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This article explores the relationship between cognitive impairment and functional limitations in individuals with haemophilia in Hong Kong. The study reveals that patients who experience difficulties with memory, attention, and executive functioning tend to have poorer performance at work or school. These cognitive functions are crucial for setting and achieving goals, as well as adapting to one's surroundings, so deficiencies in these areas can impact occupational or educational performance. The findings suggest that regular monitoring of cognitive function should be integrated into the standard care for haemophilia patients, particularly those facing occupational or vocational challenges. Further research is necessary to develop a cost-effective and clinically feasible method for monitoring cognitive function in these individuals. The study investigates how cognitive impairment affects functional limitations in haemophilia patients. The researchers discover that impairments in attention, motor processing speed, and cognitive flexibility are linked to a decline in functional status. The study proposes that regular screening for cognitive deficits and cognitive rehabilitation may be necessary for this population. However, the study has limitations, such as potential selection bias and the absence of a control group. Further research is needed to validate the findings and assess the long-term consequences of functional limitations in haemophilia patients. [Extracted from the article]

Background: Few studies have evaluated the impact of subclinical microstructural changes and psychosocial factors on cognitive function in patients with haemophilia. Objectives: To determine the prevalence and characteristics of cognitive impairment in patients with haemophilia, and identify associated risk factors. Methods: We recruited haemophilia A or B patients who were aged ≥10 years old from three public hospitals in Hong Kong. A neurocognitive battery was administered to evaluate their attention, memory, processing speed and cognitive flexibility performances. They also underwent magnetic resonance imaging to identify cerebral microbleeds. Validated self‐reported questionnaires were administered to assess their mental health status and adherence to prophylactic treatment. General linear modelling was used to investigate the association of neurocognitive outcomes with risks factors, adjusting for age and education attainment. Results: Forty‐two patients were recruited (median age 32.0 years; 78.6% haemophilia A; 80.9% moderate‐to‐severe disease). Six patients (14.3%) had developed cerebral microbleeds. A subgroup of patients demonstrated impairments in cognitive flexibility (30.9%) and motor processing speed (26.2%). Hemarthrosis in the previous year was associated with worse attention (Estimate = 7.62, 95% CI: 1.92–15.33; p =.049) and cognitive flexibility (Estimate = 8.64, 95% CI: 2.52–13.29; p =.043). Depressive (Estimate = 0.22, 95% CI: 0.10–0.55; p =.023) and anxiety (Estimate = 0.26, 95% CI: 0.19–0.41; p =.0069) symptoms were associated with inattentiveness. Among patients receiving prophylactic treatment (71.4%), medication adherence was positively correlated with cognitive flexibility (p =.037). Conclusion: A substantial proportion of patients with haemophilia demonstrated cognitive impairment, particularly higher‐order thinking skills. Screening for cognitive deficits should be incorporated into routine care. Future studies should evaluate the association of neurocognitive outcomes with occupational/vocational outcomes. [ABSTRACT FROM AUTHOR]

Background Few studies have evaluated the medication burden borne by survivors of pediatric cancer. This study aimed to describe the drug utilization pattern of chronic medications in a cohort of young pediatric cancer survivors. Methods This was a population-based study of patients diagnosed with cancer at age 18 years or younger between 2000 and 2013 in Hong Kong and who had survived at least 5 years postdiagnosis. The primary outcome is the use of any chronic medication (medications that were prescribed for ≥30 consecutive days within a 6-month period). Multivariable log-binomial models were used to identify factors associated with chronic medication use. Kaplan-Meier analysis was used to present the cumulative proportion of survivors initiated on a chronic medication across time from cancer diagnosis. Results Of the 2444 survivors (median age = 22 years, interquartile range = 16-27 years), 669 (27.4%) required at least 1 chronic medication at least 5 years postdiagnosis. Survivors who developed a chronic health condition (CHC) had a 5.48 (95% confidence interval [CI] = 4.49 to 6.71) times higher risk of taking a chronic medication than those without CHC. At 10 years postdiagnosis, the cumulative proportion of survivors being initiated a chronic medication was 33.4% (95% CI = 31.1% to 35.6%) for the overall cohort. Higher cumulative proportions were observed in survivors with endocrine (74.6%, 95% CI = 68.4% to 79.6%), renal (68.8%, 95% CI = 54.2% to 78.7%), neurological (58.6%, 95% CI = 46.1% to 68.1%), and cardiovascular (54.7%, 95% CI = 44.0% to 63.4%) disorders. Conclusion Survivors with certain CHCs had a higher risk of starting a prescription medication in the early phase of survivorship. Future studies include examining the impact of medication burden on survivors' functional status. [ABSTRACT FROM AUTHOR]

Objective: Anticancer treatment may be associated with damage to the reproductive organs and risk of infertility in children with cancer. The collectivist cultural norms of Asian societies may lead Chinese parents to have unique concerns regarding infertility. This qualitative study explored the perceptions of infertility risk and parenthood among parents of childhood cancer survivors in Hong Kong. Methods: Thirteen parents were recruited via a snowball sampling approach from a nongovernmental organization in Hong Kong, representing nine survivors of childhood cancer (leukemia n = 5, solid tumors n = 4). The in-depth semistructured interviews were audiotaped and transcribed verbatim. A thematic analysis was performed using ATLAS.ti 8. Results: Parents mostly perceived their children's fertility status as a distant concern (n = 11, 85%) but emphasized the need for timely information from clinicians when their children reach young adulthood (n = 8, 62%). They reported receiving inconsistent fertility information from different oncology practitioners (n = 9, 69%). A few parents acknowledged that under the influence of the Chinese culture, their children, especially sons, have an important duty to continue the family lineage. However, even if the cancer treatment were associated with infertility risk, almost all parents (n = 12, 92%) stated that it would still not affect their willingness to let their child undergo treatment because survival and cure were still their highest priority. Conclusion: Our findings suggest the need to proactively provide fertility information to parents both during active treatment and when survivors reach reproductive age. Future studies should evaluate the benefits of developing culturally relevant decision-making aids to address parents' informational needs regarding fertility issues. [ABSTRACT FROM AUTHOR]

Objective: To delineate the prevalence and associated risk factors of low BMD, osteoporosis/bone fragility and fracture in transfusion‐dependent thalassemia (TDT) in the Chinese population in Hong Kong. Design, Patients and Measurements: A retrospective cohort study design was employed. Patients of TDT who had serial Hologic dual‐energy X‐ray absorptiometry (DXA) from 2010 to 2016 and received regular transfusion for at least 5 years were recruited. Clinical and biochemical data, from 5 years before the first DXA scan, were retrieved from the electronic record system of the Hospital Authority, till 30 June 2020. Low bone mineral density and osteoporosis/bone fragility are defined by the ISCD 2019 position guidelines. Results: Seventy‐seven patients were included in the analysis. The fracture prevalence of TDT among the Chinese population in Hong Kong was 15.58%. Up to 55.84% of patients had low bone mineral density, and 5.19% patients had osteoporosis/bone fragility state. The median age at first fracture was 31.73 years (range 24.06–44.18 years). In the regression analysis, a higher log(10) transformation of average ferritin levels over 5 years before the first DXA scan was significantly associated with fracture occurrence regardless of bisphosphonate treatment (OR 310.73, 95% CI 3.99–24183.89, p =.010). Mean average ferritin level over 5 years was 6695.5 ± 2365.7 pmol/L (fracture group) versus 4350.7 ± 3103.2 pmol/L (non‐fracture group), p =.016. Hip and spine BMD Z‐score did not have statistically significant association with fracture occurrence. Conclusion: Iron overloading plays an important role in adverse bone health in TDT. Dual X‐ray densitometry is insufficient in predicting fracture risk. [ABSTRACT FROM AUTHOR]

Background: For survivors of childhood cancer, awareness of personal health risks is a critical component of long‐term health management. Objective: To evaluate the awareness of the diagnosis, treatment and risk of late effects among survivors of childhood cancer in Hong Kong. Methods: Between June 2019 and March 2020, this cross‐sectional study recruited 155 adult survivors (mean age = 26.9, standard deviation [SD] = 6.4 years) and 45 parents of paediatric survivors (mean age = 11.1, SD = 3.6 years) from a long‐term follow‐up clinic. At >10 years post‐treatment (mean = 13.4, SD = 7.6 years), they completed a structured questionnaire to report their cancer‐specific knowledge. Multiple linear regression analysis was conducted to identify clinical, socioeconomic and behavioural factors associated with poor awareness. Results: The majority of participants accurately recalled their diagnoses (73.5%) and major treatment modalities (chemotherapy 92.4%, radiation 82.9% and surgery 88.2%). However, less than half (45%) of the participants recognized more than 25% of the total late effects for which they were at risk. The highest levels of awareness were reported for endocrine problems (49%), neurocognitive impairment (44%) and secondary cancers (43%), and the lowest for peripheral neuropathy (21%) and vision problems (23%). Compared with survivors of haematological malignancies, those of central nervous system (CNS) tumours (standardized estimate [B] = −9.33, 95% confidence interval [95% CI]: −13.41 to −5.26) and non‐CNS solid tumours (B = −8.47, 95% CI: −12.39 to −4.94) had less knowledge about their diagnosis. Retaining medical records (P <.0001) and better medical information‐seeking habits (P =.048) were associated with better awareness. Conclusions: Survivors of childhood cancer in Hong Kong have deficient awareness of their personal health risks. They may benefit from the provision of a survivorship care plan and personalized education regarding treatment‐related late effects. Patient Contribution: Patients contributed in designing the study tools. Results were presented at a non‐governmental organization. [ABSTRACT FROM AUTHOR]

Background: Evidence on self-care ability and occupational outcomes in Chinese survivors of childhood cancer is clearly lacking. This study aims to identify clinical and behavioral factors associated with poor life functioning in this population. Methods: This was a cross-sectional study conducted at an ambulatory clinic of a public hospital in Hong Kong. Licensed occupational therapists administered the Life Functioning Assessment Inventory on survivors diagnosed with cancer <19 years old and ≥5 years post-diagnosis. Survivors' career development self-efficacy, subjective happiness, and motivation were evaluated using structured questionnaires. Clinical information was obtained from medical records. Multivariable linear regression was used to evaluate factors associated with life functioning outcomes, adjusting for clinically relevant variables. Results: Eighty survivors were recruited (58.7% male; age at diagnosis: 6.7 [standard deviation (SD) = 4.8] years; age at evaluation: 24.4 [SD = 6.5] years). Compared to survivors of leukemia, survivors of brain tumor performed worse in social functioning (β = −0.79, standard error [SE] = 0.36; p = 0.034). Survivors who had been treated with cranial radiation also had lower worker life functioning than those who had not (β = −0.91, SE = 0.031; p = 0.021). Higher activity motivation was significantly associated with better leisure functioning (β = 0.086, SE = 0.03; p = 0.008), social functioning (β = 0.036, SE = 0.02; p = 0.036), and career development self-efficacy (β = 1.04, SE = 0.26; p < 0.0001). Conclusion: Survivors of brain tumors and survivors who were treated with radiation have poorer life functioning, particularly in social and work domains. Future work includes validating the study findings in a larger cohort of survivors in Hong Kong. Addressing modifiable behavioral factors include motivating survivors to engage in meaningful activities that contribute to self-care and participation in society, as well as providing at-risk survivors with ongoing support from community vocational services. [ABSTRACT FROM AUTHOR]

Background: Increasing attention has been dedicated to investigate modifiable risk factors of late effects in survivors of childhood cancer. This study aims to evaluate neurocognitive and behavioral functioning in a relatively young cohort of survivors of childhood acute lymphoblastic leukemia (ALL) in Hong Kong, and to identify clinical and socio-environmental factors associated with these outcomes. Methods: This analysis included 152 survivors of childhood ALL who were ≥5 years post-diagnosis (52% male, mean [SD] age 23.5[7.2] years at evaluation, 17.2[7.6] years post-diagnosis). Survivors completed performance-based neurocognitive tests, and reported their emotional and behavioral symptoms using the Child/Adult Behavior Checklist. Socio-environmental variables (living space, fatigue, physical activity, family functioning, and academic stress) were self-reported using validated questionnaires. Clinical variables and chronic health conditions were extracted from medical charts. Multivariable linear modeling was conducted to test identify factors associated with neurocognitive/behavioral outcomes, adjusting for current age, sex, age at diagnosis and cranial radiation. An exploratory mediation analysis was performed to examine the mediating effects of risk factors on neurocognitive and behavioral outcomes. Results: As compared to population norms, a minority of survivors developed mild-moderate impairment in motor processing speed (36.2%), memory (9.2%) and attention measures (4.0%-10.5%). Survivors also reported attention problems (12.5%), sluggish cognitive tempo (23.7%) and internalizing (depressive, anxiety and somatic symptoms) problems (17.1%). A minority of survivors developed mild-moderate treatment-related chronic conditions (n=37, 24.3%). As compared to survivors without chronic conditions, survivors with chronic conditions had more executive dysfunction (B=5.09, standard error [SE]=2.05; P =0.014) and reported more attention problems (B=5.73, SE=1.43; P <0.0001). Fatigue and poor family functioning was associated with multiple measures of behavior problems (all P <0.001). A lower level of physical activity was correlated with more self-reported symptoms of inattention (B= -1.12, SE=0.38, P =0.004) and sluggish cognitive tempo (B=-1.22, SE=0.41, P =0.003). Exploratory analysis showed that chronic health conditions were associated with behavioral measures through fatigue as the mediator. Conclusion: The majority of young Chinese survivors of ALL had normal cognitive and behavioral function. Regular monitoring of behavioral function should be performed on survivors who develop treatment-related chronic conditions. Health behavior and socio-environment factors may be potentially modifiable risk factors associated with health outcomes in survivors. [ABSTRACT FROM AUTHOR]

Background: As survivors of childhood cancer age, development of cancer treatment-related chronic health conditions often occur. This study aimed to describe the pattern of chronic prescription medication use and identify factors associated with polypharmacy among survivors of childhood cancer. Methods: This was a retrospective study conducted at the pediatric oncology long-term follow-up clinic in Hong Kong. Eligible subjects included survivors who were (1) diagnosed with cancer before 18 years old, (2) were at least 3 years post-cancer diagnosis and had completed treatment for at least 30 days, and (3) receiving long-term follow-up care at the study site between 2015 and 2018. Dispensing records of eligible survivors were reviewed to identify medications taken daily for ≥30 days or used on an "as needed" basis for ≥6 months cumulatively within the past 12-month period. Polypharmacy was defined as the concurrent use of ≥5 chronic medications. Multivariable log-binomial modeling was conducted to identify treatment and clinical factors associated with medication use pattern and polypharmacy. Results: This study included 625 survivors (mean current age = 17.9 years, standard deviation [SD] = 7.2 years) who were 9.2 [5.2] years post-treatment. Approximately one-third (n = 219, 35.0%) of survivors were prescribed at least one chronic medication. Frequently prescribed medication classes include systemic antihistamines (26.5%), sex hormones (19.2%), and thyroid replacement therapy (16.0%). Overall prevalence of polypharmacy was 5.3% (n = 33). A higher rate of polypharmacy was found in survivors of CNS tumors (13.6%) than in survivors of hematological malignancies (4.3%) and other solid tumors (5.3%) (P =.0051). Higher medication burden was also observed in survivors who had undergone cranial radiation (RR = 6.31; 95% CI = 2.75–14.49) or hematopoietic stem-cell transplantation (HSCT) (RR = 3.53; 95% CI = 1.59–7.83). Conclusion: Although polypharmacy was observed in a minority of included survivors of childhood cancer, chronic medication use was common. Special attention should be paid to survivors of CNS tumors and survivors who have undergone HSCT or cranial radiation. These individuals should be monitored closely for drug–drug interactions and adverse health outcomes that may result from multiple chronic medications, particularly during hospitalization in an acute care setting. [ABSTRACT FROM AUTHOR]

Introduction: Langerhans cell histiocytosis (LCH) is a rare disease with diverse clinical courses. Despite improvement in survival outcomes in the recent decades, sequelae of the disease remain a concern. This study aimed to provide information on the long-term outcomes in patients with LCH, particularly on the sequelae and any associated factors.Method: Medical records of patients with diagnosis of LCH and being managed in our centre were retrospectively reviewed. Data on the courses of illness, mortality, intervention, types and time of late events were collected and analysed.Results: 70 patients were included with a mean observation time of 12 years (median 10.7 years, range 1-31.3 years). Sequelae related to LCH were present in 56% (n=39), being more common in multisystem diseases and patients with reactivations. Prevalence of sequelae is as follows: orthopaedic related 27%, diabetes insipidus 19%, growth retardation 13%, cosmetic 10%, neurological 7%, hearing 7%, anterior pituitary hormone deficiency 7%, hepatobiliary 4% and ophthalmological 3%. Neurological sequelae could manifest even 10 years after initial diagnosis of LCH. Reactivations, presence of central nervous system (CNS) risk lesions and treatment with radiotherapy were associated with a higher rate of sequelae. The cumulative incidence of reactivation was 34%. Most reactivations occurred in the first 2.5 years after diagnosis.Conclusion: Sequelae were common after LCH, although some were mild. Neurological sequelae could be particularly severe and debilitating. Vigilant long-term follow-up would be essential for optimising patient outcomes. Further studies on the prevention and treatment of CNS disease of LCH are warranted. [ABSTRACT FROM AUTHOR]

The hypothesis that protection of infants from exposure to infectious agents with delayed first exposure to one or more specific agents together contribute to the aetiology of childhood leukaemia, especially common acute lymphoblastic leukaemia (cALL), has substantial indirect support from descriptive epidemiology and case-control studies in developed Western countries. A case-control study of childhood leukaemia diagnosed at ages 2-14 years has now been conducted in Hong Kong. Cases (n=98) formed a consecutive series of Chinese children diagnosed with acute leukaemia; controls (n=228) were identified following a survey using random digit dialling and required to attend for medical examination by a paediatrician. Interviews with mothers were conducted in hospital by one trained interviewer using a structured questionnaire. Odds ratios (OR) and 95% confidence intervals (CI) are reported for exposure variables capable of serving as proxies for exposure to infection in two critical time periods: first year of life, year before reference date (diagnosis for cases, corresponding date for controls). Analyses used logistic regression with adjustment for appropriate confounders. Change of area of residence reduced risk if during the first time period (OR = 0.47 [95% CI 0.23, 0.98]) and increased risk if during the second (OR=3.92, [95% CI 1.47, 10.46]). Reported roseola and/or fever and rash in the first year of life reduced risk (OR=0.33 [95% CI 0.16, 0.68]) whereas tonsillitis in the period 3-12 months before reference date increased risk (OR=2.56 [95% CI 1.22, 5.38]). Some other proxies for exposure to infection at the critical times were associated with predicted patterns of risk but day-care attendance failed to show predicted associations. These results provide support for the delayed exposure hypothesis in an affluent geographical setting in which population exposure to infectious agents is quite distinct from the settings of previous case-control studies. [ABSTRACT FROM AUTHOR]