Asians with cystic fibrosis in the UK have worse disease outcomes than clinic matched white homozygous ΔF508 controls

Abstract

These data suggest that the Asian CF phenotype is as severe as the white controls with the homozygous delta F508 phenotype but is worse in some outcomes, especially in Asian females. Socio-cultural factors and rare CF genotypes may contribute to the severity of CF in this vulnerable group.

Long-Term Benefits of Inhaled Tobramycin in Children with Cystic Fibrosis: First Clinical Observations from Poland

“…Improvement in lung function was observed after the first year of treatment, followed by a slow decline. This finding is consistent with that of Grzincich et al [14], who also showed improvement in pulmonary function in the first year and deterioration in the second year of treatment with TOBI. This waning effect of the treatment could be related to antibiotic resistance.…”

Clinical evidence that V456A is a Cystic Fibrosis causing mutation in South Asians

“…Mutation detection rate in South Asians is also lower (50% [8]) when compared to 77% in Caucasians [13] and many South Asian CF patients have undetected mutations [11].…”

“…Mei-Zahav et al [5] have described an increased incidence of poor weight gain in South Asian CF patients, despite pancreatic sufficiency, and decreased survival into adulthood. McCormick et al [11], have described that lung disease in Asian patients is severe with worse FEV 1 and FVC when compared to Caucasian patients who are homozygous to the delta F 508 genotype.…”

Developing and optimizing a decisional instrument using self-reported ancestry for carrier screening in a multi-ethnic society

“…Although uncommon, CF has been found among Africans and Asians, and HbPs have also been found among Europeans. [27][28][29][30] Some carriers, therefore, will be falsely excluded from screening on the basis of ancestry. Bobadilla et al 6 argued that disease-based data about CF prevalence in Africa may be artificial (because the condition is masked by non-survival based in other factors), and a The categories "Only Instrument A matches with interview," "Only Instrument B matches with interview," and "Both instruments incorrect" were combined in the column "No" because the number of cases in these categories were too small and there were no statistical differences found between these three categories.…”