Halil İbrahim YAKUT, Celal ÖZCAN, Ayşe METİN, Sevim ÜNAL, Ümmühan ÇAY, Mehmet Yekta ÖNCEL, Elif ÇELİKEL

Stafilooksik Omfalit İle Prezente Olan Konjenital Nötropeni Olgusu

Kostmann sendromu, ağır konjenital nötropeni, kemik iliğinde miyelosit olgunlaşmasının promyelosit aşamasında duraklama, mutlak nötrofil sayısının MNS 200/mm3’den az olması ile karakterize bir hastalıktır. Yaşamın ilk aylarından itibaren hayatı tehdit eden infeksiyonlar, özellikle tekrarlayan otitis media, mastoidit, pnömoni, omfalit, lenfadenopati, kronik gingivit ve cilt apseleri ile prezente olur. Hastaların önemli bir kısmı %90 rekombinan insan granülosit koloni stimüle edici faktör G-CSF tedavisine iyi yanıt vermektedir. Bu olgu, yenidoğan döneminde stafilokoksik omfalit ile bulgu veren Kostmann sendromunun klinik özelliklerini ve tedavisini tartışmak amacıyla sunuldu.

Anahtar Kelimeler:

Nötropeni, Bebek, Yenidoğan, Stafilokokal Enfeksiyonlar, Granülosit koloni uyarıcı faktör

The Case Of Congenitale Neutropenia Which Is Presented With Staphylococcal Omphalitis

Kostmann Syndrome, severe congenital neutropenia, is characterized by an arrest in myeloid maturation at the promyelocyte stage of bone marrow, resulting in an absolute neutrophil count of less than 200/mm3. The disorder presents with recurrent otitis media, mastoiditis, pneumonia, omphalitis, lymhadenopathy, chronic gingivitis and skin abscess following birth. The majority of patients present with life-threatening infections during the first 6 months of life The significiant amount of patients 90% give good response to granulocyte colony stimulating factor G-CSF treatment. This case is reported to discuss the clinical feature and treatment of Kostmann Syndrome which is presented with staphylococcal omphalitis in neonatal period.

Keywords:

Neutropenia, Infant, Newborn, Staphylococcal Infections, Granulocyte Colony-Stimulating Factor,

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