The Natural History of Lung Function after Lung Transplantation for α<sub>1</sub>-Antitrypsin Deficiency

Banga, Amit; Gildea, Thomas R.; Rajeswaran, Jeevanantham; Rokadia, Haala; Blackstone, Eugene H.; Stoller, James K.

doi:10.1164/rccm.201401-0031oc

Cited by 32 publications

(37 citation statements)

References 25 publications

(27 reference statements)

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“…Further, these are the shapes that we frequently observe in a temporal trend of biomakers or biologic mechanisms, such as AF, after cardiac surgery ([25], [26], [27], [28], [29]).…”

Section: A Logistic Nonlinear Mixed Effects Modelmentioning

confidence: 99%

Probability of atrial fibrillation after ablation: Using a parametric nonlinear temporal decomposition mixed effects model

Rajeswaran

Blackstone

Ehrlinger

et al. 2016

Stat Methods Med Res

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Atrial Fibrillation is an arrhythmic disorder where the electrical signals of the heart become irregular. The probability of Atrial Fibrillation (binary response) is often time varying in a structured fashion, as is the influence of associated risk factors. A generalized nonlinear mixed effects model is presented to estimate the time related probability of Atrial Fibrillation using a temporal decomposition approach to reveal the pattern of the probability of atrial fibrillation and their determinants. This methodology generalizes to patient-specific analysis of longitudinal binary data with possibly time varying effects of covariates and with different patient-specific random effects influencing different temporal phases. The motivation and application of this model is illustrated using longitudinally measured atrial fibrillation data obtained through weekly trans-telephonic monitoring from an NIH sponsored clinical trial being conducted by the Cardiothoracic Surgery Clinical Trials Network.

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“…Further, these are the shapes that we frequently observe in a temporal trend of biomakers or biologic mechanisms, such as AF, after cardiac surgery ([25], [26], [27], [28], [29]).…”

Section: A Logistic Nonlinear Mixed Effects Modelmentioning

confidence: 99%

Probability of atrial fibrillation after ablation: Using a parametric nonlinear temporal decomposition mixed effects model

Rajeswaran

Blackstone

Ehrlinger

et al. 2016

Stat Methods Med Res

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“…3 Furthermore, in a recent study comparing outcomes among AAT-replete COPD with AATD patients after LT, a trend was evident toward worsened early outcomes among AATD patients. 31 Finally, in a study by Gao et al, 32 in vitro perfusion with AAT in a rat model of ischemia-reperfusion injury appeared to attenuate inflammatory cytokine release and cell death and was also associated with significant improvements in oxygenation and pulmonary physiology. These data not only raise the possibility of a potential therapeutic role of AAT in PGD, especially among patients with AATD, but also support the concept that PGD may exert worse consequences in AATD than under other conditions for which LT is performed.…”

Section: Early Posttransplantation Carementioning

confidence: 98%

“…Reasons for the differences between studies might include varying study designs, different patient characteristics (including comorbidities), and different post-LT clinical practices. It is noteworthy that the single-center studies showing worse (or trends toward worse) outcomes among AATD patients compared with AAT-replete COPD patients are from North America, 31,45 whereas the only single-center study showing better outcomes in AATD is from Europe. 42 It has been speculated that the higher awareness of AATD in Europe might have …”

Section: Outcomes After Lung Transplantation For A-1 Antitrypsin Defimentioning

confidence: 99%

A Review of Lung Transplantation for Patients With α-1 Antitrypsin Deficiency

Banga¹,

Rokadia

Stoller

2015

Clinical Pulmonary Medicine

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a-1 antitrypsin deficiency (AATD) is an autosomal codominant condition that predisposes one to emphysema because of the lack of proteolytic defense in the lung. Emphysema leading to respiratory failure is the most common cause of death in AATD, and AATD is currently the fourth most common indication for lung transplantation (LT) in the United States. Although the intraoperative management of LT for AATD resembles that of usual (AAT replete) chronic obstructive pulmonary disease, several special circumstances regard the selection, pretransplant and posttransplant management, and outcomes after LT in AATD. The current review begins with an overview of AATD, followed by a discussion regarding indications for LT and the impact of AATD on LT candidacy. We then review the available experience with outcomes after LT for AATD, while highlighting specific considerations for posttransplant care of these patients. (Clin Pulm Med 2015;22:239-245) a-1 antitrypsin deficiency (AATD) is a genetic disease that predisposes one to emphysema and liver cirrhosis through both "toxic loss of function" and "toxic gain of function" effects, respectively. 1,2 Because severe emphysema may result from AATD, lung transplantation (LT) is frequently considered as a treatment option for such patients. Indeed, AATD is the fourth most common indication for LT after chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, and cystic fibrosis and accounts for nearly 6% of all lung transplants performed worldwide. 3 The current paper reviews LT for AATD. Specifically, AATD is first reviewed briefly, followed by a discussion of the indications for LT for COPD in general and the impact of AATD on LT candidacy. Next, reviewed is the available experience with LT for AATD and then specific considerations for the posttransplant care of patients with AATD.

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“…However, there may be individual lung transplant patients for whom this advice may not be appropriate. A recent study by BANGA et al [22] assessed the outcome of single and double lung transplantation in patients with AAT-replete COPD (n=231) and AATD (n=45). There were no significant between-group differences in overall rates of FEV1 decline ( p>0.09).…”

Section: Other Clinical Manifestations Of Aatd and The Role Of Aat Aumentioning

confidence: 99%

“…There were no between-group differences in acute cellular rejection events or in early or late mortality. Whilst additional studies are needed to better understand the apparent differences between single and double lung transplant in AATD, it is interesting to speculate that these findings indicate that patients with AATD should be eligible for lung transplantation [22]. Indeed, in our clinic we have treated a number of lung transplant patients with augmentation therapy.…”

Section: Other Clinical Manifestations Of Aatd and The Role Of Aat Aumentioning

confidence: 99%

Long-term experience in the treatment of α₁-antitrypsin deficiency: 25 years of augmentation therapy

Teschler

2015

Eur Respir Rev

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Although it is often under-recognised, α 1 -antitrypsin deficiency (AATD) represents one of the most common genetic respiratory disorders worldwide. Since the publication of studies in the late 1980s, which demonstrated that plasma-derived augmentation therapy with intravenous α 1 -antitrypsin (AAT) can reverse the biochemical deficiencies in serum and lung fluid that characterise emphysema, augmentation therapy has become the cornerstone of patient management. This article, with a focus on experience gained in clinical practice in Germany, provides an overview of some of the research highlights and clinical experience gained in the use of augmentation therapy for AATD during the past 25 years, and briefly discusses the potential role of AAT augmentation therapy in lung transplant recipients. Additionally, the goals of AAT augmentation therapy will be discussed, namely to delay the progression of emphysema, reduce the frequency of exacerbations and improve health-related quality of life. Beyond pulmonary disease, there is recent growing evidence to indicate that AATD could also play a role in rare disorders such as panniculitis, granulomatosis with polyangiitis and ulcerative colitis. @ERSpublications Overview of long-term experience gained with AAT augmentation therapy for the treatment of AATD http://ow.ly/HmJLR

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The Natural History of Lung Function after Lung Transplantation for α₁-Antitrypsin Deficiency

Cited by 32 publications

References 25 publications

Probability of atrial fibrillation after ablation: Using a parametric nonlinear temporal decomposition mixed effects model

Probability of atrial fibrillation after ablation: Using a parametric nonlinear temporal decomposition mixed effects model

A Review of Lung Transplantation for Patients With α-1 Antitrypsin Deficiency

Long-term experience in the treatment of α₁-antitrypsin deficiency: 25 years of augmentation therapy

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The Natural History of Lung Function after Lung Transplantation for α1-Antitrypsin Deficiency

Cited by 32 publications

References 25 publications

Probability of atrial fibrillation after ablation: Using a parametric nonlinear temporal decomposition mixed effects model

Probability of atrial fibrillation after ablation: Using a parametric nonlinear temporal decomposition mixed effects model

A Review of Lung Transplantation for Patients With α-1 Antitrypsin Deficiency

Long-term experience in the treatment of α1-antitrypsin deficiency: 25 years of augmentation therapy

Contact Info

Product

Resources

About

The Natural History of Lung Function after Lung Transplantation for α₁-Antitrypsin Deficiency

Long-term experience in the treatment of α₁-antitrypsin deficiency: 25 years of augmentation therapy