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1.
J Neuromuscul Dis ; 11(2): 349-359, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38363614

RESUMO

Background: Nusinersen treatment has demonstrated efficacy in improving clinical outcomes for spinal muscular atrophy (SMA), yet its impact on scoliosis progression remains unclear. Objective: This study aimed to assess the progression of scoliosis in pediatric patients with SMA undergoing nusinersen treatment. Methods: In this prospective study, data were systematically collected from Hong Kong pediatric SMA patients receiving nusinersen between 2018 and 2023. All patients had longitudinal radiographic studies pre-nusinersen, and at half-yearly or yearly intervals during treatment based on the scoliosis severity. Motor function evaluations were conducted pre-nusinersen, and after starting treatment at 6- and 12-month intervals. Results: Twenty-three patients ((SMA type 1 (SMA1) = 8, SMA type 2 (SMA2) = 7, SMA type 3 (SMA3) = 8)) with a median age of 5.8 years (range: 0.4-17.5 years) at nusinersen initiation, and median follow-up duration of 3.4 years (range: 1.1-5.2 years) were included. During the study period, motor scores remained stable or improved in 83% of patients. However, scoliosis progressed across all subtypes, with mean annual progression rates of 5.2, 11.9, and 3.6 degrees in SMA1, SMA2, and SMA3 respectively. Patients initiating nusinersen between ages 5 and 11 years exhibited the most rapid progression, with rates of 11.8, 16.5, and 7.3 degrees per year in SMA1, SMA2, and SMA3 respectively. Positive correlations were observed between the difference in CHOP-INTEND score post-nusinersen and scoliosis progression in SMA1 (rs = 0.741, p = 0.041). Conversely, negative correlations were found between the difference in HFMSE score post-nusinersen and scoliosis progression in SMA2 (rs =-0.890, p = 0.012) and SMA3 (rs =-0.777, p = 0.028). Conclusions: This study reveals that nusinersen treatment in symptomatic pediatric SMA patients with motor improvement is linked to increased scoliosis progression in SMA1, whereas it is associated with decreased progression in SMA2 and SMA3. Age, baseline Cobb angle, and motor milestone improvement are influential factors in scoliosis progression.


Assuntos
Atrofia Muscular Espinal , Oligonucleotídeos , Escoliose , Atrofias Musculares Espinais da Infância , Criança , Humanos , Lactente , Pré-Escolar , Adolescente , Estudos Prospectivos , Estudos Longitudinais , Escoliose/diagnóstico por imagem , Escoliose/tratamento farmacológico , Atrofia Muscular Espinal/tratamento farmacológico , Atrofias Musculares Espinais da Infância/tratamento farmacológico
2.
Rheumatology (Oxford) ; 63(4): 953-961, 2024 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-37632777

RESUMO

OBJECTIVES: There is little data on renal relapse in childhood-onset LN (cLN). We investigate the incidence, predictive factors and outcomes related to renal relapse. METHODS: We conducted a retrospective cohort study of all cLN diagnosed at ≤18 years between 2001-2021 to investigate the incidence and outcomes related to renal relapse. RESULTS: Ninety-five Chinese cLN patients (91% proliferative LN) were included. Induction immunosuppression was prednisolone and CYC [n = 36 (38%)] or MMF [n = 33 (35%)]. Maintenance immunosuppression was prednisolone and MMF [n = 53 (54%)] or AZA [n = 29 (31%)]. The rates of complete remission/partial remission (CR/PR) at 12 months were 78.9%/7.4%. Seventy renal relapses occurred in 39 patients over a follow-up of 10.2 years (s.d. 5.9) (0.07 episode/patient-year). Relapse-free survival was 94.7, 86.0, 80.1, 71.2, 68.3, 50.3 and 44.5% at 1, 2, 3, 4, 5, 10 and 20 years, respectively. Multivariate analysis showed that LN diagnosis <13.1 years [adjusted hazard ratio (HRadj) 2.59 995% CI 1.27, 5.29), P = 0.01], AZA maintenance [HRadj 2.20 (95% CI 1.01, 4.79), P = 0.05], PR [HRadj 3.9 (95% CI 1.03, 9.19), P = 0.01] and non-remission [HRadj 3.08 (95% CI 1.35, 11.3), P = 0.04] at 12 months were predictive of renal relapse. Renal relapse was significantly associated with advanced chronic kidney disease (stages 3-5) and end-stage kidney disease (17.9% vs 1.8%, P < 0.01). Furthermore, patients with renal relapse showed an increased incidence of infections (30.8% vs 10.7%, P = 0.02), osteopenia (38.5% vs 17.9%, P = 0.04) and hypertension (30.8% vs 7.1%, P < 0.01). CONCLUSION: Renal relapse is common among cLN, especially among young patients, and is associated with an increased incidence of morbidity and mortality. Attaining CR and the use of MMF appear to decrease the incidence of renal relapse.


Assuntos
Nefrite Lúpica , Criança , Humanos , Adolescente , Nefrite Lúpica/tratamento farmacológico , Nefrite Lúpica/epidemiologia , Nefrite Lúpica/diagnóstico , Imunossupressores/uso terapêutico , Azatioprina/uso terapêutico , Estudos Retrospectivos , Ácido Micofenólico , Resultado do Tratamento , Prednisolona/uso terapêutico , Recidiva , Ciclofosfamida , Indução de Remissão
3.
JAMA Netw Open ; 6(12): e2346106, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-38060228

RESUMO

Importance: There is limited evidence on whether the quality of life and behavior of children with special educational needs (SEN) have improved or worsened since schools reopened after COVID-19-related school closures. Objective: To describe the changes in the mental well-being of children and adolescents with SEN during the initial 6 months of resuming in-person learning after COVID-19-related school closures. Design, Setting, and Participants: This repeated cross-sectional study reported data from surveys completed by parents and caregivers of children and adolescents aged 3 to 18 years with SEN studying at special schools in Hong Kong. The first cohort was obtained during COVID-19-related school closure in April 2020 (wave 1) and the second cohort was obtained 6 months after school resumption with data collection between July and October 2021 (wave 2). Data analysis occurred from January to June 2022. Exposure: Diagnosis of a disability or disorder that required school-based special educational programming. Main Outcomes and Measures: Children's emotional and behavioral difficulties (measured with the Strengths and Difficulties Questionnaire [SDQ]), quality of life (measured with the Pediatric Quality of Life Inventory [PedsQL]), lifestyle habits, parental stress, and parental well-being (measured with the PedsQL Family Impact Module) were assessed. Cross-sectional comparisons of well-being between the 2 waves were conducted using analysis of covariance, and multiple regression analysis was performed to identify factors associated with mental health outcomes in wave 2. Results: In wave 1, a total of 456 parents and caregivers of children with SEN (mean [SD] age, 7.44 [3.98] years; 315 boys [69.1%]; 141 girls [30.9%]) responded to the surveys. In wave 2, 519 parents and caregivers of children with SEN (mean [SD] age, 8.16 [4.47] years; 365 boys [70.3%]; 154 girls [29.7%]) responded. After school resumption, preschoolers aged 3 to 5 years with SEN had significantly fewer emotional difficulties (mean [SD] SDQ score, 3.26 [2.39] vs 2.68 [2.03]; standardized mean difference [SMD] = 0.26; 95% CI, 0.07-0.46; Bonferroni-corrected P = .04) and conduct difficulties (mean [SD] SDQ score, 2.88 [1.89] vs 2.41 [1.91]; SMD = 0.25; 95% CI, 0.05-0.44; Bonferroni-corrected P = .01), whereas adolescents had more conduct difficulties (mean [SD] SDQ score, 1.62 [1.50] vs 2.37 [3.02]; SMD = 0.41; 95% CI, 0.13-0.70; Bonferroni-corrected P = .049). The overall quality of life of school-aged children with SEN aged 6 to 11 years worsened after school resumption (mean [SD] PedsQL score, 67.52 [17.45] vs 60.57 [16.52]; SMD = 0.41; 95% CI, 0.19-0.62; Bonferroni-corrected P = .002). Conclusions and Relevance: The findings of this repeated cross-sectional study suggest that preschoolers with SEN had improved emotional and behavioral functioning when school resumed after COVID-19-related closures. School-aged children with SEN, adolescents with SEN, and children with intellectual disabilities were at risk of reduced quality of life, indicating that additional support should be offered to vulnerable groups as they return to schools.


Assuntos
COVID-19 , Masculino , Criança , Adolescente , Feminino , Humanos , COVID-19/epidemiologia , Saúde Mental , Estudos Transversais , Qualidade de Vida , Pandemias
4.
Asia Pac J Clin Nutr ; 32(4): 401-407, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38135475

RESUMO

BACKGROUND AND OBJECTIVES: The usual recommended intake of vitamin D for healthy infants is 400 international unit (IU) daily. However, a high dose of vitamin D at 2000-3000 IU daily is needed for those with vitamin D deficiency (VDD). This study aimed to assess the natural history of a group of healthy infants with VDD and the associated factors for persistent VDD. METHODS AND STUDY DESIGN: Healthy infants detected to have VDD (25OHD <25 nmol/L) in a population study were followed, and their demographics and clinical data were collected. RESULTS: One hundred and thirty-one subjects (boys = 66%) were included. Their first serum 25OHD was taken at a median age of 87.5 days. None were treated with high-dose vitamin D supplements, but some have been given vitamin D at 400 IU daily. They were assessed again at the median age of 252.5 days when 15 remained to have VDD and 26 were in the insufficient range (25 - 49.9nmol/L). All persistent VDD children were on exclusive breastfeeding. Exclusive breastfeeding and no vitamin D supplementation were significant risk factors for persistent vitamin D insufficiency (<50nmol/L). CONCLUSIONS: Persistent VDD is common among infants exclusively breastfeeding and those who did not receive vitamin D supplementation.


Assuntos
Deficiência de Vitamina D , Lactente , Masculino , Feminino , Criança , Humanos , Hong Kong/epidemiologia , Vitamina D , Vitaminas , Suplementos Nutricionais
5.
Muscle Nerve ; 68(6): 857-864, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37837303

RESUMO

INTRODUCTION: Telerehabilitation provides physical training to patients through telecommunication networks. We examined the feasibility, safety, and efficacy of an integrated, personalized, respiratory and motor telerehabilitation program for pediatric patients with hereditary neuromuscular disorders (NMDs). METHODS: Stable pediatric patients were recruited for a 16-week home training program with personalized pulmonary, upper and lower limb exercises. Patients reviewed instructional videos at home and attended bi-weekly follow-ups through video or audio calls, text messages, or emails. The primary outcomes were respiratory function, Medical Research Council (MRC) grading, hand/pinch strength, 6-minute walk test, and Pediatric Quality-of-Life Inventory 3.0 Neuromuscular Module survey. The secondary outcomes were study compliance and user feedback. RESULTS: Patients with spinal muscular atrophy (n = 4), congenital myasthenic syndrome (n = 2), and Duchenne muscular dystrophy (n = 2) completed the program. The median weekly exercise time was 101.3 min (range: 30.0-266.9). No extra face-to-face physiotherapy sessions were requested by the patients. No adverse events were reported. After the study, patients showed improvements in maximal expiratory pressure (35.0 vs. 47.5 cm H2O, p = .028) and maintained their MRC grade, hand/pinch strength, and walking distance. Patients also reported improvements in the Pediatric Quality-of-Life Inventory 3.0 Neuromuscular Module survey score (74.5 vs. 87.0, p = .036). Patients rated the overall program highly (mean: 4.00/5.00) and recommended it as a standard of care (mean: 4.38/5.00). DISCUSSION: Our telerehabilitation program was feasible, safe, and possibly effective for this pilot cohort of stable pediatric patients with hereditary NMDs. Larger-scale studies for longer periods are warranted to confirm the results.


Assuntos
Doenças Neuromusculares , Telerreabilitação , Humanos , Criança , Projetos Piloto , Telerreabilitação/métodos , Terapia por Exercício/métodos , Exercício Físico , Modalidades de Fisioterapia , Qualidade de Vida
6.
JACC CardioOncol ; 5(3): 332-342, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37397078

RESUMO

Background: Anthracycline cardiotoxicity is a concern in survivors of childhood cancers. Recent evidence suggests that remote ischemic conditioning (RIC) may offer myocardial protection. Objectives: This randomized sham-controlled single-blind study tested the hypothesis that RIC may reduce myocardial injury in pediatric cancer patients receiving anthracycline chemotherapy. Methods: We performed a phase 2 sham-controlled single-blind randomized controlled trial to determine the impact of RIC on myocardial injury in pediatric cancer patients receiving anthracycline-based chemotherapy. Patients were randomized to receive RIC (3 cycles of 5-minute inflation of a blood pressure cuff placed over 1 limb to 15 mm Hg above systolic pressure) or sham intervention. The intervention was applied within 60 minutes before initiation of the first dose and before up to 4 cycles of anthracycline therapy. The primary outcome was the plasma high-sensitivity cardiac troponin T (hs-cTnT) level. The secondary outcome measures included echocardiographic indexes of left ventricular systolic and diastolic function and the occurrence of cardiovascular events. Results: A total of 68 children 10.9 ± 3.9 years of age were randomized to receive RIC (n = 34) or sham (n = 34) intervention. Plasma levels of hs-cTnT showed a progressive increase across time points in the RIC (P < 0.001) and sham (P < 0.001) groups. At each of the time points, there were no significant differences in hs-cTnT levels or LV tissue Doppler and strain parameters between the 2 groups (all P > 0.05). None of the patients developed heart failure or cardiac arrhythmias. Conclusions: RIC did not exhibit cardioprotective effects in childhood cancer patients receiving anthracycline-based chemotherapy. (Remote Ischaemic Preconditioning in Childhood Cancer [RIPC]; NCT03166813).

7.
Hum Vaccin Immunother ; 19(1): 2206278, 2023 12 31.
Artigo em Inglês | MEDLINE | ID: mdl-37157992

RESUMO

The mRNA-based BNT162b2 and inactivated whole-virus CoronaVac are two widely used COVID-19 vaccines that confer immune protection to healthy individuals. However, hesitancy toward COVID-19 vaccination appeared to be common for patients with neuromuscular diseases (NMDs) due to the paucity of data on the safety and efficacy in this high-risk patient population. Therefore, we examined the underlying factors associated with vaccine hesitancy across time for NMDs and assessed the reactogenicity and immunogenicity of these two vaccines. Patients aged 8-18 years with no cognitive delay were invited to complete surveys in January and April 2022. Patients aged 2-21 years were enrolled for COVID-19 vaccination between June 2021 and April 2022, and they recorded adverse reactions (ARs) for 7 days after vaccination. Peripheral blood was obtained before and within 49 days after vaccination to measure serological antibody responses compared to healthy children and adolescents. Forty-one patients completed vaccine hesitancy surveys for both timepoints, while 22 joined the reactogenicity and immunogenicity arm of the study. Two or more family members vaccinated against COVID-19 was positively associated with intention of vaccination (odds ratio 11.7, 95% CI 1.81-75.1, p = .010). Pain at the injection site, fatigue, and myalgia were the commonest ARs. Most ARs were mild (75.5%, n = 71/94). All 19 patients seroconverted against the wildtype SARS-CoV-2 after two doses of either vaccine, similar to 280 healthy counterparts. There was lower neutralization against the Omicron BA.1 variant. BNT162b2 and CoronaVac were safe and immunogenic for patients with NMDs, even in those on low-dose corticosteroids.


Assuntos
COVID-19 , Doenças Neuromusculares , Adolescente , Criança , Humanos , Anticorpos Antivirais , Vacina BNT162 , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Imunogenicidade da Vacina , RNA Mensageiro , SARS-CoV-2 , Vacinas de Produtos Inativados , Pré-Escolar , Adulto Jovem
8.
Kidney Int Rep ; 8(1): 141-150, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36644360

RESUMO

Introduction: Long-term data pertaining to childhood-onset lupus nephritis (cLN) remain extremely scarce. Methods: We conducted a retrospective cohort study of biopsy-proven cLN with onset age <18 years diagnosed from 2001 to 2020 to ascertain the long-term patient and kidney survival rates, and the incidence of advanced chronic kidney disease (CKD) (estimated glomerular filtration rate [eGFR] <60 ml/min per 1.73 m2). Results: A total of 92 subjects (78 female; age 13.7 ± 3.3 years; all Chinese) were included, with follow-up duration of 10.3 years (interquartile range, 5.8-15.9). Of these, 83 children (90%) had proliferative lupus nephritis (LN) (Class III/IV ± V). Mycophenolate was used for induction in 36%, whereas 34% received cyclophosphamide (CYC); 55% received mycophenolate as maintenance immunosuppression. The rates of complete remission (CR) and partial remission (PR) at 6 months and 12 months, respectively, were 65% and 20% and 78% and 8%. Two patients died (mortality rate 2.1/1000 patient-years), with a standardized mortality ratio of 22.3. Three patients (3.2%) developed end-stage kidney disease (ESKD), and advanced CKD occurred in 5 patients (5.4%). Survival rates without advanced CKD, ESKD, or death were 96.7%, 94.2%, 92.7%, 83.2% and 83.2% at 1 year, 5 years, 10 years, 15 years, and 20 years, respectively. Multivariate analysis revealed that severe kidney failure necessitating dialysis at presentation (adjusted hazard ratio 37.7, 95% confidence interval [CI] 4.0-355.6, P = 0.002), nonresponse (NR) after 12 months of treatment (adjusted hazard ratio 11.2, 95% CI 2.3-54.9, P = 0.003), and multiple nephritis flares (adjusted hazard ratio 2.6, 95% CI 1.1-6.2, P = 0.03) were predictive of advanced CKD, ESKD, or death. Other adverse outcomes included infections (2.9 episodes/100 patient-years), osteopenia (32%), hypertension (17%), short stature (14%), and avascular necrosis (7%). Conclusion: The long-term outcomes of cLN appeared to have improved in the present era with effective immunosuppression, cautious drug tapering, and assurance of medication adherence. There is still an unacceptably high prevalence of adverse outcomes.

9.
Singapore Med J ; 64(5): 319-325, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-34688228

RESUMO

Introduction: In Europe and North America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, the treatment outcomes are poor. Methods: A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed at gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, and radiotherapy to the primary and metastatic sites followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside monoclonal antibody therapy. Survival data were examined on certain clinical and laboratory factors. Results: There were 57 children (32 male) treated for high-risk neuroblastoma. Their mean age was 3.9 (range 0.7-14.9) years. The median follow-up time was 5.5 (range 1.8-13.0) years for the surviving patients. There were 31 survivors, with 27 patients surviving in first remission, and the five-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their five-year overall survival rate compared to patients whose initial chemotherapy was done elsewhere was 81.6% versus 41.1% (P = 0.011), and that of event-free survival was 69.7% versus 36.1% (P = 0.032). Published treatment results were obtained from four countries in Southeast Asia with five-year overall survival rates from 13.5% to 28.2%. Conclusion: Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective, with superior survival rates compared to previous data from Southeast Asia.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Neuroblastoma , Criança , Humanos , Masculino , Lactente , Pré-Escolar , Adolescente , Intervalo Livre de Doença , Neuroblastoma/tratamento farmacológico , Neuroblastoma/patologia , Transplante de Células-Tronco Hematopoéticas/métodos , Resultado do Tratamento , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Sudeste Asiático/epidemiologia , Terapia Combinada
10.
Artigo em Inglês | MEDLINE | ID: mdl-36554390

RESUMO

BACKGROUND: The prevalence of allergic diseases has been increasing in developing areas but has reached a plateau in many developed areas. Regular surveys are imperative to assess the disease burden for the prioritization of resource allocation. OBJECTIVES: We examined the change in the prevalence of wheezing, allergic rhinitis (AR), and eczema in school-aged children with possible associative factors and possible health effects of school air quality. METHODS: This was the third repeated cross-sectional study conducted in 2015-2016 using the International Study of Asthma and Allergies in Childhood (ISAAC) protocol. Our first and second surveys were conducted in 1994-1995 and 2000-2001, respectively. Regarding the third survey, we recruited 3698 children aged 6-7 from 33 local schools in 18 districts. Air quality, temperature, and humidity were also measured. The changes in prevalence, multiple regression, and GLIMMIX procedure were analyzed. RESULTS: From our first survey to our third survey, the increased prevalences for lifetime wheeze, current wheeze, lifetime rhinitis, current rhinitis, current rhinoconjunctivitis, lifetime chronic rash, and current chronic rash were 4.2%, 2.1%, 12.5%, 12.6%, 14.2%, 3.9%, and 4.1%, respectively. Increased prevalence of parental atopy had the strongest association with an increased prevalence of each of these seven health outcomes. There was no significant association between school air pollutant levels and the prevalence of health outcomes. CONCLUSIONS: There was an increase in the prevalence of wheezing, allergic rhinitis, and eczema across the surveys. The most important associated risk factor identified was the increased prevalence of a parental history of atopy.


Assuntos
Eczema , Exantema , Hipersensibilidade Imediata , Rinite Alérgica , Rinite , Criança , Humanos , Rinite/epidemiologia , Hong Kong/epidemiologia , Sons Respiratórios , Estudos Transversais , Eczema/epidemiologia , Rinite Alérgica/epidemiologia , Inquéritos e Questionários , Prevalência
11.
Orphanet J Rare Dis ; 17(1): 334, 2022 09 02.
Artigo em Inglês | MEDLINE | ID: mdl-36056400

RESUMO

BACKGROUND: Children with neurodegenerative conditions (CNDC) often suffer from severe neurodisability and high symptom burden with multisystemic involvement. However, their symptom burden and health-related quality of life (HRQOL) is not systematically documented in the literature, and there is no existing tool for such purposes. We designed our own tool for scoring of symptom burden amongst CNDCs and adopted the PedsQL generic score 4.0 to quantify the impact of overall symptom burden on children's overall HRQOL. METHODS: The Symptom Profile for children with neurodegnerative condition (SProND) questionnaire was developed, which consisted of 14 questions grouped according to 5 categories, namely epilepsy, neurobehavioural, movement and mobility related, breathing and swallowing, and other daily activities. CNDCs were recruited during visits to the Comprehensive Neurometabolic / Neurodegenerative Program of the Duchess of Kent Children's Hospital and Hong Kong Children's Hospital between November 2019 and March 2020. The SProND and PedsQL 4.0 Generic Core Scales were distributed to consenting parents of CNDCs. RESULTS: 36 CNDCs were recruited and matched with community controls. The response rate of subject and control were 99.5% and 98.7% respectively. The Cronbach alpha was 0.61 for the neurobehavioural domain and > = 0.7 for other domains. The greater number of symptoms each subject experiences, the worse his/ her PedsQL scores. Subjects displaying hypersalivation and swallowing difficulties had average physical health summary scores of less than 30% compared with subjects without these symptoms. On the other hand, average psychosocial health summary scores of subjects with involuntary movements, joint stiffness, hypersalivation, sleep problem and anorexia were approximately 70% compared to subjects without these symptoms. DISCUSSION AND CONCLUSION: This is one of the first studies to look at CNDCs as a group. We propose the SProND questionnaire for evaluation of symptom profile amongst CNDCs with satisfactory internal and external validity. It demonstrates how physical symptoms impact both physical and psychosocial HRQOL, and the cumulative effect of individual symptoms on the overall HRQOL. As such, CNDCs should be systematically screened for multi-systemic symptoms as a routine part of their clinical care, and care plans should be individually catered to individual patients' symptom burden and specific needs.


Assuntos
Doenças Neurodegenerativas , Sialorreia , Criança , Feminino , Humanos , Pais/psicologia , Qualidade de Vida/psicologia , Inquéritos e Questionários
12.
J Pediatr Endocrinol Metab ; 35(9): 1132-1140, 2022 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-36001345

RESUMO

OBJECTIVES: Diabetic ketoacidosis (DKA) is a life-threatening complication of type 1 diabetes (T1D). The aim of this study is to analyze the incidence, clinical characteristics, management and outcome of children presenting with DKA in new-onset T1D from 2008 to 2018 in Hong Kong. METHODS: Data was extracted from the Hong Kong Childhood Diabetes Registry. All subjects less than 18 years with newly diagnosed T1D from 1 January 2008 to 31 December 2018 managed in the public hospitals were included. Information on demographics, laboratory parameters, DKA-related complications and management were analyzed. RESULTS: In the study period, there were 556 children with newly diagnosed T1D in our registry and 43.3% presented with DKA. The crude incidence rate of new-onset T1D with DKA was 1.79 per 100,000 persons/year (CI: 1.56-2.04). Subjects presenting with DKA were younger (9.5 ± 4.5 vs. 10.5 ± 4.4, p=0.01) and had shorter duration of symptoms (4.2 ± 5.9 days vs. 10.6 ± 17.1 days, p<0.01). Regarding management, up to 12.4% were given insulin boluses and 82.6% were started on insulin infusion 1 h after fluid resuscitation. The rate of cerebral edema was 0.8% and there was no mortality. CONCLUSIONS: Younger age and shorter duration of symptoms were associated with DKA in new-onset T1D. Despite availability of international guidelines, there was inconsistency in acute DKA management. These call for a need to raise public awareness on childhood diabetes as well as standardization of practice in management of pediatric DKA in Hong Kong.


Assuntos
Diabetes Mellitus Tipo 1 , Cetoacidose Diabética , Criança , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/epidemiologia , Diabetes Mellitus Tipo 1/terapia , Cetoacidose Diabética/epidemiologia , Cetoacidose Diabética/etiologia , Cetoacidose Diabética/terapia , Hong Kong/epidemiologia , Humanos , Incidência , Insulina/uso terapêutico , Fatores de Risco
13.
Cancers (Basel) ; 14(13)2022 Jul 04.
Artigo em Inglês | MEDLINE | ID: mdl-35805042

RESUMO

Purpose: The neurocognitive outcomes of pediatric brain tumor survivors have been extensively studied but the risk and predictors for neurobehavioral impairment are less clearly defined. We systematically analyzed the rates of emotional, psychosocial, and attention problems in pediatric brain tumor survivors. Methods: PubMed, Web of Science, Embase, Scopus, and Cochrane were searched for articles published between January 2012 to April 2022. Eligible studies reported neurobehavioral outcomes for PBTS aged 2 to <23 years with a brain tumor diagnosis before 18 years of age. A random-effect meta-analysis was performed in R. Results: The search yielded 1187 unique publications, of which 50 were included in the quantitative analysis. The estimated risk of having emotional, psychosocial, and attention problems were 15% (95%CI 10−20%), 12% (95%CI 9−16%), and 12% (95%CI 9−16%), respectively. PBTS were more likely to have emotional difficulties (Hedge's g = 0.43 [95%CI 0.34−0.52]), psychosocial problems (Hedge's g = 0.46 [95%CI 0.33−0.58]), and attention problems (Hedge's g = 0.48 [95%CI 0.34−0.63]) compared to normal/healthy control subjects. There was no significant difference in the rates of neurobehavioral impairment between children with and without history of cranial radiotherapy. Conclusions: PBTS are at elevated risk of neurobehavioral impairment. Neurobehavioral monitoring should be considered as the standard of care for PBTS.

14.
Hum Vaccin Immunother ; 18(5): 2081460, 2022 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-35671466

RESUMO

Parental vaccine hesitancy is a major barrier to achieving high vaccination uptake among children, particularly in young children during the coronavirus disease 2019 (COVID-19) pandemic. Developing herd immunity is a critical concept for overcoming the current pandemic. The purpose of this study is to reduce parental vaccine hesitancy through a focused educational seminar in ZOOM and to empower parents who are concerned about vaccinating their children to communicate with medical experts during live seminars. Parents of preschoolers, teachers, and kindergarten principals from three local pre-school education and services associations attended live seminars. After attending seminars, parental willingness to vaccinate their children increased by 65%. The live Zoom seminar led by medical experts resulted in a decrease in vaccine hesitancy. Our findings support the creation of seminars that allow clients and medical specialists to communicate directly with one another. Offering an open and honest forum for people to express their concerns to medical experts could be a useful strategy for dealing with not only vaccination apprehension, but also other health-related emergencies.


Assuntos
Vacinas contra COVID-19 , COVID-19 , Criança , Pré-Escolar , Humanos , Aceitação pelo Paciente de Cuidados de Saúde , Conhecimentos, Atitudes e Prática em Saúde , COVID-19/prevenção & controle , Hong Kong/epidemiologia , Hesitação Vacinal , Pais , Vacinação
15.
Data Brief ; 42: 108069, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35341029

RESUMO

To our best knowledge, this article presents a novel data set on Hong Kong's adolescents' attitude towards the COVID-19 vaccination, excluding their parental opinions. This research used a cross-sectional questionnaire survey, which collects data from the population at a single point in time. Our questionnaire was designed in both English and Chinese for the adolescents' convenience, using a self-designed, online questionnaire website, which was sent to 30 secondary schools across Hong Kong at the beginning of June 2021, to be completed by 31st June 2021. This gathered a total of 2609 surveys, excluding those which did not fit into the criteria. As the data has identified factors that affect vaccine hesitancy, government authorities can use the data to choose effective ways to promote the COVID-19 vaccination and to educate the general population about the benefits of receiving it.

17.
J Am Soc Echocardiogr ; 35(6): 629-656, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35149208

RESUMO

BACKGROUND: Children with cancer and childhood cancer survivors (CCS) are at risk for developing chemotherapy-induced cardiomyopathy. Myocardial deformation imaging has shown potential in the early detection of subclinical myocardial damage with implications on therapeutic interventions and improvement of outcomes. The aim of this study was to perform a systemic review and meta-analysis of literature on the assessment of left ventricular and right ventricular myocardial deformation by speckle-tracking echocardiography at rest and during stress in children with cancer during and in survivors after chemotherapy. METHODS: A systematic review was performed through searching MEDLINE, Embase, the Cumulative Index to Nursing and Allied Health Literature, the Cochrane Central Register of Controlled Trials, and Scopus. Search hedges were created to cover the concepts of childhood cancer, chemotherapy, radiotherapy, anthracycline, cardiotoxicity, speckle-tracking, myocardial strain, and myocardial deformation. Two independent investigators reviewed the eligibility of articles for inclusion. The weighted mean difference in ventricular strain between pre- and postchemotherapy treatment and that between long-term CCS and healthy subjects were estimated using random-effect models with 95% CIs. Heterogeneity and publication bias were assessed using I2 statistics and the Egger test, respectively. RESULTS: Of the total of 8,703 records initially identified, 42 studies with a total of 5,430 children with cancer were included. Of these 42 studies that showed heterogeneities, nine assessed early myocardial injury during chemotherapy, 30 assessed late myocardial injury after chemotherapy with no publication bias, and three studied myocardial mechanics during stress. The main findings were as follows: (1) left ventricular systolic deformation is impaired in children with cancer during the initial treatment phase and among long-term CCS, while data on changes in right ventricular deformation are limited and inconclusive; (2) the predictive value of early reduction of myocardial strain imaging in forecasting subsequent development of cardiotoxicity is unknown, as it has not been studied; (3) limited data suggest the possibility of impaired left ventricular contractile mechanics during stress in CCS; and (4) cumulative anthracycline dose and chest-directed radiotherapy are consistently identified as factors associated with impaired myocardial deformation. CONCLUSIONS: Myocardial strain imaging by speckle-tracking echocardiography unveils early evidence of myocardial injury in children with cancer and long-term CCS. To support its adoption for clinical use, more data are required for the better understating of myocardial deformation parameters in the risk stratification of children with cancer and prediction of development of cardiomyopathy among CCS.


Assuntos
Cardiomiopatias , Neoplasias , Disfunção Ventricular Esquerda , Antraciclinas/efeitos adversos , Cardiotoxicidade , Criança , Detecção Precoce de Câncer , Ecocardiografia/métodos , Humanos , Neoplasias/tratamento farmacológico , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/diagnóstico por imagem
18.
Pediatr Diabetes ; 23(5): 556-561, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-33978300

RESUMO

OBJECTIVE: With increasing prevalence of childhood obesity worldwide, the incidence of pediatric-onset type 2 diabetes (T2D) is also increasing in many countries. We aim to analyze the time trend and incidence of T2D in children in Hong Kong from 2008 to 2017, and to characterize clinical characteristics at diagnosis. METHODS: Data were retrieved from the Hong Kong Childhood Diabetes Registry. All children with T2D diagnosed at the age of less than 18 years from January 1, 2008 to December 31, 2017 and managed in the public health care system were included in this study. RESULTS: In the incident years of 2008-2017 period, 391 children were diagnosed with T2D. The crude incidence rate was 3.42 per 100,000 persons/year [95% confidence interval (CI) 3.08-3.76], which was much higher than that in last registry of 1.27 per 100,000 persons/year in 1997-2007 (P < 0.001).Most children (76%) were asymptomatic and were diagnosed by routine screening. At presentation, a significant proportion presented with co-morbidities including fatty liver (37.9%), dyslipidaemia (35.3%), hypertension (22.5%), and microalbuminuria (12.8%). CONCLUSIONS: The incidence of T2D in children has increased significantly in Hong Kong. Most of them were asymptomatic and picked up on routine health screening. Yet, comorbidities were commonly identified at diagnosis.


Assuntos
Diabetes Mellitus Tipo 2 , Obesidade Infantil , Adolescente , Criança , Diabetes Mellitus Tipo 2/epidemiologia , Hong Kong/epidemiologia , Humanos , Incidência , Sistema de Registros
19.
Nephrology (Carlton) ; 27(2): 171-180, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34837272

RESUMO

AIM: To evaluate the demographics and long-term patient outcomes of children with end-stage kidney disease in Hong Kong. METHODS: We conducted a cohort study at the Paediatric Nephrology Centre, the designated site providing kidney replacement therapy (KRT) for children in Hong Kong. The clinical characteristics and outcomes of all children who initiated chronic KRT before 19 years, between 2001 and 2020, were analysed. RESULTS: One hundred forty-seven children (50% male) received KRT at a mean age of 11.4 ± 5.7 years. The incidence of ESKD was 6.28 per million age-related population (pmarp). The leading cause of ESKD was congenital anomalies (33%). Ten children (7%) had pre-emptive kidney transplants, 104 (71%) and 33 (22%) patients received automated peritoneal dialysis and haemodialysis as initial KRT. The incidence of ESKD increased over time, and were 4.38, 5.07, 6.15 and 9.17 pmarp during 2001-2005, 2006-2010, 2011-2015 and 2016-2020, respectively (p = .005). Ninty-seven patients (66%) received kidney transplants and the median time to receive a kidney graft was 3.7 years (95% CI 3.1-4.3). Only 10 patients had pre-emptive kidney transplants. The mortality rate was 9.1 deaths per 1000-patient-years (95%CI 4.6-16.2). The survival probabilities at 1-, 5-, 10- and 15-year were 100%, 94.8% (95%CI 90.7-98.9%), 89.7% (95% CI 83.4%-95.9%), 87.1% (95% CI 79.3%-94.9%), respectively. Standardised mortality ratio was 54.5. 72% of deaths were due to infections. Young infants and those without kidney transplants were associated with worse survival (p < .01). Multivariate analysis demonstrated that dialysis was the only factor associated with significantly increased risk of death (HRadj 12.9, 95% CI 2.7-63.2, p = .002). CONCLUSION: We observed an increasing incidence of paediatric ESKD in Hong Kong with considerable waiting time to kidney transplant. Mortality risk is comparable to other developed countries and is highest among dialysis population. Efforts should be made to facilitate early access to paediatric kidney transplantation in Hong Kong.


Assuntos
Falência Renal Crônica/terapia , Terapia de Substituição Renal , Adolescente , Criança , Estudos de Coortes , Demografia , Feminino , Hong Kong , Humanos , Masculino , Fatores de Tempo , Resultado do Tratamento
20.
Clin Infect Dis ; 75(4): 673-681, 2022 09 10.
Artigo em Inglês | MEDLINE | ID: mdl-34849657

RESUMO

BACKGROUND: Age-specific incidence of acute myocarditis/pericarditis in adolescents following Comirnaty vaccination in Asia is lacking. This study aimed to study the clinical characteristics and incidence of acute myocarditis/pericarditis among Hong Kong adolescents following Comirnaty vaccination. METHODS: This is a population cohort study in Hong Kong that monitored adverse events following immunization through a pharmacovigilance system for coronavirus disease 2019 (COVID-19) vaccines. All adolescents aged between 12 and 17 years following Comirnaty vaccination were monitored under the COVID-19 vaccine adverse event response and evaluation program. The clinical characteristics and overall incidence of acute myocarditis/pericarditis in adolescents following Comirnaty vaccination were analyzed. RESULTS: Between 14 June 2021 and 4 September 2021, 33 Chinese adolescents who developed acute myocarditis/pericarditis following Comirnaty vaccination were identified. In total, 29 (87.88%) were male and 4 (12.12%) were female, with a median age of 15.25 years. And 27 (81.82%) and 6 (18.18%) cases developed acute myocarditis/pericarditis after receiving the second and first dose, respectively. All cases are mild and required only conservative management. The overall incidence of acute myocarditis/pericarditis was 18.52 (95% confidence interval [CI], 11.67-29.01) per 100 000 persons vaccinated. The incidence after the first and second doses were 3.37 (95% CI, 1.12-9.51) and 21.22 (95% CI, 13.78-32.28 per 100 000 persons vaccinated, respectively. Among male adolescents, the incidence after the first and second doses were 5.57 (95% CI, 2.38-12.53) and 37.32 (95% CI, 26.98-51.25) per 100 000 persons vaccinated. CONCLUSIONS: There is a significant increase in the risk of acute myocarditis/pericarditis following Comirnaty vaccination among Chinese male adolescents, especially after the second dose.


Assuntos
Vacinas contra COVID-19 , COVID-19 , Miocardite , Pericardite , Adolescente , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , Criança , Estudos de Coortes , Feminino , Hong Kong/epidemiologia , Humanos , Masculino , Miocardite/complicações , Miocardite/etiologia , Pericardite/epidemiologia , Pericardite/etiologia , Vacinação/efeitos adversos
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