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There are only a few cases of steroid cell tumours that have been described in the literature. Here, we present an exceptionally rare case of a steroid cell tumour arising from the ovary in early pregnancy. Copyright © 2020 J. Weishaupt and U. Herbst.Objective To describe unusual course and unusual phenotypic features in an adult patient with Kearns-Sayre syndrome (KSS). Case Report. The patient is a 49-year-old male with KSS, diagnosed clinically upon the core features, namely, onset before the age 20 of years, pigmentary retinopathy,