Autor: |
Fabian, Elisabeth, Reisinger, Anja, Ribitsch, Werner, Stadlbauer, Vanessa, Eherer, Andreas J., Roller-Wirnsberger, Regina, Toplak, Hermann, Fickert, Peter, Krejs, Guenter J. |
Zdroj: |
Wiener Klinische Wochenschrift; Apr2023, Vol. 135 Issue 7/8, p203-209, 7p |
Abstrakt: |
Keywords: Acute intermittent porphyria; SIADH; Hemin infusion; Givosiran; Constipation EN Acute intermittent porphyria SIADH Hemin infusion Givosiran Constipation 203 209 7 04/24/23 20230401 NES 230401 Presentation of case B Dr. A.J. Eherer showing characteristic burgundy red colored urine due to formation of porphobilin after 1 hr of exposure to light and room air when the diagnosis of acute intermittent porphyria was established in 1994 in the patient who is currently receiving givosiran as the first patient in Austria described by Dr. Stadlbauer (see text). Transplantation of the liver of a patient with acute intermittent porphyria to a recipient with another cause of end-stage liver disease can result in the development of acute intermittent porphyria in the recipient [[25]]. Manifest or overt acute intermittent porphyria is considered when patients develop typical acute neurovisceral attacks, neurologic symptoms such as peripheral neuropathy but also SIADH (hyponatremia) and psychiatric disturbances including hallucinations [[6], [29]]. [Extracted from the article] |
Databáze: |
Complementary Index |
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