Cytokine storm in Chikungunya: Can we call it multisystem inflammatory syndrome associated with Chikungunya?

Paraguay is currently facing a new outbreak of Chikungunya virus. This report summarizes two severe cases of Chikungunya (CHIKV) infection, confirmed by real-time reverse transcription polymerase chain reaction. We present the cases of patients with acute CHIKV infection and multisystem involvement, with fever, rash, abdominal pain, vomiting, myocarditis, and coronary artery anomalies, very similar to the cases described in MIS-C related to SARS-CoV-2 during the COVID-19 Pandemic. Both patients received IVIG and methylprednisolone, with good clinical response. In this setting of cytokine storm in Chikungunya, can we call it "Multisystem inflammatory syndrome associated with Chikungunya"?.

Cytokine storm in Chikungunya: Can we call it multisystem inflammatory syndrome associated with Chikungunya? / Tormenta de citoquinas en Chikungunya. ¿Podemos llamarla Síndrome inflamatorio multisistémico asociado a Chikungunya?

Paraguay is currently facing a new outbreak of Chikungunya virus. This report summarizes two severe cases of Chikungunya (CHIKV) infection, confirmed by real-time reverse transcription polymerase chain reaction. We present the cases of patients with acute CHIKV infection and multisystem involvement, with fever, rash, abdominal pain, vomiting, myocarditis, and coronary artery anomalies, very similar to the cases described in MIS-C related to SARS-CoV-2 during the COVID-19 Pandemic. Both patients received IVIG and methylprednisolone, with good clinical response. In this setting of cytokine storm in Chikungunya, can we call it “Multisystem inflammatory syndrome associated with Chikungunya”?.(AU)

Paraguay se enfrenta actualmente a un nuevo brote del virus Chikungunya. Este informe resume dos casos graves de infección por Chikungunya (CHIKV), confirmados mediante reacción en cadena de la polimerasa con transcripción inversa en tiempo real. Presentamos los casos de pacientes con infección aguda por CHIKV y afectación multisistémica, con fiebre, erupción cutánea, dolor abdominal, vómitos, miocarditis y anomalías de las arterias coronarias, muy similares a los casos descritos en síndrome inflamatorio multisistémico relacionado con el SARS-CoV-2 durante la pandemia de COVID-19. Ambos pacientes recibieron IGIV y metilprednisolona, con buena respuesta clínica. En este escenario de tormenta de citoquinas en Chikungunya, ¿podemos llamarla «síndrome inflamatorio multisistémico asociado a Chikungunya»?.(AU)

Mixoma cardiaco como causa de isquemia en niños. Reporte de casos

El mixoma cardiaco constituye uno de los tumores benignos más frecuentes entre los tumores cardiacos. El mismo se caracteriza por la obstrucción del flujo sanguíneo por trombosis o émbolo, resultando en causa de insuficiencia cardiaca con disfunción ventricular, síncope ortostático, isquemia de diferentes órganos de acuerdo a la arteria afectada, e incluso muerte súbita. Presentamos 2 casos en pacientes pediátricos: el primer caso, una adolescente con ACV isquémico y el segundo caso se trata de un adolescente con isquemia a nivel de miembros inferiores por una tromboembolia en la Aorta distal. En ambos casos se realizó la exéresis del tumor, con éxito.

Cardiac myxoma is one of the most frequent benign tumors among cardiac tumors. It is characterized by the obstruction of blood flow due to thrombosis or embolus, resulting in heart failure with ventricular dysfunction, orthostatic syncope, ischemia of different organs depending on the affected artery, and even sudden death. We present 2 cases in pediatric patients: the first case, an adolescent with ischemic stroke and the second case is an adolescent with ischemia in the lower limbs due to a thromboembolism in the distal aorta. In both cases, the exeresis of the tumor was performed successfully.

Perfil de transmisión del SARS-COV-2 en una región sanitaria del Paraguay

Introducción: La transmisión viral a menudo se propaga en grupos, donde las infecciones se pueden rastrear hasta un caso índice o una ubicación geográfica, para así poder tomar medidas de prevención al respecto. Entender el perfil de transmisión del SARS-CoV-2 es esencial para desarrollar estrategias efectivas de prevención y control de la enfermedad. Objetivo: Determinar el perfil de transmisión del Sars-Cov-2, a partir de clúster con casos índices identificados, en la V región sanitaria (Caaguazu) del Paraguay, entre julio y octubre del 2020. Metodología: Se realizó un estudio observacional, descriptivo, de corte transversal, con fichas de notificación de casos de COVID-19 y resultados laboratoriales de la RT_PCR en pacientes diagnosticados con COVID-19 en la V Región Sanitaria entrejulio y octubre del 2020. Resultados: Fueron identificadas 703 personas con Sars-Cov-2 positivo. El 55,49 % de las personas no reconoció algún nexo de contagio. Con respecto al tipo de evento donde ocurrían con mayor frecuencia los contagios, ocurrieron en Eventos Sociales en 58,14 % de los casos y en el Ambiente Familiar en el 33,89 %. La transmisión secundaria se observó en el 17,40 % de los casos. Fueron identificados 58 Clústers, con una mediana de tamaño de los mismos de 3 (RIQ 2-4), y 267 personas (37,98 %) asociadas a los mismos. Discusión: La mayoría de las personas no reconocieron su nexo de contagio, sin embargo, en los que, si conocieron, se puede observar que la participación en eventos sociales fue el principal nexo, por lo que es fundamental realizar este tipo de ejercicios para hacer el seguimiento oportuno de los casos.

Introduction: Viral transmission often spreads in clusters, where infections can be traced to an index case or a geographic location, in order to take preventive measures in this regard, understanding the transmission profile of SARS-CoV-2 is essential. to develop effective disease prevention and control strategies. Objective: to determine the transmission profile of Sars-Cov-2, from clusters with identified index cases, in the V health region (Caaguazú) of Paraguay, between July and October 2020. Methodology: We carried out an observational, descriptive, cross-sectional study, with notification sheets of COVID-19 cases and laboratory results of the RT_PCR in patients diagnosed with COVID-19 in the V Sanitary Region between July and October 2020. Results: We identified 703 people with positive Sars-Cov-2. 55.49% of people did not recognize any contagion link. Regarding the type of event where infections occurred most frequently, was in Social Events in 58.14% of the cases and the Family Environment in 33.89%, secondary transmission was observed in 17.40% of the cases. 58 Clusters were identified, with a median size of 3 (IQR 2-4), and 267 people (37.98%) associated with them. Discussion: Most of the people did not recognize their contagion link, however, in those who did know, the main link was because they participated in social events, so it is essential to carry out this type of exercise to do timely follow-up. of the cases.

Caracterización clínica y epidemiológica de la epidemia de Chikungunya en el Paraguay / Clinical and epidemiological characterization of the Chikungunya epidemic in Paraguay

Durante el verano del 2022 y 2023 ocurrió la mayor epidemia de Chikungunya en Paraguay, y una de las más grandes reportadas en la región. Estuvo centralizada en el área metropolitana de Asunción en una primera etapa, pero se expandió al resto del país durante los primeros meses del 2023. Este trabajo tiene el objetivo de describir epidemiológica y clínicamente la epidemia desde su inicio en la semana epidemiológica 40 del 2022 hasta la semana 20 del 2023. Metodología: es un estudio descriptivo que utiliza los datos públicos disponibles en la página de la Dirección General de Vigilancia de la Salud. Fueron confirmados 86.761 casos, 58% femenino, 8227 ingresos hospitalarios y 248 fallecidos. La letalidad global es de 2.8 por mil casos confirmados. Los grupos etarios más afectados corresponden a la franja de 0 a 4 años (9%), sin embargo, la mayor incidencia de casos se da en mayores de 80 años. Los principales desafíos de esta enfermedad son el abordaje multidisciplinario en la gestión del manejo del vector, la evaluación de las causas de esta alta letalidad y la necesidad de una vacuna de uso poblacional.

During the summer of 2022 and 2023, the largest Chikungunya epidemic occurred in Paraguay, and one of the largest reported in the region. It was centralized in the metropolitan area of Asunción in a first stage, but it expanded to the rest of the country during the first months of 2023. This work has the objective of describing the epidemic epidemiologically and clinically from its beginning in epidemiological week 40 of 2022 to week 20 of 2023. Methodology: it is a descriptive study that uses the public data available on the page of the General Directorate of Health Surveillance. 86,761 cases were confirmed, 58% female, 8,227 hospital admissions, and 248 deaths. The global lethality is 2.8 per thousand confirmed cases. The most affected age groups correspond to the 0 to 4-year-old group (9%), however, the highest incidence of cases occurs in people over 80 years of age. The main challenges of this disease are the multidisciplinary approach in the management of the vector, the evaluation of the causes of this high lethality and the need for a vaccine for population use.

Caracterización clínica y epidemiológica de la epidemia de Chikungunya en el Paraguay

Durante el verano del 2022 y 2023 ocurrió la mayor epidemia de Chikungunya en Paraguay, y una de las más grandes reportadas en la región. Estuvo centralizada en el área metropolitana de Asunción en una primera etapa, pero se expandió al resto del país durante los primeros meses del 2023. Este trabajo tiene el objetivo de describir epidemiológica y clínicamente la epidemia desde su inicio en la semana epidemiológica 40 del 2022 hasta la semana 20 del 2023. Metodología: es un estudio descriptivo que utiliza los datos públicos disponibles en la página de la Dirección General de Vigilancia de la Salud. Fueron confirmados 86.761 casos, 58% femenino, 8227 ingresos hospitalarios y 248 fallecidos. La letalidad global es de 2.8 por mil casos confirmados. Los grupos etarios más afectados corresponden a la franja de 0 a 4 años (9%), sin embargo, la mayor incidencia de casos se da en mayores de 80 años. Los principales desafíos de esta enfermedad son el abordaje multidisciplinario en la gestión del manejo del vector, la evaluación de las causas de esta alta letalidad y la necesidad de una vacuna de uso poblacional.

During the summer of 2022 and 2023, the largest Chikungunya epidemic occurred in Paraguay, and one of the largest reported in the region. It was centralized in the metropolitan area of Asunción in a first stage, but it expanded to the rest of the country during the first months of 2023. This work has the objective of describing the epidemic epidemiologically and clinically from its beginning in epidemiological week 40 of 2022 to week 20 of 2023. Methodology: it is a descriptive study that uses the public data available on the page of the General Directorate of Health Surveillance. 86,761 cases were confirmed, 58% female, 8,227 hospital admissions, and 248 deaths. The global lethality is 2.8 per thousand confirmed cases. The most affected age groups correspond to the 0 to 4-year-old group (9%), however, the highest incidence of cases occurs in people over 80 years of age. The main challenges of this disease are the multidisciplinary approach in the management of the vector, the evaluation of the causes of this high lethality and the need for a vaccine for population use.

Intergenerational sex and early sexual debut are associated with HIV infection among transgender women in Paraguay.

INTRODUCTION: HIV prevalence among transgender women is high worldwide. The objectives of the present study were to estimate the current prevalence of HIV and identify factors associated with high HIV burden among transgender women in Paraguay. METHODS: Transgender women aged ≥15 years in four regions of Paraguay were recruited by Starfish sampling between February and March 2021. RESULTS: In total, 322 transgender women were included. Mean age was 31 years (range 15-67), and 102 had positive HIV test results (31.7%, 95% confidence interval [CI] 26.6-37.1). In multivariable analysis, factors associated with HIV infection were age at first intercourse ≤17 years (adjusted odds ratio [aOR] 5.47; 95% CI 1.05-28.42), >10 years difference in age with the last sexual partner (aOR 1.60; 95% CI 1.04-2.46), substance use (mostly cocaine) (aOR 3.00; 95% CI 1.47-6.12), higher risk perception (aOR 3.08; 95% CI 1.53-6.17), not testing for HIV (aOR 1.23; 95% CI 1.09-1.39), and accessed by a peer educator (aOR 3.86; 95% CI 1.77-8.38). CONCLUSIONS: Sexual debut as a minor and a large age difference with sexual partners are associated with high burden of HIV among transgender women in Paraguay. Our study corroborates the finding of cocaine use during sex as a risk factor for HIV. Prevention programmes must address structural and social vulnerabilities to stem the tragically high burden of HIV among transgender women.

Nefropatía por IgA en una adolescente con lupus eritematoso sistémico juvenil. Reporte de caso

Introducción: El lupus eritematoso sistémico juvenil (LESJ) es una enfermedad autoinmunitaria, multisistémica, caracterizada por la producción de autoanticuerpos y el desarrollo frecuente de glomerulonefritis mediada por inmunocomplejos. La nefritis lúpica es una complicación frecuente y grave del LESJ, con alta morbilidad, siendo causa de insuficiencia renal terminal en muchos de estos pacientes. La nefropatía por IgA representa la etiología más común en la población general y raramente se asocia con LESJ. Caso clínico: adolescente femenino con LESJ en quien se diagnosticó nefritis no lúpica (nefropatía por IgA). Conclusiones: El diagnóstico anatomopatológico es clave para establecer el pronóstico y planificar el tratamiento.

Introduction: Juvenile systemic lupus erythematosus (JSLE) is a multisystem autoimmune disease characterized by the production of autoantibodies and the frequent development of immune complex-mediated glomerulonephritis. Lupus nephritis is a frequent and serious complication of JSLE, has a high associated morbidity rate, and is the cause of end-stage renal failure in many of these patients. IgA Nephropathy represents the most common etiology in the general population and is rarely associated with JSLE. Clinical case: a female adolescent with JSLE who was diagnosed with non-lupus nephritis (IgA nephropathy). Conclusions: The anatomopathological diagnosis is key to establish the prognosis and plan the treatment.

Association of class II HLA alleles with susceptibility to develop immune-mediated diseases in Paraguayan patients.

Genetic and nongenetic factors are involved in the pathogenesis of immune-mediated inflammatory diseases (IMIDs). The best-known genetic factor for susceptibility to IMIDs is the human leukocyte antigen (HLA). The aim of the present study was to evaluate the association of HLA class II genes with the risk of systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and systemic sclerosis (SSc) in the Paraguayan population. We included 254 patients with IMIDs (101 SLE, 103 RA, and 50 SSc) and 50 healthy controls. The haplotypes of five genes corresponding to HLA class II genes and their relationship to the IMIDs studied were determined. Note that 84.6% were women, with a mean age of 43.4 ± 14 years. Among the associated HLA alleles, we found the previously identified risk factors in other populations like HLA-DRB1*03:01 and HLA-DRB1*14:02 for RA, as well as new ones not previously identified, such as DPA1*02:01 for SLE and, DB1*02:01 for RA and SSc. In the genetic association analysis, already known associations have been replicated, and unpublished associations have been identified in Paraguayan patients with IMIDs. This is the first genetic association study in Paraguayan patients with IMIDs.

HIV prevalence and associated risk factors among men who have sex with men in three regions of Paraguay, 2020.

BACKGROUND: Our study aimed to measure HIV prevalence and associated risk factors among men who have sex with men (MSM) in three regions of Paraguay in 2020. METHODS: MSM were recruited for cross-sectional surveys in three regions of Paraguay using respondent-driven sampling. Interview were conducted face-to-face to collect demographic characteristics and risk and preventive behaviors. The analysis assessed HIV prevalence and associated risk factors in the three samples of MSM within each region. RESULTS: A total of 1,207 MSM were recruited, including 559 in Asunción-Central, 245 in Alto Paraná, and 403 in Caaguazú. HIV prevalence was 24.2% (95% CI 20.6-27.9) in Asunción-Central, 10.2% (95% CI 6.7-14.6) in Alto Paraná, and 3.2% (95% CI 1.7-5.4) in Caaguazú. In Asunción-Central, associations with HIV were age ≥25 years (1.86, 95% CI 1.15-3.00), being employed (1.82, 95% CI 1.07-3.11), self-reporting as homosexual (1.90, 95% CI 1.06-3.43), having sex with a known HIV-positive partner acquisition (4.19, 95% CI 2.37-7.43), self-perceived as being at higher risk for HIV acquisition (4.15, 95% CI 2.54-6.77), and able to access condoms and lubricants (1.82, 95% CI 1.08-3.05). In Alto Paraná, associations with HIV were self-reporting as homosexual (4.33, 95% CI 1.19-15.65) and having higher HIV knowledge (2.53, 95% CI 0.97-6.61). In Caaguazú, associations with HIV were self-reporting as homosexual (7.06, 95% CI 1.53-32.46) and being diagnosed with depression (4.68, 95% CI 0.89-24.43). CONCLUSIONS: HIV prevalence among MSM in Paraguay varied by region, being highest in the capital and major metropolitan area of Asunción-Central, followed by the border area of Alto Paraná. While being self-identified as homosexual was associated with HIV in all three regions, other associations differed, indicating prevention programs need to be tailored to the locale.

Association between vitamin D deficiency and disease activity in Paraguayan patients with systemic lupus erythematosus / Asociación entre la deficiencia de vitamina D y la actividad de la enfermedad en pacientes paraguayos con lupus eritematoso sistémico

ABSTRACT Objective: To identify the association between vitamin D (VD) concentrations and the activity of systemic lupus erythematosus (SLE) and determine a supplementation dose that allows patients to maintain adequate levels of VD. Methods: Longitudinal, observational study. Serum levels of 25-hydroxy-VD were measured in 100 Paraguayan SLE patients from the Hospital de Clínicas between 2016 and 2018. To analyze the response to different doses of VD supplementation, 50 patients received 1000IU/day and the other 50 patients received 2000IU. SLE disease activity measured by SELENA-SLEDAIwas scored before and after supplementation. Results: The mean age was 27.5 ±9.8 years, 88.9% of patients presented mild disease activ ity and 11.1% presented moderate to severe activity. The mean VD concentration was 30.8 ± 11.8 ng/mL. A total of 34% of patients presented VD insufficiency and 13% VD defi ciency. There was an inverse relationship between VD concentrations and SLE disease activity (p = 0.03). Increasing levels of serum VD are associated with supplementation of 2000 IU/day (p = 0.0224). Conclusion: SLE activity was associated with low levels of VD. In our cohort, SLE patients required a supplementation dose equal to or greater than 2000 IU/day to increase their serum VD.

R E S U M E N Objetivo: Identificar la asociación entre las concentraciones de vitamina D (VD) y la actividad del lupus eritematoso sistémico (LES), además de encontrar una dosis de suplementación que les permita a los pacientes mantener niveles adecuados de VD. Métodos: Estudio observacional longitudinal. Se midieron los niveles séricos de 25-hidroxi-VD en 100 pacientes paraguayos con LES, del Hospital de Clínicas, entre los años 2016 y 2018. Para analizar la respuesta a diferentes dosis de suplementación con VD, 50 pacientes recibieron 1.000 UI/día y los otros 50 pacientes recibieron 2.000 UI/día. La actividad de la enfermedad del LES medida por SELENA-SLEDAI se puntuó antes y después de la suplementación. Resultados: La media de edad fue de 27,5 ± 9,8 años, el 88,9% de los pacientes presentó actividad leve de la enfermedad y el 11,1% presentó actividad moderada a severa. La concentración media de VD fue de 30,8 ± 11,8 ng/ml. El 34% de los pacientes presentó insuficiencia de VD y el 13%, deficiencia de VD. Hubo una relación inversa entre las concentraciones de VD y la actividad de la enfermedad del LES (p = 0,03). Los niveles crecientes de VD en suero se asocian con una suplementación de 2.000 UI/día (p = 0,0224). Conclusión: La actividad del LES se asoció con niveles bajos de VD. En nuestra cohorte, los pacientes con LES requirieron una dosis de suplementación igual o superior a 2.000 UI/día para aumentar su VD sérica.

Seguridad y supervivencia de las terapias biológicas: primer informe del registro paraguayo-uruguayo de acontecimientos adversos con terapias biológicas Biobadaguay / Safety and survival associated with biologic therapies: first report of the biobadaguay on the Paraguayan-Uruguayan registry of adverse events with biologic therapies

OBJETIVOS: Analizar los acontecimientos adversos (AA) y la supervivencia de las terapias biológicas (TB) en el registro paraguayo-uruguayo de AA, Biobadaguay. MÉTODO: Estudio observacional, prospectivo de duración indeterminada. Se han incluido pacientes al inicio de la TB y controles. Se han registrado variables clínicas, biológicas y relacionadas con el tratamiento. RESULTADOS: Se realizaron 826 registros (650 TB y 176 controles). El 70,9% fueron mujeres y el diagnóstico más frecuente fue la artritis reumatoide (AR) (63,2%). La TB más utilizada fue el adalimumab (56,6%) y la causa más frecuente de interrupción, la ineficacia (42,1%). La incidencia de AA en pacientes con TB fue de 143,9 (128,8-160,8) por 1.000 pacientes/año. En el estudio comparativo de AA en función del diagnóstico, se observó que la artritis idiopática juvenil (AIJ) se asoció a más AA globales (RTI = 2,3; IC 95%: 1,6-3,4; p = 4,27×10−6), mientras que la AR se asoció a un mayor número de AA graves (RTI = 2,20; IC 95%: 1,2-4,1; p = 1,17×10−2). Por otro lado, el tratamiento con tocilizumab se asoció a una mayor tasa de AA (RTI = 2,69; IC 95%: 1,90-3,82; p = 3,13×10−8). El diagnóstico de AIJ, el tratamiento con corticoides y el número de TB previas se asociaron a la disminución de la supervivencia de las TB. CONCLUSIÓN: En este primer informe del registro Biobadaguay, la principal causa de interrupción de la TB fue la ineficacia. Con relación al diagnóstico, la AR y la AIJ se asociaron a un mayor riesgo de AA. En este registro, se identificaron variables relacionadas a una menor supervivencia de las TB

OBJECTIVE: Analyze adverse events (AE) and survival associated with biologic therapies (BT) in the Biobadaguay, the Paraguayan Uruguayan registry of adverse events. METHODS: Prospective, observational study of undetermined duration. Patients on BT at initiation and controls were included. Clinical, biological and treatment variables were registered. RESULTS: A total of 826 registers were entered (650 BT and 176 controls); 70.9% were women and rheumatoid arthritis (RA) was the most frequent diagnosis (63.2%). The BT most often used was adalimumab and the main cause of discontinuation was loss of efficacy (42.1%). The incidence of AE of patients on BT was 143.9 (128.8-160.8) per 1000 patients/year. In the comparative study of AE related to diagnosis, juvenile idiopathic arthrosis (JIA) was associated with a higher overall number of AE (RTI = 2.3; 95%CI: 1.6-3.4; P = 4.27 ×10−6), whereas RA was associated with a higher number of serious AE (RTI = 2.2; 95% CI: 1.2-4.1; P =1.17 ×10−2). On the other hand, treatment with tocilizumab was associated with a higher rate of AE (RTI = 2.69; 95% CI: 1.9-3.82; P = 3.13 ×10−8). In JIA, treatment with corticosteroids and number of previous BT was associated with a decrease in BT survival. CONCLUSION: In this first report of the Biobadaguay registry, the main cause of BT discontinuation was loss of efficacy. In terms of the diagnosis involved, RA and JIA were associated with a higher risk of AE. In this registry, variables related to a shorter survival of BT were identified

Safety and Survival Associated with Biologic Therapies: First Report of the Biobadaguay on the Paraguayan-Uruguayan Registry of Adverse Events with Biologic Therapies. / Seguridad y supervivencia de las terapias biológicas: primer informe del registro paraguayo-uruguayo de acontecimientos adversos con terapias biológicas Biobadaguay.

OBJECTIVE: Analyze adverse events (AE) and survival associated with biologic therapies (BT) in the Biobadaguay, the Paraguayan Uruguayan registry of adverse events. METHODS: Prospective, observational study of undetermined duration. Patients on BT at initiation and controls were included. Clinical, biological and treatment variables were registered. RESULTS: A total of 826 registers were entered (650 BT and 176 controls); 70.9% were women and rheumatoid arthritis (RA) was the most frequent diagnosis (63.2%). The BT most often used was adalimumab and the main cause of discontinuation was loss of efficacy (42.1%). The incidence of AE of patients on BT was 143.9 (128.8-160.8) per 1000 patients/year. In the comparative study of AE related to diagnosis, juvenile idiopathic arthrosis (JIA) was associated with a higher overall number of AE (RTI = 2.3; 95%CI: 1.6-3.4; P = 4.27 ×10-6), whereas RA was associated with a higher number of serious AE (RTI = 2.2; 95% CI: 1.2-4.1; P =1.17 ×10-2). On the other hand, treatment with tocilizumab was associated with a higher rate of AE (RTI = 2.69; 95% CI: 1.9-3.82; P = 3.13 ×10-8). In JIA, treatment with corticosteroids and number of previous BT was associated with a decrease in BT survival. CONCLUSION: In this first report of the Biobadaguay registry, the main cause of BT discontinuation was loss of efficacy. In terms of the diagnosis involved, RA and JIA were associated with a higher risk of AE. In this registry, variables related to a shorter survival of BT were identified.

Histoplasmosis diseminada con lesiones cutáneas y osteomielitis en un niño con Leucemia Linfoblastica Aguda en mantenimiento / Disseminated histoplasmosis with skin lesions and osteomyelitis in a child with acute Lymphoblastic Leukemia Undergoing maintenance treatment

La histoplasmosis es una micosis causada por el hongo Histoplasma capsulatum y generalmente se manifiesta como una infección diseminada en pacientes inmunocomprometidos. La osteomielitis por H. capsulatum es rara y difícil de diagnosticar. Presentamos un caso inusual de histoplasmosis diseminada, causada por H. capsulatum, con lesiones en piel, mucosas y osteomielitis en un niño de 10 años de edad con Leucemia Linfoblastica Aguda en tratamiento con metotrexato vía oral a dosis baja, con adecuada respuesta a Anfotericina b y mantenimiento con Itraconazol.

Histoplasmosis is a mycosis caused by the fungus Histoplasma capsulatum and usually manifests as a disseminated infection in immunocompromised patients. Osteomyelitis due to H. capsulatum is rare and difficult to diagnose. We present an unusual case of disseminated histoplasmosis, caused by H. capsulatum, with lesions in skin, mucous membranes and osteomyelitis in a 10-yearold boy with acute Lymphoblastic Leukemia Undergoing low-dose oral methotrexate treatment, who had an adequate response to Amphotericin B and maintenance treatment with Itraconazole.

Lupus neonatal. Reporte de un caso

El lupus eritematoso neonatal (LEN) es una enfermedad rara, con incidencia de aproximadamente 2% en recién nacidos y menos del 5% de estos bebés desarrollarán lupus eritematoso sistémico en la adolescencia tardía o en la adultez temprana. Se presenta un caso de un recién nacido de término, hijo de madre sin antecedentes previos, sexo femenino, con marcadores inmunológicos positivos; nace con 3.280 gramos de peso, 40 semanas de edad gestacional, con lesiones en piel en el que el diagnóstico diferencial fue infección herpética, llegando al diagnóstico y logrando descartar de forma oportuna afectación sistémica, la que podría llegar a poner en riesgo la vida del neonato, con esta presentación de caso se concluye la importancia de agregar entre los diagnósticos diferenciales, el lupus eritematoso neonatal, entre los pacientes con lesión en piel incluso desde su nacimiento.

Neonatal lupus erythematosus (NLE) is a rare disease, with an incidence of approximately 2% in newborns. Less than 5% of these babies will develop systemic lupus erythematosus in late adolescence or early adulthood. A case of a female term newborn is presented, born to a mother with an unremarkable previous history, with positive immunological markers; birth weight of 3,280 grams, 40 weeks gestational age, with skin lesions in which the differential diagnosis was herpetic infection. The diagnosis was made and systemic involvement was ruled out in a timely manner, which could have potentially endangered the life of the newborn. This case demonstrates the importance of adding neonatal lupus erythematosus to the differential diagnosis in patients with skin lesions at birth.

Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America.

To assess epidemiology and management practices of Latin America Pediatric Rheumatologists (LAPR) about childhood-onset systemic lupus erythematosus (cSLE). A cross-sectional study was performed in 288 LAPR PANLAR members based on online survey about cSLE practices. The response rate of web-based survey by LAPR was 170/288(59%) and the majority worked in university hospitals (63%). The ACR and/or SLICC classification criteria (99%) and disease activity tools (97%) were almost universally used by LAPR, whereas damage index (70%) and CHAQ (58%) instruments were less frequently used. Laboratory exams, diagnostic imaging, and biopsies were generally available (> 75%), however low availability for densitometry (66%). Drug access was excellent for the most common prescribed medications (> 75%), except for belimumab (11%). Emerging mosquito-borne diseases were also reported: dengue (20%), chikungunya (11%), and Zika (8%). Groups were further divided in two, according to the median number of cSLE patients followed by LAPR in the last year: groups A and B (≥ 25 and < 25, respectively). Frequencies of condom in combination with other contraceptive methods were significantly higher in group A than B (p = 0.01). The frequencies of reported pregnancy (p < 0.001) and non-adherence to therapy were significantly higher in group A (p = 0.023). Alcohol intake (p = 0.004) and illicit drug use (p = 0.007) were also reported more frequently by LAPR of group A in at least one cSLE patient. This first large web-based survey demonstrated an overall excellent access for diagnosis and therapy by LAPR, probably related to their high rate of practices in tertiary care of university hospitals. Adherence to therapy, pregnancy, and substance abuse was identified as major challenges in this population, particularly in larger centers.

Frecuencia de colonización y sensibilidad de Staphylococcus aureus en un grupo de niños sanos en Asunción

Introducción: La portación nasal de Staphylococcus aureus (SA) en individuos sanos constituye una fuente potencial de infección. La prevalencia de infecciones por SA adquiridas en la comunidad ha aumentado entre los niños sanos. Aunque la epidemiología de la colonización e infección por SA en otros países se ha estudiado ampliamente, los datos son limitados en el Paraguay. Objetivos: Determinar la prevalencia de la portación nasal por SA y su susceptibilidad antimicrobiana en una población de escolares sanos en Asunción, y evaluar factores de riesgo asociados a la colonización. Material y métodos: Estudio multicéntrico, prospectivo, descriptivo y analítico, sobre la colonización nasal por SA en niños de 5 a 16 años, durante el periodo comprendido entre junio y julio del 2016. Previa autorización de los padres y la escuela, se recogieron datos demográficos y factores de riesgo para la colonización. La determinación de SA se realizó mediante muestras de hisopado de las narinas de los niños. Se realizaron pruebas de susceptibilidad antimicrobiana en Agar Mueller y la sensibilidad a la vancomicina se realizó mediante tiras de E-Test®. Los datos fueron registrados en planillas Excel y analizados con el software R v.3.4.2 Resultados: 299 niños fueron incluidos en el estudio. De estos, 58%(173) eran mujeres con una media de edad de 10,6 (±2,5) años. El 79,9% procedía de Asunción y vivía con una media de 5,1 (±1,8) habitantes en el domicilio. Se determinó la presencia de SA en el 30,8% (92/299) de los niños. El 63% de los niños refirió algún factor de riesgo para la colonización por SA. Se objetivo que el antecedente de una enfermedad previa OR=1,92 (95%IC 0,88-4,28), el uso de antibióticos previos OR=1,51 (95%IC 0,89-2,55), la convivencia con mascotas OR=1,42(95%IC 0,81-2,50) y los tratamientos crónicos OR=1,20 (95%IC 0,55-2,61) fueron las variables asociadas a un mayor riesgo de presentar colonización por SA. Conclusión: En el presente estudio se objetivó que el 30,8% de los niños incluidos en el estudio presentaba colonización por SA y que los factores asociados a un mayor riesgo de colonización han sido el antecedente de una enfermedad previa, el uso de antibióticos, la convivencia con mascotas y los tratamientos crónicos.

Introduction: Staphylococcus aureus (SA) nasal carriage in healthy individuals is a potential source of infection. The prevalence of SA infections acquired in the community has increased among healthy children. Although the epidemiology of colonization and infection by SA in other countries has been studied extensively, data are limited in Paraguay. Objectives: To determine the prevalence of nasal carriage by SA and its antimicrobial susceptibility in a population of healthy schoolchildren in Asunción, and to evaluate risk factors associated with colonization. Material and methods: This was a multicenter, prospective, descriptive and analytical study on nasal colonization by SA in children aged 5 to 16 years between June and July 2016. After obtaining authorization from parents and the school, we collected data on demographics and on risk factors for colonization. The SA colonization determination was made by swabbing samples from the children's nostrils. Antimicrobial susceptibility tests were performed on Mueller Agar and the sensitivity to vancomycin was made using E-Test® strips. The data were recorded in Excel spreadsheets and analyzed with the R v.3.4.2 software. Results: 299 children were enrolled in the study. Of these, 58% (173) were female with an average age of 10.6 (± 2.5) years. 79.9% came from Asunción and lived with an average of 5.1 (± 1.8) inhabitants at home. The presence of SA was detected in 30.8% (92/299) of the children. 63% of children reported some risk factor for colonization by SA. We found that a history of a previous disease OR = 1.92 (95% CI 0.88-4.28), the use of previous antibiotics OR = 1.51 (95% CI 0.89-2.55), cohabitation with pets OR = 1.42 (95% CI 0.81-2.50) and chronic treatments OR = 1.20 (95% CI 0.55-2.61) were the variables associated with an increased risk for colonization by SA. Conclusion: In the present study it was found that 30.8% of the children enrolled in the study were colonized by SA and that factors associated with an increased risk of colonization were a history of a previous disease, the use of antibiotics, coexistence with pets and chronic treatments.

Arteritis de Takayasu en un niño. Reporte de caso

Introducción: La arteritis de Takayasu es una vasculitis sistémica, de etiología autoinmune, que afecta principalmente a grandes vasos como la Aorta y sus ramas principales. Es poco frecuente en edad pediátrica, siendo la franja etaria más afectada la comprendida entre 10 y 18 años, con predominio del sexo femenino. Caso Clínico: Se presenta el caso de un varón de 13 años de edad, con astenia, síncopes en varias ocasiones, pérdida de peso, claudicación intermitente y dificultad respiratoria progresiva de 4 meses de evolución, hipotrófico, con palidez marcada, sudoración profusa, extremidades superiores frías y sin pulsos palpables; miembros inferiores tibios con pulsos palpables, en donde se constata cifras tensionales elevadas (240/70mmHg). A través de los análisis de laboratorio se constata anemia, con perfil inmunológico normal. Se confirma afectación de la aorta, carótidas y vasos renales por imágenes. Evolución satisfactoria con el tratamiento instaurado.

Introduction: Takayasu arteritis is a systemic vasculitis of autoimmune etiology, which mainly affects large vessels such as the aorta and its main branches. It is uncommon in pediatric age, with range between 10 and 18 years, with predominance of the female sex. Clinical Case: A 13-year-old boy with asthenia, multiple syncope, weight loss, intermittent claudication and progressive respiratory difficulty of 4 months of evolution. Hypotrophic, marked pallor, profuse sweating, superior limbs cold and without palpable pulses; Lower limbs with palpable pulses, where high blood pressure values are found (240/70 mmHg). Laboratory analysis reveals anemia, with a normal immunological profile. The involvement of the aorta, carotid and renal vessels by images is confirmed. Satisfactory evolution with the treatment established.

Síndrome de McCune-Albright. Reporte de un caso / McCune-Albright syndrome: a Case Report

El Síndrome de McCune-Albright (SMA) es una rara entidad que se caracteriza por displasia fibrosa ósea poliostótica, lesiones cutáneas hiperpigmentadas y endocrinopatías, la más frecuente es la pubertad precoz y sobre todo en niñas. Presentamos el caso de una paciente de sexo femenino de 5 años de edad, que se interna por fractura patológica del fémur derecho, constatándose lesiones líticas en fémur contralateral, pelvis, tórax y calota; manchas café con leche en regiones del tórax anterior, perineal y dorsolumbar; Tanner 2 mamario y púbico, con antecedente de sangrado vaginal en 2 oportunidades 1 mes antes; y con Rx de muñeca izquierda compatible con edad ósea de 9 años; además de microadenoma hipofisiario. El SMA resulta de mutaciones esporádicas somáticas postcigóticas en el gen que codifica la subunidad α de la proteína Gs (GNAS1). Esta proteína actúa en la transducción de señales mediante la unión a la adenil-ciclasa productora de adenosín monofosfato cíclico (AMPc). Es importante conocer esta asociación de signos a fin de obtener un diagnóstico precoz y manejo adecuado .

McCune-Albright syndrome (MAS)isa rare disease characterized by poly ostotic fibrous dysplasia of bone, hyperpigmented skin lesions, and endocrinopathies, most commonly precocious puberty, and especially in girls. We presented the case of a female patient aged 5years hospitalized for pathological fracture of the right femur with findings of lytic lesions of the contralateral femur pelvis, thorax, and calvarium, and café-au-lait spots of the anterior, perineal, and dorsolumbar thorax; Tanner stage 2 breasts and pubes, a history of vaginal bleeding on two occasionsone monthearlier, left-wrist X-ray compatible with abone age of 9years and pituitary microadenoma. MAS is caused by sporadic postzygotic somatic mutations of the gene that codifies the alpha subunit of the G(s) protein (GNAS1). This protein acts in the transduction of signals by binding with cyclic-adenosine-monophosphate (cAMP) producing adenylate cyclase. It is important to beaware of this group of associated signs in order to achiev e early diagnosis and appropriate treatment.

Síndrome DRESS y necrólisis epidérmica tóxica por el uso de lamotrigina: a propósito de dos casos / DRESS syndrome and Toxic Epidermal Necrolysis Due to Lamotrigine: a Report of Two Cases

Las reacciones adversas cutáneas a medicamentos (RACM) son una variedad de las múltiples reacciones adversas a los medicamentos (RAM). El Síndrome DRESS es una de ellas y está caracterizada por rash cutáneo, afectación de vísceras y eosinofilia. La Necrólisis Epidérmica Tóxica (NET) o Síndrome de Lyell es otra afección dentro del grupo de las RACM, parecida al Síndrome de Stevens Johnson (SSJ), del cual se diferencia por la extensión de las lesiones cutáneas en más de 30% de la piel. Estas reacciones adversas pueden ser causadas por una variedad de medicamentos entre ellos los antiepilépticos y la asociación de la Lamotrigina con el Ácido Valproico, por acumulación y alargamiento de la vida media del primero. Se presenta el caso de una niña con el antecedente de haber sido tratada con antiepilépticos, entre ellos el Ácido Valproico y que luego de 15 días de ser introducida la Lamotrigina, presentó rash cutáneo, fiebre, alteración de las enzimas hepáticas y eosinofilia importante, cumpliendo los criterios diagnósticos de Síndrome DRESS. El segundo caso corresponde a un niño que estando en tratamiento con antiepilépticos, entre ellos Ácido Valproico se le agregó Lamotrigina por una Epilepsia de difícil control y 20 días después presentó lesiones de piel con afectación de mucosas que se fueron agravando, requiriendo internación en UTI, el cuadro clínico fue compatible con NET. Ambos pacientes tuvieron una evolución favorable .

Adverse cutaneous drug reactions (ACDR) are one of many types of adverse drug reactions (ADR). DRESS syndrome is one of these, and is characterized by askin rash organ involvement, andeo sinophilia. Toxic epidermal necrolysis (TEN), or Lyell's syndrome, is another disease of the ACDR group, andis similar to Stevens-Johnson syndrome (SJS), from which TEN is differentiated by the extension of cutaneous lesions to more than 30% of the skin. These adverse reactions can becaused by avariey of medications, among them anticonvulsiveand use of lamotrigine with valproic acid, due to a resulting increased accumulation and half-life of the lamotrigine. We present the case of a female child previous lyt reated with anti convulsives,including valproic acid, who at 15 days after introduction of lamotrigine presented skinrash, fever, altered liver enzymes, and significant eosinophilia, thereby meeting the diagnostic criteria for DRESS syndrome. The second caseis that o famale child in treatment withant convulsive sincludig valproicacid, to which lamotrigine wasadded duetoo refractory epilepsy, and who 20 days later presented skin lesions with mucosal involvment which worsened with symptoms compatible with TEN and required ICU hospitalization. Both patients progressed favorably.