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Infectious and bleeding complications in patients with glycogenosis Ib.

Publication ,  Journal Article
Ambruso, DR; McCabe, ER; Anderson, D; Beaudet, A; Ballas, LM; Brandt, IK; Brown, B; Coleman, R; Dunger, DB; Falletta, JM
Published in: Am J Dis Child
July 1985

Clinical, hematologic, and immunologic findings were reviewed in 21 patients with glycogenosis Ib. Fifteen of the patients suffered from moderate to severe bacterial infections. Ten patients had excessive epistaxis or bleeding from surgical sites, and eight suffered oral and anal mucosal ulceration. Sixteen of 21 patients exhibited chronic neutropenia associated with abnormalities in myeloid maturation and decreases in the bone marrow storage and peripheral marginating pools. Diminished neutrophil motility was documented in 14 of 15 patients tested, and adherence was decreased in three patients studied. Neutrophil microbicidal activity, reduction of nitroblue tetrazolium, and ingestion were normal in all patients tested. Bleeding times were prolonged in five of eight patients, and results of platelet function studies were abnormal in five individuals. Excessive bleeding in patients with glycogenoses Ia and Ib are similar and may be secondary to the functional deficiency of glucose-6-phosphatase. However, neutropenia, neutrophil dysfunction, and the resulting infectious complications are specific for Ib disease and may be related to abnormal glucose-6-phosphate transport.

Duke Scholars

Published In

Am J Dis Child

DOI

ISSN

0002-922X

Publication Date

July 1985

Volume

139

Issue

7

Start / End Page

691 / 697

Location

United States

Related Subject Headings

  • Platelet Count
  • Platelet Aggregation
  • Platelet Adhesiveness
  • Neutropenia
  • Male
  • Infections
  • Infant, Newborn
  • Infant
  • Humans
  • Glycogen Storage Disease Type I
 

Citation

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MLA
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Ambruso, D. R., McCabe, E. R., Anderson, D., Beaudet, A., Ballas, L. M., Brandt, I. K., … Falletta, J. M. (1985). Infectious and bleeding complications in patients with glycogenosis Ib. Am J Dis Child, 139(7), 691–697. https://doi.org/10.1001/archpedi.1985.02140090053027
Ambruso, D. R., E. R. McCabe, D. Anderson, A. Beaudet, L. M. Ballas, I. K. Brandt, B. Brown, R. Coleman, D. B. Dunger, and J. M. Falletta. “Infectious and bleeding complications in patients with glycogenosis Ib.Am J Dis Child 139, no. 7 (July 1985): 691–97. https://doi.org/10.1001/archpedi.1985.02140090053027.
Ambruso DR, McCabe ER, Anderson D, Beaudet A, Ballas LM, Brandt IK, et al. Infectious and bleeding complications in patients with glycogenosis Ib. Am J Dis Child. 1985 Jul;139(7):691–7.
Ambruso, D. R., et al. “Infectious and bleeding complications in patients with glycogenosis Ib.Am J Dis Child, vol. 139, no. 7, July 1985, pp. 691–97. Pubmed, doi:10.1001/archpedi.1985.02140090053027.
Ambruso DR, McCabe ER, Anderson D, Beaudet A, Ballas LM, Brandt IK, Brown B, Coleman R, Dunger DB, Falletta JM. Infectious and bleeding complications in patients with glycogenosis Ib. Am J Dis Child. 1985 Jul;139(7):691–697.

Published In

Am J Dis Child

DOI

ISSN

0002-922X

Publication Date

July 1985

Volume

139

Issue

7

Start / End Page

691 / 697

Location

United States

Related Subject Headings

  • Platelet Count
  • Platelet Aggregation
  • Platelet Adhesiveness
  • Neutropenia
  • Male
  • Infections
  • Infant, Newborn
  • Infant
  • Humans
  • Glycogen Storage Disease Type I