Vitamin A and beta carotene levels in constitutional delay of growth and puberty

The objective of this study was to investigate the vitamin A (vit A) status and beta carotene levels of children with constitutional delay of growth and puberty (CDGP). Serum vit A and beta-carotene levels of 26 children with CDGP were measured. 20 age-matched healthy children with normal pubertal development served as controls. Except for the height SDS, which was significantly lower in the CDGP group (p0.05). Although serum vit A levels of children in both groups were within normal limits according to WHO criteria, serum vit A levels were significantly lower in the CDGP group than in controls (44.13 +/- 12.25 and 59.60 +/- 19.75 mu g/dl respectively, p<0.05). It was concluded that vit A deficiency may play a role in CDGP in developing countries

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

Partial remission phase and metabolic control in type 1 diabetes mellitus in children and adolescents

A better understanding of the remission phase, while residual beta -cell function is still present in recently diagnosed type 1 (insulin dependent) diabetes mellitus (IDDM), is very important because of the potential for pharmacological intervention to preserve this function. To evaluate the natural course and characteristics of the remission phase in children and adolescents with IDDM, a retrospective study was performed on patients diagnosed with IDDM under the age of 18 years during the years 1991-1998. Sixty-two patients whose medical records were available were included in the study. Data were collected by reviewing the hospital records of patients from the time of diagnosis through the first 24 months after diagnosis. The duration of symptoms and history of infection prior to presentation, diabetic ketoacidosis (DKA) at diagnosis, length of hospitalization, initial glucose level, basal C-peptide levels at diagnosis, daily insulin requirements per kg body weight and HbA(1c) at diagnosis and at each visit were recorded. Thirty-five patients (56.5%) entered partial remission. We observed similar remission rates in those aged 10 years at diagnosis and in boys and girls. History of infection and presentation with DKA were associated with a lower rate of remission (p 5.82 months and was much longer in boys than girls (p 3.73 months. HbA(1c) concentrations in the first year of the disease were significantly lower in patients who underwent a remission phase (7.31 +/- 1.24% vs 8.24 +/- 1.47%, p < 0.05). However, this difference was not observed during the second year of the disease. In conclusion, history of infection prior to presentation and DKA at diagnosis were associated with young age and were the most important factors negatively influencing the remission rate in newly diagnosed IDDM patients

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