Activated Phosphoinositide 3-Kinase δ Syndrome: a Large Pediatric Cohort from a Single Center in China.
Luyao QiuYanping WangWenjing TangQiuyun YangTing ZengJunjie ChenXuemei ChenLiang ZhangLina ZhouZhiyong ZhangYunfei AnXuemei TangXiao-Dong ZhaoPublished in: Journal of clinical immunology (2022)
Clinical spectrum of APDS is heterogeneous. This cohort's high incidence of localized BCG-induced granulomatous inflammation and tuberculosis indicates Mycobacterial susceptibility in APDS patients. Rapamycin is effective in improving lymphoproliferation and cytopenia. HSCT is an option for those who have severe complications and poor response to other treatments.
Keyphrases
- end stage renal disease
- mycobacterium tuberculosis
- risk factors
- newly diagnosed
- ejection fraction
- oxidative stress
- chronic kidney disease
- peritoneal dialysis
- prognostic factors
- drug induced
- high glucose
- early onset
- tyrosine kinase
- pulmonary tuberculosis
- patient reported outcomes
- hiv aids
- interstitial lung disease
- hepatitis c virus
- hematopoietic stem cell
- adverse drug