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Jordans' Anomaly As a Red Flag for Neutral Lipid Storage Diseases Publisher Pubmed



Safavi M1 ; Vasei M2 ; Motamed F3
Authors

Source: Fetal and Pediatric Pathology Published:2022


Abstract

Background: Jordans’ anomaly is characterized by lipid vacuoles in granulocytes which are observed in neutral lipid storage diseases like Chanarin-Dorfman syndrome. Case report: This six-year-old boy had skin ichthyosis, elevated liver enzymes, and prominent vacuoles in neutrophils, eosinophils, monocytes, and basophils (Jordans’ anomaly), leading to the diagnosis of Chanarin-Dorfman Syndrome, which was successfully treated with medium-chain triglyceride oil. Conclusion: Jordans’ anomaly is a red flag for the diagnosis of neutral lipid storage diseases especially in patients with ichthyosis and elevated liver enzymes. © 2020 Taylor & Francis Group, LLC.
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