Clinical Heterogeneity of Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked: Pulmonary Involvement as a Non-Classical Disease Manifestation


BARIŞ S. , Schulze I., Ozen A. O. , AYDINER E. , Altuncu E., Karasu G. T. , et al.

JOURNAL OF CLINICAL IMMUNOLOGY, cilt.34, ss.601-606, 2014 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 34 Konu: 6
  • Basım Tarihi: 2014
  • Doi Numarası: 10.1007/s10875-014-0059-7
  • Dergi Adı: JOURNAL OF CLINICAL IMMUNOLOGY
  • Sayfa Sayısı: ss.601-606

Özet

IPEX (Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked) is a rare X-linked recessive life-threatening disorder characterized by autoimmunity and early death. Pulmonary complication related with IPEX has not been elucidated exactly. Here, we report 4 IPEX patients, 3 of which died from severe pulmonary disease.