title: Late prenatal dexamethasone and phenotype variations in 46,XX CAH: concerns about current protocols and benefits for surgical procedures.
abstract: OBJECTIVE
  To describe the action of prenatal dexamethasone (PreDex) on the anatomy of female congenital adrenal hyperplasia (CAH) genitalia when started at later stages of gestation.
  
  
  METHODS
  Our group follows a large cohort of French CAH patients who underwent PreDex therapy, of whom 258 were recently reported. Four 46,XX patients with a delayed PreDex treatment presented with a virilized genitalia and required surgical reconstruction. This is a retrospective report on genital phenotyping at the time of surgery of these four patients who began PreDex therapy at 8, 12, 20, and 28 weeks of gestation.
  
  
  RESULTS
  Although this series is limited in number, the anatomical description of the length of the genital tubercle, the height of the urethra-vaginal confluence, and the degree of fusion of the genital folds seems to be dependent upon the starting date of PreDex. Most PreDex treatments prescribed up to now have covered the full duration of gestation.
  
  
  CONCLUSIONS
  Our findings suggest that PreDex therapy could be limited to the period of the partitioning window. It is hoped that further prospective multicentric clinical studies will obtain ethical approval in order to elucidate the place and protocols of PreDex therapy in the management of CAH.
authors.affiliation: Service d'Urologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, GHE, 59, boulevard Pinel, 69677 Bron Cedex, France.
authors.forename: Daniela
authors.initials: D
authors.lastname: Gorduza
authors.affiliation: Endocrinologie Moléculaire et Maladies Rares, Centre de Biologie et Pathologie Est, Centre Hospitalo-Universitaire de Lyon, 69677 Bron Cedex, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Tardy-Guidollet
authors.affiliation: Service d'Urologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, GHE, 59, boulevard Pinel, 69677 Bron Cedex, France.
authors.forename: Elsa
authors.initials: E
authors.lastname: Robert
authors.affiliation: Service d'Endocrinologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, 69677 Bron Cedex, France.
authors.forename: Claire-Lise
authors.initials: CL
authors.lastname: Gay
authors.affiliation: Service d'Endocrinologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, 69677 Bron Cedex, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Chatelain
authors.affiliation: Service d'Endocrinologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, 69677 Bron Cedex, France.
authors.forename: Michel
authors.initials: M
authors.lastname: David
authors.affiliation: Service d'Endocrinologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, 69677 Bron Cedex, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Bretones
authors.affiliation: Département de Pédiatrie Médicale, Centre Hospitalo-Universitaire de Limoges, 87042 Limoges Cedex, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Lienhardt-Roussie
authors.affiliation: Service d'Endocrinologie, Groupement Hospitalier Est, Centre Hospitalo-Universitaire de Lyon, 69677 Bron Cedex, France.
authors.forename: Aude
authors.initials: A
authors.lastname: Brac de la Perriere
authors.affiliation: Endocrinologie Moléculaire et Maladies Rares, Centre de Biologie et Pathologie Est, Centre Hospitalo-Universitaire de Lyon, 69677 Bron Cedex, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Morel
authors.affiliation: Service d'Urologie Pédiatrique, Hôpital Mère-Enfant, Centre Hospitalo-Universitaire de Lyon, GHE, 59, boulevard Pinel, 69677 Bron Cedex, France. Electronic address: pierre.mouriquand@chu-lyon.fr.
authors.forename: Pierre
authors.initials: P
authors.lastname: Mouriquand
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D003907:Dexamethasone / Q000008:administration & dosage*; D004334:Drug Administration Schedule; D005260:Female; D046128:Fetal Therapies; D005865:Gestational Age; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007223:Infant; D010641:Phenotype; D011247:Pregnancy; D012189:Retrospective Studies; D014770:Virilism / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery*
chemical_list: D005938:Glucocorticoids; D003907:Dexamethasone
keywords: CAH; Phenotype; Prenatal dexamethasone