Displaying publications 1 - 20 of 80 in total

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  1. Salahshourifar I, Sulaiman WA, Zilfalil BA, Halim AS
    Am J Med Genet A, 2011 Sep;155A(9):2302-7.
    PMID: 21834040 DOI: 10.1002/ajmg.a.34169
    Several studies have shown evidence for the contribution of interferon regulatory factor 6 (IRF6) variants to the risk of nonsyndromic oral clefts in Asians; however, this has not included the Malay population. The current study attempts to address this research gap using allele and haplotype transmission disequilibrium analyses. The results showed a strong transmission distortion for multiple haplotypes to patients with nonsyndromic cleft lip with or without cleft palate. Haplotypes carrying the 243 bp allele of D1S2136 and common alleles at the rs861019 and rs2235371 were over-transmitted to patients. By contrast, haplotypes consisting of the 251 bp allele of D1S2136 and the rare allele at rs2235371 were more under-transmitted. Furthermore, several variants and haplotypes showed excess maternal transmission, but none of them attained statistical significance in maternal relative risk analyses. In contrast, a significant child genotype effect was observed for several haplotypes, indicating fetal genotype could be the major genetic contribution rather than maternal genotype. The present study therefore further supports a role for IRF6 variants in clefting in this Southeast Asian population. Overall, Asian genetic backgrounds are most likely more susceptible to the haploinsufficiency of IRF6 variants. These variants may contribute to the condition either themselves, or they may be in linkage disequilibrium with other casual variants.
    Matched MeSH terms: Cleft Lip/genetics*; Cleft Lip/epidemiology
  2. Salahshourifar I, Halim AS, Sulaiman WA, Zilfalil BA
    Am J Med Genet A, 2010 Jul;152A(7):1818-21.
    PMID: 20583164 DOI: 10.1002/ajmg.a.33526
    We describe a chromosome 6 uniparental disomy (UPD6) in a boy, discovered during a screening for the genetic cause of cleft lip and palate. In the medical literature, almost all documented cases of UPD6 are paternal in origin, and only four were maternal. We present here a report of complete maternal chromosome 6 uniparental heterodisomy. Haplotype analysis was performed using highly polymorphic short tandem repeat (STR) markers that span both arms of chromosome 6. Analysis of these markers revealed the presence of two maternal alleles but no paternal allele, indicating an instance of maternal uniparental heterodisomy. Chromosome analysis of peripheral blood lymphocytes confirmed a normal male karyotype. Advanced maternal age at the time of the infant's birth and heterodisomy of markers around the centromere favors a meiosis-I error. No specific phenotype has been reported for maternal UPD6. Therefore, the cleft lip and palate in the present case probably occurred due to other risk factors. This report provides further evidence that maternal UPD6 has no specific clinical consequences and adds to the collective knowledge of this rare chromosomal finding.
    Matched MeSH terms: Cleft Lip/genetics*
  3. Ghani, S.H.A., Hussain, R., Hassan, S., Tan, K.K., Ahmad, M.H.
    Ann Dent, 1996;3(1):-.
    MyJurnal
    The Combined Cleft Clinic at the University Hospital, Kuala Lumpur was organised in 1992. The team consists of Plastic Surgeon, Orthodontists, Speech therapist, Ear, Nose and Throat Surgeon, Audiologist, medical officers and the nurses. We attend the clinic on a regular basis. Specialists from other medical and dental disciplines as well as the members of the Cleft Lip and Palate Association of Malaysia (CLAPAM) do occasionally participate in this set-up.The team members formulate treatment plans for each cleft patient, monitor the patient's growth and development and manage the patient at different stages according to the individual needs. To date, the idea of team approach and an establishment of a centre towards management of cleft patients seem to be the most ideal as the patients are benefiting total treatment and care from various specialists from only one place. The experience of University Hospital as a centre for cleft patients is discussed.
    Matched MeSH terms: Cleft Lip
  4. Abdul Rahman, Z.A.
    Ann Dent, 2004;11(1):-.
    MyJurnal
    The standard procedure for alveolar cleft closure in cleft lip and palate patients is by alveolar bone grafting (ABG) where the residual opening of oronasal fistula in the palate is also closed simultaneously. Occasionally there is a situation of soft tissue inadequacy and attempt to close the fistula at the same procedure as ABG may compromise the result of the bone graft itself. An unforeseen leakage at the closure may cause graft infection leading to failure. This article reports on a technique where alveolar bone grafting was done without closure of residual palatal fistula to provide adequate soft tissue coverage for the grafted bone. This may reduce the risk of leakage at the oral mucosal coverage of the graft . The residual palatal fistula will be closed at later stage by muco-palatal flap or tongue flap. We reported on a case of a failed ABG which was reconstructed using this technique. Various other techniques to ensure success of ABG were also discussed. Conclusion: The alveolar bone grafting without closure of oro-nasal fistula reduced the risk of failure of ABG in a wide alveolar cleft.
    Matched MeSH terms: Cleft Lip
  5. Abdullah S
    Ann Acad Med Singap, 1988 Jul;17(3):388-91.
    PMID: 3218930
    The results of speech, language and hearing assessment of repaired cleft palate children and adults over a 4-year period at the Speech/Language therapy unit, Department of ENT, Faculty of Medicine, UKM are reported. The subjects were 33 incomplete cleft lip palate (ICLP), 48 unilateral complete cleft lip and palate (UCLP) and 26 bilateral complete cleft lip and palate (BCLP). Results obtained for the following assessments are reported and discussed: (i) Hearing assessment, (ii) intelligibility rate, (iii) severity of hyper-nasality and (iv) types of articulation errors and school/behaviour problems and nasal grimace. Due to subject variability in ethnicity, language and dialects and the lack of normal data, it is difficult to compare or repeat previously reported results. Assessment of intelligibility, hypernasality and articulation were conducted using the Malay language. Results obtained were compared among the three groups of subjects. The significant findings were that hypernasality, intelligibility and articulation errors were more severe in the ICLP group than in the UCLP or BCLP groups of patients. This is contrary to expectations and cannot be explained in terms of the type and/or the degree of clefting. Hence, special attention should be paid to the less overt type of cleft patients in so far as their speech assessment and rehabilitation are concerned.
    Matched MeSH terms: Cleft Lip/surgery*
  6. Fong PH, Yeap CL, Lee ST
    Ann Acad Med Singap, 1983 Apr;12(2 Suppl):363-5.
    PMID: 6625521
    Cleft lip and palate is the most common congenital abnormality seen in the Department of Plastic Surgery, Singapore General Hospital. A total of 461 operative cases seen over a period of 5 years (1977 to 1981) is analysed. There is a relatively high incidence in the Singapore population of 2.0 per 1000 live births. In both cleft lip and cleft palate deformities, there is no significant difference in the sex distribution. The cleft patterns show that 78% of the cases are unilateral clefts, 53% are left sided, 25% are right sided and 22% of the cases are bilateral clefts.
    Matched MeSH terms: Cleft Lip/epidemiology*
  7. Mat Saad AZ, Chai KS, Wan Sulaiman WA, Mat Johar SFN, Halim AS
    Arch Plast Surg, 2019 Nov;46(6):518-524.
    PMID: 31775204 DOI: 10.5999/aps.2019.00227
    BACKGROUND: Anterior palatal repair performed during cleft lip repair using a vomerine flap may assist in recruiting additional soft tissue for subsequent completion of palatoplasty, especially in patients with a wide cleft. We present our early.

    RESULTS: in the hope of triggering a re-evaluation of this technique regarding its advantages for maxillary growth through further studies of patients with a wide cleft.

    METHODS: A retrospective analysis of patients with complete unilateral and bilateral cleft lip and palate was performed, including cleft and palatal measurements taken during initial surgery (lip repair together with anterior palate repair) and upon completion of palatoplasty.

    RESULTS: In total, 14 patients were included in this study, of whom nine (63.3%) had unilateral cleft lip and palate and five (37.5%) had bilateral cleft. All patients had a wide cleft palate. Lip and anterior palate repair was done at a median age of 3 months, while completion of palatoplasty was done at a median age of 10.5 months. Measurements taken upon completion of palatoplasty showed significant cleft width reduction in the mid-palate and intertubercle regions; however, the palatal arch distances at nearby landmarks showed non-significant marginal changes.

    CONCLUSIONS: Anterior palate repair using a vomerine flap significantly reduced the remaining cleft width, while the palatal width remained. Further research is warranted to explore the long-term effects of this technique in wide cleft patients in terms of facial growth.

    Matched MeSH terms: Cleft Lip
  8. Ebin LE, Zam NM, Othman SA
    Aust Orthod J, 2010 Nov;26(2):165-70.
    PMID: 21175027
    To investigate the craniofacial morphology of Malay children with repaired UCLP and compare the data with non-cleft Malay children.
    Matched MeSH terms: Cleft Lip/pathology*
  9. Abu-Rub N, Samsudin AR, Abdullah AB, Abdullah N
    Aust Orthod J, 2005 May;21(1):39-43.
    PMID: 16433080
    Presurgical orthopaedics has been employed since the 1950s as an adjunctive neonatal therapy for the correction of cleft lip and palate. It is accepted that presurgical orthopaedic plates facilitate lip repair and balanced orofacial growth.
    Matched MeSH terms: Cleft Lip/surgery; Cleft Lip/therapy*
  10. Mohamad Shah NS, Salahshourifar I, Sulong S, Wan Sulaiman WA, Halim AS
    BMC Genet, 2016 Feb 11;17:39.
    PMID: 26868259 DOI: 10.1186/s12863-016-0345-x
    BACKGROUND: Nonsyndromic orofacial clefts are one of the most common birth defects worldwide. It occurs as a result of genetic or environmental factors. This study investigates the genetic contribution to nonsyndromic cleft lip and/or palate through the analysis of family pedigrees. Candidate genes associated with the condition were identified from large extended families from the Malay population.

    RESULTS: A significant nonparametric linkage (NPL) score was detected in family 100. Other suggestive NPL and logarithm of the odds (LOD) scores were attained from families 50, 58, 99 and 100 under autosomal recessive mode. Heterogeneity LOD (HLOD) score ≥ 1 was determined for all families, confirming genetic heterogeneity of the population and indicating that a proportion of families might be linked to each other. Several candidate genes in linkage intervals were determined; LPHN2 at 1p31, SATB2 at 2q33.1-q35, PVRL3 at 3q13.3, COL21A1 at 6p12.1, FOXP2 at 7q22.3-q33, FOXG1 and HECTD1 at 14q12 and TOX3 at 16q12.1.

    CONCLUSIONS: We have identified several novel and known candidate genes for nonsyndromic cleft lip and/or palate through genome-wide linkage analysis. Further analysis of the involvement of these genes in the condition will shed light on the disease mechanism. Comprehensive genetic testing of the candidate genes is warranted.

    Matched MeSH terms: Cleft Lip/diagnosis*; Cleft Lip/genetics*
  11. Haque S, Alam MK, Khamis MF
    BMC Pediatr, 2017 May 06;17(1):119.
    PMID: 28477625 DOI: 10.1186/s12887-017-0870-4
    BACKGROUND: Cleft lip and palate (CLP) is one of the most common birth defects. Multiple factors are believed to be responsible for an unfavorable dental arch relationship in CLP. Facial growth (maxillary) retardation, which results in class III malocclusion, is the primary challenge that CLP patients face. Phenotype factors and postnatal treatment factors influence treatment outcomes in unilateral cleft lip and palate (UCLP) children, which has led to a great diversity in protocols and surgical techniques by various cleft groups worldwide. The aim of this study was to illustrate the dental arch relationship (DAR) and palatal morphology (PM) of UCLP in Bangladeshi children and to explore the various factors that are responsible for poor DAR and PM.

    METHODS: Dental models of 84 subjects were taken before orthodontic treatment and alveolar bone grafting. The mean age was 7.69 (SD 2.46) years. The DAR and PM were assessed blindly by five raters using the EUROCRAN index (EI). Kappa statistics was used to evaluate the intra- and inter-examiner agreement, chi square was used to assess the associations, and logistic regression analysis was used to explore the responsible factors that affect DAR and PM.

    RESULTS: The mean EUROCRAN scores were 2.44 and 1.93 for DAR and PM, respectively. Intra- and inter-examiner agreement was moderate to very good. Using crude and stepwise backward regression analyses, significant associations were found between the modified Millard technique (P = 0.047, P = 0.034 respectively) of cheiloplasty and unfavorable DAR. Complete UCLP (P = 0.017) was also significantly correlated with unfavorable DAR. The PM showed a significant association with the type of cleft, type of cheiloplasty and type of palatoplasty.

    CONCLUSION: This multivariate study determined that the complete type of UCLP and the modified Millard technique of cheiloplasty had significantly unfavorable effects on both the DAR and PM.

    Matched MeSH terms: Cleft Lip/pathology*; Cleft Lip/surgery
  12. Trott JA, Mohan N
    Br J Plast Surg, 1993 Apr;46(3):215-22.
    PMID: 8490700
    This paper is a preliminary report on a strategy to perform open tip rhinoplasty at the time of lip repair in bilateral cleft lip and palate deformity. This method was devised to suit unique socio-economic circumstances in the Malaysian centre of Alor Setar. Of 8 cases having surgery in 1991, 7 returned for follow-up with results being documented photographically. It is concluded that this aggressive approach is justified in severe bilateral cleft lip and palate deformity because of the cost effectiveness of limiting the number of interventions. Furthermore, it provides optimally orientated nasal tip anatomy and reduces the social stigma of cleft lip nose appearance from the earliest possible time in the child's development.
    Matched MeSH terms: Cleft Lip/surgery*
  13. Trott JA, Mohan N
    Br J Plast Surg, 1993 Jul;46(5):363-70.
    PMID: 8369872
    A method of open-tip rhinoplasty using nostril and columella rim incisions at the time of unilateral cleft lip repair is described. This preliminary report details experience gained using this technique in the Malaysian centre of Alor Setar following its introduction in September, 1991.
    Matched MeSH terms: Cleft Lip/surgery*
  14. ROSLINA R., ZAINUL AHMAD R., ZILFALIL BA, WAN AZMAN WS, AHMAD SUKARI H, SAIDI J.
    MyJurnal
    Orofacial clefts are one of the most common congenital malformations among newborns. The two main types of oral clefts are cleft lip with or without cleft (CLP) and cleft palate alone (CP). Cleft is an abnormal ssure in an anatomical structure that is normally fused. Cleft lip is the congenital failure of the maxillary and medial nasal processes to fuse, forming a ssure in the lip. Cleft palate is the congenital failure of the palate to fuse properly, forming a ssure in the roof of the mouth (Mossey, 2009).clefts are one of the most common congenital malformations among newborns. The two main types of oral clefts are cleft lip with or without cleft (CLP) and cleft palate alone (CP). Cleft is an abnormal ssure in an anatomical structure that is normally fused. Cleft lip is the congenital failure of the maxillary and medial nasal processes to fuse, forming a ssure in the lip. Cleft palate is the congenital failure of the palate to fuse properly, forming a ssure in the roof of the mouth (Mossey, 2009).
    Matched MeSH terms: Cleft Lip
  15. Othman SA, Saffai L, Wan Hassan WN
    Clin Oral Investig, 2020 Aug;24(8):2853-2866.
    PMID: 31754872 DOI: 10.1007/s00784-019-03150-1
    OBJECTIVES: To validate the accuracy and reproducibility of linear measurements of three-dimensional (3D) images and to compare the measurements with the direct anthropometry method on cleft lip and palate (CLP) patients.

    MATERIALS AND METHODS: Nineteen linear facial measurements were derived from 16 standardized surface landmarks obtained from 37 cleft patients (20 males, 17 females; mean age 23.84 years, standard deviation ± 6.02). They were taken manually with calipers and were compared with the digitally calculated distance on the 3D images captured using the VECTRA-M5 360° Imaging System with pre-marked landmarks. Another pair of 19 linear measurements were computed on the 3D images 2 weeks apart for intra- and inter-observer agreements. Statistical analyses used were paired t test, the Bland-Altman analysis, and the intra-class correlation coefficient (ICC) index.

    RESULTS: Most of the linear measurements showed no statistically significant differences between the proposed method and direct anthropometry linear measurements. Nevertheless, bias of the 3D imaging system is present in the linear measurements of the nose width and the upper vermillion height. The measurements' mean biases were within 2 mm, but the 95% limit of agreement was more than 2 mm. Intra- and inter-observer measurements generally showed good reproducibility. Four inter-observer measurements, the upper and lower face heights, nose width, and pronasale to left alar base were clinically significant.

    CONCLUSIONS: Measurements obtained from this 3D imaging system are valid and reproducible for evaluating CLP patients.

    CLINICAL RELEVANCE: The system is suitable to be used in a clinical setting for cleft patients. However, training of the operator is strictly advisable.

    Matched MeSH terms: Cleft Lip
  16. Salahshourifar I, Halim AS, Sulaiman WA, Ariffin R, Naili Muhamad Nor N, Zilfalil BA
    Cytogenet Genome Res, 2011;134(2):83-7.
    PMID: 21447942 DOI: 10.1159/000325541
    Microdeletion of the Van der Woude syndrome (VWS) critical region is a relatively rare event, and only a few cases have been reported in the medical literature. The extent of the deletion and the genotype-phenotype correlation are 2 crucial issues.
    Matched MeSH terms: Cleft Lip/genetics
  17. Gopinath VK, Samsudin AR, Mohd Noor SNF, Mohamed Sharab HY
    Eur J Dent, 2017 4 25;11(1):76-82.
    PMID: 28435370 DOI: 10.4103/ejd.ejd_238_16
    OBJECTIVES: The aim of this study was to evaluate the vertical and sagittal facial profile and maxillary arch width, depth, and length of patients with unilateral cleft lip and palate (UCLP) and to compare them with healthy noncleft children in the mixed dentition stage (7-13 years).

    MATERIALS AND METHODS: This study is conducted at Hospital Universiti Sains Malaysia. UCLP group comprised 48 patients with nonsyndromic UCLP who have had the lip and palate repaired, whereas the control group comprised 48 healthy noncleft cases. The lateral cephalometrics measurements were used to determine the vertical height, sagittal depth of the face, and cranial base length and angle. Maxillary arch dimensions were measured on the study cast including arch width, depth, and length.

    RESULTS: Vertical facial height and sagittal depth measurements showed a significant decrease (P < 0.05) in the mean growth pattern in UCLP group. The anterior cranial base length (S-N) was shorter in UCLP children (P < 0.001), while Ba-N length had no significant difference (P = 0.639). Nasion-Sella Tursica-Basion angle was significantly higher in the UCLP group (P = 0.016). Dental arch width with reference to canine-to-canine and first premolar-to- first premolar distance was significantly larger in control (P = 0.001).

    CONCLUSION: Mean vertical and sagittal facial dimensions in the UCLP children who do not undergo orthodontic treatment are significantly lesser in all directions of growth than healthy noncleft children. The maxillary dental arch had a normal depth but constricted in width and arch length.

    Matched MeSH terms: Cleft Lip
  18. Salahshourifar I, Wan Sulaiman WA, Halim AS, Zilfalil BA
    Eur J Med Genet, 2012 Jun;55(6-7):389-93.
    PMID: 22440537 DOI: 10.1016/j.ejmg.2012.02.006
    Non-syndromic oral clefts share the main clinical features of Van der Woude Syndrome (VWS), with the exception of the lower lip pit. Thus, about 15% of VWS cases are indistinguishable from cases with non-syndromic oral clefts. IRF6 mutations are the major cause of VWS; however, variants in this gene show strong association with non-syndromic oral clefts, with a higher increased risk among cases with cleft lip only (CLO). A total of 39 individuals, including 16 patients with CLO and 23 patients with a family history of cleft, were examined for IRF6 mutations in the present study. Seven variants, including five known (c.-75-4 A>; G, c.-73T>; C, c.459G>; T 5, c.820G>; A, and c.1060 + 37C>; T) and two novel (c.-75-23G>; C and c.1380G>; T), were found. Both novel variants were inherited from non-affected parents and we did not find also in the 120 control chromosomes. In silico analysis revealed that both c.1380G>; T and c.-75-23G>; C variants may disrupts a putative exonic splicing enhancer and intronic splicing binding site for SC35, respectively. Taken together, the presence of deleterious IRF6 variants in patients with non-syndromic oral clefts could be most likely an evidence for VWS. While, IRF6 variants could, at best, contribute to clefting as part of a complex inheritance pattern, with both additional genes and environmental factors having a role.
    Matched MeSH terms: Cleft Lip/genetics*
  19. Gunasegran R
    Family Physician, 1992;4:10-12.
    Matched MeSH terms: Cleft Lip
  20. Alam MK, Alfawzan AA, Haque S, Mok PL, Marya A, Venugopal A, et al.
    Front Pediatr, 2021;9:651951.
    PMID: 34026687 DOI: 10.3389/fped.2021.651951
    To investigate whether the craniofacial sagittal jaw relationship in patients with non-syndromic cleft differed from non-cleft (NC) individuals by artificial intelligence (A.I.)-driven lateral cephalometric (Late. Ceph.) analysis. The study group comprised 123 subjects with different types of clefts including 29 = BCLP (bilateral cleft lip and palate), 41 = UCLP (unilateral cleft lip and palate), 9 = UCLA (unilateral cleft lip and alveolus), 13 = UCL (unilateral cleft lip) and NC = 31. The mean age was 14.77 years. SNA, SNB, ANB angle and Wits appraisal was measured in lateral cephalogram using a new innovative A.I driven Webceph software. Two-way ANOVA and multiple-comparison statistics tests were applied to see the differences between gender and among different types of clefts vs. NC individuals. A significant decrease (p < 0.005) in SNA, ANB, Wits appraisal was observed in different types of clefts vs. NC individuals. SNB (p > 0.005) showed insignificant variables in relation to type of clefts. No significant difference was also found in terms of gender in relation to any type of clefts and NC group. The present study advocates a decrease in sagittal development (SNA, ANB and Wits appraisal) in different types of cleft compared to NC individuals.
    Matched MeSH terms: Cleft Lip
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