Nephrotic syndrome (NS) is a clinical diagnosis with proteinuria, hypoalbuminaemia and oedema. NS is rare in children, and its incidence in The Netherlands is unknown. The aim of this study was to estimate the incidence of idiopathic NS in the Netherlands. All paediatric patients (age 0-18 years) with a newly diagnosed NS in the Netherlands were registered by the Dutch Pediatric Surveillance Unit during the years 2003 until 2006, secondary NS was excluded. All paediatricians filled out questionnaires about the first clinical findings of the patients and incidences were calculated. A literature review on incidences of childhood NS was conducted. The incidence of NS in children in the Netherlands in the years 2003 until 2006 was 1.52/ 100, 000 children/ year. The median age at diagnosis was 3.88 years with a mean age of 5.08 years. A significant male:female ratio of 2.04:1 was found. This prospective study of NS in the Netherlands revealed an incidence of 1.52:100, 000 children/year, and is similar to the incidences found all over the world.
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http://dx.doi.org/10.1007/s00467-011-1851-8 | DOI Listing |
Pediatr Nephrol
January 2025
Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Background: Coenzyme Q10 (CoQ10) nephropathy is a well-known cause of hereditary steroid-resistant nephrotic syndrome, primarily impacting podocytes. This study aimed to elucidate variations in individual cell-level gene expression in CoQ10 nephropathy using single-cell transcriptomics.
Methods: We conducted single-cell sequencing of a kidney biopsy specimen from a 5-year-old boy diagnosed with a CoQ10 nephropathy caused by a compound heterozygous COQ2 mutation complicated with immune complex-mediated glomerulonephritis.
Sci Rep
January 2025
Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University, 58 Zhongshan Road II, Guangzhou, 510080, China.
Nephrotic syndrome (NS) occurs in 5-15% of patients with IgA nephropathy (IgAN), resulting in poorer long-term outcomes compared to those without NS. Clinical features and renal prognosis for patients with both NS and IgAN across different kidney pathologies have not been fully elucidated. This study included patients with primary IgAN through renal biopsy at the First Affiliated Hospital of Sun Yat-sen University from January 2001 to November 2021 presenting with NS.
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Pediatrics Department, Dr. Sulaiman Al Habib Hospital, Riyadh, SAU.
Coenzyme Q2 (CoQ2) mutations are a group of autosomal recessive mitochondria-linked diseases that result in coenzyme Q10 (CoQ10) deficiency (CoQ10: a cofactor in mitochondrial energy production). Its deficiency leads to multiple systemic clinical presentations; however, isolated steroid-resistant nephrotic syndrome (SRNS) is considerably rare. Multiple genetic mutations have been reported with different ranges of severity and prognosis, with variable responses to CoQ10 supplementation.
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Nephrology, Ibn Sina Hospital, Rabat, Rabat, MAR.
Cryoglobulinemic vasculitis is a rare small-vessel vasculitis leading to multi-organ dysfunction, often associated with chronic infections like hepatitis C virus (HCV), and autoimmune disorders. Most cases involve mixed monoclonal or polyclonal immunoglobulins, presenting symptoms such as purpura, arthralgias, and weakness. Severe organ involvement, particularly cardiac, is rare but potentially life-threatening.
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Department of Nephrology, Dialysis, Transplantation, Dr C. I. Parhon Hospital, Iasi, Romania.
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