Paget disease of bone: Diagnosis and drug therapy

Seton, Margaret

doi:10.3949/ccjm.80a.12142

Cited by 26 publications

(30 citation statements)

References 78 publications

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“…Dünya çapında değişken bir coğrafi dağılıma sahiptir ve görülme sıklığı yaş ile artmaktadır. Elli beş yaş üzerindeki bireylerde prevalansı %1-3 civarında iken, 80 yaş üzerinde bu oran %8'e kadar çıkmaktadır (3). Erkeklerde kadınlara oranla daha sık görülür.…”

Section: Introductionunclassified

“…Bu aktivite hem kemik yapımını yansıtan ALP'nin serum seviyelerinde artışa ve hem de artmış kemik rezorpsiyonunun belirteçi olan kolajen yıkım ürünlerinin üriner atılımında artışa neden olur. Laboratuvarda serum kalsiyum, fosfor, magnezyum ve parathormon düzeyleri genellikle normaldir (3,11,12). Uzun süreli immobil kalan hastalarda veya kırık sonrası hiperkalsemi görülebilmektedir (12).…”

Section: Introductionunclassified

“…En sık görülen komplikasyonlar; patolojik kırık, kemik deformiteleri, dejeneratif artrit, işitme kaybı, baziller invajinasyon, sinir kökü basıları, kord kompresyonu, ciddi kemik tutulumu olan olgularda kardiak output artışına bağlı kalp yetmezliği ve osteosarkomdur. Sarkomatöz değişim oranı, sınırlı hastalıkta %0,1 iken, yaygın hastalıkta %5-10 arasındadır (2,3,11). Tedavide ilk seçenek ilaçlar nitrojen içeren bifosfonatlardır.…”

Section: Introductionunclassified

“…Tedavi için başlıca endikasyon semptomların varlığıdır (2,3,12,14). Kalsiyum ve D vitamini replasmanının bifosfonat tedavisi ile birlikte uygulanması şarttır (10).…”

Section: Introductionunclassified

“…Potent bifosfonatların intravenöz uygulanması kemik döngüsünün kısa dönemde kontrolünü ve uzun dönem takipte de remisyonunu sağlar. Zoledronik asit paget hastalığı tedavisinde kullanılan bir bifosfanat olup intravenöz olarak tek dozda kullanımı, hasta uyumunun iyi olması, klinik ve laboratuvar hızlı yanıt alınması ve gastrointestinal yan etkilerinin daha az olması nedeniyle tercih edilebilir (3,12). Hastaların yaklaşık %40'ında infüzyon sonrası grip benzeri tablo gelişir.…”

Section: Introductionunclassified

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Paget’s Disease of the Bone: A Case Report

Özer¹,

Erol²,

Çelik³

2016

Tod

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Section: Introductionunclassified

“…Tedavi için başlıca endikasyon semptomların varlığıdır (2,3,12,14). Kalsiyum ve D vitamini replasmanının bifosfonat tedavisi ile birlikte uygulanması şarttır (10).…”

Section: Introductionunclassified

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Paget’s Disease of the Bone: A Case Report

Özer¹,

Erol²,

Çelik³

2016

Tod

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A 49‐Year‐Old Man With Debilitating Aches and Pains and a Mysterious Culprit

Abi‐Ghanem

Chouairy

Meguerian

et al. 2019

Arthritis Care & Research

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Clinical Characteristics and Evolution of Giant Cell Tumor Occurring in Paget's Disease of Bone

Rendina

Filippo

Ralston

et al. 2014

Journal of Bone and Mineral Research

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Patients with Paget's bone disease (PDB) have an increased risk of developing giant cell tumor (GCT). This study was performed to evaluate the clinical characteristics and evolution of GCT complicating PDB and to compare these clinical characteristics to those observed in two large PDB cohorts, the PDB Italian Registry and the United Kingdom's Multi-Centre Randomised Controlled Trial of Symptomatic Versus Intensive Bisphosphonate Therapy for Paget's Disease (PRISM) study. A systematic literature review identified 117 cases of PDB complicated by GCT (PDB-GCT), which involved the skeletal sites affected by PDB (110 patients) or the extraskeletal tissues adjacent to affected bones (7 patients). In contrast to what previously reported for GCT patients without GCT patients (83.2%) were white and one-fourth of them (24.8%) had multifocal GCTs. Compared to PDB patients without GCT, PDB-GCT patients showed a higher male/female ratio (2.1 versus 1.2) and more severe disease (age at PDB onset 52.1 AE 12.1 versus 63.3 AE 10.6 years; number of affected sites 6.1 AE 2.9 versus 2.34 AE 1.6; prevalence of polyostotic PDB 93.3% versus 60.6%). The mortality rate of PDB-GCT patients was higher than those occurring in GCT patients without PDB (about 50% versus 0% to 5% at 5 years) or in PDB patients without GCT (log rank ¼ 29.002). Moreover, up to 98% of PDB-GCT cases had elevated total alkaline phosphatase levels at neoplasm diagnosis, suggestive of active PDB. Importantly, PDB-GCT patients from Southern Italy (45.6% of all GCT patients) showed a higher prevalence of multifocal GCT (51.7%) and of positive familial history for PDB (70.8%) and GCT (65.0%). Finally, indirect evidence suggests a decline in the incidence of GCT in PDB patients. The occurrence of GCT in PDB patients is associated with severe disease and reduced life expectancy of affected patients. The increased prevalence of familial diseases in PDB-GCT patients from Southern Italy suggests a founder effect. The observed changes over time in the incidence of GCT in PDB patients could be related to improved clinical management and/or living conditions of patients.

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Paget disease of bone: Diagnosis and drug therapy

Cited by 26 publications

References 78 publications

Paget’s Disease of the Bone: A Case Report

Paget’s Disease of the Bone: A Case Report

A 49‐Year‐Old Man With Debilitating Aches and Pains and a Mysterious Culprit

Clinical Characteristics and Evolution of Giant Cell Tumor Occurring in Paget's Disease of Bone

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