Faculty Bibliography
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The purpose of this study was to determine whether children with borderline hypothyroidism in the neonatal period had persistent hypothyroidism after 3 years of levothyroxine replacement therapy. Fourteen term infants with slightly abnormal newborn screening results (thyroxine <10th percentile, thyroid stimulating hormone [TSH] <40 mu U/mL) were identified. The subsequent serum confirmatory TSH results of 12 subjects were modestly elevated (5.3 to 18.8 mu U/mL, normal 0.6 to 4.6), whereas 2 subjects who had borderline confirmatory: TSH (4.6 and 4.7 mu U/mL) had abnormal TSH responses to thyrotropin releasing hormone testing. After 3 years of therapy, levothyroxine was discontinued in 13 patients, and repeat thyroid function tests were obtained 1 month later. Levothyroxine was not discontinued in one patient because he had an elevated random TSH (10 mu U/mL) while receiving therapy. At 3 years of age, 13 patients had persistently abnormal thyroid function tests (TSH >4.6 mu U/mL or a thyroid releasing hormone test result consistent with primary hypothyroidism), and levothyroxine was reinitiated. Only one patient had normal thyroid function studies. Although prospective studies are still lacking, we recommend levothyroxine replacement in neu borns with borderline hypothyroidism. (J Pediatr 2000; 136:53-6).
Recent reports indicate that girls with premature adrenarche are at risk of developing functional ovarian hyperandrogenism and polycystic ovarian syndrome (PCOS). As insulin and insulin-like growth factors (IGFs) have been implicated in the pathogenesis of PCOS, we hypothesize that they may also have a role in the hyperandrogenism of premature adrenarche. Thirty-five prepubertal girls (23 Caribbean Hispanics and 12 Black African-Americans) underwent a 60-min ACTH and LH-releasing hormone test. Insulin sensitivity (S-I) was assessed using the frequently sampled iv glucose tolerance test with tolbutamide. Fasting levels of IGF-I, IGF-binding protein-1 (IGFBP-1), IGFBP-3, sex hormone-binding globulin, and free testosterone (T) were also obtained.
Short stature is a common finding in patients with Ullrich-Turner syndrome. Structural abnormalities involving the terminal short arms of the X and Y chromosomes have been shown to lead to short stature, A putative locus affecting height called PHOG/SHOX has been localized to a 170-kb, critical region within the pseudoautosomal region (PAR), It contains a homeodomain and functions as a transcription factor. We have studied a 10-year-old boy with idiopathic short stature who was found to have a unique Y;13 translocation, Southern blot analysis using cDNA probes indicated that most of the PAR, including PHOG/SHOX, was lost as a result of this translocation, We conclude that haploinsufficiency for this gene is responsible for the growth failure in our patient, Treatment with recombinant growth hormone has resulted in greatly improved growth velocity, (C) 1999 Wiley-Liss, Inc.
In a multicenter study the metabolic effects of 5 yr of GH therapy in children with idiopathic short stature were evaluated. Patients received 0.3 mg.kg.week recombinant human GH. Of the 121 patients who entered the study, data for 62 were analyzed at the final 5 yr point. Routine laboratory determinations were available for all 62 subjects at the 5 yr point. Special laboratory determinations, such as postprandial glucose and insulin, were available for only a subset of patients. Mean insulin-like growth factor I levels rose to 283 +/- 101 mu g/L, within the normal range using age-appropriate reference standards. T-4, cholesterol, triglycerides, blood chemistries, and blood pressure showed no significant changes during the 5-yr period. Mean baseline and 2-h postprandial glucose levels remained unchanged. Both fasting and postprandial insulin levels rose substantively from low normal levels to the normal range (median, 4.9-43 mU/L). Mean hemoglobin A(1c) levels remained within the normal range throughout the study.
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Total Results:
116
Value of growth hormone treatment in Turner's syndrome
PMID: 10905378
ISSN: 1355-008x
CID: 3886262
Metabolic consequences of growth hormone treatment in paediatric practice [Meeting Abstract]
No metabolic side-effects of clinical significance have been reported during a 5-year study of growth hormone (GH) therapy in children with GH deficiency, Turner syn drome, idiopathic short stature or chronic renal insufficiency. In particular, insulin levels increase but remain within the normal range, as do glucose and haemoglobin A(1c). A recent study showed that the effects of growth on insulin sensitivity in prepubertal children with idiopathic short stature represent the changes in carbohydrate tolerance observed during normal adolescence. Thus, GH treatment may lead to prolongation of the physiological state of insulin resistance observed in normal puberty. Insulin levels during the fasting state and 2 h after a standard glucose load showed no further rise after the first 3 years of continuous GH therapy. The hyperinsulinaemia observed during GH therapy may, therefore, amplify the anabolic effects of insulin on protein metabolism during puberty.
ISI:000088760100012
ISSN: 0301-0163
CID: 3492102
Three-year follow-up of borderline congenital hypothyroidism [Meeting Abstract]
ISI:000084910000014
ISSN: 0022-3476
CID: 3492072
Commentary - Growth-promoting strategies Turner's syndrome [Editorial]
ISI:000084134100008
ISSN: 0021-972x
CID: 3492062
The roles of insulin sensitivity, insulin-like growth factor I (IGF-I), and IGF-binding protein-1 and -3 in the hyperandrogenism of African-American and Caribbean Hispanic girls with premature adrenarche
ISI:000080674000057
ISSN: 0021-972x
CID: 3492052
The relationship between birth weight (BW), body mass index (BMI) and insulin sensitivity (S-I) in prepubertal Caribbean Hispanic (CH) and black African-American (BAA) girls with premature adrenarche (PA) [Meeting Abstract]
ISI:000079476700517
ISSN: 0031-3998
CID: 3492022
Prolonged growth response to CB (Saizen (R)) in pediatric subjects with GHD who responded poorly to GHRH (Geref (R)) therapy [Meeting Abstract]
ISI:000079476700547
ISSN: 0031-3998
CID: 3492032
Marked increase in the ability of the beta-cells to secrete insulin in response to glucose and FFA occurs post-puberty [Meeting Abstract]
ISI:000079476700577
ISSN: 0031-3998
CID: 3492042
Deletion of the pseudoautosomal region in a male with a unique Y;13 translocation and short stature
ISI:000078009200007
ISSN: 0148-7299
CID: 3492012
Metabolic consequences of 5-year growth hormone (GH) therapy in children treated with GH for idiopathic short stature [Meeting Abstract]
ISI:000075840700019
ISSN: 0021-972x
CID: 3492002
