title: Failure of cortisone acetate therapy in 21-hydroxylase deficiency in early infancy.
abstract: BACKGROUND
  Pediatric endocrinologists initially treat congenital adrenal hyperplasia with either cortisone acetate (CA) or hydrocortisone (HC). Despite high doses of CA, we noted that 17-hydroxyprogesterone (17-OHP) and corticotropin were not fully suppressed in serum from neonates with 21-hydroxylase deficiency (21-OHD) until they were 40- to 80-days-old. In contrast, serum concentrations of 17-OHP were suppressed immediately by oral treatment with HC.
  
  
  METHODS
  We sought to understand the reason for this discrepancy. Serum cortisol (F), cortisone (E), and 17-OHP were measured by radioimmunoassay or high-performance liquid chromatography in seven neonates with 21-OHD and in 118 normal subjects. From the time of diagnosis, CA was administered to four of the neonates with 21-OHD, while HC was given to the other three.
  
  
  RESULTS
  In normal subjects serum E concentrations were greater than F during the first 2 months after birth, whereas F concentrations exceeded E after 2 months of age. Although infants receiving CA initially were given a high dose, serum F concentrations were extremely low, while 17-OHP concentrations were high until about 2 months of age. Then serum F exceeded E, and 17-OHP became fully suppressed even though infants received only a moderate dose of CA. In contrast, HC administration successfully normalized serum 17-OHP in the neonatal period. With temporary switching of neonates from HC to CA, serum F concentrations immediately decreased and 17-OHP concentrations increased.
  
  
  CONCLUSIONS
  Conversion of E to F may be limited during early infancy, adversely affecting treatment with CA. Cortisone acetate may be inappropriate as a glucocorticoid replacement during early infancy in patients with 21-OHD.
authors.affiliation: Department of Pediatrics, Hiroshima General Hospital of West Japan Railway Company, Hiroshima, Japan. Kazuhiko.Jinno@ma4.seikyou.ne.jp
authors.forename: K
authors.initials: K
authors.lastname: Jinno
authors.forename: N
authors.initials: N
authors.lastname: Sakura
authors.forename: S
authors.initials: S
authors.lastname: Nomura
authors.forename: M
authors.initials: M
authors.lastname: Fujitaka
authors.forename: K
authors.initials: K
authors.lastname: Ueda
authors.forename: M
authors.initials: M
authors.lastname: Kihara
mesh_terms: D019326:17-alpha-Hydroxyprogesterone / Q000037:antagonists & inhibitors / Q000097:blood; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000188:drug therapy*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000367:Age Factors; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D003348:Cortisone / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000097:blood / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007231:Infant, Newborn; D008297:Male; D017211:Treatment Failure
chemical_list: D000893:Anti-Inflammatory Agents; D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D003348:Cortisone; D006854:Hydrocortisone