[Home ] [Archive]    
:: Main :: About :: Current Issue :: Archive :: Search :: Submit :: Contact ::
:: Volume 3, Issue 1 (Journal of Research in Dental & Maxillofacial Sciences winter 2018) ::
J Res Dentomaxillofac Sci 2018, 3(1): 34-38 Back to browse issues page
Hyper-IgM Immunodeficiency with Enamel Defects: a Case Report
N Rahmani *1, K Salem2, F Moazemi3
1- Postgraduate Student Pediatric Dentistry Dept,Dental Branch of Tehran, Islamic Azad University , Tehran, Iran , nastaran67rah@gmail.com
2- Assistant prof, Pediatric Dept, Dental Branch of Tehran, Islamic Azad University, Tehran, Iran., Dental Branch of Tehran, Islamic Azad University , Tehran, Iran
3- Dentist, Dental Branch of Tehran, Islamic Azad University , Tehran, Iran
Abstract:   (763 Views)
Background: Hyper-Immunoglobulin M (IgM) syndrome (HIGM) is a rare primary immunodeficiency in which defective B-cell isotype switching results in a phenotype characterized by elevated or normal serum IgM levels and low levels of other Ig classes, leading to an increased susceptibility to infection, neutropenia, autoimmune disorders, and malignancies. In this disease, a mutation occurs in the CD40 gene, leading to combined B-cell and T-cell immunodeficiencies. The oral manifestations include wound-like lesions, oral candidiasis, gingivitis, periodontitis, and enamel defects. Theoretically, systemic conditions affecting ameloblastic activity during enamel mineralization, such as abnormal oxygen levels resulting from hypoventilation in various respiratory diseases, result in enamel defects.
Case presentation: We report a 10-year-old male with hyper-IgM immunodeficiency. The patient had suffered from frequent infections, respiratory problems, and bronchopneumonia from the age of 2 years. At 4 years of age, type 1 diabetes mellitus was diagnosed. During dental examinations, enamel defects were found in seven permanent teeth.
Conclusion: A meticulous dental evaluation of children with systemic diseases is mandatory in order to discover possible developmental dental defects and to plan early interventions.
 
Keywords: Hyper-IgM Immunodeficiency Syndrome, Immunoglobulins, Enamel Hypoplasia
Full-Text [PDF 141 kb]   (195 Downloads) |   |   Full-Text (HTML)  (42 Views)  
Type of Study: Case report |
* Corresponding Author Address: Dental Branch of Tehran, Islamic Azad University , Tehran, Iran
References
1. Notarangelo LD, Duse M, Ugazio AG. Immunodeficiency with hyper IgM (HIM). Immunodefic Rev. 1992; 3:101–121.
2. Mayer L, Kwan SP, Thompson C, etc. 1986 Evidence for a defect in “switch” T cells in patients with immunodeficiency and hyperimmunoglobulinemia M. N Engl J Med 314:409–413
3. Subauste CS, Wessendarp M, Sorensen RU, Leiva LE: CD40-CD40 ligand interaction is central to cell-mediated immunity against Toxoplasma gondii: patients with hyper IgM syndrome have a defective type 1 immune response that can be restored by soluble CD40 ligand trimer. J Immunol. 1999, 162: 6690-6700..
4. ACKERMAN BD. DYSGAMMAGLOBULINEMIA: REPORT OF A CASE WITH A FAMILY HISTORY OF A CONGENITAL GAMMA GLOBULIN DISORDER. Pediatrics. 1964 Aug;34:211–219.
5. Winfield JB, Cohen PL, Bradley L, Finkelman FD, Eisenberg RA, Wistar R, Jr, Whisnant JK. IgM cryoprecipitation and anti-immunoglobulin activity in dysgammaglobulinemia type I. Clin Immunol Immunopathol. 1982 Apr;23(1):58–69. [PubMed]
6. Brahmi Z, Lazarus KH, Hodes ME, Baehner RL. Immunologic studies of three family members with the immunodeficiency with hyper-IgM syndrome. J Clin Immunol. 1983 Apr;3(2):127–134.
7. Slepian IK, Schwartz SA, Weiss JJ, Roth SL, Mathews KP. Immunodeficiency with hyper IgM after systemic lupus erythematosus. J Allergy Clin Immunol. 1984 Jun;73(6):846–857
8. Notarangelo LD, Peitsch MC 1996 CD40lbase: a database of CD40L gene mutations causing X-linked hyper-IgM syndrome. Immunol Today 17:511–516
9. Winkelstein JA, Marino MC, Ochs H, Fuleihan R, Scholl PR, Geha R, Stiehm ER, Conley ME 2003 The X-linked hyper IgM syndrome: clinical and immunologic features of 79 patients. Medicine (Baltimore) 82:373–384
10. Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, Resnick I, Fasth A, Baer M, Gomez L, Sanders EA, Tabone MD, Plantaz D, Etzioni A, Monafo V, Abinun M, Hammarstrom L, Abrabamsen T, Jones A, Finn A, Klemola T, DeVries E, Sanal O, Peitsch MC, Notarangelo LD 1997 Clinical spectrum of X-linked hyper-IgM syndrome. J Pediatr 131:47–54
11. Aghamohammadi A, Moin M, Karimi A, Naraghi M, Zandieh F, Isaeian A, et al. Immunologic evaluation of patients with recurrent ear, nose, and throat infections. Am J Otolaryngol 2008; 29(6):385-92.
12. Engstrom GN, Engstrom PE, Hammarstrom L, Smith CI. Oral conditions in individuals with selective immunoglobulin A deficiency and common variable immunodeficiency. J Periodontol 1992; 63(12):984-9.
13. Sicher, Harry: Orban’s Oral Histology and Embryology, 6th ed., St. Louis: C. V. Mosby Company, 1966.
14. Spouge, J. D.: Oral Pathology, Chapter 6, St. Louis: C. V. Mosby Company, 1973.
15. Sarnat, B. G. and Schour, Isaac: Enamel hypoplasia (chronic enamel aplasia) in relation to systemic disease: a chronologic, morphologic and etiologic classification, J Am Dent Assoc, 28: 1,989-2,000, 1941; 29:66-75, 1942.
16. [No authors listed]. An epidemiological index of developmental defects of dental enamel (DDE Index). Commission on Oral Health, Research and Epidemiology. Int Dent J. 1982; 32: 159-167.
17. [No authors listed]. A review of the developmental defects of enamel index (DDE Index). Commission on Oral Health, Research & Epidemiology. Report of an FDI Working Group. Int Dent J. 1992; 42: 411-426.
18. Suckling G. Defects of enamel in sheep resulting from trauma during tooth development. J Dent Res. 1980; 59: 1541-1548.
19. Suckling GW, Purdell-Lewis DJ. The pattern of mineralization of traumatically-induced developmental defects of sheep enamel assessed by microhardness and microradiography. J Dent Res. 1982; 61: 1211-1216.
20. Suckling G, Elliott DC, Thurley DC. The production of developmental defects of enamel in the incisor teeth of penned sheep resulting from induced parasitism. Arch Oral Biol. 1983; 28: 393-399.
21. Suckling GW, Thurley DC, Nelson NGA. The macroscopic and scanning electron microscopic appearance and microhardness of the enamel, and the related histological changes in the enamel organ of erupting sheep incisors resulting from a prolonged low daily dose of fluoride. Arch Oral Biol. 1988; 33: 361-373.
22. Den Besten PK. Effects of fluoride on protein secretion and removal during enamel development in the rat. J Dent Res. 1986; 65: 1272-1277.
23. Sarnat BG, Schour I. Enamel hypoplasia (chronologic enamel aplasia) in relation to systemic disease: a chronologic, morphologic and etiologic classification (part I). J Am Dent Assoc. 1941; 28: 1989-2000.
24. Sarnat BG, Schour I. Enamel hypoplasia (chronologic enamel aplasia) in relation to systemic disease: a chronologic, morphologic and etiologic classification (part II). J Am Dent Assoc. 1942; 29: 67-75.
25. Holan G, Topf J, Fuks AB. Effect of root canal infection and treatment of traumatized primary incisors on their permanent successors. Endod Dent Traumatol. 1992; 8: 12-15.
26. Lo EC, Zheng CG, King NM. Relationship between the presence of demarcated opacities and hypoplasia in permanent teeth and caries in their primary predecessors. Caries Res. 2003; 37: 456-461.
27. Pajari U, Lanning M, Larmas M. Prevalence and location of enamel opacities in children after anti-neoplastic therapy. Community Dent Oral Epidemiol. 1988; 16: 222-226.
28. Churchill HV. Occurrence of fluorides in some waters of the United States. IndEng Chem. 1931; 23: 996-998.
29. Alaluusua S, Lukinmaa PL, Koskimies M, Pirinen S, Hölttä P, Kallio M, et al. Developmental dental defects associated with long breast feeding. Eur J Oral Sci. 1996; 104: 493-497.
30. Lai PY, Seow WK, Tudehope DI, Rogers Y. Enamel hypoplasia and dental caries in very-low birthweight children: a case-controlled, longitudinal study. Pediatr Dent. 1997; 19: 42-49.
31. Marshall JA. Dental hypoplasia: its occurrence, histopathology and aetiology. J Am Dent Assoc. 1936; 23: 2074-2082.
32. Bousfiha A, Jeddane L, Al-Herz W, Ailal F, Casanova JL, Chatila T, et al. The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies. J Clin Immunol . 2015 Nov. 35 (8):727-38.
33. Armitage RJ, Fanslow WC, Strockbine L, Sato TA, Clifford KN, Macduff BM, Anderson DM, Gimpel SD, Davis-Smith T, Maliszewski CR, et al. Molecular and biological characterization of a murine ligand for CD40. Nature. 1992 May 7;357(6373):80–82.
34. Hollenbaugh D, Grosmaire LS, Kullas CD, Chalupny NJ, Braesch-Andersen S, Noelle RJ, Stamenkovic I, Ledbetter JA, Aruffo A. The human T cell antigen gp39, a member of the TNF gene family, is a ligand for the CD40 receptor: expression of a soluble form of gp39 with B cell co-stimulatory activity. EMBO J. 1992 Dec;11(12):4313–4321.
35. Armitage RJ, Sato TA, Macduff BM, Clifford KN, Alpert AR, Smith CA, Fanslow WC. Identification of a source of biologically active CD40 ligand. Eur J Immunol. 1992 Aug;22(8):2071–2076.
36. Lane P, Traunecker A, Hubele S, Inui S, Lanzavecchia A, Gray D. Activated human T cells express a ligand for the human B cell-associated antigen CD40 which participates in T cell-dependent activation of B lymphocytes. Eur J Immunol. 1992 Oct;22(10):2573–2578.
37. Lee WI, Torgerson TR, Schumacher MJ, Yel L, Zhu Q, Ochs HD. Molecular analysis of a large cohort of patients with the hyper immunoglobulin M (IgM) syndrome. Blood. 2005;105:1881–90.
38. Hayward AR, Levy J, Facchetti F, Notarangelo L, Ochs HD, Etzioni A, Bonnefoy JY, Cosyns M, Weinberg A. Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency lwith hyper-IgM. J Immunol. 1997;158:977–83.
39. Jesus AA, Duarte AJ, Oliveira JB. Autoimmunity in hyper-IgM syndrome. J Clin Immunol. 2008;28 Suppl 1:S62–6/
40. Bussone G, Mouthon L. Autoimmune manifestations in primary immune deficiencies. Autoimmun Rev. 2009;8:332–6.
41. Eren M, Saltik-Temizel IN, Yüce A, et al. Duodenal appearance of giardiasis in a child with selective immunoglobulin A deficiency. Pediatr Int. 2007;49(409)
42. Korponay-Szabó IR, Dahlbom I, Laurila K, et al. Elevation of IgG antibodies against tissue transglutaminase as a diagnostic tool for coeliac disease in selective IgA deficiency. Gut. 2003;52(1567)
43. Kumar V, Jarzabek-Chorzelska M, Sulej J, et al. Celiac disease and immunoglobulin A deficiency: how effective are the serological methods of diagnosis? Clin Diagn Lab Immunol. 2002;9(1295)
44. William T. Shearer, Thomas A. Fleisher, Rebecca H. Buckley, et al. Recommendations for live viral and bacterial vaccines in immunodeficient patients and their close-contacts. J Allergy Clin Immunol. 2014 Apr; 133(4): 961–966
45. Moss-Salentijn L, Hendricks-Klyvert M. Dental and oral tissue: an introduction. 2nd edition. Lea &Febiger:Philadelphia. 1990.
46. Fearnhead RW, Kawasaki K, Inoue K. Comments on the porosity of human tooth enamel. J Dent Res. 1982; Spec No: 1524-1531.
47. Robinson C, Kirkham J, Briggs HD, Atkinson PJ. Enamel proteins: from secretion to maturation. J Dent Res. 1982; Spec No: 1490-1495.
48. Fejerskov O, Josephen K. In: Human oral embryology and histology. Mjör IA, Fejerskov O, editors. Munksgaard: Copenhagen. 1986: 31-49.
49. Weerheijm KL, Duggal M, Mejare I, etc. Judgement criteria for molar incisor hypomineralisation (MiH) in epidemiologic studies: a summary of the European meeting on MiH held in athens, 2003. Eur. Paediatr. dent. 2003; 4, 110–113.
50. Wong HM, McGrath C, King NM. Dental practitioners' views on the need to treat developmental defects of enamel. Community Dent Oral Epidemiol. 2007; 35: 130-139.
51. Jälevik B, Klingberg GA. Dental treatment, dental fear and behaviour management problems in children with severe enamel hypomineralization of their permanent first molars. Int J Paediatr Dent. 2002; 12: 24-32.
Send email to the article author

Add your comments about this article
Your username or Email:

CAPTCHA



XML     Print


Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

Rahmani N, Salem K, Moazemi F. Hyper-IgM Immunodeficiency with Enamel Defects: a Case Report. J Res Dentomaxillofac Sci. 2018; 3 (1) :34-38
URL: http://jrdms.dentaliau.ac.ir/article-1-170-en.html


Volume 3, Issue 1 (Journal of Research in Dental & Maxillofacial Sciences winter 2018) Back to browse issues page
Journal of Research in Dental and Maxillofacial Sciences
Persian site map - English site map - Created in 0.05 seconds with 30 queries by YEKTAWEB 3921