About 30% of the cases of steroid resistant nephrotic syndrome display a genetically determined disease and will not recur after kidney transplant; the other cases with fully or partially immunological pathogenesis display a high risk of post transplant recurrence. Although lots of studies were carried out in the last 50 years the pathogenetic mechanism is still obscure and the therapeutic approach mostly empirical. The cornerstones principles of the therapies are based on removal of a still undefined "permeability factor" through plasma-exchange or other apheresis techniques and inhibition of its synthesis by the immunological system through different drugs. The probability of successfully inducing persistant remission is nowadays around 30%through the different schemes experimented so far which mostly include plasmapheresis. Rituximab in the last years has significantly increased the efficacy of the treatments. Non responders are rapidly evolving to graft loss and will most probably recur also in subsequent transplant. Apart from genetics no other risk factors are predictive for recurrence.
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http://dx.doi.org/10.1016/j.transci.2017.07.010 | DOI Listing |
CEN Case Rep
December 2024
Division of Nephrology and Hypertension, Department of Internal Medicine, The Jikei University School of Medicine, Minato-Ku, Tokyo, 105-8461, Japan.
Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide with heterogeneous histopathological phenotypes. Although IgAN with membranoproliferative glomerulonephritis (MPGN)-like features has been reported in children and adults, treatment strategies for this rare IgAN subtype have not been established. Here, we present the case of a 56-year-old man with no history of kidney disease who initially presented with nephrotic syndrome.
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December 2024
Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582, Japan.
Neuron-derived neurotrophic factor (NDNF) was discovered as a target antigen in membranous nephropathy (MN) caused by syphilis. However, there have been few reports of NDNF-positive MN in Japan. A 19-year-old female patient was admitted to our hospital with nephrotic syndrome and acute kidney injury.
View Article and Find Full Text PDFPediatr Nephrol
December 2024
Department of Pediatric Nephrology, Great North Children Hospital, Newcastle Upon Tyne, UK.
Background: Optimal dosing of rituximab when given with mycophenolate mofetil (MMF) for frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome (FRNS/SDNS) remains uncertain.
Methods: This was a prospective, non-inferiority, open-label randomized controlled multicentre study. Children (2-18 years old) with difficult FRNS/SDNS were randomized to group A (rituximab 375 mg/m once) or group B (rituximab 375 mg/m twice; 7-14 days apart) followed by continuous MMF and 3 months of tapered steroids.
Med Sci (Basel)
December 2024
Department of Nephrology, Hospital Cayetano Heredia, Lima 15002, Peru.
Background: Lupus podocytopathy (LP) is a non-immune complex-mediated glomerular lesion in systemic lupus erythematosus (SLE), characterized by the diffuse effacement of podocyte processes without immune complex deposition or with only mesangial immune complex deposition. LP is a rare cause of nephrotic syndrome in SLE patients with implications for prognosis and treatment.
Case Report: We present the case of a 28-year-old woman with a medical history of type 1 diabetes mellitus (T1DM) who presented with lower limb edema, dyspnea, hypercholesterolemia, with nephrotic range proteinuria, without acute kidney injury, and laboratory findings compatible with auto-immune hemolytic anemia.
This epidemiological study aimed to identify the primary categories of kidney pathology diagnosis and their prevalence among patients admitted to Shahid Labbafinezhad Teaching Hospital. We included 1006 kidney biopsy findings from 2019 to 2022. The majority of kidney patients (78%) were between the ages of 20 and 60 years.
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