ISSN: 1308-5727 | E-ISSN: 1308-5735
Volume : 6 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: 6 (3)
Volume: 6  Issue: 3 - 2014
1.Cover

Page I

REVIEW
2.Overweight and Obesity in Children and Adolescents
Neslihan Koyuncuoğlu Güngör
doi: 10.4274/jcrpe.1471  Pages 129 - 143
Obesity among children, adolescents and adults has emerged as one of the most serious public health concerns in the 21st century. The worldwide prevalence of childhood obesity has increased remarkably over the past 3 decades. The growing prevalence of childhood obesity has also led to appearance of obesity-related comorbid disease entities at an early age. Childhood obesity can adversely affect nearly every organ system and often causes serious consequences, including hypertension, dyslipidemia, insulin resistance, dysglycemia, fatty liver disease and psychosocial complications. It is also a major contributor to increasing healthcare expenditures. For all these reasons, it is important to prevent childhood obesity as well as to identify overweight and obese children at an early stage so they can begin treatment and attain and maintain a healthy weight. At present, pharmacotherapy options for treatment of pediatric obesity are very limited. Therefore, establishing a comprehensive management program that emphasizes appropriate nutrition, exercise and behavioral modification is crucial. The physician’s role should expand beyond the clinical setting to the community to serve as a role model and to advocate for prevention and early treatment of obesity.

ORIGINAL ARTICLE
3.Thyroid Abnormalities in Survivors of Childhood Cancer
Ayla Akça Çağlar, Aynur Oğuz, Faruk Güçlü Pınarlı, Ceyda Karadeniz, Arzu Okur, Aysun Bideci, Ülker Koçak, Hüseyin Bora
doi: 10.4274/jcrpe.1326  Pages 144 - 151
Objective: To investigate the late side effects of childhood cancer therapy on the thyroid gland and to determine the risk factors for development of thyroid disorder among childhood cancer survivors.
Methods: One hundred and twenty relapse-free survivors of childhood cancer (aged 6-30 years) were included in this study. The diagnoses of patients were lymphoma, leukemia, brain tumor, rhabdomyosarcoma and nasopharyngeal carcinoma (NPC). The patients were divided into two groups depending on the treatment: group 1-chemotherapy (ChT) only (n=52) and group 2-combination therapy of ChT + radiotherapy (RT) (head/neck/thorax) (n=68). Thyroid function tests, urinary iodine levels, and thyroid gland ultrasound examinations were evaluated in both groups.
Results: Incidence of thyroid disease was 66% (n=79) in the survivors. The thyroid abnormalities were: hypothyroidism (HT) (n=32, 27%), thyroid nodules (n=27, 22%), thyroid parenchymal heterogeneity (n=40, 33%), autoimmune thyroiditis (n=36, 30%), and thyroid malignancy (n=3, 2%). While the incidence of HT and thyroid nodules in group 2 was significantly higher than in group 1, the incidence of thyroid parenchymal heterogeneity and autoimmune thyroiditis was similar in the two patient groups. HT and thyroid malignancy were seen only in group 2. In multivariate logistic regression analysis, a history of Hodgkin lymphoma (HL), brain tumor and NPC, as well as cervical irradiation and 5000-5999 cGy doses of radiation were found to constitute risk factors for HT. History of HL and 4000-5999 cGy doses of radiation were risk factors for thyroid nodules. Head/neck irradiation and treatment with platinum derivatives were risk factors for autoimmune thyroiditis. In univariate analysis, a history of NPC, cervical + nasopharyngeal irradiation, and treatment with platinum derivatives were risk factors for thyroid parenchymal heterogeneity.
Conclusion: Our results indicate that there is especially an increased risk of HT and thyroid nodules in patients treated with combination therapy of ChT with head/neck/thorax RT. Although chemotherapeutic agents per se do not seem to cause HT, longer follow-up is needed to assess whether or not there is an increased risk for autoimmune thyroiditis and thyroid parenchymal heterogeneity after antineoplastic therapy.

4.Thyroid Function in Obese Children with Non-Alcoholic Fatty Liver Disease
Hüseyin Bilgin, Özgür Pirgon
doi: 10.4274/jcrpe.1488  Pages 152 - 157
Objective: To investigate the relationships between thyroid function and metabolic risk factors in obese adolescents with non-alcoholic fatty liver disease (NAFLD).
Methods: One hundred sixty obese adolescents and 40 control subjects were enrolled in the study. The obese subjects were divided into two groups based on presence or absence of liver steatosis (NAFLD group and non-NAFLD group). Serum samples were assayed for glucose, insulin, cholesterol, alanine aminotransferase, aspartate aminotransferase, free thyroxine (fT4), free triiodothyronine (fT3) and thyroid-stimulating hormone (TSH). The ratio of fT3 to fT4 was evaluated as an indirect index of deiodinase activity. Insulin resistance was evaluated by homeostasis model assessment (HOMA-IR) from fasting samples.
Results: NAFLD and non-NAFLD groups had slightly higher fasting blood glucose values than the control group. Fasting insulin levels in the NAFLD group were significantly higher than those in the non-NAFLD and control groups. The NAFLD group had significantly greater HOMA-IR values compared with the non-NAFLD group and also with the control group. The NAFLD group had significantly higher fT3/fT4 ratio values compared to both non-NAFLD and control groups. fT3/fT4 was positively correlated with serum insulin levels in the NAFLD group.
Conclusion: This study showed that obese adolescents with hepatosteatosis had elevated values for fT3/fT4 ratio. This finding suggested a high conversion of T4 to T3 due to increased deiodinase activity as a compensatory mechanism for fat accumulation.

5.Intraoperative Parathyroid Hormone Monitoring Corroborates the Success of Parathyroidectomy in Children
Ahmet Çelik, Emre Divarcı, Zafer Dökümcü, Orkan Ergün, Samim Özen, Damla Gökşen, Şükran Darcan, Yeşim Ertan
doi: 10.4274/jcrpe.1401  Pages 158 - 162
Objective: To assess the efficacy of intraoperative parathyroid hormone (PTH) monitoring in evaluating the outcome of parathyroidectomy in pediatric patients.
Methods: Intraoperative PTH monitoring during parathyroidectomy was performed in five children (3M, 2F); three had parathyroid adenomas (single gland disease) and two had primary hyperplasia. One patient had undergone two previous surgical interventions to remove the parathyroid glands, but the PTH levels had remained high with persistence of symptoms. Immunoradiometric analysis was used for PTH measurements. Preoperative PTH values were obtained to monitor the baseline levels. Serum samples were collected 20 minutes after removal of the adenoma/parathyroid gland(s) and PTH levels were compared with preoperative values. Specimens were also confirmed by frozen sectional examination.
Results: Mean age of the patients was 11 years (range: 3 months-16 years). Mean preoperative PTH values were 633.3±579 pg/mL (range: 143-1300 pg/mL). Intraoperative values decreased to 18.7±5.5 pg/mL (range: 8-27 pg/mL) following removal of the gland(s). Normal calcium levels were achieved with adequate management following surgery. One patient (with multiple surgeries and found to have an ectopic parathyroid gland) had hungry bone syndrome after the operation and was treated successfully. There were no major complications. All patients maintained normal calcium/phosphorus levels in the follow-up period, ranging from 2 to 5 years.
Conclusion: An ectopic parathyroid gland or another undetected adenoma can be overlooked during surgery. Owing to the short life of the hormone, intraoperative PTH monitoring to determine PTH clearance proved to be a feasible marker for adequacy and safety of surgery and “cure”.

6.Serum Paraoxonase/Arylesterase Activity and Oxidative Stress Status in Children with Metabolic Syndrome
Erdal Eren, Mahmut Abuhandan, Abdullah Solmaz, Abdullah Taşkın
doi: 10.4274/jcrpe.1454  Pages 163 - 168
Objective: This study aimed to measure paraoxonase/arylesterase activities and to evaluate the total oxidant and antioxidant capacities in obese children and in children with metabolic syndrome (MetS).
Methods: A total of 151 children of comparable ages (13.23±1.96 years, 13.45±1.85 years and 13.95±1.31 years) were enrolled in the study. Forty of these were children with MetS, 55 were obese and 56 were healthy controls. Diagnosis of the MetS was made according to International Diabetes Federation criteria. Paraoxonase/arylesterase activities were evaluated by using paraoxon and phenylacetate substrates. Total oxidant status (TOS) and total antioxidant status (TAS) were measured and oxidative stress index (OSI) was estimated by calculation.
Results: High levels of paraoxonase were detected in the obese group, whereas high levels of arylesterase were detected in both MetS and obese groups. Higher values for TOS, TAS and OSI were found in the MetS group (p<0.05).
Conclusion: Higher values of mean TOS and OSI in the MetS group than those in the control groups indicate that these parameters may be indicators of future risks such as atherosclerosis in patients with MetS.

7.One Base Deletion (c.2422delT) in the TPO Gene Causes Severe Congenital Hypothyroidism
Hakan Cangül, Murat Doğan, Yaman Sağlam, Michaela Kendall, Kristien Boelaert, Timothy G Barrett, Eamonn R Maher
doi: 10.4274/jcrpe.1404  Pages 169 - 173
Objective: Congenital hypothyroidism (CH) is the most common neonatal endocrine disorder and mutations in the TPO gene have been reported to cause CH. Our aim in this study was to determine the genetic basis of CH in two affected individuals coming from a consanguineous family.
Methods: Since CH is usually inherited in autosomal recessive manner in consanguineous/multi-case families, we adopted a two-stage strategy of genetic linkage studies and targeted sequencing of the candidate genes. First, we investigated the potential genetic linkage of the family to any known CH locus using microsatellite markers and then screened for mutations in linked-gene by Sanger sequencing.
Results: The family showed potential linkage to the TPO gene and we detected a deletion (c.2422delT) in both cases. The mutation segregated with disease status in the family.
Conclusion: This study demonstrates that a single base deletion in the carboxyl-terminal coding region of the TPO gene could cause CH and helps to establish a genotype/phenotype correlation associated with the mutation. The study also highlights the importance of molecular genetic studies in the definitive diagnosis and accurate classification of CH.

CASE REPORT
8.Extensive Middle Cranial Fossa Arachnoid Cysts and Different Clinical Presentation in Two Patients
Özge Yüce, Esra Döğer, Nurullah Çelik, Hamdi Cihan Emeksiz, Erkut Baha Bulduk, Mahmut Orhun Çamurdan, Aysun Bideci, Peyami Cinaz
doi: 10.4274/jcrpe.1381  Pages 174 - 176
Arachnoid cysts (ACs), particularly suprasellar cysts, cause a wide spectrum of endocrine disorders. Herein, we report two patients diagnosed with an extensive AC in the middle cranial fossa while being investigated for etiologies of precocious puberty and short stature. One of them required surgery due to his pubertal disorders associated with compression effects of cyst. After surgery, his puberty progression was regressed within one year. On the other hand, surgery was not planned for the second patient considering of his cranial imaging findings and the extremely low incidence of growth hormone (GH) deficiency caused by middle fossa AC (MFAC). We started treatment with recombinant human GH and no complication was found during treatment follow-up. Endocrine disorders associated with MFACs are extremely rare. By presenting with these two cases, we aimed to remain our fellow physcians that ACs can be possibly cause of endocrine disorders. Clinicians should be careful evaluating endocrine disorders because real cause may not be cyst itself but masked by it.

9.Unusual Course of Congenital Hypothyroidism and Route of the L-Thyroxine Treatment in a Preterm Newborn
Levent Korkmaz, Mustafa Ali Akın, Tamer Güneş, Ghaniya Daar, Osman Baştuğ, Ali Yıkılmaz, Selim Kurtoğlu
doi: 10.4274/jcrpe.1383  Pages 177 - 179
Congenital hypothyroidism (CH) is the most common endocrine pathology in neonates. Inappropriate treatment of CH is complicated by irreversible brain damage or low IQ score. Hormone replacement therapy with L-thyroxine (L-T4) is sufficient for a very large proportion of patients. However, during treatment, the patient needs to be carefully monitored for presence of factors which might affect the absorption or bio-availability of the drug as well as its dose. Herein, we report a preterm newborn with CH who presented with gastrointestinal problems mimicking necrotizing enterocolitis. The clinical course was also complicated by cholestasis. The L-T4 replacement treatment was switched from oral route to parenteral. After resolution of the cholestasis, L-T4 treatment was continued successfully by the oral route.

10.Transverse Testicular Ectopia: A Rare Presentation with Persistent Müllerian Duct Syndrome
Onur Telli, Mehmet İlker Gökçe, Perviz Hacıyev, Tarkan Soygür, Berk Burgu
doi: 10.4274/jcrpe.1479  Pages 180 - 182
Undescended testes can be found in the inguinal channel or in the abdomen. Rarely, undescended testes can present with transverse testicular ectopia (TTE) and very rarely, with residual Müllerian duct (MD) structures. This latter presentation is called persistent MD syndrome (PMDS). PMDS is mostly discovered during surgery for inguinal hernia or cryptorchidism. TTE is a rare congenital anomaly in which both testes descend through a single inguinal canal. Patients with TTE present with symptoms of unilateral cryptorchidism and contralateral inguinal hernia. Herein, we report two TTE cases: one associated with PMDS and the other having only cross ectopia. For patients with inguinal hernia and cryptorchidism associated with TTE, PMDS should be kept in mind and radiologic evaluation with ultrasonography or magnetic resonance imaging of the genitourinary system and karyotyping are recommended. Radiologic evaluation can be helpful in the diagnosis of TTE; however, it cannot diagnose the malignancy itself. Laparoscopy is very useful for both diagnosis and treatment of TTE.

11.A Case of Central Precocious Puberty Due to Concomitant Hypothalamic Hamartoma and Juvenile Pilocytic Astrocytoma
Gönül Çatlı, Ayhan Abacı, Ahmet Anık, Handan Güleryüz, Erdener Özer, İrfan Öcal, Nurullah Yüceer, Kamer Mutafoğlu
doi: 10.4274/jcrpe.1306  Pages 183 - 186
Central precocious puberty (CPP) is caused by premature activation of the hypothalamo-pituitary-gonadal axis. More than 50% of boys with CPP have an identifiable etiology. Hypothalamic hamartoma (HH), hydrocephalus, tumors, infections, congenital defects, ischemia, radiation, or injury of the brain are the most common causes of secondary CPP. In this report, we present the case of a 2 years and 9 months old male patient who had a 30x40 mm contrast-enhancing suprasellar mass and was histopathologically diagnosed with giant HH. However, since HHs are designated as non-enhancing masses, considering the possibility of an incomplete diagnosis of a glial tumor, the patient was followed up. Clinical and radiological follow-up revealed stable findings with no evidence of tumor growth until the third year after surgery when he presented with neurological deficit due to the rapid growth of the suprasellar mass. After the second surgery, histopathological examination of the biopsy specimen revealed the lesion to be a juvenile pilocytic astrocytoma (PA). The concomitance of HH and juvenile PA is very rare. To our knowledge, this is the first report of a patient with concomitant juvenile PA and HH who developed CPP and did not have gelastic epilepsy despite the rapidly growing giant mass.

12.An Unusual Combination of Klinefelter Syndrome and Growth Hormone Deficiency in a Prepubertal Child
Jayanthy Ramesh, Mudiganti Nagasatyavani, Javvadii Venkateswarlu, Jakka Nagender
doi: 10.4274/jcrpe.1225  Pages 187 - 189
Klinefelter syndrome (KS) is the most common chromosomal aneuploidy in males. It is very difficult to diagnose this disorder in childhood due to absence of significant manifestations before puberty. These patients usually present with tall stature. We report a case of KS with short stature due to growth hormone deficiency. The boy’s height was below the 3rd centile with significant delay in bone age. He responded well to growth hormone injections. In view of mental subnormality karyotyping was done, which revealed KS (47XXY).

SHORT COMMUNICATION
13.Variability of DKA Management Among Pediatric Emergency Room and Critical Care Providers: A Call for More Evidence-Based and Cost-Effective Care?
Matthew G Clark, Abdallah Dalabih
doi: 10.4274/jcrpe.1434  Pages 190 - 191
Management protocols have been shown to be effective in the pediatric emergency medicine (PEM) and pediatric critical care (PCC) settings. Treatment protocols define clear goals which are achieved with consistency in implementation. Over the last decade, many new recommendations have been proposed on managing diabetic ketoacidosis (DKA). Although no perfect set of guidelines exist, many institutions are developing DKA treatment protocols. We sought to determine the variability between institutions in implementation of these protocols.

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