Rabson-Mendenhall syndrome

Rabson-Mendenhall syndrome is an autosomal recessive disorder caused by a loss-of-function mutation of the insulin receptor. An insulin resistent diabetes mellitus ensues further characterized by hyperinsulinemia and various somatic abnormalities. There are striking similarities between Rabson-Mendenhall and Donohue syndromes. The latter being published shortly before.

Historical Aspects

The disorder was first described by Rabson and Mendenhall in 1956. The found a family of 3 affected children who later died of ketoacidosis.[Error: Macro 'ref' doesn't exist]

Systematic

Neonatal diabetes mellitus
	Leprechaunism
	Mitchell-Riley syndrome
	Neonatal diabetes mellitus with congenital hypothyroidism
	Pancreatogenic diabetes
	Permanent neonatal diabetes mellitus
	Rabson-Mendenhall syndrome
		INSR
	Renal cysts and diabetes (RCAD)
	Transient neonatal diabetes mellitus 1
	Transient neonatal diabetes mellitus 2
	Transient neonatal diabetes mellitus 3
	X-linked immunodysregulation, polyendocrinopathy, and enteropathy