Primary immune deficiency in bronchiectasis.

The primary purpose of the immune system is to protect the body from infection. Failure of the immune system can lead to repeated infections. The aim of this review is to discuss primary immune deficiency (PID) and its relationship with bronchiectasis in adults. It examines treatment options for patients with PID and provides practical details of how nurses can empower these patients to reduce their risk of respiratory infections.

Subcutaneous immunoglobulin therapy home.

Subcutaneous immunoglobulin (SClg) replacement therapy is a new clinical service development in adult respiratory care undertaken at the Royal Brompton and Harefield Foundation Trust. SCIg therapy reduces appropriate patients' susceptibility to recurrent acute and chronic respiratory infections. Patients with specific chronic respiratory conditions and antibody deficiency can be treated with SCIg therapy at home, rather than with intravenous immunoglobulin (IVIg) therapy in hospital. Using a personalised teaching programme and managing the daily demands of the clinical service promotes greater concordance with treatment. With over 34% of our immunoglobulin therapy patients now on SCIg replacement therapy, this treatment innovation has brought about high levels of patient satisfaction and cost savings to trust budgets.

HLA-C and killer cell immunoglobulin-like receptor genes in idiopathic bronchiectasis.

RATIONALE: In idiopathic bronchiectasis, lung inflammation and chronic bacterial infection lead to progressive lung damage. A possible role for natural killer (NK) cells is suggested by the observation that familial bronchiectasis occurs in a rare group of individuals with impaired HLA class I expression and consequent NK cell dysfunction. OBJECTIVE: Because the HLA-C locus and killer cell immunoglobulin-like receptors (KIRs) are of key importance for NK cell recognition, we analyzed HLA-C/KIR combinations by genotyping patients with idiopathic bronchiectasis. METHODS: Genomic DNA from 96 individuals with idiopathic bronchiectasis and 101 control subjects was analyzed by polymerase chain reaction with sequence-specific primers. High-resolution HLA-C genotyping was performed in addition to KIR analysis. RESULTS: HLA-Cw*03 alleles and, in particular, HLA-C group 1 homozygosity are associated with the presence of bronchiectasis. Analysis of the relationship between HLA-C and KIR genes suggests a shift to activatory NK cell function. CONCLUSION: This is the first demonstration of genetic susceptibility in idiopathic bronchiectasis. The association with HLA-C group 1 homozygosity, and the interplay between HLA-C and KIR genes, argue for a role for NK cells in the progressive lung damage seen in this disease. This will require further investigation using functional studies.