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Type 1 diabetes is a chronic disease caused by a cell-specific destruction of the insulin producing cells of the pancreas. Although Puerto Rico has the highest incidence of type 1 diabetes among Latin American countries, there is scanty data on the presence of antibodies against insulin producing cells. To this end, 20 children (8 males, 12 females), ages 1-15 years, admitted to the University Pediatric Hospital with type 1 diabetes de novo between November 2000 and April 2001 were prospectively studied to determine the presence of serum antibodies against Islet cells (ICA), glutamic acid decarboxylase (GAD-65) and insulin autoantibodies (IAA). IAA was found to be present in 45% of the subjects with 85% of positive rate in subjects under age 5. GAD-65 was present in 66% and ICA was present in 23% of the subjects. We found evidence of autoimmunity against islet cell surface and intracellular components among a cohort of Puerto Rican children with newly diagnosed type 1 diabetes. These findings compared favorably with reports from other ethnicities.

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OBJECTIVE: Psychosocial short stature (PSS), is the only known variant of reversible growth hormone deficiency (GHD)-like state. Herein we present three cases of Puerto Rican children with PSS, which will aid the uninitiated to the entity and assist in making the appropriate diagnosis. All of them demonstrated catch up growth and reversible GHD state as determined by increased insulin-like growth factor 1 (IGF-1) production and growth hormone secretion after pharmacologic stimulation. METHODS: Three boys ages 4.5 to 15.5 years were evaluated because of poor growth at the University Pediatric Hospital in San Juan, Puerto Rico. Medical evaluation excluded organic causes for growth failure. Psychosocial evaluation revealed the presence of repeated instances of psychological abuse by caretakers and the subjects demonstrated patterns of bizarre behavior. These findings prompted evaluation toward the possibility of PSS. The three children were removed from their caretakers' homes and placed in foster nurturing environments. RESULTS: Once relocated, the three children were able to demonstrate marked weight gain, growth acceleration, and improved social behavior. These changes were accompanied by biochemical evidence of GH-axis recovery as determined by the augmented insulin-like growth factor 1 levels and GH secretion. They have continued thriving at their foster homes. These results were felt to be compatible with PSS Type 2. CONCLUSION: We conclude that infants and children with growth failure without apparent organic cause, should be suspected of having PSS. Early relocation is critical for a successful outcome.

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Dysregulation of the growth hormone (GH)-insulin-like growth factor-I (IGF-I) axis in children and adolescents with insulin-dependent diabetes mellitus (IDDM) is well documented. Elevated levels of circulating GH, increased GH secretory amplitude, and decreased concentrations of IGF-I, IGFBP-3, and GHBP have been related to poor glycemic control. We proposed that pubertal maturation may be a more significant factor, potentially overriding the effects of metabolic control, especially during mid-puberty when the GH-IGF-I axis is maximally stimulated. We studied 24 male children and adolescents with IDDM over a 5 year period. Subjects were grouped both by pubertal stage (prepubertal vs mid-pubertal) and by level of glycemic control (hemoglobin A1 (<9%, 9-11.5%, and >11.5%). Twenty-four hour every 20 minute blood sampling for GH determination was analyzed using the Cluster algorithm, and static measures of IGF-I, IGFBP-3, and GHBP were obtained. When analyzed by pubertal status, we found no difference in the number of GH secretory peaks or the interval between concentration peaks. The sum of the peak heights and area under the curve were significantly greater in the mid-pubertal boys, as was the average GH nadir. Serum levels of IGF-I and IGFBP-3 were greater in the mid-pubertal boys, but levels of GHBP were higher in the prepubertal boys. When analyzed by level of glycemic control, we found no differences in the number of GH secretory peaks or interval between peaks among the 3 groups. However, the sum of the peak heights, area under the curve, and average GH nadir were all lower in the group with the intermediate level of glycemic control (HgbA1 9-11.5%); no differences were observed between the other 2 groups. This relationship persisted when the mid-pubertal subjects were analyzed separately. No differences were found among the 3 groups for levels of IGF-I, IGFBP-3, or GHBP. We conclude that normal increases in GH secretion and levels of IGF-I and IGFBP-3 occur during mid-puberty in boys with IDDM. A concomitant increase in average GH nadir may reflect an underlying effect of metabolic control. Greater GH secretion was observed in the groups with the lowest and highest levels of glycemic control. We speculate that this may be related to an increased incidence of severe hypoglycemic episodes in the group with the lowest levels of glycosylated hemoglobin, resulting in metabolic derangements similar to those with elevated glycosylated hemoglobin levels.

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We present the case of an adolescent with hypercalcemia secondary to unrecognized hyperparathyroidism, which lead to complications such as pancreatitis, diabetes mellitus, and nephrocalcinosis. Although hypercalcemia is not common in the pediatric age, its early recognition and intervention are crucial for the prevention of highly morbid complications.

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Diminished concentrations of growth hormone (GH) have been observed in the male BB/Wor rat with diabetes mellitus (DM). The precise mechanism(s) responsible for the altered GH levels is not entirely understood. We have therefore employed independent techniques to investigate potential alterations in: 1) the peripheral metabolism of the hormone; 2) GH release by somatotropes; and 3) hypothalamic regulation of GH secretion. An extra group of insulin-untreated animals was included for the studies of acute DM. The results demonstrate diminished circulating mean concentrations of GH (35 +/- 4 vs. 16 +/- 4 micrograms/l; mean +/- SEM; control vs. animal with DM; P = 0.006) due to impaired GH secretion. In particular, there was a decrease in the mass of GH secreted per burst (230 +/- 22 vs. 136 +/- 34 micrograms/l; P = 0.04) and in the GH secretory rate (24 +/- 4 vs. 9 +/- 3 micrograms/l/min; P < 0.01). No differences in the secretory burst frequency, (5.3 +/- 0.3 vs. 5.2 +/- 0.5 #/8-h; P = 0.68), secretory half-duration (10 +/- 2 vs. 17 +/- 2 min; P = 0.09), or serum GH half-life (8 +/- 1 vs. 6 +/- 1 min; P = 0.13) were observed. In vitro studies of acutely dispersed somatotropes obtained from rats with DM demonstrated increased sensitivity to GHRH (1 nM), as detected by a greater mean hemolytic plaque area following exposure to an EC50 dose of the secretagogue (14.3 +/- 3.3 vs. 17.4 +/- 3.5 microns 2 x 10(3); P = 0.049), and diminished sensitivity to SRIH (1 nM) inhibition of GH release following exposure to an EC50 dose of the secretagogue (10.0 +/- 1.2 vs. 14.9 +/- 2.3 microns2 x 10(3); P = 0.026). The number of the pituitary cells (18.0 +/- 2.8 vs. 15.3 +/- 1.0 x 10(5) cells; P = 0.38) as well as the number of somatotropes (7.3 +/- 1.4 vs. 7.6 +/- 0.9 x 10(5) cells; P = 0.87) were indistinguishable between experimental groups. Hypothalamic gene transcript levels for GH-releasing hormone (GHRH) and somatotropin release-inhibiting hormone (SRIH) were evaluated by in situ hybridization histochemistry to assess cellular synthetic activity.(ABSTRACT TRUNCATED AT 400 WORDS)

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At puberty, elevated circulating GH concentrations are found, with a parallel increase in the levels of insulin-like growth factor-I (IGF-I). However, these hormonal changes are less well understood in children with insulin-dependent diabetes mellitus (IDDM) during the peripubertal years. Since the metabolic derangement is often associated with elevated circulating GH and diminished serum IGF-I levels, we sought to determine whether similar alterations occur in boys with IDDM. A multiple parameter deconvolution analysis was applied to serum GH concentrations measured at 20-min intervals for 24 h in 25 boys with IDDM. Subjects were divided into 3 pubertal groups, pre (n = 9), early (n = 8), and late (n = 11), according to Tanner stage. Glycosylated hemoglobin and body mass index-SD scores were indistinguishable among groups. Forty nondiabetic peripubertal boys served as controls. Similar to those in the normal boys, circulating GH concentrations and serum IGF-I levels increased during puberty in the boys with IDDM. The augmented circulating GH concentrations occur due to an increase in GH secretion, as determined by calculated daily GH production rates (760 +/- 119 vs. 1025 +/- 121 vs. 1821 +/- 266 micrograms/day, respectively for the 3 groups). IGF-I levels were decreased in prepuberty in the boys with IDDM and were overcome with increasing pubertal development (0.68 +/- 0.13 vs. 0.78 +/- 0.11 vs. 1.53 +/- 0.20 U/mL; P < 0.05). There was an increase in the maximal rate of GH secretion per burst (amplitude) during prepuberty (0.54 +/- 0.05 vs. 0.88 +/- 0.17 microgram/L.min, control vs. IDDM; P = 0.03) and early puberty (0.64 +/- 0.10 vs. 0.88 +/- 0.10 microgram/L.min; p = 0.04). The differences in amplitude between the controls and the boys with IDDM were absent once puberty was well established (1.00 +/- 0.10 vs. 1.02 +/- 0.14 microgram/L.min; P > 0.05). The metabolic clearance of GH was increased in the late pubertal boys with IDDM compared to that in their controls (GH half-life, 24.0 +/- 1.0 in control vs. 19.8 +/- 0.5 min in diabetics; P = 0.006). We conclude that comparable increments in GH secretion and serum IGF-I levels in boys with IDDM in moderate glycemic control and controls are presumably related to increased levels of testosterone in both groups. However, differences exist with respect to GH secretory burst amplitude (augmented) and serum IGF-I concentrations (decreased) before puberty is reached. These alterations disappear with the establishment of puberty.

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