Publications by authors named "Pauline Adnot"

Objectives: To prospectively assess, using polysomnography (PSG), the evolution in obstructive sleep apnea (OSA) in infants with Robin Sequence (RS) during their first year of life and to evaluate the role of PSG in OSA treatment.

Methods: Prospective and longitudinal study conducted in 2 tertiary hospitals (2018-2021). Data from 2 PSG (PSG1 0-3 months of life, PSG2 6-10 months of life) performed in RS infants in different sleep positions/conditions (without treatment: supine [SP]; with treatment: lateral [LP], prone [PP], respiratory support) were analyzed.

View Article and Find Full Text PDF

Aim: We aimed to investigate the developmental outcome of children with Robin sequence (RS) for whom continuous positive airway pressure was the main strategy to release upper airway obstruction.

Methods: We included children with isolated RS or RS associated with Stickler syndrome who were aged 15 months to 6 years. We used the French version of the Child Development Inventory and calculated the developmental quotient (DQ) for eight different domains and the global DQ (DQ-global).

View Article and Find Full Text PDF

Mortality outcomes of children with isolated neonatally operated congenital heart defects (CHDs) born with a low (LBW), moderately low (MLBW) or very-low birthweight (VLBW) remain ambiguous. We searched Medline and Embase (inception until October 2021) and included studies that evaluated early mortality. The risk of bias was assessed using the Critical Appraisal Skills Program cohort checklist.

View Article and Find Full Text PDF
Article Synopsis
  • - The study analyzed infant mortality rates (IMR) in France from 2001 to 2019 using data on births and deaths in the first year of life, finding an average IMR of 3.63/1000, with higher rates for males compared to females.
  • - Key findings included a sharp decrease in IMR until 2005, a slower decrease until 2012, and a notable increase thereafter, particularly among early neonatal deaths.
  • - The authors emphasize the need for urgent investigation into the recent uptick in IMR since 2012 to identify causes and implement corrective measures.
View Article and Find Full Text PDF
Article Synopsis
  • * Of the 20 guidelines reviewed, 41% contained missing statements, and 4% were unclear, with variations in the definition of sentinel injuries and the recommended diagnostic tests.
  • * Overall, while there was general agreement on some procedures, significant discrepancies exist in specific diagnostic evaluations and tests across guidelines, indicating a need for standardization.
View Article and Find Full Text PDF

Background & Aim: The canalicular bile salt export pump (BSEP/ABCB11) of hepatocytes is the main adenosine triphosphate (ATP)-binding cassette (ABC) transporter responsible for bile acid secretion. Mutations in ABCB11 cause several cholestatic diseases, including progressive familial intrahepatic cholestasis type 2 (PFIC2) often lethal in absence of liver transplantation. We investigated in vitro the effect and potential rescue of a BSEP mutation by ivacaftor, a clinically approved cystic fibrosis transmembrane conductance regulator (CFTR/ABCC7) potentiator.

View Article and Find Full Text PDF