Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.31 (beta-glucuronidase)
 7,680 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two newborn infants with hyperleukocytosis and blast cells in the peripheral blood are compared. Case 1 represents a connatal leukemia, which could be characterized as monocytic by the naphthol-ASesterase reaction, which was inhibited by NaF. The child died after a few hours. Case 2 must be diagnosed as "pseudoleukemia" in mongolism. The blast cells were classified as lymphoblastic on the basis of a strong granular beta-glucuronidase reaction. The patient achieved spontaneous remission.
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PMID:[Connatal leukaemia and pseudoleukaemia--two cases with cytochemical findings (author's transl)]. 57 34

A neonate with Down's syndrome confirmed by chromosomal analysis had leukocytosis, with a WBC count of 48,000/cu mm, at age 13 days. Differential counts showed 70% to 90% blast forms by light microscopy. The blast-cell granules stained negatively with Sudan black B and alpha-napthyl butyrate esterase. Myeloperoxidase activity evaluated light and electron microscopy was negative. The granules stained positively with PAS, beta-glucuronidase, and tartrate-sensitive acid phosphatase. Indirect immunofluorescence for terminal-deoxynucleotidyl-transferase was negative. Our results show that the blast cells of pseudoleukemia may be of lymphoid origin.
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PMID:Pseudoleukemia. Ineffective regulation of granulopoiesis. 645 Dec 9