Biochemical predictors of neurocognitive outcomes in Hurler syndrome

2021 ◽  
Vol 132 (2) ◽  
pp. S68
Author(s):  
Troy Lund ◽  
Lynda Polgreen ◽  
Elizabth Braunlin ◽  
Patricia Dickson ◽  
Jakub Tolar ◽  
...  
2019 ◽  
Vol 21 (11) ◽  
pp. 2552-2560 ◽  
Author(s):  
Julie B. Eisengart ◽  
Elizabeth I. Pierpont ◽  
Alexander M. Kaizer ◽  
Kyle D. Rudser ◽  
Kelly E. King ◽  
...  

2018 ◽  
Vol 123 (2) ◽  
pp. S41
Author(s):  
Julie B. Eisengart ◽  
Weston Miller ◽  
Alex Kaizer ◽  
Kyle Rudser ◽  
Kelly King ◽  
...  

2008 ◽  
Author(s):  
Douglas Woods ◽  
John Walkup ◽  
Lawrence D. Scahill ◽  
Alan Peterson ◽  
Sabine Wilhelm ◽  
...  

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yoo Jinie Kim ◽  
Seung Han Shin ◽  
Eun Sun Lee ◽  
Young Hwa Jung ◽  
Young Ah Lee ◽  
...  

AbstractPrematurity, size at birth, and postnatal growth are important factors that determine cardiometabolic and neurodevelopmental outcomes later in life. In the present study, we aimed to investigate the associations between the size at birth and growth velocity after birth with cardiometabolic and neurodevelopmental outcomes in preterm infants. Fifty-six preterm infants born at < 32 weeks of gestation or having a birth weight of < 1500 g were enrolled and categorized into small for gestational age (SGA) and appropriate for gestational age (AGA) groups. Anthropometric and cardiometabolic parameters were assessed at school-age, and the Korean Wechsler Intelligence Scale for Children, fourth edition (K-WISC-IV) was used for assessing the intellectual abilities. The growth velocity was calculated by changes in the weight z-score at each time period. Multivariate analysis was conducted to investigate the associations of growth velocity at different periods with cardiometabolic and neurodevelopmental outcomes. Forty-two (75%) were classified as AGA and 25% as SGA. At school-age, despite the SGA children showing significantly lower body weight, lean mass index, and body mass index, there were no differences in the cardiometabolic parameters between SGA and AGA groups. After adjusting for gestational age, birth weight z-score, weight z-score change from birth to discharge and sex, change in weight z-score beyond 12 months were associated with a higher systolic blood pressure, waist circumference, and insulin resistance. Full-scale intelligent quotient (β = 0.314, p = 0.036) and perceptional reasoning index (β = 0.456, p = 0.003) of K-WISC-IV were positively correlated with postnatal weight gain in the neonatal intensive care unit. Although cardiometabolic outcomes were comparable in preterm SGA and AGA infants, the growth velocity at different time periods resulted in different cardiometabolic and neurocognitive outcomes. Thus, ensuring an optimal growth velocity at early neonatal period could promote good neurocognitive outcomes, while adequate growth after 1 year could prevent adverse cardiometabolic outcomes in preterm infants.


2014 ◽  
Vol 24 (9) ◽  
pp. 274-278 ◽  
Author(s):  
Louise Simcox ◽  
Alexander E.P. Heazell

1992 ◽  
Vol 2 (2) ◽  
pp. 196-199
Author(s):  
Louis Tsun-cheung Chow ◽  
Wing-Hing Chow

SummaryWe studied the cardiac conduction system in a case of Hurler syndrome. There was dense fibrosis of the supporting matrix of the sinus node and accumulation of mucopolysaccharide in the nodal cells. The bundle branches showed prominent hydropic degeneration, being encased and punctuated by dense fibrous tissue. These changes in the conduction system may predispose to the development of arrhythmias, accounting for the sudden deaths in Hurler syndrome.


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