RUSSELL-SILVER SYNDROME: A CASE REPORT

In 1953 and 1954, Silver and Russell independently described groups of small children whose pregnancies had complicated by intrauterine growth retardation with a both genetically and clinically heterogeneous disorder named Russel Silver Syndrome [RSS/SRS; OMIM 180860]. This case report describes the dental complications of the syndrome. A 4-yearold girl diagnosed with RSS at the Medical Faculty Hospital of Marmara University was directed to Marmara University Department of Pediatric Dentistry for dental examination. Severe dentin caries and pulpitis were detected in many teeth of the primary dentition as an indication of the child being in the high caries risk group (dft/dfs = 13/37). RSS-diagnosed children first experience nutritional difficulty and later face severe dental caries as a consequence.

Anahtar Kelimeler:

russel Silver Syndrome, hypoglicemia

PDF

___

[1] Silver HK, Kiyasu W, George J, Deamer WC. Syndrome of congenital hemihypertrophy, shortness of stature, and elevated urinary gonadotropins. Pediatrics. 1953;12(4):368-76.
[2] Russell A. A syndrome of intra-uterine dwarfism recognizable at birth with craniofacial dysostosis, disproportionately short arms, and other anomalies (5 examples). Proc R Soc Med. 1954;47(12):1040-4.
[3] Binder G, Begemann M, Eggermann T, Kannenberg K. Silver-Russell syndrome. Best Pract Res Clin Endocrinol Metab. 2011;25(1):153-60.
[4] Price S, Stanhope R, Garrett C, Preece M, Trembath R. The spectrum of SilverRussell syndrome: a clinical and molecular genetic study and new diagnostic criteria. Journal of medical genetics. 1999;36(11):83742.
[5] Hansen KK, Latson LA, Buehler BA, Latson LA. Silver-Russell syndrome with unusual findings. Pediatrics. 1987;79(1):1258.
[6] Wee SA. Russell-Silver syndrome. Dermatology online journal. 2007;13(1):16.
[7] Blissett J, Harris G, Kirk J. Feeding problems in Silver-Russell syndrome. Developmental Medicine & Child Neurology. 2001;43(1):39-44.
[8] Sato C, Ogawa T, Tsuge R, Shiga M, Tsuji M, Baba Y, et al. Systemic and maxillofacial characteristics of 11 Japanese children with Russell-Silver syndrome. Congenit Anom (Kyoto). 2016;56(5):217-25.
[9] Bergman A, Kjellberg H, Dahlgren J. Craniofacial morphology and dental age in children with Silver–Russell syndrome. Orthodontics & Craniofacial Research. 2003;6(1):54-62.
[10] Kotilainen J, Hölttä P, Mikkonen T, Arte S, Sipilä I, Pirinen S. Craniofacial and dental characteristics of Silver-Russell syndrome. American Journal of Medical Genetics. 1995;56(2):229-36.
[11] World Health O. Oral health surveys: basic methods: World Health Organization; 2013.
[12] Flykanaka-Gantenbein C. Hypoglycemia in childhood: long-term effects. Pediatr Endocrinol Rev. 2004;1 Suppl 3:530-6.
[13] Touger-Decker R, van Loveren C. Sugars and dental caries. Am J Clin Nutr. 2003;78(4):881S-92S.