Alphai-antitrypsin

Carrell RW, Lomas DA Alphai-antitrypsin deficiency—a model for conformational diseases. N Engl J Med 2002 346 45. 

Blood-group proteins Alphai -antitrypsin Calprotectin Cystein peptidases... 

Genetic mechanisms A genetic disposition is suspected in HCC, as in other malignant tumours. Some 22% of patients suffering from HCC had other organ tumours as well. (135) Several hereditary metabolic diseases, with or without cirrhosis, may increase the risk of HCC considerably. These include tyrosinaemia (type I), glycogenosis (type I), alphai-antitrypsin deficiency, galactos-aemia, porphyria cutanea tarda, acute intermittent porphyria and idiopathic haemochromatosis. Patients... 

Eriksson, S.X, Carlson, X, Velez, R. Risk of cirrhosis and primary liver cancer in alphai-antitrypsin deficiency. New Engl. X. Med. 1986 314 736-779... 

Pasteurization is the heating of aqueous, stabilized protein solutions. Pasteurization has been used for bulk preparations of Factor VIII, antithrombin III, and alphai-antitrypsin but wet heat treatment of these... 

Alphai-antitrypsin neutralizes the activity of neutrophil elastase and is used to treat patients with homozygous deficiency of Alphai-antitrypsin (1). Adverse effects occur only rarely (2). 

A 34-year-old woman developed jaundice 11 days after starting to take omeprazole for ulcer dyspepsia (19). Her hver function tests suggested acute hepatitis with large rises in transaminases. Investigations excluded virus infections, autoimmune disorders, copper overload, and alphai antitrypsin deficiency. On withdrawal of omeprazole, the transaminases gradually normalized. 

Carlson ), Eriksson S. Chronic cryptogenic Ever disease and malignant hepatoma in intermediate alphai. antitrypsin deficiency identified by a Pi Z-spe-cific monoclonal antibody. Scand J Gastroenterol 1985 20 835-42. 

Graziadei I, Joseph J, Wiesner R, Therneau T, Batts K, Porayko M. Increased risk of chronic liver failure in adults with heterozygous alphai-antitrypsin deficiency. Hepatology 1998 28 1058-63. 

S-100 protein and NSE reactivity. Both the adult and congenital types share positivity for alphai-antitrypsin. 

Adverse reactions to alphai-antitrypsin concentrate as replacement therapy are rare. In an international randomized clinical trial, 29% of the patients had adverse reactions (0.004 drug-related adverse events per infusion) of which more than 90% were classified as mild or moderate there was one case of psoriasis [25 "]. 

Augmentation therapy for alphai-antitryp-sin uses partially purified plasma, which is highly enriched with alphai-antitrypsin. Adverse reactions to this are rare (under 0.03 events per patient-month) and generally mild. They include headache, dizziness, nausea, and dyspnea [34 ]. 

A non-smoking 61-year-old man with severe emphysema due to alphai-antitrypsin deficiency was given intravenous human alphai-antitrypsin 60-120 mg/kg once a week or every second week for 10 years [35 ]. His plasma alphai-antitrypsin concentration was restored. There were no significant changes in FEVi, vital capacity, or the ratio of FEVi to vital capacity, and the emphysema progressed, but there were fewer exacerbations. There were no adverse reactions. 

Drug administration route Six healthy subjects, seven patients with alphai-antitrypsin deficiency, and seven patients with cystic fibrosis were given alphai-antitrypsin by nebulization. One healthy subject developed tongue vesicles and dysphagia and one patient with cystic fibrosis had mild headache, which was possibly related to alphai-antitrypsin [36 ]. 

Brand P, Schulte M, Wencker M, Herpich CH, Klein G, Raima K, Meyer T. Lung deposition of inhaled alphai-proteinase inhibitor in cystic fibrosis and alphai-antitrypsin deficiency. Eur Respir J 2009 34(2) 354-60. 

Matthys H. Definition and assessment of asthma. Lung 1990 168 51-56. Mossberg B, Philipson K, Camner P. Traeheobronehial elearanee in patients with emphysema assoeiated with alphai-antitrypsin defieieney. Seand J Respir Dis 1978 59 1 7. 

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