Laryngological presentations and patient-reported outcome measures in Ehlers-Danlos syndrome.

OBJECTIVE: This study aimed to characterise the laryngological presentations of Ehlers-Danlos syndrome and conduct a preliminary exploration of patient-reported outcome measures. METHODS: This paper describes a retrospective case series of patients with Ehlers-Danlos syndrome seen by the senior author between 2005 and 2019. A literature review was conducted to summarise the existing findings. RESULTS: Twenty-one patients met the inclusion criteria. All reported symptoms were grouped; this showed that swallowing, voice and hyolaryngeal skeletal complex problems were commonest. Patient-reported outcome measures were available for eight patients, which showed large variations in: the Reflux Severity Index (median = 25.5; range = 0-33), Eating Assessment Tool score (median = 21.5; range = 0-35) and Voice Handicap Index (median = 21.5; range = 0-104). Twelve studies met our literature review inclusion criteria, involving at least 91 patients with laryngological presentations of Ehlers-Danlos syndrome. CONCLUSION: Ehlers-Danlos syndrome patients experience musculoskeletal issues, which in the throat manifest as hyolaryngeal skeletal complex problems. Future studies with larger patient numbers are required to validate laryngological patient-reported outcome measure tools in Ehlers-Danlos syndrome.

Using positive youth development constructs to design a money management curriculum for junior secondary school students in Hong Kong.

This paper aims to discuss the relationships between the selected positive youth development constructs and the enhancement of Hong Kong junior secondary school students' money management skills, values, and attitudes. Various issues of money management of adolescents are reviewed. These issues include the need for money management programs for adolescents, the content and coverage of an appropriate money management program, and its relationships with the selected positive youth development constructs. The curriculum units for secondary 3 students are taken as examples to illustrate the design of the program. It is believed that promoting cognitive competence, self-efficacy, and spirituality could be an effective way to enhance students' money management skills, values, and attitudes, thus preparing them better for facing the finance-related issues in life.

Outcome after partial hepatectomy for hepatocellular cancer within the Milan criteria.

BACKGROUND: There is a trend to offer liver transplantation to patients with hepatocellular carcinoma (HCC) with tumour status within the Milan criteria but with preserved liver function. This study aimed to evaluate the outcome of such patients following partial hepatectomy as primary treatment. METHODS: A retrospective analysis was performed on all adult patients with HCC and tumour status within the Milan criteria undergoing partial hepatectomy at a single centre from 1995 to 2008. Their outcomes were compared with those of similar patients having right-lobe living donor liver transplantation (LDLT) as primary treatment. RESULTS: A total of 408 patients with HCC were enrolled. Some 384 patients with a solitary tumour 5 cm or less in diameter had a better 5-year survival rate than 24 patients with oligonodular tumours (2-3 nodules, each 3 cm or less in size) (70·7 versus 46 per cent; P = 0·025). Multivariable analysis identified younger age (65 years or less), lack of postoperative complications, negative resection margin, absent microvascular invasion and non-cirrhotic liver as predictors of favourable overall survival. The 5-year survival rate of 287 younger patients with chronic liver disease and R0 hepatectomy was 72·8 per cent, comparable to that of 81 per cent in 50 similar patients treated by LDLT (P = 0·093). CONCLUSION: Partial hepatectomy for patients with HCC and tumour status within the Milan criteria achieved a satisfactory 5-year survival rate, particularly in younger patients with solitary tumours and R0 hepatectomy. Patients with oligonodular tumours have a worse survival and might benefit from liver transplantation.

Population-based information on emergency colorectal surgery and evaluation on effect of operative volume on mortality.

INTRODUCTION: Population-based information on emergency surgery for colorectal conditions was limited. The present study was designed to review comprehensively the outcomes of emergency colectomy in Hong Kong population and evaluate the effect of case load on mortality after considering case mixes. METHODS: All adult patients older than aged 18 years who underwent emergency colectomy in 2003 in all 15 Hong Kong public hospitals were included. Demographics and perioperative variables were reviewed and analyzed. Hospitals were classified into low, middle, or high operative volume groups according to their yearly caseloads, and their performance in terms of mortality were compared by using CR-POSSUM. RESULTS: A total of 864 patients with a mean age of 67.8 years were included. Bowel obstruction and peritonitis were the two major clinical indications for the surgery. The crude in-hospital mortality rate was 18.9%; individual hospitals varied from 8.7% to 33.3%. With the risk adjusted model, all hospital groups performed within 95% confidence limits of prediction. There was no statistical difference for mortality of hospitals of different case volume. CONCLUSION: A crude in-hospital mortality of 18.9% after emergency colorectal surgery was observed in Hong Kong public hospitals. Higher hospital case load is not significantly associated with better outcomes in emergency colectomy.

Effects of cognitive-behavioural therapy on anxiety for children with high-functioning autistic spectrum disorders.

INTRODUCTION: Children with autistic spectrum disorders (ASD) often exhibit one or more comorbid disorders, including anxiety, disruptive behaviour, mental retardation, and depression. Various studies have documented the effectiveness of cognitive-behavioural therapy (CBT) in treating children with anxiety. Although studies have indicated a high prevalence of anxiety in individuals with ASD, there is a lack of systematic studies substantiating the effectiveness of cognitive-behavioural interventions among children with high-functioning autism. METHODS: This pilot study investigated the effects of a 16-session CBT programme on six high-functioning children diagnosed with ASD (mean age 11.50 years, standard deviation 0.84 years). These children were diagnosed with ASD or Asperger's syndrome by the DSM-IV criteria. Measures on levels of child's anxiety, parental and teacher stress were administered at pre- and post-treatment. RESULTS: Children showed lower levels of anxiety at post-treatment. Parents and teachers also reported lower levels of stress following the CBT programme. CONCLUSION: Findings from the present study provided some evidence of the effects of CBT for high-functioning autistic children in reducing anxiety, parental and teacher stress. Interpretation of the findings, recommendations for future research and implications of the present study are presented.

The impact of psychological abuse by an intimate partner on the mental health of pregnant women.

OBJECTIVE: The objective of this first population-based study in Hong Kong was to assess the impact of psychological abuse by an intimate partner on the mental health of pregnant women. DESIGN: Survey. SETTING: Antenatal clinics in seven public hospitals in Hong Kong. POPULATION: Three thousand two hundred and forty-five pregnant women. METHODS: The Abuse Assessment Screen (AAS) and demographic questionnaires were administered face-to-face at 32-36 weeks of gestation. At 1 week postpartum, the AAS, Edinburgh Postnatal Depression Scale and SF-12 Health Survey were administered by telephone. MAIN OUTCOME MEASURES: Intimate partner violence, postnatal depression and health-related quality of life. RESULTS: Two hundred and ninety six (9.1%) of the participants reported abuse by an intimate partner in the past year. Of those abused, 216 (73%) reported psychological abuse only and 80 (27%) reported physical and/or sexual abuse. Forty six (57.5%) in the physical and/or sexual abuse group also reported psychological abuse. Women in the psychological abuse only group had a higher risk of postnatal depression compared with nonabused women (adjusted OR: 1.84, 95% CI: 1.12-3.02). They were also at a higher risk of thinking about harming themselves (adjusted OR: 3.50, 95% CI: 1.49-8.20) and had significantly poorer mental health-related quality of life (P < 0.001). The higher risks of postnatal depression and thinking of harming themselves were not observed in the physical and/or sexual abuse group although significantly poorer mental health-related quality of life (P < 0.001) was observed. CONCLUSIONS: Psychological abuse by an intimate partner against pregnant women has a negative impact on their mental health postdelivery. Furthermore, psychological abuse in the absence of physical and/or sexual abuse can have a detrimental effect on the mental health of abused women. The findings underscore the importance of screening pregnant women for abuse by an intimate partner and the need for developing, implementing and evaluating interventions to address psychological abuse.

Improved operative and survival outcomes of surgical treatment for hilar cholangiocarcinoma.

BACKGROUND: The aim of the present study was to assess whether an aggressive surgical approach in the management of patients with hilar cholangiocarcinoma was associated with improved operative and survival outcomes. METHODS: Eighty-two patients with hilar cholangiocarcinoma treated between 1989 and 1998 (period 1), and 60 patients treated between 1999 and 2004 (period 2), were evaluated. Modifications to management in period 2 primarily included percutaneous biliary drainage instead of endoscopic drainage for relief of obstructive jaundice, preoperative right portal vein embolization before right-sided hepatectomy, routine total caudate lobectomy and radical lymphadenectomy during surgical resection of the tumour. RESULTS: The surgical resection rate was significantly higher in period 2 than in period 1 (45 versus 16 per cent; P < 0.001). All patients in period 2 underwent major hepatectomy with concomitant caudate lobectomy. The operative morbidity and hospital mortality rates decreased significantly in period 2 compared with period 1, with significantly better survival outcomes. In multivariate analysis, resection of the tumour in period 2 and operative blood loss of 1.5 litres or less were the significant independent factors associated with improved survival. CONCLUSION: An aggressive surgical approach was associated with improved operative and survival outcomes for patients with hilar cholangiocarcinoma.

Efficacy and safety of radiofrequency ablation for perivascular hepatocellular carcinoma without hepatic inflow occlusion.

BACKGROUND: The role of radiofrequency ablation (RFA) for perivascular (up to 5 mm from the major intrahepatic portal vein or hepatic vein branches) hepatocellular carcinoma (HCC) is unclear because of possible incomplete tumour ablation and potential vascular damage. This study aimed to evaluate the safety and efficacy of RFA for perivascular HCC without hepatic inflow occlusion. METHODS: Between May 2001 and November 2003, RFA using an internally cooled electrode was performed on 52 patients with perivascular HCC (group 1) through open (n = 39), percutaneous (n = 9), laparoscopic (n = 2) and thoracoscopic (n = 2) approaches. Hepatic inflow occlusion was not applied during the ablation procedure. The perioperative and postoperative outcomes were compared with those of 90 patients with non-perivascular HCC (group 2) treated by RFA during the same period. RESULTS: The morbidity rate was similar between groups 1 and 2 (25 versus 28 per cent; P = 0.844). One patient in group 1 (2 per cent) and two in group 2 (2 per cent) had developed thrombosis of major intrahepatic blood vessels on follow-up computed tomography scan. There were no significant differences between groups 1 and 2 in mortality rate (2 versus 0 per cent; P = 0.366), complete ablation rate for small HCC (92 versus 98 per cent; P = 0.197), local recurrence rate (11 versus 9 per cent; P = 0.762) and overall survival (1-year: 86 versus 87 per cent; 2-year: 75 versus 75 per cent; P = 0.741). CONCLUSION: RFA without hepatic inflow occlusion is a safe and effective treatment for perivascular HCC.

Evaluation of outcome of laparoscopic colorectal resection with POSSUM, Portsmouth POSSUM and colorectal POSSUM.

BACKGROUND: This study evaluated the Physiological and Operative Severity Score for the enUmeration of Mortality and morbidity (POSSUM), Portsmouth (P) POSSUM and colorectal (CR) POSSUM in laparoscopic colorectal resection. METHODS: Observed mortality and morbidity rates in 400 patients who underwent laparoscopic colorectal resection were compared with those predicted by POSSUM, P-POSSUM and CR-POSSUM. RESULTS: Observed mortality and morbidity rates were 0.5 and 19.0 per cent respectively. Mortality rates predicted by POSSUM, P-POSSUM and CR-POSSUM were 10.8, 4.0 and 5.6 per cent respectively, and the morbidity rate predicted by POSSUM was 43.0 per cent. The predicted and observed mortality and morbidity rates showed significant lack of fit. The conversion rate to open surgery was 11.5 per cent. The mortality rate for patients having conversion was 2 per cent and was not significantly different to that predicted by P-POSSUM (4 per cent; P = 0.493) or CR-POSSUM (5 per cent; P = 0.370). In this group, the observed and POSSUM-predicted morbidity rates were also similar (43 versus 48 per cent respectively; P = 0.104). CONCLUSION: POSSUM, P-POSSUM and CR-POSSUM overestimated mortality and morbidity in patients who underwent laparoscopic colorectal resection. However, the mortality rate in patients who required conversion fitted the models of P-POSSUM and CR-POSSUM, and the morbidity rate was comparable to that predicted by POSSUM.