Fig. 3.

a-g Patient’s radiographs. a. Left hand of the patient at the age 5 years. There is marked metaphyseal involvement of the distal radius and ulna. Dissociation of the carpal bone age with absent ossification of the proximal carpal raw is demonstrated. Only four small carpal ossification centres are present. b. Knees of 8-year-old patient reveal marked metaphyseal involvement and severe coxa valga. c-f Patient at age 10 years. c. Lateral spine with lumbar lordosis and trapezoid shape of vertebral bodies. d. a-p view of spine that shows progress of spinal scoliosis in spite of 6 years of bracing. e. Pelvis. Note osteoporosis, absent capital femoral ossification and delayed ossification of the pubic bones. f. Left leg. Osteoporosis, severe metaphyseal changes and genu valgum deformity are visible. g. Left hand of 11-year old patient. The tubular bones of the hands are slightly affected. There is severe metaphyseal involvement of the forearm bones and dissociation of the delayed carpal bone age with absent ossification of the proximal carpal raw