Search

The pathology of an unusual form of nonneuropathic familial amyloidosis is presented. The disease, occurring in two sisters, is similar to a form of familial amyloidosis described by Ostertag in 1950 and is characterized by progressive renal failure and an autosomal dominant mode of inheritance. Widespread vascular amyloidosis and dense renal glomerular amyloid deposits characterize the disease pathologically. Renal interstitial foam cells and glomerular giant cells were found in the kidneys of one sister. The giant cells, probably of macrophage origin, contain amyloid fibrils within poorly formed non-membrane-bounded cytoplasmic vacuoles. Transitions to well-formed membrane-bounded cytoplasmic vacuoles with nonfibrillar material were also present. It is suggested that these cells function to degrade and resorb amyloid, and that as amyloid is changed to a nonfibrillar state, well-defined membranes can form around the degraded material.

Twenty-five cases of severe pneumonitis occurring among children aged 3 to 18 months were observed in Auckland, New Zealand, in 1965. An apparent relationship was established between a uniform disease picture and recovery of adenovirus type 21. Two died, and of the survivors 60% had permanent lung damage, probably resulting from obliterative bronchiolitis. Twenty per cent. were left with proved bronchiectasis. The possible relationships of adenovirus pneumonia to malnutrition or a genetic factor on the one hand and to the development of bronchiectasis on the other are discussed.