Distribution and protective role of hlab40 in Iranian patients with kawasaki disease; a report from southern Iran

Kawasaki disease [KD] clinically presents as a systemic vasculitis syndrome with significant cardiovascular involvement. With different incidence among different ethnic groups, the role of certain human leukocyte antigens and their products has been considered as a crucial predisposing factor in the immune responses in this disease. We determined the distribution of human leukocyte antigens type B for 90 Iranian patients with Kawasaki disease in order to evaluate a possible association between these antigens and this disease in our area. We used the polymerase chain reaction [PCR] sequence specific primers [PCR-SSP] technique for antigen typing. Distribution of these antigens for 89 healthy Iranians used as control. While 7 [3.9%] of our patients were positive for human leukocyte antigen type B 40, there were 18 [10.1%] subjects from the control group who had this antigen with statistically significant difference between patients and control group [CI= 95%, RR=1.15 and P= 0.02]. Data were analyzed by Pearson chi-square test and Fisher's exact test. SPSS version 15 was used for statistical analysis and a P value less than 0.05 considered statistically significant. The presence of higher frequency of allele type-B40 in the control group may represent a protective role for this antigen with resultant decreased susceptibility to KD in our area

Cardiovascular involvement in children with osteogenesis imperfecta

Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with Osteogenesis imperfecta. The present study aimed to investigate the prevalence of cardiovascular abnormalities in these patients. 24 children with Osteogenesis imperfecta and 24 normal children who were matched with the patients regarding sex and age were studied. In both groups, standard echocardiography was performed, and heart valves were investigated. Dimensions of left ventricle, aorta annulus, sinotubular junction, ascending and descending aorta were measured and compared between the two groups. The results revealed no significant difference between the two groups regarding age, sex, ejection fraction, shortening fraction, mean of aorta annulus, sinotubular junction, ascending and descending aorta, but after correction based on the body surface area, dimensions of aorta annulus, sinotubular junction, ascending and descending aorta in the patients were significantly higher than those in the control group [P<0.05]. Two [8.3%] patients had aortic insufficiency and five [20%] patients had tricuspid regurgitation, three of whom had gradient >25 mmHg and one patient had pulmonary insufficiency with indirect evidence of pulmonary hypertension. According to Z scores of aorta annulus, sinotubular junction and ascending aorta, 5, 3, and 1 out of 24 patients had Z scores >2 respectively. The prevalence of valvular heart diseases and aortic root dilation was higher in children with Osteogenesis imperfecta. In conclusion, cardiovascular investigation is recommended in these children

prevalence of hypomagnesaemia in pediatric patients with mitral valve prolapse syndrome and the effect of Mg therapy

A paucity of data exists regarding the prevalence and relationship of hypomagnesaemia with clinical symptoms of mitral valve prolapse [MVP] in pediatric patients. In this study we evaluated the prevalence of magnesium [Mg] deficiency in pediatric patients with MVP syndrome and attempted to clarify the effect of Mg therapy on alleviating their symptoms. The present study was conducted from April 2010 to January 2012, and included 230 patients [90 males and 140 females] with symptoms of mitral valve prolapse and mean age of 11.6 +/- 3.66. Serum magnesium [Mg] level less than 1.5 mg/dl was defined as hypomagnesaemia. Patients with 2 mm leaflet displacement and maximum leaflet thickness of 5 mm in echocardiography were considered to have classic MVP, while those with leaflet thickness less than 5 mm were considered as non-classic MVP. Patients with hypomagnesaemia were orally treated with 4.5 mg/kg/day Mg chloride for 5 weeks followed by re-evaluation of symptoms of chest pain, palpitation, fatigue and dyspnea. Hypomagnesaemia was found in 19 [8.2%] of 230 patients with mitral valve prolapse. The re-evaluation of patients with Hypomagnesaemia after 5 weeks of Mg therapy, showed statistically significant relief of chest pain [P=0.01]. However, no significant changes was detected in regard to palpitation [P=0.06], fatigue [P= 0.5] and dyspnea [P=0.99]. This study revealed that the prevalence of hypomagnesaemia in pediatric patients with mitral valve prolapse is relatively low compared to adults, but treatment with oral Mg in patient with hypomagnesaemia decreases chest pain

relationship of coronary sinus dilation with pulmonary artery pressure in pediatric patients

Increased right atrial pressure due to pulmonary hypertension may impair venous drainage with resultant coronary sinus dilatation. The aim of this study was to search for correlations between coronary sinus diameter and estimated pulmonary artery pressure in children. In a prospective study, 100 children who were referred consecutively for transthoracic echo car diography were included in this study. Ratios of coronary sinus diameter to weight, body surface area and aortic annulus were calculated for in each patient. Correlation between coronary sinus diameter and estimated pulmonary artery pressure was studied by person correlation. A tricuspid regurgitation peak gradient more than 36 mmHg or pulmonary regurgitation peak gradient more than 25 mmHg were considered as pulmonary hypertension. Sixty-eight of our participants had no pulmonary hypertension and 32 did. Mean age was 7.6 years in the patients without pulmonary hypertension and 8.0 years in the patients with pulmonary hypertension [P=0.11]. Mean coronary sinus diameter to aortic annulus diameter ratio was 0.49 +/- 0.13 in the patient with pulmonary hypertension versus 0.38 +/- 0.12 in the patient without pulmonary hypertension [P<0.001]. The coronary sinus diameter to body surface area ratio was 1.3 +/- 0.59 versus 0.7 +/- 0.28 [P<0.001], and coronary sinus diameter to weight ratio was 0.06 +/- 0.03 versus 0.02 +/- 0.01 [P<0.001]. coronary sinus dilation was documented in pediatric patients with pulmonary hypertension. The ratios of coronary sinus diameter to aortic annulus diameter, body surface area and weight correlated significantly with pulmonary hypertension

Evaluation of pulsed doppler-versus tissue doppler-derived tei index of right and left ventricle in fetuses

The myocardial performance index [MPI], also known as the Tei index, was introduced by Tei et al. to evaluate cardiac function in adults with dilated cardiomyopathy. This index is defined as the sum of isovolumic contraction time [ICT] and isovolumic relaxation time [IRT], divided by ejection time [ET]. To determine the correlation between pulsed Doppler [PD]- and tissue Doppler imaging [TDI]-derived Tei indices in fetuses. Right and left ventricle PD and TDI echocardiographic data were obtained from 59 fetuses [11 pregnant women who were positive for anti-SSA-Ro or anti-SSB-La antibodies, 18 women who were referred due to dysrhythmia, and 30 women who had normal clinical findings]. Mean fetal gestational age was 27 +/- 6.4 weeks. Mean PD Tei index of the mitral and tricuspid valve was 0.58 +/- 0.05 and 0.53 +/- 0.08, respectively. Mean TDI indices for the mitral and tricuspid valve were 0.56 +/- 0.09 and 0.55 +/- 0.08, respectively. There were no significant differences between mitral and tricuspid PD- and TDI-derived Tei indices [P = 0.87, P= 0.21], but the Bland-Altman diagrams showed no fine agreement between the indices [the mean difference +/- 1 standard deviation of the right ventricular PD- and TDI-derived Tei indices was 0.24 +/- 0.02 and 0.29 +/- 0.04 for the left ventricle]. There were no significant differences in PD- and TDI-derived Tei indexes between groups of evaluated fetuses [Mitral valve: PD-Tei P=0.69, TDI-Tei P=0.49; Tricuspid valve: PD-Tei P=0.41, TDI-Tei P=0.36]. Although the mean values of the two indices did not differ significantly, the TDI-derived and PD-derived Tei indices did not have fine agreement

Estimation of right atrial pressure from the inspiratory collapse of the inferior vena cava in pediatric patients

Paucity of data exists between mean right atrial pressure [RAP] and inferior vena cava [IVC] size and collapsibility in pediatric patients with congenital heart disease. In a prospective study, fifty consecutive pediatric patients with different congenital heart diseases who had right side cardiac catheterization were studied, comparing right atrial pressure with simultaneous M-mode echocardiographic measurement of inferior vena cava diameter. Mean age of the patients was 4.96 +/- 4.05 years [30 male and 20 female]. Patients were categorized into two groups according to their right atrial pressure [RAP] as measured by cardiac catheterization: Group 1 [40 patients] were those with mean RAP <8 mmHg and group 2 [10 patients] who had a mean RAP> 8 mmHg. In M-mode echocardiography IVC size was statistically different [P=0.004 and 0.009] in inspiration and expiration in the two groups. Mean RAP was estimated to be > 8 mmHg when IVC diameter in inspiration was >3.6 [sensitivity of 100%, specificity of 47.5%, +LR=1.9] or if IVC diameter was >6mm in expiration [sensitivity of 70%, specificity of 87%, +LR=4.67]. This study showed that measurement of IVC size in inspiration and expiration can be used as a reliable method for estimation of mean right atrial pressure

Relation of mean right atrial pressure to doppler parameters of right atrial and hepatic venous flow in pediatric patients with congenital heart disease

A paucity of data exists regarding the relation of mean right atrial pressure [RAP] to Doppler parameters of right atrial and ventricular filling in pediatric patients with congenital heart disease. Fifty patients [30 male and 20 female] with mean age of 4.96 +/- 4.05 who were admitted in the pediatric cardiology ward of Nemazee Hospital affiliated to Shiraz University of Medical Sciences, were included in this study. Patients were categorized into two groups according to their RAP measured by cardiac catheterization: Group 1 [40 patients] were those with mean RAP <8 mmHg and group 2 [10 patients] who had mean RAP 38 mmHg. Data gathered from hepatic venous flow, tricuspid diastolic flow and pulse tissue Doppler of lateral tricuspid annulus of each patient were then compared with right atrial pressure obtained by cardiac catheterization. If change of peak S wave velocity of hepatic vein in respiration was more than 38%, sensitivity and specificity of a RAP more than 8 mmHg was 90% and 51.3% respectively with likelihood ratio [LR] equal to 1.85; a peak S wave velocity of less than 70 mm/sec also showed a RAP more than 8 mmHg with sensitivity and specificity of 70 and 82.1 respectively [LR=3.9]. A peak expiratory D wave velocity of hepatic vein more than 63 mm/sec was indicator of RAP more than 8 mmHg with sensitivity and specificity of 60% and 92.3% respectively [LR=7.8]. This study showed that hepatic venous flow can be valuable for estimation of mean RAP in pediatric patients with congenital heart disease