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静息型遗传性粪卟啉病和杂合性卟啉病患者进行25小时禁食,并发症风险较低。

A 25-Hour Fast Among Quiescent Hereditary Coproporphyria and Variegate Porphyria Patients is Associated With a Low Risk of Complications.

作者信息

Edel Yonatan, Mamet Rivka, Sagy Iftach, Snast Igor, Kaftory Ran, Mimouni Tomer, Levi Assi

机构信息

Porphyria Center, Rabin Medical Center, Petah Tikva, Israel.

Medicine B, Assuta Ashdod Medical Center, Ashdod, Israel.

出版信息

Rambam Maimonides Med J. 2023 Jan 29;14(1):e0003. doi: 10.5041/RMMJ.10490.

Abstract

OBJECTIVE

In patients with acute hepatic porphyria (AHP), prolonged fasting is a known trigger of AHP attacks. Despite this, some Jewish AHP patients-mainly hereditary coproporphyria (HCP) and variegate porphyria (VP) patients-fast for 25 consecutive hours during the traditional Jewish holy day known as Yom Kippur. In this study, we evaluated the effect of the fast on these patients.

METHODS

A retrospective study and survey of AHP patients in Israel was carried out. Patients were asked whether they have fasted and whether any symptoms were induced by this fast. Patients' medical records were reviewed for an emergency department (ED) visit following Yom Kippur between 2007 and 2019. Only 3 acute intermittent porphyria (AIP) patients reported fasting; they were excluded from analysis.

RESULTS

A total of 21 HCP patients and 40 VP patients completed the survey; 30 quiescent patients reported they fast, while 31 did not fast. The majority of fasting patients (96.67%) reported no symptoms following a fast. We found no statistically significant association between ED visits 1 week (0.26% in both fasting and non-fasting patients) or 1 month (2.1% visits in non-fasting versus 0.78% in fasting patients) following Yom Kippur. Of the symptomatic ED visits following a fast, none were defined as severe attacks.

CONCLUSION

A 25-hour fast in stable HCP and VP patients did not increase the risk of an acute attack and can probably be regarded as safe.

摘要

目的

在急性肝卟啉病(AHP)患者中,长时间禁食是已知的AHP发作诱因。尽管如此,一些犹太AHP患者——主要是遗传性粪卟啉病(HCP)和杂合性卟啉病(VP)患者——在传统犹太圣日赎罪日期间连续禁食25小时。在本研究中,我们评估了禁食对这些患者的影响。

方法

对以色列的AHP患者进行了一项回顾性研究和调查。询问患者是否禁食以及此次禁食是否诱发了任何症状。查阅了患者在2007年至2019年赎罪日后到急诊科就诊的病历。只有3例急性间歇性卟啉病(AIP)患者报告禁食;他们被排除在分析之外。

结果

共有21例HCP患者和40例VP患者完成了调查;30例病情静止的患者报告禁食,31例未禁食。大多数禁食患者(96.67%)报告禁食后无症状。我们发现在赎罪日后1周(禁食和非禁食患者均为0.26%)或1个月(非禁食患者就诊率为2.1%,禁食患者为0.78%)内,急诊科就诊情况无统计学显著关联。在禁食后出现症状的急诊科就诊病例中,没有一例被定义为严重发作。

结论

稳定的HCP和VP患者禁食25小时不会增加急性发作风险,可能可视为安全。

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本文引用的文献

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Porphyria: What Is It and Who Should Be Evaluated?卟啉病:它是什么以及谁需要接受评估?
Rambam Maimonides Med J. 2018 Apr 19;9(2):e0013. doi: 10.5041/RMMJ.10333.
4
Porphyria.卟啉病
N Engl J Med. 2017 Nov 23;377(21):2101. doi: 10.1056/NEJMc1712682.
5
Acute Porphyrias.急性卟啉病
J Emerg Med. 2015 Sep;49(3):305-12. doi: 10.1016/j.jemermed.2015.04.034. Epub 2015 Jul 7.
6
Porphyrias: A 2015 update.卟啉病:2015年最新进展
Clin Res Hepatol Gastroenterol. 2015 Sep;39(4):412-25. doi: 10.1016/j.clinre.2015.05.009. Epub 2015 Jul 2.
8
Posterior reversible encephalopathy syndrome in acute intermittent porphyria.急性间歇性卟啉病中的后部可逆性脑病综合征
Pediatr Neurol. 2014 Sep;51(3):457-60. doi: 10.1016/j.pediatrneurol.2014.05.016. Epub 2014 May 21.
10
Modern diagnosis and management of the porphyrias.卟啉病的现代诊断与管理
Br J Haematol. 2006 Nov;135(3):281-92. doi: 10.1111/j.1365-2141.2006.06289.x. Epub 2006 Sep 4.

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