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Background. Recent reports about cisapride have raised some concerns about the safety and efficacy of this medication in children. The aim of this study was to identify electrocardiographic changes and a predisposition to develop arrhythmias in children.Methods. Patients were divided in 2 groups: 1) 63 children (mean age: 29 months) who received cisapride (0.2 mg/kg/dose 3 times/day), and 2) 57 children (mean age: 27 months) who did not receive cisapride (they served as controls). Both groups did not have any associated disease. Electrocardiogram (EKG) was performed to children when they were included in the study. The QT interval was corrected using Bazett's formula. Twenty-four-hour Holter recording was performed in children with prolonged QT interval (PQTI). When PQTI was identified in group 1, cisapride was discontinued and a new EKG was performed.Results. Five children from group 1 and 6 from group 2 had PQTI. In 3 children with PQTI, the QTc interval returned to normal values when cisapride was discontinued. In children under 4 months of age, a statistical difference was found, with QTc interval being longer in group 2 (without cisapride) than in group 1. Holter recordings were normal in all children with PQTI.Conclusion. PQTI can be found in normal children with or without cisapride. In our study PQTI was not associated with any life-threatening event. [ABSTRACT FROM AUTHOR]

Objectives: To describe the clinical presentation, management, and outcomes of Kawasaki disease (KD) in Latin America and to evaluate early prognostic indicators of coronary artery aneurysm (CAA)., Study Design: An observational KD registry-based study was conducted in 64 participating pediatric centers across 19 Latin American countries retrospectively between January 1, 2009, and December 31, 2013, and prospectively from June 1, 2014, to May 31, 2017. Demographic and initial clinical and laboratory data were collected. Logistic regression incorporating clinical factors and maximum coronary artery z-score at initial presentation (between 10 days before and 5 days after intravenous immunoglobulin [IVIG]) was used to develop a prognostic model for CAA during follow-up (>5 days after IVIG)., Results: Of 1853 patients with KD, delayed admission (>10 days after fever onset) occurred in 16%, 25% had incomplete KD, and 11% were resistant to IVIG. Among 671 subjects with reported coronary artery z-score during follow-up (median: 79 days; IQR: 36, 186), 21% had CAA, including 4% with giant aneurysms. A simple prognostic model utilizing only a maximum coronary artery z-score ≥2.5 at initial presentation was optimal to predict CAA during follow-up (area under the curve: 0.84; 95% CI: 0.80, 0.88)., Conclusion: From our Latin American population, coronary artery z-score ≥2.5 at initial presentation was the most important prognostic factor preceding CAA during follow-up. These results highlight the importance of early echocardiography during the initial presentation of KD., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects patients younger than 5 years. In the absence of an available, affordable diagnostic test, detailed clinical history and physical examination are still fundamental to make a diagnosis., Methods: We present five representative cases with KD-like presentations: systemic onset juvenile idiopathic arthritis, mycoplasma-induced rash and mucositis, staphylococcal scalded skin syndrome, BCGosis, and the recently described multisystemic inflammatory syndrome in children (MIS-C) associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) virus., Results: Rash, fever, and laboratory markers of inflammation can be present in several childhood diseases that may mimic KD., Conclusion: The term 'Kawasaki syndrome' instead of 'Kawasaki disease' may be more appropriate. Physicians should consider an alternative diagnosis that may mimic KD, particularly considering MIS-C during the present pandemic, as an aggressive diagnostic and therapeutic approach is needed., (© 2020 Japan Pediatric Society.)

Background: Recently, hoarseness affecting the supraglottic structure has been reported in Kawasaki disease (KD). The objective of this study was to characterize the frequency of hoarseness in acute KD patients in Latin America., Methods: We used prospective data from the multinational Red de Enfermedad de Kawasaki en America Latina (REKAMLATINA) network. A total of 865 patients from 20 countries were enrolled during the 3 year study period. Data on hoarseness were available in 858 (99.2%) patients. The clinical and laboratory characteristics between hoarse and non-hoarse KD were compared., Results: Hoarseness was documented in 100 (11.6%) patients. Hoarse patients were younger than those with KD without hoarseness (median age 18 vs 26 months; P = 0.002) and presented with lower hemoglobin (10.7 g/dL vs 11.3 g/dL; P = 0.040) and hematocrit levels (32% vs 33%, P = 0.048)., Conclusions: Hoarseness was found to be prevalent as a presenting sign of acute KD in younger children. Anemia may indicate the presence of active inflammation., (© 2020 Japan Pediatric Society.)

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. KD can be complicated with macrophage activation syndrome. The optimal treatment for this KD complication has not been established, and a variety of treatments have been used. Infliximab, a chimeric monoclonal antibody that binds tumor necrosis factor, has proved to be efficacious in IV gammaglobulin resistant KD. We present 2 cases of KD complicated with macrophage activation syndrome, including 1 patient with DiGeorge syndrome successfully treated with a combined treatment of IV gammaglobulin, corticosteroids, cyclosporine, and infliximab., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Frequency of Kawasaki disease (KD) in infants is low in almost all countries. These patients are at higher risk of developing cardiac complications., Aim: To evaluate the clinical features, treatment used and cardiac outcome in infants under one year of age attending for KD in a third level pediatric hospital in Mexico City, Mexico., Methods: A cross-sectional study was conducted in our hospital from August 1995 to August 2019. We analyzed the clinical features, laboratory results, treatment used and cardiac outcomes in infants younger than one year of age and compared them with older patients., Results: We included 687 patients, 152 were younger than one year of age (22.1%). There was a delayed diagnosis in younger patients with an increased frequency of incomplete clinical presentations. Coronary artery abnormalities were most common in younger infants who also had an increased frequency of giant coronary artery aneurysms. Two patients in the younger group died in the acute phase of KD of myocardial infarction., Conclusions: Diagnosis of KD in infants younger than 1 year of age is a clinical challenge with an increased rate of incomplete clinical presentations and also an increased risk of development of severe cardiac complications.

Objective: To characterize the use of adjunctive therapy in Kawasaki disease (KD) in Latin America. Methods: The study included 1,418 patients from the Latin American KD Network (REKAMLATINA) treated for KD between January 1, 2009, and May 31, 2017. Results: Of these patients, 1,152 received only a single dose of IVIG, and 266 received additional treatment. Age at onset was similar in both groups (median 2 vs. 2.2 years, respectively). The majority of patients were male (58 vs. 63.9%) and were hospitalized with the first 10 days of fever (85.1 vs. 84.2%). The most common adjunctive therapy administered was steroids for IVIG-resistance, followed by additional doses of IVIG. The use of biologics such as infliximab was limited. KD patients who received adjunctive therapy were more likely to have a lower platelet count and albumin level as well as a higher Z score of the coronary arteries. Conclusion: This is the first report of adjunctive therapies for KD across Latin America. IVIG continues to be the initial and resistance treatment, however, steroids are also used and to a lesser extent, biological therapy such as infliximab. Future studies should address the barriers to therapy in children with acute KD throughout Latin America., (Copyright © 2020 Fortuna-Reyna, Bainto, Ulloa-Gutierrez, Garrido-García, Estripeaut, del Águila, Gómez, Faugier-Fuentes, Miño-León, Beltrán, Cofré, Chacón-Cruz, Saltigeral-Simental, Martínez-Medina, Dueñas, Luciani, Rodríguez-Quiroz, Camacho-Moreno, Viviani, Alvarez-Olmos, Marques, López-Medina, Pirez, Tremoulet and the Kawasaki Disease REKAMLATINA Network Study Group.)

Background: Kawasaki disease (KD) is an acute febrile illness that largely affects young children before 5 years of age. Younger children with KD are reported to have a higher prevalence of coronary artery abnormalities. Little is known about infants in the first 3 months of age diagnosed with KD. Methods: A retrospective study was conducted at the National Institute of Pediatrics in Mexico City from 1995 to 2019. Clinical features, laboratory results and cardiac outcomes were recorded. Infants in the first 3 months of age were compared with older patients. Wilcoxon-Mann-Whitney analysis was performed for continuous variables and Fisher's exact test for categorical variables. Results: Six hundred and eighty-eight cases of KD were included in this study. Fourteen cases were diagnosed in the first three months of age. Heart failure and KD shock-syndrome was found in five cases (35.7%). Giant coronary artery aneurysms were found in six cases in the younger group (42.9%). Conclusions: Diagnosis of KD in children younger than 3 months of age is rare. In most cases, an incomplete presentation contributed to a delay diagnosis, treatment, and complications. Younger patients with KD have an increased risk of presenting cardiac complications, including giant coronary artery aneurysms, shock, and death., (Copyright © 2020 Garrido-García, Gutiérrez-Alanis, Ramírez-Perea, Tremoulet and Yamazaki-Nakashimada.)

Introduction: QT interval prolongation is associated with ventricular arrhythmias and sudden death syndrome., Objective: To determine the value of QTc interval in healthy newborns in a general hospital in Jalisco, Mexico, and their outcome during their first months of life., Methods: The study included healthy newborns from March to November 2016, in the Hospital General of Occidente in Jalisco, Mexico. A 12-lead electrocardiogram was performed at a speed of 25mm/s during the first 48h of life. The QT interval was measured in lead DII, and the QTc interval was calculated using the Bazett formula. Patients detected with QTc prolongation were assessed monthly with an ECG and echocardiogram. If they persisted with prolonged QTc interval, they were re-evaluated at 6 months with an ECG, 24h Holter, and electrocardiography study on parents and siblings. Those who persisted with prolonged QTc interval were evaluated with an ECG at 9 months., Results: The study included 548 patients. The mean QTc interval at birth was 459 ms; during this period 33 patients has a QTc greated that 470 ms; which were evaluated monthly with a new electrocardiographic study, obtaining a mean QTc interal of 446 ms. At 6 months 16 patients were evaluated, with a mean QTc interval of 434ms. At 9 months, 6 patients were found to have a mean QTc interval of 438ms, and only 4 patients persisted with a prolonged QTc interval., Conclusions: The QTc interval in our population is prolonged compared to other populations and with a gradual return to normal., (Copyright © 2017 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.)

Objectives: To describe the cardiac manifestations in the acute phase of patients with Kawasaki disease treated in a third level Children's hospital in Mexico City, Mexico., Methods: A cross-sectional study was conducted in patients with a diagnosis of Kawasaki disease treated in this hospital from August 1995 to December 2016. Information included patient demographics, clinical features, treatment used, electrocardiographic findings, extra-coronary echocardiographic findings, and the development of coronary artery aneurysms in the acute phase of the disease., Results: The study included 508 cases of Kawasaki disease, with a mean age at diagnosis of 37.64±35.56 months (range from 2 to 200 months). Almost two-thirds (65.4%) of the patients were male, with a male/female ratio of 1.88:1. Complete Kawasaki disease was diagnosed in 79.2% of cases. Almost all cases (92.4%) received intravenous immunoglobulin. Twenty-eight patients (5.5%) developed arrhythmias, ST changes developed in 29 patients (5.6%), and 5 patients presented with ischaemic changes. In the initial echocardiographic evaluation, 51 patients (9.9%) were diagnosed with myocarditis, 72 patients (14.0%) with pericarditis and 77 cases (15.0%) developed pericardial effusion. Coronary artery anomalies were detected in 169 cases (32.9%). 32 cases were diagnosed as giant coronary aneurysms. Four patients died from cardiac complications in the acute phase of the disease., Conclusions: There has been an increase in the diagnosis of Kawasaki disease in Mexico. They presented with more cardiac complications than reported in literature. An increased knowledge of Kawasaki disease is required in Mexico in order to establish the cardiac outcomes of this group of patients., (Copyright © 2018 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.)

Background: One of the most important complications of Kawasaki disease is the development of giant coronary aneurysms. Risk factors for their development are still not clear., Methods: A retrospective analysis was conducted at the National Institute of Paediatrics in Mexico City, Mexico. It included all patients with a diagnosis of acute Kawasaki disease between August, 1995 and August, 2015. Clinical and laboratory findings, as well as echocardiographic measurements, were recorded. Patients with giant coronary aneurysms (z-score⩾10) were compared with the rest of the patients. A value of p<0.05 was considered statistically significant. Odds ratios and their 95% confidence intervals were calculated to define risk factors., Results: During the study period, 416 patients were diagnosed with Kawasaki disease. Of them, 34 developed giant coronary aneurysms during the acute stage of the disease. In the multivariate analysis, patients younger than 1 year, those with a higher duration of illness at the time of diagnosis, and those who received additional intravenous immunoglobulin showed a significantly higher frequency of giant coronary aneurysms., Conclusions: One of the main factors associated with the development of giant coronary aneurysms was the delay in the diagnosis of Kawasaki disease. This finding highlights the importance of maintaining a high suspicion of the disease, which would enable an early diagnosis and prompt treatment and decrease the risk for developing giant coronary aneurysms.

Background: Kawasaki disease (KD) is an acute self-limited systemic vasculitis that primarily affects children <5 years old. There is no specific diagnostic test. A reaction at the Bacille Calmette-Guérin (BCG) inoculation site has been reported as a common finding in patients with KD where BCG vaccination is mandatory. The purpose of this study was to evaluate the frequency of reactivation of the BCG in Mexican children diagnosed with KD., Methods: A retrospective study of all patients diagnosed with KD from August 1, 1995, to August 31, 2015, at our Institution was performed. The clinical profile, laboratory results, treatment used and coronary artery abnormalities in the BCG reactive and the BCG nonreactive groups were analyzed and compared., Results: We included 399 patients with KD. Ninety-seven (24.3%) had BCG reaction at the inoculation site. The BCG(+) group was younger than the BCG(-) group (P < 0.000). There were 18 (18.55%) incomplete cases in the BCG(+) group compared with 65 (21.52%) in the BCG(-) group without statistical significance. The BCG+ group developed coronary artery aneurysms in 37 cases and the BCG(-) group developed coronary artery aneurysms in 111 cases without statistical significance. Multivariate analysis showed that younger age at diagnosis was the only variable associated with a reaction at the BCG inoculation site in patients with KD., Conclusions: In Mexico, a country with a National BCG Vaccination Program and a low incidence of KD, reaction at the BCG inoculation site could be a useful diagnostic sign of KD.

Kawasaki disease is a small-to-medium-vessel vasculitis of unknown origin that predominantly affects children, although the disease can occur in adults. We report the case of a 26-year-old paediatric resident with fever, exanthema, ocular changes, arthralgia, and desquamation of palms and soles. Diagnosis was established after the fever resolved, and no treatment with intravenous gammaglobulin was administered. His echocardiogram showed normal coronary arteries. Acute Kawasaki disease in adults is a rare and under-recognised condition. It is important to consider the disease in patients with prolonged fever associated with unusual clinical features.

Pompe disease or glycogen-storage disease type 2 (GSD2, OMIM 232300) is an autosomal recessive disorder caused by mutations in the acid alpha-glucosidase gene. Late-onset GSD2 resembles some limb-girdle and Becker muscular dystrophies. The screening of GSD2 through the measurement of acid alpha-glucosidase activity in dried blood spots was applied to a selected sample of 5 Mexican patients with proximal myopathies of unknown etiology. Only 1 male patient showed a low level of acid alpha-glucosidase activity and a compound heterozygote genotype for the c.-32-13T>G splicing mutation present in most white late-onset Pompe disease cases and the novel mutation p.C558S. To our knowledge, this is the first report of a Mexican patient with late-onset GSD2. The identification of c.-32-13T>G in our patient could reflect the genetic contribution of European ancestry to the Mexican population. The enzymatic screening of GSD2 could be justified in patients with myopathies of unknown etiology.

Kawasaki disease (KD) is one of the most common vasculitic disorders in children. The diagnosis is made based on the presence of clinical criteria. We present an exceptional case that fulfilled the clinical criteria of KD except the presence of fever. We consider that the criteria should be revised, with fever not to be considered as an obligate criterion. The similarities of KD with recurrent toxin-mediated perineal erythema are discussed, the latter being a possible attenuated form of KD.

We report the results of radiofrequency catheter ablation in 203 patients under 18 years of age with supraventricular tachyarrhythmias between April 1992 and June 1997. The presence of an accessory pathway caused the tachyarrhythmia in 181 patients (89.1%) with a total of 187 accessory pathways; atrioventricular nodal reentry caused the arrhythmia in 18 patients (8.8%) and atrial flutter in only 4 patients (1.9%). We eliminated the accessory pathway in 171 patients (91.4%), 23 patients showed recurrence of the tachycardia and we had complications in 4 patients (2.1%). The procedure was successful in the treatment of the atrioventricular nodal reentry in the 18 cases, with ablation of the slow pathway in 17 cases and in only one patient of the fast pathway, one patient showed total A-V block, and recurrence of the arrhythmia in 3 cases (16.6%). Finally the procedure was successful in the 4 cases of atrial flutter with one recurrence (25%). In the total of the series, the radiofrequency catheter ablation was successful in 193 patients (95%), with recurrence of the arrhythmia in 27 cases (13.3%) and with complications in only 5 patients (2.6%). Radiofrequency catheter ablation is a safe and effective procedure for the definitive treatment of supraventricular tachyarrhythmias in children.