Alpha-1 Antitrypsin Deficiency

Updated: May 29, 2023

Background

Epidemiology

Anatomy

Pathophysiology

Etiology

Genetics

Prognostic Factors

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

alpha 1 proteinase inhibitor

Indicated for Alpha-1 Antitrypsin Deficiency
60 mg/kg intravenous infusion every week
It is indicated for the chronic augmentation and also maintenance treatment in adults with the emphysema
Intravenous infusion rate
Glassia: 0.04ml/kg/min
Prolastin C, Aralast NP, Zemaira: It should not exceed 0.08 ml/kg/min
Type-1 Diabetes Mellitus
Prolastin C: For treatment of DM type-1 with the residual beta cell function as an orphan designation
Glassia (IV): For treatment of DM type-1 (<15 years) with the residual beta cell function
Deficiency of Alpha-1 Proteinase Inhibitor
It is indicated as an inhalation treatment for congenital deficiency of the alpha-1 proteinase inhibitor
Bronchopulmonary Dysplasia
It is generally indicated for the prevention of the bronchopulmonary Dysplasia
Graft vs Host Disease
Glassia: It is used as a treatment for Graft vs Host Disease