Perioperative care following strabismus surgery for the patient with severe congenital neutropenia

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Perioperative care following strabismus surgery for the patient with severe congenital neutropenia

A 10-year-old girl presented with esotropia. She had 35 prism diopters of esodeviation at far and near with left inferior oblique muscle overaction (+3). Cycloplegic refraction was +0.5D OD and +0.75D OS, and visual acuity was 20/25 in each eye. The patient was being treated with G-CSF (Leufokine) due to her severe congenital neutropenia (WBC 3350, neutrophil 7.3%, and ANC 170/ul). Despite of l...

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Laryngeal stenosis in a patient with severe congenital neutropenia.

OBJECTIVES/HYPOTHESIS We report the first case of laryngeal stenosis with granuloma in a patient with severe congenital neutropenia (SCN). STUDY DESIGN Case report and retrospective review. METHODS Review of medical records. RESULTS A 6-year-old female who was diagnosed with SCN presented with a cough and wheezing. An endoscopic study revealed laryngeal stenosis with granuloma. Tracheotom...

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Severe congenital neutropenia: case report

Introduction Neutropenia is defined in the literature as absolute neutrophil counts in peripheral blood of less than 1500 cells/mm3 in more than one year old and less than 2000cells/mm3 in children in the first year old of life. Neutropenia is classified as mild, moderate or severe, and may be congenital or acquired, persistent or not. Kostmann syndrome is a severe neutropenia, the incidence va...

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Granulopoiesis in severe congenital neutropenia.

The pathogenesis of the granulopoietic failure in three children with severe congenital neutropenia was studied. Mature neutrophils were absent from both peripheral blood and bone marrow. Assay of bone marrow granulocyte colony-forming cells (CFU-C) in a methylcellulose tissue culture system using colony-stimulating activity (CSA) from peripheral blood leukocytes demonstrated normal or increase...

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Severe congenital neutropenia: new lane for ELANE.

In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutropenia (SCN).

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ژورنال

عنوان ژورنال: Case Reports in Clinical Medicine

سال: 2013

ISSN: 2325-7075,2325-7083

DOI: 10.4236/crcm.2013.21017