Combination therapy with carfilzomib, lenalidomide and dexamethasone (KRd) results in an unprecedented purity of the stem cell graft in newly diagnosed patients with myeloma.

Still, many physicians give 4 cycles of combination therapy to multiple myeloma patients prior to collection of stem cells for autologous bone marrow transplant. This tradition originates from older doxorubicin-containing regiments which limited the number of cycles due to cumulative cardiotoxicity. Using older regiments, most patients had residual myeloma cells in their autologous stem-cell grafts during collection.

Leiomyosarcoma: a rare sinonasal malignancy.

Leiomyosarcomas (LMS) are rare sinonasal malignant tumours with 20 cases of nasal LMS previously reported in the literature. An 81-year-old man presented with an incidental left nasal lesion associated with left nasal obstruction and occasional left-sided blood-stained rhinorrhoea. Nasendoscopic examination showed green coloured polyps filling the left nasal cavity with biopsies showing LMS.

Birth weight, gestational age and late adolescent liver function using twin status as instrumental variable in a Hong Kong Chinese birth cohort: "Children of 1997".

Birth weight (BW) is inversely associated with diabetes and liver function in Mendelian Randomization studies. Observationally, lower BW is usually also associated with poorer liver function. However, these studies could be confounded by socioeconomic position.

The Impact of Vocal and Laryngeal Pathologies Among Professional Singers: A Meta-analysis.

Objective: Professional singers are more likely to develop laryngeal pathologies and symptoms associated with misuse and overuse of the voice. However, different studies have shown conflicting evidence. We aim to perform a systematic review and quantitative meta-analysis to determine the prevalence and risk of laryngeal pathologies and symptoms among professional singers.

Patient Satisfaction in the Preoperative Period: Preparing for Hand Surgery.

The potential impact of the number and type of preoperative encounters on satisfaction rates prior to elective surgical procedures is unclear, specifically scheduling and medical clearance encounters. Questionnaires investigating satisfaction with the preoperative process were collected for 200 patients presenting for elective hand surgery. The number of telephone, surgeon, and medical clearance encounters were recorded, and satisfaction was determined for each type based on a 4-category Likert scale.

Disconnect Between Genes Associated With Ischemic Heart Disease and Targets of Ischemic Heart Disease Treatments.

Background: Development of pharmacological treatments to mitigate ischemic heart disease (IHD) has encompassed disappointing results and expensive failures, which has discouraged investment in new approaches to prevention and control. New treatments are most likely to be successful if they act on genetically validated targets. We assessed whether existing pharmacological treatments for IHD reduction are acting on genetically validated targets and whether all such targets for IHD are currently being exploited.

Baseline mutational patterns and sustained MRD negativity in patients with high-risk smoldering myeloma.

Early results of a prospective phase 2 clinical trial of carfilzomib, lenalidomide, and dexamethasone followed by lenalidomide maintenance in high-risk smoldering myeloma showed promising results that were previously published. Here, we provide novel insights into the genetic landscape of high-risk smoldering myeloma and information on sustained minimal residual disease (MRD) negativity with an expanded cohort of patients. Eighteen patients with high-risk smoldering myeloma were enrolled between 29 May 2012, and 14 January 2014.

Genetic variation in predicts phenytoin-induced maculopapular exanthema in European-descent patients.

Objective: To characterize, among European and Han Chinese populations, the genetic predictors of maculopapular exanthema (MPE), a cutaneous adverse drug reaction common to antiepileptic drugs.

Methods: We conducted a case-control genome-wide association study of autosomal genotypes, including Class I and II human leukocyte antigen (HLA) alleles, in 323 cases and 1,321 drug-tolerant controls from epilepsy cohorts of northern European and Han Chinese descent. Results from each cohort were meta-analyzed.

The Associations of Breast Feeding with Infant Growth and Body Mass Index to 16 years: 'Children of 1997'.

Background: Controversial findings concerning associations of breast feeding with growth have been reported. This study examined whether the associations of breast feeding with early growth trajectories and body mass index to 16 years differed by sex or age.

Methods: In Hong Kong's 'Children of 1997' population-representative birth cohort, contemporaneously reported breast-feeding status in the first 3 months was classified as exclusive breast feeding (BF) (n = 470), mixed feeding (MF) (n = 2693), and formula feeding (FF) (n = 4204).

Age-period-cohort analysis of trends in blood pressure and body mass index in children and adolescents in Hong Kong.

Background: The declining or fluctuating trend in blood pressure (BP) despite the rising trend in body mass index (BMI) during childhood and adolescence is unexplained. We decomposed trends in BP and BMI to identify the relevance of early-life and contemporaneous factors.

Methods: We assessed the relative contribution of age, period and cohort to secular trends in BP in children and adolescents (9-18 years) from 1999 to 2014 and BMI (6-18 years) from 1996 to 2014 in Hong Kong, China.

USP7 small-molecule inhibitors interfere with ubiquitin binding.

The ubiquitin system regulates essential cellular processes in eukaryotes. Ubiquitin is ligated to substrate proteins as monomers or chains and the topology of ubiquitin modifications regulates substrate interactions with specific proteins. Thus ubiquitination directs a variety of substrate fates including proteasomal degradation.

Revision gastric bypass after laparoscopic adjustable gastric band: a 10-year experience at a public teaching hospital.

Background: In Australia, there is limited access to public revisional bariatric procedures. However, the need for such procedures is rising. We investigated the safety and efficacy of band-to-bypass procedures in our experience at a public teaching hospital over a period of 10 years.

Host-related immunodeficiency in the development of multiple myeloma.

Host-related immunodeficiency is known to play a role in the development of multiple myeloma (MM) from its precursor conditions (monoclonal gammopathy of undetermined significance, MGUS, smoldering multiple myeloma, SMM). In order to understand the underlying immune changes in this process, we characterized immune patterns from MGUS to SMM to MM. We further sought to identify potential novel immune biomarkers that may predict progression of SMM to MM.

The association of breastfeeding with insulin resistance at 17 years: Prospective observations from Hong Kong's "Children of 1997" birth cohort.

Breastfeeding has many benefits for mother and infant. Whether breastfeeding also protects against type 2 diabetes is unclear. To clarify the role of breastfeeding in type 2 diabetes, we assessed the association of breastfeeding with insulin resistance in late adolescence in a birth cohort from a non-Western setting where breastfeeding was not associated with higher socio-economic position.

Evolution of the Reactor Antineutrino Flux and Spectrum at Daya Bay.

The Daya Bay experiment has observed correlations between reactor core fuel evolution and changes in the reactor antineutrino flux and energy spectrum. Four antineutrino detectors in two experimental halls were used to identify 2.2 million inverse beta decays (IBDs) over 1230 days spanning multiple fuel cycles for each of six 2.

Divergent secular trends in blood pressure and body mass index in children and adolescents in Hong Kong.

Secular trends in blood pressure (BP) and body mass index (BMI) during childhood and adolescence are sentinels for the future population cardiovascular disease burden. We examined trends in BP z-score (ages 9-18 years from 1999 to 2014) and BMI z-score (ages 6-18 years from 1996 to 2014) in Hong Kong, China. Overall, BP z-score fell, systolic BP from 0.

Endovascular Fenestration for Distal Aortic Sealing After Frozen Elephant Trunk With Thoraflex.

We describe a case of total arch replacement with frozen elephant trunk for chronic type B aortic dissecting aneurysm, which resulted in inadvertent landing of the frozen elephant trunk into the false lumen. A radiofrequency puncture system-assisted controlled endovascular fenestration of the dissection flap was performed at the upper abdominal aorta and subsequent thoracic endovascular stenting, successfully redirecting the blood flow from the false to the true lumen. Our case illustrated a possible way to seal distal reentry in chronic type B aortic dissection.

Dynamic changes in clonal cytogenetic architecture during progression of chronic lymphocytic leukemia in patients and patient-derived murine xenografts.

Subclonal heterogeneity and clonal selection influences disease progression in chronic lymphocytic leukemia (CLL). It is therefore important that therapeutic decisions are made based on an understanding of the CLL clonal architecture and its dynamics in individual patients. Identification of cytogenetic abnormalities by FISH remains the cornerstone of contemporary clinical practice and provides a simple means for prognostic stratification.

USP7 inhibition alters homologous recombination repair and targets CLL cells independently of ATM/p53 functional status.

The role of deubiquitylase ubiquitin-specific protease 7 (USP7) in the regulation of the p53-dependent DNA damage response (DDR) pathway is well established. Whereas previous studies have mostly focused on the mechanisms underlying how USP7 directly controls p53 stability, we recently showed that USP7 modulates the stability of the DNA damage responsive E3 ubiquitin ligase RAD18. This suggests that targeting USP7 may have therapeutic potential even in tumors with defective p53 or ibrutinib resistance.

Median philtrum sinus: a rare presentation and review of management options.

Nasal dermoids are rare congenital abnormalities, and there is only one previously reported case of a philtrum sinus tract extending to the skull base. A 2-month-old boy was presented with an incidental finding of a median philtrum sinus with no infective features and no other abnormal clinical findings. MRI demonstrated a sinus tract extending from the philtrum to the crista galli.

SNP-based HLA allele tagging, imputation and association with antiepileptic drug-induced cutaneous reactions in Hong Kong Han Chinese.

Human leukocyte antigen (HLA) genes control the regulation of the human immune system and are involved in immune-related diseases. Population surveys on relationships between single nucleotide polymorphisms (SNP) and HLA alleles are essential to conduct genetic association between HLA variants and diseases. Samples were obtained from our in-house database for epilepsy genetics and pharmacogenetics research.

Pubertal testis volume, age at pubertal onset, and adolescent blood pressure: Evidence from Hong Kong's "Children of 1997" birth cohort.

Objectives: A warning from Health Canada suggests that testosterone increases blood pressure (BP). No evidence from large randomized controlled trials is available, and observational studies are confounded by ill-health lowering serum testosterone. To address the evidence gap, we assessed the association of pubertal testicular volume, as a reflection of testosterone production, with BP.

Genetically predicted milk consumption and bone health, ischemic heart disease and type 2 diabetes: a Mendelian randomization study.

Background/objectives: Milk provides protein and micronutrients, and is recommended by some dietary guidelines, particularly for bone health. Meta-analysis of small randomized controlled trials suggests that milk may increase bone mineral density, but they are very heterogeneous. No randomized controlled trial has assessed the effects of milk on major chronic diseases.