Anthropometric and nutritional evaluation of short statured children from low socio-economic class

The objective of this study was to evaluate the anthropometric parameters of short statured children examined in our outpatient clinic. 367 children were evaluated. Body weight for age, weight for height, height for age and body mass index of subjects were expressed as percent of standards. Wasting and stunting were established according to Waterlow's criteria /4/. Of 367 children, 27.8% were wasted and stunted; 59.1% were stunted; 3.8% had constitutional delay of growth and 9.3% had growth hormone deficiency. Ninety-seven percent of growth hormone deficient children had deficiency in weight as well as height. This study indicates that most short statured children and some growth hormone deficient children present with anthropometric indices of chronic. nutritional deficiency in a country where nutritional deprivation is frequent

Helicobacter pylori infection in children with constitutional delay of growth and puberty

Helicobacter pylori is a gastroduodenal pathogen strongly associated with chronic gastritis and duodenal ulceration. It is thought that H, pylori infection might be one of the causes of growth retardation in children. The aim of this study was to evaluate the seroprevalence of H, pylori in children with constitutional delay of growth and puberty (CDGP). H. pylori seropositivity was studied in 24 children with CDGP (22 M, 2 F) and 32 healthy age-matched children with normal pubertal development. Mean age of the children with CDGP was 14.53 +/- 1.12 yr and all of them had been diagnosed as CBGP after physical and laboratory assessment. H, pylori IgG positivity was detected in 16 of the 24 children with CDGP (66.6%) and 12 of 32 controls (37.5%) (p <0.05), This finding is consistent with the hypothesis that H, pylori infection could be one of the environmental factors causing CDGP

SPONDYLOMETAPHYSEAL DYSPLASIA, TYPE-VII

Spondylometaphyseal dysplasia was first described by Kozlowski et al. in 1967 as a new dysplastic bone disease, characterized by metaphyseal dysplasia associated with generalized platyspondyly in the vertebral column [1]. Kozlowski ct ai. have pointed out the autosomal dominant transmission of this disorder at that time. However, later reports showed that thc manner of genetic transmission and the degree of the manifestation of the radiological findings could be variable and accordingly seven types of spondylometaphyseal dysplasia were described [2]. In this article, three cases displaying one of the rare forms of spondylometaphyseal dysplasia,"type VII" Lire presented and the diagnostic findings as well as the differential diagnostic criteria are discussed

Precocious puberty in a patient with indicanuria

A 7-(5)/12 year-old girl, who was followed-up after diagnosis of indicanuria, presented with symptoms of bilateral breast enlargement. Her breast development was at Tanner stage II. No pubic or axillary hair was observed, Pelvic ultrasonograpy revealed multiple follicles on both ovaries. Basic endocrinological evaluation and cranial magnetic resonance imaging (MRT) were normal. The diagnosis of precocious puberty was established with respect to the pubertal response to GnRH stimulation test

Maroteaux-Lamy syndrome associated with growth hormone deficiency

Growth retardation is a common feature of mucopolysaccharide storage disorders, mostly considered to be a consequence of skeletal changes, Maroteaux-Lamy disease is a subtype of mucopolysaccharidosis, demonstrating somatic changes and skeletal deformities. We present a case with Maroteaux-Lamy disease associated with growth hormone deficiency, Magnetic resonance imaging study revealed marked signal changes in white matter due to the storage in brain and empty sella appearance in sellar region, In the presence of empty sella syndrome, hypothalamic-pituitary dysfunction due to the storage material may have led to growth hormone deficiency in this patient. Therefore, we recommend patients with mucopolysaccharidosis, especially those who have growth retardation, to be evaluated by hormonal and radiological studies

Assessment of protein-energy malnutrition in children with chronic arthritis

Protein-energy malnutrition (PEM) has been estimated to occur in 10 to 50% of children with juvenile chronic arthritis (JCA). Thirty-eight children with JCA were evaluated and their nutritional status determined, and they were compared with 23 healthy sex and age-matched children as controls. A standardized, 9-parameter comprehensive nutritional assessment profile was used. The simple anthropometric measurements, height and weight for age, were abnormal in 30% and 27% of the patients, respectively. A detailed evaluation revealed that 71% had abnormal somatic protein stores, and that they also had significantly low levels of visceral protein stores, when compared to their healthy peers. The results were consistent with the fact that inflammation put the JCA patients at significant risk for developing complicated malnutrition and it might result in PEM without any obvious signs of malnutrition. A nutritional screening test would be very useful in detecting early PEM in children with chronic arthritis

Successful therapy with L-T-4 in a 5 year-old boy with generalized thyroid hormone resistance

Resistance to thyroid hormone (RTH) is a rare dominantly inherited disorder caused by mutations in the thyroid hormone receptor P gene which lead to impaired tissue responsiveness to thyroid hormone (TH). RTH is characterized by elevated free thyroid hormone and unsuppressed thyrotropin (TSH) levels. Two types of the disorder have been recognized: selective pituitary resistance and generalized resistance to TH (GRTH). Triiodothyroacetic acid has been used in patients showing hypermetabolism, and L-T-4 treatment in high doses has been suggested in GRTH if patients have signs of clinical hypothyroidism such as growth retardation and developmental delay. The outcome of long-term treatment of GRTH with L-T-4 has not yet been reported. We report a 5 year-old boy who presented with severe growth retardation, fatigue and speech delay. He had hyperactivity despite feeling tired easily. Elevated TH levels with unsuppressed TSH and delayed bone age were determined by laboratory evaluation and he was diagnosed as GRTH. There was no clinicical evidence of hypermetabolism. We could not demonstrate any mutation in thyroid receptor beta1, beta2 or alpha gene of this patient and his parents. L-T-4 treatment was started at conventional doses (6 mug/kg), and after 3 months of treatment T-4 and TSH levels were suppressed successfully. In 12 months of treatment, no side effects were improved without requirement for high doses of L-T-4

Chronic hypoxemia leads to reduced serum IGF-I levels in cyanotic congenital heart disease

Cyanotic congenital heart disease in children commonly causes more pronounced growth retardation in comparison with acyanotic congenital heart disease. Chronic hypoxemia has been suggested as the cause of poor growth in these patients, but the relationship between serum IGF-I levels and chronic hypoxemia is unclear. Serum IGF-I concentrations, oxygen saturation and nutritional status were evaluated in 29 patients with cyanotic congenital heart disease, and serum IGF-I levels were compared with a group of 20 well-nourished, age-matched control children to assess the relationship between IGP-I levels and chronic hypoxemia, The nutritional status of each patient was determined by using anthropometric parameters and calorie and protein intake ratios. The patients were divided into malnourished and well-nourished groups (21 and 8 patients, respectively) according to their nutritional status. Serum IGP-I concentrations were measured in the two patient groups and the controls. The malnourished group had the lowest IGF-I levels (48.14 +/- 21.8 ng/ml, p < 0.05), However, the well-nourished group's IGP-I levels were significantly lower than the control subjects' despite improved nutritional status (85.5 +/- 30.2 and 107 +/- 19.7 ng/ml, respectively, p < 0.05), In addition, me found a positive correlation between serum IGF-I levels and oxygen saturation of the patients (r = 0.402, p < 0.05), These findings indicate that chronic hypoxemia has a direct or indirect effect to reduce serum IGF-I concentrations and this may be a cause of the increased growth failure in patients with cyanotic congenital heart disease