Duodenal neuroendocrine tumour associated with minimal change glomerulonephritis

Paraneoplastic glomerular disease is an increasingly well-recognised entity, and a wide range of both solid and haematological malignancies have been implicated. The most common glomerular disease associated with cancer is membranous nephropathy. Only a few case reports have described an association between neuroendocrine tumours (NETs) and glomerulonephritis and only one paediatric case in relation to minimal change disease. A 76-year-old woman with a well-differentiated duodenal NET presented with nephrotic syndrome and renal biopsy was suggestive of minimal change glomerulonephritis. Standard therapy with corticosteroids brought little benefit, but a dramatic improvement was seen following initiation of systemic anticancer therapy with lanreotide, a somatostatin analogue. Less than 1 month after initiation of lanreotide, the patient was no longer in a nephrotic state, and after a further 2 months of follow-up had shown no sign of relapse.

Rituximab Use in Nephrotic Syndrome

Background: Nephrotic syndrome may be caused by primary (idiopathic) renal disease or by a variety of secondary causes. Patients present with marked edema, proteinuria, hypoalbuminemia, and often hyperlipidemia. Treatment of most patients should include fluid and sodium restriction, oral or intravenous diuretics, and angiotensin-converting enzyme inhibitors. Adults with nephrotic syndrome may benefit from corticosteroid treatment. The treatment of patients with the steroid-resistant nephrotic syndrome (SRNS) and steroid-dependent nephrotic syndrome (SDNS) is challenging. On the basis of suggestions that B lymphocytes are crucial in the pathogenesis of the nephrotic syndrome, rituximab (a monoclonal antibody against CD20 antigen) is used in treatment of these patients. Aim of study: To evaluate the role of rituximaband mycophenolic acid in treatment of patientswith steroid-resistant (SRNS) and steroid-dependent nephrotic syndrome (SDNS), whom not respond or relapse after calcineurin inhibitor (CNI) (tacrolimus or cyclosporine) had been used. Patients and methods: Case series study was done between 2012 - 2015 in AL-Sadder Teaching Hospital Nephrology Center and record 40 patients with different age groups, males and females with different histopathological types (Minimal Change Glomerulonephritis, Focal Segmental Glomerulosclerosis, Mesengeo Prolifrative Glomerulonephritis). These patients were taking prednisilone and\or calcineurin inhibitor (tacrolimus “prograf”) or (cyclosporine “sandimmune”), and they get either Steroid Dependent Nephrotic Syndrome or Steroid Resistant Nephrotic Syndrome with frequent admission more than four time per year. To these patients we start rituximab intravenous infusion monthly for at least six months with the use of steroid and mycophenolate mofetil during these six months. The patients followed up for 3-12 months after initiation of rituximab by different investigations and the patients were classified according to their response into complete, partial and no response. After one year stop rituximab treatment, follow the patients clinically and by investigations for (1-2) years to determine which patients get relapse. Results: Majority (80%) of patients with nephrotic syndrome who had good response to rituximab were younger age group < 15 years. Better response to rituximab associated with Minimal Change Glomerulonephritis. There was significant reduction in blood urea, serum creatinine, urine (protein/creatinine) ratio and serum cholesterol. Serum albumin was significant elevated. Response to rituximab was not significantly associated with gender or steroid response. Majority of patients with good response not relapse and need more time for follow up. Relapsing after stopping rituximab not significantly associated with age, gender, histopathological type and steroid response. Conclusion: Rituximab and mycophenolate mofetil used in steroid-resistant nephrotic syndrome to get ride from side effects of calcineurine inhibitor (tacrolimus or cyclosporine). Rituximab and mycophenolate mofetil used in steroid-dependent nephrotic syndrome after calcineurine inhibitorto get ride from side effects of steroid. Improvement in renal function is result from stopping of calcineurine inhibitor (nephrotoxic drugs) and/or from rituximab and mycophenolate mofetil. Cost of rituximab is less than the cost that needed if the patients had frequent admissions to the hospital or developed renal failure and ended with dialysis.

Steroid Induced Bilateral Femoral Head Avascular Necrosis in a Patient of Minimal Change Glomerulonephritis: Detection by Radionuclide Bone Scan

One of the commonest causes of avascular necrosis of bones in patients with renal transplants are glucocorticoids. Early detection and management of this complication is crucial to the progression and prevention of collapse of femoral heads. In the early diagnosis of avascular necrosis radionuclide bone scintigraphy provides an useful information on the aetiology of the painful hip before changes appear on X ray examination. We present a case of proved glomerulonephritis taking glucocorticoids with difficulty in walking and pain around hip joints. A planar whole body and a SPECT Technetium 99-m (Tc99-m) bone scans were done which revealed avascular necrosis of both femoral heads. JMS 2012;15(1):61-64.

Das nephrotische Syndrom im Kindesalter

ZusammenfassungDas nephrotische Syndrom (NS) ist eine seltene, aber wichtige Nierenerkrankung im Kindesalter. Es ist definiert durch eine massive Proteinurie (>1 g/m²/Tag), die zur Hypoalbuminämie (<25 g/l) und Ödemen führt. Im ersten Lebensjahr liegen meist genetisch bedingte oder syndromale Störungen vor. Die häufigste Ursache bei Kindern jenseits des ersten Lebensjahres ist das idiopathische nephrotische Syndrom, welches bei 90 % die Minimal-Change-Glomerulonephritis (MCNS) und bei etwa 10 % die fokale segmentale Glomerulosklerose (FSGS) umfasst. Die MCNS ist in der Regel steroidsensibel, allerdings kann der Verlauf durch häufige Rezidive kompliziert werden, sodass eine erweiterte und oftmals lang andauernde immunsuppressive Therapie erforderlich werden kann. Hier sind als Therapieoptionen Levamisol, Cyclophosphamid, und Cyclosporin A zu nennen (Stufenschema). Neuere Behandlungsmöglichkeiten umfassen die Mycophenolsäure oder den monoklonalen Antikörper Rituximab, der bei besonders therapierefraktären Patienten eingesetzt wird. Bei Steroidresistenz und FSGS spielt die Behandlung mit Cyclosporin A eine entscheidende Rolle und hat die Prognose in den letzten Jahren deutlich verbessert. Bei den genetisch bedingten Formen ist bislang keine kausale Therapie möglich, hier sind insbesondere Maßnahmen zur Reduktion der Proteinurie (z. B. ACE-Inhibitoren) sinnvoll. Zusammenfassend ist das Spektrum nephrotischer Erkrankungen eine klinische Herausforderung. Fortschritte sowohl der Grundlagenforschung als auch der klinischen Forschung haben sich jedoch positiv auf die Prognose niedergeschlagen.