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Background Strategies to mitigate the impact of COVID‐19 in special populations are complex and challenging. Few studies have addressed the impact of COVID‐19 on pediatric patients with cancer in low‐ and middle‐income countries. Methods Multicenter observational cohort study with prospective records and retrospective analyses starting in April 2020 in 21 pediatric oncology centers distributed throughout Brazil. Participants: Patients under 18 years of age who are infected by the SARS‐CoV‐2 virus (confirmed diagnosis through reverse transcriptase‐polymerase chain reaction [RT‐PCR]) while under treatment at pediatric oncology centers. The variables of interest included clinical symptoms, diagnostic and therapeutic measures. The repercussions of SARS‐CoV‐2 infection on cancer treatment and general prognosis were monitored. Results One hundred seventy‐nine patients were included (median age 6 [4–13] years, 58% male). Of these, 55.9% had acute leukemia and 34.1% had solid tumors. The presence of SARS‐CoV‐2 was diagnosed by RT‐PCR. Various laboratory markers were analyzed, but showed no correlation with outcome. Children with low or high BMI for age had lower overall survival (71.4% and 82.6%, respectively) than those with age‐appropriate BMI (92.7%) (p = .007). The severity of presentation at diagnosis was significantly associated with outcome (p

Although frequently disseminated to other anatomical sites, neuroblastoma (NB) is rarely reported as involving the central nervous system (CNS), which may reflect insufficient research in poorly controlled systemic disease. Here we demonstrate the involvement of the CNS in patients with NB over 18 months of age at diagnosis of extensive systemic disease. Meningeal metastases were observed even in the presence of complete systemic control. Although no improvement in patient’s survival was observed, radiotherapy was effective in preventing CNS recurrence after observation of actual or previous dural disease. In conclusion, this study uncovered the uncommon pathologic involvement of the CNS in children with advanced NB and underscores the meningeal surface as a potential pathway for this to occur.

OM is a painful inflammatory condition of the oral mucosa, derived from the toxic effects of chemotherapy and radiotherapy. High OM severity is frequently present in HSCT pediatric patients, who exhibit multiple painful ulcers that limit their mastication and swallowing, leading to poor nutritional status. Few studies have demonstrated OM clinical outcomes in young patients undergoing HSCT. Feasibility of oral care and LLLT on OM prophylaxis and treatment is also poorly discussed. The aim of this study was to describe a specialized oral care protocol that included LLLT for pediatric patients undergoing transplantation and to demonstrate the clinical outcomes after OM prevention and treatment. Data from OM-related morbidity were collected from 51 HSCT pediatric patients treated daily with LLLT, followed by standard oral care protocols. All the patients, even infants and young children, accepted the daily oral care and LLLT well. The majority (80.0%) only exhibited erythema in the oral mucosa, and the maximum OM degree was WHO II. Patients who had undergone autologous and HLA-haploidentical transplants showed OM with the lowest severity. The frequency of total body irradiation and methotrexate prescriptions was higher in adolescents when compared with infants (p = 0.044), and adolescents also exhibited OM more severely than infants and young children. We found that good clinical outcomes were obtained using this therapy, mainly in regard to the control of OM severity and pain reduction in the oral cavity. Specialized oral care, including LLLT, is feasible and affordable for HSCT pediatric patients, although some adaptation in the patient's oral hygiene routine must be adopted with help from parents/companions and clinical staff.

Neuroblastoma (NB) is the most common extra cranial solid tumor of childhood and often lethal in childhood. Clinical and biologic characteristics that are independently prognostic of outcome in NB are currently used for risk stratification to optimally the therapy. It includes age at diagnosis, International Neuroblastoma Staging System tumor histopathology and MYCN amplification. However, even in patients with theoretically good prognosis, such as localized tumor and non-amplified MYCN, either disease progress or recurrence may occur. Potential genetic determinants of this unfavorable behavior are not yet fully clarified. The presence of elevated expression of AHCY, PKMYT1, and BLM has accompanied poor prognosis MYCN-amplified neuroblastoma patients. Considering the potential implication of these genes on the clinical management of NB, we hypothesize that the identification of genetic variations may have significant impact during development of the recurrent or progressive disease. Using targeted DNA sequencing, we analyzed the mutation profiles of the genes PKMYT1, AHCY, and BLM in tumor samples of five patients with MYCN amplified and 15 MYCN non-amplified NB. In our study, BLM germline variants were detected in two patients with MYCN-non-amplified neuroblastoma. Our data allow us to hypothesize that, regardless of MYCN status, these mutations partially abolish BLM protein activity by impairing its ATPase and helicase activities. BLM mutations are also clinically relevant because BLM plays an important role in DNA damage repair and the maintenance of genomic integrity. We also found a novel variant in our cohort, PKMYT1 mutation localized in the C-terminal domain with effect unknown on NB. We hypothesize that this variant may affect the catalytic activity of PKMYT1 in NB, specifically when CDK1 is complexed to cyclins. The prognostic value of this mutation must be further investigated. Another mutation identified was a nonsynonymous variant in AHCY. This variant may be related to the slow progression of the disease, even in more aggressive cases. It affects the maintenance of the catalytic capacity of AHCY, leading to the consequent functional effects observed in the NB patients studied. In conclusion, our hypothesis may provide that mutations in BLM, AHCY and PKMYT1 genes found in children with MYCN-amplified or MYCN-non amplified neuroblastomas, may be associated with the prognosis of the disease.

Introduction: Hematopoietic stem cell transplantation (HSCT) was offered primarily as a therapeutic option for severe sickle cell disease in the context of myeloablative matched sibling donor transplants over the last two decades and helped to establish the benefits of transplantation for this disorder. In recent years, the transplant community has set out to explore ways to make stem-cell transplantation more available to patients with the disease, define indications and better timing, and offset toxicities with novel approaches to conditioning and better supportive care. In this context, neurological complication such as stroke disease and blood flow alteration in medial cerebral artery constitute the main indications of HSCT but neurological complications are the main causes of TRM in D+100 and is important to find ways to prevent this problem. Objective: Describe early neurological complications in sickle patients undergoing related HSCT on single specialized center and its following after the early diagnosis. Material and Methods: Seven patient were undergone related HSCT for sickle disease from 2011 to 2013. All patients filled inclusion criteria in the study and signed agreement term. Results: Seven HSCT were developed in the period, being four males. The average age was 13 years old (7-24). The HSCT indications were previous stroke, cerebral flow alteration on Doppler, acute chest syndrome and alloimunization. All patient were on blood transfusion therapy. The conditioning regimen was BuCy + ATG and the GVHD prophylaxis was MTX and CSA. Related donors were chosen with 10/10 HLA match and graft source marrow. The median of the neutrophil engraftment was D+ 25 and the platelets engraftment was in D+60. Two patient died, one by intestinal and liver GVHD on D+120, and another with sagittal sinus thrombosis and hemorrhagic stroke on D+3. Other two patients showed PRES syndrome related to cyclosporine use. The patients showed generalized seizures with tomographic neurological alteration. After the imunossupressor change to tacrolimus and the change of Phenytoin to Lamotrigine, the patient had total resolution of neurological complications without development of neurological sequelae. Patients who used Lamotrigine since the beginning of the conditioning have not shown neurological alterations. Conclusion: The PRES Syndrome and Stroke are two of the main causes of mortality related to the use of calcineurin inhibitors. Patients with sickle disease have shown endothelial and cerebral microcirculation changes, which made them highly susceptible to neurological complication. The control of blood pressure, maintenance of 50.000 platelets level and the use of Lamotrigine as prophylaxis of seizures seems to decrease the risk of neurological complications. Prospective studies with lamotrigine as primary prevention of PRES Syndrome must be performed. Patients with severe neurological alteration as vessels stenosis more than 90% and Moya-Moya Syndrome must be better evaluated before the conditioning because of high TRM risk. Disclosures No relevant conflicts of interest to declare.