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.

Pseudo-renal failure following total abdominal hysterectomy.

Intraperitoneal urinary bladder perforation should be in the differential diagnosis of acute oliguric renal failure soon after gynecological surgery. We present a case of reversible acute pseudo-renal failure after total abdominal hysterectomy for uterine fibroid. Biochemical features of uremia occur as a result of intraperitoneal extravasation of urine, which is in turn reabsorbed through the peritoneum. Early recognition and surgical repair, as opposed to dialysis therapy, are warranted in such clinical setting. Nephrologists, who are often the first to encounter those patients with presumably acute renal failure, should be aware of this condition. Prompt recovery of the serum biochemistry is to be expected, in contradistinction to genuine renal failure or kidney insults.

Clinico-biochemical prediction of biliary cause of acute pancreatitis in the era of endoscopic ultrasonography.

BACKGROUND: Occult biliary stones escape detection on conventional investigations, and clinico-biochemical systems proposed for predicting biliary pancreatitis has low predictive values. AIM: To evaluate the accuracy of clinico-biochemical parameters for prediction of biliary pancreatitis in patients undergoing endoscopic ultrasonography. METHODS: Early endoscopic ultrasonography was performed on 139 patients presenting with acute pancreatitis within 24 h of admission. The aetiologies of all patients were determined after complete evaluations, and clinico-biochemical characteristics of patients with a biliary cause (biliary group) and non-biliary causes (non-biliary group) were compared. RESULTS: Biliary pancreatitis was diagnosed in 107 patients and 32 patients had non-biliary causes. The biliary group belonged to a significantly older age group, had a female predominance, significantly more derangement of liver function and a higher incidence of severe attack of acute pancreatitis. On multivariate analysis, female sex, age >58 years and serum alanine aminotransferase >150 U/L were independent predictive factors for biliary cause of acute pancreatitis. Using these three factors for prediction of biliary cause, the sensitivity was 93% and overall accuracy was 85%. CONCLUSION: Clinico-biochemical prediction for biliary cause of acute pancreatitis improves in the era of endoscopic ultrasonography with a higher sensitivity and overall accuracy. In centres where endoscopic ultrasonography is inaccessible or local expertise is unavailable, clinico-biochemical prediction of biliary cause of acute pancreatitis may provide a useful alternative in the initial management of this group of patients.

Laparoscopic surgery for common surgical emergencies: a population-based study.

BACKGROUND: Despite being controversial in the past, many reports on the safe use of laparoscopic surgery in emergency settings have been published. The aim of this study was to investigate the diffusion of laparoscopic surgery in three common surgical emergency operations, namely, appendectomy, cholecystectomy, and simple repair of perforated peptic ulcer (PPU), in a stable population. METHODS: This was a retrospective analysis of the central database of the Hospital Authority (HA) in Hong Kong. Data for patients managed in 14 HA hospitals from 1998 to 2002 were studied. The operation record and discharge record of each patient were also investigated to verify the data. RESULTS: A total of 12,708 patients underwent appendectomy, 2631 patients underwent cholecystectomy, and 2260 patients had simple repair of PPU performed. During the study period, 37.2% of appendectomies, 46.5% of cholecystectomies, and 23.1% of simple repairs of PPU were performed laparoscopically. More than a two-fold increase in the proportion of laparoscopic surgery was observed in each of these three operations. By the end of 2002, the percentage of laparoscopic surgery had increased to 53.5% for appendectomies, 61.3% for cholecystectomies, and 32.9% for simple repairs of PPU. Significantly lower hospital mortality rates and shorter postoperative hospital stay were consistenty observed in patients with laparoscopic surgery of the three emergencies. A wide variation in the use of laparoscopic surgery, ranging from 3.7% to 73.1%, was observed among the 14 HA hospitals. However, there was no correlation in the use of laparoscopic surgery with the volume of operation performed in each hospital (p = 0.933). CONCLUSION: A high diffusion rate on the use of laparoscopic surgery for common surgical emergency was observed in Hong Kong. However, there was also a wide variation in the diffusion rate among the 14 HA hospitals. Efforts to reduce hospital variation for the better dissemination of safe laparoscopic technique may be warranted.

Gallbladder cancer presenting with acute cholecystitis: a population-based study.

BACKGROUND: The role of laparoscopic cholecystectomy (LC) in acute cholecystitis remains controversial. The aim of the present study was to determine the incidence, clinicopathological characteristics, and outcome of patients with gallbladder cancer presenting with acute cholecystitis. METHODS: We performed a retrospective analysis of patients with gallbladder cancer who presented with acute cholecystitis and were treated at the public hospitals in Hong Kong between 1998 and 2002. RESULTS: Among 2,700 patients with acute cholecystitis managed with cholecystectomy (1,347 open and 1,353 LC), 63 patients (2.3%) were found to have gallbladder cancer. There were 44 women and 19 men with a mean age of 74.7 (+/-12.8) years. Adenocarcinoma (90.5%) was the most common cancer. The overall median survival was 5 months (95% CI = 2.6-7.4). The 5-year survival rate was 20.8%. Laparoscopic cholecystectomy was attempted in 11 patients and was completed successfully in six of them. There was no difference between the LC and open groups in the complication rate, hospital mortality rate, or survival rate. CONCLUSIONS: In the ethnic Chinese population of Hong Kong, the incidence of gallbladder cancer presenting with acute cholecystitis is higher than the same finding in patients undergoing elective cholecystectomy for cholelithiasis. Long-term survival is possible because such patients may be diagnosed at an early stage of the disease.

Delayed portal vein thrombosis after experimental radiofrequency ablation near the main portal vein.

BACKGROUND: Portal venous blood flow may protect adjacent tumour cells from thermal destruction with radiofrequency ablation (RFA). This study aimed to investigate the local effect of RFA on the main portal vein branch, and the completeness of cellular ablation in its vicinity, with or without a Pringle manoeuvre using a porcine model. METHODS: This was an in vivo study on 23 domestic pigs. RFA using a cooled-tip electrode was performed 5 mm from the left main portal vein branch under ultrasonographic guidance for 12 min with (n = 10) or without (n = 10) a Pringle manoeuvre. Ten pigs were killed 4 h after the procedure to study the early effects of RFA and ten others were killed 1 week later to determine any delayed effect. As a control, sham operations with a Pringle manoeuvre for 12 min were performed on three pigs. The flow velocity changes of portal vein and hepatic artery were measured using Doppler ultrasonography, and the completeness of cellular ablation around the portal vein was assessed qualitatively by histochemical staining and quantitatively by measuring intracellular levels of adenosine 5'-triphosphate (ATP). RESULTS: In the absence of the Pringle manoeuvre, there was no significant change in mean(s.d.) portal vein flow velocity before RFA (20.0(3.5) cm/s) and at 4 h (18.5(2.5) cm/s) (P = 0.210) and 1 week (19.5(2.2) cm/s) (P = 0.500) after the procedure. Gross and histological examination of the portal vein branches showed no damage without the Pringle manoeuvre. In all pigs that underwent RFA with a Pringle manoeuvre, the portal vein was occluded 1 week after the operation; histological examination of the affected portal vein showed severe thermal injury and associated venous thrombosis. The local effect of RFA on the hepatic artery was similar. With intact portal blood flow during RFA, complete ablation of liver tissue around the pedicle was demonstrated by histochemical staining and measurement of the intracellular ATP concentration. CONCLUSION: RFA was safe when applied close to the main portal vein branch without a Pringle manoeuvre, with complete cellular destruction. Use of the Pringle manoeuvre resulted in delayed portal vein and hepatic artery thrombosis and injury to the hepatic artery and bile duct.

SIADH associated with ovarian immature teratoma: a case report.

Immature teratoma in association with SIADH is rare. A 17-year-old patient presented with a pelvic mass and serum sodium concentration of 121 mmol/l. Subsequent investigation confirmed SIADH and grade 2 ovarian immature teratoma. No other causes of SIADH were found apart from the immature teratoma. There was no further recurrence of SIADH after the curative surgery and chemotherapy. We postulate that immature teratoma consists of neurohypophyseal structures which account for the ADH release.

Different presentation of hepatitis B-related hepatocellular carcinoma in a cohort of 1863 young and old patients - implications for screening.

AIM: To compare the clinico-pathological features of hepatitis B virus-related hepatocellular carcinoma in young and old patients. METHODS: The clinico-pathological characteristics of hepatitis B virus-related hepatocellular carcinoma were compared in 1863 consecutive patients (121 patients, 40 years) seen at a single institution over the last 13 years. RESULTS: Young patients presented more often with pain (P < 0.0001), hepatomegaly (P = 0.01) and ruptured hepatocellular carcinoma (P = 0.02), whereas old patients presented with ankle oedema (P = 0.001), ascites (P = 0.002) and by routine screening (P = 0.035). Liver function, Child-Pugh grading and indocyanine green test were better preserved in young patients. They also had a higher alpha-foetoprotein concentration (P = 0.001), larger tumour size (P = 0.001) and more frequent metastasis (P = 0.008), but a similar surgical resection rate (33.6% vs. 28%), to old patients. There was no difference between the two groups in the overall post-resection survival rate, but there was a shorter survival in young patients with unresectable disease (3.6 months vs. 4.6 months, P = 0.004). Young patients with hepatocellular carcinoma often show a later presentation, but a higher resectability rate and similar survival rates, than old patients. The screening programme should include young hepatitis B virus carriers, even in the absence of cirrhosis.

Validation of POSSUM scoring systems for audit of major hepatectomy.

BACKGROUND: The aim of the study was to validate the use of Physiological and Operative Severity Score in the enUmeration of Mortality and morbidity (POSSUM) and Portsmouth (P) POSSUM scoring systems to predict postoperative mortality in a group of Chinese patients who had a major hepatectomy for hepatocellular carcinoma. METHODS: A retrospective analysis was performed on data collected prospectively over a 6-year interval from January 1997 to December 2002. The mortality risks were calculated using both the POSSUM and the P-POSSUM equations. RESULTS: Two hundred and fifty-nine patients underwent major hepatectomy; there were 17 (6.6 per cent) postoperative deaths. Of 32 preoperative and intraoperative variables studied, age, smoking habit, serum creatinine concentration, American Society of Anesthesiologists grade, and physiological and operative severity scores were found to be significant factors predicting mortality. On multivariate analysis only the physiological and operative severity scores were independent variables. The POSSUM system overpredicted mortality risk (14.2 per cent) and there was a significant lack of fit in these patients (chi(2) = 14.1, 3 d.f., P = 0.003). The mortality rate predicted by P-POSSUM was 4.2 per cent and showed no significant lack of fit (chi(2) = 7.6, 3 d.f., P = 0.055), indicating that it predicted outcome effectively. A new logistic equation was derived from the present patient data set that requires testing prospectively. CONCLUSION: P-POSSUM significantly predicted outcome in Chinese patients who had major hepatectomy for hepatocellular carcinoma. A modified disease-specific equation requires further testing.

Impact of radiofrequency ablation on the management of patients with hepatocellular carcinoma in a specialized centre.

BACKGROUND: Radiofrequency ablation (RFA) has been used increasingly in the treatment of hepatocellular carcinoma (HCC). The aim of this study was to investigate changes in the treatment pattern of primary HCC following the implementation of RFA in a specialized surgical centre. METHODS: This was a retrospective analysis of all 894 patients admitted for treatment of primary HCC over 36 months. RESULTS: There was no difference in the age, sex ratio, liver function according to Child-Pugh grade, serum alpha-fetoprotein concentration, hepatitis B surface antigen status and tumour size among patients before and after the introduction of RFA therapy. Fifty-one patients (10.6 per cent) with primary HCC received RFA treatment after its implementation. There was a 6.8 per cent reduction in the number of patients who had supportive treatment (P = 0.041) and a 3.2 per cent reduction in surgical treatment. The hospital mortality rates for RFA and surgery were 2.0 and 4.9 per cent respectively. The overall survival rates at 6, 12 and 18 months for patients treated with RFA were 92.2, 73.4 and 61.2 per cent respectively. The corresponding survival rates for the 213 patients who had surgery were 88.0, 77.0 and 71.5 per cent. These values were no different from those in patients who had RFA (P = 0.718). Patients treated with RFA or surgery survived longer than those who had other treatments. CONCLUSION: RFA had a significant impact on the management of primary HCC, increasing the number of patients suitable for liver-directed therapy and leading to survival benefit. RFA may become the treatment of choice for patients with irresectable HCC.

Prevalence and characteristics of familial hepatocellular carcinoma caused by chronic hepatitis B infection in Hong Kong.

BACKGROUND: Hepatitis B virus infection is an important aetiological factor for hepatocellular carcinoma. Clusters of hepatocellular carcinoma have been observed in families infected with hepatitis B virus. AIM: To investigate the prevalence and characteristics of hepatocellular carcinoma associated with familial hepatitis B virus in Hong Kong. METHODS: Hepatitis B virus patients were screened for familial hepatocellular carcinoma using a standardized questionnaire. The clinical features of patients with familial hepatocellular carcinoma were compared with those of 118 patients with sporadic hepatocellular carcinoma attending the clinic during the same period. RESULTS: A total of 5080 patients were interviewed. Validation of the questionnaire indicated that the reliability was high. There were 22 families with familial hepatocellular carcinoma, giving a prevalence of 4.3 families/1000 hepatitis B virus carriers. The mean age of onset was 48.5 +/- 13 years in familial hepatocellular carcinoma and 62 +/- 11 years in sporadic hepatocellular carcinoma (P = 0.005). The ages of onset were 59 +/- 11, 40 +/- 10 and 18 +/- 4 years in the first, second and third generations, respectively (P < 0.0001), suggesting an anticipation phenomenon. Familial hepatocellular carcinoma patients were more likely to present with pain (70% vs. 10%, P < 0.0001), but not on routine screening (14% vs. 52%, P < 0.0001), than sporadic hepatocellular carcinoma patients. CONCLUSION: The prevalence of familial hepatocellular carcinoma is significant in Hong Kong. These patients show specific clinical features when compared with patients with sporadic hepatocellular carcinoma.