Nephrology
John D Firth, Professor Ian Gilmore in MRCP Part 1 Self-Assessment, 2017
The diagnosis is minimal change glomerulonephritis, also known as minimal change nephropathy, minimal change disease, lipoid nephrosis and idiopathic nephrotic syndrome. Standard initial therapy is with corticosteroids, typically prednisolone at a dose of about 1 mg/kg/day, which introduces remission in around 80% of patients. Supportive therapies are also given, diuretics to clear oedema will clearly be appropriate in this case, and some nephrologists would prescribe warfarin to reduce risk of thromboembolism and a statin to reduce hypercholesterolaemia, although many would elect to see whether or not the patient went into a rapid remission that would render these agents unnecessary. Ciclosporin is useful as a steroid-sparing agent in patients who frequently relapse but would not be used as initial treatment.
Practice Paper 1: Answers
Anthony B. Starr, Hiruni Jayasena, David Capewell, Saran Shantikumar in Get ahead! Medicine, 2016
Nephrotic syndrome, not to be confused with nephritic syndrome, is characterized by loss of large amounts of protein in the urine due to an excessively leaky glomerular basement membrane. The features of nephrotic syndrome include proteinuria; peripheral oedema; hypoalbuminaemia; hypertension; hyperlipidaemia; hypercoagulability; and an increased risk of infection, including spontaneous bacterial peritonitis. In children, the main cause of nephrotic syndrome is minimal-change glomerulonephritis (>90% of cases). Minimal-change disease is thought to be secondary to abnormal T-cell activity that causes a reduction in the synthesis of anion channels within the glomerular basement membrane, thereby making it more permeable. On renal biopsy, the histological appearance is normal. Electron microscopy of the sample may reveal fused and abnormal podocytes. The treatment of minimal-change disease involves steroid therapy, fluid restriction, a low-salt diet, penicillin prophylaxis, ACE inhibitors to reduce proteinuria and statin therapy for hyperlipidaemia. Immunosuppression is occasionally indicated in severe and refractory disease.
Diabetic Nephropathy
Jahangir Moini, Matthew Adams, Anthony LoGalbo in Complications of Diabetes Mellitus, 2022
Nephrotic syndrome is of different causes based on patient age. The most common primary causes include minimal change disease, focal segmental glomerulosclerosis, and membranous nephropathy. Minimal change disease causes a fast onset of edema and heavy proteinuria, and usually affects children, with renal function remaining normal in most cases. Secondary causes only make up fewer than 10% of childhood cases. However, secondary causes make up more than 50% of adult cases, and include diabetic nephropathy and preeclampsia. Amyloidosis causes 4% of all cases. HIV-associated nephropathy occurs in patients with AIDS, and is a form of focal segmental glomerulosclerosis.
Significance of M2 macrophage in tubulointerstitial disease secondary to primary Sjogren's disease
Published in Renal Failure, 2018
Jun Li, Ya-Fen Yu, Chang-Hua Liu, Cui-Mei Wang
Formalin-fixed, paraffin-embedded renal tissues were obtained from patients with pSS (n = 10), drug-associated chronic interstitial nephritis (CIN, n = 8, the drugs includes proton pump inhibitor and Chinese herbs). Renal tissues samples of minimal change disease patients (MCD, n = 5), and those obtained from normal control kidneys (n = 3) were used as negative controls. Morning urine samples before renal biopsy were collected for the detection of urinary osmotic pressure, urinary NAG and urinary β2- microglobulin. All patients with pSS fulfilled the international classification criteria for Sjögren’s syndrome (2012 American College of Rheumatology classification criteria for Sjögren’s syndrome) [9]. Exclusion criteria were the presence of malignancy, acute inflammation and sepsis. All the patients agreed and signed the informed consent. This study was performed in accordance with the Declaration of Helsinki. This study obtained the permission of the Ethics Committee of the affiliated hospital of Jiangnan University and the Clinical Medical College of Yangzhou University.
Contribution of Electron Microscopy to the Clinicopathologic Diagnosis in Childhood Glomerular Renal Diseases
Published in Fetal and Pediatric Pathology, 2019
Secil Arslansoyu Camlar, Mehtat Ünlü, Alper Soylu, Duygu Karaca, Sulen Sarioglu, Salih Kavukcu
EM contributes to diagnosis of nephrotic syndrome in adults by 50% [16]. In histopathological diagnosis of glomerulonephritis, the role of EM differs in childhood. Its contribution is higher in children and has been reported as essential in 73% of childhood nephrotic syndrome [4]. FSGS is diagnosed primarily by LM and DIF, EM confirms the widespread effacement of foot processes of the podocytes and excludes secondary causes of sclerosis by absence of electron-dense deposits [4, 5]. Minimal change disease (MCD) and FSGS both show foot process effacement. In MCD, the foot process effacement is often extensive, whereas in FSGS it is more likely to be focal [4]. Other features more suggestive of FSGS are foot process separation from the basement membrane, hyaline deposits in the mesangium and occasionally in the capillary lumen/subendothelially, wrinkling and/or collapse of the basement membrane, and intralumenal foam cells. Findings about podocytes are quite similar in both diseases. Our most commonly used and distinctive finding is collapsed glomerular capillaries with thick and wrinkled basal lamina and sometimes foamy cells adherent between Bowman’s capsule and the capillary tuft. Even though some studies has stated that the number of vacuoles was higher in the FSGS compared with MCD, we did not diagnose segmental sclerosis with only this finding in patients who did not have capillary collapse. In our study population, although LM was normal in 2 of 13 patients with nephrotic syndrome, EM examination disclosed FSGS. In MCD and FSGS, when LM and DIF were unremarkable, foot process effacement could be demonstrated only by the ultrastructural study [5].
Efficacy of extracorporeal plasma therapy for adult native kidney patients with Primary FSGS: a Systematic review
Published in Renal Failure, 2023
Jing Miao, Pajaree Krisanapan, Supawit Tangpanithandee, Charat Thongprayoon, Michael A. Mao, Wisit Cheungpasitporn
Outcomes of different EPT modality are shown in Table 4. Of the 31 patients who underwent PE, the response rate was 65% (n = 20) in ≥14 months of follow-up duration. CR and PR rates with PE were 27% (n = 8) and 37% (n = 11) in 30 non-hemodialysis patients, respectively. Of the 61 patients who underwent LDL-A, the response rate was 54% (n = 33) but it was primarily reported with a short follow-up period (≤4 months). Only 1 case series study followed 2 years after LDL-A therapy, and the long-term response rate was 43% (n = 12/28). CR and PR rates with LDL-A were 41% (n = 12) and 3% (n = 1) in 29 non-hemodialysis patients, respectively. A significant reduction of LDL and total cholesterol was also reported after LDL-A therapy [27,30,34,35]. Of the 10 patients with IA, the response rate (all presenting with PR) was 40% (n = 4) with 6 months of follow-up. Only one study, consisting of 6 patients (2 minimal change disease (MCD), 2 FSGS, 1 membranous nephropathy (MN), and 1 MN and FSGS), reported the efficacy of LCAP in primary kidney disease. LCAP was performed twice in two consecutive weeks and then followed with corticosteroid therapy with or without cyclosporine [36]. Of the 2 patients with FSGS, one achieved complete remission, and the other one did not respond and progressed to renal failure at the end of follow-up (47 and 40 months, respectively). The patients with MN and FSGS obtained PR, another 2 patients with MCD achieved CR, and the patient with MN died from pneumonia after LCAP therapy. T cells (especially activated T cells) decreased significantly after LCAP therapy in the response group [36].
Related Knowledge Centers
- Albumin
- Edema
- Hypoalbuminemia
- Kidney Failure
- Nephrotic Syndrome
- Protein
- Proteinuria
- Kidney Disease
- Incidence
- Signs & Symptoms