Yılmaz TABEL, İLKE MUNGAN AKIN, Mümtaz AKTAŞ, Neşe KARADAĞ, Hale KIRIMLIOĞLU

Hiperimmünglobulin E sendromlu sekiz yaşında erkek çocukta nefrotik sendrom: Vaka sunumu

Hiperimmünglobulin E sendromu, yüksek serum immün-globulin E seviyeleri, kronik egzema ve tekrarlayan piyojenik infeksiyonlarla karakterize ender bir immün yetmezlik hastalığıdır. Bu makalede; nefrotik sendrom bulguları ile başvuran, Hiperimmünglobulin E sendromu ve minimal lezyon hastalığı tanısı alan, steroid tedavisinden yarar gören sekiz yaşında erkek hasta sunularak, ender görülen bu immün yetmezlik hastalığında ender bir tutulum olan böbrek hastalığı konusunda dikkatli olmayı, gerekirse tanıda böbrek biyopsisi yapılmasını ve tedavide de immünsupresif tedavilerin kullanılmasını vurgulamak amaçlanmıştır.

Anahtar Kelimeler:

Çocuk, Job sendromu, Erkek, Nefrotik sendrom, Steroidler, Tedavi sonucu

Nephrotic syndrome in eight years old male child with hyperimmunoglobulin E syndrome: Case report

Hyperimmunoglobulin E syndrome is a rare multisystem immunodeficiency disease characterized by high serum immunoglobulin E levels, chronic eczema, and recurrent pyogenic infections. In this manuscript, an eight years old male who was, admitted with nephrotic syndrome findings, and was diagnosed as hyperimmunoglobulin E syndrome and minimal change disease and responded to steroid treatment is presented. With this case, we wanted to draw attention to the possibility of renal disease in this rare immune deficiency syndrome, and to the importance of renal biopsy for diagnosis and immunosuppressive drugs for treatment.

Keywords:

Child, Job's Syndrome, Male, Nephrotic Syndrome, Steroids, Treatment Outcome,

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