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)

A patient with an unusual myeloproliferative disorder, characterized by hepatosplenomegaly, myelofibrosis, ringed sideroblasts, and conversion to acute myeloblastic leukemia developed cardiac tamponade secondary to pericardia extramedullary hematopoiesis. Diagnostic criteria are discussed. Irradiation and systemic chemotherapy were successful in controlling the effusion.
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PMID:Cardiac tamponade resulting from pericardial extramedullary hematopoiesis: a case report and review of the literature. 38 7

An unusual case of chronic myelogeous leukemia (CML) is reported which was characterized by leukocytosis without a shift to the left, elevated leukocyte alkaline phosphatase, positive indirect Coombs' test, anemia and thrombocytosis, as well as the absence of hepatosplenomegaly. The diagnosis of CML was ascertained by the presence of Philadelphia chromosome with translocation of its deleted arms on the short arms on the short arms of a chromosome No. 6. The possible relationship between the chromosomal aberration and the unusual hematological and clinical features of this case is discussed.
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PMID:Chronic myelogenous leukemia with elevated leukocyte alkaline phosphatase, positive indirect Coombs' test, neutrophilic leukocytosis and unusual cytogenetical findings. 40 33

Seventy children with hepatomegaly, between the ages of 3 months and 13 years, were investigated including a liver biopsy in 60, to study the general pattern of liver disease in children. Thirty percent had acute viral hapatitis, 20 percent cirrhosis, 17.6 percent pulmonary tuberculosis, 18 percent hereditary diseases and 14 percent miscellaneous diseases involving the liver. None of the cases met the criteria for Indian childhood cirrhosis. It was concluded that in Karachi pulmonary tuberculosis was a common case of hepatosplenomegaly in children and that the aetiology of cirrhosis was probably multifactorial.
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PMID:Aspects of paediatric liver disease in Karachi. 40 63

The first case of gamma-heavy chain disease described in Spain is here reported. The patient, a 36-year-old woman, presented fever, enlarged regional lymph nodes, and hepatosplenomegaly, without bone marrow abnormalities but with lymphopenia. Serum electrophoresis did not disclose any M-component. The abnormal gamma-chain protein had a alpha2-globulin mobility and was immunochemically related to the Fc fragment. It belonged to the IgG 4 subclass, its molecular weight was about 60,000. Proteinuria was minimal but the electrophoresis of concentrated urine showed a homogeneous peak of alpha2-globulin mobility constituted by the gamma-chain fragment. Biopsy of an axillary lymph node disclosed features of immunoblastic sarcoma. The course was malignant, resulting in death in 8 months.
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PMID:A new case of gamma-heavy chain disease. 41 49

A 42-year-old woman died after an episode of anaphylaxis associated with a raised serum histamine level. A diagnosis of systemic mastocytosis was established, with lymphadenopathy and hepatosplenomegaly, not associated with the usually pre-existing skin lesions of urticaria pigmentosa.
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PMID:Fatal anaphylaxis in systemic mastocytosis. 42 77

From July, 1974 to February, 1978, we managed 12 infants with listeriosis. This infection presented in two distinct forms: an early-onset type (nine patients), often representing a congenital infection following maternal illness, and a late-onset type in which the patient presented with meningitis (three patients). Of our nine infants with early-onset disease, three died, three developed permanent sequelae, and only three were normal at follow-up. Appropriate early management in the perinatal period may improve the outlook in this condition. Affected infants were often premature and had pneumonia, rash, and hepatosplenomegaly at birth. Prenatal clues to the diagnosis included maternal fever, abdominal pains, and leukocytosis with meconium staining of the preterm amniotic fluid. Examination of gastric aspirate at birth showed gram-positive coccobacilli. Antibiotic therapy should be started prenatally and continue for three weeks after birth to prevent recurrences of the late-onset type. This late-onset disease presented as meningitis after the second week of life and responded well to antibiotics. Our three patients recovered without sequelae.
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PMID:Perinatal listeriosis--a review of twelve patients. 43 5

A 27-year-old woman presented with high spiking fevers, progressive malaise, weight loss, and marked hepatosplenomegaly. The clinical picture and laboratory findings (including hepatic scintiscan, coeliac arteriography and abdominal lymphangiography) prompted surgical exploration of hypochondriac organs. On laparotomy, both liver and spleen surface exhibited extensive inflammatory lesions. Histopathologic investigations on hepatic tissue showed microscopic caseating granulomata. Specific stains and cultures for mycobacterium, fungi etc. were negative in tissue preparations. Antituberculous and steroid therapy elicited a rapid response. Follow-up observations indicated the resolution of specific hepatic lesions.
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PMID:[Isolated hepatosplenic tuberculosis: 1 case]. 44 14

Two brothers are described with ichthyosis, hepatosplenomegaly and signs of cerebellar degeneration. The ichthyosis was similar to autosomal dominant ichthyosis both clinically and biochemically. No metabolic abnormality has yet been identified to account for this previously undescribed triad of abnormalities.
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PMID:A syndrome of ichthyosis, hepatosplenomegaly and cerebellar degeneration. 44 32

A two and a half-year old child is described who presented with signs of portal hypertension (hematemesis, hepatosplenomegaly, ascites). Her subsequent work-up revealed that the "pressure-head" originated within the pulmonary arterial bed. Indeed, severe changes of primary pulmonary hypertension were found at autopsy. What is unique about this case is the absence of cardiopulmonary symptoms prior to the development of suprahepatic portal hypertension. In addition, the pulmonary disease developed in the absence of underlying chronic hepatic disease or extrahepatic portal vein thrombosis which, reportedly, can lead to pulmonary hypertension.
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PMID:Primary pulmonary hypertension presenting as portal hypertension. 45 36

A child with Niemann-Pick disease type B, diagnosed at the age of two years and followed up for three years is described. Despite extensive visceral involvement--as deduced from marked hepatosplenomegaly and pulmonary infiltration--the child is in general good health and has no apparent neurologic abnormality. Biochemical studies revealed a tenfold increase of sphingomyelin content in his liver biopsy specimen and a markedly reduced sphingomyelinase activity in peripheral blood leukocytes. His parents were found to be heterozygous carriers of the disease, showing sphingomyelinase activity values intermediate between those of patient and healthy subjects. The relevant literature, concerning patients with the non-neuronopathic type B form, is reviewed.
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PMID:Clinical and biochemical study of a child with the non-neuronopathic-Type B form of Niemann-Pick disease. 45 85


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