PMID- 37045725 OWN - NLM STAT- MEDLINE DCOM- 20230705 LR - 20230705 IS - 1579-2129 (Electronic) IS - 0300-2896 (Linking) VI - 59 IP - 7 DP - 2023 Jul TI - Estimated Worldwide Prevalence of the PI*ZZ Alpha-1 Antitrypsin Genotype in Subjects With Chronic Obstructive Pulmonary Disease. PG - 427-434 LID - S0300-2896(23)00117-5 [pii] LID - 10.1016/j.arbres.2023.03.016 [doi] AB - INTRODUCTION: The prevalence of alpha1-antitrypsin PI*ZZ genotypes in patients with COPD is only partially known. We aimed to estimate this prevalence worldwide. METHOD: A systematic review of the literature was conducted for studies investigating the prevalence of COPD and the prevalence of severe alpha-1-antitrypsin deficiency (AATD) PI*ZZ genotype. Results are shown in tables and on a whole world interpolation map. RESULTS: Studies from 48 countries with available data (21 from Europe, 9 from the Americas, 5 from Africa, 11 from Asia and 2 from Australasia) were selected. About 235,000 individuals with PI*ZZ genotypes were accounted: 50% in Europe, 37% in America, 9% in Asia, 3% in Australasia and 1% in Africa. The estimated crude prevalence of COPD in adults older than 40 years was 12.45% in Europe, 13.51% in America, 13.22% in Africa, 11.70% in Asia and 11.86% in Australasia. The highest PI*ZZ weighted average prevalence among COPD subjects (expressed as 1/x [95% confidence intervals]) were found in Northern Europe (395 [252-576]) followed by Western (797 [538-1165]), Southern (944 [600-1475]) and Central Europe (1096 [687-1738]). Outside Europe, high values were found in Australia-New Zealand (1007 [684-1509]), Saudi Arabia (1276 [563-2961]), United States (1298 [1094-1540]), Canada (1482 [1057-2083]) and Thailand (1807 [717-4692]). In the rest of the world, prevalence was significantly lower, especially in vast regions of Asia and Africa where the PI*Z gene is practically non-existent. CONCLUSIONS: Severe AATD is associated with a significant number of cases of COPD, especially in Europe, USA, Canada, New Zealand and Australia. CI - Copyright (c) 2023 SEPAR. Published by Elsevier Espana, S.L.U. All rights reserved. FAU - Blanco, Ignacio AU - Blanco I AD - Alpha1-Antitrypsin Deficiency Spanish Registry, Spanish Society of Pneumology and Thoracic Surgery (SEPAR), Barcelona, Spain. FAU - Diego, Isidro AU - Diego I AD - Materials and Energy Dept, School of Mining Engineering, Oviedo University, Oviedo, Principality of Asturias, Spain. FAU - Castanon, Cesar AU - Castanon C AD - Materials and Energy Dept, School of Mining Engineering, Oviedo University, Oviedo, Principality of Asturias, Spain. FAU - Bueno, Patricia AU - Bueno P AD - Internal Medicine Dept, County Hospital of Jarrio, Jarrio, Principality of Asturias, Spain. FAU - Miravitlles, Marc AU - Miravitlles M AD - Pneumology Dept, Hospital Universitari Vall d'Hebron/Vall d'Hebron Research Institute (VHIR), Barcelona, Catalonia, Spain. Electronic address: marcm@separ.es. LA - eng LA - spa PT - Journal Article PT - Review DEP - 20230330 PL - Spain TA - Arch Bronconeumol JT - Archivos de bronconeumologia JID - 0354720 RN - 0 (alpha 1-Antitrypsin) SB - IM MH - Adult MH - Humans MH - *alpha 1-Antitrypsin Deficiency/epidemiology/genetics/complications MH - Prevalence MH - alpha 1-Antitrypsin/genetics MH - *Pulmonary Disease, Chronic Obstructive/epidemiology/genetics/complications MH - Genotype MH - Europe/epidemiology OTO - NOTNLM OT - Alpha-1 antitrypsin deficiency OT - COPD OT - Chronic obstructive pulmonary disease OT - Geographic Information Systems (GIS) technology OT - Inverse distance weighted (IDW) interpolation OT - PI*ZZ genotype OT - Prevalence OT - SERPINA1 EDAT- 2023/04/13 06:00 MHDA- 2023/07/05 06:42 CRDT- 2023/04/12 22:04 PHST- 2023/02/01 00:00 [received] PHST- 2023/03/11 00:00 [revised] PHST- 2023/03/21 00:00 [accepted] PHST- 2023/07/05 06:42 [medline] PHST- 2023/04/13 06:00 [pubmed] PHST- 2023/04/12 22:04 [entrez] AID - S0300-2896(23)00117-5 [pii] AID - 10.1016/j.arbres.2023.03.016 [doi] PST - ppublish SO - Arch Bronconeumol. 2023 Jul;59(7):427-434. doi: 10.1016/j.arbres.2023.03.016. Epub 2023 Mar 30.