Congenital unilateral renal agenesis is a relatively frequent condition at birth diagnosed mostly incidentally. Despite the excellent prognosis, unilateral renal agenesis is associated with an increased risk of other structural abnormalities, including genital malformations. The authors present two cases of asymptomatic adolescents with known congenital unilateral renal agenesis and associated genital malformations solely diagnosed during puberty—a man with Zinner syndrome and a female with Mayer-Rokitansky-Küster-Hauser syndrome.

RESUMOIntrodução: O abandono de doentes do serviço de urgência pediátrico antes da observação médica constitui um problema multifatorial. Procurámos comparar características sociodemográficas de crianças que abandonaram a urgência e das que não abandonaram, assim como avaliar os motivos, recurso subsequente a cuidados de saúde e outcome clínico. Material e Métodos: Estudo caso-controlo prospetivo de amostra aleatória de crianças que abandonaram a urgência e de controlos pareados durante um período de três meses. Foi realizado um questionário telefónico para recolha de informação relacionada com os motivos para o abandono, outcomes clínicos e opiniões gerais. Material and Methods:This was a prospective case-control study of a random sample of children who left without being seen and their matched controls from an emergency department during a three-month period. We performed a phone questionnaire to obtain information concerning reasons for leaving, patient outcomes and general feedback.Results: During the study period, 18 200 patients presented to the emergency department, of whom 92 (0.5%) left without being seen. Fifty-five (59.8%) completed the questionnaire and there were 82 controls. The most common reasons for leaving were 'excessive waiting time' (92.7%) and 'problem could wait' (21.8%). A significantly higher number of patients who left sought further medical care (78.2% vs 11%) but they did not experience higher levels of unfavourable outcomes. Discussion: The waiting time seems to be the major factor that drives the decision to leave. The fact that parents felt safe in leaving and the low level of adverse outcomes highlights the low-acuity nature of the majority of patients who leave. Conclusion:Reducing the waiting times may be the logical strategic mean to decrease the rates of patients who leave without being seen. However, our data seems to indicate that the concerns surrounding clinical outcome after leaving may be partly unwarranted.

Introduction: Multisystem inflammatory syndrome in children (MIS-C) is a rare and severe manifestation of coronavirus disease 2019 (COVID-19). The aim of this study was to describe the characteristics of children with MIS-C admitted to a pediatric tertiary hospital in Portugal.Material and Methods: Observational descriptive study of MIS-C patients admitted between April 2020 and April 2021. Demographic and clinical characteristics, diagnostic tests, and treatment data were collected. The diagnosis of MIS-C was based on the World Health Organization and Centers for Disease Control and Prevention criteria.Results: We reported 45 children with MIS-C. The median age was seven years (IQR 4 - 10 years) and 60.0% were previously healthy. SARS-CoV-2 infection was confirmed in 77.8% by RT-PCR or antibody testing for SARS-CoV-2, and in 73.3%, an epidemiological link was confirmed. All the patients had a fever and organ system involvement: hematologic (100%), cardiovascular (97.8%), gastrointestinal (97.8%), mucocutaneous (86.7%), respiratory (26.7%), neurologic (15.6%), and renal (13.3%) system. Neurological (p = 0.035) and respiratory (p = 0.035) involvement were observed in patients with a more severe presentation. There was a significant difference of medians when comparing disease severity groups, namely in the values of hemoglobin (p = 0.015), lymphocytes (p = 0.030), D-dimer (p = 0.019), albumin (p < 0.001), NT-proBNP (p = 0.005), ferritin (p = 0.048), CRP (p = 0.006), procalcitonin (p = 0.005) and IL-6 (p = 0.002). From the total number of children, 93.3% received intravenous immunoglobulin, 91.1% methylprednisolone, and one patient (2.2%) received anakinra. Thirteen patients (28.8%) required intensive care and there were no deaths. Of the 21 patients evaluated, 90.4% had reduction of exercise capacity and of the 15 patients who underwent cardiac magnetic resonance, 53.3% had sequelae of cardiac injury.Conclusion: We observed a large spectrum of disease presentation in a group of patients where most were previously healthy. A small percentage of patients (28.9%) had a severe presentation of the disease. MIS-C is a challenge in current clinical practice and its diagnosis requires a high level of clinical suspicion as the timely initiation of therapy is essential to prevent complications. However, there is no scientific consensus on the treatment and follow-up of these patients.

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