Tooth retention was evaluated in 12/44, present in 2 individuals (17%), pulpal obliteration, evaluated in 24/44 and present in 24 (100%). [ojrd.biomedcentral.com]

Presents at preschool age (tarda). Hearing deficit in 50%. [orthobullets.com]

Both primary and permanent teeth present pulp obliteration. Patients with this form of DGI have no normal teeth. Sensorineural hearing loss has also been found in some patients. [orpha.net]

Most of the cases affected with DGI require a comprehensive interdisciplinary planning dictated by the age at the time of presentation, clinical presentation, amount of morbidity, patient's expectations, and resources. [ijdr.in]

• Prolonged Bleeding

  One individual (3%) reported a history of prolonged bleeding. Two individuals (6%) had experience of spraining. One 13-year-old girl reported a previous radius fracture (3%). [ojrd.biomedcentral.com]

• Osteoporosis

  Abnormal collagenous maturation results in bone with a thin Cortex, fine trabeculation and diffuse osteoporosis. [juniordentist.com]

  Fractures tend to decrease in frequency after puberty, but their frequency may increase later in life when age- and sex-related osteoporosis is superimposed. Over a lifetime, numbers of fractures can range from one to 60 or more. [emedicine.staging.medscape.com]

  More detailed information on them can be found in Pediatric Bone: Biology and Diseases, Glorieux et al, 2003 3. osteoporosis-pseudoglioma syndrome : severe form of OI that also causes blindness Cole-Carpenter syndrome : OI with craniosynostosis and ocular [radiopaedia.org]

  Bisphosponates which is a type of drug to treat osteoporosis, is often prescribed for patients with osteogenesis imperfect to strengthen the bones. [intelligentdental.com]

  Other rare types of osteogenesis imperfecta include: Osteoporosis-pseudoglioma Bruck-syndrome Cole-Carpenter syndrome. Complications of osteogenesis imperfecta: They are joint laxity, which causes chronic joint pain, deafness and brain compression. [geneticdisorders.info]

• Narrow Pelvis

  Eventually, the wide rib cage overlaps the narrow pelvis. Basilar invagination is an uncommon but potentially fatal occurrence in OI. Vertigo is common in patients with severe OI. [emedicine.staging.medscape.com]

• Saber Shin

  shins skull radiographs reveal wormian bones coxa vara Evaluation Labroratory mildly elevated ALP Histology increased diameter of haversion canals and osteocyte lacunae replicated cement lines increased number of osteoblasts and osteoclasts decreased [orthobullets.com]

Treatment of Dentinogenesis Imperfecta in Children Picture 2 – Dentinogenesis imperfecta Image There are published cases of treatment of this disorder in children. [medicalsubstance.com]

At the moment, there is no standard treatment for amelogenesis imperfecta. Treatment depends on the type and severity of the condition. [healthline.com]

The multidisciplinary approach would help to provide the optimal treatment outcome. [ijmdent.com]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

Challenges are associated with root canal treatment of teeth affected by DI due to pulp chamber and root canal obliteration, or narrowing of such spaces. [en.wikipedia.org]

Prognosis Prognosis depends primarily on the age of diagnosis and the quality of management. Early diagnosis and regular dental care cannot always prevent premature tooth loss. [orpha.net]

Although pulpal pathosis is rarely reported with dentinogenesis imperfecta, endodontic treatment is occasionally necessary and has a guarded prognosis if initiated after pulp canal obliteration has occurred. [streetdirectory.com]

Early diagnosis and treatment intervention helps in better prognosis and rehabilitation in such cases. According to Wei, stainless steel crowns are commonly used to prevent the attrition of the deciduous teeth and young permanent teeth [11]. [ispub.com]

An efficient stomatognathic system was restored aiming at establishing a more favorable prognosis for such a complex anomaly. 1. Tahmassebi JF, Day PF, Toumba KJ, Andreadis GA. Paediatric dentistry in the new millennium: 6. [jisppd.com]

Adapted Sillence Classification of Osteogenesis Imperfecta (Open Table in a new window) Type Genetic Teeth Bone Fragility Bone Deformity Sclera Spine Skull Prognosis IA AD * Normal Variable but less severe than other types Moderate Blue 20% scoliosis [emedicine.staging.medscape.com]

Etiology OI type I is caused by mutations in the COL1A1 and COL1A2 genes (17q21.31-q22 and 7q22.1 respectively). Genetic counseling Transmission is autosomal dominant. [orpha.net]

ETIOLOGY –DGI 1 CAUSED BY MUTATION IN THE DENTIN SIALO PHOSPHO PROTEIN( DSPP) gene ENCODING DENTIN PHOSPHOPROTEIN AND DENTIN SIALOPROTIEN 8. [slideshare.net]

etiology of DI to DEJ and follow Shields classification. [jomfp.in]

Summary Epidemiology The overall prevalence of OI is estimated at between 1/10,000 and 1/20,000 but the prevalence of type I is unknown. [orpha.net]

In addition, the absence of wide epidemiological studies in the literature and the variation of small studies results make it difficult to compare and analyze the obtained results with reference values. [jispcd.org]

Epidemiology Incidence is approximately 1/15,000-1/20,000 live births but this may be underestimated, as milder forms can evade diagnosis [ 7 ]. It is the leading cause of lethal short-limbed dwarfism and skeletal dysplasia. [patient.info]

However, in the past 10 years discoveries of further (mainly recessive) causative genes have lent support to a predominantly collagen-related pathophysiology [ 1 ]. [patient.info]

Osteogenesis Imperfecta: pathophysiology and treatment. Wien Med Wochenschr 2015; 165(13-14): 278-284. Sillence DO, Senn A, Danks DM. Genetic heterogeneity in osteogenesis imperfecta. J Med Genet 1979; 16(2):101-16. [biolifesas.org]

optimizing esthetics,and preventing the common dental problems associated with caries and periodontal disease. [streetdirectory.com]

Preventive efforts can limit pathology occurring within the pulp, which may render future endodontic procedures less challenging, with better outcomes. [en.wikipedia.org]

The early diagnosis and appropriate treatment is of paramount significance to prevent psychological and functional morbidity to the patient. [ijmdent.com]

A mutation in any one of these genes can prevent it from making the correct protein, leading to enamel that is thin or soft. [healthline.com]

It often requires a collaborative approach, with an orthodontist and a prosthodontist, for the completion of the entire dental restoration and prevention procedure. [medicalsubstance.com]