The Journal of pediatric endocrinology最新文献

Thirty-seven prepubertal children evaluated for severe growth retardation were studied by assessment of total granulocyte, monocyte and lymphocyte count, lymphocyte subsets CD3+, CD3+Dr+, CD3+Dr-, CD4+, CD8+, CD8+CD57+, CD8+CD57-, CD16+, CD20+ and CD23+, serum immunoglobulin concentrations, and phagocytic activity of circulating neutrophils and monocytes (by a flow cytometric assay). Idiopathic GH deficiency was diagnosed in 21 of 37 patients; the remaining 16 healthy subjects served as controls. Fourteen patients received biosynthetic GH (rhGH), and their immune parameters were assessed at baseline and after 6 months of therapy. Phagocytic function mediated by both polymorphonuclears and monocytes was significantly impaired in GH-deficient subjects compared to controls (p < 0.003 for neutrophils, p < 0.007 for monocytes), while a significant increase of phagocytic activity was obtained during long-term rhGH replacement therapy (p < 0.02 for neutrophils, p < 0.001 for monocytes), thus suggesting that GH may affect the functional activity of circulating phagocyte cells. No significant differences were found in total granulocyte, monocyte and lymphocyte counts, T- and B-lymphocyte subsets and immunoglobulin levels, between GH-deficient patients and controls, and between values observed before and during rhGH substitution treatment.

Children with IDDM have diminished glucagon responses to hypoglycemia. We evaluated possible mechanisms in 60 children and adolescents with IDDM (age 15.4 +/- 2.6 years, duration 7.8 +/- 3.5 years [mean +/- SD]) and without diabetic complications. These were: 1) suppression by hyperinsulinism, 2) autonomic neuropathy, 3) a pan-islet cell defect, and 4) a glucotoxic effect. Glucagon and pancreatic polypeptide responses to hypoglycemia (insulin bolus 0.15-0.75 U/kg) were studied after insulin withdrawal and 3 days of intensive insulin therapy. Responses to arginine and mixed meal were also studied. The control group consisted of children with non-growth hormone deficient short stature. IDDM children had lower glucagon responses to hypoglycemia than controls (p < 0.001), the response to arginine did not differ from controls, and was greater than the response to hypoglycemia (p < 0.001). Responses to hypoglycemia after insulin withdrawal and intensive therapy did not differ. Basal pancreatic polypeptide levels were lower in IDDM than in controls (p < 0.05) but responses to hypoglycemia did not differ between groups. Thus the diminished glucagon response to hypoglycemia reflects a defect in hypoglycemic recognition or response by the alpha cells.

This case report concerns a prepubertal patient (height 148.7 cm) with congenital anterior panhypopituitarism diagnosed at the chronological age of 25.2 years (bone age 12.5). In spite of his advanced age on initiation of hormonal replacement therapy this patient achieved an adult height (172.8 cm), exceeding those of his father and brothers, because of his markedly delayed bone age. This satisfactory height outcome confirms that final height in hypopituitarism depends on both height at onset of puberty and height with respect to bone age but not chronological age at initiation of growth hormone treatment.

Children with long-standing IDDM have impaired counterregulatory responses to hypoglycemia. To determine whether children with new onset IDDM also have altered counterregulation, we studied the counterregulatory responses to hypoglycemia in twenty children with new onset IDDM (5-6 days, age 12.6 +/- 2.9 yr, mean +/- SD), and compared these responses to 47 subjects with long-standing IDDM (duration 7.8 +/- 3.6 yr, age 15.3 +/- 2.5 yr) and 21 controls (age 14.2 +/- 2.8 yr). Six new onset subjects were restudied three months later during their remission. Glucose nadir in new onset (2.7 +/- 0.1 mmol.l-1) was similar to controls (2.4 +/- 0.1 mmol.l-1), but was higher than in long-standing IDDM (2.2 +/- 0.1 mmol.l-1). Both groups of diabetic subjects had lower glucagon responses to hypoglycemia than controls (p < 0.005). Glucagon responses in new and long-standing diabetes did not differ. Epinephrine was diminished in new IDDM compared to controls (p < 0.01). Glucose recovery was faster in new onset than in long-standing IDDM (p < 0.001) and the same as in controls. Responses remained diminished 3 months after diagnosis despite increased C-peptide and lower glycosylated hemoglobin. Thus, children with IDDM have diminished counterregulatory responses to hypoglycemia at diagnosis, that are similar to those in long-standing IDDM. The reasons for this impairment and its clinical application in childhood require further investigation.

We measured T4, T3, TSH, and TBG in 53 children (both asymptomatic and symptomatic) with human immunodeficiency virus (HIV) antibodies, and 17 controls. Although most had normal T3 and T4 levels, two children with acquired immuno-deficiency syndrome (AIDS), who were very ill when studied, had low T3 values. TBG and TSH levels were higher in children with AIDS than in other HIV-infected children or controls (P < 0.005). Increased TSH levels were found in 5 children with AIDS who were recovering from severe illnesses. TSH levels returned to normal without treatment. In summary: 1) the pattern of thyroid abnormalities in children with AIDS was different from that seen in healthy controls, critically ill children, other HIV-infected children, and HIV-infected adults; 2) if an increased TSH is found, measurement should be repeated before instituting thyroxine therapy; 3) an increased TBG is not seen in HIV-infected children until clinically evident AIDS is present.

This study investigated the relationships of neonatal sex-steroid hormones to preschool children's size. Two measures of size were considered: a unidimensional measure of height and a weight-for-height ratio. Results showed a significant positive relationship between girls' neonatal progesterone and the weight-for-height ratio at age four. A negative association of this ratio with an estrogen composite measure was also found. High testosterone girls had higher weight-for-height ratios than low testosterone girls. A significant positive association between the estrogen composite and height was found for boys. These findings indicate that neonatal hormone levels are important complements to environmental factors (e.g., nutrition) in predicting later growth.

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is associated with hormonal imbalance which predisposes affected females to prenatal development of genital ambiguity. Because the disease is usually not lethal and can be treated with glucocorticoids, affected pregnancies are seldom terminated. Dexamethasone can be administered to the pregnant mother and is effective in correcting the fetus's adrenal hormone imbalance during gestation. Nearly a decade's experience with prenatal treatment of CAH indicates that the risk-benefit ratio is favorable for mother and fetus with careful medical supervision of gestationally administered dexamethasone.

Pituitary-hypothalamic abnormalities due to impaired cerebrospinal fluid circulation have long been recognized. The aim of this study was to assess pituitary, thyroid, adrenal, and gonadal function in 46 prepubertal (22 M and 24 F) and 10 pubertal (4 M and 6 F) subjects with myelomeningocele (MMC). Basal serum levels of FT3, FT4, TSH, PRL, LH, FSH, T or E2, cortisol, 17-OH-P and DHEA-S were measured by routine radio-immunoassay methods. Twenty-two prepubertal patients had a TRH test for TSH and PRL evaluation, and eight underwent a GnRH test. Three patients presented with precocious puberty. Six subjects had modest elevations of serum TSH together with normal free thyroid hormone levels. In three cases, TSH responses to TRH were significantly exaggerated and prolonged: in two patients, TSH responses were delayed. The mean basal plasma FSH level in females with ventriculo-peritoneal shunt was significantly higher than in controls. In six cases FSH responses to GnRH were significantly higher than in controls. Both basal and stimulated PRL levels were elevated in patients with shunts; in patients without shunts, basal PRL was normal, but peak PRL levels following TRH stimulation were elevated. Our data show an abnormal hypothalamic-pituitary function in MMC subjects. These findings reinforce the importance of physical examination, hormonal evaluation and follow-up of pubertal development in patients with myelomeningocele.