Search

Amaç: Tiroid kanserleri çocuk ve adölesanlarda nadirdir. Çalışmada kliniğimizde takip ettiğmiz çocuk ve adölesan tiriod kanserlerinin klinik özelliklerini ve prognozunu değerlendirmeyi amaçladık.Gereç ve Yöntemler: 2004-2014 arası kliniğimizde izlenmekte olan 12 tiroid kanserli hasta çalışmaya dahil edildi.Bulgular: Papillar karsinom 9, folliküler karsinom 3 hastada saptandı. Boyun şişliği en sık başvuru nedeniydi. Bir hastada (%8) servikal lenf nodu metastazı, 1 hastada (%8) anjioinvazyon belirlendi. Akciğer metastazı saptanmadı. Total tiroidektomi 4 hastada, ipsilateral total ve kontralateral subtotal tiroidektomi 5 hastada, parsiyel tiroidektomi 2 hastada, tiroglossal kanal eksizyonu 1 hastada uygulanmıştı. I131 ablasyonu 8 hastada (%66) rezidüel veya metastatik hastalık nedeniyle uygulanmıştı. Hastalar hala remisyonda izlenmekte ve rekürrens görülmemiştir.Sonuç: Tiroid kanserlerinin çocuk ve adölesanlarda prognozu iyi olup total ve near total tiroidektomi ve takiben radyoaktif tedaviden fayda görmektedirler. Bu tedavi yaklaşımlarının kısa ve uzun dönem yan etkilerinin belirlenmesi için prospektif çalışmalara ihtiyaç vardır., Objective: Thyroid carcinomas are rare in childhood and adolescence. This study was conducted to evaluate the clinical features and outcomes in children and adolescents with thyroid carcinoma at our center. Material and Methods: Twelve children and adolescents treated for thyroid carcinoma in our departments from 2004 to 2014 were included in the study. results: Papillary carcinoma was found in 9 patients and follicular carcinoma in 3 patients. Neck swelling was the most common chief complaint. At the time of surgery, one patient (8%) had cervical lymph node metastasis, and one (8%) had angioinvasion. No patients had lung metastasis. Total thyroidectomy was performed in 4 patients, ipsilateral total and contralateral subtotal thyroidectomy in 5 patients, partial thyroidectomy in 2 patients and thyroglossal duct cyst excision in one patient. Eight patients (66%) required 131I ablation for residual or metastatic disease after thyroidectomy. At the time of this report, all patients were alive without recurrence.conclusion: According to our data, children and adolescents with thyroid carcinoma have a good prognosis and benefit from total or near-total thyroidectomy followed by radioiodine therapy. Future prospective studies are required to clarify the short- and long-term side effects of treatment approaches

Amaç: Çalışmada çocuklarda obeziteye eşlik eden endokrin-metabolik bozuklukların sıklıklarının belirlenmesi ve bunlarla ilişkili faktörlerin ortaya konması amaçlanmıştır.Gereç ve Yöntemler: Çalışmaya bir çocuk endokrinoloji polikliniğinde obezite tanısı alan 642 hasta dahil edilmiştir. Vücut kitle indeksi 95. persentil üzerinde olan olgular obez olarak kabul edilmiştir. Tüm olgular; yaş, bel çevresi, Vücut Kitle İndeksi (VKİ), VKİ standart deviasyon skoru, sistolik ve diyastolik kan basıncı, homeostasis model of assesment for insülin resistance (HOMA-IR) indeksi, stria, hirsutismus, akantozis nigrikans, cushingoid görünüm, erken puberte, hipertansiyon, insülin direnci, bozulmuş açlık glukozu (BAG), bozulmuş glukoz toleransı (BGT), Tip 2 Diyabet (Tip 2 DM), subklinik hipotiroidi ve Haşimato tiroiditi açısından retrospektif olarak değerlendirilmiştir.Bulgular: Çalışmada yer alan olgularda ortalama yaş 11.1 ± 3.2 yıl, erkek/kız oranı 1.01’di. Obezite ile ilişkili gözlenen en sık endokrin bozukluk insülin direnci (%44.4) iken, en sık klinik bulgu olarak akantozis nigrikans (%31.9) saptandı. Obezite ile ilişkili klinik bulgulardan stria ve erken puberte kızlarda erkeklere göre anlamlı olarak daha sık bulundu (p, Objective: This study aimed to investigate the frequency of endocrine-metabolic disorders accompanying obesity in children and to identify the factors related to these disorders.Material and Methods: A total of 642 children with a diagnosis of obesity among the endocrinology clinic patients were included in the study. Obesity was accepted as a body mass index over the 95th percentile. All patient were evaluated in term of age, waist circumference, body mass index, body mass index standard deviation score, systolic and diastolic hypertension, homeostasis model of assessment for insulin resistance (HOMA-IR) index, stria, hirsutismus, acantosis nigricans, cushingoid appearance, early puberty, dyslipidemia, hypertension, insulin resistance, impaired fasting glucose, impaired glucose tolerance, type 2 DM, subclinical hypothyroidism and Hashimato thyroiditis, retrospectively.Results: The mean age of the patients with a diagnosis of obesity was 11.1 ± 3.2 years and the male/female ratio was 1.01. The most common accompanying endocrine problem was insulin resistance (44.4%), and the most common clinical sign was acantosis nigricans (31.9%). Striae and precocious puberty were seen significantly more commonly in female than male patients (p < 0.05). Obesity-related clinical symptoms including hypertension, hirsutism, stria and acantosis nigricans were significantly more common in pubertal patients but buried penis was more commonly seen in pre-pubertals than pubertal cases (p< 0.05). We found the frequency of dyslipidemia, autoimmune thyroiditis and subclinical hypothyroidism in obese children and adolescents to be 23.2%, 4% and 1.2% respectively. The frequencies of IGT and type 2 DM were 7.5% and 0.9% respectively. This group of patients were found to be older, to have higher BMI and abdominal circumference, and to suffer more commonly from dyslipidemia, hypertension, acantosis nigricans and stria compared to the patients without insulin resistance (p=0.001). Conclusion: Obesity is a significant health problem of childhood. The incidence of endocrine and metabolic disorders is increased in obese children with age-induced obesity. These children should be screened for the possible presence of endocrine and metabolic disorders taking into account the age, pubertal status and gender. Determining children at metabolic risk and decreasing morbidity and mortality in adulthood requires prevention of obesity in childhood and careful follow-up of obese children with lifestyle modification.

Amaç: Nörofi bromatozis Tip1 (NF 1) klinik bulguları çocukluk çağında ortaya çıkmaya başlayan ve pek çok sistemi etkileyen otozomal dominant geçişli bir hastalıktır. Birçok endokrin problem özellikle de büyüme ve püberte sorunları NF 1’e eşlik edebilmektedir. Bu çalışma ile NF 1 tanılı hastalarımızın büyüme, pübertal sorunlar ve endokrin problemlerinin değerlendirilmesi amaçlanmıştır.Gereç ve Yöntemler: Kliniğimizde izlenmekte olan 38 hastanın antropometrik, klinik ve laboratuvar verileri geriye dönük olarak incelendi.Bulgular: Çalışmaya 38 olgu alındı, (18 kız, 20 erkek); %55.3’ü sporadik, %44.7’si ailevi tip NF 1 olgusuydu. Olguların 6’sında maternal geçiş, 11’de paternal geçiş öyküsü vardı. Ortalama başvuru yaşı 10.8 ± 4.4 (2.1 ile 19 yaş) ve 24 hasta (%63.2 ) püberte dönemindeydi. Boy kısalığı 38 hastanın 11’de (%28.9), obezite 5 olguda (%13.2), fazla tartılı olma 5 olguda (%28.9) saptandı. Püberte bozukluğu 7/38 (%18.4) de olup; 2 gecikmiş püberte, 1 santral püberte prekoks,1 prematür telarş,2 prematür pubarş ve 1 pübertal jinekomasti olgusu saptandı. Skolyoz 5 olguda (%13.2) görüldü. Hipotroidi 3/38 (%7.9) da görüldü; 2 olguda otoimmün troidit, 1 olguda konjenital hipotroidi (dishormonogenezis) vardı. D vitamini eksikliği ve yetersizliği sırasıyla %44.7 ve %10.5 saptandı.Sonuç: NF 1 hasta grubunda vitamin D eksikliği, obezite, boy kısalığı, erken ve geç püberte en yaygın görülen endokrin sorunları oluşturmaktadır. Bu nedenle NF 1 tanılı hastaların endokrinolojik açıdan periyodik olarak izlenmesi erken tanı ve uygun tedavi almalarını sağlayacaktır., Objective: Neurofi bromatosis type 1(NF1) is one of the most common autosomal dominant multisystem diseases. Many endocrine problems especially related to puberty and growth may accompany NF1. We evaluated growth, pubertal development and endocrine problems in patients with NF1.Material and Methods: We obtained the anthropometric variables, and clinical and laboratory data of 38 patients (18 girls and 20 boys) with sporadic (55.3%) or familial NF-1 (44.7%). Six patients had affected mothers and 11 had affected fathers). The mean age at referral was 10.8±4.4 years (range 2.10 to 19 years) and 24 patients were pubertal (63.2%). The average age at diagnosis was 6.6 years and the mean follow-up period was 4.2 years. Results: Short stature was recognized in 11 of the 38 children (28.9%). One of them had an endocrine disorder (hypothyroidism). Obesity was diagnosed in 5 cases (13.2 %) and another 5 cases were overweight (13.2%). Puberty was abnormal in 7/38 of the children (18.4%). Two cases of delayed puberty, 1 central precocious puberty (1 male with optic glioma), 1 premature telarche, 2 premature pubarche and 1 pubertal gynecomastia cases were found. Lisch nodules were seen in 9 cases (23.7%). Scoliosis was diagnosed in 5 cases (13.2%). Hypothyroidism was detected in 3/38 (7.9%) children. Two of them had autoimmune thyroiditis and one had congenital hypothyroidism (dyshormonogenesis). The frequencies of vitamin D defi ciency and insuffi ciency in the winter were 44.7% and 10.5% respectively. There vitamin D levels were not adequate in our NF1 patients. Conclusion: Vitamin D defi ciency, obesity, short stature and pubertal disorders were the most common endocrine problems in our study group. We believe that patients with NF1 should see an endocrinologist routinely

Moyamoya hastalığı (MMH) internal karotid arterler ve proksimal dallarının ilerleyici stenozuyla birlikte hastalarda felçlere zemin hazırlayan serebrovasküler bir durumdur. Moyamoya hastalığı çeşitli doğumsal ve edinsel bozukluklar ile ilişkili olarak tarif edilmiştir. Konjenital hematolojik bozukluklar (Fanconi anemisi, orak hücreli anemi), genetik sendromlar (apert sendromu, Seckel sendromu, Robinow sendromu, Down sendromu, nörofibromatosiz tip 1), homosistinüri gibi metabolik bozukluklar Moyamoya hastalığı ile ilişkili olarak bilinmektedir. Aynı zamanda sella/kiazma bölgesi tümörlerinde ve lösemi nedeniyle profilaktik radyoterapi alan hastalarda MMH geliştiği bilinmektedir. Şimdiye dek MMH ile over tümörü arasında bir ilişki tanımlanmamıştır. Burada, tekrarlayan over tümörü olan, kurtarma kemoterapisi sonrası MMH gelişen 8 yaşında bir kız vaka bildirilmiştir., Moyamoya disease (MMD) is a cerebrovascular condition that predisposes affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. Moyamoya disease has been described in association with a variety of congenital and acquired diseases. Some congenital hematological diseases (Fanconi anemia, sickle cell anemia), genetic syndromes (Apert’s syndrome, Seckel syndrome, Robinow syndrome, Down syndrome, neurofibromatosis type I), metabolic diseases (homocystinuria) are known to associated with Moyamoya syndrome. It is also known that MMD may develop after cranial radiotherapy in children with sella/chiasma region tumors or after prophylactic cranial irradiation for leukemia. However, there is no data regarding the presence of an association between ovarian tumor and MMD. Here, we report a case of an 8-year-old girl with recurrent ovarian tumor developed MMD after salvage chemotherapy

Adrenokortikal karsinom (AKK) çocukluk çağının nadir görülen malign tümörlerindendir. Klinik ve laboratuvar bulgularıyla konjenital adrenal hiperplaziye (KAH) benzerlik gösterebilir. Burada başlangıçta KAH düşünülen, ancak hemen sonrasında adrenokortikal karsinom tanısı alan 3 yaşında bir erkek hasta sunulmuştur. Hasta, altı aydır vücudunda kıllanma ve penis büyümesi şikayeti ile başvurdu. Fizik muayenesin de ciddi hipertansiyon saptanan hastanın penis boyu 90 persentilin üzerinde, testis volümleri bilateral 5 ml ve evre-2 pubarşı vardı. Laboratuvar değerlendirmede, ACTH, 0.8 pg/ml, bazal kortizol düzeyi 19, pik kortizol düzeyi 19.2 mcg/dl, pik 17OH progesteron 60.1 ng/ml, bazal 11- deoksikortizol 16.3 ng/ml (normal düzey: 0–8), serum testesteron; 27.9 ng/dl, Serum Na138mmol/L, K3.3 mmol/L bulunması üzerine 11 ß hidroksilaz eksikliğine bağlı KAH düşünüldü. Günlük 20 mg/m2 hidrokortizon başlanan hastaya bir haftalık izlem süresince antihipertansif tedaviye yanıt alınamaması ve trunkal obezitesi nedeniyle yapılan abdominal USG’de sağ adrenal yerleşimde 5x6x5 cm’lik kitle saptandı. Hasta preoperatif ve postoperatif adrenalyetmezlik riski nedeniyle steroid profilaksisi ile opere edildi. Total cerrahi rezeksiyon sonrası AKK tanısı alan hastanın, klinik ve laboratuar bulguları bir hafta içerisinde normale döndü., Adrenocortical carcinoma (AKC) is an uncommon malignancy with poor prognosis in childhood. Because of the similar clinical and laboratary findings, it can be misdiagnosed as congenital adrenal hyperplasia (CAH). Here, we present a three year old male who was firstly thought to be CAH, but later diagnosed with AKC. He was admitted to our hospital with the complaints of increase in body hair and penile enlargement six month. His physical examination revealed high blood pressure over 95 percentile for his age, streched penis length was above 90 percentile for age, his testes volumes were 5 mL/5mL and he had Taner stage 2 pubic hair. Other vital sign and physical examination findings were normal. His plasma ACTH level ( 0.8 pg/mL), basal cortisol level (19.0 mcg/dl), peak cortisol level (19.2 mcg/dl), peak 17-OH progesteron level ( 60.1 ng/mL), basal 11- deoxycortisol (16.3 ng/mL) and testosteron level (27.9 ng/dl).Were high for his age. His plasma Na and K levels were within normal limits. With these clinical and laboratory findings he was firstly diagnosed as congenital adrenal hyperplasia due to 11 ß hydroxylase deficiency. He was put on hydrocortisone tretment at a dose of 20 mg/m2 /day but after one week of treatment his blood pressure levels were still high and he had prominent truncal obesity. Abdominal ultrasonography showed a 5x6x5 cm sized solid mass in the right surrenal region. The patient was operated in pediatric Surgery Department of our hospital. while on prophylaxis with steroid treatment for adrenal insufficiency. The clinical and laboratory findings of the patient ameliorated in a week after the total surgical resection of the tumor which was pathologically proven to be ACC