دوره 10، شماره 4 - ( 9-1401 )                   جلد 10 شماره 4 صفحات 330-321 | برگشت به فهرست نسخه ها


XML English Abstract Print


Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

Nazemi Salman B, Biglar N, Mirkeshavarz M, Ansari G. Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report. J. Pediatr. Rev 2022; 10 (4) :321-330
URL: http://jpr.mazums.ac.ir/article-1-479-fa.html
Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report. Journal of Pediatrics Review 1401; 10 (4) :330-321

URL: http://jpr.mazums.ac.ir/article-1-479-fa.html


چکیده:   (348 مشاهده)

Background: Microcephalic Osteodysplastic Primordial Dwarfism type 2 (MOPD II) is a rare untreatable genetic disorder characterized by severe prenatal and postnatal growth retardation, microcephaly, bird-headed face (receding forehead and chin, a beaklike nose, and prominent eyes), skeletal abnormalities, abnormal dentition, abnormal hair and skin changes, high-pitched nasal voice, and an increased risk for insulin resistance and cerebrovascular disease. MOPDII is caused by mutations in the pericentrin gene and is inherited in an autosomal recessive manner. This study aims to report a MOPD II child patient. 
Case Presentation: A seven-year-old girl genetically diagnosed with MOPD II has been presented in this case report. Clinical, radiological, and laboratory findings with emphasis on oral features have been reported, and her dental problems management has also been described.
Conclusions: MOPD II patients have a shorter life expectancy. The main health complications which need regular care include vascular changes of the central nervous system, diabetes mellitus, renal problems, blood pressure, cardiac pathologies, and hematologic profile. MOPD II patients have a high risk of caries because they consume soft and cariogenic foods due to microdontia, oligodontia, and an incompetent masticatory system. On the other hand, dental treatment for such patients can be very challenging. MOPD II cases and their families should be aware of the importance of oral hygiene and routine dental follow-ups.

متن کامل [PDF 5257 kb]   (214 دریافت)    
نوع مطالعه: Case Report and Review of Literature |
دریافت: 1401/3/29 | پذیرش: 1401/7/10 | انتشار: 1401/9/28

ارسال نظر درباره این مقاله : نام کاربری یا پست الکترونیک شما:
CAPTCHA

بازنشر اطلاعات
Creative Commons License این مقاله تحت شرایط Creative Commons Attribution-NonCommercial 4.0 International License قابل بازنشر است.

کلیه حقوق این وب سایت متعلق به Journal of Pediatrics Review می باشد.

طراحی و برنامه نویسی : یکتاوب افزار شرق

© 2023 CC BY-NC 4.0 | Journal of Pediatrics Review

Designed & Developed by : Yektaweb