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• Journal

  Palliative Care

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  Sage

  v. 1-12, 2008-19.

  PubMed Central

  v. 7-12, 2013-19.
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• Article

  Schwerer alpha1-Antitrypsin-Mangel. Klinische Beobachtung bei 21 Patienten. [Severe alpha 1-antitrypsin deficiency: clinical observations of 21 patients].

  Dürst A, Grob PJ.

  Schweiz Med Wochenschr. 1978 Dec 23;108(51):2040-6.

  The disease course is described in 21 patients with low serum concentrations of alpha1-antitrypsin of the phenotype Z (genotype pi ZZ). 13 of these patients have long-standing disease characterized by bronchitis or dyspnea beginning before the age of 40 and progressing to emphysema (11 patients) and to corpulmonale (7 patients). The remaining 8 patients are children with hepatopathy characterized by prolonged jaundice at birth, persistent hepatomegaly and persistently elevated liver enzymes. In 2 children, the evolution to cirrhosis was ascertained by biopsy.

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