Persistent neonatal hyperinsulinism is the most common cause of refractory hypoglycemia during the first year of life. Inadequate insulin secretion is associated to mutations of four different genes, that can be diagnosed to orient patient management. We report two patients: a female newborn that presented a hypoglycemia of 16 mg/dl two hours after birth, was subjected to a subtotal pancreatectomy that did not correct hypoglycemia, requiring a total pancreatectomy. Pathological study of the pancreas showed a focal adenomatous hyperplasia. At the present time, she is three years of age and maintains euglycemia with fractionated feeding. A male newborn that had seizures at 28 hours of life and a hypoglycemia of 15 mg/dl was detected.He was also subjected to a subtotal pancreatectomy, that did not correct hypoglycemia and had to be extended to a total pancreatectomy. At the present time, he is 3 years and 11 months of age and has a normal psychomotor development (Rev Méd Chile 2004; 132: 995-1000
Graves-Basedow disease in pediatric patientsObjective: Determine differences in the epidemiology, clinical features and diagnosis of Graves-Basedow disease (GBD) in prepubertal and pubertal patients. Method: Retrospective study analyzing medical records of 38 patients with GBD at Pontificia Universidad Católica de Chile between 1992-2007. Statistical analysis was performed with non parametric test of Mann-Whitney U and proportions difference with Fisher Test (SPSS 10.0 for Windows and Graphpad Prism 4). Results: 21 patients were prepubertal and 17 were pubertal, with ages between 3 and 15,9 years. There were more girls than boys in both groups (5:2 and 15:2, respectively; p = 0.2). The most common clinical presentations were diffuse goiter, hyperactivity, frequent bowel movements, insomnia and heat intolerance. The prepubertal group had a taller stature (+2.4 SDS) compared with the pubertal group (+0.2 SDS; p = 0.03) and the most frequent ocular manifestation was exophthalmus in both groups. Conclusions: We did not find any differences in the clinical presentations of Graves-Basedow disease among prepubertal and pubertal patients. Neuropsychiatric symptoms such as hyperactivity and insomnia, together with tall stature are common features in children with GBD.
Hormone profile end pelvic ultrasonography were studied in 41 girls with premature thelarche aged 21 .2 ± 9.7 months and in 39 non affected conrrol girls of the same age and social status. Estrogenic contaminants were looked for both in girls sera and in poultry samples through serum and tissue levels of zearalenone and diethilbestrol. Total estrogenic activity was measured by radioreceptor assay. Basal serum levels or LH, FSH, prolactine and estradiol [E2] were not different between both groups. Total estrogenic activity was significantly higher in girls with thelarche [201 ± 102 vs 78 ± 20 pg E2 eauivalent), turning later to sim'lar levels than in controls in five patients in which thelarche eventually subsided. After LHRH greater FSH than LH peak response was observed (28.7 ± 1 7.5 Up/ml vs 5, ~; ± 2.7 Uu/m!|. No differences were detected in uterine size and squeletal maturation, but a greater mean ovarian volume was seen in patients with thelarquia. No estrogenic contaminants v/ere detected in poultry samples. Partial activation of ihe hypothalamus-pituitary-axis, mainly through FSH secretion, might thus be the responsible of ovarian secretion of E2 or another substance w ; tn estrogenic action.
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