http://hdl.handle.net/2440/5866 2013-05-23T09:30:42Z http://hdl.handle.net/2440/77667 Title: Hypertrophic cardiomyopathy with cardiac rupture and tamponade caused by congenital disorder of glycosylation type Ia Author: Rudaks, Laura Ivete; Andersen, Chad Campion; Khong, Teck Yee; Kelly, Andrew John; Fietz, Michael James; Barnett, Christopher Patrick Abstract: Hypertrophic cardiomyopathy (HCM) is a rare presenting feature of congenital disorder of glycosylation type Ia (CDG-Ia). We report two female siblings with CDG-Ia and cardiomyopathy. Patient no. 1 died at 12 days of age from cardiac rupture and tamponade, which has not previously been reported in CDG-Ia. The second patient died at 2 months of age from HCM. The severe cardiac manifestations seen in our patients emphasize the importance of early cardiac assessment in all patients with CDG-Ia. 2011-12-31T13:30:00Z http://hdl.handle.net/2440/77633 Title: Respiratory hospitalisation of infants supplemented with docosahexaenoic acid as preterm neonates Author: Atwell, Kerryn; Collins, Carmel Teresa; Sullivan, Thomas Richard; Ryan, Philip; Gibson, Robert Alan; Makrides, Maria; McPhee, Andrew John Abstract: AIM: To determine the effect of neonatal docosahexaenoic acid (DHA) supplementation in preterm infants on later respiratory-related hospitalisations. METHODS: We enrolled 657 infants in a multicentre, randomised, controlled trial designed to study the long-termefficacy of higher dose dietary DHA in infants born <33 weeks’ gestation. Treatment was with high DHA (~1%) compared with standard DHA (~0.3%) in breast milk or formula, given from the first week of life to term equivalent. Parent-reported hospital admissions to 18 months corrected age were recorded. The proportion of children hospitalised for lower respiratory tract (LRT) conditions and the mean number of hospitalisations per infant were determined. RESULTS: Twenty-three per cent (154/657) of infants were hospitalised for LRT conditions. Seventy-three per cent (173/238) of admissions were for bronchiolitis. There was no significant effect of higher DHA on the proportion of infants admitted for LRT conditions (high DHA 22% vs. standard DHA 25%, adjusted relative risk 0.92, 95% confidence interval (CI) 0.68–1.24, P = 0.57) or in the mean number of admissions per infant (high DHA 0.34, standard DHA 0.38, adjusted ratio of means 0.91, 95% CI 0.63–1.32, P = 0.62). The sexes responded differently to treatment (interaction P = 0.046), with reduced admissions in boys given high DHA, but this was not statistically significant (high DHA 19%, standard DHA 28%, adjusted relative risk 0.69, 95% CI 0.46–1.04, P = 0.08). CONCLUSIONS: Hospitalisation for LRT problems in the first 18 months for preterm infants was not reduced by neonatal supplementation with 1% DHA. 2012-12-31T13:30:00Z http://hdl.handle.net/2440/77582 Title: Child abuse Author: Donald, Terence George 2011-12-31T13:30:00Z http://hdl.handle.net/2440/77581 Title: Atopy Author: Gold, Michael Steven 2011-12-31T13:30:00Z