This leads to abnormal development of many body systems. Rare individuals thought to have SGS do not have a SKI gene mutation, suggesting that other genes may be associated with this condition that have not yet been identified.
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Abstract Marfan syndrome is an autosomal dominant systemic disorder of the connective tissue. Children affected by the Marfan syndrome carry a mutation in one of their two copies of the gene that encodes the connective tissue protein fibrillin Marfan syndrome affects most organs and tissues, especially the skeleton, lungs, eyes, heart, and the large blood vessel that distributes blood from the heart to the rest of the body.
A case report of Marfan syndrome has been reported with oral features.
The dental problems of the child were treated under general anesthesia and a one-month review showed intact stainless steel crowns' restorations and no signs of secondary caries. Introduction Marfan syndrome is an autosomal dominant systemic disorder of connective tissue [ 1 ].
Children affected by the Marfan syndrome carry a mutation in one of their two copies of the gene that encodes the connective tissue protein fibrillin-1 FBN 1 [ 2 ]. It mostly affects skeleton, lungs, eyes, heart, and the aorta [ 3 ].
Affected individuals often are tall and slender, have arachnodactyly, scoliosis, and either a pectus excavatum, pectus carinatum, or ectopia lentis in eyes [ 4 ]. The incidence of mitral prolapse in such patients is essentially equal in children and adults of the same sex [ 5 ].
The oral cavity shows high-arched palate that result from a narrow maxilla [ 7 ]. Connective tissue disorders have been associated with severe periodontitis [ 8 ]. Here I am presenting a case report of Marfan syndrome with dental decay which is successfully treated.
|Beals Syndrome | The Marfan Foundation||It can last only a few minutes or linger for many hours.|
|SADS - SADS Foundation||Marfan Syndrome What Is Marfan syndrome is a condition in which your body's connective tissue is abnormal. Connective tissue helps support all parts of your body.|
|Marfan Syndrome: A Case Report||Current Research Beals Syndrome Beals syndrome and Marfan syndrome are similar in many ways, but there are also some important differences, specifically how the joints are affected. It is important for people with features of Beals syndrome to obtain an accurate diagnosis so they can benefit from treatments, such as physical therapy, to improve joint mobility as soon as possible.|
Case Report A 6-year-old girl reported along with her mother to the Private Dental Clinic, Chennai, with a chief complaint of decayed teeth in both upper and lower front and back teeth region.
The medical history revealed that she has Marfan syndrome with tricuspid and mitral valve prolapse and dilation of aorta. General examination revealed elongated fingers and toes arachnodactyly Figures 1 and 2.
On intraoral examination, and there was presence of high arch palate and dental caries in 52, 54, 62, 64, 73, 74, 82, 83, and Class 1 malocclusion with crowding in lower anteriors was present. An OPG was taken Figure 3which showed dental caries involving the pulp in 54, 74, 83, 84, and Resorption of roots was seen in Pretreatment photographs were taken Figures 4 and 5.
Pulp therapy was done in 54, 73, 74, 75, and Stainless steel crown was placed in 54, 74, 75, and 84 with type I GIC.
Extraction of 64 was done under local anesthesia. Photograph was taken showing the treatment procedures under general anesthesia Figure 6.
Postoperative photographs were taken Figures 7 and 8.
The patient was reviewed after treatment for one day and she was discharged from the hospital. A one week review showed intact restorations and good retention of stainless steel crown.
A one-month review of the child showed intact restorations and no signs of secondary caries Figure 9. OPG showing grossly decayed 54 and 61, 83, 84, and 85 showed dental caries involving the pulp in 54, Intraoral preoperative photographs showing dental caries in 54 and Marfan syndrome affects most organs and tissues, especially the skeleton, lungs, eyes, heart, and the large blood vessel that distributes blood from the heart to the rest of the body.
A case report of Marfan syndrome has been reported with oral features. Conclusion: Marfan syndrome is the most common inherited connective tissue disorder with diverse clinical manifestations. Although many studies have been conducted which aimed at improving the medical aspect of management, those trials produced conflicting results and .
Raynaud's syndrome is a rare condition that limits blood circulation to some areas of your body, causing them to feel numb and cold. Click here to learn more about this condition, plus 6 natural ways to help manage symptoms. A number sign (#) is used with this entry because Loeys-Dietz syndrome-2 (LDS2) is caused by heterozygous mutation in the TGFBR2 gene on chromosome 3pFor a general phenotypic description and a discussion of genetic heterogeneity of Loeys-Dietz syndrome, see LDS1 ().
Clinical Features. Transforming growth factor beta (TGF-β) is a multifunctional cytokine belonging to the transforming growth factor superfamily that includes four different isoforms (TGF-β 1 to 4, HGNC symbols TGFB1, TGFB2, TGFB3, TGFB4) and many other signaling proteins produced by all white blood cell lineages.
Activated TGF-β complexes with other . Marfan syndrome is a disorder that affects the connective tissue in many parts of the body. Connective tissue provides strength and flexibility to structures such as bones, ligaments, muscles, blood vessels, and heart valves.