Oral tadalafil was administered easily and tolerated well and improved mean pulmonary artery pressure (MPAP) in children with PAH, which suggests that oral tadalafil may be more effective and safer than sildenafil in the treatment of PAH.

Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD), skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6) in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH) was performed and the result was: 46.XX, ish del (7q11.2) (ELN X1) (7q22 X2) ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

Infective endocarditis (IE) is an important clinical disease in children with a mortality rate of 11.6%. Prophylaxis with antibiotics is one of the most commonly used methods in children at risk of IE; therefore, the evaluation of antibiotic resistance seems necessary in view of its increasing trend. This study aimed to determine the antibiotic susceptibility pattern of oral viridans group streptococci (VGS) isolated from the dental plaque of children at risk of IE. Fifty-one plaque samples were obtained from children aged 3 to 12 years old in the period from April to July 2018. Samples were obtained with sterile swabs and were transferred to the laboratory in Brain Heart Infusion (BHI) Broth. Samples were immediately cultivated on Columbia blood agar. After identifying VGS, antimicrobial susceptibility test (AST) was performed using Mueller-Hinton agar (MHA) with sheep's blood and E-test strips for selected antibiotics. The minimum inhibitory concentration (MIC) was determined for each isolate and the results were reported as sensitive, intermediate and resistant. Fifty-one VGS bacteria were isolated from children with an average age of 7.3 ± 2.5 years. The highest resistance was observed for azithromycin in 36 (70.6%) isolates and then cefazolin in 35 (68.6%) isolates. The highest susceptibility was observed for amoxicillin in 46 (90.2%) isolates. Based on the findings of this study, amoxicillin is the most effective option for prophylaxis in children. Furthermore, cefazolin should be used with caution because bacteria resistant to this antibiotic can transfer resistance genes to other bacteria.

Introduction: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare Cardiovascular Disease presented with an incidence of 1:300000 live births. Case Presentation: In this manuscript, four cases of ALCAPA in infancy were described. Two infants were presented with respiratory distress and two with heart a murmur. Their coronary artery was derived from the pulmonary artery. Two of them died, one after surgical procedure and one before surgical intervention. Of remained cases, one of them had a good surgical outcome and another was missed in follow up. A brief review was done on case reports of ALCAPA in children. Eighteen manuscripts were found including 201 pediatric cases. Conclusions: The vast majority of affected children will die within the first year of life if ALCAPA is not diagnosed and treated correctly.

Background: Methylmalonate-semialdehyde Dehydrogenase Deficiency (MMSDHD) is an uncommon autosomal recessive disorder. MMSDH is an enzyme encoded by the protein coding gene ALDH6A1 in humans. Case Presentation: We present a 4-year-old boy with elevated liver enzymes, 3-hydroxyisobutyric aciduria (MMSDHD) and cardiac symptoms. He had a mutated ALDH6A1 gene, c.184c>G (p.Pro62Ala). Conclusions: This is one of the rare case reports in the world and the first one in Iran that reports MMSDHD with cardiac disease.

No abstract

Background and Aim: Liver directed therapy utilizing yttrium-90 microspheres represents a recently introduced in situ multidisciplinary cancer therapy that has caught the attention of many physicians faced with the challenges of treating these complex patients. Radioactive particle in capillary liver system were assessed through the in vivo test and the leakage to lung or other organ was defined. The concentration of Y-90 in liver tumor without leakage to the other organ was seen which has the important role in radioactive drug successes by radioimbolization method.