Wiskott-Aldrich syndrome: life-threatening haemorrhage from aneurysms within the liver, small bowel mesentery and kidney, requiring both surgical and radiological intervention.
Wiskott-Aldrich syndrome (WAS) is a rare, generally X-linked recessive condition, originally described by Wiskott in 1937 as a triad of discharging ears, eczema and thrombocytopoenia. Aldrich included bloody diarrhoea in his report of 1954, with severe immunodeficiency and predisposition to malignancy being recognised subsequently. The incidence currently quoted is approximately 4 per million live male births, although there is some regional variation. We report the case of a long-term survivor who had massive haemorrhage from an intrahepatic aneurysm and, on a separate occasion, the right kidney.
Loan
W
W
Department of Radiology, Royal Victoria Hospital, Belfast. w@loan.freeserve.co.uk
McCune
K
K
Kelly
B
B
Maxwell
R
R
D000328:Adult; D000783:Aneurysm / Q000000981:diagnostic imaging / Q000601:surgery; D015901:Angiography, Digital Subtraction; D004621:Embolization, Therapeutic; D006406:Hematoma / Q000000981:diagnostic imaging / Q000601:surgery; D006499:Hepatic Artery / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed; D014923:Wiskott-Aldrich Syndrome / Q000000981:diagnostic imaging* / Q000601:surgery*