Effects of Senior Simulation Suit Programme on nursing students' attitudes towards older adults: A randomized controlled trial.

BACKGROUND: As populations age worldwide, nursing educational institutions need to train nurses not only to provide health care services specific to the elderly, but also to have a positive attitude as they work. The present study aimed to investigate the efficacy of a Senior Simulation Suit Programme (SSSP). The SSSP, which focused on mimicking the physiological experiences of an 80 year-old person, was hypothesized to increase the wearer's positive attitude towards older adult care. METHODS: A single-blinded, randomized controlled trial was used to evaluate the efficacies of SSSP. One hundred and thirty-nine (139) nursing students were randomly assigned to either SSSP group (n = 69) or to a control group (n = 70) with "placebo clothing", i.e. clothing that mimicked old age but did not actually impair faculties. Two instruments-Kogan Attitudes Towards Old People Scale (KAOP) and a 1-item scale on Willingness To Care for Older People Scale (WCOP)-were used for data collection at baseline and at completion of SSSP. A Chinese version of Palmore's Facts Aging Quiz (C-FAQ) was used to assess nursing students' knowledge about adult care, and a questionnaire was developed to collect demographic information at baseline. RESULTS: No significant difference between the two groups was found. A significant increase of positive attitudes and of willingness to serve older adults was found in both the control group and the group wearing SSSP. CONCLUSION: Both the SSSP and control intervention could improve the attitudes of nursing students towards older adult care. This study suggests that wearing whatever the nursing students associate with being old, will improve their attitude towards older adult care.

Indirect calorimetry: an indispensable tool to understand and predict obesity.

Obesity is a physiological condition of chronic positive energy balance. While the regulation of energy metabolism varies widely among individuals, identifying those who are metabolically prone to weight gain and intervening accordingly is a key challenge for reversing the course of the obesity epidemic. Indirect calorimetry is the most commonly used method to measure energy expenditure in the research setting. By measuring oxygen consumption and carbon dioxide production, indirect calorimetry provides minute-by-minute energy expenditure data that makes it the most valuable tool to distinguish the various components of energy expenditure, that is, sleeping and resting metabolic rate, thermic effect of food and the energy cost of activity. Importantly, such measures also provide information on energy substrate utilization. Here we summarized some of the research that revealed resting metabolic rate, spontaneous physical activity and respiratory quotient as key metabolic predictors of weight gain and obesity. Recent studies using indirect calorimetry in response to mid-term fasting or overfeeding have identified 'thrifty' and 'spendthrift' phenotypes in people who differ in propensity to weight gain. We propose the use of indirect calorimetry data as a basis for personalized interventions that may be efficacious in slowing down the rise of global obesity.

Aetiological bases of 46,XY disorders of sex development in the Hong Kong Chinese population.

OBJECTIVE: Disorders of sex development are due to congenital defects in chromosomal, gonadal, or anatomical sex development. The objective of this study was to determine the aetiology of this group of disorders in the Hong Kong Chinese population. SETTING: Five public hospitals in Hong Kong. PATIENTS: Patients with 46,XY disorders of sex development under the care of paediatric endocrinologists between July 2009 and June 2011. MAIN OUTCOME MEASURES: Measurement of serum gonadotropins, adrenal and testicular hormones, and urinary steroid profiling. Mutational analysis of genes involved in sexual differentiation by direct DNA sequencing and multiplex ligation-dependent probe amplification. RESULTS: Overall, 64 patients were recruited for the study. Their age at presentation ranged from birth to 17 years. The majority presented with ambiguous external genitalia including micropenis and severe hypospadias. A few presented with delayed puberty and primary amenorrhea. Baseline and post-human chorionic gonadotropin-stimulated testosterone and dihydrotestosterone levels were not discriminatory in patients with or without AR gene mutations. Of the patients, 22 had a confirmed genetic disease, with 11 having 5α-reductase 2 deficiency, seven with androgen insensitivity syndrome, one each with cholesterol side-chain cleavage enzyme deficiency, Frasier syndrome, NR5A1-related sex reversal, and persistent Müllerian duct syndrome. CONCLUSIONS: Our findings suggest that 5α-reductase 2 deficiency and androgen insensitivity syndrome are possibly the two most common causes of 46,XY disorders of sex development in the Hong Kong Chinese population. Since hormonal findings can be unreliable, mutational analysis of the SRD5A2 and AR genes should be considered the first-line tests for these patients.

Social obstetrics: non-local expectant mothers admitted through accident and emergency department in a public hospital in Hong Kong.

OBJECTIVES: To review the pregnancy outcomes of non-booked, non-local pregnant women delivering in Kwong Wah Hospital via admission to the Accident and Emergency Department 1 year after the announcement by the Hospital Authority to stop antenatal booking for non-eligible persons; and to perform a literature review of local studies about non-eligible person deliveries over the last decade. DESIGN: Case series. SETTING: A public hospital in Hong Kong. PARTICIPANTS: All women who held the People's Republic of China passport or the two-way permit and those non-eligible persons whose spouses were Hong Kong Identity Card holders, who delivered in Kwong Wah Hospital from 1 April 2011 to 31 March 2012. RESULTS: Overall, 219 women who were non-eligible persons delivered 221 live births during the study period. Compared with the annual statistics of Kwong Wah Hospital in 2011, non-local mothers were of higher parity; more likely to have hypertensive disease (including pre-eclamptic toxaemia), preterm deliveries (ie at <37 weeks), babies needing admission to the special care baby unit, and macrosomic babies (ie weighing >4.0 kg). The rates of induction of labour and caesarean section were lower in this group. There was no significant difference in the maternal and neonatal outcomes between women who had no booking and those who had a booking in another Hospital Authority or private hospital. There were many incidents of near-miss obstetric complications or suboptimally managed obstetric conditions due to lack of well-structured and continuous antenatal care in this group of non-eligible persons. CONCLUSION: Non-eligible person delivering babies in Hong Kong has become a social obstetrics phenomenon. Despite the introduction of policies, reduction in the number of deliveries (quantity) did not improve the obstetric outcomes (quality). Health care professionals should continue to be prepared for managing the potential near-miss clinical complications in this group of 'travelling mothers'.

SLICC/ACR damage index independently associated with left ventricular diastolic dysfunction in patients with systemic lupus erythematosus.

Left ventricular (LV) diastolic dysfunction has been reported in both active and inactive systemic lupus erythematosus (SLE) patients without clinical evidence of cardiovascular disease. However, the relationship between the long-term inflammatory burden reflected by the SLICC/ACR damage index and LV diastolic function has not been studied. Eighty-two SLE patients and 82 controls matched for age, sex, body mass index, blood pressure and heart rate underwent echocardiography with tissue Doppler imaging (TDI). LV diastolic function was estimated by the myocardial early diastolic velocity (E') at the lateral annulus. There were 51 patients (62.2%) with nephritis, 23 patients (28.0%) with hypertension, 21 patients (25.6%) with vasculitis, 16 patients (19.5%) with pulmonary hypertension, 4 patients (4.9%) with cerebrovascular disease and 2 patients (2.4%) with diabetes mellitus. Sixty-two patients (75.6%) were taking prednisone and 35 patients (42.7%) used a immunosuppressant. Forty-five patients (54.8%) had active disease and suffered from disease-related end-organ damage. Patients with SLICC/ACR damage index ≥1 had more evidence of LV diastolic dysfunction with lower lateral annulus E' (9.6 ± 3.4 vs 12.9 ± 3.5 cm/s, p < 0.001) than those without. In addition, the proportion of patients with abnormal LV myocardial relaxation (defined as lateral E' < 10.0 cm/s) (51.1% vs 16.2%, χ(2) = 10.8, p = 0.001) were significantly higher. Multivariate analysis showed that the SLICC/ACR damage index ≥1 was independently associated with LV diastolic dysfunction (OR = 3.80, 95%CI: 1.21-11.95, p = 0.023) after adjusting for hypertension, disease duration and medical therapy. This may suggest that the overall inflammatory burden in SLE, as reflected by SLICC/ACR damage index, is associated with the development of diastolic dysfunction in SLE patients.

Successful treatment of severe fetal chylothorax resistant to repeated pleuroamniotic shunting by OK-432 pleurodesis.

We report the first case of successful fetal pleurodesis with OK-432 for recurrent severe fetal primary chylothorax after failing repeated pleuroamniotic shunting. Shunting and pleurodesis could be complementary to each other in the treatment of fetal chylothorax.

Molecular analysis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency in Hong Kong Chinese patients.

BACKGROUND: Congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency (21OHD) is an autosomal recessive disorder due to mutation in the CYP21A2 gene. OBJECTIVE: To elucidate the genetic basis of 21-hydroxylase-deficient CAH in Hong Kong Chinese patients. PATIENTS AND METHODS: Mutational analysis of the CYP21A2 gene was performed on 35 Hong Kong Chinese patients with 21OHD using direct DNA sequencing and multiplex ligation-dependent probe amplification (MLPA). RESULTS: The genetic findings of 21 male and 14 female patients are the following: c.293-13A/C>G (intron 2 splice site; 20 alleles), p.I172N (13), p.R356W (7), p.Q318X (4). A total of 20 mutant alleles contained gross deletion/conversion of all or part of the CYP21A2 gene. A novel mutation, c.1367delA (p.D456fs), was detected in one patient. One patient had only a heterozygous mutation detected. Out of 35 patients, 16 would have been incorrectly genotyped if either DNA sequencing or MLPA alone was used for molecular analysis. CONCLUSIONS: The frequency of various mutations in the studied patients differs from those reported in other Asian populations. Gross deletion/conversion accounts for nearly one-third of the genetic defects. Therefore, laboratories must include methods for detecting point mutations as well as gross deletions/conversions to avoid misinterpretation of genotype. Genotyping has increasingly been proven to be a useful tool for supplementing, if not replacing, hormonal profiling for the diagnosis of 21OHD.

Insulin-stimulated glucose uptake and pathways regulating energy metabolism in skeletal muscle cells: the effects of subcutaneous and visceral fat, and long-chain saturated, n-3 and n-6 polyunsaturated fatty acids.

AIMS: The study aims to determine the effect of long-chain saturated and polyunsaturated (PUFA) fatty acids, specifically palmitic acid (PA; 16:0), docosahexaenoic acid (DHA; 22:6n-3) and linoleic acid (LA; 18:2n-6), and their interactions with factors from adipose tissue, on insulin sensitivity and lipid metabolism in skeletal muscle. METHODS: L6 myotubes were cultured with PA, DHA or LA (0.4mmol/l), with or without conditioned media from human subcutaneous (SC) and visceral (IAB) fat. Insulin-stimulated glucose uptake, lipid content, mRNA expression of key genes involved in nutrient utilization and protein expression of inhibitor protein inhibitor kappa B (IκB)-α and mammalian target of rapamycin (mTOR) were measured. RESULTS: PA and IAB fat reduced insulin-stimulated glucose uptake and their combined effect was similar to that of PA alone. PA-induced insulin resistance was ameliorated by inhibiting the de novo synthesis of ceramide, IκBα degradation or mTOR activation. The PA effect was also partially reversed by DHA and completely by LA in the presence of SC fat. PA increased diacylglycerol content, which was reduced by LA and to a greater extent when either IAB or SC fat was also present. PA increased SCD1 whereas DHA and LA increased AMPKα2 mRNA. In the presence of SC or IAB fat, the combination of PA with either DHA or LA decreased SCD1 and increased AMPKα2 mRNA. CONCLUSIONS: PA-induced insulin resistance in skeletal muscle involves inflammatory (nuclear factor kappa B/mTOR) and nutrient (ceramide) pathways. PUFAs promote pathways, at a transcriptional level, that increase fat oxidation and synergize with factors from SC fat to abrogate PA-induced insulin resistance.

Diagnosis of 5alpha-reductase 2 deficiency: a local experience.

5Alpha-reductase 2 deficiency is an autosomal recessive disorder characterised by lack of masculinisation in XY individuals due to failure to convert testosterone to dihydrotestosterone, the bioactive androgen. Traditionally, the testosterone-to-dihydrotestosterone ratio is used to diagnose this condition but interpreting these results is not always straightforward, thus they may be inconclusive. On the contrary, urinary steroid profiling unambiguously demonstrates a significantly reduced excretion of 5alpha-reduced steroid metabolites compared to their 5beta counterparts. This analytical technique can also simultaneously confirm or rule out other causes of ambiguous genitalia due to steroidogenic defects. Making a DNA-based diagnosis by studying the SRD5A2 gene has become increasingly popular. Here, we report six Chinese patients from different families who were all diagnosed with 5alpha-reductase 2 deficiency based on urinary steroid profile findings and mutational analysis of the SRD5A2 gene. R227Q was the most commonly identified mutation in these patients. Management of sexual development disorders is also discussed.

Difference in long-term clinical outcome after cardiac resynchronisation therapy between ischaemic and non-ischaemic aetiologies of heart failure.

OBJECTIVE: To examine the impact of heart failure (HF) aetiology on long-term outcome after cardiac resynchronisation therapy (CRT). DESIGN: Prospective cohort study. SETTING: University hospital. PATIENTS: 119 patients (44% with ischaemic and 56% non-ischaemic aetiology) who underwent CRT. INTERVENTIONS: Clinical follow-up for 39 (24) months. MAIN OUTCOME MEASURES: Cardiovascular mortality, HF and cardiovascular hospitalisation were compared by Kaplan-Meier curves between the two groups, followed by Cox regression analysis for prognostic predictor(s). RESULTS: 41 (34%) patients died, in whom cardiovascular causes were identified in 32 (27%) patients. The ischaemic group had a higher cardiovascular mortality (log-rank chi(2) = 4.293, p = 0.038) and cardiovascular hospitalisation (log-rank chi(2) = 5.123, p = 0.024) when compared with the non-ischaemic group, though no difference was found in HF hospitalisation (log-rank chi(2) = 0.019, p = 0.892). At three months, left ventricular reverse remodelling occurred in 52% of the ischaemic group and 55% of the non-ischaemic group (chi(2) = 0.128, p = 0.720). By Cox regression analysis, ischaemic aetiology and absence of reverse remodelling at three months were independent predictors of cardiovascular mortality (HR = 2.698, p = 0.032; HR = 3.541, p = 0.030) and cardiovascular hospitalisation (HR = 1.905, p = 0.015; HR = 2.361, p = 0.004). Furthermore, these two factors had an incremental value in predicting cardiovascular mortality when compared with either alone (left ventricular reverse remodelling, log-rank chi(2) = 10.275 vs 6.311, p = 0.05; Ischaemic aetiology, log-rank chi(2) = 10.275 vs 4.293, p<0.05). CONCLUSION: Ischaemic aetiology of HF is an independent predictor of higher cardiovascular mortality and hospitalisation after CRT. This may implicate the progressive nature of coronary heart disease leading to a worse outcome despite similar short-term benefits of CRT.

Left ventricular long axis dysfunction in adults with "corrected" aortic coarctation is related to an older age at intervention and increased aortic stiffness.

OBJECTIVES: This study examined the prevalence of left ventricular (LV) long axis dysfunction (LAD, septal annulus pulsed-wave (PW) tissue Doppler imaging (TDI) early diastolic velocity < or =8 cm/s) in patients with "corrected" aortic coarctation and its relationship to patient demographics and aortic elastic properties. METHODS: A retrospective study of 80 consecutive patients with "corrected" aortic coarctation (aged 27 (SD 6) years, seven postballoon aortoplasty, 41 poststenting and 32 postsurgical repair) was carried out. Patients' ages at intervention, comorbidities and medications were recorded. The LV long axis motions were recorded by M-mode and PW TDI. Aortic stiffness indices were calculated from the aortic diameters and pulse pressures. RESULTS: Forty-seven patients (59%) had LAD. They were older (28 (5) vs 9 (6) years) at treatment, had stiffer aorta (stiffness index 18.4 (6.0) vs 9.2 (2.3)), thicker LV walls (146.7 (59.7) vs 103.8 (44.9) g/m2), higher wall stress (80 (6) vs 70 (7) 10(3) dynes/cm2), larger left atria (31.7 (4.6) vs 24.5 (5.3) ml/m2) and higher LV filling pressures (p<0.01 for all) compared with those without LAD, despite a similar prevalence of antihypertensive use and bicuspid aortic valves. The age at intervention (OR 2.92, 95% CI 1.29 to 6.60, p<0.01) and aortic stiffness index (OR 1.98, 95% CI 1.41 to 2.79, p<0.001) were the two independent predictors for LAD in patients on multivariate analysis. A cut-off age of > or =25 year at intervention was 89% sensitive and 76% specific in predicting LAD (AUC = 0.90, p<0.001). CONCLUSIONS: LAD is common in adults with aortic coarctation despite apparently successful treatment. Its presence is related to older age at intervention and increased aortic stiffness.

Current endovascular therapy for lower extremity peripheral arterial disease: indications, outcomes and modalities.

Atherosclerosis of the lower extremities frequently leads to lifestyle-restricting claudication and can cause critical limb ischaemia (rest pain, non-healing ulcer, or gangrene). The prevalence of peripheral arterial disease (PAD) is rising in line with an ageing population. In the USA, PAD affects 8-10 million people (approximately 12% of the adult population). There is a strong association with concomitant coronary artery and cerebral vascular disease in these patients, which represents a significant cause of mortality and morbidity in patients with PAD. Disease affecting the lower extremity peripheral vessels is most aggressive in smokers and diabetics.

Study of gene-gene interactions for endophenotypic quantitative traits in Chinese asthmatic children.

BACKGROUND: Asthma is a complex disease resulting from interactions between multiple genes and environmental factors. Study of gene-gene interactions could provide insight into the pathophysiology of asthma. METHODS: We investigated the interactions among 18 single-nucleotide polymorphisms in eight candidate genes for plasma total immunoglobulin E (IgE) concentration and peripheral blood (PB) eosinophil count in 298 Chinese asthmatic children and 175 controls. Generalized multifactor dimensionality reduction and generalized linear model were used to analyze gene-gene interactions for the quantitative traits. RESULTS: A significant interaction was found between R130Q in IL13 and I50V in IL4RA for plasma total IgE concentration, with a cross-validation (CV) consistency of nine of 10 and a prediction error of 41.1% (P = 0.013). Plasma total IgE concentration was significantly higher in the high-risk than the low-risk groups (P < 0.0001). For PB eosinophil count, significant interaction was found between C-431T in TARC and RsaI_in2 in FCERIB, with a CV consistency of nine of 10 and a prediction error of 40.2% (P = 0.009). PB eosinophil count was significantly higher in the high-risk group than the low-risk groups (P < 0.0001). Generalized linear model also revealed significant gene-gene interaction for the above two endophenotypes with P = 0.013 for plasma total IgE concentration and P = 0.029 for PB eosinophil count respectively. CONCLUSIONS: Our data suggest significant interactions between IL13 and IL4RA for plasma total IgE concentration, and this is the first report to show significant interaction between TARC and FCERIB for PB eosinophil count in Chinese asthmatic children.

Improvement of left ventricular myocardial short-axis, but not long-axis function or torsion after cardiac resynchronisation therapy: an assessment by two-dimensional speckle tracking.

AIMS: To evaluate whether short-axis function plays a part in determining left ventricular (LV) geometric and functional improvement after cardiac resynchronisation therapy (CRT). METHODS AND RESULTS: 39 patients who received CRT were enrolled. 2D speckle tracking echocardiography was performed at baseline and three months after CRT to assess mean systolic circumferential (epsilon-circum), radial (epsilon-radial) and longitudinal (epsilon-long) strain and torsion. Responders of reverse remodelling (n = 21) had higher baseline mean epsilon-circum than non-responders (p<0.05), who also had improvement in mean epsilon-circum and mean epsilon-radial (both p<0.05) after CRT. Also, the increase in mean epsilon-circum correlated with increase in ejection fraction (r = 0.57, p<0.001) and decrease in mid-cavity width (r = -0.52, p = 0.001). A baseline mean epsilon-circum of >or=6.5% predicted a gain in ejection fraction >or=5%, with a sensitivity of 73% and a specificity of 71%. The baseline epsilon-long was not different between the two groups, and remained unchanged after CRT. The torsion did not improve in responders, but was worsened in non-responders (p<0.05). CONCLUSIONS: The improvement of LV short-axis function but not long-axis function or torsion contributes to the improvement in LV global function and geometry at three-month follow up. A relatively preserved mean epsilon-circum of >or=6.5% might be useful to predict favourable responses after CRT.

Triple-A syndrome--the first Chinese patient with novel mutations in the AAAS gene.

We report on the first Chinese patient with triple-A syndrome, who presented at 22 months with status epilepticus secondary to hyponatraemia and hypoglycaemia. Subsequent endocrine investigations confirmed primary adrenal insufficiency and aldosterone deficiency. In the presence of achalasia and alacrima, this patient satisfies the diagnostic criteria of triple-A syndrome. Further molecular testing detected compound heterozygous mutations in the AAAS gene: a c.580C --> T transition in exon 7 and a c.771delG single nucleotide deletion in exon 8. Testing of parents and brother confirmed their heterozygous carrier status.

An unusual case of non-malignant superior vena cava obstruction.

An 81-year-old Chinese woman presented with a 1-week history of increasing facial puffiness. She had 2:1 second-degree heart block and a permanent pacemaker that had been inserted 3 years previously because of syncopal episodes. Physical examination revealed facial plethora, dilated upper trunk veins, and oedematous upper limbs suggestive of superior vena cava obstruction syndrome. This was confirmed on urgent computed tomographic scan of the thorax. There was no evidence of extrinsic compression or formation of intraluminal thrombus. The underlying aetiology was a pacemaker-induced fibrotic stricture that was successfully treated with balloon venoplasty. At 3-month follow-up, the patient remained symptom-free with normal pacemaker function.