Síndrome hemolítico urémico secundario a Streptococcus pneumoniae. Reporte de un caso clínico / Streptococcus pneumoniae associated hemolytic uremic syndrome. Case report

El síndrome hemolítico urémico secundario a Streptococcus pneumoniae (SHU-Sp) es una complicación poco frecuente de las enfermedades invasoras por S. pneumoniae. Presenta una alta morbimortalidad, con requerimiento de transfusiones de glóbulos rojos y plaquetas, terapia de sustitución de la función renal de inicio precoz y más prolongada, así como mayores complicaciones a largo plazo, comparado con las formas secundarias a infección entérica por Escherichia coli productora de toxina Shiga. Presentamos el caso clínico de una preescolar de dos años, previamente sana, vacunada con tres dosis de PCV13, que desarrolló una insuficiencia renal aguda, anemia hemolítica y plaquetopenia, en el contexto de una neumonía con empiema y bacteriemia por S. pneumoniae.

Streptococcus pneumoniae associated hemolytic uremic syndrome (Sp-HUS) is an uncommon complication of invasive pneumococcal infections. Patients with Sp-HUS have a higher mortality and long term morbidity than those due to HUS from Shiga toxin-producing Escherichia coli infections (STEC-HUS). They often require more red blood cells and platelet transfusions, and early initiation of renal substitution therapy, presenting a higher rate of arterial hypertension and chronic renal disease in the long term, compared to STEC-HUS. We report a healthy 2 year-old infant, vaccinated with three doses PCV13, that developed acute renal failure, hemolytic anemia and thrombocytopenia in the course of a complicated pneumococcal pneumonia with empyema and bacteremia.

Síndrome multisistémico post COVID-19 y dificultad en su diagnóstico etiológico: a propósito de un caso clínico / Multisystem post COVID-19 syndrome and its difficult etiological diagnosis: a clinical case / Síndrome multissistêmica pós COVID-19 e a dificuldade no seu diagnóstico etiologico: relato de caso clínico

El SARS-CoV-2 afecta a un reducido número de pacientes pediátricos, que en su mayoría son asintomáticos o presentan compromiso respiratorio leve y evolución favorable. Sin embargo, en niños previamente sanos puede aparecer el síndrome inflamatorio multisistémico (SIM-C) o similar a Kawasaki (Kawasaki-like) asociado a la enfermedad por COVID-19, que evolucionan al shock y requiere internación en la unidad de cuidados intensivo. Presentamos el caso de un adolescente con antecedentes ambientales de SARS-CoV-2 37 días antes de su ingreso, que no se testeó en ese momento. Recibe la primera dosis de vacuna Pfizer 15 días antes de la presentación de un cuadro de sintomatología digestiva con dolor abdominal y fiebre, con test de antígenos COVID-19 y PCR viral negativos. Luego de una laparoscopia exploratoria, al sexto día de fiebre y dolor instaló conjuntivitis bilateral no supurada y exantema en tórax, odinofagia, astenia y lengua saburral con fenotipo Kawasaki. La serológica IgG anti spike fue positiva y la IgM negativa, con parámetros inflamatorios elevados, por lo que se planteó un síndrome multisistémico post COVID-19. Recibió tratamiento con inmunoglobulina intravenosa, ácido acetilsalicílico y metilprednisolona. Dada la necesidad de descartar la posibilidad de un evento atribuible a vacunación o inmunización (ESAVI), se realizó la búsqueda serológica para SARS-CoV-2, en un estudio cualitativo, buscando anticuerpos que no se generan con la vacuna Pfizer, que fueron positivos. De esta forma confirmamos la etiología post COVID-19 y descartamos la etiología por ESAVI, realizándose además el estudio cuantitativo de los anticuerpos anti spike con disminución de estos al mes del debut.

Summary: SARS-CoV-2 affects a low number of pediatric patients, most asymptomatic or with mild respiratory compromise and favorable evolution. However, in previously healthy children, Multisystem Inflammatory Syndrome (SIM-C) or Kawasaki-like Syndrome(Kawasaki-like) may appear linked to the COVID-19 disease, progressing to shock and requiring admission to the intensive care unit. We present the case of an adolescent with an environmental history of SARS-CoV-2 37 days prior to her admission, who was not tested at that time. She had received the first dose of Pfizer vaccine 15 days before the presentation of digestive symptoms with abdominal pain and fever. She had a negative Covid-19 antigen test and viral PCR. After an exploratory laparoscopy, on the sixth day of fever and pain, she developed bilateral non-suppurative conjunctivitis and a rash on the chest, odynophagia, asthenia, and coated tongue with the Kawasaki phenotype. The anti-spike IgG serology was positive and the IgM negative, with elevated inflammatory parameters, so a post-COVID-19 multisystem syndrome was suggested. She received treatment with intravenous immunoglobulin, ASA and methylprednisolone. In order to rule out the possibility of an event allegedly caused by vaccination or immunization (ESAVI), the serological search for SARS-CoV-2 was carried out through a qualitative study, looking for antibodies that are not generated with the Pfizer vaccine. The result was positive. Therefore, we confirmed the post-Covid etiology and ruled out the ESAVI etiology, and also performed the quantitative study of the anti-spike antibodies, which showed a decrease one month after the debut.

O SARS-CoV-2 afeta um número reduzido de pacientes pediátricos, em sua maioria, assintomáticos ou apresentando comprometimento do nível respiratório e evolução favorável. Porém, em crianças previamente sãs, pode aparecer a Síndrome Inflamatória Multissistêmica (SIM-C) ou similar a Kawasaki (Kawasaki-like) associada à doença por COVID-19, que pode evoluir a choque e requer a internação na UTI. Apresentamos o caso de um adolescente com antecedentes ambientais de SARS-CoV-2 37 dias antes do seu ingresso, que não se testou nesse momento. Recebendo a dose da primeira vacina Pfizer 15 dias antes da apresentação de sintomatologia digestiva com dor abdominal e febre. Teve um teste de antígenos COVID-19 viral negativo. Depois de una laparoscopia exploratória, ao sexto dia de febre e dor, teve conjuntivite bilateral não supurada e exantema en tórax, odinofagia, astenia e língua saburral com fenótipo Kawasaki. A sorologia IgG anti-spike foi positiva e a gM negativa, com parâmetros inflamatórios superiores, indicando uma síndrome multissistêmica pós Covid 19. O tratamento recebido foi imunoglobulina intravenosa, AAS e Metilprednisolona. Com o fim de descartar a possibilidade de um evento supostamente atribuível à vacinação ou vacinação positiva (ESAVI), realizou-se uma busca sorológica para SARS COV2, através de um estúdio qualitativo, procurando anticorpos não gerados pela vacina Pfizer, os quais foram confirmados. Nesta forma, confirmamos a etiologia pós-Covid e descartamos a etiologia por ESAVI. Aliás, realizamos um estudo quantitativo dos anticorpos anti-spike e comprovamos a sua diminuição a um mês de ter acontecido o debut.

Ursodeoxycholic Acid Therapy in Pediatric Primary Sclerosing Cholangitis: Predictors of Gamma Glutamyltransferase Normalization and Favorable Clinical Course.

OBJECTIVE: To investigate patient factors predictive of gamma glutamyltransferase (GGT) normalization following ursodeoxycholic acid (UDCA) therapy in children with primary sclerosing cholangitis. STUDY DESIGN: We retrospectively reviewed patient records at 46 centers. We included patients with a baseline serum GGT level ≥50 IU/L at diagnosis of primary sclerosing cholangitis who initiated UDCA therapy within 1 month and continued therapy for at least 1 year. We defined "normalization" as a GGT level <50 IU/L without experiencing portal hypertensive or dominant stricture events, liver transplantation, or death during the first year. RESULTS: We identified 263 patients, median age 12.1 years at diagnosis, treated with UDCA at a median dose of 15 mg/kg/d. Normalization occurred in 46%. Patients with normalization had a lower prevalence of Crohn's disease, lower total bilirubin level, lower aspartate aminotransferase to platelet ratio index, greater platelet count, and greater serum albumin level at diagnosis. The 5-year survival with native liver was 99% in those patients who achieved normalization vs 77% in those who did not. CONCLUSIONS: Less than one-half of the patients treated with UDCA have a complete GGT normalization in the first year after diagnosis, but this subset of patients has a favorable 5-year outcome. Normalization is less likely in patients with a Crohn's disease phenotype or a laboratory profile suggestive of more advanced hepatobiliary fibrosis. Patients who do not achieve normalization could reasonably stop UDCA, as they are likely not receiving clinical benefit. Alternative treatments with improved efficacy are needed, particularly for patients with already-advanced disease.

Ultrasonography of the colon in pediatric ulcerative colitis: a prospective, blind, comparative study with colonoscopy.

OBJECTIVES: To evaluate the usefulness of colonic ultrasonography (US) in assessing the extent and activity of disease in pediatric ulcerative colitis (UC) and to compare US findings with clinical and endoscopic features. STUDY DESIGN: Consecutive pediatric patients (n = 60) with a diagnosis of UC and suspected disease flare-up were prospectively enrolled; of these, 50 patients were eligible for the study. All underwent clinical evaluation, bowel US with color Doppler examination and colonoscopy. Blind US was performed the day before endoscopy in all patients. The US assessed variables were bowel wall thickness >3 mm, bowel wall stratification, vascularity, presence of haustra coli, and enlarged mesenteric lymph nodes. RESULTS: The endoscopic extent of disease was independently confirmed in 47 patients by US that yielded a 90% concordance with endoscopy (95% CI 0.82-0.96). Multiple regression analysis showed that US measurements with an independent predictive value of severity at endoscopy were increased bowel wall thickness (P < .0008), increased vascularity (P < .002), loss of haustra (P = .031), and loss of stratification of the bowel wall (P = .021). Each variable was assigned a value of 1 if present. The US score strongly correlated with clinical (r = 0.94) and endoscopic activity (r = 0.90) of disease (P < .0001). CONCLUSIONS: Colonic US is a useful first line noninvasive tool to assess the extent and activity of disease in children with UC and to estimate the severity of a flare-up, prior to further invasive tests.