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一名患有弥漫性盆腔疼痛的女性患者的卟啉病诊断:病例研究

Diagnosing Porphyria in a Female Patient With Diffuse Pelvic Pain: A Case Study.

作者信息

Hoffpauir Lauren N, Olexo Rob, Hamric Hiliary

机构信息

Family Medicine, West Virginia School of Osteopathic Medicine, Lewisburg, USA.

Pediatrics, West Virginia School of Osteopathic Medicine, Lewisburg, USA.

出版信息

Cureus. 2024 Nov 30;16(11):e74839. doi: 10.7759/cureus.74839. eCollection 2024 Nov.

DOI:10.7759/cureus.74839
PMID:39737267
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11684490/
Abstract

Porphyria is a rare and often underdiagnosed metabolic disorder that leads to abdominal pain, pelvic pain, changes in neurological states, and digestive issues due to a buildup of porphyrins in the body. Diagnosis can be especially difficult in young women, where symptoms of porphyria often overlap with gynecological conditions. We present a case of a 37-year-old female patient who was experiencing persistent lower abdominal and pelvic pain, brain fog and confusion, and a rash after long sun exposure. Despite extensive evaluation from her gynecologist, including transvaginal ultrasound and pap smears, no abnormalities were found. Further evaluation performed by her primary care physician showed elevated porphyrin levels, suggesting a possible diagnosis of either hereditary coproporphyria or acute intermittent porphyria. After implementing a low-protein diet, the patient reported significant pain relief, which further supports a diagnosis of porphyria. This case highlights not only the challenges of diagnosing porphyria in young women but also the value of considering metabolic disorders in a preliminary differential diagnosis. Diagnostic laboratory testing of urine and stool remains the gold standard. However, advances in genetic testing have shown to be more conclusive, yet these tests are costly.

摘要

卟啉病是一种罕见且常被漏诊的代谢紊乱疾病,由于体内卟啉积累,会导致腹痛、盆腔疼痛、神经状态改变和消化问题。对于年轻女性而言,卟啉病的诊断尤其困难,因为其症状常与妇科疾病重叠。我们报告一例37岁女性患者,她长期暴露于阳光下后出现持续的下腹部和盆腔疼痛、脑雾和意识混乱以及皮疹。尽管她的妇科医生进行了广泛评估,包括经阴道超声检查和巴氏涂片检查,但未发现异常。她的初级保健医生进一步评估显示卟啉水平升高,提示可能诊断为遗传性粪卟啉病或急性间歇性卟啉病。实施低蛋白饮食后,患者报告疼痛明显缓解,这进一步支持了卟啉病的诊断。该病例不仅凸显了年轻女性诊断卟啉病的挑战,还强调了在初步鉴别诊断中考虑代谢紊乱的价值。尿液和粪便的诊断实验室检测仍是金标准。然而,基因检测的进展已显示出更具决定性,但这些检测成本高昂。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d648/11684490/57a524a6aa46/cureus-0016-00000074839-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d648/11684490/57a524a6aa46/cureus-0016-00000074839-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d648/11684490/57a524a6aa46/cureus-0016-00000074839-i01.jpg

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本文引用的文献

1
Acute Intermittent Porphyria: A Review and Rehabilitation Perspective.急性间歇性卟啉病:综述与康复视角
Cureus. 2023 Aug 28;15(8):e44260. doi: 10.7759/cureus.44260. eCollection 2023 Aug.