Idiopathic nephrotic syndrome in children has conventionally been associated with minimal change disease. However, recent reports have conflictingly suggested that the frequency of focal segmental glomerulosclerosis (FSGS) in children might be on the increase, as has occurred in adults. The aim of the present work was to review the medical literature to ascertain whether an increase in the frequency of FSGS is occurring and, if so, to quantify such increase. We reviewed the studies comparing the frequency of FSGS in two consecutive periods over the past three decades (period 1 versus period 2). We pooled the data of the studies and then estimated FSGS frequency in two ways: (a) including in the denominator all patients with nephrotic syndrome and (b) including only patients who had undergone kidney biopsy. Both analyses were aimed to determine the odds ratio of FSGS occurrence in the second period. Six studies fulfilled the inclusion criteria, involving 1,149 patients with nephrotic syndrome. Four studies were used to calculate FSGS frequency, including in the denominator all nephrotic patients (n = 885), yielding an odds ratio of 2.22 (95% CI = 1.18-4.18). The analysis combining five studies with the number of biopsies in the denominator (n = 603) produced an odds ratio of 1.98 (95% CI = 1.12-3.50). These results suggest that a shift in the pathological pattern of nephrotic syndrome in children might be occurring, resulting in an increase in FSGS frequency. This hypothesis has major clinical significance due to the poorer prognosis associated with FSGS.
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http://dx.doi.org/10.1007/s00467-007-0516-0 | DOI Listing |
JAMA Pediatr
January 2025
Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Importance: Cyclophosphamide and calcineurin inhibitors are the most used nonsteroid immunosuppressive medications globally for children with various chronic inflammatory conditions. Their comparative effectiveness remains uncertain, leading to worldwide practice variation. Nephrotic syndrome is the most common kidney disease managed by pediatricians globally and suboptimal treatment is associated with high morbidity.
View Article and Find Full Text PDFPak J Med Sci
January 2025
Khemchand N Moorani, MBBS, MCPS, FCPS, IPNA Pediatr Nephrol fellowship Professor, Department of Pediatric Nephrology, The Kidney Centre Post Graduate Training Institute, Karachi. 197/9, Rafiqui Shaheed Road, Karachi-75530, Pakistan.
Objectives: To determine the effectiveness of Levamisole (Leva) in maintaining short-term and long-term remission in early steroid responders (ESRs) and late steroid responders (LSRs).
Methods: This retrospective study on 106 cohorts, aged 2-14 years with frequent-relapsing (FR) and steroid-dependent nephrotic syndrome (SDNS) who received Leva over 10-years (2012-2023), was carried out at tertiary care centre, Karachi from January-August 2023. Patients were categorized based on steroid response during first episode of NS as ESRs if complete remission (CR) was achieved within two weeks of daily steroid and LSRs if CR achieved between two-four weeks.
Introduction Nephrotic syndrome, an unusual clinical presentation of IgA nephropathy (IgAN), occurs only in a few cases. The data regarding its clinical characteristics and treatment outcomes are lacking. Material and methods In this retrospective analysis, we reviewed kidney biopsies conducted between January 2007 and December 2018.
View Article and Find Full Text PDFInt Med Case Rep J
January 2025
Department of Neurology, The Lishui Hospital of Wenzhou Medical University, The First Affiliated Hospital of Lishui University, Lishui People's Hospital, Lishui, Zhejiang, 323000, People's Republic of China.
Primary amyloidosis (AL type) is a systemic disease that can lead to structural and functional damage to organs and tissues such as the kidney, heart and liver with non-specific symptoms. Most of the affected patients develop thyroid infiltration and thus diffuse enlargement of the thyroid gland, while cases leading to hypothyroidism are exceedingly rare. Some researchers have analyzed thyroid function in newly diagnosed patients with AL amyloidosis, and found that the incidence of overt hypothyroidism is only 7%.
View Article and Find Full Text PDFBiomedicines
January 2025
Second Department of Internal Medicine, Division of Nephrology, Kansai Medical University, Hirakata 573-1010, Japan.
: Charcot-Marie-Tooth (CMT) disease is an inherited peripheral neuropathy primarily involving motor and sensory neurons. Mutations in INF2, an actin assembly factor, cause two diseases: peripheral neuropathy CMT-DIE (MIM614455) and/or focal segmental glomerulosclerosis (FSGS). These two phenotypes arise from the progressive degeneration affecting podocytes and Schwann cells.
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