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      Epidemiology and clinical profile of individuals with cleft lip and palate utilising specialised academic treatment centres in South Africa

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          Abstract

          Objective

          The study was conducted to determine the epidemiology and clinical profile of individuals with cleft lip and/or palate (CLP) utilizing specialized academic treatment centres in South Africa’s public health sector.

          Materials and methods

          The Human Research Ethics Committee of the University of the Witwatersrand in Johannesburg provided ethical approval for the study. We conducted a retrospective record review of all cases of CLP treated at the specialised academic centres for the two-year period from 1 January 2013 until 31 December 2014. We used a structured, pre-tested record review form to obtain demographic, clinical and treatment information on each CLP case. We used Stata 13 to analyse the data and conducted statistical tests at 5% significance level.

          Results

          We analysed 699 records of individuals with CLP. The estimated prevalence of CLP in the South African public health sector was 0.3 per 1000 live births, with provincial variation of 0.1/1000 to 1.2/1000. The distribution of clefts was: 35.3% cleft palate; 34.6% cleft lip and palate; 19.0% cleft lip and other cleft anomalies at 2%. Of the total number of CLP, 47.5% were male and 52.5% female, and this difference was statistically significant (p<0.001). The majority of clefts occurred on the left for males (35.5%) and palate for females (43.4%), with a male predominance of unilateral cleft lip and palate (53.3%).

          Conclusion

          The study findings should inform the implementation of South Africa’s planned birth defect surveillance system and health service planning for individuals with CLP.

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          Most cited references54

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          The psychosocial effects of cleft lip and palate: a systematic review.

          This systematic review examined the published scientific research on the psychosocial impact of cleft lip and palate (CLP) among children and adults. The primary objective of the review was to determine whether having CLP places an individual at greater risk of psychosocial problems. Studies that examined the psychosocial functioning of children and adults with repaired non-syndromal CLP were suitable for inclusion. The following sources were searched: Medline (January 1966-December 2003), CINAHL (January 1982-December 2003), Web of Science (January 1981-December 2003), PsycINFO (January 1887-December 2003), the reference section of relevant articles, and hand searches of relevant journals. There were 652 abstracts initially identified through database and other searches. On closer examination of these, only 117 appeared to meet the inclusion criteria. The full text of these papers was examined, with only 64 articles finally identified as suitable for inclusion in the review. Thirty of the 64 studies included a control group. The studies were longitudinal, cross-sectional, or retrospective in nature.Overall, the majority of children and adults with CLP do not appear to experience major psychosocial problems, although some specific problems may arise. For example, difficulties have been reported in relation to behavioural problems, satisfaction with facial appearance, depression, and anxiety. A few differences between cleft types have been found in relation to self-concept, satisfaction with facial appearance, depression, attachment, learning problems, and interpersonal relationships. With a few exceptions, the age of the individual with CLP does not appear to influence the occurrence or severity of psychosocial problems. However, the studies lack the uniformity and consistency required to adequately summarize the psychosocial problems resulting from CLP.
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            Incidence of cleft lip, cleft palate, and cleft lip and palate among races: a review.

            A review of the literature pertaining to the incidence of cleft lip, cleft palate, and cleft lip and palate in different races is presented. The studies have been evaluated according to the method used to record the incidence rate. Half of the studies include in their base population livebirths, stillbirths, and abortions, or livebirths and stillbirths to record the incidence rate. In addition, in most of the studies, clefts with associated malformations and possible syndromes are included in the reported incidence. There is evidence, however, to suggest that the risk of developing clefts in stillbirths and abortions is three times as frequent as in livebirths and that clefts with associated malformations behave differently epidemiologically from clefts without associated malformations. It is suggested, therefore, that the incidence of cleft lip, cleft palate, and cleft lip and palate should be studied separately for each group, namely for livebirths, stillbirths, and abortions and should be reported separately for clefts without associated malformations, clefts with associated malformations, and syndromes. More research is needed to study the risk of developing clefts among the various groups that exhibit different epidemiologic behavior for each race.
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              Prevalence at birth of cleft lip with or without cleft palate: data from the International Perinatal Database of Typical Oral Clefts (IPDTOC).

              (2010)
              As part of a collaborative project on the epidemiology of craniofacial anomalies, funded by the National Institutes for Dental and Craniofacial Research and channeled through the Human Genetics Programme of the World Health Organization, the International Perinatal Database of Typical Orofacial Clefts (IPDTOC) was established in 2003. IPDTOC is collecting case-by-case information on cleft lip with or without cleft palate and on cleft palate alone from birth defects registries contributing to at least one of three collaborative organizations: European Surveillance Systems of Congenital Anomalies (EUROCAT) in Europe, National Birth Defects Prevention Network (NBDPN) in the United States, and International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) worldwide. Analysis of the collected information is performed centrally at the ICBDSR Centre in Rome, Italy, to maximize the comparability of results. The present paper, the first of a series, reports data on the prevalence of cleft lip with or without cleft palate from 54 registries in 30 countries over at least 1 complete year during the period 2000 to 2005. Thus, the denominator comprises more than 7.5 million births. A total of 7704 cases of cleft lip with or without cleft palate (7141 livebirths, 237 stillbirths, 301 terminations of pregnancy, and 25 with pregnancy outcome unknown) were available. The overall prevalence of cleft lip with or without cleft palate was 9.92 per 10,000. The prevalence of cleft lip was 3.28 per 10,000, and that of cleft lip and palate was 6.64 per 10,000. There were 5918 cases (76.8%) that were isolated, 1224 (15.9%) had malformations in other systems, and 562 (7.3%) occurred as part of recognized syndromes. Cases with greater dysmorphological severity of cleft lip with or without cleft palate were more likely to include malformations of other systems.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: Funding acquisitionRole: InvestigationRole: MethodologyRole: Project administrationRole: ResourcesRole: SoftwareRole: ValidationRole: VisualizationRole: Writing – original draftRole: Writing – review & editing
                Role: Formal analysisRole: Writing – review & editing
                Role: ConceptualizationRole: Formal analysisRole: SupervisionRole: Writing – review & editing
                Role: Editor
                Journal
                PLoS One
                PLoS ONE
                plos
                plosone
                PLoS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                9 May 2019
                2019
                : 14
                : 5
                : e0215931
                Affiliations
                [1 ] School of Oral Health Sciences, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg
                [2 ] School of Public Health, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg
                [3 ] Centre for Health Policy & DST/NRF SARChI Chair, School of Public Health, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg
                Agha Khan University, UNITED REPUBLIC OF TANZANIA
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Author information
                http://orcid.org/0000-0002-8052-9275
                Article
                PONE-D-18-36970
                10.1371/journal.pone.0215931
                6508722
                31071123
                2a0ef43a-b117-4906-8dde-87ef068d0781
                © 2019 Hlongwa et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 28 December 2018
                : 10 April 2019
                Page count
                Figures: 1, Tables: 4, Pages: 14
                Funding
                The project was funded by Wits Faculty of Health Sciences Research Office ( www.wits.ac.za); South African Society of Orthodontists Gerald Gavron Fund ( www.saso.co.za) and The South African Dental Association Dentistry Developmental Foundation of SA Trust ( www.sada.co.za) to PH. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Research Article
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Cleft Lip and Palate
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Cleft Lip and Palate
                Medicine and Health Sciences
                Otorhinolaryngology
                Cleft Lip and Palate
                People and places
                Geographical locations
                Africa
                South Africa
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Cleft Lip and Palate
                Cleft Palate
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Cleft Lip and Palate
                Cleft Palate
                Medicine and Health Sciences
                Otorhinolaryngology
                Cleft Lip and Palate
                Cleft Palate
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Cleft Lip and Palate
                Cleft Lip
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Cleft Lip and Palate
                Cleft Lip
                Medicine and Health Sciences
                Otorhinolaryngology
                Cleft Lip and Palate
                Cleft Lip
                Medicine and Health Sciences
                Public and Occupational Health
                Medicine and Health Sciences
                Congenital Disorders
                Congenital Anomalies
                Medicine and Health Sciences
                Epidemiology
                Medicine and Health Sciences
                Congenital Disorders
                Birth Defects
                Biology and Life Sciences
                Developmental Biology
                Morphogenesis
                Birth Defects
                Custom metadata
                All relevant data are within the paper and its Supporting Information files.

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