MENSTRUAL-CYCLE AND GONADAL-STEROID EFFECTS ON SYMPTOMATIC HYPERAMMONEMIA OF UREA-CYCLE-BASED AND IDIOPATHIC ETIOLOGIES

被引：17

作者：

GRODY, WW

CHANG, RJ

PANAGIOTIS, NM

MATZ, D

CEDERBAUM, SD

机构：

[1] UNIV CALIF LOS ANGELES,SCH MED,DEPT PATHOL & LAB MED,LOS ANGELES,CA 90024

[2] UNIV CALIF LOS ANGELES,SCH MED,DEPT OBSTET & GYNECOL,LOS ANGELES,CA 90024

[3] UNIV CALIF LOS ANGELES,SCH MED,DEPT MED,LOS ANGELES,CA 90024

[4] UNIV CALIF LOS ANGELES,SCH MED,DEPT PSYCHIAT,LOS ANGELES,CA 90024

[5] UNIV CALIF LOS ANGELES,SCH MED,DEPT PEDIAT,LOS ANGELES,CA 90024

[6] UNIV CALIF LOS ANGELES,SCH MED,MENTAL RETARDAT RES CTR,LOS ANGELES,CA 90024

[7] NORTHRIDGE HOSP,MED CTR,DEPT MED,NORTHRIDGE,CA

[8] NORTHRIDGE HOSP,MED CTR,DEPT OBSTET & GYNECOL,NORTHRIDGE,CA

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1994年 / 17卷 / 05期

关键词：

D O I：

10.1007/BF00711592

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：566 / 574

页数：9

共 33 条

[1] HYPERAMMONEMIA IN WOMEN WITH A MUTATION AT THE ORNITHINE CARBAMOYLTRANSFERASE LOCUS - A CAUSE OF POSTPARTUM COMA [J].