Metabolizam kosti u bolesnika s hemofilijom

Boban, Ana (2011) Metabolizam kosti u bolesnika s hemofilijom. PhD thesis, Sveučilište u Zagrebu.

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Abstract

Hemophilia is a rare inherited disorder characterized by spontaneous bleedings into joints and muscles, with development of a typical hemophilic arthropathy. Several studies have shown that patients with hemophilia have reduced bone mineral density, but the etiology is unclear. We evaluated the incidence of ostoporosis and investigated possible risk factors for development of reduced mineral bone density in hemophilia patients. We evaluated 58 patients with severe, mild and moderate hemophilia A and B. Bone mineral density was measured with dual-energy X-ray absorptiomery and quantitative ultrasound of the heel. Bone turnover was evaluated by the measurement of bone formation markers (osteocalcin and bone alkaline phosphatase) and bone resorption markers (C-terminal cross-linking telopeptide of collagen type I). Infection with hepatitis B virus, verification of FVIII inhibitors and number of target joint were used for assessment of risk factors. Out of 58 patients with hemophilia 48% had normal bone mineral density, 48% osteopenia and 9% osteoporosis. The severity of osteoporosis on femoral neck correlated with severity of hemophilia. No correlation was found between bone mineral density and number of target joints, infection with hepatitis C or presence of FVIII inhibitors. When assessed with quantitative ultrasound of the heel, patients with hemophilia has significantly lower levels of QUS parameters when compared to healthy controls (p<0,000). The QUS values significantly correlated with severity of hemophilia and number of target joints, but no correlation was found in comparison with presence of FVIII inhibitors or infection of hepatitis C virus. Our results showed that patients with hemophilia have high risk of developing osteoporosis and osteopenia, which is dependent on severity of hemophilia, but not on presence of FVIII inhibitors or hepatitis C infection. Quantitative ultrasound of the heel, after modification of T- cut off values, showed high sensitivity and specificity for detection of reduced bone mineral density in hemophilia patients.

Abstract in Croatian

Hemofilija je rijetka nasljedna bolest koju karakterizira sklonost prema spontanim krvarenjima u mišiće i zglobove, s posljedičnim razvojem hemofilične artropatije i invalidnosti. Ovo istraživanje smo proveli s ciljem procjene koštanog statusa i dinamike koštane pregradnje u bolesnika s hemofilijom, te procjene rizičnih faktora koji mogu utjecati na razvoj smanjene mineralne gustoće kosti. Istraživanjem smo obradili 58 bolesnika s teškom, umjerenom i blagom hemofilijom A, te jednog bolesnika s hemofilijom B. U svrhu procjene koštanog statusa bolesnicima je učinjeno mjerenje mineralne gustoće kosti metodom denzitometrije i metodom kvantitativnog ultrazvuka petne kosti. Procjena dinamike koštane pregradnje je učinjena određivanjem markera koštane izgradnje (osteokalcina i koštane alkalne fosfataze) i markera koštane razgradnje (β-crosslaps). U svrhu procjene rizičnih faktora bolesnicima je učinjen serološki status infekcije virusom hepatitisa C, određena prisutnost protutijela na FVIII te zabilježen broj ciljnih zglobova. Među bolesnicima s hemofilijom je urednu mineralnu gustoću kosti imalo 43% bolesnika, osteopeniju 48%, a osteoporozu 9% bolesnika. Mineralna gustoća kosti je bila značajno snižena u bolesnika s hemofilijom u usporedbi sa zdravim kontroloma (p=0,002), te nije ovisila o prisutnosti protutijela na VIII, infekciji virusom hepatitisa C niti broju ciljnih zglobova. Mjereno kvantitativni ultrazvukom petne kosti, bolesnici s hemofilijom su imali značajno niže vrijednosti parametara QUI, BUA i SOS u usporedbi sa zdravim kontrolama (p>0,000). Ove vrijednosti su ovisile o težini hemofilije (p=0,001) i broju zahvaćenih ciljnih zglobova, ali ne i o prisutnosti protutijela na FVIII ili infekciji virusom hepatitisa C. Ovim smo istraživanjem pokazali da bolesnici koju boluju od hemofilije imaju visoki rizik nastanka osteopenije i osteoporoze. Rizik smanjenja mineralne gustoće kosti ovisi o težini hemofilije, te ne ovisi o prisutnosti protutijela na faktor VIII niti o infekciji virusom hepatitisa C. Metoda kvantitativnog ultrazvuka petne kosti je nakon modifikacije prijelomne T-vrijednosti za definiciju osteopenije i osteoporoze 65 pokazala visoku osjetljivost i specifičnost u otkrivanju smanjene mineralne gustoće kosti u bolesnika s hemofilijom.

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