Edel Yonatan, Mamet Rivka, Sagy Iftach, Snast Igor, Kaftory Ran, Mimouni Tomer, Levi Assi
Porphyria Center, Rabin Medical Center, Petah Tikva, Israel.
Medicine B, Assuta Ashdod Medical Center, Ashdod, Israel.
Rambam Maimonides Med J. 2023 Jan 29;14(1):e0003. doi: 10.5041/RMMJ.10490.
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.
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.
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.
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.
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。
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。
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。
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。