Search: WFRF:(Andersson Nadine G) >
Long-term joint out...
Long-term joint outcomes in adolescents with moderate or severe haemophilia A
-
- Schmidt, David E. (author)
- Karolinska Institute,Karolinska University Hospital
-
- Michalopoulou, Aikaterini (author)
- Aghia Sophia Children's Hospital
-
- Fischer, Kathelijn (author)
- University Medical Center Utrecht
-
show more...
-
- Motwani, Jayashree (author)
- Birmingham Children's Hospital
-
- Andersson, Nadine G. (author)
- Lund University,Lunds universitet,Pediatrisk hematologi,Forskargrupper vid Lunds universitet,Paediatric Haematology Research Unit,Lund University Research Groups,Skåne University Hospital
-
- Pergantou, Helen (author)
- Aghia Sophia Children's Hospital
-
- Ranta, Susanna (author)
- Karolinska Institutet,Karolinska Institute,Karolinska University Hospital
-
show less...
-
(creator_code:org_t)
-
- 2022-08-04
- 2022
- English.
-
In: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 28:6, s. 1054-1061
- Related links:
-
http://dx.doi.org/10... (free)
-
show more...
-
https://lup.lub.lu.s...
-
https://doi.org/10.1...
-
http://kipublication...
-
show less...
Abstract
Subject headings
Close
- Introduction: Favourable joint outcomes are expected with modern haemophilia A (HA) management. Evaluation of long-term treatment outcomes is hampered by the delay between bleeding episodes during childhood and resulting joint outcomes in adulthood. Aim: To measure the long-term joint health of adolescents with moderate and severe HA, according to severity and inhibitor status. Methods: Pilot cross-sectional study of five European PedNet centres in moderate and severe HA patients aged 10–19 years. Structured assessment of joint status by physical examination (HJHS) and ultrasound (HEAD-US). Results: In total, 141 HA patients were evaluable, 100 without inhibitors (81 severe, 19 moderate HA), and 41 severe HA with current/past inhibitors. On physical examination, 12/81 (15%) of severe HA without inhibitors, 3/19 (16%) of moderate HA, and 13/41 (32%) of severe HA patients with inhibitors exhibited joint abnormalities. Inhibitor persistence, longer inhibitor duration, and a high peak inhibitor level were associated with impaired joint health. Ultrasound showed joint damage (bone or cartilage) in 13/49 (27%) of severe HA without inhibitors, 1/12 (8%) of moderate HA, and 10/28 (36%) of severe HA patients with inhibitors. A discordant ankle evaluation by ultrasound versus physical examination was present in 53/169 joints (31%). Conclusions: Most adolescents with severe or moderate HA show favourable joint health. Future research with combined ultrasound and/or MRI is needed to better understand joint outcomes in the remaining patients. Patents with inhibitors showed a two-fold increased proportion with joint deterioration. Ultrasound paired with physical examination increases sensitivity for detection of joint damage.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Keyword
- adolescent
- arthropathy
- haemophilia A
- paediatrics
- ultrasonography
Publication and Content Type
- art (subject category)
- ref (subject category)
Find in a library
To the university's database