Internal medicine (Tokyo, Japan) · 2026
A Delayed Progression of Porphyria-Associated Kidney Disease After Hemin Therapy in a Patient with Acute Intermittent Porphyria: A Case Report.
СтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Ishimura Takuya, Wells Ken Jc, Shimada Arisa, Shikano Yumika, Hanafusa Ai, Morita Hajime, Handa Takaya, Kan Youngna, Suzuki Hiroyuki, Matsubara Takeshi, Muso Eri, Tsukamoto Tatsuo
Аннотация
Acute intermittent porphyria (AIP) is a condition characterized by the neurotoxic accumulation of δ-aminolevulinic acid and porphobilinogen. However, chronic kidney disease is an under-recognized complication. A 50-year-old woman experienced recurrent abdominal pain with repeated acute kidney injury and hyponatremia, resulting in a rapid decline in renal function. Marked elevations of both metabolites, as well as her genetic mutation, confirmed AIP. The administration of hemin markedly decreased the frequency of porphyria attacks and significantly attenuated the annual estimated glomerular filtration rate decline. Although the patient eventually developed end-stage kidney disease, hemin treatment slowed the progression of renal failure.