CASE-REPORTS OF SUCCESSFUL PREGNANCY IN WOMEN WITH MAPLE SYRUP URINE DISEASE AND PROPIONIC ACIDEMIA

被引：40

作者：

VANCALCAR, SC

HARDING, CO

DAVIDSON, SR

BARNESS, LA

WOLFF, JA

机构：

[1] UNIV WISCONSIN HOSP & CLIN, WAISMAN CTR MENTAL RETARDAT & HUMAN DEV, DEPT PEDIAT & MED GENET, MADISON, WI USA

[2] UNIV WISCONSIN HOSP & CLIN, DEPT OBSTET & GYNECOL, MADISON, WI USA

来源：

AMERICAN JOURNAL OF MEDICAL GENETICS | 1992年 / 44卷 / 05期

关键词：

ORGANIC ACIDEMIA; MAPLE SYRUP URINE DISEASE; CARNITINE SUPPLEMENTATION;

D O I：

10.1002/ajmg.1320440523

中图分类号：

Q3 [遗传学];

学科分类号：

071007 ; 090102 ;

摘要：

We report on 2 women with organic acidemias, one with classical maple syrup urine disease and another with mild propionic acidemia in which protein restricted diets and carnitine supplementation were successfully employed to manage pregnancies. Healthy infants were delivered without maternal metabolic decompensation.

引用

页码：641 / 646

页数：6

共 31 条

[1]

[Anonymous], 1989, RECOMMENDED DIETARY

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

ARN, PH ;

HAUSER, ER ;

THOMAS, GH ;

HERMAN, G ;

HESS, D ;

BRUSILOW, SW .

NEW ENGLAND JOURNAL OF MEDICINE, 1990, 322 (23) :1652-1655

[3]

BRANDT IK, 1974, PEDIATRICS, V53, P391

[4] PLASMA CARNITINE AND RENAL-CARNITINE CLEARANCE DURING PREGNANCY [J].

CEDERBLAD, G ;

FAHRAEUS, L ;

LINDGREN, K .

AMERICAN JOURNAL OF CLINICAL NUTRITION, 1986, 44 (03) :379-383

[5] URINARY-EXCRETION OF L-CARNITINE AND ACYLCARNITINES BY PATIENTS WITH DISORDERS OF ORGANIC-ACID METABOLISM - EVIDENCE FOR SECONDARY INSUFFICIENCY OF L-CARNITINE [J].

CHALMERS, RA ;

ROE, CR ;

STACEY, TE ;

HOPPEL, CL .

PEDIATRIC RESEARCH, 1984, 18 (12) :1325-1328

[6]

CHALMERS RA, 1982, ORGANIC ACIDS MAN, P350

[7]

DALLAIRE L, 1974, J OBSTET GYN BR COMM, V81, P761

[8]

DROGARI E, 1987, LANCET, V2, P927

[9]

GHADIMI H, 1964, PEDIATRICS, V33, P500

[10] RENAL EXCRETION OF NUTRIENTS IN PREGNANCY [J].

HYTTEN, FE .

POSTGRADUATE MEDICAL JOURNAL, 1973, 49 (575) :625-629

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