ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume : 5 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: 5 (3)
Volume: 5  Issue: 3 - 2013
ORIGINAL ARTICLE
1.Pubertal Gynecomastia Coincides with Peak Height Velocity
Yehuda Limony, Michael Friger, Ze’ev Hochberg
doi: 10.4274/Jcrpe.958  Pages 142 - 144
Objective: Pubertal gynecomastia (PG) occurs in up to 65% of adolescent boys. In this study, we investigated the relationship between the ages at which PG and peak height velocity occur in pubertal boys.
Methods: This was a prospective study that was designed to detect PG within three months of its emergence. We examined one hundred and six boys who were followed for short stature and/or delayed puberty at three month intervals, and gynecomastia was observed in 43 of these boys (40.5%).
Results: PG occurred in the 43 boys within a year of their peak height velocity, and most of these boys were at Tanner stage 3 for pubic hair and had testicular volumes between 8-10 mL.
Conclusion: It is recommended that evaluation of height growth be included in the diagnostic approach to PG in boys with short stature and/or delayed puberty. The coincidence of age of peak height velocity and PG suggests a causal relationship between the two events and a role of insulin-like growth factor-1.

2.Microvascular Complications in Adolescents with Type 1 Diabetes Mellitus
Fatma Demirel, Derya Tepe, Özlem Kara, İhsan Esen
doi: 10.4274/Jcrpe.994  Pages 145 - 149
Objective: Screening of complications is an important part of diabetes care. The aim of this study was to investigate diabetic complications and related risk factors in adolescents with type 1 diabetes mellitus (T1DM).
Methods: This cross-sectional study was conducted on type 1 diabetics who were over 11 years of age or had a diabetes duration of 2 years and included 155 adolescents with T1DM (67 male, 88 female). The mean age of the patients was 14.4±2.1 years. Mean diabetes duration was 6.3±2.9 years. The patients were screened for diabetic nephropathy, retinopathy and peripheral neuropathy.
Results: Mean glycosylated hemoglobin (HbA1c) level of the study group was 8.4%. The frequency of microalbuminuria and peripheral neuropathy were 16.1% and 0.6%, respectively. None of the patients had diabetic retinopathy. Dyslipidemia and hypertension rates were 30.3% and 12.3%, respectively. Risk factors associated with microalbuminuria were hypertension, higher HbA1c levels, longer diabetes duration and dyslipidemia.
Conclusion: Early diagnosis and treatment of hypertension and dyslipidemia as well as achieving a better metabolic control are important in prevention or postponement of complications in patients with T1DM. Yearly screening for diabetic nephropathy should be started 2 years after the onset of the diabetes.

3.Hyperinsulinemic Hypoglycemia: Experience in A Series of 17 Cases
Sebahat Yılmaz Ağladıoğlu, Şenay Savaş Erdeve, Semra Çetinkaya, Veysel Nijat Baş, Havva Nur Peltek Kendirci, Aşan Önder, Zehra Aycan
doi: 10.4274/Jcrpe.991  Pages 150 - 155
Objective: Hyperinsulinemic hypoglycemia (HIH) is a genetically heterogeneous disorder with both familial and sporadic variants. Patients with HIH may present during the neonatal period, infancy, or childhood and may show transient, prolonged, and persistent features. In this study, we aimed to discuss our experience with HIH patients, based on a series of 17 patients.
Methods: We retrospectively analyzed the clinical and laboratory characteristics at the time of diagnosis and during treatment and evaluated the neurodevelopmental outcomes during follow-up in 17 HIH patients, who presented or were referred to the Pediatric Endocrinology Clinic of Dr. Sami Ulus Training and Research Children’s Hospital between 1998 and 2011. The patients (7 male, 10 female) were aged between the first day of life and 7 years - 10 were in their first week of life, 6 in their infancy, and 1 in childhood.
Results: None of the mothers had gestational diabetes. Hypoglycemic seizure (76.5%) was the most common presenting symptom. Medical treatment failed in two patients, and was stopped in eight patients. Of two diazoxide-unresponsive patients, one underwent near-total pancreatectomy, but hypoglycaemic episodes continued after surgery. The parents of other patient refused surgery, the medical treatment was continued, nevertheless, severe motor and mental retardation developed. At follow-up, 23.5% of the patients were found to have mild or moderate psychomotor retardation, and 23.5% developed epilepsy. There was no marked difference in neurological results between cases with onset in the neonatal period or in infancy.
Conclusions: Clinical course and treatment response in HIH cases are very heterogeneous. Long-term careful monitoring is needed to detect and treat the complications.

4.Relationship of Epicardial Adipose Tissue Thickness with Early Indicators of Atherosclerosis and Cardiac Functional Changes in Obese Adolescents with Metabolic Syndrome
Bedir Akyol, Mehmet Boyraz, Cevriye Aysoy
doi: 10.4274/Jcrpe.1064  Pages 156 - 163
Objective: Epicardial adipose tissue thickness (EATT) is suggested as a new cardiometabolic risk factor. Carotid intima-media thickness (IMT) is a potential indicator of subclinical atherosclerosis in patients with metabolic syndrome (MS). We investigated the association of EATT with carotid IMT and cardiac functional changes in obese adolescents with MS.
Methods: One hundred thirty-eight obese adolescents and 63 lean subjects were enrolled in the study. The obese subjects were divided into two subgroups based on the presence or absence of MS (MS group and non-MS group). All subjects underwent transthoracic echocardiographic examination for determination of left ventricular (LV) function, LV mass index (LVMI), and myocardial performance index (MPI). EATT and carotid IMT were also measured during echocardiography.
Results: The average LVMI measurements were higher in both MS and non-MS obese patients in comparison with the lean children. The MS group had significantly higher LVMI measurements than the non-MS and lean groups (88.5±23.0, 67.5±24.8 g/m2, and 62.4±18.2 g/m2, respectively; p<0.01). Carotid IMT was higher in both the MS and non-MS obese patients in comparison with the lean group. The MS group had significantly higher carotid IMT measurements than the non-MS and lean groups (0.91±0.23, 0.78±0.18, and 0.52±0.08 mm, respectively; p<0.01). The EATT was also increased significantly in patients with MS compared to lean adolescents (7.42±1.55 vs. 4.28±0.79mm; p=0.001). EATT was positively correlated with body mass index-SDS, waist circumference, fasting glucose, insulin, homeostasis model assessment-insulin resistance, triglyceride levels, LV thickness, LVMI, and MPI in the MS obese group. EATT was the only independent predictor of carotid IMT in the multivariate analysis (ß= 0.69, p<0.001).
Conclusion: The findings of the present study demonstrate a close relationship of EATT with carotid IMT and early cardiac dysfunction in obese adolescents with MS. Assessment of EATT and carotid IMT in routine echocardiographic examinations is suggested as a feasible and reliable method for the evaluation of obesity with MS and its related cardiovascular risks in children and adolescents.

5.Effects of ACE Inhibitors on Insulin Resistance and Lipid Profile in Children with Metabolic Syndrome
Eda Çelebi Bitkin, Mehmet Boyraz, Necati Taşkın, Arzu Akçay, Korkut Ulucan, Mehmet Bedir Akyol, Teoman Akçay
doi: 10.4274/Jcrpe.1020  Pages 164 - 169
Objective: The aim of this study was to evaluate the effects of using ACE inhibitors on insulin resistance, glucose metabolism, body fat composition, and lipid profile in children over 10 years of age with obesity-associated metabolic syndrome (MS).
Methods: A total of 53 children with MS, who had been followed for at least one year were included in the study. The sample was divided into two groups: Group 1-30 obese children (13 female, 17 male) who were not using an ACE inhibitor and Group 2-23 obese children (13 female, 10 male) who were using an ACE inhibitor. Anthropometric and laboratory data obtained at baseline and at the 3rd, 6th, and 12th months of follow-up were compared in the two groups.
Results: Comparison of the data in the two groups at 3rd, 6th, and 12th months revealed no statistically significant differences in terms of weight standard deviation score (SDS), body mass index SDS, weight for height percentile, body fat percentage, and very low-density lipoprotein (VLDL)values. However, there were statistically significant differences in mean glucose and insulin levels, homeostasis model assessment for insulin resistance, LDL and high-density lipoprotein values, and highly significant differences in mean triglyceride values.
Conclusions: The positive effects of ACE inhibitor drugs, particularly on hypertriglyceridemia and insulin resistance, might bring them forth as first-line drugs in the treatment of obese and hypertensive children. Randomized, controlled, double-blind, and long-term studies are needed for a definitive conclusion.

6.The Role of Initial Clinical and Laboratory Findingsin Infants With Hyperthyrotropinemia to PredictTransient or Permanent Hypothyroidism
Tolga Ünüvar, Korcan Demir, Ayhan Abacı, Atilla Büyükgebiz, Ece Böber
doi: 10.4274/Jcrpe.931  Pages 170 - 173
Objective: Studies on the clinical course of children with hyperthyrotropinemia are scarce. We aimed to evaluate the role of presentation findings in such infants to predict eventual outcome.
Methods: Files of infants diagnosed as suspicious congenital hypothyroidism (CH) in the neonatal or early infancy period in the past ten years were analyzed retrospectively, and 37 patients (M/F: 20/17) with hyperthyrotropinemia diagnosed at a median age of 3.2 months were included in the study. Criteria for inclusion were: normal free thyroxine (fT4) levels and thyrotropin (TSH) levels between 10-20 µIU/mL during the initial neonatal screening (or TSH<10 µIU/mL afterwards). Cases with permanent CH (Group 1) were compared to those with transient hyperthyrotropinemia (Group 2) regarding age at the time of diagnosis, sex, gestational age, birth weight, symptoms, ultrasonographic and scintigraphic findings, initial thyroid function tests, and state of mental and motor development.
Results: Of the total group, 20 patients (54%) were eventually diagnosed as permanent CH. T4 doses that maintained normal thyroid function tests were significantly higher at the end of the first and second years of life in this group. Age, TSH and fT4 levels at the time of diagnosis, sex, gestational age, birth weight, symptoms, ultrasonographic and scintigraphic findings, and the state of mental and motor development were similar in the two groups.
Conclusions: T4 dose required to maintain a euthyroid state was the only parameter which distinguished between transient and permanent CH.

7.Serum Adiponectin and hsCRP Levels and Non-InvasiveRadiological Methods in the Early Diagnosis ofCardiovascular System Complications in Children andAdolescents with Type 1 Diabetes Mellitus
Damla Gökşen, Ertürk Levent, Sakine Kar, Samim Özen, Şükran Darcan
doi: 10.4274/Jcrpe.1003  Pages 174 - 181
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.

8.Relationship Between Aspartate Aminotransferase-to-Platelet Ratio Index and Carotid Intima-Media Thickness in Obese Adolescents with Non-Alcoholic Fatty Liver Disease
Ahmet Sert, Özgür Pirgon, Ebru Aypar, Hakan Yılmaz, Bumin Dündar
doi: 10.4274/Jcrpe.891  Pages 182 - 188
Objective: There is increasing evidence for an association between non-alcoholic fatty liver disease (NAFLD) and an increased risk of cardiovascular morbidity and mortality. The aim of this study was to investigate the association between aspartate aminotransferase-to-platelet ratio index (APRI) and carotid intima-media thickness (IMT) in obese adolescents with NAFLD.
Methods: Seventy-six obese adolescents and 36 lean subjects were enrolled in this cross-sectional single-centre study. The obese subjects were divided into two subgroups based on the presence or absence of fatty liver with high transaminase levels (NAFLD group and non-NAFLD group). Fasting blood samples were assayed for transaminase, glucose, and insulin levels. Insulin resistance was calculated by the homeostasis model assessment (HOMA-IR).
Results: APRI values were higher in both obese groups (NAFLD and non-NAFLD) in comparison with the lean group. The NAFLD group had significantly higher APRI values than the non-NAFLD obese group and the lean group. Carotid IMT was higher in both obese groups (NAFLD and non-NAFLD) in comparison with the lean group. The NAFLD group had significantly higher measurements of carotid IMT than the non-NAFLD group and the lean group. APRI was positively correlated with most of the metabolic parameters (total cholesterol, low-density lipoprotein cholesterol, glucose, insulin, HOMA-IR) and with carotid IMT in the NAFLD obese group.
Conclusions: This study demonstrated that a significant relationship exists between APRI and carotid IMT in obese adolescents with NAFLD. We suggest that an increased APRI score in obese adolescents with NAFLD can possibly serve to predict a more adverse cardiovascular risk profile.

9.Increased Resistin Serum Concentrations in Patients with Type 1 Diabetes Mellitus
İclal Geyikli, Mehmet Keskin, Yılmaz Kör, Müslüm Akan
doi: 10.4274/Jcrpe.1072  Pages 189 - 193
Objective: Adiponectin, leptin, and resistin are adipokines which play a significant role in the regulation of lipid and carbohydrate metabolism in patients with type 2 diabetes, while little is known about their role in type 1 diabetes mellitus (T1DM). The aim of this study was to measure serum adiponectin, leptin, and resistin levels and to investigate their relationships with some parameters in patients with T1DM and healthy controls.
Methods: Fifty children and adolescents with T1DM (21 boys and 29 girls) and 33 healthy control subjects (18 boys and 15 girls) participated in the study. All subjects were patients followed in the Pediatric Endocrinology and Metabolism Unit of Gaziantep University Faculty of Medicine. None of the subjects had hypertension, obesity, hyperlipidemia, anemia, or infection. Adiponectin, leptin, and resistin levels were analyzed with ELISA.
Results: There were no statistically significant differences related with age, sex, pubertal status, or body mass index distribution between the diabetic and control groups. Resistin levels were significantly higher in the diabetic group compared to controls (5.26±3.15 ng/mL vs. 3.50±1.26 ng/mL; p<0.01).
Conclusion: Of the three investigated adipokines, only resistin was associated with T1DM. Resistin may play a role in the process of inflammation and also in the pathophysiology of T1DM.

CASE REPORT
10.Remission with Cabergoline in Adolescent Boys with Cushing’s Disease
Ayla Güven, Feyyaz Baltacıoğlu, Fatma Dursun, Ayşe Nurcan Cebeci, Heves Kırmızıbekmez
doi: 10.4274/Jcrpe.1007  Pages 194 - 198
Cabergoline is a long-acting dopamine receptor agonist used for treatment of patients with uncured Cushing’s disease (CD) and, as a first-line treatment, was used in only limited numbers of patients. This report presents two adolescent boys with CD who were treated with cabergoline. Two adolescent boys with clinical and laboratory findings of CD are presented. No pituitary adenoma was detected by radiological investigation in either patient. Adrenocorticotropic hormone (ACTH) hypersecretion and lateralization was found by inferior petrosal sinus sampling in both patients. The initial cabergoline dose was 1mg/week and was adjusted up to 1.5 mg/week in the second patient, based on his urinary free cortisol (UFC) level. The patients responded to cabergoline treatment with normal UFC levels on the 4th and 6th months of treatment. The boys reached complete remission at the end of the 17th and 24th months, respectively. Cabergoline is effective in the control of cortisol secretion and can be considered as a first-line treatment in cases of CD.

11.Novel Mutation in the SLC19A2 Gene in an Iranian Family with Thiamine-Responsive Megaloblastic Anemia: A Series of Three Cases
Nosrat Ghaemi, Martha Ghahraman, Mohammad Reza Abbaszadegan, Alireza Baradaran-Heravi, Rahim Vakili
doi: 10.4274/Jcrpe.969  Pages 199 - 201
Thiamine-responsive megaloblastic anemia (TRMA) is a clinical triad characterized by megaloblastic anemia, non-autoimmune diabetes mellitus, and sensory-neural hearing loss. Mutations in the thiamine transporter gene, solute carrier family 19, member 2 (SLC19A2), have been associated with TRMA. Three pediatric patients from a large consanguineous Iranian family with hyperglycemia, anemia, and hearing loss were clinically diagnosed with TRMA. In all three patients, TRMA was confirmed by direct sequencing of the SLC19A2 gene that revealed a novel missense homozygous mutation c.382 G>A (p.E128K). This mutation results in the substitution of glutamic acid to lysine at position 128 in exon 2 and was not detected in 200 control chromosomes. Thiamine therapy reversed the anemia and alleviated the hyperglycemia in all three patients. We recommend sequence analysis of the SLC19A2 gene in individuals with a clinical triad of diabetes mellitus, hearing loss, and anemia. The administration of thiamine ameliorates the megaloblastic anemia and the hyperglycemia in patients with TRMA.

12.Long-Term Follow-Up of Cushing’s Disease: A Case Report
Veysel Nijat Baş, Sebahat Yılmaz Ağladıoğlu, Aşan Önder, Pınar Özışık, Havva Nur Peltek Kendirci, Semra Çetinkaya, Zehra Aycan
doi: 10.4274/Jcrpe.993  Pages 202 - 205
Cushing’s disease is a condition in which hypercortisolism develops due to excessive hypophyseal adrenocorticotropic hormone production. It is rare in childhood. In this paper, we report the case of a 10-year-old male patient with hypophyseal microadenoma-related Cushing’s disease who presented with obesity and was found to show poor height growth at follow-up. The diagnosis was confirmed with inferior petrosal sinus sampling, and the adenoma was successfully removed by transsphenoidal surgery. While adrenal axis suppression continued for approximately 1 year, clinical improvement was clearly observed after the third month following surgery. The findings in this patient demonstrate that decreased growth rate despite rapid weight gain in children can be early sign of Cushing’s disease and emphasize the importance of monitoring of growth in obese children.

13.Neonatal Seizure as a Manifestation of Unrecognized Maternal Hyperparathyroidism
Hüseyin Anıl Korkmaz, Behzat Özkan, Demet Terek, Ceyhun Dizdarer, Sertaç Arslanoğlu
doi: 10.4274/Jcrpe.1037  Pages 206 - 208
Maternal hypercalcemia suppresses parathyroid activity in the fetus resulting in impaired parathyroid responsiveness to hypocalcemia after birth. Resultant hypocalcemia may be severe and prolonged and rarely may lead to convulsions. Here, we present a newborn infant admitted to the pediatric emergency department at age two weeks with recurrent tonic convulsions due to asymptomatic maternal hyperparathyroidism and vitamin D deficiency. Physicians should be aware that undiagnosed maternal hyperparathyroidism can cause severe hypocalcemia in the newborn.

14.Multiple Pituitary Hormone Deficiency Due to Gunshot Injury in a 6-Year-Old Girl
Hüseyin Demirbilek, Mehmet Nuri Özbek, Rıza Taner Baran, Ahmet Baran
doi: 10.4274/Jcrpe.1027  Pages 209 - 211
Gunshot injuries (GSI) of the cranial area have an extremely high mortality rate. Herein, we present a girl who has been living with a bullet in the posterior sellar region. A 6-year-old girl was admitted with complaints of headache, polyuria and polydypsia, which started after a GSI. At the time of admission the patient’s anthropometric, physical and neurological examinations were normal. Urine output was 5.5 L/m2/24h. A water deprivation test suggested central diabetes insipidus, which responded to treatment. Evaluation of other pituitary hormones revealed central hypothyroidism and growth hormone deficiency. Pituitary hormone deficiency must be kept in mind in patients injured by a gunshot to the sellar/parasellar region.

15.Medical Management of Thyroid Ectopia: Report of Three Cases
Deep Dutta, Manoj Kumar, Anubhav Thukral, Dibakar Biswas, Rajesh Jain, Sujoy Ghosh, Satinath Mukhopadhyay, Subhankar Chowdhury
doi: 10.4274/Jcrpe.1103  Pages 212 - 215
Thyroid ectopia (TE) is an embryological aberration of the thyroid gland migration most commonly observed in the lingual region followed by the sublingual, hyoid, and mediastinal regions. TE is often complicated by local compressive symptoms resulting in dysphagia, dysphonia, and dyspnea. Surgical removal of TE is frequently complicated by difficulties in intubation, increased perioperative bleeding, and severe primary hypothyroidism; on the other hand, I131 ablation is limited by high doses needed and the concern for long-term effects especially in children. We report three children with TE who all presented with compressive symptoms and were managed conservatively with levothyroxine resulting in resolution of compressive symptoms and favorable outcomes. Levothyroxine supplementation is effective and has an important role in managing TE, not only in correcting the associated hypothyroidism but also in resolving the associated compressive symptoms by reducing the size of the ectopic thyroid tissue.

OTHER
16.ERRATUM

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