Pegademase bovine (PEG-ADA) for the treatment of infants and children with severe combined immunodeficiency (SCID

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Pegademase bovine (PEG-ADA) for the treatment of infants and children with severe combined immunodeficiency (SCID)

Adenosine deaminase deficiency (ADA) is a rare, inherited disorder of purine metabolism characterized by immunodeficiency, failure to thrive and metabolic abnormalities. A lack of the enzyme ADA allows accumulation of toxic metabolites causing defects of both cell mediated and humoral immunity leading to ADA severe combined immune deficiency (SCID), a condition that can be fatal in early infanc...

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Long-term efficacy of enzyme replacement therapy for adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID).

Adenosine deaminase (ADA)-deficient Severe Combined Immunodeficiency (ADA-deficient SCID) is characterized by impaired lymphocyte development and function resulting from the adenosine metabolism defect. Enzyme replacement therapy with polyethylene glycol-conjugated adenosine deaminase (PEG-ADA) minimizes infectious complications of ADA-deficient patients who have not received bone marrow transp...

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BTT_3103_pegademase bovine (PEG-ADA)

Correspondence: HB Gaspar Molecular Immunology Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH Tel +44 207 905 2319 Fax +44 207 905 2810 email [email protected] Abstract: Adenosine deaminase deficiency (ADA) is a rare, inherited disorder of purine metabolism characterized by immunodeficiency, failure to thrive and metabolic abnormalities. A lack of the enzyme ADA a...

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A case of severe combined immunodeficiency presenting with CMV pneumonia

A five month-old girl was admitted in Ali Asghar Children’s Hospital with a history of three months of fever, cough and dyspnea that her symptoms have exacerbated since two weeks before admission. She was the first child of the family born to consanguineous parents. She was clinically healthy in the past and had gained weight normally and undergone vaccination program with no complication unti...

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Dental treatment of children with severe combined immunodeficiency.

The prolonged survival of children having primary and secondary immunodeFiciencies will require many of these youngsters to seek the attention of dentists. Two children with severe combined immunodeFiciency (SCID), a disease with profound abnormalities of both cellular and humoral immunity, are presented to illustrate how dental and oral lesions cause major difficulties in clinical management. ...

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ژورنال

عنوان ژورنال: Biologics: Targets & Therapy

سال: 2009

ISSN: 1177-5475

DOI: 10.2147/btt.s3103