Orellaniinimürgistusest põhjustatud neerukahjustus – haigusjuhtude kirjeldus ja kirjanduse ülevaade

2020. aasta augustis diagnoositi Eestis seitse seenemürgistusjuhtu, mille põhjuseks oli orellaniinimürgistus, mis oli tekkinud vöödikute tarvitamisest. Orellaniinimürgistus kulgeb esmalt väheste sümptomitega, kuid põhjustab ägeda neerukahjustuse ning patsiendid satuvad haiglaravile alles nädalate möödudes, kui jääkained on kuhjunud ning esinevad olulised elektrolüütide häired. Tartu Ülikooli Kliinikumi hospitaliseeriti lühikese ajavahemiku jooksul neli patsienti, Põhja-Eesti Regionaalhaiglasse kolm patsienti. Kuus patsienti vajasid hemodialüüsravi ning kolmel patsiendil on krooniline neeruasendusravi vajalik ka 10 kuud pärast mürgistust. Neerukahjustuse kujunemiseks kulub tavaliselt mõni nädal, seetõttu võib mürgistuse algpõhjus jääda märkamata ning käesoleva haigusjuhtude kirjeldamise eesmärk on olla abiks edasiste mürgistusjuhtude diagnoosimisel ja käsitlusel

Increased Mitochondrial Protein Levels and Bioenergetics in the Musculus Rectus Femoris of Wfs1-Deficient Mice

Wfs1 deficiency leads to a progressive loss of plasma insulin concentration, which should reduce the consumption of glucose in insulin-dependent tissues, causing a variety of changes in intracellular energy metabolism. Our objective here was to assess the changes in the amount and function of mitochondrial proteins in different muscles of Wfs1-deficient mice. Mitochondrial functions were assayed by high-resolution oxygraphy of permeabilized muscle fibers; the protein amount was evaluated by liquid chromatography tandem mass spectrometry (LC/MS/MS) analysis and mRNA levels of the uncoupler proteins UCP2 and UCP3 by real-time PCR; and citrate synthase (CS) activity was determined spectrophotometrically in muscle homogenates. Compared to controls, there were no changes in proton leak and citrate synthase activity in the heart and m. soleus tissues of Wfs1-deficient mice, but significantly higher levels of both of these factors were observed in the m. rectus femoris; mitochondrial proteins and mRNA of UCP2 were also higher in the m. rectus femoris. ADP-stimulated state 3 respiration was lower in the m. soleus, remained unchanged in the heart, and was higher in the m. rectus femoris. The mitochondrial protein amount and activity are higher in Wfs1-deficient mice, as are mitochondrial proton leak and oxygen consumption in m. rectus femoris. These changes in muscle metabolism may be important for identifying the mechanisms responsible for Wolfram syndrome and diabetes