II. Indications
III. Contraindications
- IgA deficiency AND formed Antibody to IgA
IV. Mechanism
- Purified pooled plasma product, replacing deficient Alpha-1 Antitrypsin (Alpha-1 Proteinase Inhibitor)
V. Dosing
- Give 60 mg/kg IV weekly
VI. Adverse Effects
- Fever
- Pooled plasma product
- Risk of bood borne pathogens including Creutzfeld-Jakob Disease
- Complete Immunizations (including Hepatitis B Vaccine) before use
VII. Safety
- Pregnancy Category C
- Unknown Safety in Lactation
VIII. Resources
- Zemaira Alpha-1 Proteinase Inhibitor Plasma (DailyMed)
- Prolastin-C Alpha-1 Proteinase Inhibitor Plasma (DailyMed)
- Aralast NP Alpha-1 Proteinase Inhibitor Plasma (DailyMed)
IX. References
- Olson (2020) Clinical Pharmacology, Medmaster Miami, p. 90
- Hamilton (2020) Tarascon Pocket Pharmacopoeia
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Related Studies
| Definition (MSH) | Deficiency of the protease inhibitor ALPHA 1-ANTITRYPSIN that manifests primarily as PULMONARY EMPHYSEMA and LIVER CIRRHOSIS. |
| Definition (MEDLINEPLUS) |
Alpha-1 antitrypsin deficiency (AAT deficiency) is an inherited condition that raises your risk for lung and liver disease. Alpha-1 antitrypsin (AAT) is a protein that protects the lungs. The liver makes it. If the AAT proteins aren't the right shape, they get stuck in the liver cells and can't reach the lungs. Symptoms of AAT deficiency include
Some people have no symptoms and do not develop complications. Blood tests and genetic tests can tell if you have it. If your lungs are affected, you may also have lung tests. Treatments include medicines, pulmonary rehab, and extra oxygen, if needed. Severe cases may need a lung transplant. Not smoking can prevent or delay lung symptoms. NIH: National Heart, Lung, and Blood Institute |
| Definition (NCI) | A genetic disorder characterized by decreased alpha-1 antitrypsin activity in the lungs and blood and deposition of alpha-1 antitrypsin protein in the hepatocytes. These abnormalities result from defective production of alpha-1 antitrypsin and lead to the development of emphysema, cirrhosis, and liver failure. |
| Definition (CSP) | autosomal recessive trait leading to destruction of lung tissue by neutrophil elastase and eventual emphysema; second only to cystic fibrosis as most common lethal genetic disorder among Caucasians of northern European ancestry. |
| Concepts | Disease or Syndrome (T047) |
| MSH | D019896 |
| ICD9 | 273.4 |
| ICD10 | E88.01 |
| SnomedCT | 30188007, 154771007 |
| English | Deficiencies, alpha 1-Antitrypsin, Deficiency, alpha 1-Antitrypsin, alpha 1 Antitrypsin Deficiency, alpha 1 antitrypsin deficiency, alpha 1-Antitrypsin Deficiencies, ALPHA ANTITRYPSIN DEFICIENCY 01, ALPHA 1 ANTITRYPSIN DEFIC, alpha 1-antitrypsin deficiency, alpha 1-antitrypsin deficiency (diagnosis), Alpha-1-antitrypsin deficiency (disorder), alpha-1-Antitrypsin deficiency (disorder), alpha-1-Antitrypsin deficiency, Alpha-1-antitrypsin deficiency, Alpha-1 anti-trypsin deficiency, Alpha-1 proteinase inhibitor deficiency, Alpha-1-antitrypsin def, A-1ATD, A1AD, AAT deficiency, alpha 1-Antitrypsin Deficiency [Disease/Finding], alpha-1 antitrypsin deficiency, alpha-1-antitrypsin deficiency, 1 alpha antitrypsin deficiency, alpha-1 anti-trypsin deficiency, ALPHA-1-ANTITRYPSIN DEFICIENCY, AATD, Alpha-1 Antitrypsin Deficiency, alpha 1-Antitrypsin Deficiency, Alpha 1-antitrypsin deficiency, Alpha-1-Antitrypsin Deficiency, alpha-1-Proteinase inhibitor deficiency, deficiency; alpha-1-antitrypsin, alpha-1-antitrypsin; deficiency, Deficiency;A1 antitrypsin |
| Italian | Deficit di alfa-1 antitripsina, Deficit dell'inibitore della alfa-1 proteinasi, Deficit di alfa-1-antitripsina |
| Dutch | alfa-1-proteïnase-inhibitordeficiëntie, alfa-1-antitrypsine; deficiëntie, deficiëntie; alfa-1-antitrypsine, alfa-1-antitrypsinedeficiëntie, Alfa-1-antitrypsinedeficiëntie, Antitrypsinedeficiëntie, alfa-1-, Deficiëntie, alfa-1-antitrypsine- |
| French | Carence en inhibiteurs de la protéinase alpha-1, Déficit en alpha 1-antitrypsine, Déficit en alpha-1-antitrypsine, Déficit en alpha-1 antitrypsine |
| German | Alpha-1 Proteinase-Inhibitormangel, ALPHA ANTITRYPSINMANGEL 01, Alpha-1 Antitrypsinmangel, Alpha-1-Antitrypsinmangel |
| Portuguese | Carência de inibidor de alfa-1-proteinase, Deficiência de 1-Antitripsina alfa, Deficiência de alfa-1-antitripsina, Deficiência de alfa 1-Antitripsina |
| Spanish | Déficit del inhibidor de la alfa-1 proteínasa, deficiencia de alfa-1-antitripsina, deficiencia de inhibidor de alfa - 1 - proteinasa, deficiencia de alfa - 1 - antitripsina (trastorno), deficiencia de alfa-1-antitripsina (trastorno), Deficiencia de alfa 1-Antitripsina, deficiencia de alfa - 1 - antitripsina, Déficit de alfa-1 anti-tripsina |
| Japanese | α１蛋白分解酵素阻害欠損症, α１抗トリプシン欠損症, ｱﾙﾌｧ1ﾀﾝﾊﾟｸﾌﾞﾝｶｲｺｳｿｿｶﾞｲｹｯｿﾝｼｮｳ, ｱﾙﾌｧ1ｺｳﾄﾘﾌﾟｼﾝｹｯｿﾝｼｮｳ, アルファ1-アンチトリプシン欠損症, α1-アンチトリプシン欠損症 |
| Swedish | Alfa 1-antitrypsinbrist |
| Finnish | Alfa-1-antitrypsiinin puutos |
| Russian | AL'FA 1-ANTITRIPSINOVAIA NEDOSTATOCHNOST', АЛЬФА 1-АНТИТРИПСИНОВАЯ НЕДОСТАТОЧНОСТЬ |
| Czech | Deficit alfa-1-antitrypsinu, Deficit inhibitoru alfa-1-proteinázy, alfa-1-antitrypsin - nedostatečnost, deficit alfa-1 antitrypsinu, deficit alfa-1- antitrypsinu, deficit α1-antitrypsinu, deficit alfa1-antitrypsinu |
| Polish | Niedobór alfa-1-antytrypsyny |
| Hungarian | Alpha-1 antitrypsin hiány, Alpha-1 proteinase inhibitor hiány |
| Norwegian | Alfa-1-antitrypsinmangel |
