MORQUIO'S SYNDROME

also called mucopolysaccharidosis IV uncommon hereditary disorder of intracartilaginous bone development that results in severe malformation of the skeleton and dwarfing. The disease is recognized within the first two years of life and is usually progressive until bone growth ceases in late adolescence. The vertebral bodies are wedge shaped and flattened, and back deformity is usual. The heads of the thighbones are small and malformed, sometimes resulting in dislocation of the hip; knock-knees and asymmetrical development of paired bones are also common. Associated findings include clouded corneas, circulatory malformations, and a tendency to compression of the spinal cord when back deformity is severe, but intelligence and life expectancy are apparently unaffected.