MyMedR

Maternal uniparental heterodisomy of chromosome 6 in a boy with an isolated cleft lip and palate

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.

Dried irradiated human amniotic membrane as a biological dressing for facial burns--a 7-year case series

Bujang-Safawi E, Halim AS, Khoo TL, Dorai AA

Burns, 2010 Sep;36(6):876-82.

PMID: 20236771 DOI: 10.1016/j.burns.2009.07.001

Facial burns are common and have a significant impact on patient function and psychosocial well being. Human amnion has been used for many years as a temporary biological wound dressing in the management of partial thickness burns. The observed advantages of human amnion treatment include pain relief, ease of use, prevention of infection and acceleration of wound healing.

The application of glycerol-preserved skin allograft in the treatment of burn injuries: an analysis based on indications

Khoo TL, Halim AS, Saad AZ, Dorai AA

Burns, 2010 Sep;36(6):897-904.

PMID: 20299154 DOI: 10.1016/j.burns.2009.03.007

Glycerol-preserved skin allograft (GPA) plays a crucial role in the management of burns. Its indications include wound-bed preparation, definitive dressing and sandwich grafting technique.

Fulltext Biologic and synthetic skin substitutes: An overview

Halim AS, Khoo TL, Mohd Yussof SJ

Indian J Plast Surg, 2010 Sep;43(Suppl):S23-8.

PMID: 21321652 DOI: 10.4103/0970-0358.70712

The current trend of burn wound care has shifted to more holistic approach of improvement in the long-term form and function of the healed burn wounds and quality of life. This has demanded the emergence of various skin substitutes in the management of acute burn injury as well as post burn reconstructions. Skin substitutes have important roles in the treatment of deep dermal and full thickness wounds of various aetiologies. At present, there is no ideal substitute in the market. Skin substitutes can be divided into two main classes, namely, biological and synthetic substitutes. The biological skin substitutes have a more intact extracellular matrix structure, while the synthetic skin substitutes can be synthesised on demand and can be modulated for specific purposes. Each class has its advantages and disadvantages. The biological skin substitutes may allow the construction of a more natural new dermis and allow excellent re-epithelialisation characteristics due to the presence of a basement membrane. Synthetic skin substitutes demonstrate the advantages of increase control over scaffold composition. The ultimate goal is to achieve an ideal skin substitute that provides an effective and scar-free wound healing.

Fulltext Wound contraction effects and antibacterial properties of Tualang honey on full-thickness burn wounds in rats in comparison to hydrofibre

Khoo YT, Halim AS, Singh KK, Mohamad NA

BMC Complement Altern Med, 2010;10:48.

PMID: 20815896 DOI: 10.1186/1472-6882-10-48

Full-thickness burn wounds require excision and skin grafting. Multiple surgical procedures are inevitable in managing moderate to severe full-thickness burns. Wound bed preparations prior to surgery are necessary in order to prevent wound infection and promote wound healing. Honey can be used to treat burn wounds. However, not all the honey is the same. This study aims to evaluate the wound contraction and antibacterial properties of locally-produced Tualang honey on managing full-thickness burn wounds in vivo.

Fulltext Antibacterial properties of tualang honey and its effect in burn wound management: a comparative study

Nasir NA, Halim AS, Singh KK, Dorai AA, Haneef MN

BMC Complement Altern Med, 2010;10:31.

PMID: 20576085 DOI: 10.1186/1472-6882-10-31

The use of honey as a natural product of Apis spp. for burn treatment has been widely applied for centuries. Tualang honey has been reported to have antibacterial properties against various microorganisms, including those from burn-related diagnoses, and is cheaper and easier to be absorbed by Aquacel dressing. The aim of this study is to evaluate the potential antibacterial properties of tualang honey dressing and to determine its effectiveness as a partial thickness burn wound dressing.

Fulltext Evaluations of bacterial contaminated full thickness burn wound healing in Sprague Dawley rats Treated with Tualang honey

Sukur SM, Halim AS, Singh KK

Indian J Plast Surg, 2011 Jan;44(1):112-7.

PMID: 21713196 DOI: 10.4103/0970-0358.81459

AIM: The effect of Tualang honey on wound healing in bacterial contaminated full-thickness burn wounds was evaluated in 36 male Sprague Dawley rats.

MATERIALS AND METHODS: The rats were randomly divided into three groups (n = 12/group). Three full-thickness burn wounds were created on each rat. Each group of rats was inoculated with a different organism in the burn wounds: Group A was inoculated with Pseudomonas aeruginosa, Group B was inoculated with Klebsiella pneumoniae and Group C was inoculated with Acinetobacter baumannii. One wound on each rat was dressed with either Tualang honey, Chitosan gel or Hydrofibre silver. Each wound size was measured on day 3, 6, 9, 12, 15, 18 and 21 of the study.

RESULTS: The mean wound size of the Tualang honey-treated wounds was not statistically different than that of the Chitosan gel or Hydrofibre silver-treated wounds when the wounds were compared throughout the entire experiment (P > 0.05). However, comparing the mean wound size on day 21 alone revealed that the Tualang honey-treated wounds were smaller in comparison to that of the Chitosan gel and Hydrofibre silver-treated groups.

CONCLUSIONS: This study shows that topical application of Tualang honey on burn wounds contaminated with P. aeruginosa and A. baumannii gave the fastest rate of healing compared with other treatments.

The use of glycerol-preserved skin allograft in conjunction with reconstructive and flap surgery: seven years of experience

Saad AZ, Halim AS, Khoo TL

J Reconstr Microsurg, 2011 Feb;27(2):103-8.

PMID: 20976667 DOI: 10.1055/s-0030-1268208

Major reconstructive surgery may be extensive and prolonged, and it may cause edema and compromise the flap pedicle if closed under tension. Glycerol-preserved skin allograft (GPA) can provide a means for tension-free closure and temporary cover of the wound. Seven years of analysis on GPA used in conjunction with major reconstruction was undertaken to highlight its indications, results, and outcomes. Forty-seven patients were included, aged between 9 and 73 years. Majority of patients had reconstruction following tumor resection and trauma. The main indication for use of GPA was temporary, loose cover of the wound in 44% of cases; flap pedicle protection in 31% of cases; donor site wound cover in 10%; flap monitoring in one case; and management of flap-related complications in 6% of cases. Free flap reconstruction was performed in 72% of cases. In conclusion, GPA is a useful adjunct in reconstructive surgery. It can be used temporarily to allow tension-free wound closure, as well as to protect the flap pedicle until edema subsides and the pedicle becomes stable. This latter approach allows secondary wound closure and good esthetic outcome.

Fulltext Contribution of 6p24 to non-syndromic cleft lip and palate in a Malay population: association of variants in OFC1

Salahshourifar I, Halim AS, Sulaiman WA, Zilfalil BA

J Dent Res, 2011 Mar;90(3):387-91.

PMID: 21297019 DOI: 10.1177/0022034510391798

Non-syndromic cleft lip, with or without cleft palate, is a heterogeneous, complex disease with a high incidence in the Asian population. Several association studies have been done on cleft candidate genes, but no reports have been published thus far on the Orofacial Cleft 1 (OFC1) genomic region in an Asian population. This study investigated the association between the OFC1 genomic region and non-syndromic cleft lip with or without cleft palate in 90 Malay father-mother-offspring trios. Results showed a preferential over-transmission of a 101-bp allele of marker D6S470 in the allele- and haplotype-based transmission disequilibrium test (TDT), as well as an excess of maternal transmission. However, no significant p-value was found for a maternal genotype effect in a log-linear model, although single and double doses of the 101-bp allele showed a slightly increased cleft risk (RR = 1.37, 95% CI, 0.527-3.4, p-value = 0.516). Carrying two copies of the 101-bp allele was significantly associated with an increased cleft risk (RR = 2.53, 95% CI, 1.06-6.12, p-value = 0.035). In conclusion, we report evidence of the contribution of the OFC1 genomic region to the etiology of clefts in a Malay population.

Tualang honey has higher phenolic content and greater radical scavenging activity compared with other honey sources

Kishore RK, Halim AS, Syazana MS, Sirajudeen KN

Nutr Res, 2011 Apr;31(4):322-5.

PMID: 21530807 DOI: 10.1016/j.nutres.2011.03.001

Many chronic diseases are associated with increased oxidative stress caused by an imbalance between free-radical production and the antioxidant level. Antioxidants, which are abundant in natural honey, are free-radical scavengers that either reduce the formation of or neutralize free radicals. The composition and source of honey greatly dictates its biochemical properties. We performed a comparative analysis of the total phenolic content and antioxidant potential of common commercially available honeys along with Malaysian tualang honey. In vitro biochemical analysis of the phenolic content by the Folin-Ciocalteau method revealed a significantly elevated phenolic content (83.96 ± 4.53 mg gallic acid equivalents per 100 g) in tualang honey. In addition, the antioxidant capacity (53.06 ± 0.41 mg ascorbic acid equivalents per gram) of tualang honey was greater, as assessed by the phosphomolybdenum method, 2,2-diphenyl-1-picryl-hydrazyl assay, and ferric reducing/antioxidant power assay. Peroxynitrite and superoxide radical scavenging activity was determined by spectrophotometric analysis in different honey types. Our data suggest that the elevated free-radical scavenging and antioxidant activity observed in tualang honey is due to the increased level of phenolic compounds. In addition to its antibacterial, anticarcinogenic, and anti-inflammatory properties, our study highlights the favorable antioxidant properties of tualang honey, which may be important to human nutrition and health.

A prospective, randomised, double-blinded trial to study the efficacy of topical tocotrienol in the prevention of hypertrophic scars

Khoo TL, Halim AS, Zakaria Z, Mat Saad AZ, Wu LY, Lau HY

J Plast Reconstr Aesthet Surg, 2011 Jun;64(6):e137-45.

PMID: 20869928 DOI: 10.1016/j.bjps.2010.08.029

Despite widespread beliefs regarding the use of topical tocotrienol in the prevention of hypertrophic scars, there is very little evidence from well controlled and randomised clinical trials to justify its benefits for surgical scars.

Breakage of an intramedullary rod after bone union in congenital pseudoarthrosis of the tibia: a report of two cases

Sulaiman AR, Simbak N, Wan Ismail WF, Wan Z, Halim AS

J Orthop Surg (Hong Kong), 2011 Aug;19(2):250-3.

PMID: 21857057

We report 2 patients with congenital pseudoarthrosis of the tibia who underwent intramedullary Rush rod transfixation through the ankle joint following refracture and nonunion of vascularised fibular grafting 6 and 8 months earlier. After 9 and 5 years, both Rush rods were broken at the level of the ankle joints, while the reconstructed area was solidly united. The growth of the distal tibia increased the distance of the tips of the broken rod and hence the ankle joint motion. The broken tips may damage the articular cartilage and result in valgus deformity of the ankle and limb length discrepancy.

Contribution of variants in and near the IRF6 gene to the risk of nonsyndromic cleft lip with or without cleft palate in a Malay population

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.

Contribution of MSX1 variants to the risk of non-syndromic cleft lip and palate in a Malay population

Salahshourifar I, Halim AS, Wan Sulaiman WA, Zilfalil BA

J Hum Genet, 2011 Nov;56(11):755-8.

PMID: 21866112 DOI: 10.1038/jhg.2011.95

Oral clefts are clinically and genetically heterogeneous disorders that are influenced by both genetic and environmental factors. The present family-based association study investigated the role of the MSX1 and TGFB3 genes in the etiology of non-syndromic oral cleft in a Malay population. No transmission distortion was found in the transmission disequilibrium analysis for either MSX1-CA or TGFB3-CA intragenic markers, whereas TGFB3-CA exhibited a trend to excess maternal transmission. In sequencing the MSX1 coding regions in 124 patients with oral cleft, five variants were found, including three known variants (A34G, G110G and P147Q) and two novel variants (M37L and G267A). The P147Q and M37L variants were not observed in 200 control chromosomes, whereas G267A was found in one control sample, indicating a very rare polymorphic variant. Furthermore, the G110G variant displayed a significant association between patients with non-syndromic cleft lip, with or without cleft palate, and normal controls (P=0.001, odds ratio=2.241, 95% confidence interval, 1.357-3.700). Therefore, these genetic variants may contribute, along with other genetic and environmental factors, to this condition.

De novo interstitial deletion of 1q32.2-q32.3 including the entire IRF6 gene in a patient with oral cleft and other dysmorphic features

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.

Fulltext Antiproliferative effect of methanolic extraction of tualang honey on human keloid fibroblasts

Nurul Syazana MS, Halim AS, Gan SH, Shamsuddin S

BMC Complement Altern Med, 2011;11:82.

PMID: 21943200 DOI: 10.1186/1472-6882-11-82

Keloid is a type of scar which extends beyond the boundaries of the original wound. It can spread to the surrounding skin by invasion. The use of Tualang honey is a possible approach for keloid treatment. The objective of this study was to determine the antiproliferative effect of methanolic extraction of Tualang honey to primary human keloid fibroblasts and to identify the volatile compounds in methanol extraction of Tualang honey.

Fulltext Resection and reconstruction of malignant tumor involving sternum

Faisham WI, Ziyadi MG, Azman WS, Halim AS, Zulmi W, Biswal BM

Med J Malaysia, 2012 Apr;67(2):224-5.

PMID: 22822652 MyJurnal

We present a series of four cases of chest wall tumor, which underwent sternum resection. The methods of resection and reconstruction chest wall defect are discussed and the final outcome highlighted.

Fulltext Keloid scarring: understanding the genetic basis, advances, and prospects

Halim AS, Emami A, Salahshourifar I, Kannan TP

Arch Plast Surg, 2012 May;39(3):184-9.

PMID: 22783524 DOI: 10.5999/aps.2012.39.3.184

Keloid disease is a fibroproliferative dermal tumor with an unknown etiology that occurs after a skin injury in genetically susceptible individuals. Increased familial aggregation, a higher prevalence in certain races, parallelism in identical twins, and alteration in gene expression all favor a remarkable genetic contribution to keloid pathology. It seems that the environment triggers the disease in genetically susceptible individuals. Several genes have been implicated in the etiology of keloid disease, but no single gene mutation has thus far been found to be responsible. Therefore, a combination of methods such as association, gene-gene interaction, epigenetics, linkage, gene expression, and protein analysis should be applied to determine keloid etiology.

Fulltext Wound bed preparation from a clinical perspective

Halim AS, Khoo TL, Saad AZ

Indian J Plast Surg, 2012 May;45(2):193-202.

PMID: 23162216 DOI: 10.4103/0970-0358.101277

Wound bed preparation has been performed for over two decades, and the concept is well accepted. The 'TIME' acronym, consisting of tissue debridement, infection or inflammation, moisture balance and edge effect, has assisted clinicians systematically in wound assessment and management. While the focus has usually been concentrated around the wound, the evolving concept of wound bed preparation promotes the treatment of the patient as a whole. This article discusses wound bed preparation and its clinical management components along with the principles of advanced wound care management at the present time. Management of tissue necrosis can be tailored according to the wound and local expertise. It ranges from simple to modern techniques like wet to dry dressing, enzymatic, biological and surgical debridement. Restoration of the bacterial balance is also an important element in managing chronic wounds that are critically colonized. Achieving a balance moist wound will hasten healing and correct biochemical imbalance by removing the excessive enzymes and growth factors. This can be achieved will multitude of dressing materials. The negative pressure wound therapy being one of the great breakthroughs. The progress and understanding on scientific basis of the wound bed preparation over the last two decades are discussed further in this article in the clinical perspectives.

Facial dimensions of Malay children with repaired unilateral cleft lip and palate: a three dimensional analysis

Zreaqat M, Hassan R, Halim AS

Int J Oral Maxillofac Surg, 2012 Jun;41(6):783-8.

PMID: 22424709 DOI: 10.1016/j.ijom.2012.02.003

This comparative cross-sectional study assessed the facial surface dimensions of a group of Malay children with unilateral cleft lip and palate (UCLP) and compared them with a control group. 30 Malay children with UCLP aged 8-10 years and 30 unaffected age-matched children were voluntarily recruited from the Orthodontic Specialist Clinic in Hospital Universiti Sains Malaysia (HUSM). For the cleft group, lip and palate were repaired and assessment was performed prior to alveolar bone grafting and orthodontic treatment. The investigation was carried out using 3D digital stereophotogrammetry. 23 variables and two ratios were compared three-dimensionally between both groups. Statistically significant dimensional differences (P<0.05) were found between the UCLP Malay group and the control group mainly in the nasolabial region. These include increased alar base and alar base root width, shorter upper lip length, and increased nose base/mouth width ratio in the UCLP group. There were significant differences between the facial surface morphology of UCLP Malay children and control subjects. Particular surgical procedures performed during primary surgeries may contribute to these differences and negatively affect the surgical outcome.

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