In Peru, median latency to diagnosis was comparable to that described in developing countries, where the index of suspicion for childhood cancer remains low. It is crucial to establish strategies to optimize early diagnoses using associated factors.
Background: Macrophage activation syndrome (MAS) is characterized by excessive activation of macrophages and lymphocytes, leading to multiorgan dysfunction. As the initial manifestation of systemic lupus erythematosus (SLE), MAS is rare in children. Due to the COVID-19 pandemic, it is vital to identify the MAS as it shares similar characteristics with the multisystem inflammatory syndrome in children (MIS-C). Case report:We report the case of an 11-year-old male adolescent with symptoms of MIS-C. Although with negative results of RT-PCR (reverse transcription-polymerase chain reaction) and serology for SARS-CoV-2, contact with a positive COVID-19 relative was reported. When admitted to a referral hospital center, the patient received standard treatment for MIS-C. Although the same scheme was given on three occasions, the patient showed no response to initial therapy. Thus, the patient was classified as a refractory case. When the study was extended to other differential diagnoses, we found MAS associated with SLE. Therefore, the patient was treated with etoposide, cyclosporine, dexamethasone, and methotrexate and showed a good clinical response. Conclusions: MAS associated with SLE is rare in the pediatric population. MAS shares inflammatory markers with the MIS-C and is often confused with rheumatologic, infectious, and neoplastic entities. Reporting this case is important to identify differential diagnoses in patients presenting as MIS-C and decide on timely treatment, as it could be harmful or even fatal if a definitive diagnosis is not obtained on time.
Background Diagnosis delay in children and adolescents with cancer is a public health problem in Peru that leads to high rates of advanced disease and mortality. We aimed to assess the implementation feasibility and potential utility of ONCOpeds®, a mobile application that provides consultations with pediatric oncologists, in reducing the latency to diagnosis (LD) and referral time (RT) among children and adolescents in Peru diagnosed with cancer. Material and Methods A prospective pilot study was conducted in the region of Callao between November 2017 and April 2018. Primary and secondary care providers were trained on the use of ONCOpeds in five educational sessions. Patients younger than 18 years who resided in Callao and were diagnosed with cancer at four pediatric cancer units in Lima were analyzed by referral type: ONCOpeds facilitated or conventional. Results ONCOpeds was successfully installed in the smartphones of 78 primary and secondary care providers of Callao. During the study period, 23 new cases of cancer in children and adolescents from the region were diagnosed. Ten patients received ONCOpeds‐facilitated referrals and 13 received conventional referrals. The RT decreased among those who received ONCOpeds‐facilitated referrals by 66% (P = 0.02); however, the LD did not significantly decrease with the use of ONCOpeds. Conclusions The implementation of ONCOpeds was found to be feasible in this pilot study, having a potential utility in improving early diagnosis and referral in children and adolescents newly diagnosed with cancer. Directions for future research include multicenter studies with a larger population to further test the application's effectiveness.
Ovarian and paraovarian neoplasms are uncommon in children, mainly originating from germ cell tumors and, least frequently, epithelial tumors. There is an association between genital tract tumors and Proteus syndrome, a rare, sporadic, and progressive entity, characterized by a postnatal overgrowth in several tissues caused by a mosaic mutation in the AKT1 gene. We describe a 20-month-old asymptomatic infant with Proteus syndrome who developed an endometrioid paraovarian borderline cystic tumor. This is the youngest patient so far reported in the literature with this rare syndrome and an adnexal tumor of borderline malignancy. A total of nine patients have been described with female tract tumors and associated Proteus syndrome, which includes bilateral ovarian cystadenomas and other benign masses. A paraovarian neoplasm is extremely rare in children and could be considered a criterion for Proteus syndrome. Standardized staging and treatment of these tumors are not well established; however, most authors conclude that these neoplasms must be treated as their ovarian counterparts.
Introduction Due to higher survival rates among patients with bone tumors, there is a growing interest in determining these individuals' limb functionality and psychosocial prognosis. Objectives This study aimed to analyze the differences in functionality and quality of life (QoL) related to health in patients diagnosed with a malignant bone tumor during childhood, according to the type of surgery performed. Materials and Methods A cross-sectional study was performed for patients older than 14 years who treated for osteosarcoma or Ewing's sarcoma of the lower limb by who receiving surgery. To assess lower limb functionality and QoL among patients surviving malignant bone tumors, 19 patients surviving osteosarcoma or Ewing's sarcoma of the lower extremity were studied. An evaluation of functionality and QoL was done using the “Enneking and Medical Outcomes Study Short-Form 36 scales.” We compared the functional results according to the surgical technique used. Categorical variables were compared according to the Mann–Whitney and Kruskal–Wallis tests, with an established 95% level of significance. Results QoL among patients who had conservative surgery was not significantly better than amputee patients in the physical or mental aspects, nor in any of their components. Limb functionality, according to Enneking's staging, was significantly higher in non-amputee patients (p = 0.035). Conclusion According to the data analysis done in this study, the QoL was found to be not significantly different, based on the type of surgery performed; however, there were differences in limb functionality.
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