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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A preliminary report is presented on a current prospective virological and clinical study of congenital and acquired infant cytomegalovirus (CMV) infections. During a 1-year period, 7/2200 newborn Swedish infants investigated (0.3%) had a congenital CMV infection as shown by positive virus isolation. Two of them had typical symptoms, hepatosplenomegaly and petechiae in one case and splenomegaly in the other one. All of them had a normal birth weight and normal head circumference. No sequelae have been observed during an observation period of up to 9 months. Five out of 10 control infants followed-up acquired a CMV infection within a few months. 5/7 mothers of the congenitally infected infants and 3/14 mothers of the control infants were primiparas.
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PMID:A prospective study on congenital and acquired cytomegalovirus infections in infants. 22 34

Two distinct and characteristic cases of osteopetrosis are presented. The first concerns a fourth month old baby with the malignant form of the disease, initiated since the newborn period. He was admitted because of abnormal ocular movements and probably deafness. At the physical examination he showed bilateral optic atrophy, abnormal ocular movements, hepatosplenomegaly and petechia. The diagnosis was confirmed by clinical means and radiological studies of virtually all of his skeleton. Several fractures were found. Laboratory tests showed marked anemia, trombocytopenia "tear cells", evidence of medullary erythropoiesis and myelofibrosis. An increased alkaline phosphatase was detected in serum and in granulocytes. The second case corresponds to a 26 year-old female with the benign form of the disease. She had multiple pathological fractures specially of her lower limbs, that produced severe impotence in her gait. In this case, the diagnosis is suspected on clinical basis and confirmed with the characteristic radiological findings. Some of the more distinct features of the disease are discussed, specially those concerning the genetic mode of inheritance and consanguinity, the etiopathogenic, pathophysiology, clinical, laboratory, prognosis and treatment, with particular reference to the radiological and hematologic problems related with this metabolic disorder.
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PMID:[Osteopetrosis (report of 2 cases)]. 91 41

Knowledge of the natural history of symptomatic congenital cytomegalovirus (CMV) infection in the newborn is essential in order to anticipate complications and assess the potential benefit from antiviral therapy. To define the disease course we reviewed data on 106 neonates with symptomatic congenital CMV infection diagnosed and managed by the investigators. Petechiae, jaundice and hepatosplenomegaly were each noted in 70% or more patients. Microcephaly was noted in 54 of 102 (53%) at birth. Elevated alanine aminotransferase, conjugated hyperbilirubinemia and thrombocytopenia were seen in 83, 81 and 77%, respectively. Eighty-six percent had at least two of the manifestations highly suggestive of congenital infection. Platelet count fell to its nadir during the second week of life whereas elevated alanine aminotransferase and direct bilirubin persisted past the first month. In spite of the difficulty in assessing central nervous system function in the newborn, evidence of damage was present in the majority. Seventy-two had microcephaly, poor suck, lethargy/hypotonia or seizures. Abnormal computerized tomographic scan was present in 16 of 20 (80%) and decreased hearing in 20 of 39 (56%). Cerebrospinal fluid protein was greater than 120 mg/dl in 24 of 52 (46%) and this elevation was associated with neurologic abnormalities as well as hearing loss. The mean length of hospital stay was 13 and 22.4 days for term and preterm infants, relatively. Thirteen infants (12%) died during the first 6 weeks of life. Disseminated CMV infection with multiorgan involvement was evident in 7 of 9 at postmortem examination. We conclude that neonates with symptomatic congenital CMV infection have a multi-system disease with significant morbidity and mortality.
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PMID:Symptomatic congenital cytomegalovirus infection: neonatal morbidity and mortality. 131 Oct 66

The cytomegalovirus (CMV) is an abiquitous agent that infects almost all human beings at some time during their lives. In developing area of the world, 90% or more of the population is infected during childhood. However, in developed countries the infection is acquired at a lower rate. In neonates, CMV infection can be divided into congenital and perinatal infection. Congenital CMV infection is the result of transplacental transmission, CMV can be transmitted to the fetus following reactivation as well as primary infection during pregnancy. The incidence of congenital infection is 0.2% to 2.2%. Symptomatic congenital CMV infection is more likely to be the result of primary as opposed to reactive CMV infection during pregnancy. The clinical manifestations of symptomatic CMV infection are hepatosplenomegaly, microcephaly, jaundice, petechiae, small for gestation age, periventricular calcification and chorioretinitis. Mortality may be as high as 30% among the most severely affected infants. In the survivors, about 90% will develop mild to severe handicaps. Perinatal CMV infection can be acquired from exposure to virus in the maternal genital tract at delivery, breast milk, or through blood transfusion. In premature infants who require prolonged and intensive medical care, blood transfusions are an important iatrogenic cause of CMV infection. Transfusion-acquired perinatal CMV infection can cause significant morbidity and mortality, particularly in premature infants with a birth weight of less than 1250 gm born to CMV-seronegative mothers. So CMV acquisition can be prevented either by providing these infants blood products from seronegative donors or by using frozen deglycerolized red blood cells. Two cases of neonatal CMV infection are reported, one with congenital infection, the other with perinatal infection.
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PMID:[Cytomegalovirus infection in neonate--report of two cases]. 256 49

Corynebacterium sp. are found as normal flora in skin and mucosal sites. They have been isolated in empyemas, brain abscesses, blood cultures and ventricular shunts. About 9-10% of early-onset and 4-5% late-onset prosthetic valve endocarditis are due to different species of the so-called "diphteroids". A 30 year-old white female was admitted after 30 days with fever of undetermined origin. A mitral prosthesis had been fitted in 1977. On physical examination a protomesosystolic mitral murmur, petechiae, retinal hemorrhages and hepatosplenomegaly were detected. Laboratory tests showed 37% hematocrit, 14,800/mm3 white blood cells, 78 mm ESR, urinary sediment: less than 30/h.p.f. red blood cells. A new first-degree A-V block was detected. Blood cultures were negative. Due to persistent fever, progressive anemia, leukocytosis and new vegetations on echocardiogram, surgery was performed. A mitral valve ring abscess was found. Corynebacterium xerosis was isolated from surgical specimens. The strain was found susceptible to penicillin, ampicillin, oxacillin, ticarcillin, piperacillin, cephalotin, cefoxitin, cefoperazone, rifampin, gentamicin, amikacin, and norfloxacin. Studies with clindamycin, disclosed MIC and MBC = 0.25 mg/l. The patient received 1800 mg/day clindamycin for 4 weeks. Serum cidal studies showed a peak concentration 1/128 and a titre of trough 1/4. Negative control blood cultures were obtained. She has remained well for nine months after treatment. Corynebacterium sp. can cause "apparently" negative blood cultures. Blood samples should be incubated for more than 15 days before they can be considered negative. Almost 50% of previously described cases have been detected during the six months after cardiac surgery. Mortality has been high (48%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Endocarditis due to Corynebacterium xerosis]. 263 Aug 75

A seven year-old boy with hereditary stomatocytosis complicated with aplastic anemia was reported. He was admitted to our hospital because of pale and general fatigue. On physical examination, he had severe anemia, petechiae, but no hepatosplenomegaly. Peripheral blood cell count revealed pancytopenia; RBC 103 X 10(4)/microliters, Hb 3.5 g/dl, Ret 21%, WBC 1,200/microliters, Pl 1.3 X 10(4)/microliters, and bone marrow revealed markedly hypocellular marrow. Red cell morphology demonstrated stomatocytosis. Red cell life span (51Cr T1/2) was 12 days, Coombs' test and Ham's test were negative. Indirect bilirubin was 1.1 mg/dl and marked decrease of haptoglobin was found. Family studies showed that his father and sister had stomatocytosis on peripheral blood examination, but no anemia. The patient had severe anemia because of complicated aplastic anemia. Congenital stomatocytosis with aplastic anemia is extremely rare. The authors are interested in a possible relationship between hereditary stomatocytosis and aplastic anemia although the precise mechanism remains to be elucidated.
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PMID:[Congenital stomatocytosis associated with aplastic anemia]. 279 85

Three cases of overt congenital cytomegalovirus infection, each with a different mode of presentation, are documented. Common features of presentation were hepatosplenomegaly, skin petechiae, neonatal jaundice and gross hepatic dysfunction. One infant presented with hydrops fetalis, a feature not described before. Two infants survived and are at present developing normally.
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PMID:Congenital cytomegalovirus infection. 628 May 77

A 2-month-old girl presented with enlarged head girth, generalized petechiae, anemia, coagulopathy and hepatosplenomegaly. Imaging studies showed a huge, dumbbell-shaped intracranial hemangioma located between the falx, and involving the supra- and infra-tentorium, extending through the posterior fontanel to involve the subgaleal area. A urine culture grew cytomegalovirus. Severe thrombocytopenia was refractory to a massive platelet transfusion, intravenous immunoglobulin and corticosteroid therapy. Hypertension, pulmonary hemorrhage and sepsis complicated the course. After establishing a diagnosis of Kasabach-Merritt syndrome, subcutaneous injections of alpha-interferon were given with an initial dose of 1 x 10(6) IU/m2 followed by 3 x 10(6) IU/m2 per day for 12.5 mo. Her platelet count rose gradually and became stable after 1.5 mo of interferon treatment. The intracranial hemangioma regressed remarkably and the hepatosplenomegaly was also resolved. The infant showed good growth and development, without obvious side-effects during the 23-month follow-up period. The treatment with recombinant alpha-interferon appeared to be effective in reversing thrombocytopenia associated with the patient's massive intracranial hemangioma.
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PMID:Recombinant alpha-interferon treatment of intracranial hemangioma and Kasabach-Merritt syndrome in an infant with cytomegalovirus. 761 60

Systemic lupus erythematosus (SLE) is not uncommon in Thai children and adults but neonatal lupus erythematosus has never been reported in Thailand. A case of NLE born of a mother without prior history of SLE was reported. He was initially misdiagnosed as congenital self-healing histiocytosis because of seborrheic dermatitis-like lesions, petechiae, hepatosplenomegaly and thrombocytopenia. Features supporting the diagnosis of NLE in this case were presented.
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PMID:Neonatal lupus erythematosus in Thailand. 782 97

Using a passive surveillance system, 15 cases of symptomatic congenital cytomegalovirus (CMV) infection were identified in the Louisville area over approximately 3 years. When compared to mothers delivering healthy infants in Jefferson County, mothers of infants with congenital CMV were younger, more often nonwhite, and from lower socioeconomic status. Affected infants had a lower mean gestational age and birth weight, and most demonstrated classic features of congenital CMV infection such as jaundice, petechiae, hepatosplenomegaly, intrauterine growth retardation, and microcephaly. The overall infant mortality rate was 33%. Maternal and infant characteristics were similar to those of 167 cases in a national database and 106 cases reported from Birmingham, Alabama. The estimated minimal incidence of symptomatic congenital CMV disease in this area was 1.16/10,000 live births for white and 6.06/10,000 for nonwhites, establishing this as a significant public health problem in this part of Kentucky.
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PMID:Congenital cytomegalovirus disease in the Louisville area: a significant public health problem. 799 40


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