Nijmegen breakage syndrome-like disorder

Orpha code: 240760OMIM code: 613078

Definition

Nijmegen breakage syndrome-like disorder is a rare, genetic multiple congenital anomalies/dysmorphic syndrome characterized by growth retardation, short stature, developmental delay, intellectual disability, craniofacial dysmorphism (i.e. severe microcephaly, sloping forehead, prominent eyes, broad nasal ridge, hypoplastic nasal septum, epicanthal folds), spontaneous chromosomal instability, cellular hypersensitivity to ionizing radiation and radioresistant DNA synthesis, without severe infections, immunodeficiency or cancer predisposition. Additional reported features include mild spasticity, slight and nonprogressive ataxia, hyperopia, multiple pigmented nevi, widely spaced nipples, and clinodactyly.

Disease data
Classification

Malformation syndrome

Synonyms
Microcephaly and chromosomal instability without immunodeficiency
Małogłowie i niestabilność chromosomalna bez niedoboru odporności
NBSLD
Niedobór RAD50
Zaburzenie podobne do NBS
NBS-like disorder
NBSLD
RAD50 deficiency
ORPHA code
240760
OMIM code
613078
ICD10 code
-
ICD11 code
-

No additional description.

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