trichothiodystrophy


trich·o·thi·o·dys·tro·phy

(trik'ō-thī'ō-dis'trŏ-fē), [MIM*234050 and MIM*601675] Congenital brittle hair resulting from low sulfur-containing amino acid (cysteine) content sometimes associated with mental impairment and short stature; autosomal recessive inheritance. [tricho- + thio- + G. dys, bad, + trophē, nourishment]

BIDS syndrome

An acronym for an autosomal recessive genodermatosis (MIM:234050) defined by its characteristic disorders: 
▪ Brittle hair;
▪ Intellectual impairment;
▪ Decreased fertility;
▪ Short stature.
A condition characterised by brittle, sulfur-poor hair, mental and physical retardation, onychodystrophy, ichthyotic skin, caused by an inherited defect in the ‘excision repair’ pathway