hrp0098p2-298 | Late Breaking | ESPE2024

A real-world data analysis of 37 pediatric patients with pheochromocytoma and paraganglioma: Evaluation of the concordance between current diagnostic and treatment algorithms and clinical management practices in resource-limited settings

Kurt Ilknur , Gurpinar Tosun Busra , Gul Uslu Nihal , Ozalp Kizilay Deniz , Dikmen Ibrahim , Ozguc Comlek Fatma , Bugrul Fuat , Bezen Digdem , Hatipoglu Nihal , Dogan Murat , Deniz Papatya Cakir Esra , Ucar Ahmet , Ozcabi Bahar , Yildirim Cakar Didem , Hatun Aytac Kaplan Emel , Sutcu Zumrut , Nur Hepokur Merve , Catli Gonul , Ahmet Ucakturk S , Demir Senol , Siklar Zeynep , Haliloglu Belma , Turan Serap , Bereket Abdullah , Guran Tulay

Background: Pheochromocytomas and paragangliomas (PPGLs) are rare pediatric neuroendocrine tumors. Data on the diagnosis, treatment and follow-up of PPGL in children are limited and lack standardization. Furthermore, variations in access to diagnostic and therapeutic resources across centers contribute to inconsistencies in clinical management.Aim: To identify variations in the clinical management of paediatric PPGL with...

hrp0092p3-65 | Diabetes and Insulin | ESPE2019

Insulin-Induced Oedema in a Child with Newly Diagnosed Diabetes Mellitus

Cakir Meltem Didem , Baysal Ozlem

Oedema is a care complication of insulin therapy. It affects mainly patients with newly diagnosed type 1 diabetes mellitus, poorly controlled diabetes mellitus or patients on large doses of insulin. There are only a few case report showing that it is an uncommon and probably an under-reported complication. Two mecanisms are known: the sodium and water retention and vasoactive mediators release. The oedema tends to develop shortly after initiation of insulin therapy and resolve...

hrp0092p2-101 | Diabetes and Insulin | ESPE2019

Evaluation of the Effect of Knowledge Levels of Adolescents Diagnosed with Type 1 Diabetes Melllitus on Hba1c and Life Quality Score

KESKIN Meliksah , YILDIRIM Nurdan , CETINKAYA Semra , AYCAN Zehra

Introduction: Type 1 Diabetes Mellitus (T1DM) is a chronic disease in children and adolescents.There are studies showing that quality of life is affected in T1DM. In our study, we aimed to evaluate the effect of diabetes knowledge levels on quality of life and metabolic status.Methods: Patients aged >10 years who were diagnosed with diabetes mellitus in our clinic and who were given diabetes education and who had reg...

hrp0084p3-612 | Adrenals | ESPE2015

11β-Hydroxylase Deficiency: 20 Years Follow-Up

Guven Ayla , Helvacioglu Didem , Kilinc Suna

Background: We presented 20-year follow-up of two patients with 11β- hydroxylase deficiency, one of them has novel mutation.Case 1: The male patient was diagnosed at the age of 9-months. He presented with penil growth and pubic hair. At diagnosis blood pressure was normal, his hormonal profile was distinctive of 11β-OHD, with elevated serum levels of 11-deoxycortisol (DOC 134 ng/ml, N: 0–1.18). Hydrocortisone was introduced immediately aft...

hrp0084p3-668 | Bone | ESPE2015

Treatment of Life Threatening Hypercalcaemia in Two Infants

Helvacioglu Didem , Kilinc Suna , Guven Ayla

Introduction: Physicians often treat patients with high doses of vitamin D for various ailments and on occasion the prescribed doses far exceed the requirements of the patients. We present here two infants with vitamin D intoxication.Case presentation: A 6 month-girl child was brought to the hospital with complaints of persistent vomiting and refusal to feed. She was clinically dehydrated. She was administered 150.000 IU every day for 8 days. Serum calci...

hrp0084p3-798 | DSD | ESPE2015

‘I am a Boy Since 8-Years-Old’: Female During Childhood, Virilization at Puberty

Kilinc Suna , Guven Ayla , Helvacioglu Didem

Background: 5α-reductase-deficiency is an autosomal recessive disorder with clinical spectrum ranges from a male phenotype with hypospadias to a female phenotype. Many different mutations of SRD5A2 gene has been described in affected patients and clinical signs can vary depending on the degree of enzyme deficiency.Case presentation: 14-years-old girl admitted to our clinic because of feeling as a boy since 8-years-old. Parents were first degree cous...

hrp0097p2-182 | Diabetes and Insulin | ESPE2023

Evaluation of clinical outcomes in children and adolescents with type 1 diabetes switching from Insulin Glargin to Insulin Detemir

Turan Hande , Kaya Didem , Ercan Oya

Basal-bolus insulin therapy is currently, the most common treatment modality in children and adolescents with type 1 diabetes mellitus (T1DM) worldwide. Insulin glargine and detemir are two different molecules, produced with small modifications, of human insulin. In this prospective longitudinal study, we aimed to compare HbA1c, time in range in CGM, basal insulin doses and hypoglyccemia frequencies in children and adolescents with T1DM who underwent switching from insulin det...

hrp0094p2-422 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2021

17-hydroxylase deficiency and diabetes in a case with 46 XY female

Yildirim Ahmet , Zeytun Hikmet , Albayrak Serpil , Karaoğlan Murat , Keskin Mehmet

A 13-year-old patient was admitted due to delayed puberty. Ovary and uterus had not seen in USG and MRI. On physical examination; body weight: 57 kg (79 P), height 157.7 cm (45P), BMI: 22.9 (83P), TA: 120/80 mmHg. The patient was externally in female appearance and there was no puberty development. Labia minora was seen. The urethral and vaginal openings were in the vestibulum and bilateral gonads could not be palpated in the inguinal canal. Mother and father was cousin. Fathe...

hrp0086p2-p875 | Syndromes: Mechanisms and Management P2 | ESPE2016

Tricho-Rhino-Phalangeal Syndrome Type I in a Girl with Growth Hormone Deficiency

Cakir Meltem Didem , Altintas Zuhal , Bozdogan Sevcan Tug

Tricho-rhino-phalangeal syndrome Type I (TRPS I) is a rare autosomal dominant genetic disorder clinically characterized by craniofacial and skeletal abnormalities, associated with cone-shaped epiphyses, brachydactyly and short stature. Although patients with TRPS I present various degrees of short stature, there are only four reports of growth hormone deficiency in patients with TRPS I. We present the case of TRPS I and partial GH deficiency. A 15-year-old female was referred ...

hrp0082p3-d3-693 | Bone (2) | ESPE2014

The Use of Pamidronate in Pediatric Patients with Disease of Other than Osteogenesis Imperfecta: the Experience of Our Center

Guven Ayla , Helvacioglu Didem , Hancili Suna , Yildiz Metin

Background: Bisphosphonate treatment for bone fragility has expanded beyond the children with osteogenesis imperfecta (OI) to those with other causes of low bone mass. Pamidronate is effective such as Paget’s disease, hypercalcaemia of malignancy, osteolytic bone metastasis, steroid-induced osteoporosis and idiopathic osteoporosis.Objective and hypotheses: The experience with bisphosphonates treatment other than OI in children is limited although th...