CONGENITAL ADRENAL HYPERPLASIA

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Presentation transcript:

CONGENITAL ADRENAL HYPERPLASIA WILSON C. CUA MD DPPS DPSPME

CAH ONE OF THE DISEASES INCLUDED IN THE PHILIPPINE NEWBORN SCREEN 21 HYDROXYLASE DEFICIENCY 90-95% LIFE THREATENING IF NOT DIAGNOSED ON TIME

PRESENTATION USUALLY FEMALES - VIRILIZATION MALES - NORMAL MAYBE SALT LOSING OR NOT

PRESENTATION GENITAL 21 HYDROXYLASE 11 HYDROXYLASE 3 B HYDROXYSTEROID FEMALE - AMBIGUITY MALE - NORMAL 11 HYDROXYLASE SAME AS 21 HYDROXYLASE HYPERTENSION 3 B HYDROXYSTEROID FEMALE - VIRILIZED MALE - UNDER VIRILIZED 17 ALPHA HYDROXYLASE/17,21 LYASE FEMALE - NORMAL MALE UNDER VIRILIZED

THE DEGREE OF AMBIGUITY THE SEVERITY OF ENZYME DEFICIENCY PRESENTATION THE DEGREE OF AMBIGUITY THE SEVERITY OF ENZYME DEFICIENCY

ADRENAL CRISIS LOW SODIUM HIGH POTASSIUM LOW BLOOD SUGAR

TREATMENT ABC’S CORTISOL REPLACMENT HYDROCORTISONE 20 MG/M2 IN CASES OF EMERGENCIES HYDROCORTISONE 100MG LOADING AND 50 MG EVERY 6 HRS MAINTAINANCE.

TREATMENT FOR MAINTAINANCE SALT SUPPLIMENT HYDROCORTISONE 9 ALPHA FLUDROCORTISONE ACETATE PHILIPPINES – PREDNISONE SALT SUPPLIMENT

MONITORING PROGESTERONE 17 OH PROGESTERONE PLASMA RININ ACTIVITY ANDROSTENEDIONE TESTOSTERONE

UNTREATED CASES PROGRESSIVE PENILE OR CLITORAL ENLARGEMENT ADVANCE BONE AGE TALL CHILD SHORT ADULT

UNDIAGNOSED FEMALES PRECOSCIOUS PUBERTY (ISO OR HETEROSEXUAL) SIGNS OF HYPERANDROGENISM INFERTILITY/MENSTRUAL PROBLEMS SEVERE REFRACTORY ACNE MALE PATTERN BOLDNESS POLYCYSTIC OVARIAN DISEASE PATIENT

TAKE HOME DO NOT LABEL THE SEX, IF YOU ARE NOT SURE ALWAYS DO NEWBORN SCREEN ALWAYS DO CHROMOSOMAL ANALYSIS IN CAH STEROID IS YOUR FRIEND