# Adrenogenital disorder - Category: chronic - Review status: reviewed (every claim checked against a document named on this page) - Sources: Congenital Adrenal Hyperplasia - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK448098/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class TD99.02 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care · Congenital Adrenal Hyperplasia - StatPearls - NCBI Bookshelf (NBK448098) - Verified date: 2026-08 ## Verified against - No dose - referral pathway, no medicine given in primary care - Adrenogenital disorder - disease-level clinical article (adrenogenital-syndrome-clinical.txt) ## Treatment metadata - Referral & safety-netting (no drug therapy) - Hydrocortisone — injection ## Complete treatment card ```text ADRENOGENITAL DISORDER Sources: Congenital Adrenal Hyperplasia - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK448098/ · ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class TD99.02 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care · Congenital Adrenal Hyperplasia - StatPearls - NCBI Bookshelf (NBK448098) Review status: REVIEWED against No dose - referral pathway, no medicine given in primary care, Adrenogenital disorder - disease-level clinical article (adrenogenital-syndrome-clinical.txt) (2026-08) IS IT THIS? - reference only, to read alongside your own examination SYMPTOMS - what the patient reports (6) - Poor feeding, darkened skin, vomiting, or a sudden collapse in infancy can signal cortisol deficiency [collapse · poor feeding · vomiting] - Salt-wasting forms cause dehydration and low blood volume that can progress to shock [dehydration · shock] - Some forms instead cause high blood pressure, a hypertensive crisis, and heart failure from mineralocorticoid excess [hypertension] - Early pubic hair and a growth spurt in childhood can result from excess androgen - Genetic females can have irregular periods and difficulty conceiving due to lack of ovulation [abnormal uterine bleeding · infertility · irregular periods] - In young women the condition can show up as irregular periods, polycystic ovary syndrome, or excess hair growth [abnormal uterine bleeding · excess hair · irregular periods] SIGNS - what you find (5) - Ambiguous genitalia at birth with an enlarged clitoris can be seen in affected girls - Genetic males may show a normally sized penis with marked virilization but no sperm production [virilisation] - Some enzyme defects leave genetic males under-masculinized, appearing anywhere from mild hypospadias to fully female genitalia - Some newborn boys instead show darkened skin and an enlarged penis at birth - Prader staging and the External Masculinization or Genitalia Score standardize how the genitals are documented [virilisation] TESTS (9) - Newborn screening measures 17-OHP levels two to four days after birth - Classic 21-hydroxylase deficiency typically pushes 17-OHP above 10,000 ng/dL, while the milder form runs 1,000 to 10,000 - An ACTH (cosyntropin) stimulation test is used when 17-OHP is only mildly or ambiguously elevated - 21-deoxycortisol is a more reliable screening marker than 17-OHP because it is less affected by gestational age or sample timing - All classic forms show high ACTH with low cortisol, since glucocorticoid production is deficient - Salt-wasting forms show low sodium, high potassium, acidosis, low aldosterone, and high plasma renin activity - Imaging is generally not needed unless bleeding into the adrenal gland is suspected - A pelvic ultrasound can define the internal anatomy in a newborn with ambiguous genitalia - Genetic testing is done to establish genotypic sex in infants with ambiguous genitalia IF NOT THIS - what else fits (5) - Other causes of primary adrenal failure - adrenal hypoplasia, autoimmune destruction, bleeding into the gland - need to be considered - Genetic or acquired kidney disease causing high potassium or low sodium can look similar - Ambiguous genitalia can also come from a placental enzyme defect or a maternal androgen- secreting tumor - Polycystic ovary syndrome is the differential for irregular periods with excess androgen and infertility - High blood pressure with low potassium and suppressed renin points instead to Liddle syndrome or a DOC-producing tumor Source StatPearls "Congenital Adrenal Hyperplasia" - disease-level clinical article Status traced to the source above Rx: Main treatment | Emergency stress dose in adrenal crisis MAIN TREATMENT 1. REFERRAL & SAFETY-NETTING (NO DRUG THERAPY) [1st line] Adult Includes congenital adrenal hyperplasia and related virilising disorders; primary care recognises signs (ambiguous genitalia, early puberty, virilisation) and refers to paediatric endocrinology. - Refer, with advice Peds Children follow the same pathway: recognise and refer. No primary-care medicine is implied. Source No dose - referral pathway, no medicine given in primary care Why Includes congenital adrenal hyperplasia and related virilising disorders; primary care recognises signs (ambiguous genitalia, early puberty, virilisation) and refers to paediatric endocrinology. Caution Salt-wasting congenital adrenal hyperplasia in a newborn is a life-threatening emergency needing urgent referral. Referral is the pathway; the medicines listed alongside are what primary care can give before or while it happens. RED FLAG - Adrenal crisis is the most common cause of mortality in CAH; patients must be educated on stress dosing, wear medical ID, and carry an emergency hydrocortisone injection kit. EMERGENCY STRESS DOSE IN ADRENAL CRISIS 2. HYDROCORTISONE [1st line] Adult 50 to 100 mg/m2 by intramuscular or intravenous injection, as a single stress dose Peds Children get the same 50 to 100 mg/m2 stress dose; a newborn is given 25 mg, then 100 mg/m2 per day divided every 6 hours. Dosing here is by body surface area, not per kilogram. Source Congenital Adrenal Hyperplasia - StatPearls - NCBI Bookshelf (NBK448098) Why A known CAH patient who is vomiting, febrile or injured can decompensate within hours, and the injection has to be given before transfer rather than after it. That is the one moment a GP has to act. Caution Do not use it where there is known hypersensitivity to hydrocortisone or an untreated systemic infection. This is the emergency dose only. The daily replacement doses in congenital adrenal hyperplasia are set and adjusted by an endocrinologist and must not be started in the clinic. Give it alongside fluid resuscitation and arrange transfer; the injection alone does not treat the salt loss or the low blood sugar. Egypt SOLU-CORTEF 100MG/2ML VIAL EIPICO > PFIZER 16.00 EGP HYDROCORTISONE SODIUM SUCCINATE 100MG I.V./I.M.VIAL EIPICO 26.00 EGP SIGMACORTIN 500MG PD. FOR I.M./I.V. SIGMA-TEC > SIGMA 34.00 EGP Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy. ``` --- Dawaa Reference is a reference for prescribers, not a medical device, and does not replace clinical judgement. [Privacy policy](/privacy)