Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A prospective study, aimed to investigate the aetiology of an unusual clustering of cases of severe acute haemolytic anaemia affecting a high percentage of the adult population, was carried out in two isolated Yanomamo communities of the Upper Orinoco basin in Venezuela. Twenty-six patients with active or recent episodes of severe haemolysis were evaluated. All of them exhibited massive liver and spleen enlargement and fulfilled the diagnostic criteria of the hyperreactive malarious splenomegaly (HMS) syndrome. In four cases with advanced non-alcohol-related chronic liver disease, hypersplenism, severe haemolytic anaemia and acanthocytosis, the characteristic clinical and laboratory findings of spur cell anaemia were documented. Chronic infection by the HBV and HCV was present in three of them. However, in most of the 22 additional HMS cases, the acute haemolytic condition appeared associated with the occurrence of a cold agglutinin-mediated autoimmune response. The clustering of a significant number of cases of severe acute haemolysis in HMS patients from this small isolated aboriginal community is most unusual, and represents a serious complicating factor for a population already beleaguered by a high prevalence of malaria due to multiresistant strains of Plasmodium falciparum. Moreover, the coexistence of HMS and severe chronic HBV or HCV infection may further aggravate the course of the haemolytic disorder, because of the occurrence of spur cell anaemia.
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PMID:Spur cell anaemia and acute haemolysis in patients with hyperreactive malarious splenomegaly. Experience in an isolated Yanomamo population of Venezuela. 1111 87

Background/Aims: In patients with chronic liver disease, heterogeneous enhancement of liver parenchyma is often noted on computed tomography during arterial portography (CTAP). We investigated the factors contributing to the heterogeneous enhancement and its relationship with postoperative histopathological findings. Methodology: Eighty-seven patients who had undergone a right lobectomy for liver tumor after CTAP were evaluated. The heterogeneity of hepatic parenchymal enhancement on CTAP was assessed quantitatively using standard deviation of mean CT numbers for five ROIs (S.D.) set in the right hepatic lobe, and comparatively evaluated among three histological groups (liver cirrhosis (LC, n=41), chronic hepatitis (CH, n=33), and normal liver (Normal, n=13)). Severity of fibrosis and degree of splenomegaly (Sp) were taken up as factors contributory to the heterogeneity, and were assessed for correlation with the S.D. Results: The range (mean) of S.D. was LC, 3.07-17.64 (9.10); CH, 1.83-11.12 (6.77); and Normal, 2.06-8.89 (5.64) (Scheffe's F-test: LC vs CH, P<0.0005; LC vs Normal, P<0.0002). The higher fibrosis group exhibited significantly greater S.D. values as compared with the lower fibrosis group (Scheffe's F-test: P<0.00003). Coefficient of correlation between the S.D. and the Sp was 0.295 (P<0.005). Conclusion: There was a fair possibility of LC in patients with heterogeneous enhancement of liver parenchyma on CTAP. The severity of liver fibrosis and the degree of splenomegaly were considered to be factors contributing to the heterogeneous enhancement.
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PMID:Heterogeneity of hepatic parenchymal enhancement on computed tomography during arterial portography: quantitative analysis of correlation with severity of hepatic fibrosis. 1134 53

The hyperdynamic circulation begins in the portal venous bed as a consequence of portal hypertension due to the increased resistance to flow from altered hepatic vascular morphology of chronic liver disease. Dilatation of the portal vein is associated with increased blood flow, as well as the opening up or formation of veno-venous shunts and splenomegaly. At the same time, portal hypertension leads to subclinical sodium retention resulting in expansion of all body fluid compartments, including the systemic and central blood volumes. This blood volume expansion is associated with vasorelaxation, as manifested by suppression of the renin--angiotensin--aldosterone system, initially only when the patient is in the supine position. Acute volume depletion in such patients results in normalisation of the hyperdynamic circulation, whilst acute volume expansion results in exaggerated natriuresis. As liver disease progresses and liver function deteriorates, the systemic hyperdynamic circulation becomes more manifest with activation of the renin--angiotensin--aldosterone system. The presence of vasodilatation in the presence of highly elevated levels of circulating vasoconstrictors may be explained by vascular hyporesponsiveness due to increased levels of vasodilators such as nitric oxide, as well as the development of an autonomic neuropathy. However, vasodilatation is not generalised, but confined to certain vascular beds, such as the splanchnic and pulmonary beds. Even here, the status may change with the natural history of the disease, since even portal blood flow may decrease and become reversed with advanced disease. The failure of these changes to reverse following liver transplantation may be due to remodelling and angiogenesis.
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PMID:The hyperdynamic circulation in cirrhosis: an overview. 1151 77

Hepatitis C virus (HCV) is considered the most common etiology of chronic liver disease (CLD) in Egypt, where prevalence of antibodies to HCV (anti-HCV) is approximately 10-fold greater than in the United States and Europe. Reported are results that show the role of HCV in both overt and occult CLD, the risk factors for CLD and for HCV infection, and the relative importance of chronic HCV, hepatitis B, or both in causing hepatic morbidity. Case patients included 237 new outpatients at the National Liver Institute. Controls comprised 212 sex- and age-matched neighbors without liver disease. Case patients were more likely than controls to report a history of blood transfusions, schistosomiasis, or parenteral therapy for schistosomiasis; to have anti-HCV, HCV RNA, hepatitis B surface antigen, and serum alanine aminotransferase (ALT) elevations; and to have abdominal ultrasound findings of cirrhosis, portal hypertension, and splenomegaly. Anti-HCV-positive case patients were more likely than anti-HCV-negative patients to be male, older, and farmers: to have received a blood transfusion or parenteral therapy for schistosomiasis; to have ALT elevations; and to have ultrasound findings of cirrhosis, portal hypertension, and spleen enlargement. Anti-HCV-positive controls were more likely than anti-HCV-negative controls to have received parenteral therapy for schistosomiasis. These data support the belief that HCV is the predominant cause of CLD in Egypt and suggest there is a large underlying reservoir of HCV-caused liver disease.
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PMID:Role of hepatitis C infection in chronic liver disease in Egypt. 1245

A total of eighty-one consecutive cases of Kala-azar admitted in all four medicine units of Mymensingh Medical College Hospital during the period from January 2002 to mid August 2002 were included in this study. The number of the patients clearly indicates that the burden of Kala-azar in this region is significant and expanding, which constituted 1.90% of total admission in all 4 medicine units during this period. Majority of the patients were of 20-29 years of age. Male to female ratio was 1.38:1. Maximum number of the patients were of poor socio-economic group with history of housing made up of mud and having close proximity with cattle house. Fever and splenomegaly (100%) were the predominant features. Hepatomegaly was found in 91.36% of the cases. Other clinical manifestations were weight loss (79.01%), normal or increased appetite (65.43%), generalized weakness (72.84%), pallor (69.13%), cough (25.92%), jaundice (17.28%), abdominal Pain (12.34%), hyperpigmentation (9.88%), ascites (4.94%) and bleeding manifestations (4.94%). Notable concomitant illnesses were urinary tract infection (7.40%), pulmonary tuberculosis (3.70%), malaria (1.23%), scabies (4.94%), heart failure (3.70%) and chronic liver disease (2.47%). Due to wide diversity of clinical presentations, clinical features of kala-azar should be evaluated in details which will pave the hidden cases into light.
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PMID:Clinical profile of Kala-azar in adults: as seen in Mymensingh Medical College Hospital, Mymensingh, Bangladesh. 1271 42

Pulmonary hypertension associated with increased pulmonary vascular resistance occurring in the setting of portal hypertension, referred to as "portopulmonary hypertension", is a complication of chronic liver disease, and occurs in 2% to 3% of patients with portal hypertension. Portal hypertension is a relatively common finding in patients with chronic myeloproliferative disorder (CMPD). Pulmonary hypertension is also an occasional finding in CMPD patients. Latent myeloproliferative disorder, on the other hand does not fulfill the diagnostic criteria of classical CMPD and is characterized by younger age of onset, slow disease progression, a high risk of thrombosis, platelet dysfunction, and normal or increased platelet count in spite of the presence of splenomegaly. We report findings in a 50-year-old woman with portal hypertension for which there were three major etiological findings-increased splenic blood flow, infiltration of hematopoietic cells in the liver, and thrombosis in the portal or hepatic vein-over a 25-year clinical course, during which there was also reversible stenosis of the portal vein. Twenty-three years after her first admission, her condition was diagnosed as latent myeloproliferative disorder, and she developed pulmonary hypertension. Her clinical history and data indicated that the portopulmonary hypertension was due to the latent myeloproliferative disorder.
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PMID:A 25-year clinical history of portopulmonary hypertension associated with latent myeloproliferative disorder. 1276 93

HCC is the most frequent primary malignancy of the liver and one of the most common cancers in the world. HCC is substantially a complication of liver cirrhosis, and because HBV and HCV are the predominant causes of chronic liver disease and cirrhosis worldwide, they have a propensity to lead to HCC. Common sites of HCC metastases include the lung, lymph nodes, and portal vein. Bony metastases are rare, and when they do occur the disease is usually far advanced and is associated with clinical manifestations of abdominal pain, weight loss, jaundice, hepato-splenomegaly, ascities, deranged LFTs, and elevated AFP. We report here a patient with asymptomatic advanced HCC, normal LFTs, and normal AFP values presenting with spinal cord compression.
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PMID:Asymptomatic advanced hepatocellular carcinoma presenting with spinal cord compression. 1574 89

In this study we retrospectively assessed the prevalence of impaired liver function in all 49 patients suffering from Mediterranean Spotted Fever (MSF) consecutively admitted to our department over the last four years. The main parameters of liver function and ultrasound of upper abdomen were performed at entry and at the end of treatment. At admission mean values of transaminases were above the normal limits and significantly higher when compared to mean serum levels at recovery. 55% and 51% of patients had serum values of GOT and GPT, respectively, above the normal limits versus 1% and 2% at the end of treatment. Mean serum values of alkaline phosphatase (AP) were within the normal limits at entry in hospital, but 22 of them had serum values above the normal limits. The same proportion was seen for gamma glutamiltranspeptidase values. Eighteen patients (36.7%) had both transaminases and AP above the normal limits. There were no significant differences among serum values of albumin, bilirubin and gamma globulin before and after therapy. Platelet count, on the contrary, was significantly reduced at admission (p < 0.0001). At ultrasound half of the patients showed hepatomegaly with a hepatitis-like pattern and 39% of patients had splenomegaly. In conclusion, this study confirms previous data from the literature showing a high frequency of liver impairment during the course of MSF, which is usually mild-moderate. In a few cases, however, the increase of transaminases could be serious and the recovery delayed, but never, in our experience, has there been progression toward chronic liver disease.
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PMID:[Abnormal liver function in Mediterranean spotted fever]. 1759 97

Most cases of portal hypertension follow chronic liver disease (cirrhosis) while non cirrhotic causes are occasionally seen. A case of portal hypertension secondary to non-cirrhotic portal vein thrombosis is reported. The patient was managed at the Aminu Kano Teaching Hospital Kano, Nigeria in the year 2006. She presented with recurrent massive upper gastrointestinal bleeding and was resuscitated, followed by clinical, radiological and endoscopic evaluation. She had massive splenomegaly and grade four oesophageal varices with evidence of recent bleed. There were, however, no other stigmata of chronic liver disease or portal hypertension. Abdominal computed tomography scan confirmed portal vein thrombosis. Splenectomy and ligation of short gastric veins was performed and the patient has not had any repeat episode of haematemesis or malaena six months post surgery.
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PMID:Non-cirrhotic portal vein thrombosis, an uncommon cause of portal hypertension - a case report. 1816 51

Patent ductus venosus (PDV) is a very rare congenital vascular anomaly of the liver. We report a 17-year-old male patient presenting with elevated liver enzymes and multiple liver masses. Physical examination was normal except for pectus excavatum deformity. Ultrasonography revealed multiple tumor-like lesions of the liver and a dilated portal vein with mild splenomegaly. Liver biopsy obtained from liver parenchyma revealed minimal hepatocellular damage. PDV was diagnosed by CT (computed tomography) portography. The radiologic findings of chronic liver disease and multiple liver masses were attributed to presence of PDV. Although it is very rare, PDV might be a diagnostic possibility in patients with mass lesions in the liver.
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PMID:A case of patent ductus venosus complicated with tumor-like lesions of the liver. 1827 18


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