Activated protein C resistance and false type 2 protein C deficiency detected after multiple shunt failures in a patient with hydrocephalus.
A 2-year-old hydrocephalic boy who had suffered multiple shunt failures was evaluated for hypercoagulability after a thrombus was removed from his right atrium. The work-up revealed that the patient had the heterozygous form of activated protein C resistance and false type 2 protein C deficiency by the clotting method. His protein C activity was normal by the chromogenic method. We suggest that patients having hydrocephalus, shunt-associated thrombus formation, or both should be evaluated for thrombophilic disorders, and protein C activity should be measured by chromogenic assay in patients with documented activated protein C resistance.
Kayiran
S M
SM
Department of Pediatrics, Başkent University, Faculty of Medicine, Bahçelievler, Ankara, Turkey.
Ozbek
N
N
Caner
H
H
Tokel
K
K
Mercan
S
S
Alehan
F
F
D020016:Activated Protein C Resistance / Q000097:blood / Q000150:complications* / Q000235:genetics; D001780:Blood Coagulation Tests / Q000379:methods; D002352:Carrier Proteins / Q000097:blood*; D002557:Cerebrospinal Fluid Shunts / Q000009:adverse effects*; D002675:Child, Preschool; D004868:Equipment Failure; D005189:False Positive Reactions; D006325:Heart Atria; D006331:Heart Diseases / Q000209:etiology* / Q000628:therapy; D006801:Humans; D006849:Hydrocephalus / Q000628:therapy*; D008297:Male; D013927:Thrombosis / Q000209:etiology* / Q000628:therapy
D002352:Carrier Proteins; C020723:citrate-binding transport protein