Exploring congenital sucrase-isomaltase deficiency in autism spectrum disorder patients with irritable bowel syndrome symptoms: A prospective SI gene sequencing study.

Congenital sucrase-isomaltase deficiency (CSID) is an inherited metabolic disorder causing chronic gastrointestinal symptoms and malnutrition when untreated. Most CSID patients are likely to remain under- or misdiagnosed. This study aimed to investigate prevalence of CSID among patients with autism spectrum disorder (ASD) presenting with irritable bowel syndrome (IBS) symptoms via prospective SI gene sequencing.

Use of ivabradine in children with junctional ectopic tachycardia after pediatric cardiac surgery; two-centre experience.

Introduction: Although amiodarone is traditionally used in the treatment of postoperative junctional ectopic tachycardia (JET), the search for new treatments is ongoing. We present our experience with ivabradine at two medical centers.

Materials And Methods: Between January 2022 and January 2023, patients who developed JET after pediatric cardiac surgery were prospectively followed up and documented.

Management of patients developing axillary pseudoaneursym after ductal stenting: Report of three cases.

Pseudoaneurysms develop as a result of disruption of the arterial wall due to trauma or iatrogenic reasons such as catheterization, and it is important due to the high risk of bleeding and rupture. Until recently, the main treatment of pseudoaneurysms was surgical repair. However, in recent years, minimally invasive methods such as ultrasound-guided compression and percutaneous thrombin injection have been used more frequently.

Assessment of the frequency and risk factors of gastrointestinal bleeding after cardiopulmonary bypass in paediatric cases.

Introduction And Aim: Gastrointestinal bleeding is a potential complication in paediatric patients undergoing cardiopulmonary bypass, as it develops secondary to low gastrointestinal perfusion. This study aimed to examine the incidence of gastrointestinal bleeding and identify its risk factors in these patients.

Method: This retrospective study was undertaken to examine the demographic features, clinical findings, and operative data of paediatric patients under years old who had undergone congenital heart surgery with cardiopulmonary bypass between November 1, 2021, and November 1, 2023.

Impact of high-flow nasal oxygen therapy on postoperative atelectasis and reintubation rate after paediatric cardiac surgery.

Introduction: Airway problems emerging after congenital cardiac surgery operations may have an impact on mortality and morbidity. Recently, to improve alveolar gas exchange and reduce respiratory effort, high-flow nasal cannula (HFNC) has started to be used in paediatric cases. This study aimed to evaluate the potential effects of high-flow nasal oxygen therapy on postoperative atelectasis development and reintubation rate in paediatric cardiac surgery patients.

Myocardial infarction and narrowed peripheral arterial vessels secondary to generalised arterial calcification syndrome in a two-month-old girl.

Generalised arterial calcification of infancy, an autosomal recessive disorder characterised by abnormal calcification of medium and large-sized arteries, represents a rare cause of dilated cardiomyopathy. We present the case of a two-month-old girl diagnosed posthumously with dilated cardiomyopathy. Studies suggest that early initiation of treatment can improve prognosis in generalised arterial calcification of infancy, so clinicians should be alert to the condition, especially in patients displaying generalised narrowing of medium and large-sized arteries.

Results of a hybrid approach for high risk term newborn patients with interrupted aortic arch (IAA) with left ventricular outflow tract obstruction.

This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated.

Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers.

Pulmonary Hypertension Due to High-Output Heart Failure: Hereditary Hemorrhagic Telangiectasia.

Pulmonary hypertension (PH) is a complex disorder that should be managed with a multidisciplinary approach. Although most of the underlying causes of left heart disease can be easily diagnosed with cardiac imaging, some pathologies might necessitate careful investigation to go beyond the obvious. High-output heart failure (HF) due to arteriovenous malformation (AVMs) is an unnoticeable cause for HF and PH.

Factors Predicting Early Major Adverse Events in the Intensive Care Unit After Successful Cardiac Surgery for Congenital Heart Disease in Full-Term Neonates.

Objective: In this study, we aimed to evaluate the factors affecting major adverse event (MAE) development after full-term neonatal cardiac surgery.

Methods: This study was conducted retrospectively on newborns who underwent congenital heart surgery between June 1, 2020, and June 1, 2022. MAE was defined as the presence of at least one of the following: cardiac arrest, unplanned reoperation, emergency chest opening, admission to the advanced life support system, and death.

A rare ventriculoarterial connection: double outlet of both ventricles.

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.

A unique bizarre subtype of mixed total anomalous pulmonary venous connection.

Mixed total anomalous pulmonary venous connection is a rare CHD with a wide variation in pulmonary venous anatomy and drainage. We present a ten-day-old newborn with a rare bizarre subtype of mixed total anomalous pulmonary venous connection.

Comparison of the effects of conventional method and primary sutureless techniques on early postoperative rhythm problems in patients with total abnormal pulmonary venous return anomaly.

Background: Total abnormal pulmonary venous return anomaly is a CHD characterised by abnormal pulmonary venous flow directed to the right atrium. In this study, we aimed to compare the effects of these techniques on early rhythm problems in total abnormal pulmonary venous return anomaly cases operated with conventional or primary sutureless techniques.

Method: Seventy consecutive cases (median age 1 month, median weight 4 kg) who underwent total abnormal pulmonary venous return anomaly repair with conventional or primary sutureless technique between May 1 2020 and May 1 2022 were evaluated.

Assessment of the factors that affect fast-track or early extubation following pediatric cardiac surgery.

Background: This study aims to evaluate the early extubation rate and the factors affecting early extubation in pediatric patients undergoing cardiac surgery.

Methods: Between August 1st, 2020 and December 1st, 2021, a total of 528 pediatric patients (264 males, 264 females; median age: 4 months; range, 2 days to 24 months) who were followed in the pediatric cardiac intensive care unit after congenital heart surgery were retrospectively analyzed. Demographic and clinical characteristics of the patients including operation and intensive care data were obtained from the medical records.

Evaluation of perfusion index and left ventricular output changes in low cardiac output syndrome after arterial switch operation.

Introduction: Transposition of great arteries is one of newborns' most common cyanotic CHDs, and its treatment is arterial switch operation in the first days of life. Low cardiac output syndrome may develop in the early postoperative period. In this study, we evaluated perfusion index and left ventricular output blood flow changes in patients who underwent arterial switch operation and developed low cardiac output syndrome.

The comparison of three different acute kidney injury classification systems after congenital heart surgery.

Background: We aimed to compare the frequency of acute kidney injury (AKI) and its effects on mortality and morbidity with different classification systems in pediatric patients who had surgery under cardiopulmonary bypass for congenital heart disease.

Methods: This study included children younger than 18 years old who were followed up in the pediatric cardiac intensive care unit between September 1 and December 1, 2020, after congenital heart surgery with cardiopulmonary bypass. Each case was categorized postoperatively in terms of AKI using Pediatric-Modified Risk, Injury, Failure, Loss, and End-Stage (pRIFLE), Acute Kidney Injury Network (AKIN), and Kidney Disease: Improving Global Outcomes (KDIGO).

Comparison of early-stage stenosis and regurgitation results among patients who underwent aortic valve repair.

Introduction: This study aimed to analyse the results of paediatric aortic valve repairs in our institution.

Method: The data of 57 patients under 18 years of age who underwent aortic valve repair between 2014 and 2019 were retrospectively analysed. Early postoperative reoperation and hospital mortality rates were evaluated based on the ages of the patients, their preoperative diagnoses, and the surgical techniques used.

Evaluation of postoperative renal functions and its effect on body perfusion in patients with double aortic cannulation.

Background: The optimal visceral preservation method during aortic arch reconstruction is still controversial. It has been thought that double aortic cannulation is effective. Herein, it was aimed to evaluate this technique in providing distal perfusion.

F-Fluorodeoxyglucose Positron Emission Tomography/Magnetic Resonance Imaging Appearance of Gastrointestinal Behcet's Disease.

Behcet's syndrome (BS) is a variable vessel vasculitis with multi-organ involvement. Recurrent episodes of oral and genital ulcers, papulopustular and erythema nodosum-like skin lesions, and arthritis are relatively more frequent, whereas uveitis, venous and arterial lesions, nervous system, and gastrointestinal involvement are less common, but are severe manifestations. The frequency of gastrointestinal involvement shows important variation between countries as more common in the Far East and the United States, and much less common in Turkey and the Middle East.

The effect of somatostatin analogs and acromegaly on the upper gastrointestinal system.

Purpose: To evaluate the effects of somatostatin analogs and disease activity status on the upper gastrointestinal system in patients with acromegaly.

Methods: One hundred eighty-one patients with acromegaly were retrospectively assessed. The demographic, biochemical, pathologic, and radiologic data of the patients were evaluated.