ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume : 3 Issue : 3 Year : 2024
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Turkish Society for Pediatric Endocrinology and Diabetes
JOURNAL OF CLINICAL RESEARCH IN PEDIATRIC ENDOCRINOLOGY - J Clin Res Pediatr Endocrinol: 3 (3)
Volume: 3  Issue: 3 - 2011
REVIEW
1.Current Concepts in Disorders of Sexual Development
Gönül Öçal
doi: 10.4274/jcrpe.v3i3.22  Pages 105 - 114
Disorders of sex development (DSD) with or without ambiguous genitalia require medical attention to reach a definite diagnosis. Advances in identification of molecular causes of abnormal sex, heightened awareness of ethical issues and this necessitated a re-evaluation of nomenclature. The term DSD was proposed for congenital conditions in which chromosomal, gonadal or anatomical sex is atypical. In general, factors influencing sex determination are transcriptional regulators, whereas factors important for sex differentiation are secreted hormones and their receptors.The current intense debate on the management of patients with intersexuality and related conditions focus on four major issues: 1) aetiological diagnosis, 2) assignment of gender, 3) indication for and timing of genital surgery, 4) the disclosure of medical information to the patient and his/her parents. The psychological and social implications of gender assignment require a multidisciplinary approach and a team which includes ageneticist, neonatologist, endocrinologist, gynaecologist, psychiatrist, surgeon and a social worker. Each patient should be evaluated individually by multidisciplinary approach.

ORIGINAL ARTICLE
2.Inflammatory Cytokine Profiles During Exercise in Obese, Diabetic, and Healthy Children
Pietro R. Galassetti, Jaime S. Rosa, Shirin Heydari, Stacy R. Oliver, Rebecca L. Flores, Andria M. Pontello, Milagros Ibardolaza
doi: 10.4274/jcrpe.v3i3.23  Pages 115 - 121
Objective: Modulation of inflammatory status is considered a key component of the overall health effects of exercise. This may be especially relevant in children with obesity (Ob) or type 1 diabetes (T1DM), in which an imbalance between pro- and anti-inflammatory mediators could accelerate onset and progression of cardiovascular complications. To date, exercise-induced alterations in immuno-modulatory mediators in Ob and T1DM children remain largely unknown.
Methods: In this study, we monitored the kinetic profiles of 8 pro-and anti-inflammatory cytokines (TNF-a, IL-6, IL-2, IL-8, IL-5, IL-13, IL-10, IL-4) during a standardized exercise challenge (ten 2-min cycling bouts at 80% VO2max, separated by 1-min intervals) in 23 Ob (12 females, 11 males), 23 T1DM (10 females and 13 males) patients and 20 healthy (CL, 10 females and 10 males) children. Blood glucose of T1DM patients was kept in the 4.4-6.1 mM range for at least 90 minute prior to and during exercise. Blood samples were drawn at rest and after every other exercise bout.
Results: In Ob, TNF-a and IL-2 were significantly greater (p<0.0167) as compared to T1DM and CL, both at baseline and throughout exercise. All other variables, while not significant, were quantitatively elevated in Ob vs. CL. In T1DM, IL-4 and IL-8 levels were similar to Ob, IL-2 and TNF-a similar to CL, and IL-6, IL-5, IL-13, IL-4 levels were intermediate between the Ob and CL groups.
Conclusions: During exercise, therefore, both Ob and T1DM children displayed exaggerated pro-inflammatory responses, although with clearly different magnitude and involved mediators. Our data support the necessity to identify specific exercise formats through which each at-risk pediatric population can draw maximal beneficial health effects.

3.Anogenital Distance in Turkish Newborns
Behzat Özkan, Belkıs Konak, Atilla Çayır, Murat Konak
doi: 10.4274/jcrpe.v3i3.24  Pages 122 - 125
Objective: Anogenital distances are considered to be a sensitive indicator of external genitalia exposure to factors such as anti- androgens, and/or endocrine distruptors during the prenatal period. Exposure to such factors can lead to changes in the anogenital measurements (AGM) of newborn infants. These measurements can be used to predict masculinization of the external genitalia in healthy newborns. The goal of this study was to determine normal values for AGM in Turkish newborns of both genders.
Methods: One hundred fifteen female and 135 male term newborns with no congenital defects were included in this study. A well-trained observer measured the anogenital distance by using a sliding Caliper graduated in millimeters. Anogenital distance was measured from the center of the anus to the posterior convergence of the fourchette in females and from the center of the anus to the junction of the smooth perineal skin with the base of the scrotum in males.
Results: Anogenital distance in males and females was 23±0.6 mm and 10.3±0.2 mm, respectively. There were significant differences in anogenital distance values between male and female newborns (p<0.05).
Conclusion: The findings of this study provide data that can be used as reference standards with regard to AGM of the posterior genital structures in Turkish male and female newborns. These data will also serve in postnatal evaluations to determine the effects of prenatal exposures to factors affecting development of genitalia.

4.The Distribution of Exon 3-Deleted/Full-Length Growth Hormone Receptor Polymorphism in the Turkish Population
Firdevs Baş, Fatih Keleşoğlu, Özlem Timirci, Sema Kabataş Eryılmaz, Nilüfer Bozkurt, Banu Küçükemre Aydın, Rüveyde Bundak, Turgay İsbir, Feyza Darendeliler
doi: 10.4274/jcrpe.v3i3.25  Pages 126 - 131
Objective: The exon 3-deleted/full-length (d3/fl) growth hormone receptor (d3/fl-GHR) polymorphism has been associated with responsiveness to GH therapy in some children and also with adult height variation in the general population. We aimed to evaluate the distribution of d3/fl-GHR polymorphism in a Turkish population.
Methods: The study included 477 (54 females/423 males) healthy adults with a mean±SD age of 31.1±9.0 years (range: 18-57). Height and body mass index (BMI) were expressed as standard deviation score (SDS) according to national standards. All adults had normal height and BMI SDSs (between -2 and +2). GHR exon 3 isoforms were studied by simple multiplex polymerase chain reaction method. Insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 (IGFBP-3) values were also measured and expressed as SDS.
Results: The distribution of the GHR exon 3 genotypes in the Turkish healthy adults was 35% (n=167) for fl/fl, 39% (n=186) for fl/d3, and 26% (n=124) for d3/d3. There was no difference between genders in GHR exon 3 genotypes. Frequencies of fl allele and d3 allele were 54.5% and 45.5%, respectively. There were no differences in height SDS and BMI SDS among the three d3/fl-GHR genotype groups. There was a significant difference in IGFBP-3 SDS between fl/fl and fl/d3 groups (p=0.022).
Conclusions: This study presents the results of GHR polymorphism in a Turkish population as a reference for further studies. The distribution was similiar to European populations. There were no correlations between GHR isoforms and height SDS or other clinical/biochemical characteristics of the individuals except for higher IGFBP-3 levels in the fl/d3 group as compared to the fl/fl group. Whether this finding implies an abnormality, needs further investigation.

5.The Relationship Between Metabolic Syndrome and Left Ventricular Mass Index in Obese Children
Mehmet Emre Atabek, Beray Selver Eklioğlu, Esra Akyüz, Derya Çimen
doi: 10.4274/jcrpe.v3i3.26  Pages 132 - 138
Objective: To investigate the relationships between metabolic syndrome (MS), other metabolic features and left ventricular mass index (LVMI) in a population of obese children and adolescents with MS.
Methods: Two hundred and eight obese children and adolescents (119 girls and 89 boys, mean age: 11.9±2.7 years) and control subjects (24 girls and 26 boys, mean age: 11.4±2.9 years) were enrolled in the study. The insulin sensitivity index and LVMI were determined. The International Diabetes Federation criteria were used to diagnose MS.
Results: The obese patients were divided into MS group (n = 55) and non-MS (n = 153) group. The values of LVMI in the MS group were significantly higher than those in the non-MS group (p = 0.014). The present LVMI cut-off point of 33g/m2 for the diagnosis of MS yielded a sensitivity of 97% and a specificity of 98%. LVMI was found to be positively correlated in univariate analysis with height, weight, body mass index (BMI)¬ SDS, fasting insulin level, homeostasis model assessment of insulin resistance (HOMA-IR) and fasting glucose insulin ratio (FGIR) and negatively correlated with quantitative insulin sensitivity check index (QUICK-I).
Conclusions: We suggest that our optimal LVMI cut-off value for identifying MS may be considered as a sensitive index in screening obese children and adolescents for pediatric MS. Assessment of LVMI in obese children and adolescents may be used as a tool in predicting the presence of MS and its associated cardiovascular risks.

6.Complementary and Alternative Medicine in Children with Type 1 Diabetes Mellitus
Belma Haliloğlu, Pınar İşgüven, Metin Yıldız, İlknur Arslanoğlu, Müferet Ergüven
doi: 10.4274/jcrpe.v3i3.27  Pages 139 - 143
Objective: Complementary and alternative medicine (CAM) isconditions. Studies on CAM in diabetes have mainly focused on the adult population and its application in children has not been well increasingly utilized in adults and children for treatment of various established. The aim of this study was to examine the prevalence and characteristics of CAM use in Turkish children with type 1 diabetes mellitus (T1DM).
Methods: The information was acquired by a questionnaire completed by a face-to-face interview with the parents of children with T1DM.
Results: A total of 195 subjects (mean age: 14.02±4.7 years; F/M: 103/92) were included in this survey. Use of CAM was reported in 85 subjects (43.6%). Herbal medicines were used in 64 subjects (75.3%). Sixty-nine subjects (81.2%) did not inform the diabetes specialist about CAM use. Thirty-eight subjects (44.7%) evaluated CAM as efficacious. Only 3 subjects (3.5%) interrupted the insulin injections to use CAM. No relationships were found between CAM use and parental education or insulin dose. There were significant correlations between CAM use and higher family income (p=0.027), urban residence (p=0.05), presence of complications (p=0.03), dissatisfaction with medical therapy (p=0.034) and prior CAM use among parents (p=0.001).
Conclusion: CAM use is a frequent practice among diabetic children, which is usually not shared with their physicians and sometimes leads to cessation of medical treatment.

7.Absence of Increase in Carotid Artery Intima-Media Thickness in Infants of Diabetic Mothers
Mehmet Emre Atabek, Havva Hasret Çağan, Beray Selver Eklioğlu, Bülent Oran
doi: 10.4274/jcrpe.v3i3.28  Pages 144 - 148
Objective: Infants of diabetic mothers (IDM) are considered as a risk group for atherosclerosis. Increased aortic intima-media thickness has been reported in IDM. The purpose of this study was to assess carotid artery intima-media thickness (CA-IMT), left ventricular mass index (LVMI) and atherosclerotic risk factors in IDM.
Methods: Thirty IDM and 25 healthy controls were included in the study. Of these infants, 14 were appropriate-for-gestational age (AGA) and 16 were large-for-gestational age (LGA). CA-IMT and LVMI were obtained by M-mode echocardiographic examination. The relationship between parameters of atherosclerosis and echocardiographic measurements was assessed by Pearson’s correlation analysis.
Results: LVMI was higher in LGA IDM when compared to AGA IDM and controls. CA-IMT was not significantly different between the groups and was also not related to atherosclerotic risk factors. Serum lipid and insulin levels were higher in LGA IDM when compared with AGA IDM and controls. There were no correlations between CA-IMT, LVMI and atherosclerotic risk factors.
Conclusions: In contrast to previous reports indicating an increase in CA-IMT in IDM, no differences were found between IDM and controls in this study. Our results indicate that macrosomic IDM are prone to hypertrophic cardiomyopathy but not to atherosclerotic changes in the blood vessels.

8.Efficiency of Fluid Treatments with Different Sodium Concentration in Children with Type 1 Diabetic Ketoacidosis
Şenay Savaş-Erdeve, Merih Berberoğlu, Pembe Oygar, Zeynep Şıklar, Tanıl Kendirli
doi: 10.4274/jcrpe.v3i3.29  Pages 149 - 153
Objective: The management of children with diabetic ketoacidosis (DKA) continues to be a controversial issue with regard to amount of intravenous fluid to be given, rate of delivery of fluid, and type of fluid to be used. We aimed to analyze the results obtained by administration of rehydration fluids of two different sodium (Na) concentrations (75 mEq/L vs. 100 mEq/L ) in the treatment of children with DKA.
Methods: Thirty-two children with DKA were assessed for efficacy and safety of fluid treatment. After an initial rehydration time, intravenous fluids were switched to a 5% dextrose solution with a Na content of 75 mEq/L (Group I, n=19) or 100 mEq/L (Group II, n=13). Venous blood samples were collected from all subjects at diagnosis and at the 4th, 8th, 16th and 24th hours of treatment.
Results: Changes in blood glucose levels did not differ significantly between the two groups at the 4th, 8th, 16th and 24th hours of the follow-up. Nadir effective plasma osmolality (Peff osm) and Peff osm levels also did not show statistically significant differences. Plasma sodium (PNa) level did not drop lower than the level at diagnosis in both groups. The changes in PNa concentrations in the two groups were not statistically significant at diagnosis or in follow-up samples (p=0.74). pH, anion gap, pCO2 and HCO3 levels were also similar in Group I and Group II. The duration of a pH level of <7.3 was shorter in Group II, but this was not statistically significant (p=0.65). None of the patients enrolled in this study developed cerebral edema.
Conclusion: The efficacy and safety of rehydration fluids with Na concentrations of 75 or 100 mEq/L did not reveal any differences in children with DKA.

CASE REPORT
9.3M Syndrome: A Report of Four Cases in Two Families
Ayla Güven, Ayşe Nurcan Cebeci
doi: 10.4274/jcrpe.v3i3.30  Pages 154 - 159
3M syndrome is a rare entity characterized by severe growth retardation, dysmorphic features and skeletal changes as its major components. It is differentiated from other types of dwarfism by its clinical features and by the typical slender long bones and foreshortened vertebral bodies that can be visualized radiographically. 3M syndrome has an autosomal recessive mode of inheritance. An early diagnosis is important for genetic counseling. In this report, we present four children (3 males, 1 female) from two families who were aged between 411/12 and 1011/12 years and had clinical findings of 3M syndrome. One of these patients had received growth hormone (GH) treatment which was discontinued due to an inadequate height gain. Physicians should be aware of this entity in the differential diagnosis of children with severe short stature and mild skeletal changes.

10.Fetal Adrenal Suppression Due to Maternal Corticosteroid Use: Case Report
Selim Kurtoğlu, Dilek Sarıcı, Mustafa Ali Akın, Ghaniya Daar, Levent Korkmaz, Şeyma Memur
doi: 10.4274/jcrpe.v3i3.31  Pages 160 - 162
During pregnancy, steroids are usually used in maternal diseases such as adrenal failure or other autoimmune diseases, e.g. idiopathic thrombocytopenic purpura (ITP), Crohn’s disease, systemic lupus erythematosus, dermatomyositis, scleroderma, Addison’s disease and hyperemesis gravidarum, HELLP syndrome. Endogenous or exogenous maternal steroids are metabolized by the placental enzyme 11 beta-hydroxy steroid dehydrogenase type 2. Prednisolone and methylprednisolone are highly sensitive to this enzyme, while dexamethasone and betamethasone are less well metabolized. Steroids which can cross the placental barrier are administered in cases like fetal lupus, congenital adrenal hyperplasia and for enhancement of fetal lung maturation, whereas steroids used in maternal diseases are usually the ones with low affinity to the placenta; however, in case of long-term use or in high doses, placental enzyme saturation occurs and thus, resulting in fetal adrenal suppression. Antenatal steroids can lead to low birth weight, as observed in our patient. Here, we report a case with fetal adrenal suppression due to maternal methylprednisolone use presenting with early hypoglycaemia and late hyponatremia in neonatal period and requiring three-month replacement therapy.

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