Table 1.
Study | Patients and CAH form | Age range (years) | Mean HC dose (mg/m2/day) | Mean FC dose (μg/m2/day) | Technique | Outcomes |
---|---|---|---|---|---|---|
Finkielstain et al. (8) |
-139 Classic (97 SW, 42 SV) -31 Non-classic |
0.6–17 | 15 | n.a. | n.a. | ↑ Prevalence of obesity (35%) vs. normal pediatric population (17%), with no difference between Classic and NC CAH; No difference between males and females ↑ HOMA-IR ↑ BP (about 40% Classic and 20% NC CAH) |
Gonçalves et al. (11) |
-28 Classic females (18 SW, 10 SV) -112 Controls |
4–23 | 15–20 | n.a. | BIA | ↑ BMI vs. controls, ↑ fat mass and waist/hip ratio vs. controls |
Stikkelbroeck et al. (12) |
-27 Classic (24 SW, 3 SV) -30 Controls |
17–25 | n.a. | n.a. | DXA | ↑ BMI vs. controls, ↑ fat mass vs. controls |
Völkl et al. (16) |
-89 Classic (78 SW, 11 SV) |
0.2–17.9 | 14.7 | 63.1 | n.a. | ↑ Prevalence of obesity (16.8%) vs. normal population (2.27%) No difference between males and females |
Roche et al. (21) |
-38 Classic (SW) | 6.1–18.2 | 17.5 (prepubertal) 17.3 (pubertal) 17.8 (postpubertal) |
120 (prepubertal) 113 (pubertal) 103 (postpubertal) |
n.a. | ↑ BMI vs. normal population ↑ SPB and DBP vs. normal population ↓ nocturnal dip in BP No difference between males and females |
Subbarayan et al. (25) |
-107 Classic (79 SW, 28 SV) |
0.4-20.5 | 13.3 | 102 | n.a. | ↑ Prevalence of obesity (23.6%) vs. normal population (14.8-17.1 %) ↑ SBP vs. normal population 9.5% high plasma triglycerides No difference between males and females ↓ HOMA-IR vs. controls |
Isguven et al. (26) |
-16 Classic (SW) -18 Controls |
1.4–10.5 | 15 | n.a. | BIA | ↑ BMI vs. controls, No difference between males and females ↑ Fat mass in female patients vs. controls |
Janus et al. (27) |
-61 Classic (51 SW, 10 SV) -9 Non-classic |
3–17.9 | 17.2 (SW) 19.5 (SV) 11.9 (NC) |
66.5 (SW) 28.6 (SV) |
BIA | Normal BMI, no differences in body composition between classic and NC forms; No difference between males and females No hypertension |
Williams et al. (28) |
-25 Classic -12 Non -classic -41 Controls |
0.5–15.8 | 13.9 8.2 |
82 μg/day 18 μg/day |
DXA | ↑ fat mass in classic CAH vs. controls, ↑ lean mass in NC CAH vs. controls Comparable HOMA-IS between CAH (classic and NC) and controls ↑ stimulated glucose and insulin in NC CAH vs. controls Comparable BP and lipids between classic CAH and controls ↑ SBP in NC CAH vs. controls |
Abd El Dayem et al. (29) |
-28 Classic (27 SW, 1 SV) -11 Controls |
3–18 | 15.5 | 50–200 μg/day | DXA | ↑ BMI, ↑ fat mass and ↓ lean mass vs. controls No difference between males and females |
Marra et al. (30) |
-20 Classic (15 SW, 5 SV) -20 Controls |
11.1–16.1 | 15 | 54.8 | DXA | ↑ BMI and ↑ fat mass and waist/height ratio vs. controls; ↑ HOMA-IR vs. controls; No difference between males and females Comparable lipids and BP between CAH and controls ↓ Exercise capacity vs. controls LV diastolic dysfunction in CAH male patients |
Ariyawatkul et al. (31) |
-21 Classic (10 SW, 11 SV) -21 Controls |
9–28 | 21.4 | 50–150 μg/day | DXA | BMI, fat and lean mass comparable between CAH and controls ↑ Waist/hip and waist/height ratio vs. controls Comparable HOMA, BP, lipids, leptin between CAH and controls |
Halper et al. (32) |
-32 Classic (19 SW, 13 SV) -10 Non- classic -101 controls |
7.6–17.7 | 11.3 | n.a. | DXA | ↓ BMI vs. controls, VAT comparable between patients and controls |
Kim et al. (33) |
-28 Classic (20 SW, 8 SV) -28 Controls |
12.4–18.8 | 19.5 | 210 μg/day | CT | 60.7% of patients obese or overweight; ↑ VAT, SAT, and VAT/SAT vs. controls; No difference between males and females Elevated HOMA in 18% of CAH |
Mooij et al. (23) |
-26 Classic -(24 SW, 2 SV) |
8-16 | 11.2 | 98.5 | n.a. | ↑ BMI vs. reference population (25.9% obese and 14.8% overweight) 44.4% of patients HOMA-IR > 75th and 29.6% HOMA-IR >90th percentile ↑ SBP in 18.5%, lower than reference population 48.1% ↓ nocturnal dip |
Amr et al. (24) |
-32 Classic (24 SW, 8 SV) -32 Controls |
3-17 | 15 | 50-100 | n.a. | ↑ BMI in patients (22% obese) vs. controls ↑ HOMA-IR, CIMT, SBP vs. controls ↑ Stimulated glucose levels vs. controls Comparable lipids, stimulated insulin vs. controls |
CAH, Congenital Adrenal Hyperplasia; HC, hydrocortisone; FC, fludrocortisones; SW, salt wasting; SV, simple virilizing; NC, non-classic; HOMA, homeostatic model assessment; HOMA-IS, HOMA insulin sensitivity; BP, blood pressure; BIA, bioelectrical impedance analysis; BMI, body mass index; DXA, dual X-ray absorptiometry; SBP, systolic blood pressure; DBP, diastolic blood pressure; HOMA-IR, HOMA insulin resistance; LV, left ventricle; HDL, high density lipoproteins; T-COL, total cholesterol; VAT, visceral adipose tissue; SAT, subcutaneous adipose tissue; CIMT, carotid intima media thickness; CRP, C reactive protein. n.a., not available/not applicable. ↑ increased; ↓ decreased.