PMID- 1323921
OWN - NLM
STAT- MEDLINE
DCOM- 19920911
LR  - 20191028
IS  - 0951-3590 (Print)
IS  - 0951-3590 (Linking)
VI  - 6
IP  - 2
DP  - 1992 Jun
TI  - The incidence of non-classical 21-hydroxylase deficiency in hirsute adolescent 
      girls.
PG  - 99-106
AB  - Non-classical adrenal hyperplasia due to 21-hydroxylase enzyme deficiency 
      (NC21OHD) causes hirsutism, acne and menstrual irregularities in women. 
      Clinically, patients with NC21OHD may be indistinguishable from other 
      hyperandrogenic women, as they all present with similar symptoms. An elevated 
      response of cortisol precursors like 17 alpha-hydroxyprogesterone (17-OHP) to 
      ACTH stimulation is a valuable diagnostic criteria. In this study, 32 hirsute 
      adolescent girls, aged 13-19 years, underwent i.v. adrenocorticotrophic hormone 
      (ACTH) (Synacthen 0.25 mg) stimulation test. The results were compared with those 
      of the controls. The plasma levels of 17 alpha-hydroxyprogesterone, cortisol, 
      dehydroepiandrosterone sulphate (DHEA-S), androstenedione, testosterone, follicle 
      stimulating hormone (FSH), luteinizing hormone (LH) and prolactin were 
      established before, and 60 min after the infusion of ACTH to both patients and 
      controls. Six patients demonstrated an increase in both the 17 
      alpha-hydroxyprogesterone levels and the 17 alpha-hydroxyprogesterone/cortisol 
      ratio on ACTH stimulation, almost twice that of the mean +/- 2SD in the control 
      group and ten times that in one patient. Six patients with abnormal elevation of 
      17 alpha-hydroxyprogesterone were considered heterozygotes for 21-hydroxylase 
      enzyme deficiency, and one patient was presumed to have NC21OHD. Human leukocyte 
      antigen (HLA) analysis supported these diagnoses. In this study, the incidence of 
      NC21OHD in hirsute adolescent girls in our population was investigated, and 
      NC21OHD was found in only one of 32 patients.
FAU - Akinci, A
AU  - Akinci A
AD  - Pediatric Endocrinology Unit, Dr Sami Ulus Children's Hospital, Ankara, Turkey.
FAU - Yordam, N
AU  - Yordam N
FAU - Ersoy, F
AU  - Ersoy F
FAU - Ulusahin, N
AU  - Ulusahin N
FAU - Oguz, H
AU  - Oguz H
LA  - eng
PT  - Journal Article
PL  - England
TA  - Gynecol Endocrinol
JT  - Gynecological endocrinology : the official journal of the International Society 
      of Gynecological Endocrinology
JID - 8807913
RN  - 0 (Hydroxyprogesterones)
RN  - 3XMK78S47O (Testosterone)
RN  - 409J2J96VR (Androstenedione)
RN  - 459AG36T1B (Dehydroepiandrosterone)
RN  - 57B09Q7FJR (Dehydroepiandrosterone Sulfate)
RN  - 68-96-2 (17-alpha-Hydroxyprogesterone)
RN  - 9002-60-2 (Adrenocorticotropic Hormone)
RN  - 9002-62-4 (Prolactin)
RN  - 9002-67-9 (Luteinizing Hormone)
RN  - 9002-68-0 (Follicle Stimulating Hormone)
RN  - WI4X0X7BPJ (Hydrocortisone)
SB  - IM
MH  - 17-alpha-Hydroxyprogesterone
MH  - Adolescent
MH  - *Adrenal Hyperplasia, Congenital
MH  - Adrenocorticotropic Hormone
MH  - Adult
MH  - Androstenedione/blood
MH  - Dehydroepiandrosterone/analogs & derivatives/blood
MH  - Dehydroepiandrosterone Sulfate
MH  - Female
MH  - Follicle Stimulating Hormone/blood
MH  - Hirsutism/*enzymology
MH  - Humans
MH  - Hydrocortisone/blood
MH  - Hydroxyprogesterones/blood
MH  - Luteinizing Hormone/blood
MH  - Prolactin/blood
MH  - Testosterone/blood
EDAT- 1992/06/01 00:00
MHDA- 1992/06/01 00:01
CRDT- 1992/06/01 00:00
PHST- 1992/06/01 00:00 [pubmed]
PHST- 1992/06/01 00:01 [medline]
PHST- 1992/06/01 00:00 [entrez]
AID - 10.3109/09513599209046392 [doi]
PST - ppublish
SO  - Gynecol Endocrinol. 1992 Jun;6(2):99-106. doi: 10.3109/09513599209046392.