Multisystem inflammatory syndrome in children across 16 Latin American countries: A multicenter study from the REKAMLATINA Network.

Objectives: Our aim was to describe the epidemiology and outcomes of multisystem inflammatory syndrome in children (MIS-C) in Latin America. Methods: We conducted an observational, retrospective, and prospective multicenter study that gathered information from 84 participating centers across 16 Latin American countries between August 1, 2020 and June 30, 2022. Results: Of the 1239 reported children with MIS-C, 84.18% were previously healthy. The most frequent clinical manifestation in our studied population was abdominal pain (N = 804, 64.9%), followed by conjunctival injection (N = 784, 63.3%). The median duration of fever at the time of hospital admission was 5 days and a significant number of subjects required admission to an intensive care unit (N = 589, 47.5%). Most of the subjects (N = 1096, 88.7%) were treated with intravenous immunoglobulin, whereas 76.7% (N = 947) were treated with steroids, of whom 10.6% (N = 100) did not receive intravenous immunoglobulin. The death rate attributed to MIS-C was 4.88%, with a rate of 3.39% for those initially diagnosed with MIS-C and 8.85% for those whose admission diagnosis was not MIS-C (P <0.001, odds ratio 2.76, 95% confidence interval 1.6-4.6). Conclusions: One of the most significant findings from our study was the death rate, especially in those not initially diagnosed with MIS-C, in whom the rate was higher. This highlights the importance of increasing awareness and making an earlier diagnosis of MIS-C in Latin America.

Daño renal agudo y colestasis asociados a la enfermedad de Kawasaki en una paciente de 9 años; reporte de caso / Acute kidney injury and cholestasis associated with Kawasaki disease in a 9-year-old: case report

La enfermedad de Kawasaki (EK) es una vasculitis sistémica frecuente en niños menores de 5 años, involucra arterias coronarias y otros vasos de mediano calibre, además existe evidencia de lesión inflamatoria y proliferativa de la vía biliar e infiltración linfocitaria en el intersticio renal. Se presenta el caso de una niña de 9 años con fiebre de alto grado; desarrollando inyección conjuntival bilateral no purulenta, lengua «aframbuesada», eritema y descamación en dedos de manos y pies, síndrome colestásico, así como edema e incremento de azoados. La EK continúa siendo un reto diagnóstico para el pediatra. En todo paciente con síndrome febril, colestasis y daño renal agudo la EK debe considerarse como diagnóstico diferencial, aunque es necesario realizar más estudios para evaluar esta atípica asociación

Kawasaki disease (KD) is a systemic vasculitis frequent in children younger than 5 years of age. It involves coronary arteries and other medium-sized vessels. There also exists evidence of inflammatory and proliferative changes affecting the biliary tract and lymphocyte infiltration of the renal interstitial. We describe the case of a 9-year-old girl who developed high-grade fever, bilateral non-purulent conjunctivitis, «strawberry» tongue, desquamation of the fingers and toes, cholestatic syndrome, edema and elevated serum creatinine. KD is a diagnostic challenge for the pediatrician. In every patient with high-grade fever, cholestasis and acute kidney injury, KD should be included in the differential diagnosis, even though more research is necessary to evaluate this atypical association

Acute kidney injury and cholestasis associated with Kawasaki disease in a 9-year-old: Case report. / Daño renal agudo y colestasis asociados a la enfermedad de Kawasaki en una paciente de 9 años; reporte de caso.

Kawasaki disease (KD) is a systemic vasculitis frequent in children younger than 5 years of age. It involves coronary arteries and other medium-sized vessels. There also exists evidence of inflammatory and proliferative changes affecting the biliary tract and lymphocyte infiltration of the renal interstitial. We describe the case of a 9-year-old girl who developed high-grade fever, bilateral non-purulent conjunctivitis, «strawberry¼ tongue, desquamation of the fingers and toes, cholestatic syndrome, edema and elevated serum creatinine. KD is a diagnostic challenge for the pediatrician. In every patient with high-grade fever, cholestasis and acute kidney injury, KD should be included in the differential diagnosis, even though more research is necessary to evaluate this atypical association.

Fiebre manchada de montañas rocosas: experiencia en 5 años de vigilancia activa en un hospital pediátrico de segundo nivel en el noreste de México / Rocky mountain spotted fever: five years of active surveillance experience in a second level pediatric hospital in northeastern Mexico

Resumen: Introducción: La fiebre manchada de las montañas rocosas (FMMR) es causada por Rickettsia rickettsii. En Coahuila, la región de la Comarca Lagunera se considera una zona endémica. No se han reportado casos en la zona sur del estado, específicamente en la ciudad de Saltillo. Métodos: Estudio prospectivo, reporte de casos. Se incluyeron los casos de niños atendidos en el Hospital del Niño Dr. Federico Gómez Santos en la ciudad de Saltillo, Coah., del mes de septiembre de 2012 a septiembre 2017 con diagnóstico confirmado clínicamente y por reacción en cadena de la polimerasa (PCR) de FMMR. Se presentan los antecedentes epidemiológicos, las características clínicas y evolución de los pacientes. Resultados: Se confirmaron 14 pacientes. La relación masculino:femenino fue de 1.8:1, la edad promedio de los pacientes fue de 7.6 años (18 meses a 13 años). El 42.8% de los pacientes refirió el contacto con perros y el 57.1% afirmó tener contacto con garrapatas. En todos los casos hubo fiebre y exantema purpúrico; alrededor del 70% manifestaron mialgias y artralgias; el 28% tuvo sangrado del tubo digestivo, y el 11% alteraciones neurológicas graves. El 64.2% de los casos recibió tratamiento adecuado con doxiciclina. Fallecieron 8 pacientes, con una tasa de letalidad de 57.1%. Conclusiones: La zona sur de Coahuila debe considerarse una zona endémica para FMMR. El retraso en el diagnóstico y tratamiento favorecen una mayor letalidad.

Abstract: Background: Rocky Mountain Spotted Fever (RMSF) is caused by Rickettsia rickettsii. In Coahuila, Comarca Lagunera is considered an endemic zone; no cases have been reported in the southern zone of the state, specifically in the city of Saltillo­. Methods: Prospective study, cases report. Children evaluated in the Hospital del Niño Dr. Federico Gómez Santos from September 2012 to September 2017, with clinically and laboratory (by polymerase chain reaction, PCR) confirmed diagnosis of FMMR were included. The epidemiological antecedents, clinical characteristics and patient's evolution are presented. Results: 14 patients were confirmed. The male: female ratio was 1.8: 1, the average age of the patients was 7.6 years (18 months to 13 years). 42.8% reported contact with dogs and 57.1% confirmed contact with ticks. In all cases, there was fever and purpuric rash; around 70% manifested myalgias and arthralgias; 28% presented digestive tract bleeding and 11% had severe neurological alterations. 64.2% of the cases received adequate treatment with doxycycline. Eight patients died with a case fatality rate of 57.1%. Conclusions: The southern zone of Coahuila should be considered an endemic area for FMMR. The delay in diagnosis and treatment favor a greater lethality.

[Fiebre manchada de montañas rocosas: experiencia en 5 años de vigilancia activa en un hospital pediátrico de segundo nivel en el noreste de México]. / Rocky mountain spotted fever: five years of active surveillance experience in a second level pediatric hospital in northeastern Mexico.

Introducción: La fiebre manchada de las montañas rocosas (FMMR) es causada por Rickettsia rickettsii. En Coahuila, la región de la Comarca Lagunera se considera una zona endémica. No se han reportado casos en la zona sur del estado, específicamente en la ciudad de Saltillo. Métodos: Estudio prospectivo, reporte de casos. Se incluyeron los casos de niños atendidos en el Hospital del Niño Dr. Federico Gómez Santos en la ciudad de Saltillo, Coah., del mes de septiembre de 2012 a septiembre 2017 con diagnóstico confirmado clínicamente y por reacción en cadena de la polimerasa (PCR) de FMMR. Se presentan los antecedentes epidemiológicos, las características clínicas y evolución de los pacientes. Resultados: Se confirmaron 14 pacientes. La relación masculino:femenino fue de 1.8:1, la edad promedio de los pacientes fue de 7.6 años (18 meses a 13 años). El 42.8% de los pacientes refirió el contacto con perros y el 57.1% afirmó tener contacto con garrapatas. En todos los casos hubo fiebre y exantema purpúrico; alrededor del 70% manifestaron mialgias y artralgias; el 28% tuvo sangrado del tubo digestivo, y el 11% alteraciones neurológicas graves. El 64.2% de los casos recibió tratamiento adecuado con doxiciclina. Fallecieron 8 pacientes, con una tasa de letalidad de 57.1%. Conclusiones: La zona sur de Coahuila debe considerarse una zona endémica para FMMR. El retraso en el diagnóstico y tratamiento favorecen una mayor letalidad. Background: Rocky Mountain Spotted Fever (RMSF) is caused by Rickettsia rickettsii. In Coahuila, Comarca Lagunera is considered an endemic zone; no cases have been reported in the southern zone of the state, specifically in the city of ­Saltillo. Methods: Prospective study, cases report. Children evaluated in the Hospital del Niño Dr. Federico Gómez Santos from September 2012 to September 2017, with clinically and laboratory (by polymerase chain reaction, PCR) confirmed diagnosis of FMMR were included. The epidemiological antecedents, clinical characteristics and patient's evolution are presented. Results: 14 patients were confirmed. The male: female ratio was 1.8: 1, the average age of the patients was 7.6 years (18 months to 13 years). 42.8% reported contact with dogs and 57.1% confirmed contact with ticks. In all cases, there was fever and purpuric rash; around 70% manifested myalgias and arthralgias; 28% presented digestive tract bleeding and 11% had severe neurological alterations. 64.2% of the cases received adequate treatment with doxycycline. Eight patients died with a case fatality rate of 57.1%. Conclusions: The southern zone of Coahuila should be considered an endemic area for FMMR. The delay in diagnosis and treatment favor a greater lethality.

Dermatomiositis juvenil y calcinosis extensa. Tratamiento con metilprednisolona y metotrexato / Juvenile dermatomyositis and extensive calcinosis. Treatment with methylprednisolone and methotrexate

La dermatomiositis juvenil (DMJ) es una enfermedad multisistémica de etiología incierta, que resulta en una inflamación crónica no supurativa del músculo estriado, la piel y el tracto gastrointestinal. Las calcificaciones distróficas ocurren en un 30-70% de los niños con DMJ. Presentamos el caso de una paciente de 4 años de edad, con diagnóstico de DMJ según criterios de Bohan y Peter, en una edad muy temprana de presentación, con calcinosis extensas que le impedían sentarse, sin flexión de articulación de rodillas, con clase funcional 3. Recibió tratamiento con pulsos intravenosos de metilprednisolona cada 14 días, además de metotrexato vía oral, con mejoría clínica. A pesar de que la calcinosis es frecuente en enfermedades del tejido conectivo y puede llevar a discapacidad severa, no se han desarrollado protocolos terapéuticos para su manejo. El uso simultáneo de metilprednisolona y metotrexato permite un control más rápido de la enfermedad, con mejoría en la fuerza muscular y el eritema y regresión de las calcinosis, sin efectos colaterales importantes (AU)

Juvenile dermatomyositis (JDM) is considered a multisystemic disease of uncertain etiology. The clinical manifestation is a non-suppurative inflammation of the striated muscle, gastrointestinal tract and skin. Dystrophic calcifications are present in 30%-70% of children with JDM. The clinical case we are presenting is a 4 years old female with diagnosis of JDM in accordance to the Bohan and Peters criteria (very early presentation age) with extensive calcinosis, classified as functional class III, without being able to sit down or flex her knees. She was treated with IV methylprednisolone (MPS) bolus every 14 days and oral methotrexate, with improvement of her clinical condition. Even though calcinosis is a frequent finding in connective tissue disease and can cause severe disability, there are no treatment protocols at this time. The simultaneous use of IV MPS and oral methotrexate allows for a faster control of the disease, improvement in muscular force, reduction of erythema and regression of the calcinosis without important collateral effects (AU)

Lupus eritematoso sistémico en una lactante de 6 meses / Systemic lupus erythematosus in a 6 year old female child

El lupus eritematoso sistémico (LES) es poco frecuente, particularmente en la primera década de la vida; su inicio antes de 1 año de edad es muy raro y conlleva un diagnóstico difícil de documentar, ya que las manifestaciones pueden ser muy variables y parecerse a las de una gran variedad de enfermedades. En la edad pediátrica la incidencia de LES se estima en 0,36-0,9/100.000 niños, con prevalencia mayor en mujeres que en varones (aproximadamente 3:1 en menores de 12 años y 10:1 en niños mayores). Sin embargo, el inicio de LES antes de los 4 años ocurre con muy baja incidencia. Presentamos el caso de una lactante de la que se documentaron claramente más de cuatro criterios diagnósticos de LES desde los 6 meses de edad. Por lo revisado en la literatura, podría ser el caso de la paciente más pequeña que se haya publicado. Los médicos deben estar atentos al mayor riesgo de complicaciones sistémicas en niños con LES que se inicia a una edad temprana y realizar un diagnóstico oportuno de esta enfermedad (AU)

Systemic lupus erythematosus (SLE) is an uncommon disease, particularly in the first decade of life. Finding it before the first year of life is very rare and it entails a difficult diagnosis to document because the clinical manifestations can be very variable and can simulate a great variety of diseases In the pediatric age, incidence of SLE is 0.36-0.9/100 000 children, with greater prevalence in females 3:1 under 12 years of age and 10:1 in patients over 12 years. There is a very low incidence of SLE before 4 years of age. We present the clinical case of a 9 month-old female in whom four criteria of SLE were clearly documented at 6 months of age. In a review of literature, this is the youngest patient reported with SLE. Physicians must be very alert of the higher risk of systemic complications in children that are diagnosed with SLE at an early age (AU)

[Juvenile dermatomyositis and extensive calcinosis. Treatment with methylprednisolone and methotrexate]. / Dermatomiositis juvenil y calcinosis extensa. Tratamiento con metilprednisolona y metotrexato.

Juvenile dermatomyositis (JDM) is considered a multisystemic disease of uncertain etiology. The clinical manifestation is a non-suppurative inflammation of the striated muscle, gastrointestinal tract and skin. Dystrophic calcifications are present in 30%-70% of children with JDM. The clinical case we are presenting is a 4 years old female with diagnosis of JDM in accordance to the Bohan and Peters criteria (very early presentation age) with extensive calcinosis, classified as functional class III, without being able to sit down or flex her knees. She was treated with IV methylprednisolone (MPS) bolus every 14 days and oral methotrexate, with improvement of her clinical condition. Even though calcinosis is a frequent finding in connective tissue disease and can cause severe disability, there are no treatment protocols at this time. The simultaneous use of IV MPS and oral methotrexate allows for a faster control of the disease, improvement in muscular force, reduction of erythema and regression of the calcinosis without important collateral effects.

[Systemic lupus erythematosus in a 6 year old female child]. / Lupus eritematoso sistémico en una lactante de 6 meses.

Systemic lupus erythematosus (SLE) is an uncommon disease, particularly in the first decade of life. Finding it before the first year of life is very rare and it entails a difficult diagnosis to document because the clinical manifestations can be very variable and can simulate a great variety of diseases. In the pediatric age, incidence of SLE is 0.36-0.9/100 000 children, with greater prevalence in females 3:1 under 12 years of age and 10:1 in patients over 12 years. There is a very low incidence of SLE before 4 years of age. We present the clinical case of a 9 month-old female in whom four criteria of SLE were clearly documented at 6 months of age. In a review of literature, this is the youngest patient reported with SLE. Physicians must be very alert of the higher risk of systemic complications in children that are diagnosed with SLE at an early age.