there are few reports about congenital indifference to pain or hereditary and sensory autonomic neuropathy (hsan). several investigations for pathophysiology of this syndrome have been performed and different classifications about it. in this report we present a case of hsan type ii with general absence of pain and self amputations and leprosy–like damage of extremities which was suspected to b...

background: hypoalbuminemia may be caused by liver disease, nephrotic syndrome, burns, protein-losing entropathy, malnutrition, and metabolic stress. alterations in albumin in metabolic stress such as cardiac surgery have been previously investigated. we studied serum albumin concentration in children with congenital heart disease and also the association of hypoalbuminemia with mortality and m...

background : due to the increased prevalence of celiac disease in chromosomal anomalies and other congenital anomalies, this study was conducted to evaluate the seroprevalence of celiac disease (cd) in patients with congenital heart defects (chd). methods: this case-control study was done on 1002 children in two groups of chd patients (n=402) and controls (n=600). the serum tissue transglutamia...

catheter ablation is an accepted, highly effective modality of treatment for cardiac arrhythmias in children. the success rate depends on the operator’s experience, especially in cases involving complex anatomies. we hereby report our recent experience of successful ablation of accessory pathways in three children with complex congenital heart diseases. the first case was a 7-year-old girl with...

background the prevalence of celiac disease (cd) is remarkably varied in down syndrome(ds)patientscompared with other diseases.  this study aimed to assess celiac disease prevalence in down syndrome children with and without congenital heart defects (chd) and its comparison with controls. materials and methods this case-control study was performed at a single center on 132 participants in three...

background: there is some evidence indicating improvement in myocardial performance after atrial septal defect closure, either device closure or surgical, but ventricular dyssynchrony has not been evaluated before and after surgical closure. the aim of this study was to evaluate ventricular mechanical dyssynchrony in patients with atrial septal defect before and after surgical closure. methods:...

background: brain type natriuretic peptide (bnp) is a cardiac hormone that is secreted mainly by the ventricles in response to volume expansion and pressure load. it can predict post-operative complications after heart surgery in adults. we sought to investigate the prognostic value of bnp in children after heart surgery. methods: we measured the bnp serum levels in 96 children with congenital ...

Partial muscular inter-ventricular septal defect (VSD) or Pacman heart is a rare congenital or occasionally acquired anomaly. Concurrent Pacman heart and Shone's complex are extremely rare and have never been reported until now. We described a 37-year-old male patient with congenital Pacman heart, flail mitral valve (FMV), and a history of multiple congenital anomalies, including subvalvular ao...

aortopulmonary window (apw) is a rare congenital malformation. it results from an incomplete division between the ascending aorta and the pulmonary artery. we describe a 26-year-old male, who presented with a grade ii exertional dyspnea and palpitations. echocardiography revealed an apw with an ascending aorta aneurysm. he underwent surgery under cardiopulmonary bypass without aortic cross-clam...

conclusions surgical repair of the congenital mv disease yields acceptable early and intermediate-term satisfactory valve function and good survival at intermediate-term follow-up. strong predictors for poor surgical outcome and death were age smaller than 1 year, weight smaller or equal than 6 kg, and associated cardiac anomalies such as pulmonary stenosis. objectives the aim of this study was...