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Diabetic Nephropathy
Published in Jahangir Moini, Matthew Adams, Anthony LoGalbo, Complications of Diabetes Mellitus, 2022
Jahangir Moini, Matthew Adams, Anthony LoGalbo
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.
Respiratory, endocrine, cardiac, and renal topics
Published in Evelyne Jacqz-Aigrain, Imti Choonara, Paediatric Clinical Pharmacology, 2021
Evelyne Jacqz-Aigrain, Imti Choonara
Nephrotic syndrome develops when an abnormality of glomerular permeability results in the development of heavy proteinuria, hypoalbuminaemia and generalised oedema. Large studies in the 1970’s reported that 80% of cases of childhood nephrotic syndrome are attributable to minimal change disease (MCD), so called because renal biopsy shows no significant abnormality at light microscopy level. This disorder is more common in males than females (ratio 3:2) and is predominantly a disease of younger children with a median age at presentation of 3 years. The incidence of MCD, in the UK, is around 2–4 per 100,000 children in the Caucasian population, but is approximately six times higher in children of Asian origin [1]. MCD is associated with response to oral steroid therapy in over 90% of cases and is usually associated with a favourable long-term outcome, although at least 70% of patients will experience a chronic, relapsing-remitting course [2, 3].
Nephrology
Published in John D Firth, Professor Ian Gilmore, MRCP Part 1 Self-Assessment, 2017
John D Firth, Professor Ian Gilmore
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.
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].
Spectrum of renal disease in HIV-infected children: report of five cases
Published in Paediatrics and International Child Health, 2018
Karalanglin Tiewsoh, Ankur Kumar Jindal, Dhrubajyoti Sharma, Sunil Arora, Ranjana W. Minz, Parimal Agrawal, Ritambhra Nada, Deepti Suri
Laboratory investigations. Urine protein was 43 mg/m2/hr, renal function was normal and there was a high HIV viral load (Table 1). Kidney biopsy demonstrated minimal change disease. In view of clinical and virological failure, the cART regimen was changed to abacavir, lamivudine and lopinavir/ritonavir but she continued to have proteinuria. Five months later, she developed fever, cough and respiratory distress. Contrast-enhanced computed tomography (CECT) of the chest demonstrated enlarged mediastinal lymph nodes, and lymph node biopsy confirmed high-grade non-Hodgkin lymphoma (NHL). Her parents refused further therapy and she was lost to follow-up.