ABSTRACT
BACKGROUND: Mulibrey-Nanism (Muscle-liver-brain-eye Nanism = dwarfism; MUL) is a rare genetic syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. In the largest published series of MUL, 8% patients were reported to develop Wilms tumor (WT). The published literature lacks data regarding the best treatment protocol and outcome of this cohort of children with WT and MUL. We report here a 2-year-old boy with WT and MUL and present a review of literature on WT in MUL. CASE: Our patient had associated cardiac problems of atrial septal defect, atrial flutter and an episode of sudden cardiac arrest. We managed him successfully with chemotherapy, surgery and multi-speciality care. He is alive and in remission at follow-up of 6 months. CONCLUSION: A total of 14 cases (including present case) of WT have been reported in MUL and treatment details were available for six cases. They were managed primarily with surgery, chemotherapy with/without radiotherapy, and all achieved remission. The outcome data is available only for two cases, one has been followed up till 15 years post treatment for WT and other is our patient.
Subject(s)
Kidney Neoplasms , Mulibrey Nanism , Wilms Tumor , Child , Child, Preschool , Humans , Kidney Neoplasms/diagnosis , Kidney Neoplasms/therapy , Male , Mulibrey Nanism/complications , Mulibrey Nanism/genetics , Mulibrey Nanism/pathology , Nuclear Proteins/genetics , Tripartite Motif Proteins , Ubiquitin-Protein Ligases , Wilms Tumor/complications , Wilms Tumor/diagnosis , Wilms Tumor/therapyABSTRACT
Introduction Coronavirus disease 2019 (COVID-19) affects children but mostly has mild course. There is meagre published data on the impact of COVID-19 illness in children with Severe Aplastic anemia (SAA). We describe our experience of managing COVID-19 in children with SAA. Method Three children of SAA who developed SARS-CoV-2 infection are included in this study. Results Patient 1 was post Immunosuppressive therapy (IST) for SAA and had an asymptomatic course and uneventful recovery. Patient 2 was several months post IST with no response and had an asymptomatic COVID-19 illness but had delayed viral clearance, however he succumbed to bacterial sepsis soon after. Patient 3 was awaiting IST and while he contracted severe acute respiratory syndrome-Coronavirus-2 (SARS-CoV-2) infection, he had symptomatic COVID-19 illness followed by bacterial and fungal sepsis to which he succumbed. Conclusion: COVID-19 in children with SAA can be mild to fatal course and virus may have delayed clearance. It can lead to delay in therapy of SAA.
ABSTRACT
Introduction The clinical outcomes of COVID-19 infection in children with cancer have been variable worldwide. Therefore, we aimed to collect data from all regions in India through a national collaborative study and identify factors that cause mortality directly related to COVID-19 infection. Methods Data was collected prospectively on children across India on cancer therapy and diagnosed with COVID-19 infections from 47 centers from April 2020 to October 2021. Information was recorded on the demographics, the number of children that required intervention, and the outcome of the infection. In addition, we analyzed the impact of the delta variant in 2021. Results A total of 659 children were studied, of whom 64% were male and 36% were female. The data from the eastern region was sparse, and this was a collection bias. COVID-19 infection was predominantly seen in children less than five years. The delta variant had a higher impact in the southern region, and this was statistically significant. Of the 659 children, 30 children died (4.5%), however only 7 of the deaths were directly attributed to COVID-19 infection (1%). Conclusion The study reports the largest nationally representative cohort of children with cancer and COVID-19 to date in India. We identified demographic and clinical factors associated with increased all-cause mortality in patients with cancer. Complete characterization of the cohort has provided further insights into the effects of COVID-19 on cancer outcomes. The low mortality allows us to recommend that specific cancer treatments be continued without delays in therapy.
Subject(s)
COVID-19/complications , Neuroblastoma/complications , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/complications , Adolescent , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Asparaginase/adverse effects , Asparaginase/therapeutic use , COVID-19/diagnosis , Carboplatin/adverse effects , Carboplatin/therapeutic use , Child, Preschool , Cisplatin/adverse effects , Cisplatin/therapeutic use , Cyclophosphamide/adverse effects , Cyclophosphamide/therapeutic use , Daunorubicin/adverse effects , Daunorubicin/therapeutic use , Etoposide/adverse effects , Etoposide/therapeutic use , Humans , Male , Neuroblastoma/drug therapy , Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Prednisone/adverse effects , Prednisone/therapeutic use , Reinfection/complications , Reinfection/diagnosis , SARS-CoV-2/isolation & purification , Vincristine/adverse effects , Vincristine/therapeutic useABSTRACT
With the recent Pandemic of Covid-19 affecting more than 180 countries across the globe, services to children with blood disorders and cancers are likely to be affected. Children, in general, are at lesser risk of developing severe diseases and mortality, and mostly recover from it;fortunately, till date, very few cases have been reported in children with cancers. However, since this is an unprecedented pandemic, there is a felt need for guidance on the challenges faced in providing care to children with cancers and various blood disorders including those requiring Hematopoietic Stem Cell Transplantation (HSCT). This document will provide guidance on how to maintain continuity of care for children with blood disorders & cancers during the COVID-19 pandemic while exercising all precautions to reduce the risk of Covid-19 in this vulnerable group and if infected, what optimum care should be provided to these children.