Publications by authors named "A Besancon"

Background: Highly effective CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor (ETI), herald a new era in therapeutic strategy of cystic fibrosis (CF). ETI impact on glucose tolerance remains controversial.

Methods: All the participants underwent a baseline oral glucose tolerance test (OGTT) before ETI initiation (M0) and 12 months (M12), and at 24 months if possible.

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Objective: Adreno CorticoTropic Hormone (ACTH)-independent Cushing's syndrome (CS) in children is very rare but potentially fatal. In bilateral nodular hyperplasia, synchronous bilateral adrenalectomy (SBA) represents the definitive treatment to correct hypercortisolism. We aim to report the multidisciplinary management of this rare condition.

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Article Synopsis
  • Generalized arterial calcification of infancy (GACI) is a rare genetic disorder caused by mutations in the ENPP1 and ABCC6 genes, leading to abnormal mineralization in arteries and soft tissues, resulting in serious cardiovascular issues.
  • Nearly 50% of affected infants do not survive past six months due to these complications, highlighting the severe prognosis linked to early diagnosis.
  • Current treatment focuses on symptom management with options like bisphosphonates and requires ongoing support from specialized medical teams, while research aims to develop targeted therapies to correct the underlying causes of the disease.
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Objective: Adamantinomatous craniopharyngioma mainly affects children. Excessive weight gain is a major long-term complication. The primary objective of this study was to assess long-term weight changes in children treated for craniopharyngioma.

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Objective: Isolated childhood growth hormone deficiency (GHD) can persist into adulthood, and re-testing at the transition period is needed to determine whether continued growth hormone therapy is indicated. Here, our objective was to identify predictors of permanent GHD.

Design: Retrospective single-centre study of patients with childhood-onset GHD who were re-tested after adult height attainment.

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