Motor development in children 0-2 years pre- and post-LTX, a prospective study.

To determine prospectively gross and fine motor development of children <2 years of age, who undergo LTX. In this prospective study, children aged <2 years who undergo LTX were tested using the motor scale of the Bayley Scales of infant and toddler development, 3rd edition Dutch version. Testing was done during screening pre- and post-LTX: at the time of hospital discharge (2-6 weeks), at 3 months, 6 months, and 1 year. Z-scores were calculated. Twenty-nine children participated in this study, 14 boys, median age 6 months, at screening for LTX. Gross motor skills were delayed pre-LTX (Z-score -1.3). Fine motor skills were normal (Z-score 0.3). Immediately post-LTX, both skills reduced, and at 1 year post-LTX, gross motor skills Z-score was -1.0 and fine motor skills Z-score 0.0. Both gross and fine motor skills Z-scores decline post-LTX and tend to recover after 1 year, gross motor skills to low normal and fine motor skills to normal levels. Monitoring of gross motor development and attention on stimulating gross motor development post-LTX remains important, to enable participation in physical activity and sport for health benefits later in life.

Muscle strength is reduced in children with pulmonary arterial hypertension.

Muscle strength is decreased in adults with pulmonary arterial hypertension (PAH). We aim to investigate muscle strength in children with PAH in relation to a cohort of healthy children, and investigate correlations with disease severity markers. This prospective study included children with PAH aged 4-18 years, who visited the Dutch National Referral Center for Pulmonary Hypertension in Childhood between October 2015 and March 2016. Muscle strength was assessed using handgrip strength and maximum voluntary isometric contractility (MVIC) of four peripheral muscles. Dynamic muscle function was evaluated with the Bruininks-Oseretsky test of motor proficiency (BOT-2). These measurements were compared with those in two cohorts of healthy children and correlated with 6-minute walk distance (6MWD), World Health Organization functional class (WHO-FC), N-terminal pro-brain natriuretic peptide (NT-proBNP), and time since diagnosis. Eighteen children with PAH aged 14.0 [interquartile range: 9.9-16.0] years showed reduced muscle strength. Handgrip strength z-score -2.4 ± 1.2, p < 0.001, total MVIC z-score -2.9 ± 1.2, p < 0.001, and BOT-2 z-score -1.0 ± 0.9, p < 0.001. 6MWD (67 ± 11% predicted) correlated with most muscle measurements (r = 0.49-0.71, p = 0.001). Dynamic muscle function (BOT-2) differed between WHO-FC, whereas handgrip strength and MVIC did not. NT-proBNP and time since diagnosis did not show significant correlations with muscle strength measurements. Muscle strength was significantly reduced in children with PAH and correlated with 6MWD, but not with disease severity markers WHO-FC and NT-pro-BNP. The nature of this reduced muscle strength is yet unclear, but its occurrence in children with seemingly mild or well-controlled PAH supports the concept of PAH being a systemic syndrome involving peripheral skeletal muscles.

Variation in Measurement Results Using Bioimpedance Spectroscopy to Determine Extracellular Fluid of Upper Extremity.

Background: The L-Dex U400 is a recently developed measurement device to aid in the clinical assessment of unilateral lymphedema. Until now, little is known about variation in measurement results of the L-Dex U400. The aim of this study was to determine variation in measurement results in determining extracellular fluid of the arm with the L-Dex U400 under different measurement conditions on 2 days and to determine the reliability of these measurements in healthy subjects. Methods and Results: Thirty participants were included. Participants were measured on 2 days, 2 weeks apart. Each day they were measured six times, 1st basic measurement, 2nd after a 10-minute rest period, 3rd second observer, 4th after drinking 200 mL of coffee and rest for 30 minutes on the examination couch, 5th after 30 minutes of cycling on an exercise bike at 50 W and 50-60 rounds per minute, and 6th after a 10-minute rest period. The variance due to participants was 68% of the total variance and 32% was error variance. Lin's concordance coefficient (CCC), a reliability measure, ranged from 0.935 (first day, 1st and 2nd measurement of observer 1) to 0.517 (first and second day after a 10-minute rest period after cycling). In two CCCs, the lower limits of the 95% confidence interval were higher than 0.750. Repeatability coefficient was smallest for the basic observations on day 2 (4.6) and largest after cycling (8.0). Conclusions: Clinical decision-making based on L-Dex U400 measurements should be regarded with caution because of moderate reliability.