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)

An 8-month-old male infant with severe anemia and thrombocytopenia was brought to our hospital due to fever and pallor. The physical examination on admission showed pale conjunctivae, skin rash, lymphadenopathy and hepatosplenomegaly. Subsequent laboratory studies showed pancytopenia, impaired liver function, elevated lactate dehydrogenase level but without evidence of disseminated intravascular coagulation. Bone marrow aspiration demonstrated increased macrophages and prominent hemophagocytosis. The serological studies revealed a recent infection of human herpesvirus-6 which was confirmed by blood mononuclear cell culture and polymerase chain reaction. This infant was also found to have beta-thalassemia which was confirmed by hemoglobin electrophoresis performed before first transfusion. After treatment with intravenous immunoglobulin and other supportive therapies, the bone marrow abnormalities has completely recovered after 5 days, and the hemogram improved. This report demonstrates the human herpesvirus-6 as the etiology of hemophagocytic syndrome.
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PMID:Human herpesvirus-6 associated hemophagocytic syndrome in beta-thalassemia: report of one case. 860 65

Clinical course and histopathologic features of a typical case of familial hemophagocytic lymphohistiocytosis (FHLH) are presented. FHLH, initially known as familial hemophagocytic reticulosis (FHR), is rare and without proper treatment is invariably rapidly fatal, usually accompanied by fever, anorexia, vomiting, irritability and pallor. Sporadic examples with prolonged survival have been reported. Other significant findings include hepatosplenomegaly, progressive anemia, leukopenia, thrombocytopenia, hyperlipidemia and hypofibrinogenemia. Varying degrees of hemophagocytosis by widely disseminated histiocytes in different organs and structures is one hallmark of the disease. Hemophagocytosis may also occur in viral and bacterial infections and in certain malignant processes. Very high parental consanguinity in FHLH was mentioned in two relatively recent reports. A probable immunologic defect has been the focus of recent investigations. The genetic defect is believed to be transmitted as an autosomal recessive trait.
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PMID:Familial hemophagocytic lymphohistiocytosis (FHLH). 909 88

This report describes a full-term newborn with massive fetomaternal hemorrhage. Fetal movements were decreased 48 hr prior to delivery. On the day of delivery, they were absent. The nonstress test was abnormal with low biophysical profile and decreased beat-to-beat variability. The infant presented with extreme pallor, hypotonia, hepatosplenomegaly, and ascites. The initial hemoglobin was 2.2 g/dL, the Kleihauer-Betke stain was 27.6% (highest level ever reported). Right temporal and cerebellar hemorrhages were present. Sequelae include severe developmental delay and asymmetric double hemiplegia.
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PMID:Massive fetomaternal hemorrhage. 925 42

A 24 year old woman living in the Mediterranean region of Turkey present with a three-month history of weight loss and irregular fever that was peaking at 40 degrees C with shivering. No definite aetiology could be identified in a local hospital. A bacterial infection had been suspected, but antibiotic therapy, at first with sulbactam-ampicillin and later with azithromycin, had no influence on the fever. Physical examination revealed an emaciated patient with fever (39 degrees C), pallor, and hepatosplenomegaly (spleen 9 cm and liver 5 cm palpable below the costal margin). No peripheral lymphadenopathy was present. The laboratory examinations are summarised in the table. Notably, a prominent increase of macrophages containing intracellular micro-organisms (figures 1 and 2) was seen in the bone marrow smears. The same micro-organisms were also identified within the Kupffer cells in liver biopsy.
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PMID:Fever, hepatosplenomegaly and pancytopenia in a patient living in the Mediterranean region. 968 80

A retrospective study of all children admitted with the diagnosis of acquired immunodeficiency syndrome (AIDS) at Jos University Teaching Hospital (JUTH) between August 1995 and October 1996 was carried out. Forty three (1.5% out of a total of 2793 children were diagnosed with HIV infection during the study period. However, only the records of 23 out. Of the 43 positive cases were available for analysis. Of the 23 cases whose records were available, 8 presented in 1995, while the remaining 15 presented between January and October 1996. The ages of the children ranged between 1 and 15 years (Mean 3.0 +/- 4.1 Years). There were 12 males and 11 females (M:F = 1:1). Sixteen (69.6 percent) out of the 23 patients were aged between 1 month and 2 years. Sixteen (69.6%) of the 23 patients acquired the infection vertically, 2 (8.9%) acquired the infection through blood transfusion, 1 (4.3%) from sexual abuse, while in 4 (17.4%) the source of infection could not be established due to inadequate data. Majority of the children presented with weight loss, chronic diarrhoea and fever, while the common findings included wasting, oralthrush, pallor, hepatosplenomegaly and lymphadenopathy. Six (26.1 percent) out of the 23 children died, 8 (34.8 percent) were discharged against medical advice and have not been seen since, 9 (39.1%) improved and were discharged to out-patient clinic followup, but all except 2 of these have been lost to follow-up. It is concluded that AIDS is increasingly becoming a major cause of childhood morbidity and mortality in our environment. All children in our environment who present with features of malnutrition should be screened for AIDS. Campaigns aimed at preventing vertical (maternal-child) transmission, including health education of young men and women on the risk of unprotected sex must be vigorously pursued and sustained.
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PMID:Paediatric AIDS in Jos, Nigeria. 992 Oct 95

A total of 405 cases of fever who were either admitted to the Hospital or attended in paediatric out patient Department or Emergency of Medical College Hospital, Calcutta between January '95 and November '95 were included in the study. Majority of cases presented with usual features of malaria like fever with chill and rigor, hepatosplenomegaly, pallor. Apart from these, complicated manifestations like shock, convulsion D.I.C and jaundice were also observed. Some unusual presentations with severe diarrhoea, dehydration and features like that of acute viral respiratory tract infection were highly confusing in terms of clinical diagnosis. P. falciparum was observed in 35.5% of cases. Overall therapeutic response to chloroquin was good, However, two patients died of cerebral Malaria. Five cases of severe malaria were caused by P. vivax however, other etiological features could not be found to attribute the severe nature of these illnesses.
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PMID:Resurgence of malaria in Calcutta in 1995: a hospital based study. 1038 10

We report a case of massive chronic foetomaternal hemorrhage. The labor course was uncomplicated. The newborn presented with pallor. tachypnea, and moderate hepatosplenomegaly. The initial hemoglobin was 6.5 g/dl. The Kleihauer-Betke stain on a maternal blood sample was 12%, which is equivalent to 540 ml of fetal blood in the maternal circulation. A clot in the umbilical vein was demonstrated sonographically. The possible association of foetomaternal hemorrhage with umbilical vein thrombosis is discussed.
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PMID:Anemia due to massive chronic foetomaternal hemorrhage. 1043 73

We present a case of juvenile myelomonocytic leukemia (JMML) with ocular infiltration. A 1-month-old boy presented with myeloid precursors in peripheral blood and a white blood cell count >10x10(9)/l. His peripheral blood monocyte count was >1x10(9)/l, bone marrow blasts were <20%, and no Ph chromosome was identified. The boy also presented with hepatosplenomegaly, pallor, fever, and skin rash. We diagnosed this case as JMML, although hemoglobin F was within the normal range and no spontaneous colony growth was observed from peripheral blood mononuclear cells. Neither Epstein-Barr (EB) virus nor cytomegalovirus was detected by PCR in bone marrow aspirate or peripheral blood. The patient had several lesions into which JMML cells might have infiltrated, including skin, liver, spleen, oral cavity, right lung, sigmoid colon, and both eyes. To our knowledge, this is the first reported case of JMML with ocular involvement. Since infiltration of JMML cells into both eyes causes blindness, further consideration of the timing of bone marrow transplantation (BMT) in JMML is necessary.
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PMID:A case of juvenile myelomonocytic leukemia with ocular infiltration. 1064 83

A 29 day old male infant presented with a history of fever, cough, increasing respiratory distress and abdominal distension from the 10th day of life. Examination revealed failure to thrive, marked tachypnea, pallor, hepatosplenomegaly and harsh vesicular breath sounds. Chest skiagram showed extensive broncho-pneumonic changes. As the infant did not respond to antibiotics, he was investigated for tuberculosis. The gastric aspirate smear showed plenty of acid fast bacilli (AFB) and culture showed M. tuberculosis growth. Endometrial biopsy of the mother showed tuberculous granuloma and acid fast bacilli and culture of the aspirate from endometrium grew M. tuberculosis. The need for endometrial biopsy of mothers of infants with congenital tuberculosis is highlighted.
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PMID:Congenital tuberculosis. 1079 50

Various complications related to gastrointestinal system, central nervous system, and skeletal system may be observed during typhoid fever, but splenic abscess and pleural effusion are rarely encountered. A 12-year-old boy was admitted with fever, fatigue and pallor. On examination he had hepatosplenomegaly and severe anemia. He was diagnosed as having Salmonella typhi infection complicating with splenic abscess, pleural effusion and severe anemia, and successfully treated with percutaneous drainage with ultrasonography and antibiotics. In conclusion we would like to emphasize that typhoid fever should also be considered in patients with hepatosplenomegaly and severe anemia, and percutaneous drainage with ultrasonography may successfully be used in management of splenic abscess in typhoid fever.
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PMID:Splenic abscess, pleural effusion and severe anemia caused by Salmonella typhi. 1141 95


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