Primordial Dwarfism

Description: Primordial has been defined as belonging to or being characteristic of the earliest stages of development of an organism. Therefore primordial dwarfism is a class of disorders where growth delay occurs at the earliest stages of development. Unlike some of the other forms of dwarfism where newborn infants can have average lengths, children with primordial dwarfism are born smaller than average and have intrauterine growth retardation (IUGR). Unlike some of the other specific conditions, primordial dwarfism is not a specific diagnosis. It is in fact a class of disorders to which at least 5 different conditions are currently grouped: Russell-Silver syndrome, Seckel syndrome, Meier-Gorlin syndrome, and Majewski osteodysplastic primordial dwarfism (MOPD) Types I/III and Type II. The Russell-Silver, Seckel and Meier-Gorlin syndromes are relatively well defined entities and we will not discuss them here. We will limit our discuss to MOPD Type II and most of the information below can be examined in more detail in Hall et. al (1).