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- Title
Varied Clinical Manifestations of LRBA Deficiency (Immune Dysregulation Disorder).
- Authors
Munirathnam, Deenadayalan; Kumar, Vimal; Sivasankaran, Meena; Agrawal, Silky
- Abstract
Long-term outcome of LRBA deficiency in 76 patients after various treatment modalities as evaluated by the immune deficiency and dysregulation activity (IDDA) score. LRBA deûciency is an autosomal recessive disorder caused by biallelic mutations in the LRBA gene leading to immune dysregulation. Patients with LRBA deficiency show CTLA4 loss and immune dysregulation responsive to abatacept therapy. INDIAN PEDIATRICS 285 VOLUME 58 MARCH 15, 2021 CLINICAL CASE LETTERS intravenous antibiotics and catheter drainage remain the mainstays of treatment of neonatal empyema, VATS can be safely considered as a primary treatment modality to promote earlier recovery and shorten antibiotic therapy.
- Subjects
IMMUNOLOGIC diseases; CHILD patients; DIAGNOSIS; EMPYEMA; STEM cell transplantation; LYMPHOCYTE subsets
- Publication
Indian Pediatrics, 2021, Vol 58, Issue 3, p285
- ISSN
0019-6061
- Publication type
Letter
- DOI
10.1007/s13312-021-2175-9