primary immunodeficiencies (pid) are a group of disorders, characterized by an unusual susceptibility to infections. delay in diagnosis results in increased morbidity and mortality in affected patients. the purpose of this study was to determine the mortality rate of iranian immunodeficient patients referred to children medical center hospital affiliated to tehran university of medical sciences...

chondroectodermal dysplasia (ellis-van creveld syndrome) is a rare autosomal recessive congenital abnormality. this syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. it is imperative to not overlook the cardiac complications in patients with this syndrome during den...

OBJECTIVES We sought to investigate whether patients with syndrome X have an abnormal perception of cardiac pain. BACKGROUND Previous studies have reported an increased sensitivity to potentially painful cardiac stimuli in patients with syndrome X. However, it is not clear whether this increase is due to an increased perception of pain or to an enhanced tendency to complain. METHODS We asse...

Background Down syndrome is the most common chromosomal anomaly. Dysmorphic features can occur in several organs in this syndrome. Cardiac anomalies with a prevalence of 50% are the most common anomalies responsible for death during the first two years of life. We aimed to determine the prevalence of cardiac anomalies among Down syndrome patients admitted to two tertiary hospitals in Kermanshah...

In cardiac syndrome X, the presence of ischaemic-like ST segment changes during chest pain, in the absence of epicardial coronary stenoses, suggests that myocardial ischaemia caused by coronary microvascular dysfunction is responsible for angina. This view is supported by the documentation of abnormalities in myocardial perfusion on radionuclide studies 3 and abnormal coronary blood flow respon...

introduction turner syndrome is the most common chromosomal abnormality in females. girls with turner syndrome have an abmormal or missing x choromosme that causes short stature, ovarian failure, renovascular and cardiac anomalies. it has been shown that there is a tendency to obesity in the turner syndrome. this prospective study has been done to evaluate whether body mass index (bmi) is diffe...

What is interesting in a patient with chest pain and normal coronary arteries on the angiogram? Not much for the Y factor, especially as there is not much we can see, investigate, measure or treat. The publication by Vermeltfoort et al. in this issue is very reassuring [1]. The group in Tilburg in collaboration with the VU University Medical Center in Amsterdam did an excellent job in highlight...

Cardiac syndrome-X is a clinical entity characterized by angina-like chest discomfort that is often prolonged in duration, normal coronary arteries as assessed by arteriography, non-inducible coronary vasospasm with ergonovine provocation and ST segment depression on treadmill exercise testing. The pathogenesis of cardiac syndrome-X has been ascribed to myocardial ischaemia that may be caused b...