Scoliosis and Kyphosis Prevalence in Turner Syndrome: A Retrospective Review at a Pediatric Tertiary Care Medical Center.

Background: The prevalence of major coronal and sagittal spinal curves (scoliosis and kyphosis) in Turner syndrome (TS) is not well established due to limited reporting. The relationship between growth hormone (GH) therapy and its effect on TS spinal curve incidence is also not well established.

Methods: A retrospective chart review of 306 TS patients from 2007 to 2021 evaluated major coronal and sagittal spinal curves, progression of the curve, and treatment with GH.

Comparison of Insulin Pump Bolus Calculators Reveals Wide Variation in Dose Recommendations.

Background: The introduction of insulin pumps with bolus calculators (BCs) has improved glycemic outcomes and quality of life for those with type 1 diabetes. Despite the increased reliance on BCs, the formulas used to derive recommended boluses are not standardized. Our objective was to examine whether recommendations from different pump BCs vary significantly for identical clinical scenarios.

The Fontan operation: the pursuit of associated lesions and cumulative trauma.

Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients.

Urgent permanent pacemaker implantation in critically ill preterm infants.

The management of complete heart block in premature low birth-weight infants, particularly those with hydrops fetalis, is challenging. We report emergent implantation of permanent epicardial pacemakers in the first 48 hours of life in two premature infants (one with hydrops fetalis) with birth weights of 1,400 grams and 1,000 grams.

Transforming patient and family access to medical information: utilisation patterns of a patient-accessible electronic health record.

Objective: The purpose of this study was to evaluate the utilisation of a web-based multimedia patient-accessible electronic health record, for patients with congenital cardiac disease.

Patients And Methods: This was a prospective analysis of patients undergoing congenital cardiac surgery at a single institution from 1 September, 2006 to 1 February, 2009. After meetings with hospital administration, physicians, nurses, and patients, we configured a subset of the cardiac program's web-based clinical electronic health record for patient and family access.

Hybrid ventricular decompression in pulmonary atresia with intact septum.

Initial palliation for pulmonary atresia with intact ventricular septum continues to evolve in the face of significant early and late morbidity. In patients with suitable anatomy, decompression of the right ventricle may be the first step in treatment. A hybrid approach to right ventricular decompression, combining surgery and interventional catheterization techniques is described.

Hybrid treatment of superior vena cava syndrome in a child.

A 10-year-old boy with a history of renal failure and hemodialysis by indwelling superior vena cava (SVC) catheters was diagnosed with SVC obstruction and clinically severe SVC syndrome. During attempted recanalization of the SVC in the cardiac catheterization laboratory, he suffered a perforation of his SVC with pericardial tamponade. After treatment of the perforation and relief of tamponade, he underwent a hybrid procedure to recanalize his SVC.

Midterm results for collaborative treatment of pulmonary atresia with intact ventricular septum.

Background: We report a single-institution experience using a collaborative surgical and catheter-based approach to the initial treatment of pulmonary atresia with intact ventricular septum.

Methods: A retrospective review was conducted of all neonates admitted with pulmonary atresia with intact ventricular septum from 1996 to March 2007.

Results: We identified 24 patients with a mean age at first intervention of 4.