Endocrine Abstracts

Medical History of thirty five patients aged from one to sixteen years diagnosed with CAH have been analyzed in the Republican Center for Pediatric Endocrinology. Among the thirty five patients participating in the study (boys–fifty seven percent, girls –forty-three) seventy-one percent is with salt wasting form (SWF), twenty-two percent with virilising form (VF), three percent with non-classical form (NF). The mean weight SDS at birth is minus eight hundredths ± one point seventeen, the mean height SDS is twelvehundredths ± one point six. Age of diagnosis SWF is seventeen point four ± sixteen point three days, VF is one point three hundredths ± four point six years. The most frequent signs of manifestation in SWF were regurgitation, body weight loss, vomiting, dry skin, hypotension, exicosis; in VF – premature pubarche. At the time of diagnosis, seventeen-hydroxyprogesterone (SOHP) was higher than normal range in all groups: SWF –eight hundredforty ± two hundred ninety-eightnmol/l, VF – three hundred eighty-six ± eighty-one nmol/l, NF –thirty-five nmol/l; increase morning values of adrenocorticotropic hormone (seventy-four ± two pg/ml). It was revealed that before treatment there were changes in electrolytes (K, Na) only in group with SWF (six point seven ± one point six mmol/l andone hundred twenty-six ± eleven mmol/l respectively). Blood glucose and pH in children with SWF were three point two ± one point forteen mmol/l and seven point three ± sixty-seven thousandths co-responsibly. All children received hydrocortisone replacement therapy at a starting dose of sixty-four ± twenty-eight mg/m² in SWF group; twenty-two ± twenty-one mg/square meter in VF group; twenty-one mg/m² in NF group. Patients with SWF additionally took fludrocortisone at a starting dose of seven hundred eighty-seven ± one hundred thirty-one mkg/square meter. Fourteen children from the total sample have reached puberty age. Height SDS is minus point four ± one point three, weight SDS one point thirteen ± one point thirty-three, BMI twenty-four point two ± three point six kg/square meter, SOHP –thirteen point fifty-six ± thirteen point forty seven nmol/l. Late diagnosis of the disease in patients was revealed regardless of the form of СAH. Metabolic acidosis, hyponatremia and hyperkalemia, high level of SOHP are registered in the SWP group at the time of manifestation There were noted compensation of metabolic parameters, achievement of target growth indices, when children receive adequate replacement hormonal therapy.