Severe labetalol overdose in an 8-month-old infant

To access publisher full text version of this article. Please click on the hyperlink in Additional Links fieldWe report a case of a large labetalol overdose in an eight-month-old infant that was being treated for hypertension following surgery for coarctation of the aorta. Labetalol, both alpha and beta adrenergic blocking agent was used for treating postoperative hypertension. By mistake, the patient was given an extremely high dose of labetalol intravenously (17.2 mg.kg(-1)). Remarkably, the medication error had a surprisingly limited clinical effect on the infant who survived the incident. We discuss the pharmacokinetic, pharmocodynamic and possible explanations for this fortunate turn of events

The Asiatic part of the Russian Empire from the maps published by the Imperial Academy of St. Petersburg with the new discoveries of Captn. Cook &c. [cartographic material].

Map, showing route of Pilot Petuszcoff, with relief shown by hachures.; Prime meridian: Ferro.; Also available in an electronic version via the Internet at: http://nla.gov.au/nla.map-rm1519

Don't forget there's someone in Australia [music] /

H.D.M.P.Co.33. (Publisher number). H.D.M.P.Co.34. (Publisher number). For voice and piano.; No collective t.p., titles transcribed from individual captions.; Pl. no.'s: H.D.M.P.Co.33 ; H.D.M.P.Co.34.; "Featured by Nora Delany"; Printed on recto and verso.; Scores have been physically removed from a larger, unknown volume.; Also available online http://nla.gov.au/nla.mus-vn4464178

Congenital cardiac malformations in Iceland from 1990 through 1999

To access publisher full text version of this article. Please click on the hyperlink in Additional Links fieldINTRODUCTION AND BACKGROUND: About 1% of live-born children have congenital malformations of the heart. The aim of our study was to investigate the incidence of such defects in children born in Iceland during a period of 10 years, extending from 1990 through 1999. MATERIALS AND METHODS: Information about the patients was obtained from medical records from two hospitals that cover the whole country, a private clinic of pediatric cardiologists, an echocardiography database, autopsy reports, and death certificates. We investigated the distribution of specific malformations, the age at diagnosis, the symptoms leading to the diagnosis, the source of referral, and treatment and quality of life. RESULTS: Between 1990 and 1999, there were 44,013 live births in Iceland, of which 740 patients were diagnosed with congenital cardiac malformations, accounting for 1.7% of the live-born children. The distribution was made up of 338 patients with ventricular septal defect (45.7%), 90 with atrial septal defect (12.2%), 85 with patency of the arterial duct (11.5%), 48 with pulmonary valvar stenosis (6.5%), 38 with a bicuspid aortic valve (5.1%), 28 with aortic coarctation (3.8%), 22 with tetralogy of Fallot (3.0%), 14 with transposed great arteries (1.9%), 11 with aortic stenosis (1.5%), 10 with atrioventricular septal defect and common atrioventricular orifice (1.4%), 9 with mitral valvar regurgitation (1.2%), 7 with sub-aortic stenosis (0.9%), and 5 with hypoplasia of the left heart (0.7%). Extracardiac anomalies were seen in 89 patients (12.0%). Chromosomal defects were seen in 36 patients, of whom 28 had Down's syndrome. DISCUSSION: The annual incidence of diagnosis of patients with congenital cardiac malformations increased during the period of study. This was noted for minor defects, but the incidence of the major anomalies did not alter. Our observed yearly incidence, at 1.7%, was higher than noted in a previous study covering the years 1985 through 1989, and is also higher than in other population-based studies. The most likely explanation is the fact that access to pediatric cardiologists in Iceland is very good. Diagnosis, registration, and follow-up are conducted by only a few cardiologists, and take place at a single center for pediatric cardiology

A rare missense mutation in MYH6 associates with non-syndromic coarctation of the aorta.

To access publisher's full text version of this article, please click on the hyperlink in Additional Links field or click on the hyperlink at the top of the page marked DownloadCoarctation of the aorta (CoA) accounts for 4-8% of congenital heart defects (CHDs) and confers substantial morbidity despite treatment. It is increasingly recognized as a highly heritable condition. The aim of the study was to search for sequence variants that affect the risk of CoA. We performed a genome-wide association study of CoA among Icelanders (120 cases and 355 166 controls) based on imputed variants identified through whole-genome sequencing. We found association with a rare (frequency = 0.34%) missense mutation p.Arg721Trp in MYH6 (odds ratio = 44.2, P = 5.0 × 10-22), encoding the alpha-heavy chain subunit of cardiac myosin, an essential sarcomere protein. Approximately 20% of individuals with CoA in Iceland carry this mutation. We show that p.Arg721Trp also associates with other CHDs, in particular bicuspid aortic valve. We have previously reported broad effects of p.Arg721Trp on cardiac electrical function and strong association with sick sinus syndrome and atrial fibrillation. Through a population approach, we found that a rare missense mutation p.Arg721Trp in the sarcomere gene MYH6 has a strong effect on the risk of CoA and explains a substantial fraction of the Icelanders with CoA. This is the first mutation associated with non-familial or sporadic form of CoA at a population level. The p.Arg721Trp in MYH6 causes a cardiac syndrome with highly variable expressivity and emphasizes the importance of sarcomere integrity for cardiac development and function.deCODE genetics/Amgen, Inc