Şeyma Kayalı

Regression of Symptomatic Multiple Cardiac Rhabdomyomas Associated with Tuberous Sclerosis Complex in a Newborn Receiving Everolimus

UNLABELLED Cardiac rhabdomyoma is the most common primary cardiac tumor, is considered to be a hamartoma of developing cardiac myocytes. Cardiac rhabdomyoma is associated with tuberous sclerosis complex (TSC) in 50-86% of cases. Mutations in TSC-1/TSC-2 genes result in increased mammalian target of rapamycin (mTOR) pathway activation responsible for the hamartomatous lesions of tuberous sclerosis complex. Therapy with mTOR inhibitors is currently under investigation as a treatment option for tumors associated with TSC. In this report we present a case with multiple symptomatic rhabdomyomas associated with tuberous sclerosis complex, deemed to be ineligible for surgical removal, treated with everolimus (mTOR inhibitor). CONCLUSION As we observed in our patient, in cases with inoperable symptomatic rhabdomyomas associated with TSC, everolimus, an mTOR inhibitor, may be the treatment of choice, which should be confirmed with additional studies.

Massive Pericardial Effusion and Tamponade Can Be a First Sign of Familial Mediterranean Fever

Abstract Cardiac involvement is a rare initial presentation of familial Mediterranean fever (FMF). We described 2 children with massive pericardial effusion and cardiac tamponade, who were later diagnosed as having FMF based on clinical and laboratory findings. Therefore, in children presenting with massive pericardial effusion, FMF should be considered as one of the differential diagnoses. In addition, massive pericardial effusion is a serious clinical condition that requires emergency therapeutic approach including pericardiocenthesis. For medical treatment, colchicine is the first line therapy, but in resistant cases, other anti-inflammatory drugs can be used for extra anti-inflammatory effect.

Persistent left superior vena cava accompanying congenital heart disease in children: Experience of a tertiary care center

This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD).

Anne Sütü Alan İnfantlarda Demir Takviyesinin Etkisi: Randomize Kontrollü Çalışma

Amac Bu calismanin amaci, cogunlukla anne sutu alan infantlarda demir eksikligi ve demir eksikligi anemisinden korunmada gunluk oral demir takviyesinin etkisini ve demir eksikligi gelisimini etkileyen faktorleri incelemektir. Metod Bu calisma Ocak 2009-Mart 2010 tarihleri arasinda, 65 infant (35 demir destegi alan ve 30 destek almayan kontrol grubu) ile yurutulmustur. Tum infantlara 4, 6 ve 9. aylarinda detayli fizik muayene yapildi. Demirden zengin beslenme onerileri infantlar 6 ve 9. ayinda iken tum ailelere verildi. Antropometrik olcumler, Denver gelisimsel tarama testi, tam kan sayimi ve demir parametreleri degerlendirildi. Sonuclar Demir destegi alan ve kontrol grubu infantlar arasinda demir eksikligi ve demir eksikligi anemisi gelisimi yonunden belirgin fark yoktu (31.44%, 46.6% sirasiyla) (p>0,05). Gruplar arasinda ayni zamanda, antropometrik olcumler, hematolojik parametreler, Denver gelisimsel tarama testi sonuclari arasinda belirgin fark izlenmedi. Tartisma Term, anne sutu alan infantlarda, 4 ayliktan itibaren verilen rutin demir takviyesi ile birlikte demirden zengin ek besinlerin tuketimi demir durumunu duzeltebilir. Diyetle yeterli demir alimi demir eksikligi anemisinden korumada etkin bir yoldur.

Aneurysm of the muscular septum associated with Wolf-Parkinson-White syndrome presenting as dilated cardiomyopathy; A report of two cases

Muscular septal aneurysms are extremely rare without a ventricular septal defect and are diagnosed accidentally in most cases. Reported cases generally have rhythm disturbance or electrocardiographic findings of Wolf-Parkinson-White (WPW) syndrome. Presently described are 2 cases of ventricular septal aneurysm associated with WPW syndrome, which presented as dilated cardiomyopathy. Pre-excitation disappeared gradually in first patient. There was also concurrent decrease in degree of bulging of the interventricular septum and improvement of left ventricular systolic function. Second patient had complaints of palpitation and was referred for ablation of accessory pathway. Our findings suggest that presence of pre-excitation may lead to ventricular dyssynchrony and abnormal ventricular septal movement, resulting in appearance of aneurysm.

PP-150 Effect of Pulmonary Hypertension Related Congenital Heart Disease with Left to Right Shunt on the Serum Levels of High Sensitive Cardiac Troponins in Children

The American Journal of Cardiology MARCH 26e29, 2015 11 IN AND C was attempted in 7 of the patients (2 teratoma, 1 papillerfibroelastoma, 1 fetal type rhabdomyoma, 1 TBS associated rhabdomyoma producing left ventricular outflow tract obstruction, 1 cystic tumor with granulomatous inflammation and 1 undiagnosed solid mass). Of the patients 3 died in perioperative period. Conclusion: Most of cardiac tumours during childhood are benign. Spontaneus regression is most probably possible in cases of TBS associated rhabdomyoma. Surgical intervention is only required for children who develop clinical symptoms due to hemodynamic instability.

PP-078 Pericardial Effusion in Childhood: Experience from Tertiary Care Center in Ankara

Pericardial Effusion in Childhood: Experience from Tertiary Care Center in Ankara. Tamer Yoldas, Ozkan Kaya, Şeyma Kayali, Ilker Ertu grul, Vehbi Do gan, Senem Ozgur, Utku Arman Orun, Selmin Karademir, Murat Koc. Department of Pediatric Cardiology, Dr. Sami Ulus Maternity and Children Research and Training Hospital; Department of Pediatric Cardiovascular Surgery, Dr. Sami Ulus Maternity and Children Research and Training

Multiple Gallstone Formation due to Ceftriaxone Treatment in a Child Diagnosed with Infective Endocarditis

Ceftriaxone, a third-generation cephalosporin, has been known to cause reversible sludge in the gallbladder. This sludge is referred to as biliary pseudolithiasis. In this article, a six-year-old child diagnosed with infective endocarditis is presented. He suffered from abdominal pain during the treatment with ceftriaxone and we performed an abdominal ultrasonographic examination that revealed multiple stones in the gallbladder. His symptoms quickly disappeared after the cessation of the treatment with ceftriaxone and we observed a significant decrease in size and number of the gallstones. Ceftriaxone is a commonly used drug in childhood and may cause gallstone formation. These gallstones usually resolve spontaneously and do not necessitate any surgical procedure.