Infectious and Bleeding Complications in Patients With Glycogenosis lb

Daniel R. Ambruso, Edward R B Mccabe, Donald Anderson, Arthur Beaudet, Donald H. Mahoney, Lawrence M. Ballas, Ira K. Brandt, Barbara Brown, Rosalin Coleman, Thomas R. Kinney, Henry S. Friedman, John M. Falletta, Morey W. Haymond, Patricia Simmons, James P. Keating, J. V. Leonard, D. B. Dunger, Reuben Matalon, Thomas F. Roe, Alfred E. Slonim

Research output: Contribution to journalArticle

74 Citations (Scopus)

Abstract

Clinical, hematologic, and immunologic findings were reviewed in 21 patients with glycogenosis lb. 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 myelold 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 la and lb 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 lb disease and may be related to abnormal glucose-6-phosphate transport.

Original languageEnglish (US)
Pages (from-to)691-697
Number of pages7
JournalAmerican Journal of Diseases of Children
Volume139
Issue number7
DOIs
StatePublished - 1985
Externally publishedYes

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Glycogen Storage Disease
Hemorrhage
Neutrophils
Neutropenia
Glycogen Storage Disease Type I
Nitroblue Tetrazolium
Glucose-6-Phosphate
Epistaxis
Bleeding Time
Patient Compliance
Bacterial Infections
Blood Platelets
Eating
Bone Marrow

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Ambruso, D. R., Mccabe, E. R. B., Anderson, D., Beaudet, A., Mahoney, D. H., Ballas, L. M., ... Slonim, A. E. (1985). Infectious and Bleeding Complications in Patients With Glycogenosis lb. American Journal of Diseases of Children, 139(7), 691-697. https://doi.org/10.1001/archpedi.1985.02140090053027

Infectious and Bleeding Complications in Patients With Glycogenosis lb. / Ambruso, Daniel R.; Mccabe, Edward R B; Anderson, Donald; Beaudet, Arthur; Mahoney, Donald H.; Ballas, Lawrence M.; Brandt, Ira K.; Brown, Barbara; Coleman, Rosalin; Kinney, Thomas R.; Friedman, Henry S.; Falletta, John M.; Haymond, Morey W.; Simmons, Patricia; Keating, James P.; Leonard, J. V.; Dunger, D. B.; Matalon, Reuben; Roe, Thomas F.; Slonim, Alfred E.

In: American Journal of Diseases of Children, Vol. 139, No. 7, 1985, p. 691-697.

Research output: Contribution to journalArticle

Ambruso, DR, Mccabe, ERB, Anderson, D, Beaudet, A, Mahoney, DH, Ballas, LM, Brandt, IK, Brown, B, Coleman, R, Kinney, TR, Friedman, HS, Falletta, JM, Haymond, MW, Simmons, P, Keating, JP, Leonard, JV, Dunger, DB, Matalon, R, Roe, TF & Slonim, AE 1985, 'Infectious and Bleeding Complications in Patients With Glycogenosis lb', American Journal of Diseases of Children, vol. 139, no. 7, pp. 691-697. https://doi.org/10.1001/archpedi.1985.02140090053027
Ambruso, Daniel R. ; Mccabe, Edward R B ; Anderson, Donald ; Beaudet, Arthur ; Mahoney, Donald H. ; Ballas, Lawrence M. ; Brandt, Ira K. ; Brown, Barbara ; Coleman, Rosalin ; Kinney, Thomas R. ; Friedman, Henry S. ; Falletta, John M. ; Haymond, Morey W. ; Simmons, Patricia ; Keating, James P. ; Leonard, J. V. ; Dunger, D. B. ; Matalon, Reuben ; Roe, Thomas F. ; Slonim, Alfred E. / Infectious and Bleeding Complications in Patients With Glycogenosis lb. In: American Journal of Diseases of Children. 1985 ; Vol. 139, No. 7. pp. 691-697.
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