Volume 9, Issue 4 (12-2024)                   J Res Dent Maxillofac Sci 2024, 9(4): 282-286 | Back to browse issues page


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Nematnezhad M, shakoori S, Fazlyab M. Oral Manifestations of the Donohue Syndrome: A Case Report. J Res Dent Maxillofac Sci 2024; 9 (4) :282-286
URL: http://jrdms.dentaliau.ac.ir/article-1-509-en.html
1- Department of Endodontics, Islamic Azad University, Dental Branch, Tehran, Iran
2- Department of Endodontics, Islamic Azad University, Dental Branch, Tehran, Iran , drsh.shakoori@gmail.com
Abstract:   (347 Views)
Background and Aim: The Donohue syndrome, also known as leprechaunism, is a rare autosomal recessive genetic disorder of insulin resistance. The severe form of insulin resistance seen in this syndrome is due to a mutation in the insulin receptor gene. This syndrome has symptoms such as delayed growth before and after birth, premature growth, lipoatrophy, hypertrichosis, acanthosis nigricans, and dysmorphic face.
Case Presentation: This report presents oral manifestations (such as macrodontia, severe crowding, supernumerary tooth, etc.) and management of a 10-year-old patient with the Donohue syndrome.
Conclusion: Considering the extensive oral manifestations of the Donohue syndrome, good oral hygiene and regular dental follow-ups are necessary for such patients.
 
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Type of Study: Case report | Subject: Endodontics

References
1. Kirkwood A, Stuart G, Harding L. Donohue syndrome: A review of literature, case series, and anesthetic considerations. Paediatr Anaesth. 2018 Jan;28(1):23-7. [DOI:10.1111/pan.13273] [PMID]
2. Imamura T, Kobayashi M. [Donohue's syndrome (Leprechaunism)]. Nihon Rinsho. 1994 Oct;52(10):2643-7.
3. Sánchez-Hernández RM, Martín-Frías M, Castaño L, Lamas A, Barrio R. Donohue syndrome. Extreme insulin resistance in the neonatal period. Endocrinol Nutr. 2016 Jan;63(1):45-6. [DOI:10.1016/j.endoen.2015.10.010]
4. Wakimoto P, Block G. Dietary intake, dietary patterns, and changes with age: an epidemiological perspective. J Gerontol A Biol Sci Med Sci. 2001 Oct;56 Spec No 2:65-80. [DOI:10.1093/gerona/56.suppl_2.65] [PMID]
5. Odeh R, Alassaf A, Al-Qudah AA. Donohue syndrome: a new case with a new complication. J Pediatr Endocrinol Metab. 2015 Jul;28(7-8):951-4. [DOI:10.1515/jpem-2014-0405] [PMID]
6. Li W, Mai R. A syndrome of insulin resistance resembling Donohue syndrome with patent ductus arteriosus. Clin Lab. 2014;60(2):315-7. [DOI:10.7754/Clin.Lab.2013.130326] [PMID]
7. Nijim Y, Awni Y, Adawi A, Bowirrat A. Classic Case Report of Donohue Syndrome (Leprechaunism; OMIM *246200): The Impact of Consanguineous Mating. Medicine (Baltimore). 2016 Feb;95(6):e2710. [DOI:10.1097/MD.0000000000002710] [PMID] []
8. Hacıhamdioğlu B, Baş EG, Delil K. Homozygous Mutation in the Insulin Receptor Gene Associated with Mild Type A Insulin Resistance Syndrome: A Case Report. J Clin Res Pediatr Endocrinol. 2020 Feb 5;13(1):100-3. [DOI:10.4274/jcrpe.galenos.2020.2019.0213] [PMID] []
9. Huggard D, Stack T, Satas S, Gorman CO. Donohue syndrome and use of continuous subcutaneous insulin pump therapy. BMJ Case Rep. 2015 Oct 27;2015:bcr2015210019. [DOI:10.1136/bcr-2015-210019] [PMID] []
10. Adams JM, Gordon LP, Dutton RV, Rosenberg HS, Rudolph AJ. Leprechaunism (Donohue's syndrome) in a low birth weight infant. South Med J. 1977 Aug;70(8):998-1001. [DOI:10.1097/00007611-197708000-00031] [PMID]
11. He L, Que G, Yang X, Yan S, Luo S. Prevalence, clinical characteristics, and 3-dimensional radiographic analysis of supernumerary teeth in Guangzhou, China: a retrospective study. BMC Oral Health. 2023 Jun 2;23(1):351. [DOI:10.1186/s12903-023-03032-9] [PMID] []
12. Zhao L, Liu S, Zhang R, Yang R, Zhang K, Xie X. Analysis of the distribution of supernumerary teeth and the characteristics of mesiodens in Bengbu, China: a retrospective study. Oral Radiol. 2021 Apr;37(2):218-23. [DOI:10.1007/s11282-020-00432-3] [PMID]
13. Hamada A, Mukasa H, Taguchi Y, Akagi E, Obayashi F, Yamasaki S, Kanda T, Koizumi K, Toratani S, Okamoto T. Identification of a familial cleidocranial dysplasia with a novel RUNX2 mutation and establishment of patient-derived induced pluripotent stem cells. Odontology. 2022 Jul;110(3):444-51. [DOI:10.1007/s10266-021-00674-5] [PMID] []
14. Kim Y, Jeong T, Kim J, Shin J, Kim S. Effects of mesiodens on adjacent permanent teeth: a retrospective study in Korean children based on cone-beam computed tomography. Int J Paediatr Dent. 2018 Mar;28(2):161-9. [DOI:10.1111/ipd.12317] [PMID]
15. Jo C, Bae D, Choi B, Kim J. Removal of Supernumerary Teeth Utilizing a Computer-Aided Design/Computer-Aided Manufacturing Surgical Guide. J Oral Maxillofac Surg. 2017 May;75(5):924.e1-924.e9. [DOI:10.1016/j.joms.2016.11.002] [PMID]
16. Pescia R, Kiliaridis S, Antonarakis GS. Spontaneous eruption of impacted maxillary incisors after surgical extraction of supernumerary teeth: a systematic review and meta-analysis. Clin Oral Investig. 2020 Nov;24(11):3749-59. [DOI:10.1007/s00784-020-03369-3] [PMID]
17. Küchler EC, Risso PA, Costa Mde C, Modesto A, Vieira AR. Studies of dental anomalies in a large group of school children. Arch Oral Biol. 2008 Oct;53(10):941-6. [DOI:10.1016/j.archoralbio.2008.04.003] [PMID]
18. Acharya S, Kumar Mandal P, Ghosh C. Bilateral molariform mandibular second premolars. Case Rep Dent. 2015;2015:809463. [DOI:10.1155/2015/809463] [PMID] []
19. Dugmore CR. Bilateral macrodontia of mandibular second premolars: a case report. Int J Paediatr Dent. 2001 Jan;11(1):69-73. [DOI:10.1046/j.1365-263x.2001.00215.x] [PMID]
20. Pace A, Sandler PJ, Murray A. Macrodont management. Dent Update. 2013 Jan-Feb;40(1):18-20, 23-6. [DOI:10.12968/denu.2013.40.1.18] [PMID]
21. Robertson S. FLNB Disorders. 2008 Oct 9 [updated 2020 Feb 13]. In: Adam MP, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2023.
22. Saleh D, Yarrarapu SNS, Cook C. Hypertrichosis. StatPearls (Internet): StatPearls Publishing; 2022.
23. Khalil R. Ubiquitin-Proteasome Pathway and Muscle Atrophy. Adv Exp Med Biol. 2018;1088:235-48. [DOI:10.1007/978-981-13-1435-3_10] [PMID]
24. Jones ES, Gibson JAG, Dobbs TD, Whitaker IS. The psychological, social and educational impact of prominent ears: A systematic review. J Plast Reconstr Aesthet Surg. 2020 Dec;73(12):2111-20. [DOI:10.1016/j.bjps.2020.05.075] [PMID]
25. Segura-Egea JJ, Cabanillas-Balsera D, Martín-González J, Cintra LTA. Impact of systemic health on treatment outcomes in endodontics. Int Endod J. 2023 Mar;56 Suppl 2:219-35. [DOI:10.1111/iej.13789] [PMID]

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