Figure 4.

Small hands with distinctive brachydactyly, short middle and distal phalanges, small nails, single transverse palm creases, and 5th finger clinodactyly. A. and D. Patient 1 Male hand X-ray at age 6 months showed delayed carpal ossification. B. and E. Patient 5 Male hand X-ray at age 30 months showed short 2^nd, 3^rd and 4^th distal phalanges with severe delay in the bone age and disharmonic osseous maturation. C. and F. Patient 6 Male hand X-ray at 7 years, 5 months showed short 2^nd and 5^th middle phalanges with 5^th finger clinodactyly. The 2^nd middle phalanx showed a coned epiphysis, and bone age was severely delayed to 4 years 9 months. G. and J. Patient 9 Male hand X-ray at age 8 years, 8 months showed carpal and phalangeal ossification centers were markedly delayed to 5–6 years (3.6 SD below mean for age). H. and K. Patient 7 Female hand X-ray at age 12 years showed shortening of the 3^rd, 4^th and 5^th metacarpals with milder shortening of the 2^nd metacarpal. Proximal phalanges appeared normal, but there was shortening of the 2^nd, 4^th and 5^th middle phalanges and shortening of the 2^nd, 3^rd, 4^th and 5^th distal phalanges. There was disharmonic osseous maturation with small carpal centers and short thumbs. I. and L. Hand from a 12-year-old girl with MOPD II and biallelic loss-of-function mutations in the centrosomal pericentrin gene (PCNT) showing similar radiographic features resulting in disharmonic osseous maturation (Figures courtesy of Anita Rauch with permission from Science).