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Öğe 8-year old patient with giant hydatid cyst consulted with chest wall asymmetry(WILEY-BLACKWELL, 2015) Uygun, Saime Sundus; Aribas, Olgun Kadir; Pekcan, SevgiCystic echinococcosis, a zoonotic disease, is common in Turkey and is caused by the Echinococcus granulosus tapeworm. We describe the case of an 8-year-old girl who was admitted to hospital with chest wall asymmetry after a fall from height, without any other symptoms. Cystic lesion was identified on radiology, and serology supported the diagnosis of hydatid cyst. The patient underwent thorax surgery. Postoperatively, she was treated with benzimidazoles for 2 months. Rarely, hydatid cysts can reach a size sufficiently large to cause chest deformity.Öğe Evaluation of congenital anomalies accompanying gastrointestinal system malformation(Cukurova Medical Journal, 2019) Konak, Murat; Sert, Ahmet; Gunduz, Metin; Soylu, Hanifi; Uygun, Saime SundusPurpose: The aim of this study was to review accompanied congenital anomalies to gastrointestinal pathologies. Materials and Methods: The medical records of patients who were admitted to neonatal intensive care unit with gastrointestinal system surgical pathologies during 2015-2018 were retrospectively evaluated for associated congenital anomalies. Results: 67 patients were identified during the study period. The most common gastrointestinal system pathology was anal atresia in 15 cases (22.4%), followed by diaphragmatic hernia in 14 cases (20.9%), esophageal atresia (n: 10) and ileal atresia (n: 10) (14.9%). Thirty two patients (48.8%) have had accompanied congenital anomalies to gastrointestinal system pathologies. Congenital heart disease was the most common anomaly and the most frequent one was atrial septal defect in 18 cases (26.8%). In addition, there was complex congenital heart disease in (n: 13) 19.4%. In 12 patients (17.9%), accompanied extracardiac anomalies were detected. Hydronephrosis and sludge in gall bladder were detected in three patients (4.5%). Vesicoureteral reflux or ectopic kidney or polysplenia was detected as a separate anomaly in each case. In our cases, prematurity rate was found in 54%. Conclusion: Gastrointestinal system pathologies are frequently associated with multiple anomalies, especially with congenital heart diseases. In addition, these patients are likely to be preterm baby.Öğe Hypertrophic cardiomyopathy with Jeune syndrome: The first reported case(TURKISH SOC CARDIOLOGY, 2016) Guvenc, Osman; Uygun, Saime Sundus; Cimen, Derya; Aslan, Eyup; Annagur, AliJeune syndrome (Asphyxiating thoracic dysplasia) is a rare dystrophy of the skeleton, inherited as an autosomal recessive condition. Patients develop a narrowed thorax, rhizomelic dwarfism, and hepatic, renal, and pancreatic abnormalities. High rates of pulmonary hypoplasia and pulmonary hypertension have been reported. Some patients die in early stages of life due to respiratory failure. The case of a patient referred with a history of severe asphyxiating birth, who had been diagnosed with Jeune syndrome and later hypertrophic cardiomyopathy (HCM) upon echocardiographic examination is described in the present report. This rare disease is discussed with respect to the current literature, as the present is the first reported case to be accompanied by HCM.Öğe Meckel Gruber syndrome: A case report with review of literature(MODESTUM LTD, 2017) Uygun, Saime Sundus; Sivri, Mesut; Topsakal, Ahmet; Dikener, Ahmet Hakan; Soylu, Hanifi; Annagur, AliMeckel-Gruber syndrome, which is firstly described by Friedrich Meckel and Georg B. Gruber, is an autosomal recessive disorder that is characterized triad of occipital encephalocele, bilateral renal dysplasia and polydactyly. Because of the genetic heterogeneity, multiple organs can be affected. The worldwide incidence varies from 1 in 13,250 to 1 in 140,000 live births. We have reported a male baby born at 37-week gestation, who has occipital encephalocele, polydactyly and cystic dysplasia of both kidneys. As a result of these clinical findings, Meckel-Gruber Syndrome is suspected for his baby. This study is presented to draw attention to the Meckel Gruber Syndrome which has high risk of recurrence and early diagnosis by ultrasonographic screening can be confident.