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IntroductionMultisystem inflammatory syndrome in children associated with coronavirus disease 2019 (MIS-C), a novel hyperinflammatory condition secondary to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, is associated with severe outcomes such as coronary artery aneurysm and death.MethodsThis multicenter, retrospective, observational cohort study including eight centers in Mexico, aimed to describe the clinical characteristics and outcomes of patients with MIS-C. Patient data were evaluated using latent class analysis (LCA) to categorize patients into three phenotypes: toxic shock syndrome-like (TSSL)-MIS-C, Kawasaki disease-like (KDL)-MIS-C, and nonspecific MIS-C (NS-MIS-C). Risk factors for adverse outcomes were estimated using multilevel mixed-effects logistic regression.ResultsThe study included 239 patients with MIS-C, including 61 (26%), 70 (29%), and 108 (45%) patients in the TSSL-MIS-C, KDL-MIS-C, and NS-MIS-C groups, respectively. Fifty-four percent of the patients were admitted to the intensive care unit, and 42%, 78%, and 41% received intravenous immunoglobulin, systemic glucocorticoids, and anticoagulants, respectively. Coronary artery dilatation and aneurysms were found in 5.7% and 13.2% of the patients in whom coronary artery diameter was measured, respectively. Any cause in-hospital mortality was 5.4%. Hospitalization after ten days of symptoms was associated with coronary artery abnormalities (odds ratio [OR] 1.6, 95% confidence interval [CI] 1.2–2.0). Age ≥10 years (OR: 5.6, 95% CI: 1.4–2.04), severe underlying condition (OR: 9.3, 95% CI: 2.8–31.0), platelet count 1.2 (OR: 3.8, 95% CI: 1.05–13.9), and serum ferritin concentration >1,500 mg/dl at admission (OR: 52, 95% CI: 5.9–463) were risk factors for death.DiscussionMortality in patients with MIS-C was higher than reported in other series, probably because of a high rate of cases with serious underlying diseases.

BackgroundThe distribution of RUNX1-RUNXT1, PML-RARA, CBFB-MYH11, BCR-ABL1p210, and KMT2A-MLLT3 in the pediatric population with acute myeloid leukemia (AML) in many countries of Latin America is largely unknown. Therefore, we aimed to investigate the frequency of these fusion genes in children with de novo AML from Mexico City, which has one of the highest incidence rates of acute leukemia in the world. Additionally, we explored their impact in mortality during the first year of treatment.MethodsWe retrospectively analyzed the presence of RUNX1-RUNXT1, PML-RARA, CBFB-MYH11, BCR-ABL1p210, and KMT2A-MLLT3 by RT-PCR among 77 patients (

Abstract Background Spontaneous bleedings occurring into joints (hemarthrosis) are the most common manifestations of hemophilia and causes severe joint damage ultimately resulting in joint disfunction known as hemophilic arthropathy. Among available therapeutic options for reducing recurrent hemarthrosis-associated damage, radiosynoviorthesis (RS) has proven effective in improving joint function. Aim To assess the impact of RS with Yttrium(90) citrate (C-Y(90)) on frequency of hemarthroses and joint function in a group of pediatric patients. Methods Between November 1998 and February 2017, we evaluated 27 pediatric patients with mild, moderate or severe hemophilia with haemophilic arthropathy. Overall, RS was applied in 60 joints. Some patients received more than one single intra-articular injection with C-Y(90). Results During the follow-up, one patient showed joint bleeding 15 months after RS, one patient after 12 months and one patient after 45 days. The episodes of hemarthrosis were reduced and joint function significantly improved in all patients. Conclusion RS with C-Y(90) is a simple and safe treatment for reducing the frequency of hemarthroses in patients with hemophilia. It decreases the use of factor VIII / IX and improves joint function.

ETV6::RUNX1 is a genetic rearrangement of good prognosis in children with acute lymphoblastic leukemia (ALL). In Mexico, its prevalence is low in comparison with Caucasian populations. We developed a novel TaqMan one-step RT-qPCR approach to assess the prevalence of four genetic rearrangements in a cohort of Hispanic children with ALL from Mexico City. The prevalence of common fusion gene transcripts was as follows: TCF3::PBX1 7.7%; BCR::ABL1p190 3.3%; and KMT2A::AFF1 2.8%, and ETV6::RUNX1was observed with low prevalence (10.5%) in comparison to that reported for developed countries. This is consistent with previous findings on Mexican children with ALL and similar to those reported on children from Hispanic populations. The confirmation of a low prevalence of ETV6::RUNX1 in children of a Hispanic origin represents an advancement in the description of genetic factors of ALL in these populations.

Abstract Background Gorham-Stout disease is a rare condition characterized by unifocal and massive type IV osteolysis (variant of idiopathic nonhereditary osteolytic disease) with a slow progression, which is self-limiting for some years. It is characterized by recurrent vascular tumors with disruption of the anatomical architecture and intraosseous proliferation of vascular channels that leads to the destruction and resorption of the bone matrix. The aim of this study is to present the clinical features of this disease, as well as the importance of prompt diagnosis and treatment, with a review of the reported cases. Case reports We describe two cases of Gorham-Stout disease between 2013 and 2017 with surgical interventions, follow-up and results. Case one involves an 11-year-old male with involvement of the left iliac bone, with adequate evolution after a surgical procedure with a lyophilized cadaveric tricortical bone allograft. Case two involves a 6-year-old male with cervical spine C1-C3 repercussion; in the protocol for surgical treatment, he presented with signs of spinal cord compression and died. Conclusion Diagnosis of Gorham-Stout disease is made by exclusion, and its clinical presentation varies widely, from spontaneous remission to a fatal outcome.

Introduction. Down syndrome (DS) is one of the most frequent genomic disorders around the globe (∼1:700 births). During the COVID-19 pandemic, it has been recognized that children with DS are patients with a greater risk of presenting SARS-CoV-2 infection-related poor outcomes. Nonetheless, a few cases with DS and SARS-CoV-2 infection have been reported. Our aim is to describe the unfavorable clinical course of a child with DS infected with SARS-CoV-2 virus. Case. Female, 2 years old, karyotype 47,XX,+21[30], previously diagnosed with a cyanotic congenital heart disease (tricuspid atresia and infundibular pulmonary stenosis, type Ib) who started with diarrhea, developed shortness of breath, and cyanosis and was admitted to the hospital presenting low-oxygen saturation (33%) requiring invasive mechanical ventilation support. The patient tested positive for SARS-CoV-2 infection. During hospitalization, the patient presented hypotension, anuria, retarded capillary filling, and metabolic acidosis; management with vasoactive drugs was needed. Nonetheless, the patient developed respiratory and cardiac failure, acute renal injury (AKIN-III), and septic shock. After 24 days of hospitalization, the patient died. Conclusions. Multiple organ failure observed in the patient presented could be related to the triple gene dose of four interferon receptors (IFNAR1, IFNAR2, IFNGR2, and IL10RB) located at 21q22.11. Additionally, overexpression of TMPRSS2 at the pulmonary level, located also at 21q22.3, could be related with an increased susceptibility for the development of SARS-CoV-2 infection in DS patients.

Central venous catheter (CVC) installation is essential for the treatment of critically ill neonates; however, it is associated with the development of neonatal intracardiac thrombosis, which is a complication that is associated with a poor prognosis. We aimed to identify specific risk factors for the development of intracardiac thrombosis in the right atrium (RA) and superior vena cava (SVC) related to the use of CVC in critically ill neonates. Methods: A case–control study was conducted at the tertiary referral neonatal intensive care unit of the Pediatric Hospital Siglo XXI in Mexico City, Mexico from 2008 to 2013. The included cases (n = 43) were de novo patients with intracardiac thrombosis in the RA and SVC diagnosed by echocardiography. The controls (n = 43) were neonates without intracardiac thrombosis or thrombosis at other sites. A logistic regression analysis was conducted, and odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated. Results: The independent risk factors for intracardiac thrombosis in the RA and SVC were the surgical cut-down insertion technique (OR = 2.98; 95% CI: 1.18–9.10), a maternal history of gestational diabetes/diabetes mellitus (OR = 10.64; 95% CI: 1.13–121.41), Staphylococcus epidermidis infection (OR = 7.09; 95% CI: 1.09–45.92), and CVC placement in the SVC (OR = 5.77; 95% CI: 1.10–30.18). Conclusion: This study allowed us to identify several contributing factors to the development of intracardiac thrombosis in the RA and SVC related to the installation of a CVC in a subgroup of critically ill neonates. Multicenter and well-designed studies with a larger number of patients could help validate our findings and/or identify other risk factors that were not identified in the present study.