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Menstrual cycle and gonadal steroid effects on symptomatic hyperammonaemia of urea‐cycle‐based and idiopathic aetiologies
Author(s) -
Grody W. W.,
Chang R. J.,
Panagiotis N. M.,
Matz D.,
Cederbaum S. D.
Publication year - 1994
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf00711592
Subject(s) - menstruation , urea cycle , menstrual cycle , ovulation , endocrinology , medicine , biology , physiology , hormone , biochemistry , amino acid , arginine
Summary 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.