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Background Reports on long-term complications of childhood-onset nephrotic syndrome (NS), such as obesity, osteoporosis, growth failure, and hypertension, are mostly from developed countries not representing South Asian ethnicities. Furthermore, data on cardiovascular health among patients with childhood-onset NS are limited. Methods This was an observational study involving patients attending a tertiary care center. Patients aged 15 years and older were examined for long-term complications and remission of NS at their visit in December 2021. Childhood-onset NS meant onset of NS before 10 years of age. Long-term complications included obesity, growth failure, low bone mineral density (BMD) Z score, hypertension, and increased carotid intima-media thickness (cIMT). Long-term remission was defined as no relapse for the last [Formula omitted] 3 consecutive years without immunosuppressive medication to maintain remission. Results Of 101 patients studied (~ 80% with frequent relapsing (FR)/steroid-dependent (SD) NS), the mean age was 17.6 (± 2.4) years at the time of study. Long-term complications were noted in 89.1% of patients which included one or more of the following: obesity (22.7%), growth failure (31.7%), low BMD Z score (53.5%), hypertension (31.7%), and high cIMT (50.5%). Thirty-nine patients (38.6%) were in long-term remission at the time of the study. Growth failure and low BMD Z scores were less frequent in patients with long-term remission compared to those without long-term remission. Conclusions In patients with childhood-onset NS (predominantly FR/SDNS) who were studied at [Formula omitted] 15 years of age, ~ 90% had long-term complications which included high cIMT in 50%. Only ~ 40% were in long-term remission. Graphical abstract, Author(s): Joyita Bharati [sup.1] , Karalanglin Tiewsoh [sup.2] , Lesa Dawman [sup.2] , Tarvinder Singh [sup.3] , Ujjwal Gorsi [sup.3] , Arun Prabhahar Rajarajen [sup.1] , Aakanksha Sharma [sup.1] , [...]

This article examines the role of B cells in the development and treatment of primary podocytopathies, specifically minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). The study focuses on adults with new-onset nephrotic syndrome (NS) caused by primary podocytopathy and compares them to healthy controls. The findings indicate that B cell proportions differ between children and adults, and specific B cell subsets are associated with disease activity and predict response to steroid treatment. These findings have implications for targeted therapies for primary podocytopathies. However, more research is needed to confirm these results. [Extracted from the article]

Systemic mastocytosis (SM) is a disorder of excessive mast cell accumulation in tissues due to a somatic gain-of-function mutation, commonly in the KIT gene, which prevents apoptosis of mast cells. Whereas bone marrow, skin, lymph nodes, spleen and gastrointestinal tract are commonly involved, kidneys are rarely involved directly by SM. However, there are increasing reports of indirect kidney involvement in patients with SM. Novel anti-neoplastic agents to treat advanced forms of SM include non-specific tyrosine kinase inhibitors, which are reported to be associated with kidney dysfunction in some patients. SM is also associated with immune-mediated glomerulonephritis (GN) such as mesangioproliferative GN, membranous nephropathy and diffuse proliferative GN. Kidney injury, in the form of monoclonal deposition disease and primary light chain amyloidosis, is reported in SM associated with plasma cell dyscrasia. In this narrative review we discuss the various ways kidneys (and the urinary tract) are involved in patients with SM. [ABSTRACT FROM AUTHOR]

The article discusses a proposed new classification for glomerulonephritis (GN), a type of kidney disease. The authors argue that the current focus on kidney pathology in diagnosing GN should not be downplayed, as kidney biopsies provide important information about the disease. They also highlight the limitations of non-invasive biomarkers in accurately diagnosing GN. The proposed classification categorizes GN into five groups based on the origin of pathogenesis, but the authors raise concerns about discrepancies and the inclusion of certain entities. They acknowledge the need for improvements in the classification of GN and hope that their critique will contribute to its refinement. [Extracted from the article]

Systemic AA amyloidosis is associated with systemic inflammatory processes such as autoimmune disorders or chronic infections. In addition, AA amyloidosis can develop in a localized or systemic form in patients with malignant neoplastic disorders, and usually involves kidneys impacting renal function. Among solid tumors, renal cell carcinoma (RCC) appears to be responsible for one-quarter to half of all cancers associated with amyloidosis. Among other solid cancers, various clinical presentations and pathological types of lung cancer and basal cell carcinoma skin were reported with AA amyloidosis more often than isolated case reports on other cancers with AA amyloidosis. Symptoms from kidney involvement rather than from the tumor per se were the presenting manifestations in cases of RCC associated with AA amyloidosis. Among hematological malignancies, clonal B cell/plasma cell dyscrasias such as monoclonal gammopathy and lymphoma were noted to be associated with AA amyloidosis. In addition, AA amyloidosis was reported in a substantial number of cases treated with immune checkpoint inhibitors such as pembrolizumab and nivolumab. The mechanism of association of cancer and AA amyloidosis seems to be mediated by the immune response exacerbated from the tumor and its microenvironment or immune therapy. The mainstay of treatment consists of therapy directed against the underlying malignancy or careful withdrawal of the offending agent. This review will discuss this rare but highly morbid clinical condition. [ABSTRACT FROM AUTHOR]

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological phenomenon commonly associated with kidney diseases, especially chronic kidney disease. A consequence of endothelial dysfunction, PRES is usually associated with uncontrolled blood pressures and can rarely have atypical radiological findings involving the brain stem and spinal cord, called posterior reversible encephalopathy with spinal cord involvement (PRES-SCI). These atypical features may be confused with other etiologies causing a delay in diagnosis and management. We describe a young male patient who presented with neurological symptoms suggestive of PRES; however, the atypical radiological findings along with concomitant rapidly progressive glomerulonephritis led to a diagnostic dilemma. Repeat neuro-imaging after appropriate blood pressure control showed disappearance of the lesions confirming the diagnosis of PRES-SCI, and kidney biopsy showed advanced IgA nephropathy. Knowledge of atypical features of PRES is crucial amongst nephrologists as it is a common association with kidney disease and prompt identification and management avoid irreversible sequelae and unnecessary investigations. [ABSTRACT FROM AUTHOR]