Transient nephrotic syndrome after anaesthesia resulting from a familial cryofibrinogen precipitating at 35 degrees C

Y Lolin, P A Razis, P O'Gorman, M Hjelm, A S Wierzbicki
1989 Journal of Medical Genetics  
Transient nephrotic syndrome, haematuria, and cryofibrinogenuria in a child after anaesthesia were found in association with a plasma cryofibrinogen that precipitated at 35°C. Investigation of the family showed this to be a familial trait probably with dominant inheritance. In 1955, Korst and Kratochvill coined the term cryofibrinogen to describe a plasma protein which precipitated on cooling and redissolved on rewarming to 37°C. Since then, cryofibrinogenaemia has been found to occur in 3% of
more » ... ospital patients.2 3 It has been described in association with a variety of conditions including malignancy,3 6 infection,' 7-9 autoimmune disorders,57 10 thromboembolic disease, 3 5 711 glomerulonephritis,12 13 diabetes mellitus,3 pregnancy,3 14 and in women taking oral contraceptives.15 In rare instances when no underlying disease has been found, the cryofibrinogenaemia has been termed primary or essential.3 5 16 Cold related symptoms usually sugest the diagnosis, but they may be absent.3 5 6 Heat loss is a common accompaniment of anaes-thesia18 because of the use of cold and dry gases, exposure to a cool environment of often vasodilated patients, heat loss from the operation site, the infusion of cold fluids, and abolition of the shivering reflex by muscle relaxants. It is greatest in children because of their larger surface area to volume ratio, with most heat loss occurring in the first operative hour. We report an unusual presentation of the complications of a cryofibrinogen that precipitated at 35°C in a child after anaesthesia and a hitherto undescribed familial form of the condition. Case report The proband, a seven year old girl, was admitted to the National
doi:10.1136/jmg.26.10.631 fatcat:6ilurcqt2ncbddozltjtec2jem