IPEX syndrome / Immunodysregulation polyendocrinopathy enteropathy X-linked Syndrome
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- Опубліковано 15 жов 2024
- 📌𝐅𝐨𝐥𝐥𝐨𝐰 𝐨𝐧 𝐈𝐧𝐬𝐭𝐚𝐠𝐫𝐚𝐦 :- / drgbhanuprakash
IPEX syndrome / Immunodysregulation polyendocrinopathy enteropathy X-linked Syndrome #usmle #mbbs
Immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a lethal syndrome first described as a unique entity by Powell et al in 1982. It most commonly manifests with early onset, insulin-dependent diabetes mellitus; severe watery diarrhea, often with accompanying failure to thrive; and dermatitis. Other clinical features are more variable in their expression.
Most affected children die within the first 2 years of life. IPEX syndrome is an X-linked recessive disorder with exclusive expression in males. The identification of mutations in the forkhead box protein 3 (FOXP3) gene associated with IPEX syndrome and a murine model has generated a considerable amount of interest and research. This has also extended into the therapeutic spectrum, with new immunosuppressive regimens and use of hematopoietic stem cell transplantation (HSCT).
#ipexsyndrome #usmle #usmlestep1 #medvizz #fmge #usmlepreparation #medicalstudents #nationalexitexam #usmleprep #drgbhanuprakash #neetpg2023 #mbbslectures #usmleprep
Thanks doctor
welcome
Nice
Can they have alive viral vaccines? Like MMR vaccines?
فعلاً ليس هناك أجمل من سماع القرآن الكريم خاصة باصوات جميلة. فقد وهبنى ربى هذة النعمه العظيمه فوظفتها فى تلاوة القرآن الكريم. فهل هناك من يدعمنى وجزاكم الله خيراً 🍀🌾🍀🌹🌹🌹🍀🌾🍀«
نعم القرآن جميل!