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Multisystem inflammatory syndrome in children (MIS-C) is a postinfectious condition which usually develops 4 to 6 weeks after SARS-CoV-2 infection in a genetically predisposed individual. Clinical features are heterogeneous and include fever, respiratory compromise, mucocutaneous involvement with conjunctival abnormalities and erythematous exanthem, abdominal pain, and diarrhea. Neurologic and cardiovascular symptoms can also develop, including coronary artery dilatation. Some cases involve 2 or more organs and require critical admission. Echocardiography is the mainstay of cardiac evaluation in the acute setting as well as on outpatient follow-up. We present the case of a 4-month-old female with no past medical or surgical history who presented with a prolonged febrile syndrome associated with severe respiratory illness, gastrointestinal symptoms, and mucocutaneous abnormalities. Diagnosis of MIS-C was established based on clinical findings, persistently elevated markers of systemic inflammation and positive SARS-CoV-2 molecular test and evidence of prior SARS-CoV-2 infection with SARS-CoV-2 IgG positive. Echocardiogram evidenced myopericarditis and coronary aneurysms and patient was deemed candidate for immunomodulatory therapy with intravenous immunoglobulin (IVIg), resulting in favorable clinical and paraclinical outcomes. Few cases of giant coronary aneurysms have been reported in children. There are no existing literature reports about coronary thrombosis or thrombus formation resulting from vascular aneurysmal dilations in this population. As such, the prognosis and natural history of coronary artery aneurysms in the setting of MIS-C remain largely unknown.
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SUMMARY: Multisystemic inflammatory syndrome (MIS-C) can develop as a complication of SARS CoV-2 infection, involving the gastrointestinal system mainly by vasoconstriction, edema, glandular hyperplasia, and a procoagulant state leading to direct tissue injury. METHOD: ology: a series of cases including 8 patients with MIS-C treated in two highly complex institutions is presented. These patients, had abdominal symptoms of surgical management. RESULTS: The average age was 9.5 years and the most frequent symptoms were fever, abdominal pain, diarrhea (100%); in addition, 87.5% presented shock. The diagnosis of SARS CoV-2 was confirmed by RT-PCR test in 37.5%, antigen 12.5% and the rest of the patients showed IgM and IgG antibodies. In laboratories, the increase in acute phase reactants, Erythrocyte Sedimentation Rate (ESR), C-reactive protein, procalcitonin, as well as troponin, D dimer and proBNP, is highlighted. The surgical outcome documented 2 patients with a normal appendix, 3 patients with edematous appendicitis, and 3 patients with complicated appendicitis. CONCLUSIONS: patients with MIS-C display abdominal symptoms similar to those present in surgical emergencies and a non-negligible number of cases require surgical exploration. This condition poses a new differential diagnosis to the surgical abdomen in pediatric patients.
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Dengue is a single-stranded RNA virus belonging to the Flaviviridae family. It is an endemic virus in tropical countries. In Colombia, 4 serotypes are present, and the disease is a burden for public health, social programs, and the economic sectors. The main vector is Aedes aegypti, and most infections are asymptomatic or minimally symptomatic. The hemorrhagic appearances of severe dengue are due to plasma leakage as a result of increased vascular permeability, severe thrombopenia, and hemoconcentration. In 2020, 78,979 cases of dengue were reported in Colombia. 38,836 (49.2%) of them were warning-free signs, 39,246 (49.7%) with warning signs, and 897 (1.1%) of severe dengue. As it is well-known, viral diseases are immune system activators, triggering off a loss of tolerance in it. Dengue is not an exception, and it is able to explain different autoimmune phenomena including macrophage activation. Mechanisms have been described by which an exacerbated response of the disease is triggered through the increase of infected cells, formation of immune complexes, and complement pathway activation, which lead to a cross-reaction of viral antigens with epithelial cells with platelets with subsequent endothelial dysfunction and bleeds. The first description of Evans syndrome was made in 1951 by Robert Evans. This syndrome is characterized by the combination of autoimmune hemolytic anemia, immune thrombocytopenia, and, less common/usual, immune neutropenia. This disease's etiology is unknown, and the dysregulation of the immune system is among its possibilities. Here, we present the case of an unusual hematological and immunological complication of a patient who developed Evans syndrome during severe dengue, taking into account the concomitantly limited literature available for these two diseases, the need for a broader diagnostic approach, multidisciplinary intervention, and a more complex therapeutic approach.
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Resumen Introducción: La púrpura de Henoch-Schönlein es la vasculitis más frecuente en la infancia. La afectación renal continúa siendo la causa de morbimortalidad principal en niños. El espectro del compromiso renal va desde manifestaciones leves hasta el desarrollo de síndrome nefrótico o nefrítico, o insuficiencia renal. Objetivo: Determinar las características clínicas al comienzo y el compromiso renal de los pacientes con diagnóstico definitivo de púrpura de Henoch-Schönlein, durante el primer mes, a los 3 meses y al ario de seguimiento. Métodos: Estudio descriptivo, retrospectivo, de pacientes con diagnóstico definitivo de púrpura de Henoch-Schönlein, de la consulta de Reumatología Pediátrica, en una institución de Bogotá, Colombia, en el período comprendido de 2010 a 2016. Resultados: Ochenta y seis pacientes fueron incluidos en el estudio, 42 niñas y 44 niños, edad media de presentación 5,3 años, DE 2,4 años (1a14anos). Se presentó compromiso renal en 39 pacientes (45%). Se evidenció una tendencia mayor a compromiso renal entre los pacientes con artritis (p = 0,053). El hallazgo clínico más frecuente fue proteinuria aislada (49%), seguido de proteinuria/hematuria (28%) y hematuria aislada en el 15%. Había 8/39 pacientes con compromiso renal durante el año de seguimiento que fueron llevados a biopsia, 6 (75%) con compromiso renal al inicio y 25% al mes. El promedio de seguimiento fue de 26,8 meses con una desviación estándar de 17 meses (1 a 72 meses), no hubo disfunción renal a la última valoración en ninguno de los 39 pacientes con compromiso renal. Conclusión: El compromiso renal fue más frecuente en las primeras semanas de la enfermedad, así como en las formas severas.
Abstract Introduction: Henoch-Schönlein Purpura is the most common vasculitis in children, with Henoch-Schönlein Purpura kidney involvement remaining the main cause of morbidity and mortality. The spectrum of Kidney involvement of kidney involvement ranges from mild symptoms to the development of a nephrotic and/or nephritic syndrome or kidney failure. Objective: To determine the clinic features at onset, and kidney involvement of patients with a final diagnosis of Henoch-Schönlein Purpura during the first month, 3 months, and up to the first year of follow-up. Methods: A retrospective study conducted on patients with a final diagnosis of Henoch-Schönlein Purpura in a Paediatric Rheumatology Department in an institution of Bogota, Colombia, during the period between 2010 and 2016. Results: The study included 86 patients, 42 girls and 44 boys. The median age at disease onset was 5.3 years (SD 2.4 years: range1-14years). Kidney involvement was present in 39/86 (45%) patients. A trend to kidney involvement was observed in patients with abdominal symptoms (p=,053). The most frequent clinical finding was isolated proteinuria (49%), followed of proteinuria/haematuria (28%), and isolated hematuria (15%). Renal biopsy was performed on 8/39 patients with Henoch-Schönlein Purpura nephritis. The mean follow-up was 26.8 months (SD 17 months: range 1-72). There was no evidence of kidney damage in the last assessment in any of the 39 patients with kidney involvement. Conclusion: In this group of patients, kidney involvement was more severe and common in the first weeks of the disease onset.