BACKGROUND: Alpha-1 antitrypsin deficiency is a common genetic condition that predisposes to emphysema and liver disease. Alpha-1 antitrypsin deficiency is under-recognized, so affected individuals often experience long delays in diagnosis and visits to multiple physicians before correct diagnosis. Reasoning that inadequate knowledge about alpha-1 antitrypsin deficiency could contribute to this under-recognition, we designed this study to evaluate internal medicine house officers' and respiratory therapists' (RTs) knowledge of alpha-1 antitrypsin deficiency. METHODS: We evaluated knowledge of alpha-1 antitrypsin deficiency with a Web-based test containing 30 multiple-choice questions. Invitations to take the test were sent via e-mail to all internal medicine house officers and RTs at The Cleveland Clinic main campus hospital. We assessed test scores by profession, years of training/experience, and self-assessed knowledge of alpha-1 antitrypsin deficiency. RESULTS: Of 332 invitees, 202 (61%) responded, of whom 165 (50%) provided complete responses (99 RTs, 66 physicians). The mean scores (percent of correct answers) were 54% and 52% for physicians and RTs, respectively (P = .25). The scores did not differ among the physicians when examined by subspecialty (pulmonary/critical care vs other) or post-graduate education level (P = .94). RTs who had graduated from a 4-year respiratory therapy program had a higher mean score than those who had graduated from a 2-year program (56% vs 50%, P = .02). Respondents' whose self-assessment of their knowledge about alpha-1 antitrypsin deficiency was "somewhat knowledgeable" had higher test scores than any other self-assessed knowledge level, regardless of profession. CONCLUSIONS: These results indicate a generally low level of knowledge about alpha-1 antitrypsin deficiency among physicians and RTs. Causes of under-recognition of alpha-1 antitrypsin deficiency, including the possibility of poor knowledge as a contributor, warrant further study.
BACKGROUND:Alpha-1 antitrypsin deficiency is a common genetic condition that predisposes to emphysema and liver disease. Alpha-1 antitrypsin deficiency is under-recognized, so affected individuals often experience long delays in diagnosis and visits to multiple physicians before correct diagnosis. Reasoning that inadequate knowledge about alpha-1 antitrypsin deficiency could contribute to this under-recognition, we designed this study to evaluate internal medicine house officers' and respiratory therapists' (RTs) knowledge of alpha-1 antitrypsin deficiency. METHODS: We evaluated knowledge of alpha-1 antitrypsin deficiency with a Web-based test containing 30 multiple-choice questions. Invitations to take the test were sent via e-mail to all internal medicine house officers and RTs at The Cleveland Clinic main campus hospital. We assessed test scores by profession, years of training/experience, and self-assessed knowledge of alpha-1 antitrypsin deficiency. RESULTS: Of 332 invitees, 202 (61%) responded, of whom 165 (50%) provided complete responses (99 RTs, 66 physicians). The mean scores (percent of correct answers) were 54% and 52% for physicians and RTs, respectively (P = .25). The scores did not differ among the physicians when examined by subspecialty (pulmonary/critical care vs other) or post-graduate education level (P = .94). RTs who had graduated from a 4-year respiratory therapy program had a higher mean score than those who had graduated from a 2-year program (56% vs 50%, P = .02). Respondents' whose self-assessment of their knowledge about alpha-1 antitrypsin deficiency was "somewhat knowledgeable" had higher test scores than any other self-assessed knowledge level, regardless of profession. CONCLUSIONS: These results indicate a generally low level of knowledge about alpha-1 antitrypsin deficiency among physicians and RTs. Causes of under-recognition of alpha-1 antitrypsin deficiency, including the possibility of poor knowledge as a contributor, warrant further study.
Authors: Florian M Karl; Rolf Holle; Robert Bals; Timm Greulich; Rudolf A Jörres; Annika Karch; Armin Koch; Stefan Karrasch; Reiner Leidl; Holger Schulz; Claus Vogelmeier; Margarethe E Wacker Journal: Respir Res Date: 2017-04-17
Authors: Myriam Calle Rubio; Joan B Soriano; José Luis López-Campos; Juan J Soler-Cataluña; Bernardino Alcázar Navarrete; José Miguel Rodríguez González-Moro; Marc Miravitlles; Miriam Barrecheguren; Manuel E Fuentes Ferrer; Juan Luis Rodriguez Hermosa Journal: PLoS One Date: 2018-06-28 Impact factor: 3.240
Authors: Mark Brantly; Michael Campos; Angela M Davis; Jeanine D'Armiento; Kenneth Goodman; Kathi Hanna; Miriam O'Day; John Queenan; Robert Sandhaus; James Stoller; Charlie Strange; Jeffrey Teckman; Adam Wanner Journal: Orphanet J Rare Dis Date: 2020-04-19 Impact factor: 4.123
Authors: Irene Belmonte; Alexa Nuñez; Miriam Barrecheguren; Cristina Esquinas; Mònica Pons; Rosa M López-Martínez; Gerard Ruiz; Albert Blanco-Grau; Roser Ferrer; Joan Genescà; Marc Miravitlles; Francisco Rodríguez-Frías Journal: Int J Chron Obstruct Pulmon Dis Date: 2020-10-07