Menkes disease: report of two cases.
Menkes disease is a rare, autosomal recessive disorder characterized by neuronal degeneration, abnormal hair, malformed connective tissue, mental retardation, and a life span of three years. Previously reported dental findings include a high arched palate, delayed eruption of secondary dentition, and open bite. The case of twin seven-year-old males with Menkes disease is presented, along with previously unreported dental findings of spindle-shaped root resorption patterns on the primary maxillary central and lateral incisors.
US Army, Fort Lewis, WA, USA.
F Alan
FA
Walker
Curt
C
Goho
Robert B
RB
Schanzer
D002648:Child; D004200:Diseases in Twins; D006801:Humans; D007180:Incisor / Q000002:abnormalities; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000235:genetics*; D024343:Open Bite / Q000235:genetics; D010159:Palate / Q000002:abnormalities; D012391:Root Resorption / Q000235:genetics; D014071:Tooth Abnormalities / Q000235:genetics*; D014078:Tooth Eruption; D014092:Tooth Root / Q000002:abnormalities*; D014094:Tooth, Deciduous / Q000002:abnormalities; D014430:Twins, Monozygotic