Evaluation of cerebral maturation by visual and quantitative analysis of resting electroencephalography in children with primary nocturnal enuresis.

This study was undertaken to evaluate resting electroencephalographic (EEG) changes and their relations to cerebral maturation in children with primary nocturnal enuresis. Cerebral maturation is known to be important in the pathogenesis of this disorder. Twenty-five right-handed patients with primary nocturnal enuresis, aged 6 to 14 years, and 23 age- and sex-matched healthy children were included in this cross-sectional case-control study. The abnormalities detected using such techniques as hemispheral asymmetry, regional differences, and hyperventilation response in addition to visual and quantitative EEG analysis were examined statistically by multivariate analysis. A decrease in alpha activity in the left (dominant hemisphere) temporal lobe and in the frontal lobes bilaterally and an increase in delta activity in the right temporal region were observed. We concluded that insufficient cerebral maturation is an important factor in the pathogenesis of primary nocturnal enuresis, and EEG, as a noninvasive and inexpensive method, could be used in evaluating cerebral maturation.

Lack of association between plasma leptin levels and appetite in children with iron deficiency.

A negative correlation between leptin and appetite or food intake has been shown in healthy individuals. However, the role of leptin in clinical conditions characterized by anorexia has not been established. One of the well-known clinical features of iron-deficiency anemia is poor appetite. We examined the changes in plasma leptin levels in relation to expected improvement in appetite with iron treatment in children with iron deficiency. In 24 infants and small children (mean age +/- standard deviation = 19.6 +/- 7.7 months) with iron deficiency, we studied plasma leptin levels before and after iron therapy. After 15.0 +/- 2.4 wk of iron treatment, serum ferritin levels improved significantly, with accompanying increases in their subjective appetite scores and food intakes. However, as their mean age and plasma leptin levels adjusted their body mass indexes were unchanged. Serum ferritin correlated significantly with appetite score (r = 0.680, P < 0.001) and food intake (r = 0.480, P < 0.01). Leptin correlated only with body mass index (r = 0.405, P < 0.01). Lack of association between plasma leptin levels and degree of appetite in iron-deficient children treated with iron suggests a leptin-independent mechanism for the observed increase in appetite.

Denver developmental screening test II for early identification of the infants who will develop major neurological deficit as a sequalea of hypoxic-ischemic encephalopathy.

BACKGROUND: The primary aim of this study was to find widely available, inexpensive, and non-invasive parameters for early identification or prediction of the infants with hypoxic-ischemic encephalopathy (HIE) who will have a severe adverse outcome (classified as death or a major neurological deficit). METHODS: Fifty-seven full-term or near-term newborn infants with a diagnosis of HIE were consecutively admitted to the neonatal intensive care unit and studied. Occurrence of seizures during the first 24 h, cranial ultrasonography (US) findings within the first 5 days of life, and Denver developmental screening test II (DDST II) at 6 months of age, were analyzed in relation to mortality and neurological status at 2 years of age. RESULTS: Of the 57 infants, 10 were lost to follow-up. Twenty of the remaining 47 infants had a severe adverse outcome. Among the predictors of severe adverse outcome, occurrence of seizures was found to have a poor predictive accuracy. Cranial US had 100% sensitivity, however with a rather low specificity (55%). However, DDST II at 6 months of age, yielded a very high predictive accuracy (sensitivity=100%, specificity=95%). CONCLUSION: We conclude that DDST II at 6 months of age could be used in predicting severe neurological outcome in infants with HIE.

Effect of gestational age on plasma fibronectin concentrations in the neonate.

BACKGROUND: Fibronectin (FN) is known to have important roles in host defense against infection. The risk for neonatal sepsis increases with the degree of immaturity of the host. Conflicting results have been reported on the relationship between plasma FN levels and gestational age (GA) in the preterm neonate. METHODS: In the present study, we determined plasma FN concentrations with an immunodiffusion method in 40 newborns of various gestational ages, ranging from 30 to 42 weeks. RESULTS: We found a strong direct correlation between plasma FN levels and GA (r = 0.86; P < 0.001). CONCLUSIONS: We speculate that the increased risk for neonatal sepsis with degree of prematurity may, in part, be explained by impaired defense with decreasing plasma FN levels seen with lessening GA.

Development of acute promyelocytic leukemia with isochromosome 17q after BCR/ABL positive chronic myeloid leukemia.

We describe a pediatric case of acute promyelocytic leukemia with an i(17q) after treatment of BCR/ABL positive chronic myeloid leukemia (CML) for 3.5 years. The patient was treated with Busulphan, alpha-2a interferon, hydroxyurea, and cytosine arabinoside at various times in the course of the chronic phase of CML, because he had no HLA-identical donor for bone marrow transplantation. Hematologic remission was achieved for a short time, but cytogenetic remission was never possible. When promyelocytic blast crisis was diagnosed according to the French-American-British classification, cytogenetic studies revealed an i(17q) as a new feature in our patient. The promyelocytic transformation was associated with the appearance of an i(17q) preceding CML are discussed in the light of recent literature.

Recombinant alpha-2a interferon treatment in a child with T-cell leukemia and chronic hepatitis B.

We present the case of a 12-year-old boy with T-cell acute lymphoblastic leukemia (ALL) who developed a chronic hepatitis-B virus (HBV) infection during the consolidation phase of chemotherapy and was unable to receive further therapy because of hepatotoxicity. Recombinant alpha-2a interferon (alpha-IFN) treatment (5 million units/m2 per dose, three times a week) was started for chronic HBV infection at the end of the sixth month, and vincristine (1.5 mg/m2) was administered once a month as the only well-tolerated chemotherapeutic agent. During follow-up, the dose of alpha-IFN was increased to 10 million units/m2 three times a week, depending on the patient's laboratory data. Three months later, elimination of HBe Ag and HBV DNA and seroconversion from HBe Ag to HBe Ab occurred. The duration of alpha-IFN therapy was prolonged to 18 months, since other chemotherapeutic agents caused hepatotoxicity whenever they were tried and alpha-IFN treatment can be used in children with refractory T-cell leukemia in view of its antitumor effect. Our patient has now been in complete remission for 4 years. Alpha-IFN therapy should be considered as an alternative treatment for patients with T-cell ALL who cannot receive chemotherapy because of HBV infection or for any other reasons.

Combined central retinal artery and vein occlusion in a child with systemic non-Hodgkin's lymphoma.

PURPOSE: To report on a case of systemic non-Hodgkin's lymphoma and unilateral combined central retinal artery and vein occlusion. METHOD: We examined a 14-year-old boy who experienced a sudden unilateral visual loss five months after the initial diagnosis of systemic non-Hodgkin's lymphoma. RESULT: Visual loss was due to combined central retinal artery and vein occlusion in association with tumoral optic nerve involvement. CONCLUSION: Although very rare systemic non-Hodgkin's lymphoma may present with central retinal artery and vein occlusion prior to overt central nervous system involvement.

Combination of steroid with azathioprine in treatment of giant cell autoimmune hepatitis.

Giant cell hepatitis is a rare disorder after the newborn period. Drugs, autoimmunity, and viruses (lately, paramyxovirus infection) have been implicated in its etiology. Without treatment, liver dysfunction is progressive and fatal. Immunosuppression with steroids and azathioprine has been demonstrated to sustain improvement in the disease. In this report, a one-year-old boy who has giant cell hepatitis with Coombs' positive hemolytic anemia and anti-smooth muscle antibodies is presented, and the course of the disease and the patient's response to treatment with steroid and azathioprine is reviewed.