Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The signs that may arise after perinatal infection with human immunodeficiency virus type 1 (HIV-1) have been classified by the Centers for Disease Control, but the clinical usefulness of the classification system and the prognostic importance of each disease pattern have not been established. We sought to address these issues by analysing data from the Italian Register for HIV infection in children. We studied 1887 children born to HIV-1-seropositive mothers. 1045 were identified at birth and the others were registered later (median age 4.8 [range 0.4-72] months). HIV-1-associated signs developed in 433 (81.8%) of 529 seropositive infected children at a median age of 5 (0.03-84) months. These signs appeared significantly earlier in the 102 children who died of HIV-1-related illness than in those who are still alive (median 3 [0.03-55] vs 6 [0.03-84] months; p less than 0.001). The cumulative proportion surviving at age 9 years was 49.5% (95% confidence interval 27-65%) and the median survival time was 96.2 months. Separate analysis of the 112 seropositive infected children followed from birth and older than 15 months gave similar results. Hepatomegaly, splenomegaly, lymphadenopathy, parotitis, skin diseases, and recurrent respiratory tract infections formed the mildest disease pattern. Lymphoid interstitial pneumonitis and thrombocytopenia were signs of intermediate disease. By contrast, in multivariate analysis specific secondary infectious diseases, severe bacterial infections, progressive neurological disease, anaemia, and fever were significant and independent negative predictors of survival. Growth failure, persistent oral candidosis, hepatitis, and cardiopathy were associated in univariate analysis with significantly shorter survival. Our findings suggest that the outlook for children with perinatal HIV-1 infection is better than previously thought and that a new clinical staging system of single disease patterns is needed.
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PMID:Prognostic factors and survival in children with perinatal HIV-1 infection. The Italian Register for HIV Infections in Children. 134 67

To determine the clinical factors that are of prognostic importance in protein-energy malnutrition (PEM), 150 patients admitted into the paediatric wards at the University College Hospital, Ibadan, Nigeria, were studied. Detailed clinical history, anthropometry and biochemical investigations were done immediately on admission. Case fatality rate decreased with age: 75% and 33% in those aged 12 months and below, and 30 to 36 months, respectively. The mortality in marasmus, kwashiorkor and marasmic-kwashiorkor were 35%, 47% and 60%, respectively. Hepatomegaly on admission had no significant impact on prognosis, but mortality increased with increasing hepatomegaly. Growth failure on admission, as measured by weight-for-age, weight-for-height and mid-upper-arm circumference were significantly associated with poor prognosis (p < 0.01, p < 0.01 and p < 0.001, respectively). Hypokalaemia and hyponatraemia were each separately associated with poor prognosis. When both electrolytes were low, more patients died (81%) than when both were normal (24%) (p < 0.001). Similarly, hypoproteinaemia and hypoalbuminaemia were associated with poor prognosis singly and in combination. When both serum proteins and albumin were low, the mortality was 32%, and there were no deaths when both were normal. The outcome in severe PEM is still poor. Good clinical precision in identifying clinical and biochemical prognostic factors, early intervention and good subsequent management are important in reducing mortality in PEM.
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PMID:Prognostic factors in severely malnourished hospitalized Nigerian children. Anthropometric and biochemical factors. 811 61

Growth failure in Type 1 Diabetes Mellitus (T1DM) can occur for several reasons. Mauriac syndrome is a rare cause of severe growth failure in T1DM. There may be different forms and etiologies involved in Mauriac syndrome. However, there are common features noted in these patients. We have compiled a review of cases reported in English in the last 30 years. With adequate insulin treatment there is reversal of growth failure and hepatomegaly if present. However, overly aggressive insulin delivery could result in rapid deterioration of diabetic retinopathy and nephropathy. Close monitoring of growth and pubertal maturation in children with T1DM is essential.
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PMID:Mauriac syndrome: growth failure and type 1 diabetes mellitus. 1880 15