A case is reported of a 21 year old man who suffered from Castleman's disease and systemic secondary amyloidosis. He was otherwise healthy until development of a history of edema and nocturia over 3 weeks. Physical examination was normal apart from ankle swelling. Renal biopsy showed amyloid deposits in the glomerulus with gentian violet and congo red. Positive staining by antibody against serum amyloid A protein was demonstrated. Nine months later because of persistent nephrotic syndrome, an abdominal tomography was performed and a mesenteric mass was detected. The patient underwent abdominal surgery. The histopathological examination showed plasma cell variant of Castleman disease and AA amyloidosis in the mass. After the removal of mesenteric mass, the proteinuria gradually decreased and disappeared, and no systemic findings were present.
Download full-text PDF |
Source |
---|
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.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!