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  • Title: Alpha1-antitrypsin deficiency with severe panniculitis. Report of two cases.
    Author: Rubinstein HM, Jaffer AM, Kudrna JC, Lertratanakul Y, Chandrasekhar AJ, Slater D, Schmid FR.
    Journal: Ann Intern Med; 1977 Jun; 86(6):742-4. PubMed ID: 301370.
    Abstract:
    Two patients with profound decrease of alpha1-antitrypsin (PiZZ) presented with severe pannicultis (Weber-Christian disease); one had systemic panniculitis including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and alpha1-antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease.
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