This systematic review aimed to provide an overview of the clinical profile and outcome of COVID-19 infection in patients with hemoglobinopathy. The rate of COVID-19 mortality and its predictors were ...also identified. A systematic search was conducted in accordance with PRISMA guidelines in five electronic databases (PubMed, Scopus, Web of Science, Embase, WHO COVID-19 database) for articles published between 1st December 2019 to 31st October 2020. All articles with laboratory-confirmed COVID-19 cases with underlying hemoglobinopathy were included. Methodological quality was assessed using the Joanna Briggs Institute (JBI) critical appraisal checklists. Thirty-one articles with data on 246 patients with hemoglobinopathy were included in this review. In general, clinical manifestations of COVID-19 infection among patients with hemoglobinopathy were similar to the general population. Vaso-occlusive crisis occurred in 55.6% of sickle cell disease patients with COVID-19 infection. Mortality from COVID-19 infection among patients with hemoglobinopathy was 6.9%. After adjusting for age, gender, types of hemoglobinopathy and oxygen supplementation, respiratory (adj OR = 89.63, 95% CI 2.514–3195.537,
p
= 0.014) and cardiovascular (adj OR = 35.20, 95% CI 1.291–959.526,
p
= 0.035) comorbidities were significant predictors of mortality. Patients with hemoglobinopathy had a higher mortality rate from COVID-19 infection compared to the general population. Those with coexisting cardiovascular or respiratory comorbidities require closer monitoring during the course of illness. More data are needed to allow a better understanding on the clinical impact of COVID-19 infections among patients with hemoglobinopathy.
Clinical Trial Registration:
https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42020218200
.
Low physical activity (PA) level has been reported among survivors of childhood acute lymphoblastic leukemia (ALL). The present study was performed to determine the level of participation in general ...PA and physical education in school (PES) among children with ALL who completed intensive chemotherapy and identify possible barriers that influence adherence to PA and PES.
A cross-sectional, single-center study was conducted over 1 year in a tertiary pediatric hematology and oncology referral center in Kuala Lumpur, Malaysia. A total of 47 children with ALL aged 7-18 years old who were off-treatment and attended school on a regular basis were recruited. A modified structured questionnaire adapted from the Youth Risk Behavior Surveillance System, Division of Adolescent and School Health, the Centers for Disease Control and Prevention (CDC) was used to assess the children's level of PA and PES participation.
Among the 47 children will ALL included herein, 11 (23.4%) were physically active for at least 60 min a day for 5 days or more, following CDC recommendations. The median duration from completion of intensive chemotherapy was 4.95 years (25th, 3.29; 75th, 7.95). Younger age at study entry (median, 8.7 years old vs. 12.2 years old) and younger age at diagnosis (median, 2.9 years old vs. 4.3 years old) were significantly associated with higher PA level. Almost all children (45/47, 95.7%) participated in PES. Barriers to non-participation in PES mainly included exhaustion or fear of injury.
Majority of the children with ALL included herein had low levels of daily PA after intensive chemotherapy. Nonetheless, their participation in PES was encouraging. PA should thus be promoted during and after cessation of ALL treatment to prevent long-term health risks and improve overall quality of life.
The emergence of COVID-19 pandemic has led to heightened fear and uncertainty among parents of children with cancer. This study was conducted to evaluate the parental perceptions toward effects of ...COVID-19 infection to children with cancer, determine their stress level and factors contributing to high stress level during the pandemic.
This cross-sectional study was conducted in three paediatric oncology centres in Malaysia from September 2020 until December 2022. A total of 167 parents were recruited. Parents completed a set of questionnaires to assess their perception on effect of COVID-19 infection to children with cancer and COVID Stress Scale (CSS) to assess the parents' stress level.
Patients' mean age at study entry was 8.75 years (SD 4.38). Ninety-one (54.5%) patients were still on active treatment. More than 80% of the parents obtained information regarding COVID-19 infection from mass media and social networking. Fear of their children contracting COVID-19 infection was high especially among patients who were still on treatment. Forty-nine (29.3%) parents were significantly affected by the pandemic leading to loss of job or monthly income. Twenty-nine (17.4%) patients required treatment modification during the pandemic. The median total score for CSS was 78.0 (IQR 25
64.0; 75
95.0). Ninety-one (54.5%) respondents were very/extremely stressed based on the CSS scores. Components with high scores were xenophobia (median score 18.0; IQR 25
13.0, 75
22.0), fear of danger (median score 17.0; IQR 25
14.0, 75
20.0) and contamination fears (median score 16.0; IQR 25
12.0, 75
19.0). Lower household income was associated with higher stress level (
= 0.006).
Our study demonstrated high awareness regarding risk of COVID-19 infection among parents of oncology children. Half of the parents had high stress level, with low household income identified as a factor associated with high stress level.
IntroductionValidated tools to measure caregiving knowledge among parents of children with hematological cancers are needed to measure the clinical outcome of caregiver interventions. This study ...reports the development and validation of the Hematological Oncology Parents Education Caregiving Knowledge Questionnaire (HOPE-CKQ) among Malaysian parents of pediatric leukemia and lymphoma patients.MethodsInitially, 60 items on caregiving knowledge were developed based on a qualitative needs assessment study. Content validity was evaluated using item content validity index (I-CVI) and scale content validity index (S-CVI/Ave). Parents of pediatric leukemia and lymphoma patients were invited to complete the 60-item version of the HOPE-CKQ. Exploratory factor analysis (EFA) using polychoric correlation resulted in an 18-item version of HOPE-CKQ. Confirmatory factor analysis (CFA) was used to verify the factor structure. Known-group validity was tested by comparing the scores among different levels of parent education.ResultsThe I-CVI ranged from 0.83 to 1.00 whereas the S-CVI/Ave was 0.99, indicating good content validity. A total of 167 complete responses were analyzed for factor analysis. EFA using polychoric correlations resulted in a single-factor structure consisting of 18 items. CFA confirmed that the 18-item single-factor HOPE-CKQ model had a good fit for the data. The internal consistency reliability was good (α=0.80). Parents with tertiary education level had higher caregiving knowledge (M=12.61, SD=3.37) compared to parents with secondary education and below (M=10.33, SD=3.80) (t=3.58, p<0.001).ConclusionsThe 18-item HOPE-CKQ is valid and reliable for use to measure caregiving knowledge among pediatric leukemia and lymphoma parents. This tool may be considered to measure caregiving knowledge in future preventive and intervention programs.
Patients with sickle cell disease (SCD) are at higher risk of getting severe COVID-19 infection. This systematic review and meta-analysis aimed to determine the role of serum ferritin in predicting ...ICU admission and mortality among patients with SCD following COVID-19 infection. A systematic search was conducted in PubMed, Scopus, Web of Science, Embase, WHO COVID-19 database, ProQuest, and Cochrane Library for articles published between 1st December 2019 to 31st November 2021. Methodological quality was assessed using the Joanna Briggs Institute (JBI) critical appraisal checklists. Eleven articles (7 cohorts and 4 case series) were included in this review. Pooled mean serum ferritin level on admission was 1581.62 ng/mL while pooled proportion of ICU admission and mortality were 0.10 (95% CI 0.06; 0.16, prediction interval 0.04; 0.23,
p
= 0.29,
I
2
= 17%) and 0.07 (95% CI 0.05; 0.11, prediction interval 0.04; 0.12,
p
= 0.68,
I
2
= 0%) respectively. Meta-regression showed that serum ferritin did not predict for both ICU admission (regression coefficient = 0.0001,
p
= 0.3523) and mortality (regression coefficient = 0.0001,
p
= 0.4029). Our analyses showed that serum ferritin may not be a useful marker to predict the outcomes of COVID-19 infection among patients with SCD. More data are required to identify a reliable tool to identify patients with SCD who are at risk of getting severe COVID-19 infection.
Systematic Review Registration
https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=287792
, PROSPERO Registration: CRD42021287792.
Intravenous cannulation is experientially traumatic to children. To minimize this, EMLA® is applied on the would-be-cannulated area before IV cannula insertion. However, the time to achieve its ...maximum efficacy may be affected due to incomplete cutaneous absorption and the duration of application. The latter may be a limiting factor in a busy healthcare facility. The usage of dissolvable maltose microneedles may circumvent this problem by introducing micropores that will facilitate EMLA® absorption. A randomized phase II cross-over trial will be conducted to compare the Visual Analogue Scale (VAS) pain scores and skin conductance algesimeter index between 4 different interventions (1 fingertip unit (FTU) of EMLA® with microneedle patch for 30 min before cannulation; 0.5 FTU of EMLA® with microneedle patch for 30 min; 1 FTU of EMLA® with microneedle for 15 min; 1 FTU of EMLA® with sham patch for 30 min). A total of 26 pediatric patients with thalassemia aged between 6 and 18 years old and requiring blood transfusion will be recruited in this trial. During the visits, the VAS scores and skin conductance algesimeter index at venous cannulation will be obtained using the VAS rulers and PainMonitor™ machine, respectively. The trial will commence in August 2021 and is anticipated to end by August 2022.
Asparaginase-induced hypertriglyceridemia can have a spectrum of clinical presentations, from being asymptomatic to having life-threatening thrombosis or hyperviscosity syndrome. At present, there is ...no recommendation on routine lipid monitoring during asparaginase-containing treatment phase, nor a standardized guideline on its management. Two cases are presented here to illustrate the effects of concurrent infection on asparaginase-induced hypertriglyceridemia in patients with high-risk ALL and the use of SMOFlipid infusion as a treatment option in an acute situation.
Acquired thrombotic thrombocytopenia purpura is very rarely encountered in children. It is often misdiagnosed initially when the condition is not inherited.
We describe a 3-year-old Malay boy who ...presented with simple febrile seizure and had no neurological deficit, however, he was found to have microangiopathic hemolytic anemia, thrombocytopenia, and elevated serum lactate dehydrogenase. An ADAMTS13 assay results showed zero activities (0%), and markedly high level of ADAMTS13 inhibitor (93.15 U/mL) confirming the diagnosis of secondary thrombotic thrombocytopenia purpura. He received fresh frozen plasma infusions for 3 days and subsequently his platelet levels normalized. Serial ADAMTS13 assay results showed improvement. He was also given a short course of prednisolone after which the ADAMTS13 activity normalized (> 114%) at the end of prednisolone course.
At presentation, acquired thrombotic thrombocytopenia purpura in a very young child is commonly misdiagnosed as other conditions like idiopathic thrombocytopenic purpura, Evans syndrome, atypical hemolytic-uremic syndrome, or malignancy. ADAMTS13 assay should be performed early when thrombotic thrombocytopenia purpura is suspected as this condition is associated with dire consequences.
Congenital infantile fibrosarcoma (CIF) is a rare malignant soft tissue tumor that predominantly occurs in children under 1 year of age. CIF is frequently misdiagnosed with other conditions like ...hemangioma of infancy, infantile fibromatosis, or kaposiform hemangioendothelioma. Disseminated intravascular coagulopathy (DIVC) is rarely reported to be associated with CIF.
We describe an infant who presented with a large mass over the right arm. She was initially treated conservatively as hemangioma but was later confirmed by tissue histopathological examination to have CIF as the mass rapidly increased in size. She developed massive intra-tumoral bleed with DIVC whilst receiving neoadjuvant chemotherapy requiring multiple blood products transfusion. An urgent near-total resection of the tumor was performed in view of life threatening bleeding despite multiple blood transfusions. Post-operatively, she received further adjuvant chemotherapy. Subsequently, she remained in complete remission 32 months off-treatment and has full function of the affected limb.
CIF is an important condition to be considered in infant who has large mass over the extremity. DIVC could be associated with large CIF and when it occurs can be life-threatening. Whenever feasible early surgery should be performed in very young patients with large CIF to prevent mortality from bleeding.
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