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Given the wide diversity of causes of hematuria, ranging from simple urinary tract infections with rapid recovery to severe glomerulonephritis with fast decline in kidney function, it is essential to recognize the underlying disease. The first objective of the assessment is to determine whether the cause of the hematuria is medically significant. The combination of hematuria with proteinuria, the presence of hypertension, or worsening kidney function can represent signs of progressive kidney disease. Differentiating the various causes of hematuria is often simple and obvious based on the clinical signs and gross appearance of the urine. However, in some instances, additional non-invasive investigations, such as ultrasound imaging, urinary red cell morphology, measurement of calcium and other solutes in the urine, evaluation of kidney function, and protein excretion, are needed to elucidate the nature of the hematuria. Taking a detailed family history can help in establishing the underlying cause in cases of familial hematuria. On the other hand, the decision to perform a kidney biopsy in children with asymptomatic hematuria remains a challenging issue for clinicians. Ultimately, the frequency of diagnosis of glomerular involvement causing hematuria may depend on the threshold for performing a kidney biopsy. The following review will focus on the diagnostics of hematuria, starting with difficulties regarding its definition, followed by various means to differentiate between urinary, glomerular, and other causes, and finally reviewing the most common diseases that, due to their frequency or their effect on kidney function, present a diagnostic challenge in everyday practice., Author(s): Orsolya Horváth [sup.1] , Attila J. Szabó [sup.1] , George S. Reusz [sup.1] Author Affiliations: (1) grid.11804.3c, 0000 0001 0942 9821, 1st Department of Pediatrics, Semmelweis University, , 53-54 [...]

Background There are few reports on kidney complications after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) messenger RNA (mRNA) vaccination, especially in the pediatric population. We report a pediatric case diagnosed with crescentic glomerulonephritis (CrGN) after the second dose of the SARS-CoV-2 mRNA vaccine. Case-diagnosis/treatment A 16-year-old girl was admitted due to dyspnea and headache approximately 6 weeks after receiving the second SARS-CoV-2 mRNA vaccine (Pfizer-BioNTech). She had previously experienced fever, nausea, vomiting, and dyspnea after the first vaccination, which persisted for a week. On admission, her blood pressure was 155/89 mmHg with a 7 kg weight gain in a month. She had microhematuria and proteinuria. Laboratory findings were as follows: blood urea nitrogen/creatinine, 66/9.57 mg/dL; and brain natriuretic peptide, 1,167 pg/mL. Anti-neutrophil cytoplasmic antibody (ANCA), anti-glomerular basement membrane (GBM) antibody, and antinuclear antibody findings were negative. Kidney doppler sonography revealed swelling and increased echogenicity of both kidneys with increased resistive index. Cardiac magnetic resonance imaging results showed early minimal fibrosis of myocarditis. We then started hemodialysis. Kidney biopsy showed diffuse extra capillary proliferative glomerulonephritis with diffuse crescent formation. We treated the patient with methylprednisolone pulse therapy with subsequent oral steroids and mycophenolate mofetil. Although dialysis was terminated, the patient remained in the chronic kidney disease stage. Conclusions This is the first case of ANCA-negative CrGN after SARS-CoV-2 mRNA vaccination in the pediatric population. As children are increasingly vaccinated with SARS-CoV-2 mRNA vaccines, monitoring for kidney complications is warranted., Author(s): Sujeong Kim [sup.1] , Jiwon Jung [sup.1] , Haeyon Cho [sup.2] , Jina Lee [sup.1] , Heounjeong Go [sup.2] , Joo Hoon Lee [sup.1] Author Affiliations: (1) grid.267370.7, 0000 [...]

Background Membranous nephropathy is an uncommon cause of nephrotic syndrome in pediatrics. Methods We reviewed our kidney biopsy records for patients [less than or equal to] 20 years of age with membranous nephropathy without evidence of systemic lupus erythematosus within 6 months of biopsy (January 1995-September 2020). Staining for PLA2R, NELL1, THSD7A, SEMA3B, EXT2 (3 biopsies), and IgG-subclass were performed. Results Sixteen children ([less than or equal to] 12 years) and 25 adolescents (13-20 years) were identified. Four children and 15 adolescents showed autoantigen positivity: PLA2R+/SEMA3B- (13), SEMA3B+/PLA2R+ (2), SEMA3B+/PLA2R- (1), NELL1 (1), EXT2+ (2), and THSD7A (0). Co-morbidities associated with PLA2R positivity included IPEX syndrome, active hepatitis B, Von Hippel Lindau syndrome, solitary kidney, type 1 diabetes, hyperuricemia, pregnancy (1), obesity (3), type II diabetes, H. pylori, viral prodrome, and nephrolithiasis. The SEMA3B+/PLA2R- adolescent was pregnant, the NELL1+ adolescent was obese, and the two EXT2+ adolescents eventually met the clinical criteria for lupus (4, 9 years post-biopsy). Co-morbidities among the remaining 24 patients included remote hepatitis B (2), Down's syndrome, lysinuric protein intolerance, recurrent UTIs, hypothyroidism, pregnancy (3), and obesity (2). Follow-up data was available for 12 children and 16 adolescents. Of the 12 children, 6 achieved complete remission, 4 achieved partial remission, and 2 had no response to treatment (1 transplant). Of the 16 adolescents, 4 achieved complete remission, 4 achieved partial remission, and 8 had no response to treatment (3 transplants). A child with 'full-house' immunofluorescence staining achieved spontaneous disease remission. Conclusion Our non-lupus membranous nephropathy cohort represents one of the largest pediatric studies to date. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Paul Miller [sup.1] , Li Lei [sup.1] [sup.2] , Vivek Charu [sup.1] , John Higgins [sup.1] , Megan Troxell [sup.1] , Neeraja Kambham [sup.1] Author Affiliations: (1) grid.168010.e, 0000000419368956, [...]

The microbial ecosystem within the bladder that can be measured within the urine, or urobiome, is an emerging field of study with little published data regarding children. However, investigations into urobiome research have the potential to significantly impact the understanding of the pathophysiology of genitourinary conditions, as well as potentially identify novel therapeutics. Therefore, both researchers and clinicians should be aware of pediatric urobiome research. The purpose of this review is to highlight the literature around urobiome research in urinary tract infections, nephrolithiasis, and neurogenic bladder; comment on pediatric-specific considerations when reading and interpreting the urobiome literature; and to identify new potential areas of research., Author(s): Elisabeth Cole [sup.1] , Nader Shaikh [sup.1] , Catherine S. Forster [sup.1] Author Affiliations: (1) grid.21925.3d, 0000 0004 1936 9000, Department of Pediatrics, University of Pittsburgh School of Medicine, [...]

Context.--Immunoglobulin G4 (IgG4)-related disease is rare but well characterized in adults; however, the clinical and histologic manifestations in children may differ. Objective.--To review the clinical and histologic features of IgG4-rich head and neck lesions in a pediatric population. Design.--Retrospective search for cases with IgG4 immunohistochemical staining performed at our institution from 2011 to 2019. Review of clinical courses, serology profiles, histologic patterns, and immunohistochemical staining patterns. Results.--Four pediatric IgG4-rich lesions were identified and showed distinct histologic patterns from adult IgG4-related disease, including absence of pathognomonic findings associated with the latter. One case showed intralesional immunoglobulin light-chain restriction. Clinical review showed serum IgG4 elevation in 2 of 4 cases, presence of additional autoantibody positivity, and a generally benign/treatment-responsive clinical course. Conclusions.--Pediatric IgG4-related disease shows distinct clinical, serologic, and histologic features from its adult counterpart. Pediatric IgG4-related disease involving the orbit has unique clinical characteristics, including frequently normal serum IgG4 levels and female predominance. Awareness of and evaluation for these features may improve diagnosis and treatment. (Arch Pathol Lab Med. 2022;146:611-618; doi: 10.5858/arpa.2021-0041-OA), Immunoglobulin (Ig) G4-related disease is a rare disease that typically affects middle-aged adults, with an average age of onset of 50 to 60 years and male predominance. (1,2) Owing to [...]

Author(s): Tomohiro Udagawa [sup.1] , Yaeko Motoyoshi [sup.2] Author Affiliations: (1) grid.265073.5, 0000 0001 1014 9130, Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo [...]