ESPE Abstracts

Objective and hypotheses: To establish clinical, laboratory peculiarities of manifestation, short-term effects of performance of hormonotherapy children with CACH.

Method: We examined 32 children with CACH (29 children – with salt-losing form (SLF) (boys/girls=18/11), 3 – virile form (VF) (boy/girls=1/2)) in onset and after 1 year of therapy. Weight and growth dynamics, parameters of potassium (K), sodium (Na), glucose, adrenocorticotropic hormone (ACTH), 17-Hydroxypregnenolone (17OHP), pH, buffer bases (BE)) were analyzed in blood. We used StatSoft Statistica v6.0.

Results: Age of onset: boys with SLF – 59.41±4.66 days, girls – 65.3±13.15 days (P<0.05); boy with VF – 110 days, girls – 1285±233.35 days (P<0.05). Hyperpotassemia (5.66±0.25 mmol/l, P<0.01), hyponatremia (130.19±1.81 mmol/l, P<0.005), euglycemia (4.57±0.1 mmol/l, P<0.05), 17OHP levels increasing (358.8±3912 nmol/l, P<0.05), ACTH increasing (69.6±7.95 pg/ml, P<0.05), metabolic acidosis - pH (731±0.02; P<0.05), BE (−7.96±0.77 mmol/l, P<0.05) were in children with SLF. The elevation of 17OHP values (158.83±15.11 nmol/l, P<0.05), ACTH (63±0.79 pg/ml, P<0.05) in onset of VF were revealed. Initial doses of glucocorticoid (hydrocortisone, mg/m²/d) and mineralocorticoid (fludrocortisone, mg/m²/d) in children with SLF: 26.09±2.82 and 0.46±0.03 respectively (maintenance doses one year after: 13.28±1.14 and 0.2±0.02 respectively). The initial doses of hydrocortisone (mg/m²/d) in children with VF: 15.35±3.85 (maintenance doses one year after: 12.68±3.85). The normalization of K (4.32±0.1 mmol/l), Na (138.2±0.84 mmol/l) levels were in patients with SLF after one year of treatment. Negative correlations between the age of onset of the disease and initial mineralocorticoid dose (r=−0.37, P<0.001) in patients with SLF, initial glucocorticoid dose and 17OHP levels in children with SLF (r=−0.3, P<0.05), initial glucocorticoid dose and rate of growth in patients with SLF and VF (r=−0.3, P<0.01) were noticed.

Conclusions: i) Suitably glucocorticoid and mineralocorticoid doses ensure normal growth rates in children with CACH; ii) age of onset is one of the factor defining initial mineralocorticoid dose.