alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease.

alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease. Research Abstract Details 

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alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease. Abstract Text:

alpha1-Antitrypsin (alpha1-AT) deficiency is an autosomal recessive inherited disease. The serum concentration of the protease inhibitor (Pi) alpha1-AT is controlled by a set of codominant allelic genes, constituting the so-called Pi system. Abnormal conditions reported in connection with severe alpha1-AT deficiency of the PiZZ type have been, in the newborn, cholestasis and progressive juvenile cirrhosis, and in adults, panacinar pulmonary emphysema and liver disease. Skin changes have not been described previously in connection with this disease picture. The case is persistent cutaneous vasculitis in a 2-year-old child with alpha1-AT deficiency of the PiZZ type, heterozygosity for the Duarte variant of galactose-1-phosphate uridyl transferase, and neonatal liver disease. A pathogenetic relationship may exist between the biochemical defects and both the skin and liver diseases.

alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease. Publishing Authors By Initials

For similar skin and connective tissue diseases: connective tissue diseases: alpha 1-antitrypsin deficiency research abstracts see: skin and connective tissue diseases: connective tissue diseases: alpha 1-antitrypsin deficiency research

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alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Archives of dermatology

VOLUME: 114

Page Numbers: 921-4

Journal Abbreviation: Arch Dermatol

ISSN: 0003-987X

DAY: 17

MONTH: Jun

YEAR: 1978

alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease. Information

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LANGUAGE: eng

NlmUniqueID: 372433

alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease. Keywords Mesh Terms:

KEYWORDS: alpha 1-Antitrypsin Deficiency

MESH TERMS: genetics

Chemical & Substance for Abstract: alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver disease. Information

Substance Name: alpha 1-Antitrypsin

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Country: UNITED STATES

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MEDLINETA: Arch Dermatol

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