Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report
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 abnormal...
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| Format: | Article |
| Language: | English |
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Mazandaran University of Medical Sciences
2022-12-01
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| Series: | Journal of Pediatrics Review |
| Subjects: | |
| Online Access: | http://jpr.mazums.ac.ir/article-1-479-en.html |
| _version_ | 1797946313838428160 |
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| author | Bahareh Nazemi Salman Nazila Biglar Masoumeh Mirkeshavarz Ghasem Ansari |
| author_facet | Bahareh Nazemi Salman Nazila Biglar Masoumeh Mirkeshavarz Ghasem Ansari |
| author_sort | Bahareh Nazemi Salman |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-10T21:09:03Z |
| format | Article |
| id | doaj.art-b01e8ec4fe4f4bb898cb9398b4a3cd4f |
| institution | Directory Open Access Journal |
| issn | 2322-4398 2322-4401 |
| language | English |
| last_indexed | 2024-04-10T21:09:03Z |
| publishDate | 2022-12-01 |
| publisher | Mazandaran University of Medical Sciences |
| record_format | Article |
| series | Journal of Pediatrics Review |
| spelling | doaj.art-b01e8ec4fe4f4bb898cb9398b4a3cd4f2023-01-21T08:52:14ZengMazandaran University of Medical SciencesJournal of Pediatrics Review2322-43982322-44012022-12-01104321330Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case ReportBahareh Nazemi Salman0Nazila Biglar1Masoumeh Mirkeshavarz2Ghasem Ansari3 Department of Pedodontics, School of Dentistry, Zanjan University of Medical Sciences, Zanjan, Iran. Department of Pedodontics, School of Dentistry, Zanjan University of Medical Sciences, Zanjan, Iran. Department of Oral and Maxillofacial Pathology, School of Dentistry, Zanjan University of Medical Sciences, Zanjan, Iran. Department of Pedodontics, School of Dentistry, Shahid Beheshti University of Medical Sciences, Tehran, Iran. 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.http://jpr.mazums.ac.ir/article-1-479-en.htmlgrowth disordersdwarfismmicrocephalic osteodysplastic primordial dwarfismtype iichilddentalpediatric dentistrypericentrin |
| spellingShingle | Bahareh Nazemi Salman Nazila Biglar Masoumeh Mirkeshavarz Ghasem Ansari Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report Journal of Pediatrics Review growth disorders dwarfism microcephalic osteodysplastic primordial dwarfism type ii child dental pediatric dentistry pericentrin |
| title | Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report |
| title_full | Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report |
| title_fullStr | Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report |
| title_full_unstemmed | Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report |
| title_short | Clinical Findings and Dental Manifestations Associated With Microcephalic Osteodysplastic Primordial Dwarfism Type II: A Case Report |
| title_sort | clinical findings and dental manifestations associated with microcephalic osteodysplastic primordial dwarfism type ii a case report |
| topic | growth disorders dwarfism microcephalic osteodysplastic primordial dwarfism type ii child dental pediatric dentistry pericentrin |
| url | http://jpr.mazums.ac.ir/article-1-479-en.html |
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