RECENT PULMONARY OBSERVATIONS IN α1-ANTITRYPSIN DEFICIENCY, PRIMARY BILIARY CIRRHOSIS, CHRONIC HEPATITIS C, AND OTHER HEPATIC PROBLEMS

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α1-ANTITRYPSIN DEFICIENCY

The biochemical, genetic, and molecular biology aspects of the spectrum of α1-AT deficiency were reviewed recently.16, 19 The purpose of this section is to address the recent clinical developments in classical, severe α1-AT deficiency (phenotype, PiZZ), as they relate to hepatic and pulmonary dysfunction. Specifically, I review (1) key aspects of PiZZ pathophysiology, (2) the natural clinical history, (3) medical and organ-transplant treatment expectations, and (4) the National Institutes of

Primary Biliary Cirrhosis

PBC is a chronic, cholestatic, granulomatous, and destructive inflammatory disorder of the hepatic bile ducts probably caused by an autoimmune process.48 The pulmonary manifestations of PBC have been well documented and are summarized in the following list7, 41, 70, 81

  • Interstitial lung disease

    • Lymphocytic interstitial pneumonitis

    • Fibrosing alveolitis

    • Intrapulmonary granulomas

    • Organizing pneumonitis (BOOP)

  • Obstructive airways disease

  • Chest wall deformities

  • Pulmonary hypertension

Pleural Effusions

Pleural effusions (hepatic hydrothorax) complicating liver dysfunction usually are transudates, unless the fluid becomes infected. Approximately 10% of patients with significant liver disease develop pleural effusions and they may occur with or without significant ascites.42 Because of the substantial negative inspiratory pressure of the thorax, it is not uncommon to document unilateral or bilateral effusions in the absence of clinically obvious ascites. The mechanism of fluid transfer from the

SUMMARY

Patients with metabolic, immunologic, viral, and other types of hepatic disorders can have a spectrum of complicating pulmonary abnormalities. The natural history of these associations is poorly understood. Significant reversibility in hepatic and pulmonary dysfunction, however, has been well documented in the era of organ transplantation. The continued relationship among pulmonologists, hepatologists, and transplant surgeons hopefully will provide enlightening data on these interesting

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    Address reprint requests to Michael J. Krowka, MD, Division of Thoracic Diseases, Mayo Clinic Jacksonville, 4300 San Pablo Road, Jacksonville, FL 32224

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    From the Mayo Medical School, and Division of Thoracic Diseases and Internal Medicine, Mayo Clinic Jacksonville, Jacksonville, Florida

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