الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Neutropenia is the abnormal reduction in the ANC of the blood
< 1.5 ×109 /L, severe neutropenia when ANC < 1.5 ×109 /L and is “chronic” when it lasts longer than 3 months.
Antineutrophil antibodies (ANA) are autoantibodies directed against a variety of neutrophil-specific antigens. In the literature, controversial data exists about if ANA testing is necessary for screening, confirming a diagnosis, or predicting outcomes of neutropenia. Additionally, it is not apparent if ANA testing can help to distinguish between various forms of neutropenia.
Herein, we tried to screen for the presence of ANA in children with SCN at Alexandria university children’s Hospital.
This was a cross-sectional observational prospective study that was conducted on 28 children with chronic neutropenia registered at the Hematology and immunology Clinic of Alexandria University Children’s Hospital. The study included patients who had, for at least 3 months, SCN (ANC < 0.5 x 109 /L) or moderate neutropenia (ANC < 1.0 x 109 /L) associated with recurrent infections. Their data were retrieved from patients’ records. The patients represented the four main Governorates referring to Alexandria University Children’s Hospital, namely Alexandria (71.4%), Beheira (14.3%), Matrouh (3.6%), and Kafr el-Sheikh (10.7%).
Females were more predominant than males (60,7% vs. 39.3%, respectively) with a male-to-female ratio of 1:1.5. The median age at the onset of symptoms was 4.5 months while the median age at the discovery of neutropenia was 27.2 months. More than half of the patients (53.5%) had a positive family history.
Based on patients’ clinical and routine laboratory data the preliminary classification was severe congenital neutropenia (39.3%), immune neutropenia (25%), cyclic neutropenia (14.3%), and an undetermined preliminary diagnosis (21.4%). While on the basis of genetic testing, the diagnosis was as the following severe congenital neutropenia, immune neutropenia, cyclic neutropenia, undetermined diagnosis, and resolved in 14.3%, 17.9%, 14.3%, 42.9%, and 10.7%, respectively.
Most of the patients had a history of severe infections (82.1%) and repeated hospital admissions (67.9%). Also, a history of intensive care unit admission was reported (28.6%). Fever is the most frequent symptom of presentation (92.9%), in addition to pharyngitis (78.6%), oral ulcers (71.4%), skin abscesses/ infections (53.6%), colitis (57.1%), pneumonia (53.6%), otitis (46.4%), sinusitis (39.3%), while bacteremia (32.1%), periodontitis/gingivitis (17.9%), urinary tract infections (14.3%), lung abscess (10.7%), peritonitis (10.7%), and chronic diarrhea (3.6%) were less frequent.
Concerning the outcomes, until th