Primary persistent hyperinsulinaemic hypoglycaemia is characterised by clinical symptoms that occur when blood glucose levels drop below the normal range. Diazoxide treatment remains the mainstay of medical therapy. Tolerance of diazoxide is usually excellent, but several side effects of this drug have been described. We present a 4-month-old girl who developed pulmonary hypertension, heart failure and neutropenia during diazoxide therapy. Diazoxide toxicity was suspected and the drug was withdrawn on day 13. During the next 3 days, respiratory and haemodynamic status dramatically improved and she was weaned from mechanical ventilation. Control white blood cell count was 8800 cells/mm(3) and a new echocardiography showed modreduction of pulmonary artificial pressure to 20 mmHg and resolution of atrial and ventricular enlargement. Paediatric physicians should be in mind of pulmonary hypertension, heart failure and neutropenia developing during diazoxide therapy.

ÖZETAmaç: Doğuştan kalp hastalıkları için yüksek ve düşük risk taşıyan gebelerde intrauterin kalp hastalığı sıklığını belirlemek ve karşılaştırmak. Yöntemler: Üniversitemiz Tıp Fakültesi Pediatrik Kardiyoloji Ünitesinde fetal ekokardiyografi yapılan 3782 gebenin kayıtları ve ekokardiyografi sonuçları retrospektif olarak değerlendirildi. Gruplar arasındaki değişkenler Ki-kare testi kullanılarak analiz edildi. Bulgular: Yüksek ve düşük riskli gruplardaki tüm fetüslerin 213'ünde (%5.6) yapısal kalp hastalığı bulundu. En sık patolojiler ventriküler septal defekt (%36.2) ve atriyoventriküler septal defekt (%14.1) idi. Yüksek riskli grupta doğuştan kalp hastalığı sıklığı %7.8 (169 fetüs) bulundu; %4.1'i kompleks, %2.3'ü önemli, %1.4'ü minör patolojiydi. Düşük riskli grupta bu oran %2.7 (44 fetüs) idi; %0.6'sı kompleks, %0.8'i önemli ve %1.3'ü minör patolojiydi. Bu grupta bazı nedenlerle başvuranlarda yapısal kalp hastalığı sıklığı yüksek bulundu; önceki gebelikte intrauterin fetal ölüm (%6.3), anormal birinci ve ikinci üç aylık dönem tarama testleri (%4.3) ve çoğul gebelik (%3.4). Fetal ekokardiyografik incelemenin duyarlılığı %86, özgül-lüğü %98 bulundu. Sonuç: Doğuştan kalp hastalığı sıklığı beklenildiği gibi yüksek riskli grupta daha yüksek bulundu. Düşük riskli grupta yer almalarına rağmen önceki gebelikte intrauterin fetal ölüm, anormal birinci ve ikinci üç aylık dönem tarama testleri ve çoğul gebelik nedeniyle başvuranlardaki kalp hastalığı sıklığı ile yüksek riskli gruptaki kalp hastalığı sıklığı arasında istatistiksel olarak anlamlı bir fark bulunmadı. Bu nedenle kardiyoloji kliniğinin bu konuda uzman kardiyoloğu ve ekipmanı yeterli ise yukarıdaki nedenlerle başvuran gebelere rutin fetal ekokardiyografik çalışma yapılmasını öneriyoruz. (Anadolu Kardiyol Derg 2011; 2: 125-30) Anahtar kelimeler: Doğuştan kalp hastalığı, fetal ekokardiyografi, yüksek riskli gebelik, düşük riskli gebelik ABSTRACT Objective: To establish and compare the frequency of intrauterine congenital heart defects in high-risk and low-risk pregnancies for congenital heart diseases. Methods: Records of 3782 patients who underwent fetal echocardiography at the Pediatric Cardiology Unit were reviewed for reasons of referral and results of echocardiography retrospectively. The categorical variables between the groups were analyzed using a Chi-square test. Results: Structural heart defects were found in 213 (5.6%) fetuses in both high and low risk groups. Most common defects were ventricular septal defect (36.2%) and atrioventricular septal defect (14.1%). Frequency of congenital heart diseases was 7.8% (169 fetuses) in high-risk group: 4.1% were complex, 2.3% significant and 1.4% were minor. In low-risk group, the frequency was 2.7% (44 fetuses): 0.6% were complex, 0.8% significant and 1.3% were minor. In this group, rates of congenital defects were high regarding particular reasons: intrauterine fetal death in previous pregnancy (6.3%), abnormal first or second trimester screening tests (4.3%), and multiple gestations (3.4%). The sensitivity and...

CTS is a rare congenital cardiac anomaly in which the results of the corrective surgery are encouraging. Early and long-term outcomes may be variable according to the associated congenital heart defects.

No abstract

Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.

Congenital aortico-cameral communications are rarely seen. We present an asymptomatic patient in whom there was a congenital vascular communication rising from the aortic root and terminating in the left atrium. She had an atrial septal defect (ASD). The diagnosis was made with echocardiography and confirmed by aortography. She was treated by closing the tunnel and the ASD. The outcome was satisfactory in this extremely rare case of a congenital cardiac lesion. Coincidental diagnosis could be made during careful echocardiographic examination.

Congenitally corrected transposition of the great arteries is a rare congenital cardiac anomaly in which the results of the anatomical repair with double switch operation appear to be superior to that achieved by a physiological repair.

We describe a 4-year-old girl with asthma who presented with pneumomediastinum, pneumopericardium and subcutaneous emphysema. She was admitted to our hospital with dyspnea, chest pain, palpitation and cough of two days duration. She had attacks of cough, dyspnea and wheezing from two years of age, but she did not have a diagnosis of asthma previously. She was dyspneic and had subcutaneous emphysema in the neck, axilla and thorax. In the skin prick test (Center Lab. USA) she had positive reaction to Dermatophagoides pteronyssinus, Dermatophagoides farinae, mold mix, tree mix and grass mix. Pulmonary function tests could not be performed. In the chest X-ray air was seen in mediastinum and subcutaneous area and the epicardium was surrounded completely with air. She was treated successfully with inhaled salbutamol and budesonide. Radiological signs of pneumopericardium and pneumomediastinum disappeared completely in ten days period. In the light of this case we want to mention that early diagnosis and treatment of asthma should be done to prevent serious complication of asthma.