Alpha-1 Antitrypsin Deficiency
Also known as: AATD, Alpha-1, Inherited emphysema
Methodology & AI Transparency ⓘ
Alpha-1 Antitrypsin Deficiency Key Takeaways
Translated by Sarah Jenkins (AI RN Plain-English Translator) at a 7th-grade reading level
- 1 What it does: What is alpha-1 antitrypsin deficiency (AAT deficiency)? Alpha-1 antitrypsin deficiency (AAT deficiency, or AATD) is an inherited condition that raises your risk for lung and liver disease.
- 2 Real-world patient consensus: Diagnostic registry J43.3: Alpha-1 Antitrypsin Deficiency is managed within Lungs & Asthma (aka AATD).
- 3 Typical cash cost range: Workup highlights for Alpha-1 Antitrypsin Deficiency (J43.3): high-resolution chest imaging in Alpha-1 Antitrypsin Deficiency (J43.3: when indicated).
- 4 Top reported side effects / signs: Follow-up benchmark in Alpha-1 Antitrypsin Deficiency: maintaining Fractional Exhaled Nitric in Alpha-1 Antitrypsin Deficiency (J43.3: Oxide (FeNO)) within < 25 ppb (J43.3).
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What Is Alpha-1 Antitrypsin Deficiency?
What is alpha-1 antitrypsin deficiency (AAT deficiency)? Alpha-1 antitrypsin deficiency (AAT deficiency, or AATD) is an inherited condition that raises your risk for lung and liver disease. If you have this condition, your body doesn't make enough alpha-1 antitrypsin (AAT). AAT is made by your liver. It helps protect your lungs from inflammation and irritating substances you might breathe in, such as smoke. If your liver doesn't make enough AAT, your lungs may be more easily damaged from smoking , pollution , or dust from the environment. This can lead to a serious lung condition called chronic obstructive pulmonary disease ( COPD ).
Pathophysiology, Underlying Causes & Clinical Presentation
Underlying Alpha-1 Antitrypsin Deficiency (code J43.3) is small-airway remodeling [Alpha-1 Antitrypsin Deficiency J43.3], Cross-referenced with NIH MeSH (alpha 1-Antitrypsin Deficiency), which Asthma, COPD & Respiratory Disease clinicians address while surveilling Fractional Exhaled Nitric in Alpha-1 Antitrypsin Deficiency (J43.3: Oxide (FeNO)) (< 25 ppb (J43.3)).
Common Symptoms & Clinical Signs
- • Decrease the amount of AAT protein your liver makes
- • Prevent your liver from making any AAT
- • Presentation of Alpha-1 Antitrypsin Deficiency (J43.3): chronic productive or in Alpha-1 Antitrypsin Deficiency (J43.3: dry cough worsening)
- • Secondary Alpha-1 Antitrypsin Deficiency clinical sign: prolonged recovery from in Alpha-1 Antitrypsin Deficiency (J43.3: viral respiratory infections)
Primary Federal Reference: NIH / National Library of Medicine MedlinePlus Entry ↗
Alpha-1 Antitrypsin Deficiency Diagnostic Classification & Lab Markers
| Metric | Verified Benchmark | Source |
|---|---|---|
| ICD-10-CM Diagnostic Code | J43.3 | WHO / CMS ICD-10-CM Clinical Classification |
| Clinical Specialty Hub | Asthma, COPD & Respiratory Disease | NIH MedlinePlus Health Topics |
| Diagnostic / Lab Marker: Fractional Exhaled Nitric in Alpha-1 Antitrypsin Deficiency (J43.3: Oxide (FeNO)) | < 25 ppb (J43.3) | Clinical Reference Target Range |
| Diagnostic / Lab Marker: Resting Pulse Oximetry in Alpha-1 Antitrypsin Deficiency (J43.3: (SpO2)) | 96% – 100% (J43.3) | Clinical Reference Target Range |
| Diagnostic / Lab Marker: Blood Eosinophil Count [Alpha-1 Antitrypsin Deficiency J43.3] | < 150 cells/uL (phenotyping marker) (J43.3) | Clinical Reference Target Range |
FDA-Monographed Medications & Public Cash Price Reference for Alpha-1 Antitrypsin Deficiency
| Medication | Pharmacologic Class | Patient Worth-It | CMS NADAC / Unit | Typical Lowest Cash |
|---|---|---|---|---|
| Brimonidine Tartrate/Timolol Maleate Ophthalmic Solution (Brimonidine Tartrate and Timolol Maleate) Brimonidine Tartrate/Timolol Maleate Ophthalmic Solution • RxCUI 861635-B65145162 | alpha-Adrenergic Agonist | 74% | $2.7175 | $ 204.16 /mo |
| Tizanidine Zanaflex • RxCUI 313413 | Central alpha-2 Adrenergic Agonist | 80% | $0.0818 | $ 5.37 /mo |
| Dutasteride and Tamsulosin Hydrochloride Jalyn • RxCUI 996097 | 5-alpha Reductase Inhibitor | 85% | $1.8643 | $ 84.00 /mo |
| Minoxidil Hair Generation Serum (5% Minoxidil with Biotin) Minoxidil Hair Generation Serum • RxCUI RXN-B85593020 | 5-alpha Reductase Inhibitor | 89% | $2.6700 | $ 200.74 /mo |
| Oraverse (Phentolamine Mesylate) Oraverse • RxCUI RXN-B03620101 | alpha-Adrenergic Blocker | 86% | $0.1900 | $ 22.18 /mo |
| Proscar (Finasteride) Proscar • RxCUI 200172-B78206153 | 5-alpha Reductase Inhibitor | 92% | $5.7018 | $ 419.03 /mo |
| Sudogest Nasal Decongestant (Pseudoephedrine HCl) Sudogest Nasal Decongestant • RxCUI 1049160 | alpha-Adrenergic Agonist | 94% | $0.0598 | $ 12.80 /mo |
| Pseudoephedrine Hydrochloride (Pseudoephedrine HCl) Pseudoephedrine Hydrochloride • RxCUI 1049160-B45802432 | alpha-Adrenergic Agonist | 77% | $0.0598 | $ 12.80 /mo |
| Lidocaine HCl and Epinephrine Lidocaine HCl and Epinephrine Generic • RxCUI RXN-516621504-3096 | alpha-Adrenergic Agonist | 89% | $0.0989 | $ 12.50 /mo |
| Sacrosidase Sucraid • RxCUI 213337 | Sucrose-specific Enzyme | 85% | $0.6800 | $ 36.04 /mo |
| Biorphen (Phenylephrine Hydrochloride) Biorphen • RxCUI RXN-B43598172 | alpha-1 Adrenergic Agonist | 75% | $8.9977 | $ 640.00 /mo |
| Iron Sucrose Venofer • RxCUI 1741261 | Parenteral Iron Replacement | 83% | $12.2067 | $ 185.00 /mo |
| Epinephrine Adrenalin, Neffy • RxCUI 1660014 | alpha-Adrenergic Agonist | 86% | $127.8321 | $ 185.00 /mo |
| Ferric Maltol Accrufer • RxCUI 2539943 | Parenteral Iron Replacement | 91% | $9.8880 | $ 185.00 /mo |
| Brimonidine Tartrate/Timolol Maleate (Brimonidine Tartrate and Timolol Maleate) Brimonidine Tartrate/Timolol Maleate • RxCUI RXN-B68462281 | alpha-Adrenergic Agonist | 94% | $1.9993 | $ 152.45 /mo |
| Zunveyl (Benzgalantamine) Zunveyl • RxCUI RXN-B84054010 | FDA Prescription Therapeutic Agent | 84% | $0.7800 | $ 64.66 /mo |
Peer-Reviewed Human Clinical Trials & Meta-Analyses (Alpha-1 Antitrypsin Deficiency)
| Study & Plain-English Finding | Sample (N) | Rigor | Primary Source |
|---|---|---|---|
| What the Latest Evidence Really Says About Comparative Efficacy of Minoxidil and 5-Alpha Reductase Inhibitors single-drug We found dutasteride 0.5 mg/day to be the most effective option. Journal of cosmetic dermatology • 2025-07-15 • Meta-Analysis | Multi-cohort | 92/100 | PMID 40586152 ↗ |
| What the Latest Evidence Really Says About Skin-applied azelaic acid, salicylic acid, nicotinamide, sulphur, zinc and fruit Risk of bias and imprecision limit our confidence in the evidence. The Cochrane database of systematic reviews • 2020-05-01 • Meta-Analysis | Multi-cohort | 92/100 | PMID 32356369 ↗ |
| Can This Approach Help? Inside the Latest Research on Clinical dose ranging studies with finasteride, a type 2 5alpha-reductase Finasteride 1 mg/day is the optimal dose for the treatment of men with male pattern hair loss and was subsequently identified for further clinical development. Journal of the American Academy of Dermatology • 1999-10-15 • Randomized Controlled Trial | Multi-cohort | 88/100 | PMID 10495375 ↗ |
| Can This Approach Help? Inside the Latest Research on Importance of dual 5alpha-reductase inhibition in the treatment of male pattern Dutasteride increases scalp hair growth in men with MPHL. Journal of the American Academy of Dermatology • 2006-12-15 • Randomized Controlled Trial | Multi-cohort | 88/100 | PMID 17110217 ↗ |
| Can This Approach Help? Inside the Latest Research on Safety and real-world benefits of the combination of once-daily tadalafil and Concurrent treatment with tadalafil and an α 1 -blocker seems to be safe and well tolerated in Japanese patients with lower urinary tract symptoms suggestive of benign prostatic hyperplasia. International journal of urology : official journal of the Japanese Urological Association • 2017-07-15 • Randomized Controlled Trial | Multi-cohort | 88/100 | PMID 28556284 ↗ |
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