Introduction: The COVID-19 pandemic has resulted in lifestyle changes for children. The aim of this study was to evaluate the impact of the pandemic on weight/BMI in children with severe bleeding disorders.
Methods: We conducted a retrospective review of patients age 3-18 years with severe bleeding disorders on prophylactic therapy treated at SickKids Hospital (Toronto, Canada) between February 01, 2018 and March 31, 2021. We evaluated the following pre- and post-COVID variables: weight (kg), weight percentile, BMI (kg/m ), BMI percentile, HJHS score, and prophylactic dosing (units/kg).
Results: One hundred and four patients were included in the final analysis. Diagnoses were as follows: haemophilia A (n = 92; 70.8%), haemophilia B (n = 17; 13.1%), type 3 von Willebrand disease (n = 11; 8.5%), the remainder were diagnosed with rare factor deficiencies. Median interval time from pre-COVID measurements to latest follow-up was 12.4 months (IQR 10.32-14.52 months) during which there was a statistically significant increase in median weight percentile +5.75 centiles (from 63 centile to 68.75 centile). There was a statistically significant increase in mean BMI of +1.03 kg/m (P = < .001) while median BMI percentile increased +8.82 centiles (from 53.9 centile to 62.72 centile) and mean BMI percentile increased 3.42 centiles (from 57.5 centile to 60.9 centile). The group that gained the most weight centiles, BMI and BMI centiles were 5-14 years old.
Conclusion: There was a trend to weight gain over the study period. More long-term data is required to evaluate the impact of this increase in weight and BMI on children with bleeding disorders.
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http://dx.doi.org/10.1111/hae.14449 | DOI Listing |
Haemophilia
December 2024
Advanced Center for Oncology, Hematology and Rare Disorders (ACOHRD), K.J. Somaiya Super Speciality Hospital & Research Center, Somaiya Ayurvihar, Sion East, Mumbai, Maharashtra, India.
Introduction: Mortality and morbidity in persons with haemophilia (PWH) have decreased due to improved diagnosis and treatment along with comprehensive population outreach efforts, but the impact is not uniform in different countries.
Aim: The study aims to assess all-cause and intracranial haemorrhage (ICH)-specific mortality of PWH in India.
Methods: This is a retrospective, observational, multi-centric cohort study of 1020 haemophilia patients from three centres in India.
J Pediatr Hematol Oncol
January 2025
Departments of Pediatric Hematology.
Congenital thrombotic thrombocytopenic purpura (cTTP), which is associated with mutations in the gene for a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13), is a chronic and lifelong disease. The clinical course is variable. Regularly using ADAMTS13-containing products such as fresh frozen plasma (FFP) for long-term prophylaxis is the most important treatment to prevent thrombotic microangiopathy (TMA) episodes.
View Article and Find Full Text PDFJ Pediatr Hematol Oncol
January 2025
Department of Pediatrics, The University of North Carolina at Chapel Hill, Chapel Hill, NC.
Constitutional platelet disorders have become better understood since Bernard and Soulier first described a case in 1948. Their diagnosis can also be challenging due to overlap in clinical presentation and lab findings with platelet type von Willebrand. Bernard-Soulier syndrome is a disorder caused by GPIb receptor mutations that decrease its affinity for von Willebrand factor resulting in reduced platelet function and macrothrombocytopenia.
View Article and Find Full Text PDFJ Pediatr Hematol Oncol
January 2025
Departments of Neurosurgery, Montefiore Medical Center, Albert Einstein College of Medicine.
Spontaneous epidural hematoma (EDH) is a rare sickle cell disease (SCD) complication. We report 3 pediatric cases with SCD and spontaneous EDH and 1 with subgaleal hematomas in the setting of vaso-occlusive crises and elaborate on their presentation and management. Through a scoping review, we identified 71 additional cases reported from 1970 to 2024 and highlighted notable features.
View Article and Find Full Text PDFVaccine X
January 2025
Finlay Vaccine Institute, Av. 21 #19810, Atabey, Playa, Havana 11600, Cuba.
Background: The heterologous three-dose schedule of the protein subunit anti-COVID-19 SOBERANA®02 and SOBERANA® Plus vaccines has proved its safety, immunogenicity and efficacy in pediatric population, but durability of immunogenicity is not yet dilucidated. This study reports the safety and durability of the humoral and cellular responses in children and adolescents 5-7 months after receiving the heterologous vaccine schedule of SOBERANA® 02 and SOBERANA® Plus.
Methods: Children participating in a phase I/II clinical trial were followed-up for 5-7 months after the last dose.
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