CFDB - Cystic Fibrosis DataBase

primary studies published, non RCT

Role of arterial hypoxemia and pulmonary mechanics in exercise limitation in adults with cystic fibrosis.

Study design (if review, criteria of inclusion for studies)

Observational study.

Participants

All CF patients who received a kidney transplant at the national kidney transplant center between 1993 and 2022. 14 patients received a kidney transplant over the study period. Median age at transplantation was 35 (IQR 31, 40) years.

Interventions

Kidney transplantation. Recipients of the contralateral donor kidney were selected as a control group.

Outcome measures

The peri-operative and long-term outcomes of kidney transplantation. Primary outcomes included 1-, 5-, and 10- year death-censored graft survival and overall survival. Secondary outcomes included peri-operative morbidity, acute graft rejection, delayed graft function (DGF), and length of stay (LOS).

Main results

The 1-, 5-, and 10-year death-censored graft survival was 92, 74, and 74% in the CF group compared to 100, 92, and 92% in the control group (p = .44). The 1-, 5-, and 10-year overall survival in the CF group was 85, 66, and 57% compared to 100, 92, and 82% in the control group (p = .036). There was no significant difference in peri-operative outcomes including LOS (10 vs. 11 days, p = .84), ICU admission (1 vs. 0 patients, p > .99), acute rejection episodes (2 vs. 1 patients, p > .99), and DGF (1 vs. 2 patients, p = .60).

Authors' conclusions

CF patients have good long-term graft survival, however, overall survival was worse compared to a matched cohort. These data provide important information for transplant surgeons when considering suitable donor allografts in this unique patient population.

Keywords: Adult; Caregivers; Home; Home Care Services; non pharmacological intervention - psyco-soc-edu-org; non pharmacological intervention - surg; telemedicine; transplantation; Organization;