Notes

Summary of in-capillary enzymatic reactions employing capillary electrophoresis.
Her initial serum salicylate level was significantly increased at 78.1 mg/dl (upper therapeutic limit, 19.9 mg/dl). She recovered completely following treatment with oral activated charcoal, intravenous sodium bicarbonate, and potassium replacement. CONCLUSIONS This case demonstrates that physicians should consider salicylate toxicity as a possible cause of exacerbation of neurological deficit in elderly patients.Mevalonate kinase deficiency (MKD) is a rare autosomal recessive autoinflammatory disease caused by mutations in MVK. We report two siblings with MKD, presenting with recurrent febrile illnesses, detected to have compound heterozygous variants in MVK. MKD mimics common pediatric conditions and should be considered as a differential diagnosis.Multiple cardiac masses were incidentally detected in a neonate on twelve day of life. Failure to thrive, feeding difficulty and severe dynamic right ventricular outflow tract obstruction developed at 7 months of age. Surgical resection of intracardiac masses relieved symptoms and histological studies confirmed rhabdomyoma. Progressive increase in the size of rhabdomyoma during infancy is an uncommon presentation and surgery can be life-saving.Among 893 adolescent school children from 31 schools, 78.9% were found to have normal body weight; prevalence of thinness and overweight were 8.1% and 13%, respectively. Selleckchem Neratinib Compared to the National reference, 95th percentile value of Body Mass Index was higher; while both weight and height were lower. Literacy, economic and physical activity status were the most significant predictors influencing nutritional status.Antibiotic prescriptions in 227 patients with acute group A b-hemolytic streptococcal pharyngitis in the emergency department were studied. Antibiotic prescription was inappropriate in 42% of the cases, especially due to errors in the prescription of amoxicillin. Probably the use of low-spectrum penicillins would improve this percentage.This cross-sectional study assessed distribution and pattern of echocardiography confirmed congenital heart disease, among 593 pediatric patients in outpatient departments of a tertiary care hospital in eastern India. Commonest defects were ventricular septal defect (43, 40.7%), atrial septal defect (241, 31.7%), and tetralogy of Fallot (125, 21%).Synthetic Antisense oligonucleotides (ASOs) are novel and efficient laboratory tools to regulate the expression of specific genes, and have only recently come into clinical use. These are synthetic single-stranded DNA analogs, whose sequence is complementary to a target nucleotide and alter protein synthesis by several mechanisms. We herein provide a primer on the topic for pediatricians, as this group of drugs is likely to see many more drugs for previously incurable diseases.Treatment of congenital adrenal hyperplasia (CAH) requires lifelong replacement of glucocorticoids with regular follow up to manage associated morbidities. The current review focuses on follow-up and management of infants diagnosed with classical CAH pertinent to Indian context. Early initiation of oral hydrocortisone in divided doses is recommended after diagnosis in newborn period, infancy and childhood. Fludrocortisone is recommended for all infants with classical CAH. All infants should be monitored as per protocol for disease and treatment related complications. The role of prenatal steroids to pregnant women with previous history of CAH affected infant for prevention of virilization of female fetus is controversial.JUSTIFICATION A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.OBJECTIVE To investigate the prevalence and risk factors of congenital heart disease in Yunnan, China which has diverse ethnic groups. METHODS This cross-sectional study enrolled 244,023 children from 2010 to 2015. To diagnose CHD, a conventional physical examination was used to screen suspicious cases, which were further confirmed by echocardiography. RESULTS A total of 1695 children were diagnosed with CHD. The estimated prevalence was 6.94%. Atrial septal defects were the most common cardiac abnormalities. A higher prevalence of CHD was observed with preterm birth, low birth weight, maternal age ≥35 years, and high-altitude regions. The prevalence also showed differences between diverse ethnic groups. CONCLUSIONS The prevalence of CHD in China may have ethnic differences.OBJECTIVE To identify the factors influencing brain injury in infants with congenital heart disease (CHD) after cardiac surgery. METHODS This retrospective study investigated 103 infants with CHD undergoing cardiac surgery between January 2013 and February 2016. Pre- and postoperative amplitude-integrated electroencephalography (aEEG) recordings were assessed for background pattern, sleep-wake cycle pattern and seizure activity. Logistic regression model was used to determine the influencing factors of brain injury. RESULTS Pre-operatively, most infants in our study exhibited a normal background pattern, with 16.5% showing discontinuous normal voltage, whereas this pattern was observed in only 7.8% of infants postoperatively. The improvement in background pattern after surgery was significant (P less then 0.05) in infants at no more than 39 weeks of gestational age. Infants with postoperative sepsis or severe postoperative infection were prone to show a worse sleep-wake cycle pattern after heart surgery. CONCLUSIONS The improvement in brain function of infants with CHD after cardiac surgery was associated with the gestational age and postoperative infection.
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