Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To evaluate the consequences of receiving human immunodeficiency virus type 1 (HIV-1)-seropositive blood, 90 HIV-1-seronegative recipients of HIV-1-seropositive blood (case patients) and 90 HIV-1-seronegative recipients of HIV-1-seronegative blood, matched for age, sex, number of transfusions, diagnosis, and severity of illness (controls), were followed for 12 months after transfusion at Mama Yemo Hospital in Kinshasa, Zaire. Of case patients and controls, 72% were children transfused for anemia caused by malaria. Of the 46 case patients case patients alive 6 months after transfusion and for whom HIV-1 serologic results were obtained, 44 (96%) had seroconverted. Significantly more case patients (47%) than controls (16%) died within 1 year after transfusion (P less than .001). In the first 3 months after transfusion, fatigue, diarrhea, fever, cough, pruritus, pallor, oral candidiasis, polyadenopathy, hepatosplenomegaly, and rhinorrhea were observed more often among seroconverters than controls (P less than .04). Six percent of case patients and no controls had developed clinical AIDS after 12 months of follow-up. These findings underscore the urgent need for appropriate HIV screening facilities in transfusion centers worldwide.
 ...
PMID:Seroconversion rate, mortality, and clinical manifestations associated with the receipt of a human immunodeficiency virus-infected blood transfusion in Kinshasa, Zaire. 186 35

The authors make a review of recent data in the literature and compare them to their own cases between 1983 and 1988. They analyzed eight patients with idiopathic myelofibrosis confirmed with bone marrow biopsy of the posterior iliac wrist with Jamshidi's needle. Most patients were between 55 and 60 years old (5 male and 3 female). Major symptoms were weakness and bleeding (6/8 cases), weight loss and bone distress (4/8 cases). In general, physical signs were splenomegaly and anemia (7/8 cases), hepatomegaly (5/8 cases), and jaundice (2/8 cases). Laboratory features were variable. Most cases were diagnosed in an advanced stage of the disease. Therapy with busulfan, prednisone, oxymetholone and radiotherapy of the spleen was used alone or in combination to relieve compressive abdominal symptoms. This review shows that idiopathic myelofibrosis should be included in the differential diagnosis of patients with hepatosplenomegaly and anemia.
 ...
PMID:[Idiopathic myelofibrosis]. 188 77

Two pregnant women with secondary syphilis underwent amniocentesis and evaluation for fetal syphilis. In both cases, motile spirochetes, typical of Treponema pallidum, were observed during dark-field microscopic examination of the amniotic fluid. The presence of T pallidum was confirmed by antitreponemal monoclonal antibody immunofluorescence assays and by rabbit infectivity tests using the amniotic fluid. In the first case, an infant at 35 weeks' gestation delivered within 24 hours of amniocentesis had hepatosplenomegaly, osteochondritis, and neurosyphilis. In the second case, a fetus at 24 weeks' gestation was hydropic and a fetal blood sample showed anemia, thrombocytopenia, and elevated liver enzymes. Fetal syphilis was confirmed by rabbit infectivity testing using fetal blood obtained by funipuncture. This is the first report of the diagnosis of fetal syphilis by funipuncture and confirmation of the presence of virulent T pallidum in the blood of a human fetus. The mother was treated for secondary syphilis, but the infant had residual signs of congenital infection at birth 14 weeks later. Neonatal serum from the first case and fetal serum from the second case showed specific immunoglobulin M reactivity with the 47-kd antigen of T pallidum by Western blot assays. A new wild-type strain of T pallidum, designated DAL-1, was isolated from the amniotic fluid of the first case and is available for future studies. We conclude that the presence of T pallidum in amniotic fluid or fetal blood indicates fetal-placental infection. Further investigation is necessary to determine the pathogenesis of amniotic fluid infection and its role in the prenatal diagnosis of congenital syphilis.
 ...
PMID:Identification of Treponema pallidum in amniotic fluid and fetal blood from pregnancies complicated by congenital syphilis. 192 18

Twenty-five Chinese patients with human T-cell lymphotropic virus type I (HTLV-I) associated adult T-cell leukaemia/lymphoma (ATLL) were identified in Taiwan. No patients had been outside Taiwan and none were descendants of Japanese heritage. Their ages ranged from 28 to 71 years. There were 17 men and eight women. Main clinical and laboratory features at presentation were lymphadenopathy (16), skin lesions (11), hepatosplenomegaly (11), pulmonary lesions (11), hypercalcaemia (10) and bone marrow infiltration (14). Peripheral blood was characterized by leucocytosis with presence of pleomorphic abnormal lymphocytes but rare anaemia or thrombocytopenia. The clinical subtypes were acute in 15, chronic in three, smouldering in one, and lymphoma type in six. The immunophenotypes of the ATLL cells were characterized by the expression of CD2+, CD4+, CD7-, CD8- and CD25+. The overall prognosis was poor with a median survival of 5 months. The acute form had a significantly shorter survival (2 months) than lymphoma type (13 months). Susceptibility to various infections was common. Pulmonary complications accounted for 73% of the causes of death. The clinicopathologic features of ATLL in Taiwan are indistinguishable from those in HTLV-I endemic areas. The present series adds to the knowledge of the worldwide pattern of the disease.
 ...
PMID:Human T-cell lymphotropic virus type I associated adult T-cell leukaemia/lymphoma in Taiwan Chinese. 195 72

We reported 3 fatal cases of primary Epstein-Barr virus (EBV) infection resembling histiocytic medullary reticulosis (HMR) in young children in Taiwan, where an HMR-like illness has been previously found to be prevalent. The disease ran a fulminant course, manifesting as fever, anemia, jaundice, skin rash, pulmonary infiltration, and/or hepatosplenomegaly lasting for only 1-3 weeks. Laboratory tests revealed no hemolytic anemia and Coombs test was negative. Sepsis or HMR was the main clinical differential. At autopsy, the spleen, liver, lymph node, lung, and bone marrow showed infiltration of atypical "histiocytes" or blasts, lymphocytes, and mature histiocytes with hemophagocytosis. Immunophenotype and gene rearrangement studies of the lymphoid tissues revealed that these atypical "histiocytes" were actually polyclonal B immunoblasts in one case and transformed T lymphocytes in the remaining 2 cases, representing two different types of virus-host interaction. Southern blot and in situ hybridization studies on frozen lymphoid tissues demonstrated the presence of EBV DNA in all 3 patients; the study for cytomegalovirus was negative. The young age of these patients, closely correlated with the prevalent age of primary EBV infection in the general populations in Taiwan, strongly suggest that these childhood cases of previously diagnosed HMR-like disease may actually represent a lethal form of primary EBV infection or fatal infectious mononucleosis.
 ...
PMID:Fatal primary Epstein-Barr virus infection masquerading as histiocytic medullary reticulosis in young children in Taiwan. 196 24

From 1982 to 1985, four cases of primary myelofibrosis were diagnosed in our department. Three were boys and one was a girl. Their ages ranged from 7 months to 15 years. The diagnosis was made based on anemia, leukoerythroblastic change and presence of giant platelets in the peripheral blood, and a bone marrow biopsy showing myelofibrosis. Most of them had anemia, fever, and hepatosplenomegaly on admission. The anemia was severe and refractory to repeated transfusions and steroid therapy in 3 out of the 4 cases. Splenectomy was performed in 1 case, but without satisfactory results. The clinical course and blood pictures in one case resembled leukemia of megakaryocyte lineage (M7), but results of marker studies of the blast cells ruled out the possibility of M7. Three of them underwent leukemic transformation within 2 years and died soon after. The other one died of sepsis 2 weeks after diagnosis. Myelofibrosis in childhood occurs rarely, however, when it does, it always runs a rapid and fatal course.
 ...
PMID:Primary myelofibrosis in children: report of 4 cases. 198 Dec 37

A case of bone marrow myelofibrosis with extramedullary hematopoiesis in the prostate gland is described. This 75-year-old man had an 8-year history of myelofibrosis, massive hepatosplenomegaly, and progressive anemia and a 5-year history of bladder outlet obstruction symptomatology. Transurethral resection of prostate was performed, and light microscopic examination of the prostatic chips revealed a diffuse distribution of atypical megakaryocytes, immature granulocytes, and normoblasts in the prostatic stroma. The prostatic glandular epithelium was uninvolved. Immunostaining for hemoglobin in erythroid precursors and a chloroacetate esterase stain for granulocytic precursors were useful in confirming the diagnosis and in defining the extent of prostatic involvement. Extramedullary hematopoiesis is a rare process in the prostate gland, but it should be included in the differential diagnosis of cellular prostatic stromal lesions, particularly when atypical giant cells are observed.
 ...
PMID:Extramedullary hematopoiesis in the prostate. 203 42

A 68-year-old man presented with a 6-month history of fatigue, rhinorrhoea, pruritic skin lesions, left pleural effusion, ascites, oedema and weight loss of 10 kg. Investigations revealed hepatosplenomegaly, retroperitoneal lymphadenopathy, anaemia, leucocytosis with eosinophilia, hypoprothrombinaemia, hypocholesterolaemia and elevation of both gamma glutamyltransferase and alkaline phosphatase. Biopsies of a skin lesion, bone marrow and liver revealed mast cell infiltration, allowing the diagnosis of systemic mastocytosis (SM). Hydroxyzine plus ranitidine were given without success. Hydroxyzine treatment was stopped, and ketotifen was initiated; substantial symptomatic improvement was observed within 8 d. This case report indicates the effectiveness of ketotifen in the symptomatic treatment of SM.
 ...
PMID:A case of systemic mastocytosis; therapeutic efficacy of ketotifen. 204 Aug 76

A case of chronic Salmonella enteritidis infection in a young adult patient is reported. Following an initial enteritis with bacteraemia, the illness lasted for a 7-year period and was characterized by recurrent pyrexia, anaemia, weight loss and marked hepatosplenomegaly. Investigations revealed that the patient suffers from a mild form of chronic granulomatous disease presenting in adolescence.
 ...
PMID:Relapsing Salmonella enteritidis infection in a young adult male with chronic granulomatous disease. 204 55

A 7-year-old girl with hypereosinophilic syndrome is described, the first such patient to present with inflammatory bowel disease. Hepatosplenomegaly, anaemia, and hypergammaglobulinaemia were also prominent features. Colonoscopy revealed a frank colitis of nonspecific histologic appearance. An elemental diet was unhelpful, but there was a prompt clinical and partial haematological response to systemic steroids. She has remained well receiving a small maintenance dose of prednisolone for 3 years.
 ...
PMID:Hypereosinophilic syndrome in childhood appearing as inflammatory bowel disease. 205 Dec 80


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>