RESUMO
Background: Multisystemic inflammatory syndrome in children (MIS-C) has increasingly been documented globally with the progression of the COVID-19 pandemic and a significant proportion of cases have been noted in children of Black descent. There has been a noticeable discrepancy in the presentation and outcomes of COVID-19 infection in sub-Saharan Africa compared to the rest of the world. We documented the demography, clinical features, laboratory and imaging findings, therapeutic management, and short-term outcomes of paediatric patients with MIS-C diagnosed during the COVID-19 pandemic in Lagos, Nigeria. Methods: We carried out a retrospective review of MIS-C cases seen in nine public and private hospitals in Lagos from July 10, 2020 to July 30, 2021. Data on clinical presentation, laboratory investigations, therapy as well as outcomes at 2 weeks, 6 weeks, 3 months and 6 months were analyzed. Findings: 28 children and adolescents with median age of 7·5 (IQR 2·3 - 9·4) years were diagnosed with MIS-C. MIS-C was suspected in 24 patients (85·7%) at initial clinical evaluation and mucocutaneous, gastrointestinal and cardiovascular manifestations were identified in 75·0%, 71·4% and 89·3% of patients respectively. Acute kidney injury and aseptic meningitis were noted in 32·1% and 17·9% of patients respectively. Cardiac manifestations at presentation included coronary dilatation and pericardial effusion in 46·4% each, ventricular dysfunction (32·1%), atrioventricular valve regurgitation (25·0%), prolonged QTc interval (40·0%) and first-degree atrioventricular block (16·0%). Therapy included aspirin in 89·3%, steroids in 75·0% and intravenous immunoglobulin (IVIG) infusion in 60·7%. All patients survived and were discharged after a mean of 11·14 (SD 5·65) days. Frequency of coronary dilatation had reduced from 46·4% to 7·1% by 3 months follow up and prolonged QTc interval persisted until the 6 week follow up in 4.5% of patients. Echocardiogram and electrocardiogram findings were normal in all patients assessed at 6 months follow up. Interpretation: MIS-C is an important diagnosis in children presenting with prolonged fever during the COVID-19 pandemic. Cardiovascular manifestations occurred in several children with MIS-C and improved by 6 months follow up. Early diagnosis and prompt institution of a combination of antiplatelet therapy, steroids and IVIG appear to be beneficial. Funding: None.
RESUMO
Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Our objective of this report is to describe clinical manifestation, laboratory, and radiological characteristics of PBS in our patients, to highlight the limitations to offering appropriate patient care due to parents demanding discharge against medical advice and the need to increase the awareness regarding this rare disease. We report three cases; all referred after birth with lax abdominal wall, congenital anomalies of kidney, and urinary tract. One of the patients had an absent right foot. They all had cryptorchidism, and in one, there was deranged renal function. The reported cases had both medical and radiological interventions to varying degrees. They all had an abdominal ultrasound which revealed varying degrees of hydronephrosis, hydroureters, and bladder changes. Voiding cystourethrogram showed vesicoureteric reflux in one of the reported cases. Urinary tract infections were appropriately treated with antibiotics based on sensitivity. PBS management in our setting remains a challenge because of strong cultural beliefs, and high rate of discharge against medical advice. Focus should be on parent education, early diagnosis, and multidisciplinary management approach.
