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)

Twenty five cases of Burkitt's lymphoma in Thai children were diagnosed at the Department of Pediatrics, Siriraj Hospital during the period of 13 years (January 1969 to April 1982). Males were more affected than females with the ratio of 1.7:1. The age ranged from 2 to 11 years with the median age of 4-5 years. The most common clinical manifestations were abdominal mass associated with nausea vomiting, abdominal pain, anorexia, weight loss and generalized lymphadenopathy which occurred in 50-60% of cases. Additional symptoms and signs included anemia, hepatosplenomegaly, edema and pleural effusion. Jaw tumor was found in only 37.5% of the patients. Definite diagnosis depended on the characteristic starry sky appearance of the lymph node biopsy or section of abdominal mass. In advance cases, the tumor cells could be discovered in bone marrow aspiration, ascitic fluid pleural fluid and cerebrospinal fluid. The typical blast cells were detected in the peripheral blood in 4 cases. Antibody to Epstein-Barr virus could be detected in almost all cases with high titers in some cases. Most patients responded very well to local irradiation and chemotherapy with prednisolone plus cyclophosphamide and vincristine or methotrexate. However, relapse occurred rapidly and 80% of the patients died within 3 months after diagnosis with the median survival of only 1 month. Five cases expired early before any specific treatment. The main causes of death were disease, sepsis, excessive bleeding and hyperkalemia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Burkitt's lymphoma in Thai children: an analysis of 25 cases. 631 66

Lysinuric protein intolerance is an autosomal recessive disease, due to a defect in intestinal, renal and hepatic dibasic amino acid transport. Two new cases in the same family are reported. The disease appears progressively during the first months of life with failure to thrive, anorexia, vomiting, diarrhea, hepatosplenomegaly, muscular weakness, osteoporosis, anemia, leukothrombocytopenia, hyperammonemia and orotic aciduria after a high-protein intake. Hyperdibasicamino-aciduria was associated with subnormal plasma concentrations of the same aminoacids. Oral l-arginine, l-ornithine, l-lysine, and lysyl-glycine loads confirmed the diagnosis. The supplementation of the diet with l-citrulline resulted in normal levels of blood ammonia. However, hepatosplenomegaly, muscular weakness, osteoporosis remained unchanged and growth was not improved. These may be due to lysine deficiency.
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PMID:[Lysinuric protein intolerance: a severe hyperammonemia secondary to l-arginine deficiency (author's transl)]]. 680 Mar 34

Acute clinical malaria caused by Plasmodium inui was diagnosed in an adult female cynomolgus monkey (Macaca fascicularis) 4 years after importation into the United States. Stress and immunosuppression associated with experimentation completed 2 weeks earlier may have contributed to the development of severe clinical disease. Clinical findings included severe regenerative anemia, hepatosplenomegaly, weakness, lethargy, weight loss, and anorexia. The infection was treated and successfully eliminated with chloroquine hydrochloride administered intramuscularly at a dose of 5 mg/kg base given at 0, 6, 24, 48, and 72 hours. Treatment also included a blood transfusion and intensive supportive care.
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PMID:Acute clinical malaria (Plasmodium inui) in a cynomolgus monkey (Macaca fascicularis). 683 80

In this paper we report a case of malignant lymphoma with neoplastic angioendotheliosis in the brain. A 44-year-old man with transient episodes of deafness, hypersomnia, and anorexia over a 5-month period acutely deteriorated. He presented with low grade fever, dementia, frontal lobe signs, general hyperreflexia, muscle weakness of the extremities, and ataxia. He did not have hepatosplenomegaly, lymph node swelling, or skin eruptions. On the 15th day after admission to the hospital he developed convulsions and died. Post-mortem examination revealed multiple infarcts in the central nervous system, especially in the bilateral cerebral white matter and basal ganglia, where mononuclear tumor cells were widespread within the lumens of small blood vessels, accompanied by lymphocytic infiltration and degenerative and occulsive changes of the vessels. Intravascularly in many visceral organs and in the adrenal glands, both intra- and extravascularly, proliferation of tumor cells was observed. Furthermore, a small nest of malignant lymphoma of diffuse mixed cell type was found in a para-aortic lymph node, and the lymphoma cells were identical to tumor cells observed in the brain and other organs.
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PMID:Malignant lymphoma presenting with neoplastic angioendotheliosis of the central nervous system. 685 Dec 98

Clinical analysis of 293 cases of cirrhosis from two moderate sized hospitals in the city of Dacca has been presented. Maximum number of cases were in the age group over 40 with 150 (51.2%) males and 19 (5.8%) females. Significant past history included viral hepatitis (21.5%), kala-azar (11.6%) and malaria (10.24%). History of alcoholism was present only in 16 (5.5%) cases. Weakness (84.3%), weight loss (72%) and anorexia (39.3%) constituted the most common symptoms. Ascites (45%), haematemesis (11.6%) and melaena (28.7%) were the next common symptoms. Hepatosplenomegaly was found in about one-third of the cases. Testicular atrophy was recorded in 41.63% cases whereas gynaecomastia was relatively less common (5.5%). Scanty body hair and white nails were present in almost equal number of cases (14.7% and 18%). The cases presented here are those with overt manifestation. Nevertheless, the clinical features are not materially different from those reported by other authors. In the absence of alcoholism, viral hepatitis is presumably the most important aetiological factor in our cases and the clinical features compare favourably with non-alcoholic cirrhosis of the western writers. Cryptogenic cirrhosis has been considered to be most common type constituting 43.7% of our cases.
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PMID:Cirrhosis of liver. 734 4

Angioimmunoblastic lymphadenopathy is a rare clinical entity, first described in 1974, characterized by asthenia, anorexia, fever, sweating, generalized lymph node enlargement, hepatosplenomegaly, rash, hypergammaglobulinemia, and often Coomb's positive hemolytic anemia. Main histopathologic findings are lymphoplasmocytic and immunoblastic proliferations, increased vascular neshwork and interstitial granular PAS positive material deposits. A lymph node excised from a woman with angioimmunoblastic lymphadenopathy was examined under electron microscopy. Results of ultrastructural study are compared to the typical histologic pattern observed under light microscopy. Analysis of the cellularity and the significance of fibrous collagen found in the interstitial PAS positive material are commented on.
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PMID:[Angioimmunoblastic lymphadenopathy. Case report with ultrastructural study (author's transl)]. 741 34

A total of 592 children with clinical diagnosis of typhoid fever admitted to the Dr B. C. Roy Memorial Hospital for Children, Calcutta, India during the period between February 1990 and January 1992, were screened for Salmonella typhi by blood culture. S. typhi was isolated from 221 (37.3%) cases. The majority of the strains (92.3%) showed multi-drug resistant (MDR). They were resistant to chloramphenicol, ampicillin, tetracycline and trimethoprim-sulphamethoxazole. However, all the strains were uniformly (100%) susceptible to gentamicin, amikacin, furazolidone, norfloxacin and ciprofloxacin. Minimum inhibitory concentration of the antimicrobial agents against the resistant strains of S. typhi ranged between 200 and > 1600 micrograms/ml. Phage type 0 was most frequently encountered. The rate of isolation of S. typhi was more or less the same in all the pediatric age groups. The majority of the cases came from lower socio-economic classes with poor personal hygiene. Fever was the main presenting feature in all the cases. Other associated features of the MDR typhoid fever cases, who were uncomplicated during admission, were headache (36.0%), chill and rigor (23.2%), diarrhea (37.2%), anorexia (26.2%), vomiting (23.8%), cough (18.0%) and abdominal pain (19.8%). Hepatosplenomegaly was present in 42.4% cases. However, complications were less frequently encountered among the MDR typhoid fever cases who were uncomplicated during admission and treated as in-patients. Fourteen bacteriologically-confirmed MDR typhoid fever cases had jaundice and another 18 cases had an abnormal state of consciousness during admission. Four (2.0%) bacteriologically-confirmed MDR typhoid fever patients died during the period of observation.
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PMID:Multi-drug resistant typhoid fever in hospitalised children. Clinical, bacteriological and epidemiological profiles. 795 89

We described a case of adult T cell leukemia (ATL) not associated with human T-cell leukemia virus type I (HTLV-I), a clinical entity that was first reported by Shimoyama et al. A 79-year-old male was admitted with anorexia and fever in October, 1989. Physical examination revealed marked hepatosplenomegaly and superficial lymphadenopathies. Hematological examination revealed marked leukocytosis (136,300/microliters) with abnormal lymphoid cells showing highly lobulated nuclei. Hypercalcemia (11.2 mg/dl) and elevation of lactic dehydrogenase were also recognized. Surface marker analysis showed that the abnormal lymphoid cells in the peripheral blood were positive for CD2 and CD4 but negative for CD8. Southern blot analysis of the DNA from peripheral blood leukemic cells revealed monoclonal rearrangement of T-cell receptor beta-chain gene. The clinical and hematological findings of the patient were compatible with those of acute type ATL, however, serum anti-HTLV-I antibody was negative and HTLV-I proviral DNA was not detected in the leukemic cells by Southern blot analysis. Furthermore, the polymerase chain reaction showed no integration of the HTLV-I proviral DNA in the leukemic cells.
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PMID:[HTLV-I negative adult T cell leukemia; a case report of acute type]. 829 28

Among 276 paediatric cases of brucellosis seen over a 7-year period, 16 patients (5.8%) with pancytopenia were identified. The most frequent presentations were fever, malaise, anorexia, weight loss, arthralgia, and hepatosplenomegaly. Fourteen patients (87.5%) had positive blood and/or bone marrow cultures for Brucella melitensis. Bone marrow aspiration specimens showed hypercellularity in 14 patients and normocellularity in 2. Histiocytes, eosinophils and plasma cells were increased in every marrow aspirate, and haemophagocytosis was observed in 14 patients (87.5%). Non-caseating granulomas were present in the bone marrow biopsy of 11 patients (68.8%). The pancytopenia was transient, and resolved on treatment of the Brucella infection.
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PMID:Pancytopenia in children with brucellosis: clinical manifestations and bone marrow findings. 836 1

A 62-year-old man with adult T cell leukemia (ATL) presented with complaints of anorexia and abdominal fullness. Evaluation revealed ascites and pleural effusion, but no lymph node swelling, hepatosplenomegaly, or skin involvement. The diagnosis of ATL was made by the detection of specific surface markers for T lymphocytes in pleural effusion and ascitic fluid, and by determination of human T cell leukemia virus type I (HTLV-I) proviral DNA integration in mononuclear cells of pleural effusion. This case was considered a rare type of ATL with infiltration of the pleura and peritoneum.
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PMID:Adult T cell leukemia clinically manifested with ascites and pleural effusion. 856 29


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