Article Menu

Export Article

More by Authors Links

Article Data

  • Views 1029
  • Dowloads 162

Original Research

Open Access

Wolf-Hirschhorn Syndrome; Oro-Dental Manifestations and Management

  • Roberts T1
  • Stephen LXG1
  • Fieggen K1
  • Beighton P1,*,

1Faculty of Dentistry, University of the Western Cape, Mitchell’s Plain, Cape Town Special Dental Genetic Clinic, Red Cross Memorial Children’s Hospital, Cape Town

DOI: 10.17796/jcpd.34.2.966jw1w227234756 Vol.34,Issue 2,March 2010 pp.173-176

Published: 01 March 2010

*Corresponding Author(s): Beighton P E-mail: Peter.Beighton@uct.ac.za

PDF (141.68 kB) View Full-text

Abstract

The major manifestations of the Wolf-Hirschhorn syndrome are developmental delay, short stature, mental impairment and epilepsy. Clefts of the lip and palate are sometimes present. Dental problems which are overshadowed by the major syndromic manifestations warrant appropriate management.

We have documented an affected South African boy, discussed his dental management and reviewed the oro-dental implications of the disorder.

Keywords

Wolf-Hirschhorn, Syndrome, Cytogenetic,Dental,Genetic, Malformation

Cite and Share

Roberts T,Stephen LXG,Fieggen K,Beighton P. Wolf-Hirschhorn Syndrome; Oro-Dental Manifestations and Management. Journal of Clinical Pediatric Dentistry. 2010. 34(2);173-176.

URL:

https://www.jocpd.com/articles/10.17796/jcpd.34.2.966jw1w227234756

References

1. Burgersdijk R, Tan HL. Oral symptoms of the Wolf syndrome. J Dent Chil, 45: 488–489, 1978.

2. Johnston NJ, Franklin DL. Dental findings of a child with Wolf-Hirschhorn syndrome. Int J Paed Dent, 16(2): 139–142, 2006

3. Breen GH. Taurodontism, an unreported dental finding in Wolf-Hirschhorn (4p-) syndrome. ASDC J Dent Child, 65(5): 344–345, 356, 1998.

4. Hirschhorn K, Cooper HL. Apparent deletion of short arms of one chromosome (4 or 5) in a child with defects of midline fusion. Human Chromosome Newsl, 4:14, 1961.

5. Wolf U, Porsch R, Baitsch H, Reinwein H. Deletion of short arms of a B- chromosome without “cri-du-chat” syndrome. Lancet, 3: 769, 1965.

6. Shannon NL, Maltby EL, Rigby AS, Quarrell OWJ. An epidemiological study of Wolf-Hirschhorn syndrome: life expectancy and cause of mortality. J Med Genet, 38: 674–679, 2001.

7. Wright TJ, Ricke DO, Denison K, Abmayr S, Cotter PD, Hirschhorn K, Keinanen M, McDonald-McGinn D, Somer M, Spinner N, Yaang-Feng T, Zackai E, Altherr MR. A transcript map of the newly defined 165 kb Wolf-Hirschhorn syndrome critical region. Hum Molec Genet, 6; 317–324, 1997.

8. Zollino M, Di Stefano C, Zampino G, Mastroiacovo P, Wright TJ, Sorge G, Selicorni A, Tenconi R, Zappala A, Battaglia A, Di Rocco M, Palka G, Pallotta R, Altherr MR, Neri G. Genotype-phenotype correlations and clinical diagnostic criteria in Wolf-Hirschhorn syndrome. Am J Med Genet, 94: 254–261, 2000.

9. Nieminen P, Kotilainen J, Aalto Y, Knuutila S, Pirinen S, Thesleff I. MSX1 gene is deleted in Wolf-Hirschhorn syndrome patients with oligodontia. J Dent Res, 82: 1013–1017, 2003.

10. Van Buggenhout G, Melotte C, Dutta B, Froyen G, Van Hummelen P, Marynen P, Matthijs G, de Ravel T, Devriendt K, Fryns JP, Vermeesch JR. Mild Wolf-Hirshhorn syndrome: micro-array CGH analysis of atypical 4p16.3 deletion enables refinement of the genotype-phenotype map. J Med Genet, 41: 691–698, 2004.

11. Babich SB, Banducci S, Teplitsky P. Dental characteristics of the Wolf-Hirschhorn syndrome: a case report. Spec Care Dentist, 24(4): 229–231, 2004.

12. Battaglia A Filippi T, Carey JC. Update on the clinical features and natural history of Wolf-Hirschhorn (4p-) syndrome: experience with 87 patients and recommendations for routine health supervision. Am J Med Genet C Semin Med Genet, 148C(4): 246–251, 2008.

13. Zollino M, Murdolo M, Marangi G, Pecile V, Galasso C, Mazzanti L, Neri G. On the Nosology and pathogenesis of Wolf-Hirschhorn syndrome: genotype-phenotype correlation analysis of 80 patients and literature review. Am J Med Genet C Semin Med Genet, 148C(4): 257–269, 2008.

14. Iwanowski PS, Stengel-Rutkowski S, Anderlik L, Pilch J, Midro AT. Physical and developmental phenotype analyses in a boy with Wolf-Hirschhorn syndrome. Genet Couns, 16(1): 31–40, 2005.

Abstracted / indexed in

Science Citation Index Expanded (SciSearch) Created as SCI in 1964, Science Citation Index Expanded now indexes over 9,500 of the world’s most impactful journals across 178 scientific disciplines. More than 53 million records and 1.18 billion cited references date back from 1900 to present.

Biological Abstracts Easily discover critical journal coverage of the life sciences with Biological Abstracts, produced by the Web of Science Group, with topics ranging from botany to microbiology to pharmacology. Including BIOSIS indexing and MeSH terms, specialized indexing in Biological Abstracts helps you to discover more accurate, context-sensitive results.

Google Scholar Google Scholar is a freely accessible web search engine that indexes the full text or metadata of scholarly literature across an array of publishing formats and disciplines.

JournalSeek Genamics JournalSeek is the largest completely categorized database of freely available journal information available on the internet. The database presently contains 39226 titles. Journal information includes the description (aims and scope), journal abbreviation, journal homepage link, subject category and ISSN.

Current Contents - Clinical Medicine Current Contents - Clinical Medicine provides easy access to complete tables of contents, abstracts, bibliographic information and all other significant items in recently published issues from over 1,000 leading journals in clinical medicine.

BIOSIS Previews BIOSIS Previews is an English-language, bibliographic database service, with abstracts and citation indexing. It is part of Clarivate Analytics Web of Science suite. BIOSIS Previews indexes data from 1926 to the present.

Journal Citation Reports/Science Edition Journal Citation Reports/Science Edition aims to evaluate a journal’s value from multiple perspectives including the journal impact factor, descriptive data about a journal’s open access content as well as contributing authors, and provide readers a transparent and publisher-neutral data & statistics information about the journal.

Scopus: CiteScore 1.8 (2023) Scopus is Elsevier's abstract and citation database launched in 2004. Scopus covers nearly 36,377 titles (22,794 active titles and 13,583 Inactive titles) from approximately 11,678 publishers, of which 34,346 are peer-reviewed journals in top-level subject fields: life sciences, social sciences, physical sciences and health sciences.

Submission Turnaround Time

Conferences

Top