Serum Elastase Inhibitor Levels in the Ehlers-Danlos Syndrome

Pk, Carter; Rl, Walford

doi:10.1136/ard.22.3.198

Cited by 8 publications

(2 citation statements)

References 5 publications

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“…In Ehlers-Danlos syndrotne, collagen and elastic fibers observed with electron microscope appear qualitatively normal (Hult et al 1964, Wechsler & Fisher 1964, Moreover, in opposition to our findings, serum elastase inhibitor levels were found to be normal (Carter & Walford 1963, Beighton et al 1969 or r'aised (Hall et al 1955).…”

Section: Discussioncontrasting

confidence: 87%

α₁‐Antitrypsin Deficiency and Skin Abnormalities

Ledoux-Corbusier¹,

Achten²

1975

J Cutan Pathol

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A 19-year-old Moroccan male was found to have total absence of serum alpha1-antitrypsin, a major inhibitor of elastase. This patient had chronic obstructive lung disease, hyperextensibility of the skin over the cheeks and wrists, and hyperlaxity of the hand joints. Microscopic sections of the skin revealed a thickened dermis with shortened and rarefied elastic fibers. Ultrastructural study showed collagen fibers with variable and irregular diameters. Elastic fibers were scarce and their relatively poor matrix was surrounded by numberous microfibrils. The outline of the fibers was irregular with deep recesses filled with microfibrils. The ergastoplasm of the fibroblasts was well developed. The differential diagnosis with other connective dystrophies showed the original characteristic of this case. Clinically and histopathologically, the skin abnormalities are probably related to the deficiency in elastase inhibitor.

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Section: Discussioncontrasting

confidence: 87%

α₁‐Antitrypsin Deficiency and Skin Abnormalities

Ledoux-Corbusier¹,

Achten²

1975

J Cutan Pathol

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“…Serum inhibitor of elastase was found in normal human subjects by Balo and Banga (1949). Its level is said to be increased in pregnancy (Walford and Schneider, 1959;Schneider, Walford, and Dignam, 1960;Gilfillan, Sbarra, and Bardwil, 1961) and in cases of the Ehler-Danlos syndrome (Hall, Keech, Reed, Saxl, Tunbridge, and Wood, 1955), but the latter observation was not confirmed by Carter and Walford (1963). Contradictory reports on inhibition of elastolysis by serum of patients suffering from atherosclerosis have also been published (Balo and Banga, 1953;Walford and Schneider, 1959;Balo, 1960;Banga, 1963;Gilfillan and others, 1961).…”

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confidence: 95%