ESPE Abstracts (2019) 92 P1-300

Adrenals and HPA Axis (2)

Growth Trajectory and Final Height in Children with Non Classical Congenital Adrenal Hyperplasia

Malgorzata Wasniewska1, Letteria Anna Morabito1, Federico Baronio2, Silvia Einaudi3, Maria Carolina Salerno4, Carla Bizzarri5, Gianni Russo6, Mariangela Chiarito7, Anna Grandone8, Laura Guazzarotti9, Antonietta Spinuzza1, Silvia Di Carlo1, Rita Ortolano2, Antonio Balsamo2, Enrica Abrigo3, Barbara Baldini Ferroli5, Angela Alibrandi10, Donatella Capalbo4, Maria Felicia Faienza7


1Department of Human Pathology of Adulthood and Childhood 'Gaetano Barresi', University of Messina, Messina, Italy. 2Department of Women, Children and Urological Diseases, S. Orsola Malpighi University Hospital,, Bologna, Italy. 3Department of Pediatric Endocrinology and Diabetology, Regina Margherita Children Hospital, University of Turin, Turin, Italy. 4Department of Pediatrics, University "Federico II", Naples, Italy. 5Unit of Endocrinology and Diabetes, 'Bambino Gesù' Ch, Rome, Italy. 6Department of Pediatrics, Endocrine Unit, Scientific Institute San Raffaele, Milan, Italy. 7Department of Biomedical Sciences and Human Oncology, Pediatric Section, University of Bari 'A. Moro', Bari, Italy. 8Department of Woman, Child and General and Specialized Surgery, Univeristà degli Studi della Campania "Luigi Vanvitelli", Naples, Italy. 9Department of Pediatrics, University of Padua, Padua, Italy. 10Department of Economics, Unit of Statistical and Mathematical Sciences, University of Messina, Messina, Italy

Background: Subjects with non classical congenital adrenal hyperplasia (NCCAH) often present an increased growth velocity secondary to elevation of adrenal androgens that promote early bone maturation and compromise final height (FH). The aim of the study was to analyze prognostic factors affecting growth trajectory and FH in children with NCCAH.

Design: retrospective, multicentric study

Study population: 192 (140 females) NCCAH children with confirmed molecular diagnosis followed from diagnosis up to FH.

Methods: clinical records were collected and analyzed. The study population was divided for gender, with or without hydrocortisone treatment (171 treated with hydrocortisone) and type of the mutation of CYP21A2 gene (V281L homozygosis in 55, compound heterozygosis with V281L in 85 and other mutations in 48 cases).

FH (SDS), pubertal growth (PG) (cm), growth trajectory (GT) since diagnosis to FH (SDS) and FH adjusted to target (TH) (FH-TH)(SDS) were evaluated as outcomes using stepwise linear regression models.

Results: FH SDS and FH-TH were not significantly different in both gender (-0.34 vs -0.36, P =0.98 and -0.05 vs 0.05, P=0.65, respectively).

At stepwise linear regression analysis, FH and FH-TH resulted significantly related to chronological age (CA) (P= 0.008 and 0.016), bone age (BA) / CA ratio (P= 0.004 and 0.001), height (H) (P=0.000 for both parameters) at NCCAH diagnosis and TH (P=0.013 and 0.000) .

PG was higher in males (22.59±5.74 vs 20.72±17.4 cm in females, P=0.002), as physiologically observed, and was positively related to H (P=0.027), negatively to BMI (P=0,001) and BA/CA ratio (P=0.001) at NCCAH diagnosis.

The type of the mutation of CYP21A gene and hydrocortisone doses did not influence significantly the parameters of growth of our NCCAH patients.

The comparison between treated with hydrocortisone and untreated patients did not evidence significant differences on GT, but the statistic value of these results is limited by the small number of untreated group.

Conclusion: FH and GT of NCCAH patients is significantly influenced by auxological parameters at diagnosis (CA, BA/CA ratio, H). Gender, molecular alteration, biochemical picture and hydrocortisone doses seem to have no important influence on height outcome of these NCCAH children.