Severe generalized muscular atrophia, nerve optic atrophia, ear problem and disability with Pelger-Huet anomaly

Severe generalized muscular atrophia, nerve optic atrophia, ear problem and disability with Pelger-Huet anomaly


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نویسندگان: نعیمه حمزه زاده نخجوانی , حسن محمودی

نشریه: Caspian Journal of Internal Medicine , 2-2 , 245 - 248 ,

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کد مقاله 1259
عنوان فارسی مقاله Severe generalized muscular atrophia, nerve optic atrophia, ear problem and disability with Pelger-Huet anomaly
عنوان لاتین مقاله Severe generalized muscular atrophia, nerve optic atrophia, ear problem and disability with Pelger-Huet anomaly
نوع مقاله گزارش مورد
IF
عنوان نشریه Caspian Journal of Internal Medicine
شماره مجله 2-2
دوره مجله
صفحه شروع و پایان - 248
نمایه نامه Chemical Abstract
سال انتشار شمسی
سال انتشار میلادی 2011
DOI
آدرس لینک مقاله/ همایش در شبکه اینترنت

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Background: The Pelger-Huet anomaly dominantly is a rare and benign inherited defect of terminal neutrophil differentiation. Although neutrophil migration may be minimally impaired, granulocytes function is otherwise normal association abnormalities such as ocular, musculoskeletal are reported very rare. Case: An eight year-old boy with good consciousness but severe muscular atrophia and difficulty in respiration was admitted in Amirkola Hospital at Babol University of Medical Sciences Babol, Iran. The patient was febrile at presentation. The chest x-ray was normal and other causes of respiratory problem were ruled out. The patient and his mother have 30% to 40% band and Pelger-Huet cells in peripheral blood smear. He gradually has gotten hearing loss and decreased visual acuity for three years. He has optic nerve atrophia. Conclusion: The patient is an unusual type of Pelger-Huet anomaly with multiple organ dysfunctions probably due to simultaneous muscular degenerative disease

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نویسنده نفر چندم مقاله
نعیمه حمزه زاده نخجوانیدوم
حسن محمودیاول

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