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Presents a letter to the editor about the effect of serum platelet-activating factor on type 1 diabetes.

Objective: To identify the difficulties experienced by medical caregivers of inpatients diagnosed with osteogenesisimperfecta., Methods: The descriptive, cross-sectional study was conducted at a university hospital in western Turkey from April to May, 2012, and comprised relatives providing care to patients who were diagnosed with osteogenesisimperfecta and were being treated in the paediatric endocrinology unit. Data was collected via face-to-face interviews with patient relatives. The 35-itemquestionnaire had 16 open-ended and 19 close-ended questions., Results: The mean age of the 46 caregivers was 35.52±6.65 years, and 43(93.5%) of them were mothers. All of them said they felt anxious (100%), while 44(95.7%) felt sad/sorrow, 41(89.1%) puzzled, 40(87.0%) nervous, 40(87.0%) frightened, 39(84.8%) disappointed, 29(63%) shocked, and 28(60.9%) depressed when they first heard the diagnosis. Overall, 26(56.5%) experienced physical, 45(97.8%) psychological, 45(97.8%) social, and 35(76.1%) economic changes and difficulties, while 24(52.1%) had no social support. Of all the patient relatives, 38(82.6%) were unable to obtain adequate information about the disorder from the healthcare team., Conclusions: Caregivers of patients diagnosed with osteogenesisimperfecta experienced psychological and social difficulties..

Objective: To investigate the impact of a psycho-educational program developed for the caregivers of patients diagnosed with osteogenesis imperfecta (OI)., Methods: The participants consisted of 16 caregivers. The study was designed as a quasi-experimental pre-test/post-test type study consisting of 10 semi-structured three-hour training sessions. The data were collected using the "Introductory Information Form" and appropriate scales (Burden Interview, Coping Strategies Scale, Problem-Solving Inventory and Psychosocial Adjustment to Illness Scale). The results were evaluated by descriptive statistics, correlation analysis, one-way variance analysis and Bonferroni analysis., Results: Psychosocial adjustment levels of the caregivers of OI patients before their participation in the educational program were found to be associated with styles of coping with stress, problem-solving skills and care burden. After the psycho-educational training, the majority of the participants reported favorable changes in their lives. Following the offered psycho-education resulted in positive changes in the mean scores of the caregivers (p<0.05)., Conclusion: Before the education program, the participants were not able to deal efficiently with many aspects of their caregiver responsibilities and suffered from an emotional burden due to lack of knowledge. The program appears to have provided them both with support to achieve significant psychosocial transformation and with an opportunity to reconsider their lives in multiple dimensions.

Objective: Medical nutritional therapy is important for glycemic control in children and adolescents with type 1 diabetes mellitus (T1DM). Carbohydrate (carb) counting, which is a more flexible nutritional method, has become popular in recent years. This study aimed to investigate the effects of carb counting on metabolic control, body measurements and serum lipid levels in children and adolescents with T1DM., Methods: T1DM patients aged 7-18 years and receiving flexible insulin therapy were divided into carb counting (n=52) and control (n=32) groups and were followed for 2 years in this randomized, controlled study. Demographic characteristics, body measurements, insulin requirements, hemoglobin A1c (HbA1c) and serum lipid levels at baseline and at follow-up were evaluated., Results: There were no statistically significant differences between the groups in mean HbA1c values in the year preceding the study or in age, gender, duration of diabetes, puberty stage, total daily insulin dose, body mass index (BMI) standard deviation score (SDS) and serum lipid values. While there were no differences in BMI SDS, daily insulin requirement, total cholesterol, low-density lipoprotein and triglyceride values between the two groups (p>0.05) during the follow-up, annual mean HbA1c levels of the 2nd year were significantly lower in the carb counting group (p=0.010). The mean values of high-density lipoprotein were also significantly higher in the first and 2nd years in the carb counting group (p=0.02 and p=0.043, respectively)., Conclusion: Carb counting may provide good metabolic control in children and adolescents with T1DM without causing any increase in weight or in insulin requirements.

Objective: Adiponectin and high-sensitivity C-reactive protein (hsCRP) can be used as early biochemical markers of cardiovascular diseases (CVDs). Radiologically, non-invasive flow-mediated dilation (FMD) of the brachial artery and carotid intima-media thickness (CIMT) measurements may be used as indicators in the early diagnosis of CVDs. To compare the biochemical markers of atherosclerosis with radiological markers of CVDs (CIMT, FMD, ventricular systolic and diastolic functions) and to assess the relationship of these parameters with metabolic control in diabetic children and adolescents., Methods: A total of 55 patients with type 1 diabetes mellitus (T1DM) of at least 5-year duration and 30 healthy subjects were included in the study. Serum adiponectin, hsCRP, hemoglobin A1c (HbA1c), and lipid levels were evaluated in the patients and in the controls. CIMT, FMD, ventricular systolic and diastolic functions were assessed by echocardiography., Results: Mean age of the patients with diabetes was 17.6 years; mean diabetes duration was 10.4 years. Mean serum hsCRP was elevated in children with diabetes (0.21±0.31 vs. 0.10±0.16 μg/mL, p=0.00), while no significant difference from the controls was found in adiponectin levels. Mean CIMT was significantly higher in diabetic children compared to the control group (0.53±0.11 vs. 0.34±0.46 mm, p=0.00). Mean FMD of the diabetic children was significantly lower than that of the controls (6.86±2.85% vs. 12.13±1.99%, p=0.00). Diabetes duration was positively correlated with CIMT and negatively correlated with FMD. Right ventricular (RV) and left ventricular (LV) myocardial performance index (MPI) were higher in the patient group (p=0.00)., Conclusions: Our data suggest that in addition to standard echocardiography, tissue Doppler echocardiography, FMD, and CIMT can be used as early-stage radiological markers and hsCRP as an early-stage biochemical marker of atherosclerosis in the routine follow-up of T1DM patients.

Objective: The aim of this study was to evaluate the clinical course of Hashimoto's thyroiditis (HT) in children and adolescents and the effects of levothyroxine therapy on the clinical course and laboratory findings., Methods: The clinical and laboratory data of 101 patients with HT at presentation and during a three-year follow-up period were retrospectively evaluated using patient records., Results: The mean age of the patients at the time of diagnosis was 12.3 ± 2.90 years and female/male ratio was 5.7/1. The complaint at the time of hospital presentation was goiter in 57.8% of the patients. At baseline, 36.7% of the patients were euthyroid, whereas 32.7% had subclinical hypothyroidism, 16.6 % of subjects were evaluated as hypothyroid. Twelve of the 28 patients who were initially euthyroid and not receiving therapy developed subclinical or overt hypothyroidism during the first 18 months of the follow-up period and were started on thyroid medication. At presentation, the mean anti-thyroglobulin (anti-Tg) and anti-thyroperoxidase antibody levels were 450 ± 725 IU/mL and 392 ± 428 IU/mL, respectively and at the end of the follow-up period, a significant decrease was observed in the anti-Tg levels of patients receiving levothyroxine from the beginning., Conclusions: Thyroid functions of the patients with HT should be monitored periodically for hypothyroidism. Levothyroxine therapy may positively affect the clinical course of the disease and the antibody titers.

Objective: In this study, we aimed to show the role of ghrelin in growth delay in children with constitutional delay of growth and puberty (CDGP)., Methods: Thirty male children with CDGP constituted the study group and fifteen healthy children with normal growth of similar ages-the control group. In both groups, fasting and postprandial plasma ghrelin levels, serum insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 (IGFBP-3) levels were determined., Results: There were no differences in fasting and postprandial ghrelin levels (824.23±523.46 pg/mL and 447.26±259.92 pg/mL, respectively) in children with CDGP compared to the levels in the control group (687.38±481.43 pg/mL and 365.59±260.43 pg/mL, respectively; p>0.05). Differences in fasting and postprandial ghrelin levels were also similar in the two groups (394.44±369.10 pg/mL and 346.55±338.67 pg/mL, respectively; p>0.05). Serum IGF-1 levels were significantly depressed in children with CDGP compared to those in the control group (239.5±83.95 ng/mL and 339.20±63.08 ng/mL, respectively; p<0.05)., Conclusion: Decreased appetite and feeding problems in children with CDGP were not related to depressed ghrelin levels. In addition, ghrelin levels did not increase to compensate for the decreased appetite and feeding problems in CDGP.

Background: Much epidemiological evidence has linked low birthweight with late cardiovascular risk. Intrauterine growth retardation (IUGR) is associated with the increased risk of cardiovascular disease in adult life; it is unclear whether the relationship is present at younger ages. We evaluated whether abdominal aortic stiffness was altered in patients with IUGR (born at term with birthweight small for gestational age) in younger ages., Methods: Thirty-two (24 girls and eight boys) IUGR children aged 8.77 +/- 2.05 years were enrolled in the study. The birthweight was traced from the medical records. Their gestational ages were 38.9 +/- 0.85 weeks and birthweights 2130 +/- 198 g, respectively. Thirty-one healthy subjects who had normal gestational age and birthweight, matched for age and sex were recruited as a control group. Aortic strain, pressure strain elastic modulus (Ep), and normalized Ep and aortic distensibility were measured by a sphygmomanometer and transthoracic echocardiography in all subjects from the abdominal aorta., Results: There was no statistically significant difference between the study and the control groups in sex, mean age, body mass index, lipid profile, leptin, insulin-like growth factor-1 or insulin-like growth factor binding protein 3. In IUGR children, aortic strain (0.201 +/- 0.027 vs 0.254 +/- 0.031, P < 0.001) and aortic distensibility (1.08 +/- 0.19 vs 1.42 +/- 0.24, P < 0.001) were significantly lower compared with the control group. However Ep (188 +/- 36.2 vs 146 +/- 27.1, P < 0.001) and normalized Ep (2.97 +/- 0.40 vs 2.1 +/- 0.39, P < 0.001) were significantly higher in IUGR patients., Conclusions: This study demonstrates that abdominal aortic stiffness is increased in IUGR patients. These data suggest that prenatal events could be related to cardiovascular risk in later life.

Sialic acid storage disorder, known as Salla disease, is a rare autosomal recessive lysosomal disorder produced by a defect of a proton-driven carrier that is responsible for the efflux of sialic acid from the lysosomal compartment. We report two patients with Salla disease: a two-year-old girl, presented with hypotonia, inability to speak and walk, bilateral optic atrophies, defective myelination, cerebellar atrophy, and thinning of the corpus callosum on magnetic resonance imaging (MRI), who was classified as intermediate severe Salla disease; and a four-year-old girl, presented with relatively late-onset, slight hypotonia, and delayed language and mobility development, and supported by relatively protected MRI findings, who was classified as conventional Salla disease. Diagnosis of Salla disease was confirmed by accumulation of sialic acid in fibroblast culture: 15.1 and 13.2 nmol/mg protein in the first and second patient, respectively. Optic atrophy observed in the first case may be an additional feature besides the characteristic manifestations of Salla disease.

Spontaneous adult height (AH) in Turner syndrome (TS) varies among populations. Population-specific AH data is essential to assess the efficacy of growth-promoting therapies in TS. A multicenter study was performed to establish AH of nongrowth hormone (GH)-treated Turkish patients with TS. One hundred ten patients with TS (diagnosed by karyotype) who reached AH (no growth in the previous year, or bone age > 15 years) without receiving GH treatment were included in the study. The average AH was found to be 141.6 +/- 7.0 cm at the age of 22.9 +/- 6.2 years, which is 18.4 cm below the population average and 16.4 cm below the patients' mid-parental heights. Bone age at start of estrogen replacement was 12.3 +/- 1.3 year. Karyotype distribution of the patients was 45X (43%), 45X/46XX (16%), 45X/46Xi (12%), 45XiXq (10%) and others (19%). When the patients were evaluated according to their karyotype as 45X and non-45X, no significant difference in AH was observed (142.4 +/- 6.9 cm vs 140.9 +/- 7.1 cm, respectively). Adult height of non-GH-treated Turkish TS patients obtained in this study was comparable to that of other Mediterranean populations, but shorter than that of Northern European patients. Karyotype does not seem to affect AH in TS.

Background: Insulin glargine provides effective glycemic control when administered at bedtime in adults., Objective: This study aims to investigate whether insulin glargine is equally effective if administered in the morning or at bedtime in combination with preprandial anologue insulin., Methods: Twenty-eight patients that have been treated with an intensified insulin regimen for at least one year were randomized to insulin glargine injection at breakfast (06:00-09:00) (12 patients) or bedtime (21:00-24:00) (16 patients), plus meal-time anologue insulin in the two groups. Glucose data from each day were analyzed at four different times: between 9:00 and 21:00 (t1), between 21:00 and 24:00 (t2), between 24:00 and 04:00 (t3),04:00 and 09:00 (t4) by the Minimed continuous glucose monitoring system., Results: Baseline characteristics were similar in the two groups. The sensor values were lower before breakfast in the bedtime group (180.5 ± 49.0 vs 223.8 ± 47.3 mg/dl, p=0.03). There were 13.7 events.patient (-1).day(-1) in the bedtime group and 6.9 events.patient (-1).day(-1) in the breakfast group in which glucose levels fell below 60 mg/dl (p=0.3). There were 121.6 events.patient (-1).day(-1) in the bedtime group and 162.4 events.patient (-1).day(-1) in the breakfast group in which glucose levels exceeded 180 mg/dl (p=0.05). Nighttime hypoglycemia only reached to a statistical significance between the two groups between 24:00 and 04:00. There were no significant correlations between the duration of nocturnal hypoglycemia, age, duration of diabetes, gender and HbA1c levels., Conclusion: Breakfast group is hyperglycemic during the day and hyperglycemia starts in the morning at 04:00. There is no significant difference in the frequency or duration of hypo/hyper glycemia during the day and night irrespective of the timing of glargine injection except pre-breakfast levels are significantly better in the bedtime group and hypoglycemia occurs between midnight and 04:00 in the bedtime group.

Background: Reconfirming the diagnosis of childhood onset growth hormone deficiency (GHD) in young adults is necessary to demonstrate the need for continuation of GH therapy., Objective: This nationally-based study was planned to establish GH status during adulthood in childhood-onset GH deficient patients and to evaluate factors that would predict persistency of the GHD., Methods: In this multicenter study, 70 GH deficient patients who had reached final height were evaluated after completion of GH treatment. Fifty-two patients (74%) had isolated GHD and 18 patients (26%) had multiple pituitary hormone deficiency (MPHD). Patients who had reached final height and the pubertal Tanner stage 5 were reevaluated for GH status. After at least 6 weeks of cessation of GH treatment, patients were retested with insulin induced hypoglycemia., Results: GHD was found to be transient in 64.3% of all patients. Of the isolated GH deficient patients 82.7% had transient GHD, whereas 88.9% of the MPHD patients showed persistent GHD. Comparison of isolated GH deficient and multiple hormone deficient patients indicated higher peak GH, IGF-I and IGFBP-3 levels in isolated GH deficient patients. No parameter was significantly different in the transiently and persistently GH deficient patients with respect to gender. Although specificity of IGF-I value of less than -2 SD showing persistency of GHD was lower than the specificity of IGFBP-3 value of less than -2 SD (65.7% vs 84%), negative predictive values were similar for the two parameters (85.2% and 84%, respectively)., Conclusion: Most of the cases of childhood onset GHD are idiopathic and the GHD is transient. In patients with MPHD, GHD is generally permanent. Low IGF-I and IGFBP-3 levels are supporting findings to show persistency of the GHD.

Different therapy models have been tried in order to decrease bone resorption in osteogenesis imperfecta. Bisphosphonates are a group of drugs that mainly suppress osteoclast-mediated bone resorption, thus reducing bone turnover. We assessed the effects of low-dose bisphosphonate treatment in children with osteogenesis imperfecta. Sixteen osteogenesis imperfecta patients (12 female, 4 male) with severe deformities were treated with cyclic (3-4 mg/kg/year) intravenous infusions of bisphosphonate (Aredia-Novartis) therapy for a period ranging from 0.6 to 4.7 years (mean 2.50 +/- 1.09 years). Bone mineral density increased from 0.304 +/- 0.146 g/cm2 to 0.362 +/- 0.142 g/cm2 in the first year and to 0.421 +/- 0.146 g/cm2 in the second year. A clinical response was shown with a reduction in fracture rate and improvement in mobilization scores. Fracture rates decreased from a median of 4/year (0-30/year) before treatment to 0/year (0-5/year) during treatment. Ambulation improved in 10 children and remained unchanged in three. Two of the children were fully functional before therapy and one was below two years of age. No adverse effects were seen with pamidronate infusions of 7-10 mg/kg/year (monthly) or with 4 cycles/year 3-4 mg/kg/year. Low-dose cyclical pamidronate infusions markedly increased bone density and decreased bone fracture rate and should be considered as a part of a multi-disciplinary treatment.

The aims of this study were to investigate the effect of exogenous obesity on respiratory function tests, to define the relationship between the severity of obesity and respiratory function test parameters, and to detect the incidence of airway hyperresponsiveness and exercise-induced bronchospasm in an obese study group. This cross-sectional controlled study was done with 38 exogenous obese patients, aged 9 to 15 years, and 30 healthy children. Basal respiratory function test parameters were measured with spirometry. To display airway hyperresponsiveness, 4.5% hypertonic saline provocation test was used; exercise-induced bronchospasm incidence was defined with bicycle ergometry. Basal respiratory function test parameters were lower in the study group as compared with the control group. Exercise test was positive in 31.6% of the obese group and in 3.3% of the control group (P = 0.003). The provocation test with hypertonic saline test was positive in 18.4% of the obese group. There were strong negative correlations between body mass index (BMI), relative weight, skin fold thickness, waist/hip circumference ratio and basal forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and peak expiratory flow (PEF) values. The diagnosis and management of exercise-induced bronchospasm may improve exercise performance and physical activity, assist with weight loss, and break the vicious circle.

Objective: This study was designed to evaluate iodine deficiency status in children 6-12 years in the west coast (Aegean Region) of Turkey after 5 years of mandatory iodine prophylaxis. A total of 2300 children from 72 populations (rural and urban area) were evaluated with urinary iodine excretion and thyroid volume., Design: Cross-sectional, observational study designed and performed according to surveillance methods for iodine deficiency disease (IDD) prevalence recommended by WHO/UNICEF/ICCIDD., Subjects: The study population consisted of 2300 school children age ranging from 6 to 12 years. The children were selected by multiple stage randomization from 91 primary schools of 76 zones (91 clusters). Information about the use of iodized salt was obtained from the families., Measurements: Data on the following were collected: birth date, sex, weight, height, thyroid size by palpation and ultrasonography; and urinary iodine by isotope dilution analysis method. Thyroid volumes above 97th percentile according to the WHO/ICCIDD by age and body surface area (BSA) were accepted as goitre., Results: Iodized salt consumption was 51.7%. The prevalence of goitre determined by palpation was 12.1% and by ultrasound based on BSA and age were 9.8% and 5.5%, respectively. Median urinary iodine was 53 (2-142) microg/l., Conclusion: Mild to severe degree of iodine deficiency was detected in the west coast of Turkey.

Reduced bone mass is a common complication of renal transplantation in adults but only few data are present for pediatric transplant patients. Bone mineral status of pediatric renal transplant patients ages ranging from 7.5 to 17.6 years (mean age 14.9 +/- 2.3) who were at least 6 months postrenal transplantation was examined. Bone mineral density (BMD) of lumbar vertebrea and femoral neck was determined by dual energy X-ray absorptiometry (DEXA) and z-scores according to age, puberty, height and bone age were compared to sex and ethnic specific reference data. z-scores were calculated for both areal and volumetric bone density. BMD L1-4 z-scores were more than 2 SD below the mean according to chronological age in 12 patients (63%), pubertal status in six patients (31.5%), bone age in five patients (26.3%) and height in five patients (26.3%). The BMD femoral neck z-scores were more than 2 SD below the mean according to age in 10 patients (55.5%), puberty in five patients (27.7%), bone age in three (16.6%) patients and height in five (26.3%) patients. Correction of the vertebrae and femoral neck for bone size yielded osteoporotic values for seven patients (36.8%) for lumbar BMD and for four patients (22%) for femoral neck BMD. The use of aBMD in growth-retarded children has some restrictions in determining z-scores. Deficits in spinal bone density still persisted after correcting for height, puberty, bone age and volume. In renal transplant patients who have short stature it is reasonable to give values corrected for height, puberty, bone age and bone size and interpret each of these values for each patient.

Obesity is a prevalent pathological and nutritional disease relating to clinical and sub-clinical disorders. Furthermore, its complications exert a major impact on cardiovascular risk. Myocardial performance index (MPI) has been described as noninvasive Doppler measures of left ventricular (LV) function. The purpose of this study was to assess MPI using transthoracic Doppler echocardiography in normotensive obese and hypertensive obese pediatric patients and in a control group, and to investigate the relationship between MPI and LV mass, and LV systolic and diastolic functions. The study group consisted of 25 healthy cases (M/F: 13/12) as a control group (Group I), 25 normotensive-obese patients (M/F: 13/12) (Group II) and 25 hypertensive-obese patients (M/F: 14/11) (Group III). The mean ages were 12.1+/-1.8, 11.9+/-1.5 and 12.4+/-1.4 years, respectively. Cholesterol levels and body mass index (BMI) were higher in the hypertensive-obese group. The MPI values were 0.37+/-0.04, 0.43+/-0.08 and 0.51+/-0.11 in the three groups, respectively. MPI measurements, LV mass fractional shortening (FS), ejection fraction (EF) and mitral E/A ratio were found significantly different, especially in the hypertensive-obese group. These findings may be important to determine the relationship between obesity and cardiovascular risk factors in pediatric ages. MPI may be useful in determining the relationship between them.

Primary hyperparathyroidism is a life-threatening rare disorder. It is seen as a result of neonatal primary hyperparathyroidism, familial hypocalciuric hypercalcemia, increased vitamin D levels and inactivation of calcium sensing receptor mutations. The clinical findings are hypotonia, bone demineralization, hypercalcemia and parathyroid hyperplasia. We present a six-month-old female patient, the first child of nonconsanguineous parents, who was referred for the investigation of failure to thrive, vomiting, constipation, fever, abdominal distention and hypotonia. Physical examination revealed weight under 3rd percentile, height 3rd-10th percentile, decreased subcutaneous fat, and distention of the abdomen. In neurological examination, hypotonia, motor-mental retardation, and active deep tendon reflexes were found. The biochemical values at the time of admission revealed primary hyperparathyroidism. Since hypercalcemia did not respond to calcitonin therapy and due to the mortality of hypercalcemia, parathyroidectomy was performed. Because hyperparathyroidism and hypercalcemia continued, angiography was done which revealed increased parathyroid hormone levels in the periphery of the innominate vein. Exploratory surgery followed, but hyperparathyroidism and hypercalcemia persisted after all of these procedures. Calcium-sensing receptor mutations and supernumerary gland were considered. Because hypercalcemia persisted, pamidronate therapy was initiated on a monthly basis.

In diabetes mellitus (DM), increased fatty acids have negative effects on pancreatic beta-cell functions, in addition to enhanced mitochondrial transportation of fatty acids related to decreased insulin levels. The aim of this study was to evaluate lipid metabolism in children with DM by measuring plasma fatty acids and carnitine fractions to reveal relationships between carnitine status and increased fatty acid oxidation. Increased plasma fatty acids (except for arachidonic acid, there were no significant differences in the ratio of each specific fatty acid to total fatty acids), lipoprotein (a), acyl carnitine levels and urinary total and free acyl carnitine excretion, and decreased plasma free carnitine levels, were found in children with DM. There were no correlations between the duration of DM or HbA1c and study parameters. It is recommended that plasma free carnitine determinations should be made even if the patient has good metabolic control.