Nutritional rickets in suburbia.

OBJECTIVE: Vitamin D deficiency continues to be a problem in pediatrics. This report presents four children, one Caucasian male and three African-American females aged 4 to 24 months who were treated for vitamin D deficiency rickets. METHODS: One female was diagnosed in the Emergency Department during evaluation of a viral syndrome, another presented with hypocalcemic seizures and the third was a self-referral for evaluation of widened wrists. The male had biochemical rickets discovered incidentally during a hospitalization for pneumonia. All were breastfed without formula supplements. The 24-month female had severe cow and soy protein allergies and received multivitamin supplements intermittently. Birth order was from third to sixth child. Two families practiced Islam and the mothers wore veils. The females had a weight deficit for height. The females demonstrated a rachitic rosary, widening of the wrists and leg bowing. At diagnosis the serum calcium was 5.0-8.6 mg/dl, the inorganic phosphorus was 1.5-3.9 mg/dl and the alkaline phosphatase was 408-3324 U/L. The serum intact parathormone levels and the vitamin D levels were measured at Nichols Laboratories. The 25-OH vitamin D levels were 2-22 ng/ml and the 1,25(OH)2 vitamin D levels were 14-122 pg/ml. All had elevated parathormone levels. The three females had roentgenographic evidence of rickets. Two of the children also demonstrated iron deficiency. RESULTS: All patients responded to Vitamin D supplements, beginning at 2000 IU for the male and 8,000-10,000 IU daily for the females. Two children were also given calcium supplements. The three females all showed complete healing of the rickets radiologically within six months. The serum intact parathormone demonstrated an inverse correlation with the serum calcium during recovery (r=-0.669; p<0.05). CONCLUSION: Vitamin D deficiency does still occur. Breastfed children of multiparous mothers, with increased skin pigmentation, living in the higher latitudes are at increased risk and would benefit from vitamin D supplementation while breastfeeding.

Double isochromosome 8q as single cytogenetic aberration in acute myelo-monocytic leukemia.

We report a case of double isochromosome 8q as a single cytogenetic abnormality in a patient with acute myelo-monocytic leukemia. Similarly to rare cases with tetrasomy 8, the patient showed monocytic involvement and was refractory to cytotoxic chemotherapy. We conclude that this kind of cytogenetic aberration is probably associated with distinct morphologic and clinical characteristics.

Body composition and spontaneous growth hormone secretion in normal short stature children.

This study was designed to compare the relationship of measured and estimated indices of adiposity with the spontaneous GH secretion (SGHS) in 37 normal short stature children. Fifteen of the 37 patients (10 males and 5 females) were pubertal, and 22 (17 males and 5 females) were prepubertal. All patients underwent a review of their medical history, a physical exam, laboratory tests, and a nutritional assessment that included anthropometry and evaluation of body composition by bioelectric impedance. The percentage of body fat and body fat mass index were used as measured indices of adiposity. The weight for height ratio, body mass index, and body mass index z-scores were calculated and used as estimated indices of adiposity. Our results showed that SGHS is greatly influenced by variations in adiposity in normal short stature children, and measured indices of adiposity demonstrated the strongest negative correlation with SGHS. Gender differences were apparent in the degree of adiposity that modified SGHS, and it appears that adiposity altered the amplitude of GH pulses in pubertal patients and the number of pulses in prepubertal children. These results suggest that interpretation of SGHS must take into account body composition and gender in addition to pubertal status.

Alterations in spontaneous growth hormone (GH) secretion and the response to GH-releasing hormone in children with nonorganic nutritional dwarfing.

The effects of suboptimal nutrition on the spontaneous overnight GH secretion (SGHS) and the GH response to GHRH were studied. Sixteen patients with nonorganic nutritional dwarfing (ND) were compared with 25 healthy short children with familial short stature with or without constitutional growth delay (FC). The effects of puberty were also assessed. All patients underwent an overnight study to assess SGHS with serum GH levels sampled every 20 min for 12 h, and a GHRH stimulation test was administered. Pubertal ND children had a blunted SGHS with a mean overnight GH level of 4.9 +/- 1.1 micrograms/L, significantly less than the level of 6.2 +/- 1.8 micrograms/L of the pubertal FC children (P less than 0.05). Also, prepubertal ND patients had an area under the curve in GH secretion after GHRH which was greater than that of the pubertal ND patients (2483 +/- 1581 vs. 1600 +/- 1056, P less than 0.05). The peak GH response to GHRH in the prepubertal ND patients was also higher than that of the pubertal ND patients (51.8 +/- 22.1 micrograms/L vs. 22.5 +/- 15.4 micrograms/L, P less than 0.05). This study shows that the SGHS is attenuated in ND patients during puberty but their GH response to GHRH is increased before adolescence. These abnormalities may represent compensatory mechanisms to energy restriction and may increase our understanding of the poor growth seen in ND patients.

The relationship between beta-endorphin and the growth hormone (GH) response to GH releasing hormone in prepubertal children.

Endogenous opioids are thought to participate in the regulation of growth hormone (GH) release through the mediation of growth hormone releasing hormone (GHRH). This study was intended to investigate whether the endogenous opioid beta-endorphin could modulate the GH response to GHRH and if this hypothesis could be demonstrated in children with familial short stature with or without constitutional growth delay. Seventeen children (6 female and 11 male) with stature below the fifth percentile were studied to rule out disorders in growth hormone dynamics. All had normal growth velocities, had appropriate predicted heights for their families and had normal GH levels on standard testing. Eight were prepubertal and 9 were Tanner II. All were given 0.1 mcgm/kg (1-44)hpGHRH-NH2 IV. Blood for growth hormone was obtained at 0, 15, 30, 45, 60, 90 and 120 minutes. Blood for beta-endorphin and cortisol was obtained at 0 and 60 minutes. The basal beta-endorphin level significantly correlated with the peak GH level (r = 0.868, p less than 0.05) in the prepubertal group only. In the same group of children, the degree of the negative feedback on the beta-endorphin level correlated significantly with the rise in GH level (r = 0.912, p less than 0.01). However, there was no correlation between the basal beta-endorphin and the peak GH level nor between the rise in GH level and the change in beta-endorphin in the pubertal children. These data are compatible with the hypothesis that beta-endorphin levels affect the GH response to GHRH in prepubertal children, but have no discernible effect on the GH response to GHRH in pubertal children.

A survey to determine the prevalence of abnormal growth patterns in adolescents from a suburban school district.

A longitudinal survey of 1017 (514 male, 503 female) school health records was undertaken to assess the prevalence of normal and abnormal growth patterns in adolescents attending a suburban upper middle class junior-senior high school. The vast majority (97.4% of the students) were growing and gaining weight at a steady rate, maintaining a similar percentile for height and weight throughout adolescence. However, 75% of these students had mild deficits or excesses of body weight for height, which also remained constant throughout adolescence. Approximately 10% of the students had a body weight deficit or excess for height greater than 20%. These students were growing along normal percentile patterns. This was true whether the student had short, normal, or tall stature. Only a minority (2.6%) had an abnormal growth pattern. There were 18 students who had deteriorating linear growth and had decelerated across one or two major percentile lines for height. Only eight students demonstrated accelerated growth characterized by progressive weight gain greater than 10 kg/year and crossing a major percentile line for weight.

Parental health beliefs as a cause of nonorganic failure to thrive.

Parental misconceptions and health beliefs concerning what constitutes a normal diet for infants is reported as a cause for failure to thrive. There were seven patients (four boys, three girls), 7 to 22 months of age, who were evaluated for poor weight gain and linear growth. They were only consuming 60% to 94% of the recommended caloric intake for age and sex. The children's caloric intake had been restricted by their parents. They were concerned that the children would become obese, develop atherosclerosis, become junk food dependent, and/or develop eating habits that the parents believed were unhealthy. The parents instituted diets consistent with health beliefs currently in vogue and recommended by the medical community for adults who are at risk for cardiovascular disease. These diets caused the infants to experience inadequate weight gain and have a decreased linear growth rate. With nutritional counseling, all food restrictions were removed, the caloric intake was increased to 94% to 147% of the recommended intake for age. The weight gain rate increased significantly (P less than .05) from 0.1 +/- 0.1 kg/mo to 0.4 +/- 0.3 kg/mo, and the linear growth rate increased significantly (P less than .05) from 0.4 +/- 0.4 cm/mo to 1.0 +/- 0.6 cm/mo within 3 months of therapy. Exaggerated concerns about excessive food intake in childhood and/or concern about the sequelae of eating an improper diet has resulted in this entity of failure to thrive due to parental health beliefs.

Fear of obesity. A cause of short stature and delayed puberty.

We evaluated 201 children for short stature or delayed puberty or both. Fourteen of them (nine boys and five girls, aged 9 to 17 years) fit a pattern of growth failure due to malnutrition, which was the result of a self-imposed restriction of caloric intake arising from a fear of becoming obese. All 14 patients underwent a complete history, physical examination, diagnostic laboratory evaluation, and psychiatric assessment. They were all below the fifth percentile for weight, and 11 of them were also below the fifth percentile for height. The deficit of weight for height ranged from 5 to 23 per cent. Seven of the older patients also had delayed puberty. All 14 patients had deteriorating linear growth, which was preceded by at least one to two years of inadequate weight gain. They ingested only 32 to 91 per cent of the recommended caloric intake for their age and frequently skipped meals. No gross psychiatric disease or anorexia nervosa was found; on the whole, they were good students with rather compulsive, shy personalities observed in an open-ended interview. The Diagnostic Interview for Children and Adolescents, which was conducted with seven patients, also revealed no psychiatric disease. After nutritional and psychiatric counseling, the patients resumed an adequate caloric intake for their age, and recovery occurred, as demonstrated by increased linear growth and sexual development.