Menstrual cycle and gonadal steroid effects on symptomatic hyperammonaemia of urea-cycle-based and idiopathic aetiologies |
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| Authors: | W. W. Grody R. J. Chang N. M. Panagiotis D. Matz S. D. Cederbaum |
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| Affiliation: | (1) Division of Medical Genetics, UCLA School of Medicine, 90024-1732 Los Angeles, CA;(2) Department of Pathology and Laboratory Medicine, UCLA School of Medicine, 90024-1732 Los Angeles, CA;(3) Department of Obstetrics and Gynecology, UCLA School of Medicine, 90024-1732 Los Angeles, CA;(4) Department of Medicine, UCLA School of Medicine, 90024-1732 Los Angeles, CA;(5) Department of Psychiatry, UCLA School of Medicine, 90024-1732 Los Angeles, CA;(6) Department of Pediatrics; Mental Retardation Research Center, UCLA School of Medicine, 90024-1732 Los Angeles, CA;(7) Department of Medicine, Northridge Hospital Medical Center, Northridge, CA, USA;(8) Department of Obstetrics and Gynecology, Northridge Hospital Medical Center, Northridge, CA, USA;(9) Present address: Department of Obstetrics and Gynecology, University of California, Davis |
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| Abstract: | ![]()
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. |
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