Titre
Bone Health and Linear Growth in Children with Familial Hypoparathyroidism Treated with Human Parathyroid Hormone 1-34.
Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Auteur(s)
Winer, K.K.
Auteure/Auteur
Zemel, B.S.
Auteure/Auteur
Kelly, A.
Auteure/Auteur
Reynolds, J.C.
Auteure/Auteur
Pechacek, J.
Auteure/Auteur
Webb, T.
Auteure/Auteur
Hans, D.
Auteure/Auteur
Lionakis, M.S.
Auteure/Auteur
Kalkwarf, H.J.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
1945-7197
Statut éditorial
Accepté (sous presse)
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: aheadofprint
Publication Status: aheadofprint
Résumé
Our study explores the impact of human PTH 1-34 injections (PTH therapy) on growth, areal bone mineral density (BMD), and bone quality (measured by trabecular bone score, TBS) in hypoparathyroidism due to autoimmune polyendocrine syndrome type 1 (APS-1) or an activating variant of the calcium sensing receptor (CaR).
To assess associations of 1) age and PTH therapy duration with age-standardized Z-scores for height (HAZ), BMD (BMD-Z), and TBS (TBS-Z) in CaR or APS-1, and 2) APS-1 disease severity with BMD-Z and TBS-Z.
This secondary analysis pooled linear growth and lumbar spine (LS) DXA data from studies of hypoparathyroidism with mean baseline age of 13.7±5.5y. Comparing the two diagnostic etiologies (18 APS-1 and 9 CaR), we examined the impact of age and PTH duration on HAZ, LS-BMD-Z, and LS-TBS-Z using longitudinal mixed-effects modeling.
During PTH therapy, mean HAZ remained below zero in the APS-1 group at all ages, whereas HAZ increased in the CaR group (age by group interaction p<0.0001). Mean LS-BMD-Z were normal (BMD-Z:0±1) for both groups. Mean LS-TBS-Z were near or above zero and differed by group; CaR showed an upward trajectory according to time on PTH whereas the APS-1 group maintained a LS-TBS-Z of approximately 0 (time by group interaction p=0.02). The APS-1 group with greater disease severity (≥7 manifestations) had lower LS-BMD-Z and LS-TBS-Z compared to the less severe APS-1 or CaR groups.
Our study highlights distinct growth and BMD patterns in APS-1 and CaR and underscores the need for careful monitoring and tailored treatment strategies to optimize growth and bone health.
To assess associations of 1) age and PTH therapy duration with age-standardized Z-scores for height (HAZ), BMD (BMD-Z), and TBS (TBS-Z) in CaR or APS-1, and 2) APS-1 disease severity with BMD-Z and TBS-Z.
This secondary analysis pooled linear growth and lumbar spine (LS) DXA data from studies of hypoparathyroidism with mean baseline age of 13.7±5.5y. Comparing the two diagnostic etiologies (18 APS-1 and 9 CaR), we examined the impact of age and PTH duration on HAZ, LS-BMD-Z, and LS-TBS-Z using longitudinal mixed-effects modeling.
During PTH therapy, mean HAZ remained below zero in the APS-1 group at all ages, whereas HAZ increased in the CaR group (age by group interaction p<0.0001). Mean LS-BMD-Z were normal (BMD-Z:0±1) for both groups. Mean LS-TBS-Z were near or above zero and differed by group; CaR showed an upward trajectory according to time on PTH whereas the APS-1 group maintained a LS-TBS-Z of approximately 0 (time by group interaction p=0.02). The APS-1 group with greater disease severity (≥7 manifestations) had lower LS-BMD-Z and LS-TBS-Z compared to the less severe APS-1 or CaR groups.
Our study highlights distinct growth and BMD patterns in APS-1 and CaR and underscores the need for careful monitoring and tailored treatment strategies to optimize growth and bone health.
PID Serval
serval:BIB_FBEB6D466709
PMID
Date de création
2025-02-03T13:55:22.199Z
Date de création dans IRIS
2025-05-21T05:52:19Z