Registro español de pacientes con déficit de alfa-1 antitripsina: evaluación de la base de datos y análisis de la población incluida

Lara, Beatríz; Blanco, Ignacio; Martínez, María Teresa; Rodrı́guez, Esther; Bustamante, Ana; Maldonado, Francisco Casas; Cadenas, Sergio; Lázaro, Lourdes; Torres, María Luisa García; Curi, Sergio; Esquinas, Cristina; Dası́, Francisco; Escribano, Amparo; Herrero, Inés Martínez; Martı́nez-Delgado, Beatriz; Michel, Francisco Javier; Rodríguez‐Frías, Francisco; Miravitlles, Marc

doi:10.1016/j.arbres.2016.05.003

Cited by 30 publications

(3 citation statements)

References 16 publications

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“…In our study, the frequency at baseline and the incidence during follow-up of emphysema was higher than any other AATD-associated PE. This observation supports other published studies that report that 40% to 92% of patients with AATD experience pulmonary exacerbations [12][13][14]25]. Taken together, the data from this study indicate that AATD-associated PEs were not only more severe in patients with a severe AATD clinical course, as evidenced by the need for hospitalization within 1 day of the event, but also more likely to occur in this patient population.…”

Section: Discussionsupporting

confidence: 91%

“…It is noteworthy, however, that a higher percentage of patients with a severe AATD clinical course received augmentation therapy. These findings support the results of a population-based analysis from a Spanish AATD registry showing that a higher percentage of patients with AATD who received augmentation therapy had AATDassociated COPD and emphysema compared with patients who did not receive augmentation therapy [25]. Taken together, this suggests augmentation therapy is considered more often after patients have progressed to a more severe clinical course.…”

Section: Discussionsupporting

confidence: 86%

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Alpha-1 Antitrypsin Deficiency-Associated Clinical Manifestations and Healthcare Resource Use in the United States

Herrera

Joseph

Brouwer

et al. 2021

COPD: Journal of Chronic Obstructive Pulmonary Disease

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Pulmonary events (PEs) associated with alpha-1 antitrypsin deficiency (AATD) can have a severe clinical course and increase healthcare resource use (HRU). However, AATD-associated HRU and healthcare costs have not been extensively described. This study describes and compares realworld HRU and healthcare costs among US patients with severe (requiring hospitalization after AATD-related PE) versus nonsevere AATD clinical course. Administrative healthcare claims for patients with a second primary AATD diagnosis between 6/1/2008 and 12/31/2017 were analyzed from 2 databases (requiring continuous enrollment 6 months preceding diagnosis). Patient baseline characteristics and AATD-associated PE incidence rates, HRU, and healthcare costs during follow-up were compared in patients with severe versus nonsevere AATD. Of 5109 patients with a second AATD diagnosis, 2674 (severe, n ¼ 711 [26.6%]; nonsevere, n ¼ 1963 [73.4%]) had !1 AATD-associated PE. PE incidence per 100 person-years was higher in patients with severe versus nonsevere AATD. Annual incidences (mean ± SD) of emergency department (1.2 ± 5.7 vs. 0.4 ± 1.2), inpatient (1.3 ± 2.5 vs. 0.1 ± 0.5), and outpatient (10.3 ± 15.9 vs. 5.7 ± 13.2) visits were higher in patients with severe versus nonsevere AATD. Median (interquartile range) annual costs were also higher for patients with severe versus nonsevere AATD for emergency department ($185

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 86%

Alpha-1 Antitrypsin Deficiency-Associated Clinical Manifestations and Healthcare Resource Use in the United States

Herrera

Joseph

Brouwer

et al. 2021

COPD: Journal of Chronic Obstructive Pulmonary Disease

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“…However, the number of patients diagnosed with AATD is much lower than expected according to epidemiologic studies 9 and most patients are not correctly diagnosed until late in the course of their pulmonary or liver disease, after many visits to multiple physicians. 10,11 Studies based on databases of medical records are increasingly used in clinical research, and since they represent real-life clinical practice, they may help to detect gaps or inadequacies in diagnosis and treatment. 12 In this context, we performed the present study with the aim of exploring the patterns of AATD diagnosis and their evolution over the last 5 years.…”

Section: Introductionmentioning

confidence: 99%

<p>Trends in Diagnosis of Alpha-1 Antitrypsin Deficiency Between 2015 and 2019 in a Reference Laboratory</p>

Belmonte

Núñez

Barrecheguren

et al. 2020

COPD

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Background: Alpha-1 antitrypsin deficiency (AATD) remains largely underdiagnosed despite recommendations of healthcare institutions and programmes designed to increase awareness. The objective was to analyse the trends in AATD diagnosis during the last 5 years in a Spanish AATD reference laboratory. Methods: This was a retrospective revision of all alpha-1 antitrypsin (AAT) determinations undertaken in our laboratory from 2015 to 2019. We analysed the number of AAT determinations performed and described the characteristics of the individuals tested, as well as the medical specialties and the reasons for requesting AAT determination. Results: A total of 3507 determinations were performed, of which 5.5% corresponded to children. A significant increase in the number of AAT determinations was observed from 349 in 2015 to 872 in 2019. Among the samples, 57.6% carried an intermediate AATD (50-119 mg/dL) and 2.4% severe deficiency (<50 mg/dL). The most frequent phenotype in severe AATD individuals was PI*ZZ (78.5%), and aminotransferase levels were above normal in around 43% of children and 30% of adults. Respiratory specialists requested the highest number of AAT determinations (31.5%) followed by digestive diseases and internal medicine (27.5%) and primary care physicians (19.7%). The main reason for AAT determination in severe AATD adults was chronic obstructive pulmonary disease (41.7%), but reasons for requesting AAT determination were not reported in up to 41.7% of adults and 58.3% of children. Conclusion: There is an increase in the frequency of AATD testing despite the rate of AAT determination remaining low. Awareness about AAT is probably increasing, but the reason for testing is not always clear.

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Testing Patterns and Disparities for Alpha-1 Antitrypsin Deficiency

Riley

Sriram

Brantly

et al. 2023

The American Journal of Medicine

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Registro español de pacientes con déficit de alfa-1 antitripsina: evaluación de la base de datos y análisis de la población incluida

Cited by 30 publications

References 16 publications

Alpha-1 Antitrypsin Deficiency-Associated Clinical Manifestations and Healthcare Resource Use in the United States

Alpha-1 Antitrypsin Deficiency-Associated Clinical Manifestations and Healthcare Resource Use in the United States

<p>Trends in Diagnosis of Alpha-1 Antitrypsin Deficiency Between 2015 and 2019 in a Reference Laboratory</p>

Testing Patterns and Disparities for Alpha-1 Antitrypsin Deficiency

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