急性间歇性卟啉症的姑息治疗--病例报告。

IF 1.1 Q4 HEALTH CARE SCIENCES & SERVICES
Indian Journal of Palliative Care Pub Date : 2024-07-01 Epub Date: 2024-08-23 DOI:10.25259/IJPC_14_2024
Neethu Susan Abraham, Seema Mishra, Saurabh Vig
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引用次数: 0

摘要

急性间歇性卟啉症属于一类罕见疾病,其特征是血红素的生物合成不足。卟啉症会给患者带来严重的身体和精神症状,因此姑息治疗已成为治疗卟啉症的重要手段。姑息治疗从整体上照顾患者,满足疾病过程所需的各个方面。关于卟啉症的姑息治疗,文献中存在许多空白。我们报告了一例 16 岁女性患者的病例,该患者因剧烈腹痛、腰痛和对称性双下肢疼痛而由神经内科转至姑息病房接受支持性治疗。本病例描述了姑息治疗病房在卟啉症治疗中的姑息治疗方面,从转诊到最后的治疗都取得了成功。多学科姑息治疗团队对患者进行了管理,并为患者和家属提供了必要的干预措施。AIP 中的姑息治疗需要得到重视,在这些病例中需要利用姑息治疗服务。在印度等国家无法获得特定的治疗措施--血红素,这进一步强调了为患者和家属提供长期支持性护理的必要性。该病例显示了姑息治疗在整个病程中的重要性,因为它是一种慢性疾病,发病率高,症状严重。本病例让我们认识到,对于这类慢性疾病,不应仅采用传统的治疗方法,而应纳入姑息治疗。尽早融入姑息治疗有助于探索疾病的各个领域。这是首例强调卟啉症姑息治疗的病例,填补了文献空白。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Palliative Care Aspects of Acute Intermittent Porphyria - A Case Report.

Acute intermitttent porphyria belongs to a rare group of diseases hallmarked by deficient biosynthesis of heme. It carries a significant symptom burden, both physical and emotional,and therefore palliative care has emerged as an essential tool in the armamentarium of porphyria management . It takes care of the patient as a whole and caters to all aspects that the disease process demands. There are many lacunae in the literature regarding the palliative management of porphyria. We are reporting a case of a 16-year-old female who presented with severe abdominal pain, lower backache and symmetrical bilateral lower limb pain to the palliative ward referred by the neurology department for supportive care. This case describes the palliative care aspects of porphyria management which was successfully provided in the palliative care unit right from referral till the last. A multidisciplinary palliative care team managed the patient, and the necessary interventions were provided to the patient and family. Palliative acre in AIP needs to be emphasized, and palliative care services need to be utilized in these cases. The unavailability of specific treatment measure, heme, in countries like India further emphasizes the need for long-term supportive care for the patient and family. The case shows the importance of palliative care throughout the disease course as it is a chronic disease with significant morbidity and carries a heavy symptom burden. This case provides the insight that rather than conventional management alone for such chronic diseases, palliative care should be incorportated. Early integration with palliative care helps in exploring all the domains of disease. This is one of the first cases reported highlighting palliative care in porphyria , bridging the gap in the literature.

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来源期刊
Indian Journal of Palliative Care
Indian Journal of Palliative Care HEALTH CARE SCIENCES & SERVICES-
CiteScore
2.30
自引率
0.00%
发文量
57
期刊介绍: Welcome to the website of the Indian Journal of Palliative Care. You have free full text access to recent issues of the journal. The links connect you to •guidelines and systematic reviews in palliative care and oncology •a directory of palliative care programmes in India and IAPC membership •Palliative Care Formulary, book reviews and other educational material •guidance on statistical tests and medical writing.
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