ORIGINAL ARTICLE

Evaluation of the Relationship Between Jaw Involvement and Systemic Involvement in Type 1 Gaucher Disease
Tip I Gaucher Hastalığında Çene Tutulumu ve Sistemik Tutulum Arasındaki İlişkinin Değerlendirilmesi
Received Date : 10 Feb 2021
Accepted Date : 07 Jun 2021
Available Online : 21 Jun 2021
Doi: 10.25179/tjem.2021-82213 - Makale Dili: EN
Turk J Endocrinol Metab. 2021;25:261-271
Bu makale, CC BY-NC-SA altında lisanslanmış açık erişim bir makaledir.
ABSTRACT
Objective: This study aimed to assess osteopenia and trabeculation loss in the jaws of patients diagnosed with Type 1 Gaucher disease (GD) using cone-beam computed tomography (CBCT) images, unlike the routine computed tomography, and evaluate the correlation of jaw involvement with systemic involvement of GD. Material and Methods: A total of 16 patients, five males and 11 females, who were followed up with the diagnosis of Type I GD were included in the study group. Moreover, 16 age-and sex-matched healthy individuals in the study group were included in the control group. The records of patients at the time of diagnosis were obtained, and the presence of generalized osteopenia of jaws was investigated in the CBCT images, which were taken for maxillofacial examinations. Results: In comparison, the incidence of generalized osteopenia of jaws was significantly higher in the study group than in the control group (p=0.001). In addition, six out of seven patients with hepatomegaly and seven of 11 splenomegaly/operated patients had generalized osteopenia. However, no statistically significant relationship was observed between the presence of hepatomegaly/splenomegaly and generalized osteopenia. In addition, no significant difference was observed among bone involvement, hematological parameters, and jaw involvement in patients with Type 1 GD (p>0.05). Conclusion: Trabecular bone loss and osteopenia increased in Type 1 GD; therefore, patients with Type 1 GD should be evaluated periodically in terms of jaw involvement along with other parameters.
ÖZET
Amaç: Bu çalışmada, Tip I Gaucher hastalığı (GH) tanısı konmuş hastaların rutin bilgisayarlı tomografiden farklı olarak konik ışınlı bilgisayarlı tomografi (KIBT) görüntülerinde çenelerde osteopeni ve trabekülasyon kaybının incelenmesi ve çene tutulumunun GH’nin sistemik tutulumuyla korelasyonunun değerlendirilmesidir. Gereç ve Yöntemler: Tip I GH tanısı ile izlenen 5 erkek, 11 kadın toplam 16 hasta çalışma grubuna dâhil edildi. Çalışma grubundaki hastalara benzer yaş ve cinsiyette 16 sağlıklı birey kontrol grubuna dahil edildi. Tanı anındaki dosya kayıtları elde edildi ve maksillofasiyal incelemeler için alınan KIBT görüntülerinde çenelerde generalize osteopeni varlığı sorgulandı. Bulgular: Çalışma ve kontrol grupları çenelerin generalize osteopenisi açısından karşılaştırıldığında, Tip I GH hastalarından oluşan çalışma grubunda bu bulgu kontrol grubuna göre anlamlı düzeyde yüksek bulundu (p=0.001). Hepatomegalik toplam 7 hastanın 6’sında; splenomegalik/opere olan toplam 11 hastanın 7’sinde generalize osteopeni vardı. Ancak, çalışma grubunda hepatomegali/splenomegali ile generalize osteopeni arasında istatistiksel anlamlı bir ilişki bulunmadı. Ayrıca Tip I GH hastalarının kemik tutulumu ve hematolojik parametreleri ile çene tutulumu arasında anlamlı bir farklılık bulunmadı (p>0.05). Sonuç: Tip I GH’de trabeküler kemik kaybı ve osteopeni artmıştır. Bu nedenle, Tip I GH hastaları diğer parametrelerle birlikte çenelerin tutulumu açısından periyodik olarak değerlendirilmelidir.
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