Adenosine deaminase deficiency associated severe combined immunodeficiency with disseminated varicella infection after vaccination : a case report
OBJECTIVE: To enhance the knowledge of adenosine deaminase (ADA) deficiency associated severe combined immunodeficiency (SCID) with disseminated varicella infection after vaccination
| Veröffentlicht in: | Zhonghua er ke za zhi = Chinese journal of pediatrics. - 1960. - 46(2008), 8 vom: 22. Aug., Seite 597-600 |
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| Format: | Aufsatz |
| Sprache: | Chinese |
| Veröffentlicht: |
2008
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| Zugriff auf das übergeordnete Werk: | Zhonghua er ke za zhi = Chinese journal of pediatrics |
| Schlagworte: | Case Reports English Abstract Journal Article Adenosine Deaminase EC 3.5.4.4 |
| Zusammenfassung: | OBJECTIVE: To enhance the knowledge of adenosine deaminase (ADA) deficiency associated severe combined immunodeficiency (SCID) with disseminated varicella infection after vaccination METHODS: With case report and review of literature, the background knowledge, clinical and laboratory findings, diagnosis and treatment of ADA-deficient SCID were discussed RESULTS: The patient had the condition with failure to thrive. The main complaint was more than three weeks of fever and rash. He had received the live attenuated Oka strain varicella vaccination approximately two weeks before the onset of rash. Varicella infection was confirmed with direct immunofluorescence assay. The patient had mild leukopenia, with 3% lymphocytes. The initial immunologic workup included decreased IgG, IgM and IgA, abnormal expanded lymphocyte enumeration which confirmed the reduction of CD3, CD4, CD8, CD19 and CD56. Enzyme testing for ADA activity showed remarkably low level in the hemolysate, as well as increased levels of deoxyadenosine nucleotides CONCLUSION: ADA-deficient SCID has some characteristic clinical and laboratory findings. Management options for ADA-deficient SCID include hematopoietic stem cell transplantation, enzyme replacement therapy and gene therapy. Immunodeficiency should be considered in children with severe failure-to-thrive. Live vaccine administration should be avoided in patients with immunodeficiency |
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| Beschreibung: | Date Completed 02.12.2010 Date Revised 07.12.2022 published: Print Citation Status MEDLINE |
| ISSN: | 0578-1310 |