Lumping and Splitting

In 1945, Smith and Graham at Cornell University Medical College in New York reported the case of a child with progressive uremia (urea nitrogen rose as high as 222 mg/100 mL in those pre-dialysis days), acidosis, and refractory anemia. At autopsy, the kidneys showed multiple cysts confined to the pyramids. Both kidneys were diffusely fibrotic, with partially hyalinized glomeruli and atrophied tubules. These authors did not venture to name the disorder, but by 1954 the name “medullary cystic disease” (MCD) had been given to a similar condition in other children and adults, and associated salt-wasting was observed in many of the cases (1).