RETROSPECTIVE EVALUATION OF THE CLINICAL COURSE OF PAGET’S DISEASE OF BONE

Yıl 2023, Cilt: 6 Sayı: 3, 276 - 280, 24.10.2023

Hülya Hacişahinoğulları , Gamze Bilik Oyman , Gülşah Yenidünya Yalın , Özlem Soyluk Selçukbiricik , Nurdan Gül , Ferihan Aral , Refik Tanakol , Ayşe Kubat Üzüm

https://doi.org/10.26650/JARHS2023-1342541

Öz

Objective: Paget’s disease of bone (PDB) is a focal, chronic, metabolic disorder of bones and causes complications such as bone deformity, fractures, and heart failure. This study aimed to evaluate the clinical characteristics of patients with PDB and patients’ responses to antiresorptive treatments.
Material and Methods: In the study, we retrospectively evaluated the medical records of patients who were followed up with PDB at the Istanbul Faculty of Medicine and treated with antiresorptive therapy.
Results: A total of 26 patients (12 females/14 males) with PDB were evaluated. The mean age at diagnosis was 62.9±13.5 years. The median time from the onset of symptoms to diagnosis was 17.5 months (range 1-480). The symptoms were as follows: pain in 16, swelling in 3, rubor in 2, bone fracture in 1, nephrolithiasis in 1, and hearing loss in 1 patient. Laboratory tests revealed the following results (mean±SD); Alkaline phosphatase (ALP) 512±557 U/L, bone-specific ALP 81.2±51.4 µg/L, and the mean ALP was 4.12±4 times of upper limit of the normal range. The distribution of the disease was as follows: pelvis in 58%, vertebra in 46%, skull in 35%, femur in 23%, tibia in 11.5%, humerus in 3.8% of the patients. Of 26 patients, 21 were treated with zoledronic acid alone. Relapse occurred in four patients; the mean duration from therapy to relapse was 72.25±28.7 months.
Conclusion: Zoledronic acid is a very potent antiresorptive drug and provides long-term remission of the disease even with a single dose of therapy.

Anahtar Kelimeler

Paget’s disease of bone, zoledronic acid, alkaline phosphatase

KEMİĞİN PAGET HASTALIĞININ KLİNİK SEYRİNİN RETROSPEKTİF OLARAK DEĞERLENDİRİLMESİ

Öz

Amaç: Kemiğin Paget hastalığı (KPH), fokal, kronik, metabolik bir kemik hastalığıdır ve kemik deformitesi, kırıklar ve kalp yetmezliği gibi komplikasyonlara neden olur. Bu çalışma, KPH hastalarının klinik özelliklerini ve hastaların antiresorptif tedavilere yanıtlarını değerlendirmeyi amaçladı.
Gereç ve Yöntemler: Bu çalışmada İstanbul Tıp Fakültesi’nde KPH tanısı ile takip edilen ve antirezorptif tedavi uygulanan hastaların tıbbi kayıtları retrospektif olarak değerlendirildi.
Bulgular: KPH olan toplam 26 hasta (12 kadın/14 erkek) değerlendirildi. Ortalama tanı yaşı 62.9±13.5 idi. Semptomların başlangıcından tanıya kadar geçen medyan süre 17.5 aydı (1-480 arası). Belirtilerden ağrı 16 hastada, şişlik 3 hastada, kızarıklık 2 hastada, kırık 1 hastada, nefrolitiazis 1 hastada, işitme kaybı 1 hastada mevcuttu. Laboratuvar incelemesinde ortalama alkalen fosfataz (ALP) 512±557 U/L, kemiğe özgü ALP 81.2±51.4 µg/L ve ALP üst limitin 4.12±4 katı idi. Hastalığın tutulum yerleri %58 pelvis, %46 vertebra, %35 kafatası, %23 femur, %11.5 tibia, %3.8 humerus şeklinde idi. Toplam 26 hastanın 21’i tek başına zoledronik asit ile tedavi edilmişti. Dört hastada nüks meydana geldi ve tedaviden nükse kadar geçen ortalama süre 72.25±28.7 aydı.
Sonuç: Zoledronik asit çok güçlü bir antirezorptif ilaçtır ve tek doz tedavi ile bile hastalığın uzun süreli remisyonunu sağlar.

Anahtar Kelimeler

Kemiğin Paget hastalığı, zoledronik asit, alkalen fosfataz

Kaynakça

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