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)

The aetiology of idiopathic portal hypertension or hepatoportal sclerosis is unknown. In view of the indirect evidence for underlying immunologic abnormalities 14 patients (all middle-aged females) were studied. Various auto-antibodies were demonstrated in seven patients and high levels of serum immunoglobulins, either IgG, IgM or IgA were present in ten patients. T cell responsiveness to stimulation with either autologous or allogeneic non-T cells was examined in nine of 14 idiopathic portal hypertension patients and compared with responsiveness in patients with posthepatitic cirrhosis and splenomegaly, and healthy controls. Patients with cirrhosis had levels of T cell responsiveness which were not significantly different from those in healthy controls in both autologous and allogeneic mixed lymphocyte reactions. A distinctly reduced autologous mixed lymphocyte reaction was observed in all idiopathic portal hypertension patients. These data indicate that, like many other autoimmune diseases, abnormal serological features and impaired autoreactive T cell responsiveness exist in patients with idiopathic portal hypertension.
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PMID:A defective autologous mixed lymphocyte reaction in patients with idiopathic portal hypertension. 153 30

We previously reported ultrasonographic and serologic abnormalities in 102 patients infected with Schistosoma japonicum in Leyte, The Philippines. These patients were subsequently treated with praziquantel (3 x 20 mg/kg), and changes in ultrasonographic images and the serum levels of liver function markers in 52 patients were followed up every three months for a period of 17 months. Improvement in the thickening of the portal vein wall and the intensity of echogenic bands was detected six months after treatment with praziquantel. The level of splenomegaly was also reduced in 42 patients who originally did not show the production of collateral vessels. A significant decrease in the serum total bile acid (TBA) level was detected in all patients six months after treatment with praziquantel. However, significant ultrasonographic changes could not be detected in the patients classified as type 3, with severe hepatic fibrosis caused by the long-term infection. These results clearly show that ultrasonographic examination, along with data on the serum TBA level, provides a sensitive tool to monitor the severity of hepatic fibrosis and portal hypertension caused by S. japonicum infection, as well as the improvement resulting from praziquantel treatment.
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PMID:Improvement of ultrasonographic and serologic changes in Schistosoma japonicum-infected patients after treatment with praziquantel. 153 91

Nodular regenerative hyperplasia (NRH) is a condition characterized by hepatocytic nodules distributed throughout the liver without perinodular fibrosis. The etiology is unknown, but it has been reported in patients with a variety of diseases, including autoimmune diseases and primary biliary cirrhosis (PBC). In this study, the liver biopsies of 64 patients with PBC were reviewed. Thirty-five biopsies in histological stages I or II belonging to 30 patients were suitable for study. NRH was found in 43% of biopsies (47% of patients). In 80% of these biopsies, nodular transformation was focal, while it was diffuse in the remaining 20%. Splenomegaly was more common in patients with NRH. Laparoscopy displayed evidence of portal hypertension in two of these patients and endoscopy showed esophageal varices in one patient. Serum levels of gamma-glutamyl transpeptidase were also higher in these patients. Lesions in small intrahepatic vessels, florid bile duct lesions, and portal granulomas were found more frequently in biopsies with NRH. We conclude that nodular hyperplastic changes are very common in early histological stages of PBC, and consequently, this disease should be considered in the etiology of NRH. These changes may contribute to early development of portal hypertension in these patients.
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PMID:Nodular regenerative hyperplasia of the liver in early histological stages of primary biliary cirrhosis. 155 37

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon entity of unknown origin and pathogenesis. We report here a case of NRH of the liver which was associated with portal vein thrombosis and adrenal hyperplasia. A 48-year-old man who was admitted for further examination of portal hypertension and splenomegaly, died of acute myocardial infarction. Autopsy revealed an enlarged heart with occluded coronary arteries and fresh multiple necrosis in the myocardium. The spleen and the liver were enlarged. In the liver diffusely distributed nodules of regenerative hyperplasia were noted. Organized thrombi of the portal vein and adenomatous hyperplasia of the left adrenal gland were also noted. Antinuclear antibody was positive in the serum.
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PMID:Nodular regenerative hyperplasia of the liver with portal vein thrombosis and hyperplasia of the adrenal gland. 155 40

Splenomegaly is a common clinical disorder in the Kingdom of Saudi Arabia. Its aetiology is variable and includes portal hypertension due to schistosomal periportal hepatic fibrosis, haemoglobinopathies and lymphoproliferative disorders. At King Fahd Hospital of the University, Al-Khobar in the Eastern Province of the Kingdom, splenectomy is performed frequently for various reasons on patients drawn from all the Provinces. This is a report of 104 patients (age range eight months to 65 years) who underwent splenectomy between 1982 and 1987. We reviewed the indications, operative findings, outcome, and complications of the procedure. Patients with hypersplenism formed the largest group (50%) subjected to splenectomy followed by those suffering from the haemoglobinopathies. Chest infection as the most common complication and it mainly affected patients with portal hypertension. Post-operative septicaemia occurred in four cases within six months from the date of splenectomy.
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PMID:Splenectomy for hematological disorders in the Eastern Province of Saudi Arabia. 157 81

We report the case of a patient with renal insufficiency who was admitted for the evaluation of splenomegaly. He had received a kidney allograft 6 1/2 years ago. Treatment with azathioprine and prednisolone for immunosuppression had been discontinued 1 year before admission. The underlying cause of the splenomegaly appeared to be an idiopathic portal hypertension. Until now, this disease has been described in only 13 kidney transplant patients receiving long-term immunosuppressive therapy with azathioprine. For the first time we demonstrate that azathioprine can cause this chronic liver disease even if the drug has been withdrawn some time before. Therefore, the indication for azathioprine must be considered very carefully.
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PMID:Idiopathic portal hypertension in a renal transplant patient after long-term azathioprine therapy. 160 Mar 41

Haemangioma is the commonest non-malignant tumour of the spleen. Cytopenia and coagulopathy, secondary to cell trapping and coagulation factors consumption inside the haemangioma, respectively. May occasionally occur as accompanying phenomena. A woman is presented who had splenic haemangioma associated with leucopenia and thrombocytopenia along with decrease fibrinogen and prolonged prothrombin time as well as low complement rates. She had massive splenomegaly with portal hypertension, and the partial oxygen pressure values in mesenteric and portal blood were concurrent with the presence of arteriovenous shunts. The leucocyte and platelet count, the serum complement, the fibrinogen rate and the prothrombin time recovered after splenectomy. The cavernous characteristics of the spleen, containing multiple thrombi, were confirmed upon scanning electron microscope examination. The pathophysiology of the cytopenias and clotting factors consumption is discussed, stress being laid on the ultrastructural findings. A review is presented of the clinical aspects, differential diagnosis and treatment.
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PMID:[Cavernous hemangioma of the spleen with a localized intravascular coagulation syndrome (Kasabach-Merritt syndrome). Ultrastructural study]. 162 Nov 86

A 51-year-old man had for 5 years been known to have erythropoietic protoporphyria. GPT levels were raised up to 40 U/l, gamma-GT up to 120 U/l. After lengthy exposure to sun radiation an erythema with blisters, abdominal discomfort and jaundice developed (total bilirubin 7.3 mg/dl) and biliary liver cirrhosis with portal hypertension and splenomegaly were diagnosed. Because the acute hepatobiliary complications were not improved by conservative treatment (daily 750 mg ursodeoxycholic acid and 12 g colestyramine), an orthotopic liver transplantation was performed without complication. The excised liver showed small nodular parenchymal transformation and contained reddish brown protoporphyrin pigment in the hepatocellular cytoplasm, the Kupffer cells, the canaliculi and in some biliary ducts. Bilirubin and transaminase levels in blood became normal after the transplantation, as did the urinary excretion of coproporphyrin. However, isomer I was still dominant. The protoporphyrin level in erythrocytes and plasma remained elevated. After a symptom-free interval of one year biochemical and histological tests demonstrated protoporphyrin-induced damage in the transplanted liver.
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PMID:[Liver transplantation in erythrohepatic protoporphyria]. 162 35

The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myeloid metaplasia (AMM). We searched the literature (Medline, 1970-1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or transfusion requirements in patients with AMM. We employed formal decision analysis to determine the relative value of medical and surgical treatment of advanced AMM. Results of data synthesis showed that splenectomy in AMM is associated with an operative mortality of 13.4% (95% confidence intervals (CI): 9.5-17.2%), an early morbidity of 45.3% (CI: 39.6-51.1%), and a late morbidity of 16.3% (CI: 9.9-22.5%). Almost all patients with portal hypertension and painful splenomegaly, but only about half of those with thrombopenia and anemia were reported to have experienced relief in their symptoms or signs after splenectomy. We found no evidence that splenectomy affects survival in AMM. We concluded that splenectomy in advanced AMM is a palliative procedure that carries a substantial risk. It may be considered for symptomatic patients after they have been informed about the operative mortality, morbidity, and chances of palliation. Decisions about treatment of advanced AMM should be guided predominantly by the patient's preferences.
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PMID:The choice between splenectomy and medical treatment in patients with advanced agnogenic myeloid metaplasia. 168

Liver cirrhosis was induced in rats by administering thioacetamide (TAA), and portal hypertension was maintained for at most 35 weeks to study the influence of portal hypertension on the weight of spleen and splenic tissues. TAA was intraperitoneally injected at dose of 200 mg/kg three times a week. The portal pressure was elevated and the weight of the spleen increased with the progression of hepatic fibrosis due to TAA. Liver cirrhosis was obviously observed after TAA was administered 60 times. The maximum ratio of the weight of the spleen to body weight in the group administered TAA was higher by 4.08 times than that in the control group. In all 49 rats used in the experiment there was a high correlation (r = 0.930, p less than 0.01) between the portal pressure and the ratio of spleen to body weight. Histological observation revealed that the red pulp tended to be enlarged, while the white pulp tended to be reduced, with increases in portal pressure and weight of the spleen. It also histologically revealed an increase in the splenic sinus-like structure, narrowing of the splenic cords, fibrosis due to reticular cell proliferation in the red pulp and fibrosis around the central artery in the white pulp. These histological changes in rats were similar to the histological findings of splenomegaly accompanying portal hypertension in humans. This suggests that the animal model prepared in the present experiment is useful as a model of splenomegaly due to portal hypertension.
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PMID:[Induction of experimental splenomegaly with portal hypertension in rats with liver cirrhosis]. 175 82


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