Description of the disease * EnglishPolish Pobierz sekcję do PDF 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 - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl