Association of neutrophil and complement defects in two twins with Shwachman syndrome

F. Sacchi; G. Maggiore; G. Marseglia; M. Marconi; L. Nespoli; A. G. Siccardi

Association of neutrophil and complement defects in two twins with Shwachman syndrome

F. Sacchi, G. Maggiore, G. Marseglia, M. Marconi, L. Nespoli, A. G. Siccardi

IRCCS Fondazione Policlinico San Matteo

Research output: Contribution to journal › Article › peer-review

Abstract

Immunological functions were studied in two 22-month-old dizygotic twins with the characteristic features of Shwachman syndrome. A severe defect of neutrophil motility was found in both children, but not in their parents. An impairment of the activity of the alternative pathway of complement was present in the sera of both patients. This defect, in association with the neutropenia and the chemotactic defect, might be related to the recurrent infections displayed by the twins.

Original language	English
Pages (from-to)	177-181
Number of pages	5
Journal	Helvetica Paediatrica Acta
Volume	37
Issue number	2
Publication status	Published - 1982

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

Cite this

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abstract = "Immunological functions were studied in two 22-month-old dizygotic twins with the characteristic features of Shwachman syndrome. A severe defect of neutrophil motility was found in both children, but not in their parents. An impairment of the activity of the alternative pathway of complement was present in the sera of both patients. This defect, in association with the neutropenia and the chemotactic defect, might be related to the recurrent infections displayed by the twins.",

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AU - Sacchi, F.

AU - Maggiore, G.

AU - Marseglia, G.

AU - Marconi, M.

AU - Nespoli, L.

AU - Siccardi, A. G.

PY - 1982

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N2 - Immunological functions were studied in two 22-month-old dizygotic twins with the characteristic features of Shwachman syndrome. A severe defect of neutrophil motility was found in both children, but not in their parents. An impairment of the activity of the alternative pathway of complement was present in the sera of both patients. This defect, in association with the neutropenia and the chemotactic defect, might be related to the recurrent infections displayed by the twins.

AB - Immunological functions were studied in two 22-month-old dizygotic twins with the characteristic features of Shwachman syndrome. A severe defect of neutrophil motility was found in both children, but not in their parents. An impairment of the activity of the alternative pathway of complement was present in the sera of both patients. This defect, in association with the neutropenia and the chemotactic defect, might be related to the recurrent infections displayed by the twins.

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ER -

Association of neutrophil and complement defects in two twins with Shwachman syndrome

Abstract

ASJC Scopus subject areas

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