Prevalence of vitamin D deficiency rickets in the eastern part of Turkey.

Turkey, especially its eastern part, has been accepted as endemic for vitamin D deficiency rickets (VDDR). In a study performed by our team in the region in 1998, the incidence of VDDR was 6.09% in children aged between 0-3 years. In 2005, the Ministry of Health initiated a free vitamin D supplementation campaign nationwide for every infant to eradicate VDDR. In this study, we aimed to investigate the prevalence of VDDR in children aged between 0-3 years in order to evaluate the effectiveness of this campaign. Between March 2007 and February 2008, 39,133 children aged between 0-3 years who were brought to different pediatric outpatient clinics in Erzurum, Turkey, were examined for VDDR. VDDR diagnosis was made by radiological and biochemical findings in the cases who were initially suspected of having clinical VDDR. During a one-year period, 39 (0.099%) of the 39,133 patients were diagnosed with VDDR. None of the cases with rickets was taking vitamin D supplementation. The most frequent physical findings were rachitic rosary, enlargement of the wrists, and craniotabes. The laboratory findings of the cases were compatible with VDDR; serum calcium (Ca) 7.5 +/- 1.9 mg/dL, PO4 4.4 +/- 1.3 mg/dL, alkaline phosphatase (ALP) 1,341 +/- 823, 25-hydroxyvitamin D (25 (OH) D) 5.8 +/- 2.9 ng/mL, intact parathyroid hormone (iPTH) 240 +/- 106 pg/mL. It was concluded that, although VDDR has been a continuing childhood health problem, a nationwide free vitamin D supplementation campaign initiated by the government appeared to be effective in eliminating VDDR.

Growth hormone therapy in children with chronic renal failure.

Growth is impaired in a chronic renal failure. Anemia, acidosis, reduced intake of calories and protein, decreased synthesis of vitamin D and increased parathyroid hormone levels, hyperphosphatemia, renal osteodystrophy and changes in growth hormone-insulin-like growth factor and the gonadotropin-gonadal axis are implicated in this study. Growth is adversely affected by immunosuppressives and corticosteroids after kidney transplantation. Treating metabolic disorders using the recombinant human growth hormone is an effective option for patients with inadequate growth rates.

Once-daily use of colchicine in children with familial Mediterranean fever.

Patients with familial Mediterranean fever routinely take daily multiple doses of colchicine to prevent acute attacks and to reduce the risk of amyloidosis. However, although there is no research in this regard in the literature, some clinicians believe that the use of colchicine in children as daily single dose may cause toxic side effects. The efficiency and the side effects of the administration of daily single dose versus daily multiple doses of colchicine were evaluated in the treatment of patients with familial Mediterranean fever. Thirty-nine patients being observed at Atatürk University Medical Faculty Pediatrics outpatient clinic with the diagnosis of familial Mediterranean fever were randomly divided into 2 groups. Group I consisted of 20 patients who continued taking colchicine in 2 or 3 divided doses daily as they did before the study. Group II comprised 19 patients who were given the total daily colchicine dose at 1 time. Patients were re-examined at 30-day intervals and both groups maintained this regimen for an average duration of 8 months. There was no difference between these 2 groups with respect to frequency of side effects, number of attacks, and acute phase response. Therefore, the daily colchicine dose can be given to children once daily.

Assessment of goiter prevalence, iodine status and thyroid functions in school-age children of rural Yusufeli district in eastern Turkey.

According to previous studies, Turkey has generally been accepted as a moderate endemic iodine deficient country. However, it has recently been reported that there are regions in Turkey where iodine deficiency is more severe than previously known. The current study was aimed at ascertaining the goiter prevalence by thyroid volumes, iodine status and thyroid functions in school-age children living in an area which is suspected to have moderate or severe iodine deficiency. Overall goiter was found in 47.6% of children, in 22.8% of girls and in 24.8% of boys. Mean thyroid volumes did not differ significantly according to sex. Significant correlation was found between thyroid volume and body surface area and age. There was a negative correlation between the urinary iodine concentration and thyroid volume (r = 0.45, p < 0.01). Median urinary iodine concentrations in subjects with and without goiter were 20 microg/dl and 5.2 microg/dl, respectively. While median urinary iodine levels of the subjects with goiter were consistent with severe-moderate iodine deficiency, levels in subjects without goiter were comparable to moderate-mild iodine deficiency. None of the subjects had the signs or symptoms of hyper-or hypothyroidism. The differences in the mean values of thyroid hormones and TSH levels between subjects with or without goiter were not significant (p > 0.05). No correlation was found between urinary iodine concentrations and thyroid hormone levels. A weak correlation was found between urinary iodine concentration and TSH levels (r = 0.12, p = 0.05). Individuals with goiter were investigated etiologically: biochemical hypothyroidism was detected in 2%, compensated hypothyroidism in 12.6%, autoimmune thyroiditis in 2%, nodular goiter in 3% and isolated high TSH level with autoimmune thyroiditis in 0.08%. In conclusion, although a salt iodization program has been started in Turkey, our study indicates that some regions with severe iodine deficiency are still present. This research suggests that this program should be re-evaluated for remote areas with self-contained economic systems, and should be expanded and more effectively applied nation-wide.

Cystinosis presenting with findings of Bartter syndrome.

A five-year-old boy was referred to our pediatric clinic for evaluation of failure to thrive, headache, intermittent high fever, restlessness, polyuria, and polydipsia. His weight and height measurements were under the 3rd percentile. Clinical findings consisted of frontal bossing, carious teeth, O-bain deformity of the lower extremities, and moderate dehydration. The presence of metabolic alkalosis, hypokalemia, hypochloremia, and high renin and aldosterone levels were suggestive of Bartter syndrome and a treatment regimen for Bartter syndrome was started. At follow-up, the polyuria and hyponatremia were found to persist. A reassessment of the patient revealed findings consistent with proximal renal tubular acidosis such as metabolic acidosis with a high urinary pH, proteinuria, aminoaciduria with phosphaturia and hypercalciuria. Based on the presence of parental consanguinity as well as polyuria, proteinuria, low tubular reabsorption of phosphorus, generalized aminoaciduria, light yellow skin and hair color, the probable diagnosis of cystinosis was established and was confirmed by slit-lamp examination of the cornea showing cystine crystal deposition. Our case is a good example demonstrating that development of metabolic alkalosis does not exclude cystinosis and that all findings of the patient should be thoroughly evaluated.

Lack of relation between serum parathyroid hormone levels and erythrocyte osmotic fragility in pediatric patients on peritoneal dialysis.

Secondary hyperparathyroidism and anemia are the hallmarks in uremic patients. It is suggested that parathyroid hormone increases erythrocyte osmotic fragility and induces hemolysis. The present study was undertaken to examine the possible relationship between erythrocyte osmotic fragility and secondary hyperparathyroidism in 20 pediatric patients on maintenance peritoneal dialysis. We found that erythrocyte osmotic fragility in these patients was normal. No correlation between erythrocyte osmotic fragility and hematochemical changes associated with secondary hyperparathyroidism was found. We conclude that erythrocyte osmotic fragility was normal in pediatric patients on peritoneal dialysis and excess parathyroid hormone levels do not affect erythrocyte osmotic fragility and do not cause anemia.

Carnitine supplementation improves apolipoprotein B levels in pediatric peritoneal dialysis patients.

There have been conflicting reports concerning the effect of carnitine supplementation on lipid metabolism in patients on peritoneal dialysis (PD). We investigated several parameters of lipid metabolism in pediatric PD patients supplemented with carnitine. The study included 20 patients receiving PD (treatment group) aged 2-18 years and a matched healthy control group. In the treatment group, baseline triglyceride, total cholesterol, low-density lipoprotein cholesterol, very low-density lipoprotein cholesterol, and apolipoprotein B levels were higher than in the control group. High-density lipoprotein cholesterol, free fatty acid, phospholipids, and apolipoprotein A-I levels were not different from those in the control group. The baseline plasma free carnitine level was lower and acyl-carnitine level was higher in the treatment group. No difference was found between the groups with respect to plasma total carnitine levels. Oral l-carnitine supplementation (50 mg/kg per day for 30 days) led to a significant decrease (from a baseline value of 146.6+/-51.8 mg/dl to 63.6+/-22.2 mg/dl, P<0.001) in apolipoprotein B levels, and no significant change in the other lipid parameters of the treatment group. Oral l-carnitine supplementation does not ameliorate the lipid profile in pediatric PD patients, but it causes a significant decrease in apolipoprotein B levels. Hence, carnitine supplementation may be recommended for decreasing apolipoprotein B levels in this patient population.