PEDIATRICS Vol. 47 No. 6 June 1971, pp. 1010-1018
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METACHROMASIA AND ASSAY FOR LYSOSOMAL ENZYMES IN SKIN FIBROBLASTS CULTURED FROM PATIENTS WITH CYSTIC FIBROSIS AND CONTROLS

Ilana Kraus M.D.1, Irena Antonowicz Ph.D.1, Harnish Shah M.D.1, Herbert Lazarus M.A.1, and Harry Shwachman M.D.1

1 Department of Medicine and Division of Clinical Laboratories, The Children's Hospital Medical Center, The Children's Cancer Research Foundation, and the Department of Pediatrics, Harvard Medical School

The ability of Toluidine blue 0 to stain certain cytoplasmic elements metachromatically in cultured skin fibroblasts derived from patients with cystic fibrosis (CF) has been investigated. Fifteen of 18 (83.3%) cell strains from patients with cystic fibrosis were positive, two (11.1%) borderline, and one negative (5.5%). In a control group, 8 of 18 (44.4%) were positive, three borderline (16.6%), and seven negative (38.8%). In addition to the lack of an absolute correlation between metachromasia and CF, inconsistencies in staining reaction were continually noted. Several parameters which account for these variations have been examined.

Since metachromatic granules are felt to represent lysosomal structures, a study of four lysosomal enzymes in the skin cultured fibroblasts was carried out in material from 10 patients with CF and 11 controls. No differences were noted in the activity of beta-galactosidase, beta-glucuronidase, aryl sulfatase, and acid phosphatase. While these studies do not support the possibility that CF is due to a lysosomal hydrolase deficiency in the metabolism of mucopolysaccharides, they do not exclude it either.

Submitted on October 30, 1970
Accepted on January 6, 1971




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