https://pubmed.ncbi.nlm.nih.gov/33346571/ Biliary atresia

J Pediatr Gastroenterol Nutr. 2020 Dec 17;Publish Ahead of Print. doi: 10.1097/MPG.0000000000003021. Online ahead of print.

Early Motor Repertoire in Infants with Biliary Atresia: A Nationwide Prospective Cohort Study

Lyan H Rodijk 1 , Arend F Bos, Henkjan J Verkade, Ruben H de Kleine, Jan B F Hulscher, Janneke L M Bruggink

Abstract

Objectives: To assess the neurological status in infants with biliary atresia (BA) at time of diagnosis, using Prechtl's validated General Movement Assessment (GMA).

Methods: Infants diagnosed with BA were prospectively included in a nationwide cohort study. From birth to approximately 46 weeks of postmenstrual age (PMA), General Movements (GMs) are defined as 'writhing movements'. At 46 to 49 weeks PMA, 'fidgety movements' emerge. The infant's early motor repertoire was recorded on video before Kasai portoenterostomy. We scored GM Optimality Scores (GMOS, min-max 5-42) or Motor Optimality Scores (MOS, min-max 5-28) as appropriate. We defined GMOS <36 and MOS <26 as atypical, and compared the results with two reference groups of healthy peers.

Results: We assessed GMs in 35 infants with BA (11/35 boys, gestational age 40 weeks [36-42], birth weight 3370 gram [2015-4285]). At time of diagnosis (PMA 47 weeks [42-60]), 16 infants (46%) showed atypical GMs. The proportion of infants with atypical GMs was significantly higher in BA (46%) than in two reference groups of healthy infants (vs 10%, p<0.001; vs 18%, p < 0.001). Total and direct bilirubin levels were 165 μmol/L [87-364] and 134 μmol/L [72-334], respectively, height Z-score was 0.05 [-2.90, 1.75], weight Z-score -0.52 [-2.50, -0.20] and MUAC Z-score -1.80 [-2.50, -0.20]. We found no statistically significant relation between atypical GMs and clinical variables.

Conclusions: Almost half of the infants with BA showed atypical GMs at time of diagnosis, suggesting neurological impairment. Close monitoring of these infants is warranted to determine their individual neurodevelopmental trajectories.