METHODS: Relations between skeletal movement, hyoid bone position and three-dimensional pharyngeal airway changes were retrospectively analyzed on pre- and post-surgical CBCTs in dento-skeletal class II patients who underwent orthognathic two-jaw surgery with segmentation.
RESULTS: While long-term significant reductions in length (P= 0.003), surface area (P= 0.042) and volume (P= 0.004) were found in the nasopharynx, the highly significant increases in oropharyngeal airway length, surface area, volume and the minimal cross-sectional area (P
RESULTS AND DISCUSSION: Ninety-six haemophilia patients were identified - 79(82.3%) haemophilia A(HA) and 17(17.7%) haemophilia B(HB). Severe haemophilia patients were noted in 45.6% (36/79) of HA and 64.7% (11/17) of HB. In all 44.3% of the HA and 52.9% of the HB population had no identifiable family history of haemophilia. Two-thirds of the patients with severe HA were on prophylaxis [24/36 (66.7%)] and only onethird [4/11 (36.4%)] in severe HB. Inhibitors developed in 9/79 (11.4%) of the HA population [3/79 (3.8%) high responders]. The median inhibitor titre was not significantly different between the different treatment groups - on demand versus prophylaxis (1.0BU versus 2.0BU; z statistic -1.043, p-value 0.297, Mann-Whitney test). None of the patients developed inhibitory alloantibodies to factor IX. Four HA patients (5.1%) underwent immune tolerance induction where one case had a successful outcome. Three severe HA patients received emicizumab prophylaxis and showed remarkable reduction in bleeding events with no thromboembolic events being reported. One female moderate HA patient received PEGylated recombinant anti-haemophilic factor. Eleven patients underwent radiosynovectomy. One mild HB patient succumbed to traumatic intracranial bleeding. Our data reported a prevalence (per 100,000 males) of 5.40 cases for all severities of HA, 2.46 cases for severe HA; 1.16 cases for all severities of HB, and 0.75 cases for severe HB. The overall incidence of HA and HB was 1 in 11,500 and 1 in 46,000, respectively.
CONCLUSION: This study outlines the Sarawakian haemophilia landscape and offers objective standards for forward planning. Shared responsibilities among all parties are of utmost importance to improve the care of our haemophilia population.
MATERIALS AND METHODS: A total of 119 AA patients had been identified from hospital records of the involved sites, namely Queen Elizabeth Hospital in Sabah, Sarawak General Hospital, Sibu Hospital, Miri Hospital and Bintulu Hospital in Sarawak from Jan 2006 to Dec 2017.
RESULTS: The median age at diagnosis was 46 years, and native ethnic group from Sabah, Kadazan-Dusun, recorded the highest percentage of 41.2%, which could be explained by higher frequency of HLA-DRB1*15:01, an alelle linked to increased risk of AA, among this ethnic group. The majority of patients (59.7%) received cyclosporine (CsA) as monotherapy or in combination with other non-IST agents such as danazol, which was instituted in 48.7% of the patients, while a third of them (33.7%) received antithymocyte globulin (ATG) therapy with or without CsA, and 12.4% underwent allogenic SCT. The five-year overall survival (OS) for all AA patients was 76.1%. Elderly patients >60 years old and those with severe disease had more inferior 5-year survival.
CONCLUSION: A prospective study is warranted to determine the true incidence rate, epidemiological distributions, treatment outcome and overall survival of AA patients in Malaysia. Establishment of allogenic SCT in East Malaysia is imperative to make this curative therapy more accessible to patients with severe disease and improve the outcome.
METHODS: We further investigated the association of rs10235235 with breast cancer risk in a large case control study of 47,346 cases and 47,570 controls from 52 studies participating in the Breast Cancer Association Consortium. Genotyping of rs10235235 was conducted using a custom Illumina Infinium array. Stratified analyses were conducted to determine whether this association was modified by age at diagnosis, ethnicity, age at menarche or tumor characteristics.
RESULTS: We confirmed the association of rs10235235 with breast cancer risk for women of European ancestry but found no evidence that this association differed with age at diagnosis. Heterozygote and homozygote odds ratios (ORs) were OR = 0.98 (95% CI 0.94, 1.01; P = 0.2) and OR = 0.80 (95% CI 0.69, 0.93; P = 0.004), respectively (P(trend) = 0.02). There was no evidence of effect modification by tumor characteristics. rs10235235 was, however, associated with age at menarche in controls (P(trend) = 0.005) but not cases (P(trend) = 0.97). Consequently the association between rs10235235 and breast cancer risk differed according to age at menarche (P(het) = 0.02); the rare allele of rs10235235 was associated with a reduction in breast cancer risk for women who had their menarche age ≥15 years (OR(het) = 0.84, 95% CI 0.75, 0.94; OR(hom) = 0.81, 95% CI 0.51, 1.30; P(trend) = 0.002) but not for those who had their menarche age ≤11 years (OR(het) = 1.06, 95% CI 0.95, 1.19, OR(hom) = 1.07, 95% CI 0.67, 1.72; P(trend) = 0.29).
CONCLUSIONS: To our knowledge rs10235235 is the first single nucleotide polymorphism to be associated with both breast cancer risk and age at menarche consistent with the well-documented association between later age at menarche and a reduction in breast cancer risk. These associations are likely mediated via an effect on circulating hormone levels.