ESPE Abstracts (2022) 95 P1-15

ESPE2022 Poster Category 1 Adrenals and HPA Axis (52 abstracts)

Growth from Birth to Adult Height in Patients with Classical Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency: A Collaborative Study

María Clemente 1,2,3 , María Teresa Sandino 1 , Arancha Escribano 4 , Sara Berrade 5 , Ariadna Campos 1,3 & Diego Yeste 1,2,3

1Hospital Vall d´Hebron, Barcelona, Spain; 2CIBERER, Barcelona, Spain; 3Universitat Autonòma, Barcelona, Spain; 4Hospital Universitario Virgen de la Arrixaca, Murcia, Spain; 5Hospital de Navarra, Pamplona, Spain

Objective: To evaluate growth in patients with classical CAH.

Patients and methods: Retrospective, longitudinal and multicentric study of patients with classical CAH due to 21-hydroxylase deficiency who had achieved adult height(AH).

Excluded: boys with simple virilising forms(SV)>1-year at diagnosis. Normative Control Group:Millennials Longitudinal Growth Study(2018). According to age at pubertal growth spurt (PGS)onset, patients were considered:very-early, early, intermediate, late and very-late maturers. PGS was considered normal if height Z-Score (HZS) at PGS-onset was similar (±0.5) to AH Z-Score.

Results: 50 patients(37 females); 41 salt-wasting. During the first year of life a decrease in height SDS was observed. Three groups of patients were identified according to their growth pattern from the first year: Group1:Regained growth until PGS onset, normal PGS, AH Z-Score>-2 and AH Z-Score-mean parental height(MPH) Z-Score>-1. Group 2: Non-regained prepubertal growth and normal PGS: HZS(PGSonset–3 years)< 0.5. Group 3: Accelerated prepubertal growth with disrupted PGS: HZS(PGS onset-3years)>1. All presented advanced bone age(BA) related to chronological age(>1year). Six patients were non-classifiable.

Table 1: HZS at different time-points.
  NN 1-year 3-years 7-years PGS-onset 2nd year of PGS AH AH-MPH
Group 1 n=19 0,36±0,79 -1.77±1.0 -0.99±0.94 -0.50±0.98 -0.44±0.98 -0.32±0.90 -0.53±0.75 -0.07±1.02
Group 2 n=11 0,10±0,59 -2.11±1.51 -2.26±0.79* -1.91±0.70* -1.81±0.71* -1.68±0.62* -1.67±0.42* -1.04±1.19*
Group 3 n=14 0,23±0,81 -1.90±1.70 -1.43±0.96 -0.25±1.06 0.25±1.06 -0.34±1.35 -2.06±1.01* -1.39±0.85*
All sample n=50 0.24±0.77 (-1.46,2.8) -1.84±1.34 (0.57,-6) -1.40±1.01 (2.22,-3.45) -0.65±1.15 (1.92,-3.19) -0.53±1.20 (1.87,-2.90) -0.64±1.12 (1,69,-3.89) -1.31±1.07 (1.11,-4.14) -0.68±1.19 (2.31,-3.37)
Hidrocortisone (mg/m2/day) n=39   21.1±5.1 (37.7,13.5) 18.1±4.9 (30.8,7.4) 19.06±3.7 (26.3,10.6) 16.7±4.6 (33,7.6) 15.9±4.3 (25,6.6)    
Mean±SD;(max, min).*P<0.05 vs group1 AH was 156.5±5.3 cm (168.5-139.1)in women and 168.6±7.4 cm (185.1-159.0) in men. AH Z-Score: non-statistically different in patients with and without salt wasting forms. Significantly lower in very-early maturers(-1.90±0,60) vs intermediate(-0,79±0,92) and very-late maturers(-1,16±0,40). Testosterone and androstenedione values were higher in Group3 vs Group1 at 7-years and at PGS-onset. No correlation was observed between hydrocortisone doses and HZS.

Conclusions: • Globally, loss of HZS were observed during the first year of life with partial recovery until PGS-onset • Auxological evaluation is a useful tool for monitoring disease management • The observed growth pattern heterogeneity is partially explained by hydrocortisone doses and biochemical control.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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