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| Omen Syndrome:A Case Report and Review of Related Literature |
| YUE Shu-zhen, LUO Yang-yang, LI Ke-yao, et al |
| Department of Dermatology,Hunan Children's Hospital,Changsha Hunan 410007 |
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Abstract 【Objective】 To investigate the etiology, pathogenesis, diagnostic criteria, differential diagnosis and treatment of Omenn syndrome. 【Methods】 A case of Omenn syndrome was reported and the related literature was reviewed. 【Results】 A girl aged 1 year and 2 months had recurrent cough and fever, repeated erythema and scaling all over her body. There was no special history and personal history. Her sister had a history of "autoimmune hemolysis" and died. The results of gene examination showed that RAG1 was homozygous and the therapeutic effect was poor. 【Conclusion】The prognosis of Omenn syndrome is poor, and it often dies of organ failure and severe infection.
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Received: 13 June 2022
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2022, Vol. 39 
