Menstrual cycle and gonadal steroid effects on symptomatic hyperammonaemia of urea-cycle-based and idiopathic aetiologies.
We report two female patients, one with a known inborn error of ureagenesis and the other of unknown cause, in whom recurrent, transient episodes of severe hyperammonaemia increased in frequency and severity with sexual maturity and parturition. Both responded to ovarian steroids administered continuously to suppress ovulation and menstruation, and ultimately to simple hysterectomy. These studies suggest a new therapeutic approach to defective ureagenesis in female patients and a relationship between ammonia production or disposal and the menstrual cycle.
Grody
W W
WW
Department of Pathology and Laboratory Medicine, UCLA School of Medicine 90024-1732.
Chang
R J
RJ
Panagiotis
N M
NM
Matz
D
D
Cederbaum
S D
SD
D000328:Adult; D000641:Ammonia / Q000097:blood*; D003278:Contraceptives, Oral, Hormonal / Q000627:therapeutic use*; D004997:Ethinyl Estradiol / Q000627:therapeutic use*; D019304:Ethinyl Estradiol-Norgestrel Combination; D005260:Female; D006801:Humans; D020162:Hyperargininemia; D008597:Menstrual Cycle; D009644:Norgestrel / Q000627:therapeutic use*; D014508:Urea / Q000378:metabolism*
D003278:Contraceptives, Oral, Hormonal; D009644:Norgestrel; D004997:Ethinyl Estradiol; D000641:Ammonia; D019304:Ethinyl Estradiol-Norgestrel Combination; D014508:Urea