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Nezelof syndrome; combined immunodeficiency with normal immunoglobulins
Described by Nezelof & associates in 1964.
Epidemiology:
- infants
Pathology:
- combined immunodeficiency with normal immunoglobulins
Clinical manifestations:
- recurrent infections
- failure to thrive
- diminished lymphoid tissue
- abnormal structure or agenesis of the thymus
- impaired antibody synthesis
Laboratory:
- complete blood count
- lymphopenia
- serum immunoblobulins
- normal or increased levels of one or more of the major immunoglobulin classes
Complications:
- malignancy
- death
General
syndrome
combined immune deficiency; severe combined immunodeficiency disease (SCID CID)
References
- Wikipedia: Nezelof_syndrome
http://en.wikipedia.org/wiki/Nezelof_syndrome
- Lin CY et al
Report of a case of Nezelof syndrome.
Zhonghua Min Guo Wei Sheng Wu Ji Mian Yi Xue Za Zhi. 1980 Mar;13(1):52-62.
PMID: 7449523