Nijmegen breakage syndrome

Orpha code: 647OMIM code: 251260

Definition

A rare, genetic chromosomal instability syndrome presenting at birth with microcephaly, dysmorphic facial features which become more noticeable with age, growth delay, recurring sinopulmonary infections and extremely high frequency of malignancies.

Disease data
Classification

Malformation syndrome

Synonyms
AT V1
AT V1
Ataksja-telangiektazja, wariant 1
Berlin breakage syndrome
Małogłowie - Niedobór odporności - lymphoreticuloma
NBS
Niedobór odporności- małogłowie - niestabilność chromosomowa
Zespół Seemanova typu 2
Ataxia-telangiectasia, variant 1
Berlin breakage syndrome
Immunodeficiency-microcephaly-chromosomal instability syndrome
Microcephaly-immunodeficiency-lymphoid malignancy syndrome
NBS
Seemanova syndrome type 2
ORPHA code
647
OMIM code
251260
ICD10 code
Q87.8
ICD11 code
4A01.31

No additional description.

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