So Hyun Ki

Background: Nutcracker syndrome (NCS) has been reported to coexist with various glomerulonephritis (GN). This study investigated clinical features of NCS combined with GN in a pediatric case series and the possible relationship between these two conditions.Methods: Clinical and pathologic findings of 15 children with NCS and biopsy-proven GN were analyzed. NCS was diagnosed with renal Doppler ultrasonography, abdominal computed tomography, and/or magnetic resonance imaging. Glomerular lesions were divided into two pathological categories: minor glomerular abnormalities (MGAs) and definite GN. Results: Mean age of all patients was 11 ± 3.36 years and mean follow-up duration was 53.8 ± 29.3 months. Chief complaint was proteinuria with or without hematuria. During follow-up, five patients developed left kidney enlargement. Abnormal levels in immunological tests were revealed in 10 patients. Extrarenal symptoms including gonadal varicocele, splenic cyst, syncope, and anemia were found in seven patients. On kidney biopsy, seven patients had MGAs and eight children showed definite GN (one case of focal GN, one case of mesangial proliferative GN, one case of focal segmental glomerulosclerosis, two cases of immunoglobulin A [IgA] vasculitis nephritis, and three cases of IgA nephropathy). While the MGA group showed a higher proportion of isolated proteinuria and a lower estimated glomerular filtration rate (eGFR) at the last visit, there were no differences in age, clinical features of NCS, extrarenal symptoms, immunological tests, and eGFR decline rate between the two groups.Conclusion: NCS may be associated with the presence of various GN. The causal relationship between NCS and GN should be further investigated.

Nutcracker syndrome (NCS) is a disease caused by compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by the predominance of IgA deposits in the glomerular mesangial area. Hematuria and proteinuria can be present in both diseases, and some patients can be concurrently diagnosed with NCS and IgAN; however, a causal relationship between the two diseases has not yet been clarified. Here, we report two pediatric cases of NCS combined with IgAN. The first patient presenting with microscopic hematuria and proteinuria was diagnosed with NCS at the initial visit, and the second patient was later diagnosed with NCS when proteinuria worsened. Both patients were diagnosed with IgAN based on kidney biopsy findings and treated with angiotensin-converting enzyme inhibitors and immunosuppressants. A high index of suspicion and timely imaging or biopsy are essential for the proper management of NCS combined with glomerulopathy.

2023년 9월까지 19세 이하 소아청소년 인구의 90% 이상에서 코로나바이러스감염증-19 (coronavirus disease 2019, COVID-19) 감염력이 있으며, 그중 40–60%에서 두통, 경련발작, 뇌염 등의 신경학적 증상이 보고되고 있다. 본 증례에서 저자들은 severe acute respiratory syndrome coronavirus 2 감염 후 경련과 의식 변화로 내원, COVID-19 뇌염으로 진단하여 정맥내 면역글로불린, 고용량 스테로이드, 항바이러스제, 항경련제 투여 등의 적극적인 치료로 회복된 3세 여자 환자를 보고하고자 한다. 환자는 입원 39일차 말하기와 혼자 걷기가 가능한 상태로 퇴원하였고 발병 1년이 지난 시점, 경한 언어 지연을 보이나 전반적으로 좋은 예후를 보이고 있다. COVID-19 뇌염 환자에서 조기 발견과 적극적인 치료가 긍정적인 예후와 연관됨을 본 증례를 통하여 보고하는 바이다. In Korea, >90% of children and adolescents aged <19 years have been infected with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) since 2020. Among confirmed cases of pediatric coronavirus disease 2019 (COVID-19), 40–60% of patients developed neurologic symptoms such as seizures, headache, and encephalitis. Herein, we report the case of a 3-year-old female patient with SARS-CoV-2 infection who presented with seizures and altered consciousness and was diagnosed with COVID-19 encephalitis. The patient recovered after treatment with intravenous immunoglobulin, high-dose steroids, anti-seizure drugs, and an anti-viral agent. She was discharged after regaining the ability to speak words and walk alone on hospital day 39. Complete recovery was observed at the 1-year follow-up. The findings in this case suggest that early detection and active intervention is associated with better outcomes in patients with COVID-19 encephalitis.