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:: Volume 2, Issue 2 (1-2011) ::
Caspian J Intern Med 2011, 2(2): 245-248 Back to browse issues page
Severe generalized muscular atrophia, nerve optic atrophia, ear problem and disability with Pelger-Huet anomaly
Hassan Mahmoodi Nesheli , Naimeh Nakhjavani , Tahere Galini Moghaddam
Abstract:   (8124 Views)
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.
Full-Text [PDF 146 kb]   (3998 Downloads)    
Type of Study: Original Article | Subject: Infectious Diseases
Received: 2014/01/14 | Accepted: 2014/01/14 | Published: 2014/01/14
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Mahmoodi Nesheli H, Nakhjavani N, Galini Moghaddam T. Severe generalized muscular atrophia, nerve optic atrophia, ear problem and disability with Pelger-Huet anomaly. Caspian J Intern Med 2011; 2 (2) :245-248
URL: http://caspjim.com/article-1-110-en.html

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Volume 2, Issue 2 (1-2011) Back to browse issues page
Caspian Journal of Internal Medicine
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