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1.
Cancer Rep (Hoboken) ; 5(5): e1512, 2022 05.
Article in English | MEDLINE | ID: covidwho-1913776

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/therapy
2.
Pediatric Hematology Oncology Journal ; 2022.
Article in English | ScienceDirect | ID: covidwho-1851929

ABSTRACT

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.

3.
Pediatric Hematology Oncology Journal ; 2022.
Article in English | ScienceDirect | ID: covidwho-1821452
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