Evaluating the Effect of Prophylactic Acetaminophen in the Prevention of Patent Ductus Arteriosus (PDA) in Premature Neonates: A randomized clinical trial

neonates. The purpose of this study is to determine the efficacy of prophylactic parenteral acetaminophen as a safer alternative drug for PDA in preterm infants. Methods: In a randomized clinical trial carried out in a one-year period, 60 preterm newborns under 30 weeks of gestational age with birth weights under 1500 grams, admitted in neonatal intensive care unit (NICU) of Emam Reza Hospital, Mashhad were studied. The prophylaxis group received parenteral acetaminophen for 3 days. Echocardiography was performed 96 hours after the last given dose and on the 14th day in both groups. Result: There were 30 newborns in each group. In the 4th-day echocardiography, in 33.3% of the prophylaxis group and 26.7% of the control group, the ductus arteriosus was closed (P=0.106). In the 14th-day echocardiography, the ductus was closed in 63% and 41.4% of the intervention and control group, respectively, which was not statistically significant. In addition there was not a significant difference in the ratio of left atrium to aortic root between the two groups. Conclusion: This study showed that in total, PDA was closed in more cases in the intervention group compared to the control group but the difference was not statistically significant. Acetaminophen is a new medicine for PDA closure, which may be more prevalent in future due to its cost effectiveness and safety

Partial Ectopia Cordis: A Case Report

Background: One-third of all major congenital anomalies are Congenital heart disease (CHD) and Reported CHD prevalence increased over time and in Asian countries is more than western countries. Ectopia cordis (EC) is a rare congenital anomaly with an estimated incidence of 1:100 000 live births in developed countries. EC is characterized by abnormal heart placement outside the thorax, mostly on the thoracoabdominal side. This form is often associated with pentalogy of Cantrell.Case report: We report one cases of the ectopia cordis at the Emam Reza Hospital in Mashhad. In this report, a rare case with incomplete pentalogy of Cantrell are described. It was a boy with a large omphalocele with evisceration of the heart. He had normal capillary refill and responded to stimuli. This patient was a male fetus with ectopia cordis with intracardiac anomalies; a large omphalocele with evisceration of the heart; a hypoplastic sternum and rib cage.Conclusion: Prognosis seems to be poorer in patients with the complete form of pentalogy of Cantrell, EC, and patients with associated anomalies. Intracardial defects do not seem to be a prognostic facto

Biallelic loss of LDB3 leads to a lethal pediatric dilated cardiomyopathy

Autosomal dominant variants in LDB3 (also known as ZASP), encoding the PDZ-LIM domain-binding factor, have been linked to a late onset phenotype of cardiomyopathy and myofibrillar myopathy in humans. However, despite knockout mice displaying a much more severe phenotype with premature death, bi-allelic variants in LDB3 have not yet been reported. Here we identify biallelic loss-of-function variants in five unrelated cardiomyopathy families by next-generation sequencing. In the first family, we identified compound heterozygous LOF variants in LDB3 in a fetus with bilateral talipes and mild left cardiac ventricular enlargement. Ultra-structural examination revealed highly irregular Z-disc formation, and RNA analysis demonstrated little/no expression of LDB3 protein with a functional C-terminal LIM domain in muscle tissue from the affected fetus. In a second family, a homozygous LDB3 nonsense variant was identified in a young girl with severe early-onset dilated cardiomyopathy with left ventricular non-compaction; the same homozygous nonsense variant was identified in a third unrelated female infant with dilated cardiomyopathy. We further identified homozygous LDB3 frameshift variants in two unrelated probands diagnosed with cardiomegaly and severely reduced left ventricular ejection fraction. Our findings demonstrate that recessive LDB3 variants can lead to an early-onset severe human phenotype of cardiomyopathy and myopathy, reminiscent of the knockout mouse phenotype, and supporting a loss of function mechanism

Activity of serum paraoxonase 1, lipid profile and atherogenic indexes in diabetic induced rats treated with alpha lipoic acid

Background: Paraoxonase 1 (PON1) is an enzyme attached to high density lipoprotein (HDL-C) which has antioxidant and anti-atherogenic activities. Objectives: In this study, we investigated the effects of alpha lipoic acid on PON1 activity, lipid profile and atherogenic index as well as correlation between PON1 activities and HDL-C in diabetic rats. Materials and Methods: Thirty adult male rats were distributed in three experimental groups in this study. Control (group I), diabetic (group II) and diabetic animals treated with alpha lipoic acid (group III) group. Diabetes mellitus was induced in rats in groups II and III by a single dose of alloxan monohydrate (100 mg/kg; subcutaneous) and then treatment was performed with administration of alpha lipoic acid (100 mg/kg intraperitoneally) in group III for 6 weeks. Blood samples were collected from animals to measure the levels of triglyceride (TG), total cholesterol (TC), low density lipoprotein (LDL-C), HDL-C, PON1 activity and correlation the between HDL-C and atherogenic index by PON1. Results: Statistical analysis showed that alpha lipoic acid significantly (P<0.05) inhibited the increase of TG, TC, LDL-C, VLDL, atherogenic index, atherogenic coefficient (AC), and cardiac risk ratio (CRR) when compared to diabetic rats in group II. HDL-C level was increased by alpha lipoic acid. The activity of PON1 was significantly (P<0.05) decreased in diabetic rats and treatment with alpha lipoic acid increased the PON1 activity. Moreover, the activity of PON1 correlated positively with HDL-C and negatively with AC, CRP 1 and CRP2. Conclusions: This study demonstrated that the administration of alpha lipoic acid can improve PON1 activity, lipid metabolism, atherogenic index and is able to reduce the risk of coronary artery diseases and atherosclerosis in diabetic rats

Comparison of Diagnostic Markers of Aortic Coarctation in Prenatal and Postnatal Echocardiography

Background: The prenatal diagnosis of coarctation of aorta (CoA) remains a major challenge, as the false-positive diagnosis is fairly common. The purpose of this study was to find some useful prenatal sonographic markers compatible with the postnatal diagnosis of CoA. Methods: The study included fetuses suspected of CoA in the second and third-trimester ultrasound tests. All cases were examined by fetal echocardiography at a single ultrasound clinic between 2019 and 2020. The proportion of right and left ventricular size was assessed and ductal/isthmus diameter ratio was measured. A comparison was drawn between the results of neonates with neonatal CoA and neonates without CoA. Results: Of 20 fetuses with suspected prenatal CoA, 3 (15%) had neonatal CoA. The mean ductal/isthmus ratio was significantly higher in the neonates with CoA (1.96 vs. 1.46; p< 0.001). The diagnostic power of ductal/isthmus ratio to detect CoA with a cut point of 1.53 had a sensitivity and specificity of 100% and 70.6%, respectively, a positive and negative predictive value of 37.5% and 100%, respectively, and an overall accuracy of 75%. Conclusion: The ductal/isthmus ratio diameter and ventricular disproportion are significant sonographic markers for the prenatal diagnosis of CoA, and the ductal/isthmus ratio has high sensitivity and specificity compared to postnatal findings

A general analytical solution for fluid flow and heat convection through arbitrary-shaped triangular ducts: A variational analysis

This paper presents an analytical solution for fluid flow and heat transfer inside arbitrarily-shaped triangular ducts for the first time. The former analytical solutions are limited to the special case of isosceles triangular ducts. The literature has no report about the analytical solution for the general case of arbitrarily-shaped triangular ducts. Due to the significant role of fluid flow through non-circular channels in industry and the large number of triangular shapes, a method for solving the heat transfer problem for all triangular shapes is needed. The heat transfer of a fluid flow through a channel with an arbitrary triangular cross-section for the case of constant heat flux at the walls is solved in this work for the first time, considering viscous dissipation. Here, the functionals of flow and heat transfer equations are derived, and the resulting Euler–Lagrange equations are solved using the Ritz method. The effect of the duct geometry on the velocity profile and friction coefficient is studied in detail. The effect of the Brinkman number on the temperature distribution and Nusselt number is investigated for both cooling and heating cases. The results reveal that the critical Brinkman Number distinguishes between the cooling and heating cases and represents the critical point at which the Nusselt number approaches infinity. The value of the Nusselt number decreases with the increase of the Brinkman number in both the wall cooling and heating modes. It is also found that the equilateral triangle exhibits the minimum friction coefficient and the maximum value of the Poiseuille number

Genetic Insights from Consanguineous Cardiomyopathy Families

Inherited cardiomyopathies are a prevalent cause of heart failure and sudden cardiac death. Both hypertrophic (HCM) and dilated cardiomyopathy (DCM) are genetically heterogeneous and typically present with an autosomal dominant mode of transmission. Whole exome sequencing and autozygosity mapping was carried out in eight un-related probands from consanguineous Middle Eastern families presenting with HCM/DCM followed by bioinformatic and co-segregation analysis to predict the potential pathogenicity of candidate variants. We identified homozygous missense variants in TNNI3K, DSP, and RBCK1 linked with a dilated phenotype, in NRAP linked with a mixed phenotype of dilated/hypertrophic, and in KLHL24 linked with a mixed phenotype of dilated/hypertrophic and non-compaction features. Co-segregation analysis in family members confirmed autosomal recessive inheritance presenting in early childhood/early adulthood. Our findings add to the mutational spectrum of recessive cardiomyopathies, supporting inclusion of KLHL24, NRAP and RBCK1 as disease-causing genes. We also provide evidence for novel (recessive) modes of inheritance of a well-established gene TNNI3K and expand our knowledge of the clinical heterogeneity of cardiomyopathies. A greater understanding of the genetic causes of recessive cardiomyopathies has major implications for diagnosis and screening, particularly in underrepresented populations, such as those of the Middle East