforts extremely difficult. Clinical features of schistosomiasis range from fever, headache, and lethargy to severe fibro-obstructive pathology leading to portal hypertension, ascites, and hepatosplenomegaly, which can cause premature death. Infected children are stunted and have cognitive defects impairing memory and learning ability. Current control programs are heavily based on community chemotherapy with a single dose of the drug praziquantel, but vaccines (for use in bovines and humans) in combination with other control strategies are needed to make elimination of the disease possible. In this article, we provide an overview of the biology, epidemiology, clinical features, and prospects for control of oriental schistosomiasis in the People's Republic of China.
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PMID:Schistosomiasis in the People's Republic of China: prospects and challenges for the 21st century. 1129 39

A 53-year-old man suffering from rheumatoid arthritis for 15 years presented with bleeding esophageal varices, hepatosplenomegaly and normal splenoportal venous axis. Liver biopsy revealed mild fibrosis, suggestive of non-cirrhotic portal fibrosis (NCPF). There are reports of the association of idiopathic portal hypertension, a condition similar to NCPF, with progressive systemic sclerosis, Hashimoto's thyroiditis and systemic lupus erythematosus.
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PMID:Non-cirrhotic portal fibrosis in a patient with rheumatoid arthritis. 1167 33

Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3 years, range 1-22.3 years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5 years (range 3.1-33.6 years). One and 5 years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n = 3), sclerotherapy (n = 2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n = 1). Of the nine survivors (mean age 12.8 years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.
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PMID:Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease. 1211 59

Hepatosplenic schistosomiasis involving organomegaly, portal fibrosis and portal hypertension has been observed in autopsy studies. Here, we have tested the hypothesis that hepatosplenic disease including organomegaly and markers of increased portal pressure can occur in school aged children in the absence of fibrosis. A case-only study of 96 children aged 7-20 years defined by ultrasound detectable hepatomegaly was undertaken in Makueni district, Kenya. A novel method of clinical examination that involved a consensus scoring by three or four examiners was used to classify children as presenting with severe or moderate hepatosplenic disease after palpation of livers and spleens. Ultrasound examination of livers and spleens was based on the Niamey protocol. Clinical measurements included spleen enlargement along the mid-clavicular and mid-axillary lines, liver enlargement along the mid-sternal (MSL) and mid-clavicular lines, as well as organ consistency. The clinical examination indicated that 9% and 60% of the children had severe or moderate hepatosplenomegaly, respectively. Amongst egg-positive children, all clinical measurements, except MSL liver enlargement, correlated with egg count, as did portal vein diameter, spleen length and liver length measured by ultrasound. Peri-portal fibrosis was not observed in any child, whereas 28% of the children were classified as having increased portal pressure according to World Health Organization criteria. There was no effect of malaria parasitaemia or hepatitis seropositvity on any of the observed parameters. These results indicate that hepatosplenic disease in school-aged children attributable to S. mansoni infection, involving hepatosplenomegaly and increased portal vein diameter, can occur in the absence of peri-portal fibrosis.
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PMID:Detailed clinical and ultrasound examination of children and adolescents in a Schistosoma mansoni endemic area in Kenya: hepatosplenic disease in the absence of portal fibrosis. 1507 64

Hepatopulmonary syndrome (HPS) is defined as a clinical triad including liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations. We report a 61-year-old male presented with fatigue, long-lasting fever, loss of weight, signs of portal hypertension, hepatosplenomegaly, cholestasis and progressive dyspnoea over the last year. Clinical, laboratory and histological findings confirmed the diagnosis of granulomatous hepatitis. HPS due to hepatic granuloma-induced portal hypertension was proved to be the cause of severe hypoxemia of the patient as confirmed by contrast-enhanced echocardiography. Reversion of HPS after corticosteroid therapy was confirmed by a new contrast-enhanced echocardiography along with the normalization of cholestatic enzymes and improvement of the patient's conditions. This is the first case of complete reversion of HPS in a non-cirrhotic patient with hepatic granuloma, indicating that intrapulmonary shunt in liver diseases is a functional phenomenon and HPS can be developed even in miscellaneous liver involvement as in this case.
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PMID:Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatment for granulomatous hepatitis: a case report and review of the literature. 1648 41

In systemic mastocytosis the liver, spleen, and lymph nodes may be infiltrated by mast cells, with patterns of infiltration specific for each tissue. This may result in hepatosplenomegaly and enlarged lymph nodes. Extensive involvement with mast cells may also be associated with organ dysfunction. Specifically, in the case of liver, mast cell infiltration may result in fibrosis, portal hypertension, and abdominal ascites. Clinically significant involvement of the liver, spleen, and lymph nodes appears to be more common in patients with aggressive forms of mastocytosis, including those with a hematologic disorder.
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PMID:The liver, spleen, and lymph nodes in mastocytosis. 1679 8

Hepatosplenomegaly is one of the cardinal signs of Hunter disease; however, portal hypertension has not been described. We report portal hypertension in an adult Hunter patient with the attenuated phenotype.
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PMID:Portal hypertension in a patient with Hunter disease. 1697 78


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