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Allergy and Airway |

Demographics and Clinical Profile of COPD Patients With Alpha-1 Antitrypsin Deficiency PiMZ Genotype Started on Augmentation Therapy

Navid Zaidi; Debra Greek, RN; Crystal Piepenbrink, RT
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Mercy Clinic, Carthage, MO


Chest. 2014;146(4_MeetingAbstracts):42A. doi:10.1378/chest.1987927
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Abstract

SESSION TITLE: COPD Treatment Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM

PURPOSE: Administration of Alpha-1 Antitrypsin (AAT) protein to patients with severe AAT deficiency may prevent the progression of emphysema, improve lung function and reduce mortality. Although there has been no data to support the use of augmentation therapy in patients with the PiMZ genotype (carriers), such patients are occasionally treated with augmentation therapy. The purpose of this study was to document the demographics and clinical profile of AAT-deficient COPD patients with PiMZ genotype started on augmentation therapy.

METHODS: We performed a retrospective review of all COPD patients with PiMZ genotype who were started on augmentation therapy from 2004 to 2013, as registered in the data-base of Coram, a US national specialty infusion company. All patients were diagnosed and treated by their own clinicians within United States.

RESULTS: At the initiation of augmentation therapy, there were 101 subjects: 58 men, 43 women, mean age 57, age range 18-84. All were PiMZ heterozygotes with a mean serum AAT level of 82 mg/dL (range 42-159 mg/dL). 70% were former smokers, 25% nonsmokers and 5% current smokers. Their mean FEV1 was 53% predicted (range 19-100% predicted). Their mean weight was 80 kg (range 39-139 kg). 81% were on oxygen supplementation. All received augmentation therapy with weekly IV infusions except 6 who had biweekly infusions. All were treated at home except 12 who were treated at the outpatient infusion centers.

CONCLUSIONS: This group of PiMZ heterozygotes started on augmentation therapy tended to be older, former smokers with moderately severe COPD, had a below normal serum AAT level and needed oxygen supplementation.

CLINICAL IMPLICATIONS: There is a need for prospective trials with long follow up periods, assessing meaningful clinical end points examining the role of augmentation therapy in AAT-deficient COPD patients with PiMZ genotype.

DISCLOSURE: Navid Zaidi: Grant monies (from industry related sources): Reseach grant: CSL Behring, Consultant fee, speaker bureau, advisory committee, etc.: CSL Behring Debra Greek: Employee: Coram Employee The following authors have nothing to disclose: Crystal Piepenbrink

No Product/Research Disclosure Information


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