Características del síndrome inflamatorio multisistémico asociado a COVID-19 en niños atendidos en un hospital peruano, 2020-2022 / Characteristics of COVID-19-associated multisystemic inflammatory syndrome in children treated in a Peruvian hospital, 2020-2022

RESUMEN El propósito del estudio fue describir las características del síndrome inflamatorio multisistémico asociado a COVID-19 (SIM-C) en los primeros tres años de pandemia en niños de un hospital pediátrico del Perú. Se realizó un estudio observacional, descriptivo con datos de 73 pacientes y se describieron las características clínicas, laboratoriales, tratamiento y complicaciones según la ola de la pandemia y si tuvieron shock. La mediana de edad fue 6 años, las manifestaciones gastrointestinales y mucocutáneas fueron frecuentes en las tres olas. El fenotipo similar a enfermedad de Kawasaki se presentó en 34 (46,6%) pacientes y 21 (28,8%) pacientes desarrollaron shock. El tratamiento más usado fue la inmunoglobulina (95,9%), ácido acetil salicílico (94,5%) y corticoide (86,3%). Cinco (7%) pacientes tuvieron aneurisma coronario y 17 (23,3%) ingresaron a la unidad de cuidados intensivos (UCI). Los pacientes con shock tuvieron mayor alteración laboratorial y necesidad de ventilación mecánica. En conclusión, el SIM-C ha disminuido en los primeros tres años de pandemia posiblemente por la vacunación de COVID-19 en niños.

ABSTRACT This study aimed to describe the characteristics of multisystemic inflammatory syndrome associated with COVID-19 (MIS-C) in the first three years of the pandemic in children in a pediatric hospital in Peru. We conducted an observational, descriptive study with data from 73 patients and described the clinical and laboratory characteristics, treatment and complications according to the wave of the pandemic and whether they had shock. The median age was 6 years, gastrointestinal and mucocutaneous manifestations were frequent in the three waves. Kawasaki disease-like phenotype was present in 34 (46.6%) patients and 21 (28.8%) patients developed shock. The most commonly used treatment was immunoglobulin (95.9%), followed by acetylsalicylic acid (94.5%) and corticosteroid (86.3%). Five (7%) patients had coronary aneurysm and 17 (23.3%) were admitted to the intensive care unit (ICU). Patients with shock had greater laboratorial alteration and need for mechanical ventilation. In conclusion, MIS-C has decreased in the first three years of the pandemic, possibly due to COVID-19 vaccination in children.

Diagnóstico tardío de tuberculosis diseminada: reporte de caso / Late diagnosis of disseminated tuberculosis: A case report

Introducción Los pulmones son más afectados en la tuberculosis. La infección también puede comprometer a otros órganos a través de la diseminación linfohematógena. La presentación del cuadro clínico de la tuberculosis diseminada es variable. El diagnóstico es difícil, porque las manifestaciones clínicas son diversas. Más del 50% de los pacientes acuden tardíamente, porque las pruebas microbiológicas dependen de procedimientos invasivos para el cultivo de micobacterias y la histopatología de apoyo. Caso clínico Paciente varón de 30 años, persona privada de su libertad, sin comorbilidades, ingresó al hospital por dolor intenso en muñeca izquierda, con historia previa de haber recibido glucocorticoides sistémicos durante siete meses. Desarrolló cuadro clínico de tuberculosis pulmonar en pleura, en articulación de la muñeca izquierda y en testículo izquierdo. En los análisis se confirmó presencia de . Fue intervenido quirúrgicamente en muñeca y en el testículo. Además, recibió tratamiento para tuberculosis sensible. Concomitantemente se diagnosticó secuelas de Cushing iatrogénico, anemia crónica y proctitis crónica inactiva. Conclusiones El diagnóstico de tuberculosis diseminada fue difícil debido al cuadro clínico inespecífico, a las limitaciones de herramientas de diagnóstico confirmatorio y a las evaluaciones especializadas en forma oportuna. El uso prolongado de corticoides sistémicos habría influido en la diseminación de la tuberculosis.

Introduction The lungs are most commonly involved in tuberculosis, but infection can also involve other organs through lymphohematogenous dissemination. The clinical presentation of disseminated tuberculosis is variable. Diagnosis is difficult, because clinical manifestations are diverse, more than 50% of patients present late, because microbiological testing relies on invasive procedures for mycobacterial culture and supportive histopathology. Case report A 30-year-old male patient, deprived of his liberty, with no co-morbidities, was admitted to the hospital for severe pain in the left wrist, with a previous history of having received systemic glucocorticoids for 7 months. He developed clinical symptoms of pulmonary tuberculosis, in the pleura, in the joint of the left wrist and in the left testicle, and tests confirmed the presence of M. tuberculosis. He underwent surgery on the wrist and testicle and was also treated for susceptible tuberculosis. Concomitant sequelae of iatrogenic Cushing's disease, chronic anemia and chronic inactive proctitis were diagnosed. Conclusions Diagnosis of disseminated tuberculosis was difficult due to the non-specific clinical picture, limitations of confirmatory diagnostic tools and timely specialized evaluations. Prolonged use of systemic corticosteroids may have played a role in the dissemination of tuberculosis.

Hot cross bun in pediatric age multi-system atrophy an unusual presentation

A 10-year-old female was brought to emergency department with complaint fever, cold, and cough for 5 days followed by weakness of upper limb weakness more than Lower limb associated with inability to walk/stand, brought to emergency with altered sensorium. Nervous system: Confused and altered, tone is normal, power: 3/3 in upper limbs 2/2 in lower limbs and reflexes in bilateral knee brisk, bilateral plantar-extensor; On general examination revealed hypomimia, dysarthria and bilateral bradykinesia along with ataxic gait and pyramidal signs. Blood investigation were normal and diagnosis made by neuroimaging s/o: Bilaterally symmetrical abnormal signal in both postero-medial thalami, bilateral insular cortices, pons and bilateral middle cerebellar peduncles. It is extending into pons involving transverse pontocerebellar tracts and median pontine raphe nuclei giving 'Hot cross bun sign' (HCBS). During course of treatment child had autonomic disturbances. Child was treated with supportive medication and methyl-presdnisolone followed by oral steroid. Child had responded to treatment given and child has been discharged with no neurological deficit on oral medications. We concluded autonomic dysfunction in any patient presenting with acute onset of weakness with short duration must evaluate for MSA and institute appropriate treatment.

Gastrointestinal and hepatic manifestations of COVID-19 in children: A systematic review and meta-analysis

Background@#Children with COVID-19 may present with gastrointestinal (GI) symptoms and liver dysfunction.@*Objective@#To determine the type and prevalence of gastrointestinal (GI) and hepatic manifestations of COVID-19 in children and its association with severity of illness.@*Methods@#A systematic literature search was done from inception until January 4, 2021 using PubMed, Cochrane Library, Google Scholar and prepublication repositories with no language restrictions. Studies that reported the demographic and clinical features of children with COVID-19 and provided data on their GI and hepatic signs and symptoms were included. Prevalence of GI and hepatic manifestations were pooled using Stata14.@*Results@#We included 58 studies with total of 4497 participants. Overall, one-third of children with COVID-19 presented with at least one GI symptom (33.8%; 95% confidence interval (CI) 23.0, 45.4; I2 97.5%; 42 studies, 3327 participants) with abdominal pain, nausea or vomiting, and diarrhea each occurring in approximately 20%. Children with severe COVID-19 were more likely to present with GI symptoms (odds ratio 2.59; 95% CI 1.35, 4.99; I2 24%; 4 studies, 773 participants). The pooled prevalence of elevated transaminases was 11% for both AST (11.3%, 95% CI 4.9, 19.3; I2 74.7%; 11 studies, 447 participants) and ALT (11.2%, 95% CI 7.1, 16.0; I2 40.8%; 15 studies, 513 participants). Hepatic findings such as jaundice (2-17%), hepatomegaly (2%) or behavioral changes (2%) from hepatic encephalopathy were variably reported by a few studies. The degree of heterogeneity was not improved on exclusion of studies with poor quality, but markedly improved on subgroup analysis according to geographical region and presence of MIS-C. Studies from China showed that children with COVID-19 had significantly lower pooled prevalence for any of the GI symptoms with low degree of heterogeneity, particularly for diarrhea, nausea/vomiting, and abdominal pain, all of which had I2 of 0%. Those with multisystem inflammatory syndrome in children (MIS-C) had significantly more common GI symptoms and increased transaminases than those without.@*Conclusion@#One-third of children with COVID-19 exhibit at least one GI symptom and more likely present in those with severe disease. Elevated transaminases were present in 10%. Prevalence of GI and hepatic manifestations were higher among children with MIS-C.

Clinical characteristics and risk factors for hypotension in children with multisystem inflammatory syndrome related to SARS-CoV-2 / 中国小儿急救医学

Objective:To summarize the clinical characteristics of multisystem inflammatory syndrome in children（MIS-C）related to SARS-CoV-2 and analyze the risk factors for hypotension.Methods:The clinical data of children with MIS-C who visited Beijing Children's Hospital from January 2023 to March 2023 were retrospectively collected,and the clinical characteristics and risk factors for hypotension were analyzed.Results:Thirty-six patients were included,the median age was 3.1 (2.3,5.8) years; 61.1% were boys.All cases had a history of SARS-CoV-2 infection between 1 and 9 weeks before onset.Clinical symptoms included fever (100.0%); rash (94.4%); bilateral conjunctival congestion (88.8%); lips/oral cavity changes (100.0%); swollen hands and foot (61.1%); cervical lymphadenopathy (88.8%); acute gastrointestinal symptoms (61.1%) of diarrhea,abdominal pain and vomiting; neurologic manifestations (33.3%) of lethargy,headache,and convulsions; respiratory symptoms(41.6%) of cough,sore throat and shortness of breath.Nineteen (52.8%) cases developed hypotension and needed vasoactive drugs support to maintain blood pressure.Mechanical ventilation was required in four(11.1%) cases,and one patient was intubated and treated with continuous renal replacement therapy.The level of ferritin in hypotension group was significantly higher than those in normal blood pressure group( P＜0.05),while the levels of platelet count,serum sodium and potassium in hypotension group were significantly lower than those in normal blood pressure group( P＜0.05).Logistic regression multivariable analysis found the elevated serum ferritin ( OR=20.000,95% CI:2.750-145.481, P=0.003) was an independent risk factor for hypotension. Conclusions:MIS-C may be observed following SARS-CoV-2 infection.Various symptoms and signs,including fever,multiple organ dysfunction,and hypotension,may occur in children with MIS-C.Elevated ferritin is a risk factor for hypotension.

Study on the similarities and differences of orthostatic hypotension in patients with Parkinson′s disease and multiple system atrophy / 中华神经科杂志

Objective:To summarize and compare the characteristics of orthostatic hypotension (OH)　in　patients　with　Parkinson′s disease and multiple system atrophy (MSA).Methods:The active standing test data of 210 Parkinson′s disease patients (Parkinson′s disease group) and 85 MSA patients (MSA group) admitted to the Department of Neurology, Xuanwu Hospital, Capital Medical University from January 2021 to March 2022 were retrospectively analyzed. Demographic information, clinical data, Hoehn-Yahr staging, and Unified Parkinson′s Disease Rating Scale (UPDRS), Non-Motor Symptoms Questionnaire (NMSQ), Montreal Cognitive Assessment Scale and Mini-Mental State Examination scores were collected. The comparative analysis of OH was conducted according to the changes of heart rate and blood pressure during the active standing test.Results:Among the 85 patients with MSA, 52 were found with MSA parkinsonism variant (MSA-P) and 33 with MSA cerebellar variant (MSA-C). The 210 Parkinson′s disease patients were aged (61.5±11.0) years, with 116 males (55.2%). The 85　MSA　patients　were aged (60.1±6.8) years, with 44 males (51.8%). Compared with the Parkinson′s disease group, the Hoehn-Yahr staging [2.0(2.0, 3.0) vs 3.0(2.0, 3.0), Z=-5.278, P<0.001], NMSQ[ 25.0(11.0,46.5) vs 45.0(24.0,70.0), Z=-3.632, P<0.001] and UPDRS scores [50.0(32.0,68.0) vs 65.5(44.5,78.5), Z=-3.073, P=0.003] in the MSA group were higher. The incidence of OH in the MSA group was higher than that in the Parkinson′s disease group [63.5% (54/85) vs 25.7%(54/210), χ 2= 37.284, P<0.001], but there was no statistically significant difference between the MSA-P and MSA-C groups . Compared with the Parkinson′s disease group, the MSA group had a higher incidence of classical OH [54.1%(46/85) vs 12.9%(27/210), χ 2=55.316, P<0.001] and neurogenic OH [36.5%(31/85) vs 9.0%(19/210), χ 2=32.326, P<0.001],but there was no statistically significant difference in the incidence of initial OH and delayed OH between the two groups. The incidence of severe OH in the MSA group was also higher than that in the Parkinson′s disease group [57.6%(49/85) vs 16.7%(35/210), χ 2=49.894, P<0.001], but there was no statistically significant difference in the incidence of pre-clinical OH and mild OH between the two groups. Conclusions:The incidence, time change, severity and pathophysiological basis of OH in Parkinson′s disease and MSA patients are different. Different types of OH may help to distinguish MSA from Parkinson′s disease.

Multisystem inflammatory syndrome in children in the context of coronavirus disease 2019 pandemic / 中国当代儿科杂志

Multisystem inflammatory syndrome in children (MIS-C) is a complex syndrome characterized by multi-organ involvement that has emerged in the context of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) outbreak. The clinical presentation of MIS-C is similar to Kawasaki disease but predominantly presents with fever and gastrointestinal symptoms, and severe cases can involve toxic shock and cardiac dysfunction. Epidemiological findings indicate that the majority of MIS-C patients test positive for SARS-CoV-2 antibodies. The pathogenesis and pathophysiology of MIS-C remain unclear, though immune dysregulation following SARS-CoV-2 infection is considered a major contributing factor. Current treatment approaches for MIS-C primarily involve intravenous immunoglobulin therapy and symptomatic supportive care. This review article provides a comprehensive overview of the definition, epidemiology, pathogenesis, clinical presentation, diagnosis, treatment, and prognosis of MIS-C.

Multisystem inflammatory syndrome in children related with COVID-19 infection / 中国免疫学杂志

Multisystem inflammatory syndrome in children is a disease related with severe acute respiratory syndrome corona-virus 2 infection,which can involve multiple system damage.Most cases included features of shock,cardiac dysfunction,gastrointestinal symptoms,significantly elevated markers of inflammation and cardiac damage,and positive test results for SARS-CoV-2 by serology.Although its clinical features overlap with Kawasaki disease,it is more likely to occur in older children and adolescents,and most of them need to be treated with intensive care and a variety of immunomodulators.At present,the pathogenesis and long-term prognosis of the disease need to be further studied.

Reinfección por SARS-CoV-2 en pacientes con antecedentes de MIS-C. Presentación de dos casos / MIS-C patients who were reinfected with SARS-CoV-2. Report of 2 cases

El síndrome inflamatorio multisistémico pediátrico (MIS-C, por su sigla en inglés) es una enfermedad rara. Se desconoce si los niños que se recuperaron del MIS-C tienen riesgo de recurrencia de MIS-C cuando presentan reinfección por SARS-CoV-2. El objetivo de este estudio es describir los casos de dos niñas que se recuperaron del MIS-C y presentaron reinfección por SARS-CoV-2 sin recurrencia de MIS-C.

Multisystem inflammatory syndrome in children (MIS-C) is a rare condition. It is still unknown if children who have recovered from MIS-C are at a risk of recurrence of MIS-C when they are reinfected with SARS-CoV-2. In this study, we aimed to report 2 children who recovered from MIS-C and reinfected with SARS-CoV-2 without recurrence of MIS-C.

Clinical features of multisystem inflammatory syndrome in children challenges for clinicians: a case report

A variety of complications came to light after the SARS-CoV-2 pandemic of 2020. One such post-covid complication that manifests itself in the form of a hyperinflammatory syndrome in the pediatric population is multisystem inflammatory syndrome in children (MIS-C). It results in severe inflammation of a variety of organ systems, including the heart, lungs, brain, kidneys, gastrointestinal system, skin, and eyes. Surprisingly, clinicians can easily mistake this type of presentation for many other diseases due to overlapping features, especially Kawasaki disease (KD). An interesting case report on a patient admitted to the Grodno regional infectious diseases clinical hospital, Grodno, Belarus. The patient was initially diagnosed with enteroviral infection (EVI) at the time of admission. The clinicians in charge observed the underlying cause to be masked by Kawasaki-like presentation, how they diagnosed MIS-C, Kawasaki-like phenotype: exanthema, cheilitis, scleritis, infectious cardiopathy, gastrointestinal syndrome, coagulopathy and managed this patient is described in this scientific paper. Although the presenting signs and symptoms of MIS-C overlap with other diseases, certain additional features can be helpful in differentiation. Mainly MIS-C is present in a relatively older subgroup along with gastrointestinal symptoms that are uncommon for KD. The patient was treated with IVIG and steroids after which he attained full recovery. MIS-C associated with COVID-19 is serious, rare, and potentially fatal. Clinicians, primary care physicians, and emergency department pediatricians must be quick to recognize it and treat it at the earliest by deploying immunomodulatory strategies to subdue systemic injury caused by hyper-inflammation.

Características clínicas y demográficas de los niños hospitalizados con infección por SARS-CoV-2 / Clinical and demographic characteristics of children hospitalized with SARS-CoV-2 infection / Características clínicas e demográficas de crianças hospitalizadas com infecção por SARS CoV-2

Introducción: en marzo de 2020 se registraron los primeros casos de infección por SARS-CoV-2 en Uruguay y se decretó la emergencia sanitaria. Objetivo: describir las características clínicas demográficas de los menores de 15 años hospitalizados con infección por SARS-CoV-2 en el período 13 de marzo de 2020 al 30 de septiembre de 2021 en el Hospital Pediátrico del Centro Hospitalario Pereira Rossell, centro de referencia público de Uruguay. Metodología: estudio descriptivo, retrospectivo, en el que se describen edad, manifestaciones clínicas, comorbilidades, severidad, tratamiento y evolución. Resultados: se hospitalizaron 207 niños con una frecuencia de 1,6%. La mediana (rango intercuartil) de edad fue 1,5 años (3 meses - 8 años); <1 año 44%; 54% de sexo masculino. Presentaron comorbilidades, 59 niños. Fueron sintomáticos, 71%. De los sintomáticos, presentaron síntomas leves 48%. Las manifestaciones clínicas fueron respiratorias en 96 (65%) y no respiratorias en 51 (fiebre sin foco 15, digestivas 19, exantema viral 3, SIM-Ped S 10 y atípicas 3). Treinta niños ingresaron a unidad de cuidados intensivos (UCI) y tres requirieron ventilación invasiva. Estos pacientes presentaron comorbilidades, tuvieron más días de fiebre y necesitaron oxigenoterapia que los que no requirieron UCI. Un paciente de 2 años con comorbilidades falleció. Conclusión: la frecuencia de hospitalizaciones fue de 1,6%. La mayoría de los niños sintomáticos presentaron formas leves. En los sintomáticos las manifestaciones fueron respiratorias. Los hallazgos en esta serie aportan al conocimiento del comportamiento de la infección por SARS-CoV-2 en niños.

Introduction: in March 2020, the first cases of SARS CoV-2 infection were registered in Uruguay and a health emergency was decreed. Objective: To describe the clinical and demographic characteristics of children under 15 years of age hospitalized with SARS-CoV-2 infection from March 13, 2020, to September 30, 2021, at Pereira Rossell Pediatric Hospital, a public reference center in Uruguay. Method: descriptive, retrospective study describing age, clinical manifestations, comorbidities, severity and treatment. Results: a total of 207 children were hospitalized, with a frequency of 1.6%. The median (interquartile range) age was 1.5 years (3 months - 8 years); <1 year accounted for 44%, and 54% were male. Comorbidities were present in 59 children. 71% of them were symptomatic, and among the symptomatic cases, 48% presented mild symptoms. Clinical manifestations were respiratory in 96 (65%) cases and non-respiratory in 51 (fever without a focus 15, gastrointestinal 19, viral exanthem 3, pediatric inflammatory multisystem syndrome 10, and atypical 3). Thirty patients were admitted to the Intensive Care Unit (ICU), and 3 required invasive ventilation. These patients had comorbidities, more days of fever, and required oxygen therapy compared to those who did not need ICU. One 2-year-old patient with comorbidities died. Conclusion: the hospitalization frequency was 1.6%. Most symptomatic children had mild forms of the disease. Among the symptomatic cases, respiratory manifestations were predominant. The findings from this series contribute to the understanding of the behavior of SARS-CoV-2 infection in children.

Introdução: Os primeiros casos de infecção por SARS CoV-2 no Uruguai foram registrados em março de 2020 quando foi decretada a emergência sanitária. Objetivo: descrever as características clínicas e demográficas das crianças menores de 15 anos internadas com infecção por SARS CoV-2 no período 13 de março de 2020 - 30 de setembro de 2021 no Hospital Pediátrico do Centro Hospitalar Pereira Rossell, centro público de referência no Uruguai. Metodologia: estudo descritivo, retrospectivo, incluindo idade, manifestações clínicas, comorbidades, gravidade, tratamento e evolução. Resultados: 207 crianças foram internadas com infecção por SARS CoV-2 correspondendo a frequência de 1,6% do total de crianças hospitalizadas no período estudado. A mediana (intervalo interquartil) de idade foi de 1,5 anos (3 meses - 8 anos) dos quais 44% eram <1 ano 44% e 54% do sexo masculino. 59 crianças apresentaram comorbidades. 71% eram sintomáticas sendo que 48% delas apresentaram sintomas leves. As manifestações clínicas foram respiratórias em 96 (65%) e não respiratórias em 51 (febre sem foco 15, digestiva 19, exantema viral 3, SIM-Ped S 10 e atípico 3). 30 crianças foram internadas na Unidade de Terapia Intensiva e 3 precisaram de ventilação invasiva; esses pacientes apresentavam comorbidades, necessitaram de oxigenoterapia e tiveram mais dias de febre do que aqueles que não necessitaram de UTI. Uma paciente de 2 anos com comorbidades faleceu. Conclusão: a frequência de internações foi de 1,6%. A maioria das crianças sintomáticas apresentou formas leves. Nas sintomáticas as manifestações foram respiratórias. Os achados desta série contribuem para o conhecimento do comportamento da infecção por SARS CoV-2 em crianças.

Clinical profile, cardiac involvement and outcome of children admitted with multisystem inflammatory syndrome in PICU at GB Pant children hospital Srinagar

Background: Multisystem inflammatory syndrome in children (MIS-C) is a severe hyper inflammatory post infectious complication of acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection, which typically occurs 2-6 weeks after exposure to SARS-CoV-2. Aim was to determine the clinical profile, cardiac involvement and outcome of children admitted with multisystem inflammatory syndrome in pediatric intensive care unit.Methods: This prospective observational study was conducted in pediatric intensive care unit over period of two years. After informed consent from parents, all those patients meeting inclusion criteria were subjected to complete history, general and systemic physical examination. Routine baseline investigations included CBC, LFT, KFT, ABG, serum calcium and phosphorous, and other investigations like echocardiography, COVID-19 RAT and RTPCR and various inflammatory markers like serum ferritin, pro-calcitonin, CRP and ESR whenever required were done.Results: In our study out of 77 MIS-C patients 40 were males and 37 were females with a male female ratio of 1.1:1. The mean average age was 7.4 years. Out of them 47 (61%) patients had a history of COVID-19 infection/contact with positive COVID-19 cases 3 to 4 weeks before presentation. In our study gastrointestinal, respiratory, and cardiac systems were mostly involved. Rash and conjunctival congestion was seen in 81% of MIS-C patients. On echocardiography out of 77 MIS-C patients, 15 (19.5%) had pericardial effusion, 25 (32.5%) had coronary artery dilatations and 32 patients (41.5%) had left ventricular systolic dysfunction with LVEF <55%.Conclusions: Pediatric multisystem inflammatory syndrome is a serious and life-threatening illnesses having a significant impact on morbidity and mortality.

Inmunización con vacunas ARN mensajero contra la COVID-19 en pacientes adolescentes con antecedente de síndrome inflamatorio multisistémico: serie de casos / Immunization with messenger RNA vaccines against COVID-19 in adolescents with a history of multisystem inflammatory

Los niños cursan mayormente la infección por el virus SARS-CoV-2 en forma leve. Sin embargo, de forma muy infrecuente algunos pueden desarrollar una patología con marcada gravedad denominada síndrome inflamatorio multisistémico en niños relacionado temporalmente con COVID-19 (SIM-C). Dado su reciente surgimiento, aún hay aspectos de su fisiopatología que se desconocen. La posibilidad de recidiva en caso de reinfección o ante la vacunación contra SARS-CoV-2 son nuevos interrogantes a los que nos enfrentamos. Reportamos una serie de casos de 4 pacientes adolescentes que cursaron SIM-C y meses después han sido vacunados contra SARS-CoV-2 con plataformas ARN mensajero (ARNm) sin presentar recurrencia de la enfermedad ni efectos adversos cardiológicos

In most cases, children with SARS-CoV-2 have a mild infection. However, very rarely, some children may develop a severe disease called multisystem inflammatory syndrome in children temporally associated with COVID-19 (MIS-C). Given its recent emergence, some aspects of its pathophysiology are still unknown. The possibility of recurrence in case of reinfection or SARS-CoV-2 vaccination are new questions we are facing. Here we report a case series of 4 adolescent patients who developed MIS-C and, months later, received the SARS-CoV-2 vaccine with messenger RNA (mRNA) platforms without disease recurrence or cardiac adverse events.

Síndrome inflamatorio multisistémico pos-COVID como abdomen agudo quirúrgico en pediatría / Multisystem inflammatory syndrome post-COVID as acute abdominal pain in pediatrics

RESUMEN Se presenta el caso de un adolescente masculino, de 15 años, que ingresa por Guardia por dolor abdominal y fiebre de una semana de evolución con RT PCR negativa bajo el diagnóstico presuntivo de apendicitis aguda, que se descarta tras estudios complementarios, y se realiza diagnóstico de síndrome inflamatorio multisistémico asociado a infección por COVID-19.

ABSTRACT We report the case of a 15-year-old adolescent male patient who was admitted to the emergency department due to abdominal pain and fever that started one week before, with negative RT-PCR. The suspected diagnosis was acute appendicitis that was ruled out with complementary tests. The final diagnosis was multisystem inflammatory syndrome associated with COVID-19.

COVID-19 vaccination in children: a public health priority

Abstract Objective: Covid-19 had a direct impact on children's health. The aim of this review was to analyze epidemiological and clinical data, the consequences of the pandemic, and vaccination aspects in this group. Sources of data: The searches were carried out from January 2020 to November 2022, in the MEDLINE databases (PubMed) and publications of the Brazilian Ministry of Health and the Brazilian Society of Pediatrics. Summary of findings: Covid-19 has a mild presentation in most children; however, the infection can progress to the severe form and, in some cases, to MIS-C. The prevalence of the so-called long Covid in children was 25.24%. Moreover, several indirect impacts occurred on the health of children and adolescents. Vaccination played a crucial role in enabling the reduction of severe disease and mortality rates. Children and adolescents, as a special population, were excluded from the initial clinical trials and, therefore, vaccination was introduced later in this group. Despite its importance, there have been difficulties in the efficient implementation of vaccination in the pediatric population. The CoronaVac vaccines are authorized in Brazil for children over three years of age and the pediatric presentations of the Pfizer vaccine have shown significant effectiveness and safety. Conclusions: Covid-19 in the pediatric age group was responsible for the illness and deaths of a significant number of children. For successful immunization, major barriers have to be overcome. Real-world data on the safety and efficacy of several pediatric vaccines is emphasized, and the authors need a uniform message about the importance of immunization for all children.

Neurological manifestations of multisystem inflammatory syndrome in children associated with COVID-19 in a tertiary care centre

Multisystem inflammatory syndrome in children (MIS-C) and adolescents temporally related to COVID-19” is a new entity characterized by fever, multisystem organ involvement, laboratory evidence of inflammation, and laboratory or epidemiological evidence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, in individuals aged 0–19 years with no alternative diagnosis. Neurological manifestations are not part of the diagnostic criteria of MISC and hence remain poorly described. So, we wanted to note down the neurological involvement in multisystem inflammatory syndrome in children (MIS-C) related to severe acute respiratory syndrome with coronavirus infection. Here we describe 6 cases of COVID MISC who presented as acute febrile illness with drowsiness, irritability, convulsions and serious ones with encephalopathy. Focal neurological signs, abnormal brain magnetic resonance imaging (MRI) was present in four patients. MRI brain was normal in 2 cases CSF study was normal in all cases. These patients received intravenous methylprednisolone at 30 mg/kg/day for 3 days. Cases 3 to 6 were given intravenous immunoglobulin (IVIG), the clinical picture rapidly improved in the first three days, and all neurological symptoms disappeared within 10 and 30 days with some sequel in cases 4 to 6. In conclusion we describe clinical and laboratory parameters in these patients with neurological manifestations and we documented an increase in pro inflammatory markers correlating with severity of neurological presentation in children with MISC. High index of suspicion is needed to diagnose neurological manifestations of MISC following COVID-19 pandemic. Neurological spectrum can be broad range of manifestations though outcome was favorable with early treatment.

Polymorphism in cytolytic pathway genes in patients with pediatric inflammatory multisystemic syndrome temporally associated with SARS-CoV-2 / Polimorfismos em genes da via citolítica em pacientes com síndrome inflamatória multissistêmica pediátrica temporariamente associada ao SARS-CoV-2

Introduction: Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a systemic hyperinflammatory disease that occurs in a small number of children after being infected with SARS-CoV-2. Macrophage activation syndrome, an aggressive condition characterized by the excessive inflammation and activation of well-differentiated macrophages, has been shown to occur in patients infected by SARS-CoV-2. Considering the clinical and pathophysiological similarities between these diseases, our main objective was to determine whether gene polymorphisms associated with macrophage activation syndrome were also present in patients with PIMS-TS. Methods: DNA from 10 pediatric patients with PIMS-TS (case group) and ten COVID-19 patients without PIMS-TS (control group) were genotyped by Real-time PCR analysis (TaqMan®) for single nucleotide polymorphisms (SNP) in four genes associated with macrophage activation syndrome: perforin 1 (PRF1), granzyme B (GZMB), syntaxin 11 (STX11), and syntaxin binding protein 2 (STXBP2). The SNP analysis was performed using the additive, dominant, and recessive models. Results: A significantly higher frequency of an SNP (C wild allele in rs6573910) in the GZMB gene was observed in both the additive and dominant models in the PIMS-TS group than controls. A borderline significant difference was also observed for the G allele in rs7764017 of the STX11 gene in the PIMS-TS group in the additive model. Conclusions: This study indicated the presence of two polymorphisms in genes associated with macrophage activation syndrome (GZMB and STX11) in patients who developed PIMS-TS. If the presence of these SNPs is validated in a larger number of PIMS-TS cases, they can be used as potential biomarkers for early identification of pediatric patients with a higher probability of developing PIMS-TS associated with SARS-CoV-2 infection.

Introdução: A síndrome multissistêmica inflamatória pediátrica temporariamente associada ao SARS-CoV-2 (SIMP-TS) é uma doença hiperinflamatória sistêmica que ocorre em um pequeno número de crianças após serem infectadas pelo SARS-CoV-2. A síndrome de ativação de macrófagos (SAM), uma condição agressiva caracterizada pela inflamação excessiva e ativação de macrófagos bem diferenciados, demonstrou ocorrer em pacientes infectados por SARS-CoV-2. Considerando as semelhanças clínicas e fisiopatológicas entre essas doenças, neste estudo o nosso principal objetivo foi determinar se polimorfismos gênicos associados à SAM também estavam presentes em pacientes com SIMP-TS. Métodos: DNA de dez pacientes pediátricos com SIMP (grupo caso) e dez pacientes COVID-19 sem SIMP (grupo controle) foram genotipados por análise de PCR em tempo real (tecnologia TaqMan®) para polimorfismos de nucleotídeo único (SNPs) em quatro genes selecionados associados com SAM: perforina 1 (PRF1), granzima B (GZMB), sintaxina 11 (STX11) e proteína de ligação de sintaxina 2 (STXBP2). A análise dos SNPs foi realizada utilizando o modelo aditivo, dominante e recessivo. Resultados: Uma frequência significativamente maior de um SNP (alelo selvagem C em rs6573910) no gene GZMB foi observada pelos modelos aditivo e dominante no grupo SIMP quando comparado aos controles. Além disso, uma significância limítrofe foi observada para o alelo G em rs7764017 do gene STX11 no grupo SIMP pelo modelo aditivo. Conclusões: Nosso estudo indicou a presença de dois polimorfismos em genes associados à SAM (GZMB e STX11) em pacientes que desenvolveram SIMP-TS. Uma vez validada a presença desses SNPs em um número maior de casos de SIMP-TS, eles podem ser usados como potenciais biomarcadores para a identificação precoce de pacientes pediátricos com maior probabilidade de desenvolver SIMP-TS associado à infecção por SARS-CoV-2.

Langerhans cell histiocytosis presenting as anterior neck mass in a child: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#Thyroid involvement in Langerhans Cell Histiocytosis (LCH) is rare. We report a 10-year-old Filipino male who presented with a rapidly enlarging goiter. Computed tomography scan showed thyroid and bilateral submandibular masses with malignant features, pulmonary blebs and hepatic cysts. Ultrasound-guided core needle biopsy findings were consistent with LCH and chemotherapy was initiated. This case demonstrates that LCH should be considered in patients with goiter. Multidisciplinary management is warranted to achieve proper diagnosis and institute timely treatment.

Progress on diagnosis and treatment of neonatal-onset multisystem inflammatory disease / 国际儿科学杂志

Neonatal onset multisystem inflammatory disease(NOMID), also known as chronic infantile neurological cutaneous and articular syndrome(CINCA), originates from perinatal period and mainly manifests urticaria, joint lesions, and central nervous system lesions.It is an autoinflammatory disease associated with mutations of NLRP3 located on chromosome 1q44.The early atypical clinical symptoms are prone to misdiagnosis.NOMID/CINCA should be differentiated from infectious diseases, familial cold autoinflammatory syndrome, Muckle-Wells syndrome, systemic juvenile idiopathic arthritis, mevalonate-kinase deficiency, tumor necrosis factor receptor-associated periodic syndrome, and other diseases.NOMID/CINCA is mainly diagnosed based on clinical symptoms, while genetic testing provides an essential supplementary for patients with atypical clinical manifestations.IL-1 targeted therapies including anakinra, rilonacept, and canakinumab, have been proven with sustained efficacy in treating NOMID/CINCA.This article reviews the progress on diagnosis and treatment of NOMID/CINCA.

A case of multisystem inflammatory syndrome in children and literature review / 中国综合临床

Objective:To improve clinicians' understanding of Multisystem Inflammatory Syndrome in Children (MIS-C) and to reduce missed and misdiagnosis.Methods:The clinical data of a patient with Multisystem inflammatory syndrome in children admitted to Maternal and Child Health Hospital of Hubei Province, Tongji Medical College, Huazhong University of Science and Technology in January 2023 were analyzed retrospectively, together with literature review.Results:A 10-year-old male patient was admitted to hospital with a fever for 5 days. This patient had a history of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection 1 month prior to admission; after admission, he continued to have recurring high fevers, accompanied by abdominal pain and Kawasaki disease like manifestations, shock, multiple organ function impairment, and a significant increase in inflammatory indicators. Finally, the patient was diagnosed with MIS-C, treated with intravenous immunoglobulin, glucocorticoids and aspirin, and discharged with clinical improvement.Conclusion:MIS-C was a novel syndrome with Kawasaki-like manifestations.Currently, the overall prognosis for MIS-C is good, but its pathogenesis is unclear and the therapeutic consensus remains limited. More studies are needed to clarify the best diagnosis and treatment methods and true prognosis.