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)

Since 1974 an epidemic of tertian malaria has been spreading around the Adana and Tarsus townships in southern Turkey, with a peak incidence of 115 500 cases in 1977. A further increase is to be expected because the insect vectors have become resistant to insecticides. Since 1975 eleven children and three adults have been treated for P. vivax malaria. They had all stayed in the epidemic area during the transmission season which lasts from July to October. Because of a long primary latent period seven patients only developed first manifestations of the disease six to nine months after leaving Turkey. The classical malarial paroxysms were missing during the first weeks of the primary attack. Several children had a febrile illness over weeks with headache, vomiting, abdominal pain, hepatosplenomegaly, high blood-sedimentation rate and severe haemolytic anaemia, so that appendicitis or septicaemia had been suspected. Tetracyclines and trimethroprimsulphamethoxazole were able to suppress the disease without preventing relapses.
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PMID:[Tertian malaria in children and adults from an epidemic region in southern Turkey (author's transl)]. 36 41

The Budd-Chiari syndrome caused by occlusion of the major hepatic veins, often of unknown etiology, is typically characterized by massive ascites, hepatomegaly and abdominal pain due to intense congestion of the liver. The outcome has almost always been fatal. This report describes an evaluation of side-to-side portacaval shunt in dogs with experimental Budd-Chiari syndrome and in six patients with hepatic vein thrombosis. In the animal studies, side-to-side portacaval shunt was very effective in relieving massive ascites, hepatomegaly, hepatic congestion and portal hypertension produced by ligation of the hepatic veins. Only one of 24 dogs with side-to-side anastomosis reformed ascites, 67% of the animals survived until the study was concluded after one year, and liver biopsies showed reversal of the severe pathologic abnormalities. In contrast, all 20 control dogs subjected to a sham laparotomy, and all 20 dogs that underwent end-to-side portacaval shunt reformed massive ascites and died within six months with continued hepatic congestion and necrosis. All six patients with the Budd-Chiari syndrome due to hepatic vein occlusion had massive ascites (4.4-15.9 l), hepatomegaly, abdominal pain and disturbed liver function. In all six, angiography demonstrated occlusion of the hepatic veins with a patent inferior vena cava (IVC) and a normal IVC pressure, and liver biopsy showed intense centrilobular congestion and necrosis. The most valuable diagnostic study was angiography of the IVC and hepatic veins with pressure measurements. Side-to-side portacaval shunt was performed from four to 14 weeks after the onset of symptoms, and produced dramatic and sustained relief of ascites in five of the six patients during follow-up periods of from eight months to seven years. Liver function returned to normal, hepatosplenomegaly disappeared, none of the survivors developed portal-systemic encephalopathy, and follow-up liver biopsies showed disappearance of congestion and necrosis, but mild to moderate fibrosis. One patient died following an emergency IVC thrombectomy and portacaval shunt, which was undertaken when, during the course of his workup, his condition deteriorated suddenly because the thrombotic process extended from the hepatic veins into the IVC. The everpresent risk of this complication, and the dangers associated with delaying operation were emphasized by this case. It is concluded that side-to-side portacaval shunt, which decompresses the liver by converting the portal vein into an outflow tract, provides effective treatment of the Budd-Chiari syndrome when the occlusive process is confined to the hepatic veins.
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PMID:Treatment of Budd-Chiari syndrome by side-to-side portacaval shunt: experimental and clinical results. 69 34

A patient with immunoblastic lymphadenopathy (IL) had an unusual course of illness, with frequent episodes, over a 20-month period, of chills, fever, abdominal pain, hepatosplenomegaly and weight loss. The episodes were short-lived and many resolved spontaneously. Eventually generalized lymphadenopathy and profound monoclonal IgG gammopathy developed, with atypical mononuclear cells in the peripheral blood and increased numbers of plasmacytoid cells in the bone marrow. Lymph node biopsy revealed the morphologic triad typical of IL: proliferation immunoblasts, proliferation of small blood vessels and the deposit of an amorphous acidophillic material in the vascular walls and the interstitium. Up to October 1976 110 cases had been reported of this disorder, first described 3 years ago, which indicates that IL is not rare. Remissions have occurred spontaneously and after steroid therapy or chemotherapy or both. However, death has been reported in almost 50% of the cases, and the best approach to therapy remains to be determined.
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PMID:Immunoblastic lymphadenopathy: case report and literature review. 86 11

Louse-borne relapsing fever seems to have become endemic in the southern Sudan. The epidemic history of the disease in the Sudan is reviewed. We have studied 363 Sudanese patients involved in an outbreak of louse-borne relapsing fever in Khartoum (Sudan) between January and June 1974. 318 of the 363 patients were new immigrants from the soughern Sudan to Khartoum. The clinical presentation varied. The common clinical fetures of the disease were: fever (94%), headache (85%), hepatosplenomegaly (74%), body and joint pains (66%), abdominal pain and tenderness (63%), jaundice (46%) and epistaxis (40%). Thrombocytopenia was common. Biochemical evidence of hepatocellular and renal damage was present in most patients. The mortality rate was 5-5% with treatment. Post-mortem examination was performed on six cases. The organs predominantly involved were the liver, spleen, brain and lungs. The common causes of death were severe hepatic damage, lobar pneumonia, subarachnoid haemorrhage and splenic rupture.
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PMID:Louse-borne relapsing fever: I. A clinical and laboratory study of 363 cases in the Sudan. 87 Oct 32

A case of Budd-Chiari syndrome in a young woman taking oral contraceptives is described. Her main complaints were abdominal pain and ascites without hepatosplenomegaly and the subsequent development of shock. Diagnosis was established by selective hepatic arteriogram and vena cavagram. She was treated with supportive measures, anticoagulants and neomycin. At the time of this report, the patient is slowly convalescing, taking coumadin, diuretics and Aldactone, as well as supplementary vitamins. Reviewed are 14 cases of Budd-Chiari syndrome occurring while patients were taking oral contraceptives.
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PMID:Budd-chiari syndrome after taking oral contraceptives. A case report and review of 14 reported cases. 87 28

A survey for schistosomiasis in a village in the Gezira area of the Sudan in 1973 showed that there has probably been a great increase in the incidence of S. mansoni infection in the last 25 years. Severe morbidity was uncommon in this sample but overt infection was associated with the symptoms of bloody diarrhoea, tiredness, and possibly abdominal pain, and with a reduced level of haemoglobin. Hepatosplenomegaly was common and schistosomiasis is considered to have contributed to this, although hyperendemic malaria must also have been important in its causation.
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PMID:Infection with Schistosoma mansoni in the Gezire area of the Sudan. 95 63

A 15-year old Black teenager came to a clinic at the University of Alabama's School of Medicine in Tuscaloosa requesting oral contraceptives (OCs). The physical examination indicated that she was in good health and the physician prescribed an OC (1 mg norethindrone and .035 mg ethinyl estradiol). 21 months later she returned complaining of yellow eyes for 3 weeks. The oral mucosa was also jaundiced. She had considerably high levels of bilirubin and alkaline phosphatase. She had no hepatitis virus antibodies. 5 months later she returned for the physical examination required to renew the OC prescription. She did not have jaundice at this time. 10 months later she complained of malaise and muscular pain. Her alkaline phosphatase level was high, but her bilirubin level was normal. She had mild hepatosplenomegaly without focal defects. After reviewing her medical records, the physician diagnosed intrahepatic cholestasis and discontinued her OC prescription. Liver function tests were normal within 3 months. 14 months later, she returned complaining of malaise and reported taking OCs obtained at another clinic 3 months earlier. The physician advised her about the complications of OCs and about other contraceptive methods. The same physician also examined a 32-year-old Black woman who had intermittent epigastric and right-upper quadrant abdominal pain for 2 weeks. Eating worsened the pain, which lasted for up to 15 minutes. She had used an OC for 12 years. Ultrasound revealed a 4.2 cm hypoechoic mass in the left upper lobe of the liver. The physician discontinued the OCs. The tumor regressed over 12 months. Active liver disease is a contraindication to OC use. Women who had cholestatic jaundice while pregnant or have first degree relatives with cholestatic jaundice of pregnancy should not use OCs. Physicians may introduce OCs to closely monitored women with a history of liver disease whose liver function tests are normal. Women with a family history of biliary excretion defects should not use OCs.
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PMID:Hepatobiliary complications of oral contraceptives. 133 97

The case of a 58-years-old patient with AIDS is discussed. He presented a progressive disease with discomfort, abdominal pain, hiporexia, fever and weight loss. At the time of admittance in our hospital he had hepatosplenomegaly. The patient worsened and presented asthenia, fever, oedema, ascites, pulmonary congestion and finally jaundice and died. Autopsy findings were indicative of disseminated histoplasmosis with pseudotumoral appearance of the adrenal glands.
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PMID:[Terminal jaundice in progressive disseminated histoplasmosis associated with AIDS. A report of an autopsy case]. 134 Aug 16

Fifteen endoscopic gastric biopsies (GBx) from 12 patients with small lymphocytic infiltrates morphologically raising a differential of indeterminate lymphocytic infiltrate versus mucosa-associated lymphoid tissue (MALT) lymphoma were analyzed genotypically after frozen-section identification of the abnormal lymphocytic infiltrate. Frozen-section immunoperoxidase immunophenotyping was equivocal in each case. All patients had abdominal pain attributable to superficial gastric ulceration, most often antral, without peripheral lymphadenopathy or hepatosplenomegaly. Rearrangement of the immunoglobulin heavy-chain gene (JH-R, seven patients) or kappa light-chain gene (JK-R, eight patients), was found in eight GBx from eight (seven stage IAE; one stage IBE) of 12 patients, establishing, in conjunction with the histologic features, a diagnosis of low-grade B-cell lymphoma. This diagnosis had not been tenable on multiple prior GBx, ranging from one to five per patient, over intervals of 1 month to 6.5 years (median 4.5 months). The T-cell receptor beta-chain gene retained germline configuration in all cases. Insufficient DNA for molecular studies was extracted from the GBx of two patients, one with JK-R (JH-G) on subsequent GBx and one without further GBx. One patient had two GBx, each demonstrating a single additional band in HindIII digests hybridized with the JH probe. No rearrangements were detected in either the BamHI or the EcoRI digests. Uninvolved tissue from this patient was not available for the exclusion of restriction fragment length polymorphism. Three GBx (two patients) showed germline JH genes (JH-G). One had a partial gastrectomy (histology: MALT lymphoma) in 1981 followed by GBx in 1983 (histologically benign) and in 1990 (JH-G), and negative esophagogastroduodenoscopy (EGD) in 1991 without biopsy. The other patient (two GBx with JH-G) had multiple subsequent abnormal EGD, but no biopsies since December 18, 1990. Adequate DNA for gene rearrangement studies can be extracted from GBx samples weighing as little as 20 mg. The two samples with insufficient DNA weighed 1 and 16 mg, respectively. Practically speaking, the remainder of a frozen block from a single GBx is adequate, thus allowing the screening of multiple endoscopic GBx by sequential frozen sections to determine which one contains the most extensive lymphocytic infiltrate for molecular study. Consistent results are obtained on samples weighing 40 to 60 mg. This method is a suitable alternative to kappa/lambda frozen-section immunoperoxidase immunostaining, which can be uninterpretable on endoscopic biopsies or small biopsies from other sites.
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PMID:Practicality of molecular studies to evaluate small lymphocytic proliferations in endoscopic gastric biopsies. 135 96

From December 1964 to November 1989, 71 children from 3 to 17 years of age with the eventual diagnosis of hepatocellular carcinoma (HCC) presented at the National Taiwan University Hospital (Taipei, Taiwan, Republic of China). Forty-three of them had pathologic proof, whereas 28 were diagnosed on a clinical and laboratory basis. A male predominance (M:F = 3.2:1) was noticed. Most patients presented in a late, advanced stage. Abdominal pain and abdominal mass were the major symptoms and signs, followed by anorexia, fever, and internal bleeding. Hydrocele, purpura, and obstructive jaundice were rare presenting symptoms. Hepatosplenomegaly, superficial venous engorgement, and ascites were the main physical signs. The prognosis for such children with HCC was very poor. Only 10% of the patients survived longer than 1 year after the onset of the initial symptom. Among 49 patients who could be followed, only two had long-term survival of over 5 years. One patient had a small HCC with internal bleeding, whereas the other had a large HCC with abdominal distention. Both received surgical resection, and a resection was repeated for tumor recurrence in the patient with the large mass. The resectability of these 71 patients was low (9.8%). Resectability and nonicterus seemed to be the factors indicating favourable prognosis. Observation indicated that the prognosis for children with symptomatic HCC is grave but surgical resection, whenever possible, should be carried out.
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PMID:Hepatocellular carcinoma in childhood. Clinical manifestations and prognosis. 165 24


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