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)

The treatment of choice, in the Northeast of Brazil, of patients with a history of upper GI bleeding from ruptured esophageal varices (EV) and with hepatosplenomegaly secondary to schistosomiasis (HSS), is splenectomy and left gastric vein ligation (SLGL). However, the effect of this procedure on the EV pressure, the parameter that best correlates to re-bleeding risk, has not yet been evaluated. With the introduction of a minimally invasive technique to measure the EV pressure, it has become possible to assess the effect of this surgery without an increased risk to the patient. SLGL was performed in twenty two patients with a history of HSS and upper GI Bleeding secondary to esophageal varices. The non-invasive endoscopic pneumatic balloon was used to measure the EV pressure before surgery and the results were then compared with measurements made between five and eight days post-operatively. The pre-operative EV pressure ranged from 20.0 mmHg to 28.7 mmHg (mean 24.35 +/- 2.36 mmHg), with no correlation between the pressure and the calibre of the varices. In the post-operative period, a significant decrease in EV pressure was observed, ranging from 14.6 mmHg to 21.5 mmHg (mean 17.29 +/- 1.75 mmHg, p < 0.001). These results support the use of SLGL in patients with HSS and a history of variceal bleeding. The operation results in, at least for the short term and in the majority of cases, a reduction in the EV pressure, and therefore a reduced risk of repeating upper GI Bleeding.
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PMID:Splenectomy and ligation of the left gastric vein in schistosomiasis mansoni: the effect on esophageal variceal pressure measured by a non-invasive technique. 1212 10

The long-term effect of single-dose praziquantel on morbidity and mortality from Schistosoma mansoni was investigated in surveys in 1987 and 1994 in central Sudan. Prevalence of infection dropped from 53% to 34%, and intensity of infection (> or = 400 eggs/g of faeces) from 31% to 18%. There was a reduction in hepatomegaly and hepatosplenomegaly, although splenomegaly alone was unchanged. Prevalence of periportal fibrosis decreased from 14% to 10%. Endoscopic investigation of patients with fibrosis showed a reduction in oesophageal varices from 47% to 30%. Mortality due to bleeding varices was high (community-wide, up to 11/100 infected patients with bleeding). Thus praziquantel mass treatment can be spaced to a much longer period, reducing the expense of treatment, delivery and distribution.
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PMID:Effects of single-dose praziquantel on morbidity and mortality resulting from intestinal schistosomiasis. 1219 50

The purpose of this study was to analyse the course of pregnancy of a 20-year-old woman suffering from congenital hepatic fibrosis (CHF), a genetic disease with recessive autosomic transmission, attributed to the group of hepato-nephro-pancreatic fibropolycystic familial diseases. This was the only time such a case had ever been encountered in a pregnancy in the history of Turin University's Obstetrics and Gynaecological Clinic. The case of a 20-year-old woman is considered. She was admitted to our observation at the Obstetrics and Gynaecological Department of Faculty "B" of the University of Turin, suffering from CHF, a primigravid in the eighth week of gestation. Haematochemical tests carried out on the patient showed a platelet deficiency without evident changes in liver and renal function. Objective examination revealed a slight hepatosplenomegaly confirmed by abdominal echotomography. The haematochemical examinations subsequently carried out confirmed thrombocytopenia due to hepatosplenic hyperhaemocataresis. In agreement with the will of the patient and her family, and after having heard the opinion of our hospital's anaesthetists and internists, a conservative approach was decided on and close monitoring of maternal and foetal conditions was planned, with hospitalisations scheduled for every fortnight. Together with the internists and anaesthetists, it was decided to allow the pregnancy to proceed as far as foetal maturity but not to term, to avoid problems relating to the excessive increase in uterine volume which would have led to pressure on the portal tree and so favoured haemorrhaging due to rupture of the oesophageal varices. Caesarean delivery was planned for the 37th week of gestation. The birth went according to plan and the foetus was a live, vital male with Apgar index of 9/9. The placenta presented normal characteristics and a weight of 590 g. No haemorrhagic complications were observed either in the course of surgery or in the postoperative phase, notwithstanding the preoperative finding of a platelet count of 64,000 platelets/ mm3. Although in our case neither the pregnancy nor the delivery constituted a serious danger for the life of the mother, possible obstetric complications are among the most serious encountered in obstetric pathology and can lead both to maternal death and to the intrauterine death of the foetus. For these reasons we believe it essential to provide correct, clear and adequate information about the risks involved for patients suffering from congenital hepatic fibrosis, especially if they are associated with renal polycystic disease, and who are admitted to our wards with the desire to programme a pregnancy.
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PMID:[Congenital hepatic fibrosis in pregnancy]. 1243 35

In recent years, cases of severe morbidity (fibrosis, haematemesis, hepatosplenomegaly, ascites) caused to Schistosoma mansoni infections are on the rise in Northern Senegal. The neuropeptide somatostatin is reported to decrease portal pressure, control variceal bleeding and fibrosis, and reduce Schistosoma-caused clinical morbidity in the rodent model. The aim of this study was to delineate the role of somatostatin in S. mansoni-caused pathogenesis, by studying host levels of somatostatin in the peripheral blood of uninfected and S. mansoni-infected individuals. Subjects from the district dispensary at Richard Toll, in the Medical Region of Saint-Louis, Senegal, infected with S. mansoni and suffering from severe morbidity were selected. A separate group consisted of individuals resident in the same region but uninfected at the time of the study. Significantly lower somatostatin levels were detected in severe morbidity patients, compared with the exposed but uninfected subjects. In patients with schistosomiasis physiological levels of somatostatin may determine disposition of particular individuals towards severe morbidity, as opposed to others. Host pathology can thus be alleviated by the therapeutic ability of somatostatin to treat bleeding oesophageal varices, reduce portal pressure and prevent progression to severe fibrosis.
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PMID:Lower levels of the circulating neuropeptide somatostatin in Schistosoma mansoni infected patients may have pathological significance. 1253 48

A 37-year old woman with systemic lupus erythematosus (SLE) complicated by pulmonary hypertension (PHT) was admitted to evaluate abnormal liver function. Radiological imaging study, including ultrasonography, computed tomography and magnetic resonance imaging and upper gastrointestinal endoscopy, revealed multiple hepatic nodules, hepatosplenomegaly and esophageal varices. Percutaneous needle liver biopsy showed non-cirrhotic hepatic nodules with hyperplastic hepatocytes surrounded by atrophic hepatocytes, confirming the diagnosis of nodular regenerative hyperplasia (NRH) associated with non-cirrhotic portal hypertension (PT). NRH of the liver is known to be a very rare hepatic manifestation in rheumatic diseases. This case shows the association of NRH with porto-pulmonary hypertension in SLE.
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PMID:Association of nodular regenerative hyperplasia of the liver with porto-pulmonary hypertension in a patient with systemic lupus erythematosus. 1712 May 97

We report a patient with fever, progressive jaundice and abdominal distension, having marked pallor, icterus, ascites and hepatosplenomegaly. Investigations revealed pancytopenia and deranged liver functions. Doppler study revealed portal hypertension and endoscopy showed grade II oesophageal varices. Liver biopsy suggested leishmanial hepatitis and bone marrow demonstrated multiple LD bodies. Diagnosis of "visceral leishmaniasis with leishmanial hepatitis with portal hypertension" was made. The case is being reported because of its rarity apart from it being an unusual presentation of kala-azar.
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PMID:Visceral leishmaniasis masquerading as chronic liver disease. 1724 62

Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. A 52-year-old woman presented with urticaria-like skin symptoms, anemia, and thrombocytopenia. Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Chemotherapy with cytosine arabinoside and idarubicin was ineffective and the patient died of multi-organ failure with rapidly progressing hepatosplenomegaly and large-volume ascites 3 months after admission. At autopsy the bone marrow, spleen, liver, and lymph nodes were extensively infiltrated by atypical tumor cells with occasional bi- or multi-lobated nuclei. They were positive for mast cell tryptase and possessed an activating mutation of the c-kitgene (D816V). Ascites (2200 mL) and non-ruptured esophageal varices with submucosal hemorrhage indicated the presence of severe portal hypertension. Although there was no evidence of liver cirrhosis, the hepatic sinusoids were clogged with tumor cells, with a tendency to be more severe in the perivenular areas, and the lumens of central veins were obliterated by tumor cell infiltration. The present case demonstrates that non-cirrhotic portal hypertension due to blocking of sinusoidal and venous flow could be a serious complication in mast cell leukemia.
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PMID:Mast cell leukemia with rapidly progressing portal hypertension. 1988 34

A 27-year-old man was admitted to our hospital for evaluation and treatment of liver dysfunction with jaundice and hepatosplenomegaly. The patient had severe photosensitivity from childhood. Upper gastrointestinal endoscopy revealed mild esophageal varices. The clinical manifestations, analyses of the urine and blood for porphyrins and skin biopsy led us to a diagnosis of erythropoietic protoporphyria. Because of acute deterioration of liver function, living donor liver transplantation was performed. The resected liver revealed cirrhosis. We report a rare case of erythropoietic protoporphyria with severe liver dysfunction and present a review of the literature.
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PMID:[A case of erythropoietic protoporphyria with severe liver dysfunction]. 2155 48

Gauchers disease is a rare lysosomal storage disorder characterized by abnormal accumulation of lipid-laden macrophages in different organs. Though hepatosplenomegaly is commonly found, symptomatic presentation with portal hypertension is rare. We report a child with liver cirrhosis and bleeding esophageal varices who was diagnosed with Gaucher's disease.
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PMID:Gauchers disease presenting with portal hypertension. 2208 Jun 81

A 75-year old man had been diagnosed at 42 years of age as having polycythemia vera and had been monitored at another hospital. Progression of anemia had been recognized at about age 70, and the patient was thus referred to our center in 2008 where secondary myelofibrosis was diagnosed based on bone marrow biopsy findings. Hematemesis due to rupture of esophageal varices occurred in January and February of 2011. The bleeding was stopped by endoscopic variceal ligation. Furthermore, in March of the same year, hematemesis recurred and the patient was transported to our center. He was in irreversible hemorrhagic shock and died. The autopsy showed severe bone marrow fibrosis with mainly argyrophilic fibers, an observation consistent with myelofibrosis. The liver weighed 1856 g the spleen 1572 g, indicating marked hepatosplenomegaly. The liver and spleen both showed extramedullary hemopoiesis. Myelofibrosis is often complicated by portal hypertension and is occasionally associated with gastrointestinal hemorrhage due to esophageal varices. A patient diagnosed as having myelofibrosis needs to be screened for esophageal/gastric varices. Myelofibrosis has a poor prognosis. Therefore, it is necessary to carefully decide the therapeutic strategy in consideration of the patient's concomitant conditions, treatment invasiveness and quality of life.
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PMID:Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension. 2285 73


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