Growth dynamics of transversal body dimensions and proportions, with related clinical determinants in children with X-linked hypophosphatemia treated with phosphate supplements and active vitamin D
Publikation: Beitrag in Fachzeitschrift › Forschungsartikel › Beigetragen › Begutachtung
Beitragende
- Klinik und Poliklinik für Kinder- und Jugendmedizin
- Medizinische Hochschule Hannover (MHH)
- Universität zu Köln
- Westfälische Wilhelms-Universität Münster
- Universität zu Lübeck
- Universitätsklinikum Jena
- Universität Heidelberg
- Asklepios Klinik Hamburg Nord-Heidberg
- Universitätsmedizin Mainz
- Barbara Children’s Hospital
- Christliches Kinderhospital Osnabrück
- Universitätsklinikum Augsburg
- Kinder- und Jugendkrankenhaus Auf der Bult
- Ernst-Moritz-Arndt-Universität Greifswald
- Evangelisches Klinikum Bethel (EvKB)
- KiPraHi - Kinderpraxen Hirschaid
- Universität Rostock
- Universitätsklinikum Tübingen
- Georg-August-Universität Göttingen
- Universitätsklinikum Carl Gustav Carus Dresden
- Ammersee Ärzte Diessen
- Universität Zürich
- Endokrinologikum Hamburg
- Universitätsklinikum der Friedrich-Alexander-Universität Erlangen-Nürnberg
- Zentrum für Kinder- und Jugendendokrinologie
- Universitätsklinikum Schleswig-Holstein Campus Kiel
- Helios Klinikum Schwerin
- Klinikum Bremen-Mitte
- Klinikum Bremen-Nord
- Universitätsklinikum Magdeburg
- Pediatric Practice Rostock - Endocrinology and Diabetology
- Klinikum Stuttgart
- University of Zagreb
- Charité – Universitätsmedizin Berlin
Abstract
Background: Children with X-linked hypophosphatemia (XLH) present with rickets, leg deformities, and growth failure. Bone stability depends on balanced bone growth in both length and width. Data on body proportions, including transverse body dimensions, in children with XLH treated with phosphate supplements and active vitamin D are lacking. Methods: Six major transverse body dimensions of the trunk and extremities, and the frame index (FI), i.e., ratio between bicondylar humerus diameter and height, were measured annually along with clinical characteristics in 109 pediatric patients with XLH, all on supplementation therapy, participating in a prospective multicenter observational study conducted since 1998. Associations between anthropometric and clinical parameters were investigated using linear mixed-effects models. Results: Children with XLH exhibited persistent hypophosphatemia and elevated alkaline phosphatase z scores despite supplementation treatment. This was associated with disproportionate transversal skeletal growth, which was most pronounced during adolescence (13–17 years). Bicondylar diameter z scores (tubular bone width) and FI progressively increased with age (each p < 0.05). In addition, FI was identified as a superior indicator of stunting when compared to other measures of transversal dimensionality across all age groups. In young children (2–6 years), transversal growth was most synchronized and associated most strongly with clinical characteristics. Conclusions: Our data show disproportionate growth in transversal body dimensions despite supplementation treatment in children with XLH, suggesting compensatory widening of tubular bones as adaptation for mineral loss caused by persisting rickets. The FI can be used as a general indicator of bone health in children with XLH in clinical practice and trials.
Details
| Originalsprache | Englisch |
|---|---|
| Seiten (von - bis) | 3187-3200 |
| Seitenumfang | 14 |
| Fachzeitschrift | Pediatric nephrology |
| Jahrgang | 40 |
| Ausgabenummer | 10 |
| Publikationsstatus | Veröffentlicht - Okt. 2025 |
| Peer-Review-Status | Ja |
Externe IDs
| PubMed | 40493262 |
|---|
Schlagworte
ASJC Scopus Sachgebiete
Schlagwörter
- Alkaline phosphatase, Body disproportion, Frame index, Phosphate, Rickets, Transversal body dimension, XLH