Simultaneous occurrence of diabetes, liver cirrhosis, and 47, XX,21 +/46, XX chromosomal pattern

A girl with juvenile diabetes, liver cirrhosis, and 47,XX,21 +/46,XX chromosomal mosaicism is described. The immunoglobulin pattern suggested an autoimmune process. After prednisolone treatment the IgG level and liver function tests became normal and the liver histology improved.