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妊娠与急性卟啉病

Pregnancy and the acute porphyrias.

作者信息

Brodie M J, Moore M R, Thompson G G, Goldberg A, Low R A

出版信息

Br J Obstet Gynaecol. 1977 Oct;84(10):726-31. doi: 10.1111/j.1471-0528.1977.tb12480.x.

DOI:10.1111/j.1471-0528.1977.tb12480.x
PMID:921907
Abstract

An analysis is presented of the obstetric histories of 50 women with acute porphyria, comprising 39 with acute intermittent porphyria, 3 with variegate porphyria and 8 with hereditary coporporphyria. Fifty-four per cent of the women with acute intermittent porphyria had an acute attack of porphyria in pregnancy and/or the puerperium. Only one maternal death was recorded. One patient with variegate prophyria and two with hereditary coproporphyria had an attack related to pregnancy. The total fetal wastage was 13 per cent. The babies born to mothers with acute intermittent porphyria, who experienced an acute attack during pregnancy, were smaller than those in which no such attack occurred (P less than 0.001). In 13 non-porphyric primigravidae there was a rise in urinary excretion of delta-aminolaevulinic acid, porphobilinogen and coproporphyrin up to the 28th week of gestation. It is probable that pregnancy has some deleterious effects in acute prophyria but the prognosis of the porphyric pregnancy is much better than the literature suggests.

摘要

本文对50例急性卟啉病患者的产科病史进行了分析,其中包括39例急性间歇性卟啉病患者、3例杂合性卟啉病患者和8例遗传性粪卟啉病患者。54%的急性间歇性卟啉病女性在妊娠和/或产褥期发生了卟啉病急性发作。仅记录到1例孕产妇死亡。1例杂合性卟啉病患者和2例遗传性粪卟啉病患者发生了与妊娠相关的发作。总胎儿丢失率为13%。妊娠期间发生急性发作的急性间歇性卟啉病母亲所生的婴儿,比未发生此类发作的母亲所生的婴儿体重轻(P<0.001)。在13例非卟啉病初产妇中,直至妊娠28周,δ-氨基-γ-酮戊酸、卟胆原和粪卟啉的尿排泄量均有所增加。妊娠可能对急性卟啉病有一些有害影响,但卟啉病妊娠的预后比文献报道的要好得多。

相似文献

1
Pregnancy and the acute porphyrias.妊娠与急性卟啉病
Br J Obstet Gynaecol. 1977 Oct;84(10):726-31. doi: 10.1111/j.1471-0528.1977.tb12480.x.
2
Variegate porphyria.
Ann Intern Med. 1978 Aug;89(2):238-44. doi: 10.7326/0003-4819-89-2-238.
3
[Urinary excretion of porphyrins and their precursors. Longterm observations in acute intermittent porphyrias (author's transl)].[卟啉及其前体的尿排泄。急性间歇性卟啉病的长期观察(作者译)]
MMW Munch Med Wochenschr. 1979 Mar 30;121(13):457-8.
4
Composition of urinary coproporphyrin isomers I-IV in human porphyrias.人类卟啉症中尿粪卟啉异构体I-IV的组成
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Hormonal oral contraceptives, urinary porphyrin excretion and porphyrias.激素口服避孕药、尿卟啉排泄与卟啉病
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6
Delta-amino-levulinic acid, porphobilinogen, and porphyrin excretion throughout pregnancy in a patient with acute intermittent porphyria with "passive porphyria" in the infant.患有急性间歇性卟啉症且婴儿患有“被动性卟啉症”的患者在整个孕期中δ-氨基乙酰丙酸、胆色素原和卟啉的排泄情况。
J Lab Clin Med. 1961 Sep;58:437-44.
7
The porphyrias.卟啉病
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8
Diagnosis of porphyric syndromes: a practical approach in the era of molecular biology.卟啉症综合征的诊断:分子生物学时代的实用方法
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Porphyria: genetic and acquired.卟啉病:遗传性和后天性。
IARC Sci Publ. 1986(77):549-66.
10
Urinary excretion of porphyrins, porphobilinogen and δ-aminolaevulinic acid following an attack of acute intermittent porphyria.急性间歇性卟啉症发作后卟啉、卟啉原和 δ-氨基酮戊酸的尿排泄。
J Clin Pathol. 2014 Jan;67(1):60-5. doi: 10.1136/jclinpath-2012-201367. Epub 2013 Aug 1.

引用本文的文献

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Acute hepatic porphyrias: Recommendations for evaluation and long-term management.急性肝卟啉病:评估与长期管理建议
Hepatology. 2017 Oct;66(4):1314-1322. doi: 10.1002/hep.29313. Epub 2017 Sep 4.
2
Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study.患有卟啉症的女性不良妊娠结局的风险增加:基于人群的队列研究。
J Inherit Metab Dis. 2011 Feb;34(1):217-23. doi: 10.1007/s10545-010-9231-2. Epub 2010 Oct 27.
3
A retrospective analysis of outcome of pregnancy in patients with acute porphyria.
急性卟啉症患者妊娠结局的回顾性分析。
J Inherit Metab Dis. 2010 Oct;33(5):591-6. doi: 10.1007/s10545-010-9142-2. Epub 2010 Jun 22.
4
Diagnosis and management of porphyria.卟啉病的诊断与管理
BMJ. 2000 Jun 17;320(7250):1647-51. doi: 10.1136/bmj.320.7250.1647.
5
Acute intermittent porphyria in pregnancy--a case report.
Ir J Med Sci. 1982 May;151(5):155-7. doi: 10.1007/BF02940167.
6
Effect of stanozolol on delta-aminolaevulinic acid synthase and hepatic monooxygenase activity in man and rat.司坦唑醇对人和大鼠δ-氨基乙酰丙酸合酶及肝脏单加氧酶活性的影响。
Eur J Clin Pharmacol. 1984;26(5):587-90. doi: 10.1007/BF00543490.
7
Management of attacks of acute porphyria.急性卟啉病发作的管理。
Drugs. 1987 Nov;34(5):604-16. doi: 10.2165/00003495-198734050-00007.
8
The effects of age on the structure and porphyrin synthesis of the harderian gland of the female golden hamster.年龄对雌性金黄仓鼠哈德氏腺结构和卟啉合成的影响。
J Anat. 1988 Oct;160:157-66.
9
Changes in Harderian gland activity in the female golden hamster during the oestrous cycle, pregnancy and lactation.雌性金黄地鼠在发情周期、怀孕和哺乳期时哈德氏腺活动的变化。
Biochem J. 1979 Mar 15;178(3):597-604. doi: 10.1042/bj1780597.