Search

  • HOME
  • Search
Review Article
Pulmonology
Evidence-based management guidelines for noncystic fibrosis bronchiectasis in children and adolescents
Eun Lee, Kyunghoon Kim, You Hoon Jeon, In Suk Sol, Jong Deok Kim, Taek Ki Min, Yoon Ha Hwang, Hyun-Ju Cho, Dong In Suh, Hwan Soo Kim, Yoon Hee Kim, Sung-Il Woo, Yong Ju Lee, Sungsu Jung, Hyeon-Jong Yang, Gwang Cheon Jang
Clin Exp Pediatr. 2024;67(9):418-426.   Published online January 23, 2024
· We suggest offering long-term macrolides to children with noncystic fibrosis bronchiectasis with frequent exacerbations (conditional recommendation, moderate quality of evidence).
· We do not recommend the routine use of mucolytic agents, inhaled corticosteroids, or nonsteroidal anti-inflammatory drugs to prevent exacerbation of bronchiectasis in children (inconclusive, very low quality of evidence).
· We recommend the use of nebulized hypertonic saline to prevent exacerbations and improve the lung function of children with noncystic fibrosis bronchiectasis (weak recommendation, moderate quality of evidence).
Case Report
Immunology
A novel BTK gene mutation, c.82delC (p.Arg28 Alafs*5), in a Korean family with X-linked agammaglobulinemia
Jeongeun Lee, Minhee Rhee, Taek Ki Min, Hae In Bang, Mi-Ae Jang, Eun-Suk Kang, Hee-Jin Kim, Hyeon-Jong Yang, Bok Yang Pyun
Clin Exp Pediatr. 2016;59(Suppl 1):S49-S52.   Published online November 30, 2016

X-linked agammaglobulinemia (XLA) is a hereditary humoral immunodeficiency that results from Bruton’s tyrosine kinase (BTK) gene mutations. These mutations cause defects in B-cell development, resulting in the virtual absence of these lymphocytes from the peripheral circulation. Consequently, this absence leads to a profound deficiency of lg all isotypes, and an increased susceptibility to encapsulated bacterial infections. A 15-month-old Korean boy...