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Erschienen in: Orphanet Journal of Rare Diseases 1/2019

Open Access 01.12.2019 | Correction

Correction to: Natural history of fibrodysplasia ossificans progressiva: cross-sectional analysis of annotated baseline phenotypes

verfasst von: Robert J. Pignolo, Geneviève Baujat, Matthew A. Brown, Carmen De Cunto, Maja Di Rocco, Edward C. Hsiao, Richard Keen, Mona Al Mukaddam, Kim-Hanh Le Quan Sang, Amy Wilson, Barbara White, Donna R. Grogan, Frederick S. Kaplan

Erschienen in: Orphanet Journal of Rare Diseases | Ausgabe 1/2019

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The original article can be found online at https://​doi.​org/​10.​1186/​s13023-019-1068-7
Corretion to: Orphanet J Rare Dis (2019) 14:98
https://doi.org/10.1186/s13023-019-1068-7
The original version of this article [1] unfortunately included an error to an author’s name. Author Maja Di Rocco was erroneously presented as Maja DiRocco.
The correct author name has been included in the author list of this Correction article and is already updated in the original article.
For additional clarity, the Correction article also provides below an updated Table 2.
Table 2
Demographics and Baseline Disease by Age Category
 
<8 Yrs (N=17)
8 to <15 Yrs (N=36)
15 to <25 Yrs (N=34)
≥25 to ≤65 Yrs (N=27)
Total (N=114)
Males, n (%)
9 (52.9)
24 (66.7)
16 (47.1)
13 (48.1)
62 (54.4)
Age (years)
 Mean ±SD
5.9 ±1.1
11.4 ±2.1
18.9 ±3.1
31.7 ±6.7
17.6 ±9.7
 Median (min, max)
6.0 (4, 7)
11.0 (8, 14)
18.5 (15, 24)
30.0 (25, 56)
15.0 (4, 56)
Age at 1st flare-up
 Mean ±SD
2.9 ±2.1
4.4 ±3.6
5.6 ±4.8
7.1 ±5.0
5.2 ±4.4
 Median (min, max)
2.0 (1, 6)
4.0 (0, 13)
3.5 (0, 17)
5.0 (0, 20)
4.0 (0, 20)
Years since last flare-up
 Mean ±SD
0.7 ±0.9
1.5 ±2.8
1.5 ±1.9
2.3 ±3.5
1.6 ±2.6
 Median (min, max)
0.3 (0, 3)
0.5 (0, 14)
0.7 (0, 7)
0.9 (0, 15)
0.5 (0, 15)
Number of flare-ups in the past 12 months (in those with flare-ups)
 n
13
25
21
17
76
 Mean ±SD
2.9 ±2.6
6.8 ±11.2
2.2 ±1.8
1.9 ±1.6
3.8 ±6.9
 Median (min, max)
2.0 (1, 10)
2.0 (1, 40)
1.0 (1, 8)
1.0 (1, 7)
2.0 (1, 40)
CAJIS total score1
 Mean ±SD
5.6 ±3.9
9.2 ±4.6
13.9 ±6.7
16.5 ±7.3
11.8 ±7.0
 Median (min, max)
6.0 (1, 15)
8.5 (1, 20)
13.5 (1, 26)
18.0 (1, 30)
10.5 (1, 30)
FOP-PFQ % worst total score2
 Mean ±SD
34.8 ±26.1
44.4 ±20.2
43.1 ±31.0
58.4 ±35.8
46.3 ±27.1
 Median (min, max)
41.8 (1, 84.6)
45.2 (1.9, 82.7)
38.4 (0, 100)
54.5 (0, 100)
45.5 (0, 100)
PROMIS Global Physical Health (adult) or PROMIS Global Health (pediatric, parent proxy) (T-score)3
 Mean ±SD
47.6 ±9.4
41.8 ±8.7
44.1 ±9.0
42.5 ±7.9
NA
 Median (min, max)
48.3 (33, 24)
41.7 (24, 57)
44.9 (24, 68)
42.3 (27, 54)
 
PROMIS Global Mental Health (adult) (T-score)3
 Mean ±SD
NA
NA
53.6 ±9.9
51.4 ±8.5
52.6 ±9.3
 Median (min, max)
  
53.3 (28, 68)
53.3 (39, 68)
53.3 (28, 68)
Total body HO volume, excluding head (mm3)
 Mean ±SD
61951 ±75221
159303 ±161779
380751 ±363142
651913 ±674454
324631 ±440977
 Median (min, max)
21692 (0, 224019)
130509 (0, 828262)
258543 (0, 1504849)
481524 (48844, 2833946)
173536 (0, 2833946)
Number of regions with HO4
 Mean ±SD
3.1 ±2.6
5.1 ±2.7
7.4 ±3.4
8.8 ±3.7
6.4 ±3.7
 Median (min, max)
4.0 (0, 7)
5.0 (0, 14)
8.0 (0, 13)
8.5 (3, 14)
6.0 (0, 14)
1CAJIS assessed range of motion across 12 joints (left and right shoulders, elbows, wrists, hips, knees, and ankles) and three body regions (jaw, cervical spine [neck], and thoraco-lumbar spine). Each noted as: 0=uninvolved; 1=partially involved; 2=completely ankylosed. Total scores range from 0 to 30 with higher scores indicating more severe limitations in mobility.
2FOP-PFQ used transformed scores expressed as a percentage of the worst possible score. Lower percentages indicate better functioning; higher percentages indicate worse functioning.
3Distributions standardized such that a T-score of 50 (SD of 10) represents the average for the United States general population. Higher T-scores indicate better physical/mental health.
4Fifteen possible regions (neck, lower spine/abdomen, upper spine/chest; and both shoulders, elbows, wrists, hips, knees, and ankles).
Abbreviations: CAJIS=Cumulative Analogue Joint Involvement Scale, HO=heterotopic ossification, max=maximum, min=minimum, FOP-PFQ=Fibrodysplasia Ossificans Progressiva-Patient Function Questionnaire, NA=not applicable, PROMIS=Patient-Reported Outcome Measure Information System, SD=standard deviation, Yrs=years
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://​creativecommons.​org/​licenses/​by/​4.​0/​), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://​creativecommons.​org/​publicdomain/​zero/​1.​0/​) applies to the data made available in this article, unless otherwise stated.
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Titel
Correction to: Natural history of fibrodysplasia ossificans progressiva: cross-sectional analysis of annotated baseline phenotypes
verfasst von
Robert J. Pignolo
Geneviève Baujat
Matthew A. Brown
Carmen De Cunto
Maja Di Rocco
Edward C. Hsiao
Richard Keen
Mona Al Mukaddam
Kim-Hanh Le Quan Sang
Amy Wilson
Barbara White
Donna R. Grogan
Frederick S. Kaplan
Publikationsdatum
01.12.2019
Verlag
BioMed Central
Erschienen in
Orphanet Journal of Rare Diseases / Ausgabe 1/2019
Elektronische ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-019-1096-3

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