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 39-year-old male with bleeding esophageal varices due to portal hypertension was observed. The patient had taken an arsenical preparation during a period of 12 yr because of psoriasis and subsequently developed keratotic changes of the palms and soles of his feet and an epithelioma of the scrotum. Physical examination was unremarkable except for splenomegaly and skin lesions. Liver function tests were normal; a needle biopsy of the liver (right lobe) showed nonspecific changes. Combined hepatic and umbilicoportal catheterization revealed, on splenography and portography, huge esophageal varices and patent portal vein; dilation, distortion, and cut-off of many intrahepatic portal branches were found. A marked gradient existed between the free portal venous pressure (25 mm Hg) and the wedged hepatic venous pressure (9.5 mm Hg). Hepatic blood flow, portal PO2, cardiac output, cardiac index, and blOOD volume were within normal range. Arteriographies did not reveal arteriovenous shunts in the splanchnic or splenic vessels. A splenorenal shunt were performed and a wedged biopsy of the liver (left lobe) revealed nonspecific changes. Three years later the patient had not experienced any episode of hemorrhage or hepatic encephalopathy but developed an epithelioma of the tongue. No known cause could be incriminated in the pathogenesis of the portal hypertension. However, there was unequivocal chronic arsenic intoxication. Toxic hepatitis, cirrhosis, noncirrhotic portal hypertension, and hemangiosarcoma of the liver have been reported with the intake of arsenicals. Thus, it is suggested that in this patient, presinusoidal portal hypertension was secondary to chronic arsenical intake associated with marked intrahepatic vascular changes seen on portography.
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PMID:Noncirrhotic presinusoidal portal hypertension associated with chronic arsenical intoxication. 112 3

Misaka strain was isolated as the causative agent from a patient with spotted fever group rickettsiosis in Japan by using nude mice on Awaji Island, Hyogo in September 1988. The nude mice infected with the isolate showed weakness and splenomegaly and died in two or three weeks after the infection. The cyclophosphamide-treated mice infected with the isolate died between four and seven days after the infection. The infected normal mice recovered and acquired immunity. The infected adult male guinea pigs were feverish and showed swelling and redness of the scrotum between two and eight days after the infection, and recovered. The Misaka strain was propagated well in Vero cells in tissue culture. The rickettsial particles were seen as diplobacillary and diplococcal forms growing predominantly in the cytoplasm and occasionally in the nucleus of infected cells. The serological characteristics of the Misaka strain were analyzed by the cross-immunofluorescent antibody method. The Misaka strain, the Katayama strain first isolated in Tokushima in 1987, and the representative strains of spotted fever group rickettsiae in the world; R. rickettsii Smith, R. sibirica 246, R. conorii Moroccan, R. akari MK (Kaplan), R. australis Phillips, R. montana Tick and Thai TT-118 strains were used as antigens. And immune mouse serum samples against the Misaka, Katayama, 246, Phillips and TT-118 strains were used as antisera. The result revealed that these strains showed cross-reaction and share a common antigen of spotted fever group rickettsiae. Furthermore, it became obvious that the Misaka strain and the Katayama strain have the same serotype-specific antigen different from the strains of other spotted fever group rickettsiae using Anti-Katayama monoclonal antibodies.
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PMID:[The causative agent from a patient with spotted fever group rickettsiosis in Japan on Awaji Island, Hyogo]. 220 68

Rickesttsial diseases are a group of diseases caused by obligate intracellular gram negative bacilli and transmitted to man by arthropod vectors (except Q fever). It is increasingly realised that rickesttsial diseases are underdiagnosed. It is now well documented that rickettsial disease is prevalent all over India, in pockets. The hallmark of rickettsial infection is microvasculitis, causing microinfarcts in various organs. Usually the patients present with classical triad of Fever, Headache & Rash. Apart from this, pain in legs, oedema, Gastro-intestinal symptoms, hepato-splenomegaly, anaemia, necrotic rash, gangrene of digits, toes, earlobes, scrotum, painless eschar and lymphadenopathy are other manifestations. Complications include encephalitis, ARDS, pneumonia, Myocarditis, Renal failure and Vascular collapse. Endocarditis is seen in Q fever. Gold standard test for confirmation of diagnosis is I.F.A. Weil felix test is widely available but unacceptable for accurate diagnosis. Weil Felix test can be used in developing countries where other tests are not available. ELISA Should be preferred and is now available in India. The drug of choice for all age group is doxycycline. Rickettsia are potentially dangerous pathogens and unfortunately, specific serological tests are available in only a few specialized laboratories. Hence, it is imperative to have a high index of suspicion for Rickettsial diseases and make a clinical diagnosis based on prudent history taking and appropriate physical findings. A therapeutic trial with a specific agent in these patients is justified because a delay in initiating treatment may prove fatal. A rapid and favorable response is suggestive of a correct diagnosis.
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PMID:Childhood rickettsiosis. 2096 15

Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by periodic episodes of fever and recurrent polyserositis. It is caused by a dysfunction of pyrin (or marenostrin) as a result of a mutation within the MEFV gene. It occurs mostly in individuals of Mediterranean origin; however, it has also been reported in non-Mediterranean populations. In this report, we describe the first case of FMF in a Korean child. As eight-year-old boy presented recurrent febrile attacks from an unknown cause, an acute scrotum and renal amyloidosis. He also showed splenomegaly, lymphadenopathy, pleural effusion, ascites and elevated acute phase reactants. After MEFV gene analysis, he was diagnosed as FMF combined with amyloidosis.
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PMID:The first case of familial Mediterranean fever associated with renal amyloidosis in Korea. 2231 40

A 19-year-old man had an unexplained fever, dizziness, headache, fatigue, and pain in the scrotum. An FDG PET/CT imaging was acquired to assess fever of unknown origin. The images showed multiple foci of increased FDG activity in the enlarged lymph nodes in the body. In addition, mildly increased activity in the enlarged spleen and lung bases was also noted. The patient was eventually diagnosed with scrub typhus based on positive results of the Weil-Felix agglutination test, eschar in the scrotum, and effective therapy.
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PMID:Imaging of Scrub Typhus by PET/CT. 2625 22