Table 2.

Basic characteristics of adult and paediatric patients with completed patient diaries and adrenal crisis.

CharacteristicsAdult patientsPaediatric patients
Total number (n (%))145 (100)29 (100)
Median age (range) in years32.0 (16.0-68.0)12.1 (0.7-18.9)
Sex (n (%))
 Female85 (58.6)15 (51.7)
 Male60 (41.4)14 (48.3)
Phenotype (n (%))
 SW88 (60.7)23 (79.3)
 SV49 (33.8)6 (20.7)
 NC8 (5.5)0 (0)
First glucocorticoid preparation (n (%)
Subgroups (n)
 Hydrocortisone65 (44.8)
45 SW; 17 SV; 3 NC
27 (93.1)
21 SW; 6 SV
 Plenadren®7 (4.8)
4 SW; 2 SV; 1 NC
1 (3.4)
1 SW
 Efmody®13 (9.0)
5 SW; 8 SV
 Prednison9 (6.2)
5 SW; 4 SV
 Prednisolon42 (29.0)
21 SW; 17 SV; 4 NC
1 (3.4)
1 SW
 Dexamethason9 (6.2)
8 SW; 1 SV
Second glucocorticoid preparation (n (%))
Subgroups (n)
 None133 (91.7)
79 SW; 47 SV; 7 NC
29 (100)
 Hydrocortisone1 (0.7)
1 SW
 Prednisolone8 (5.5)
6 SW; 2 SV
 Dexamethasone3 (2.1)
2 SW; 1 NC
HDE in mg/day (Md (IQR))30.1 (13.8)22.5 (17.5)
 SW30.0 (10.0)22.5 (15.0)
 SV30.0 (15.0)26.3 (23.5)
 NC15.0 (8.8)
Mineralocorticoid medication
 Yes90 (62.1)23 (79.3)
 No55 (37.9)6 (20.7)
Mineralocorticoid in mg/day (Md (IQR))0.05 (0.10)0.07 (0.06)
Total number of AC31 in 370.6 py3 in 58.6 py
8.4 in 100 py5.1 in 100 py
CharacteristicsAdult patientsPaediatric patients
Total number (n (%))145 (100)29 (100)
Median age (range) in years32.0 (16.0-68.0)12.1 (0.7-18.9)
Sex (n (%))
 Female85 (58.6)15 (51.7)
 Male60 (41.4)14 (48.3)
Phenotype (n (%))
 SW88 (60.7)23 (79.3)
 SV49 (33.8)6 (20.7)
 NC8 (5.5)0 (0)
First glucocorticoid preparation (n (%)
Subgroups (n)
 Hydrocortisone65 (44.8)
45 SW; 17 SV; 3 NC
27 (93.1)
21 SW; 6 SV
 Plenadren®7 (4.8)
4 SW; 2 SV; 1 NC
1 (3.4)
1 SW
 Efmody®13 (9.0)
5 SW; 8 SV
 Prednison9 (6.2)
5 SW; 4 SV
 Prednisolon42 (29.0)
21 SW; 17 SV; 4 NC
1 (3.4)
1 SW
 Dexamethason9 (6.2)
8 SW; 1 SV
Second glucocorticoid preparation (n (%))
Subgroups (n)
 None133 (91.7)
79 SW; 47 SV; 7 NC
29 (100)
 Hydrocortisone1 (0.7)
1 SW
 Prednisolone8 (5.5)
6 SW; 2 SV
 Dexamethasone3 (2.1)
2 SW; 1 NC
HDE in mg/day (Md (IQR))30.1 (13.8)22.5 (17.5)
 SW30.0 (10.0)22.5 (15.0)
 SV30.0 (15.0)26.3 (23.5)
 NC15.0 (8.8)
Mineralocorticoid medication
 Yes90 (62.1)23 (79.3)
 No55 (37.9)6 (20.7)
Mineralocorticoid in mg/day (Md (IQR))0.05 (0.10)0.07 (0.06)
Total number of AC31 in 370.6 py3 in 58.6 py
8.4 in 100 py5.1 in 100 py

Abbreviations: AC, adrenal crisis; HDE, hydrocortisone dose equivalent; NC, non-classic; py, patient years; SV, simple virilizing; SW, salt wasting.

Table 2.

Basic characteristics of adult and paediatric patients with completed patient diaries and adrenal crisis.

CharacteristicsAdult patientsPaediatric patients
Total number (n (%))145 (100)29 (100)
Median age (range) in years32.0 (16.0-68.0)12.1 (0.7-18.9)
Sex (n (%))
 Female85 (58.6)15 (51.7)
 Male60 (41.4)14 (48.3)
Phenotype (n (%))
 SW88 (60.7)23 (79.3)
 SV49 (33.8)6 (20.7)
 NC8 (5.5)0 (0)
First glucocorticoid preparation (n (%)
Subgroups (n)
 Hydrocortisone65 (44.8)
45 SW; 17 SV; 3 NC
27 (93.1)
21 SW; 6 SV
 Plenadren®7 (4.8)
4 SW; 2 SV; 1 NC
1 (3.4)
1 SW
 Efmody®13 (9.0)
5 SW; 8 SV
 Prednison9 (6.2)
5 SW; 4 SV
 Prednisolon42 (29.0)
21 SW; 17 SV; 4 NC
1 (3.4)
1 SW
 Dexamethason9 (6.2)
8 SW; 1 SV
Second glucocorticoid preparation (n (%))
Subgroups (n)
 None133 (91.7)
79 SW; 47 SV; 7 NC
29 (100)
 Hydrocortisone1 (0.7)
1 SW
 Prednisolone8 (5.5)
6 SW; 2 SV
 Dexamethasone3 (2.1)
2 SW; 1 NC
HDE in mg/day (Md (IQR))30.1 (13.8)22.5 (17.5)
 SW30.0 (10.0)22.5 (15.0)
 SV30.0 (15.0)26.3 (23.5)
 NC15.0 (8.8)
Mineralocorticoid medication
 Yes90 (62.1)23 (79.3)
 No55 (37.9)6 (20.7)
Mineralocorticoid in mg/day (Md (IQR))0.05 (0.10)0.07 (0.06)
Total number of AC31 in 370.6 py3 in 58.6 py
8.4 in 100 py5.1 in 100 py
CharacteristicsAdult patientsPaediatric patients
Total number (n (%))145 (100)29 (100)
Median age (range) in years32.0 (16.0-68.0)12.1 (0.7-18.9)
Sex (n (%))
 Female85 (58.6)15 (51.7)
 Male60 (41.4)14 (48.3)
Phenotype (n (%))
 SW88 (60.7)23 (79.3)
 SV49 (33.8)6 (20.7)
 NC8 (5.5)0 (0)
First glucocorticoid preparation (n (%)
Subgroups (n)
 Hydrocortisone65 (44.8)
45 SW; 17 SV; 3 NC
27 (93.1)
21 SW; 6 SV
 Plenadren®7 (4.8)
4 SW; 2 SV; 1 NC
1 (3.4)
1 SW
 Efmody®13 (9.0)
5 SW; 8 SV
 Prednison9 (6.2)
5 SW; 4 SV
 Prednisolon42 (29.0)
21 SW; 17 SV; 4 NC
1 (3.4)
1 SW
 Dexamethason9 (6.2)
8 SW; 1 SV
Second glucocorticoid preparation (n (%))
Subgroups (n)
 None133 (91.7)
79 SW; 47 SV; 7 NC
29 (100)
 Hydrocortisone1 (0.7)
1 SW
 Prednisolone8 (5.5)
6 SW; 2 SV
 Dexamethasone3 (2.1)
2 SW; 1 NC
HDE in mg/day (Md (IQR))30.1 (13.8)22.5 (17.5)
 SW30.0 (10.0)22.5 (15.0)
 SV30.0 (15.0)26.3 (23.5)
 NC15.0 (8.8)
Mineralocorticoid medication
 Yes90 (62.1)23 (79.3)
 No55 (37.9)6 (20.7)
Mineralocorticoid in mg/day (Md (IQR))0.05 (0.10)0.07 (0.06)
Total number of AC31 in 370.6 py3 in 58.6 py
8.4 in 100 py5.1 in 100 py

Abbreviations: AC, adrenal crisis; HDE, hydrocortisone dose equivalent; NC, non-classic; py, patient years; SV, simple virilizing; SW, salt wasting.

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