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Hamostaseologie 2023; 43(03): 208-214
DOI: 10.1055/a-1972-8983
Original Article

Osteoporosis Remains Constant in Patients with Hemophilia—Long-Term Course in Consideration of Comorbidities

Anna C. Strauss‡   *
1   Department of Diagnostic and Interventional Radiology, University Hospital Bonn, Bonn, Germany
,
Pius Muellejans‡   *
2   Department of Orthopaedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany
,
Sebastian Koob
2   Department of Orthopaedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany
,
Georg Goldmann
3   Institute of Experimental Haematology and Transfusion Medicine, University Clinic Bonn, Bonn, Germany
,
Peter H. Pennekamp
2   Department of Orthopaedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany
,
Thomas A. Wallny
2   Department of Orthopaedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany
,
Johannes Oldenburg
3   Institute of Experimental Haematology and Transfusion Medicine, University Clinic Bonn, Bonn, Germany
,
Andreas C. Strauss
2   Department of Orthopaedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany
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Abstract

Introduction Patients with hemophilia (PWHs) suffer from an increased risk of osteoporosis. Multiple hemophilia and hemophilic arthropathy associated factors correlate with a low bone mineral density (BMD) in PWHs. The aim of this study was to assess the long-term development of BMD in PWH as well as to analyze potentially influencing factors.

Methods A total of 33 adult PWHs were evaluated in a retrospective study. General medical history, specific-hemophilia-associated comorbidities, joint status using the Gilbert score, calcium level, and vitamin D level as well as at least two results of bone density measurements with a minimum range of 10 years per patient were taken into account.

Results The BMD did not change significantly from one point of measurement to the other. A total of 7 (21.2%) cases of osteoporosis and 16 (48.5%) cases of osteopenia were identified. The two following significant correlations could be revealed: the higher the patients' body mass index, the higher their BMD (r = 0.41; p = 0.022). Moreover, a high Gilbert score came along with a low BMD (r = −0.546; p = 0.003).

Conclusion Even if PWHs frequently suffer from a reduced BMD, our data suggest that their BMD remains constant on a low level in the course of time. A risk factor of osteoporosis often found in PWHs is a vitamin D deficiency and joint destruction. Therefore, a standardized screening of PWHs on BMD reduction by collecting vitamin D blood level and assessing joint status seems appropriate.

Zusammenfassung

Einleitung Hämophilie-Patienten leiden an einem erhöhten Osteoporose-Risiko. Verschiedene Hämophilie- und Arthropathie-bedingte Faktoren korrelieren mit der Minderung der Knochendichte (BMD: Bone Mineral Density). Das Ziel dieser Studie war es, den Langzeitverlauf der Knochendichte von Hämophilie-Patienten und mögliche Einflussfaktoren darauf zu untersuchen.

Methode In einer retrospektiven Studie wurden 33 erwachsene Hämophilie-Patienten untersucht. Allgemeine und Hämophilie-spezifische Erkrankungen, der Gelenkstatus gemessen mit dem Gilbert-Score, Kalzium- und Vitamin D-Werte und zwei Knochendichtemessungen mit einem Mindestabstand von zehn Jahren wurden erfasst.

Ergebnisse Die Knochendichte änderte sich zwischen dem ersten und zweiten Messpunkt nicht signifikant. Bei 7 (21,2%) Probanden wurde eine Osteoporose und bei 16 (48,5%) Probanden eine Osteopenie diagnostiziert. Es konnten zwei signifikante Zusammenhänge ermittelt werden: Je höher der Body Mass Index (BMI) war, desto höher war die Knochendichte (r = 0.41; p = 0.022) und je höher der Gilbert Score war, desto niedriger war die Knochendichte (r = −0.546; p = 0.003).

Schlussfolgerung Auch wenn Hämophilie-Patienten häufig an einer reduzierten Knochendichte leiden, lässt diese Studie darauf schließen, dass die Knochendichte über die Zeit auf einem erniedrigten Level konstant bleibt. Da ein Vitamin D-Mangel und Arthropathien als Risikofaktoren für das Auftreten einer Osteoporose häufig bei Hämophilie-Patienten gefunden werden, sollten regelmäßige Screenings des Vitamin D-Spiegels und des Gelenkstatus erfolgen, um bei den betroffenen Patienten anhand der Ergebnisse ggf. die Knochendichte mittels DXA zu bestimmen.

Keywords

hemophilia - osteoporosis - osteopenia - follow-up

Schlüsselwörter

Hämophilie - Osteopenie - Osteoporose - Langzeit follow-up

* These authors contributed equally.




Publikationsverlauf

Eingereicht: 13. Juni 2022

Angenommen: 01. November 2022

Artikel online veröffentlicht:
02. März 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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