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Alpha-1 Antitrypsin Deficiency and Pulmonary Morbidity in Patients with Primary Immunodeficiency Disease: A Single-Center Experience.

Georg EversArik Bernard SchulzeMichael ThrullJan-Philipp HeringChristoph SchülkeRainer WiewrodtHelmut WittkowskiLars Henning SchmidtMichael Mohr
Published in: Canadian respiratory journal (2020)
In Germany, the estimated percentage for PI∗ZZ and PI∗MZ is 0.01% and 1.9%, respectively. As demonstrated, the ratio in our study group was even higher. We identified seven patients with AATD. Since AATD contributes to pulmonary morbidity in PID patients, systematic underdiagnosis of the coexistence might yield a strong clinical impact. Hence, AAT analysis should be offered to all patients with confirmed PID diagnoses. To strengthen this finding, we suggest the investigation of larger databases.
Keyphrases
  • end stage renal disease
  • pulmonary hypertension
  • ejection fraction
  • newly diagnosed
  • chronic kidney disease
  • prognostic factors
  • peritoneal dialysis
  • big data
  • patient reported