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Submitted by Dulce Barreto (mdulce.barreto@chlc.min-saude.pt) on 2018-12-04T13:46:17Z No. of bitstreams: 1 Port J Nephrol Hypert 2018_32_85.pdf: 866308 bytes, checksum: de61365b3b783c44458827f629d828b1 (MD5) Made available in DSpace on 2018-12-04T13:46:17Z (GMT). No. of bitstreams: 1 Port J Nephrol Hypert 2018_32_85.pdf: 866308 bytes, checksum: de61365b3b783c44458827f629d828b1 (MD5) Previous issue date: 2018 info:eu-repo/semantics/publishedVersion

Submitted by Dulce Barreto (mdulce.barreto@chlc.min-saude.pt) on 2018-12-04T11:07:27Z No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_153.pdf: 416867 bytes, checksum: f627b9e4565a45efd90523260ed3206b (MD5) Made available in DSpace on 2018-12-04T11:07:27Z (GMT). No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_153.pdf: 416867 bytes, checksum: f627b9e4565a45efd90523260ed3206b (MD5) Previous issue date: 2016 info:eu-repo/semantics/publishedVersion

Oxalate Nephropathy is characterised by the presence of tubular crystalline deposits of calcium oxalate, which can lead to both acute and chronic tubular injury and progressive renal failure. Enteric hyperoxaluria is the most common cause of moderate hyperoxaluria; it occurs in conditions associated with fat or bile acid malabsorption, which include jejunoileal bypass and other bariatric procedures such as Roux‑en‑Y gastric bypass surgery. We present the clinical case of a 69‑year‑old man who was hospitalised for non‑oliguric renal dysfunction, with a serum creatinine of 10 mg/dl and normocytic normochromic anaemia. There was no prior history of renal disease. Twenty months before admission the patient was diagnosed with a gastro‑oesophageal junction adenocarcinoma and was treated with pre‑operative chemotherapy, followed by total gastrectomy, with a Roux‑en‑Y gastric bypass reconstruction. On discharge from gastric surgery, renal function was normal. On the first day of hospital stay haemodialysis was initiated. Over the following days, the rapid unexplained renal impairment was investigated, and this workup [2] included a kidney biopsy. Histological examination of the biopsy specimen revealed a predominantly interstitial nephropathy with tubular atrophy and interstitial fibrosis, with bright intra‑tubular calcium oxalate crystals in over 50% of the tubules and so the histological diagnosis was of oxalate nephropathy. Subsequently, no recovery of renal function was observed, so the patient is currently undergoing regular haemodialysis. Oxalate nephropathy is a rare but severe complication of Roux‑en‑Y gastric bypass surgery that can lead to a rapid progression to kidney failure. Although the treatment of obesity is the main indication for this surgery, this is also the preferred approach for gastrointestinal reconstruction after total gastrectomy for treatment of gastric carcinoma. Considering the rapid progression of oxalate nephropathy to kidney failure, patients who undergo Roux‑en‑Y gastric bypass surgery should have regular follow‑up of renal function. info:eu-repo/semantics/publishedVersion

Submitted by Dulce Barreto (mdulce.barreto@chlc.min-saude.pt) on 2018-12-04T11:01:34Z No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_76.pdf: 620795 bytes, checksum: 5f01e51b3bd734e0c32c4399063472dc (MD5) Made available in DSpace on 2018-12-04T11:01:34Z (GMT). No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_76.pdf: 620795 bytes, checksum: 5f01e51b3bd734e0c32c4399063472dc (MD5) Previous issue date: 2016 info:eu-repo/semantics/publishedVersion

Submitted by Dulce Barreto (mdulce.barreto@chlc.min-saude.pt) on 2018-12-04T11:54:41Z No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_228.pdf: 1105259 bytes, checksum: c5079ffc0922dfaca191cf901918eacd (MD5) Made available in DSpace on 2018-12-04T11:54:41Z (GMT). No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_228.pdf: 1105259 bytes, checksum: c5079ffc0922dfaca191cf901918eacd (MD5) Previous issue date: 2016 info:eu-repo/semantics/publishedVersion

Myeloma-associated renal disorders are rare events among renal transplants and can occur as recurrent or de novo disease. We describe an unusual case of renal allograft dysfunction due to myeloma cast nephropathy occurring 19 years after having received a renal transplant in a patient with no prior history of multiple myeloma or monoclonal gammopathy preceding transplantation. Our patient was treated with five cycles of chemotheraphy(bortezomib, melphalan and steroids), which resulted in short-term improvement in allograft dysfunction and complete haematological remission. The longer patient and graft survival after renal transplantation make post-transplant lymphoproliferative disease more frequent. Multiple myeloma after kidney transplant is rare and an elevated index of suspicion is necessary to make a timely diagnosis of this serious disease. Further work is needed to identify the best treatment options for these patients. info:eu-repo/semantics/publishedVersion

Background: Tubulointerstitial nephritis (TIN) is a common cause of kidney injury typically seen in association with drug exposure, infection or autoimmune diseases. However, TIN with interstitial immune complex deposition, without glomerular injury, is rarely observed. Case: We report a case of a 64-yearold Indian woman admitted for dialysis-requiring renal failure, without involvement of other organs. Urinalysis showed blood 3+ and 24h proteinuria of 1.5 g. Renal ultrasound revealed normal sized kidneys with loss of parenchymal-sinus differentiation. Laboratory tests disclosed low C3, positive ANA but negative anti-dsDNA, SSA and SSB. Serum protein electrophoresis was normal. The renal biopsy showed tubulointerstitial nephritis with positive immunoglobulin staining involving the interstitium and tubular basement membrane with glomerular sparing. The patient started prednisolone (1mg/kg/day) without recovery of the renal function. Conclusion: Idiopathic hypocomplementaemic tubulointerstitial nephritis is a rare disease with few cases described in the literature. To our knowledge this is the first case reported in Portugal A nefrite tubulo-intersticial (NTI) é uma causa comum de lesão renal, tipicamente associada a toma de fármacos, infecções ou doenças auto-imunes. No entanto, as NTI com deposição de imuno-complexos no interstício, sem envolvimento glomerular são raramente observadas. Apresentámos o caso de uma doente do sexo feminino, 64 anos, etnia indiana, admitida no nosso hospital, por insuficiência renal dependente de diálise. A análise sumária de urina revelou hematúria (3+) e a proteinúria nas 24h foi quantificada em 1,5g. A ecografia renal revelou rins de dimensões normais com perda da diferenciação parenquimo-sinusal. Analiticamente salienta-se, C3 baixo, ANA positivo, mas anticorpo anti dsDNA, SSA e SSB negativo. A electroforese de proteínas séricas não apresentava alterações. O doseamento de imunoglobulinas era normal, sem evidência de componente monoclonal. O restante estudo imunológico não apresentou alterações. Durante o internamento a doente realizou uma biópsia renal que mostrou nefrite tubulo-intersticial, com depósito de imunoglobulinas envolvendo o interstício e a membrana basal tubular, poupando os glomérulos. A doente iniciou terapêutica com prednisolona (1mg/Kg/dia), sem recuperação da função renal. A nefrite tubulo-intersticial hipocomplementémica idiopática é uma doença rara, com poucos casos descritos na literatura. Tanto quanto sabemos, este é o primeiro caso descrito em Portugal

Background: Tubulointerstitial nephritis (TIN) is a common cause of kidney injury typically seen in association with drug exposure, infection or autoimmune diseases. However, TIN with interstitial immune complex deposition, without glomerular injury, is rarely observed. Case: We report a case of a 64-yearold Indian woman admitted for dialysis-requiring renal failure, without involvement of other organs. Urinalysis showed blood 3+ and 24h proteinuria of 1.5 g. Renal ultrasound revealed normal sized kidneys with loss of parenchymal-sinus differentiation. Laboratory tests disclosed low C3, positive ANA but negative anti-dsDNA, SSA and SSB. Serum protein electrophoresis was normal. The renal biopsy showed tubulointerstitial nephritis with positive immunoglobulin staining involving the interstitium and tubular basement membrane with glomerular sparing. The patient started prednisolone (1mg/kg/day) without recovery of the renal function. Conclusion: Idiopathic hypocomplementaemic tubulointerstitial nephritis is a rare disease with few cases described in the literature. To our knowledge this is the first case reported in Portugal.

Circulating anti-phospholipase A2 receptor antibodies (anti-PLA2R) have been described in 70% to 80% of the patients with idiopathic membranous nephropathy (iMN), but not in patients with secondary membranous nephropathy or other glomerular diseases. The goal of this study was to evaluate the sensitivity and specificity of the assay for anti-PLA2R in the diagnosis of iMN. Anti-PLA2R IgG, Elisa and immunofluorescence tests were used to detect circulating anti-PLA2R. These tests were applied in 53 patients who had a kidney biopsy. Of these, 38 had histological diagnosis of membranous nephropathy (MN) and the remaining had other glomerular diseases. The MN was classified as idiopathic in 33 patients after clinical exclusion of secondary causes. Anti-PLA2R were positive in 57.6% of the patients with iMN. All patients with secondary membranous nephropathy or other glomerular diseases did not show circulating anti-PLA2R. The sensitivity was 57.6% (CI 39.2-74.5) and specificity 100% (CI 47.8-100), AUC 0.788; p < 0.0001 for the detection of iMN. 71.4% of the iMN patients that tested negative for anti-PLA2R were in partial or complete remission. The detection of anti-PLA2R in the studied population had a specificity of 100% for the iMN diagnosis. Prior treatments seem to make the test negative and contribute to a lower sensitivity.

Introduction: The Henoch-Schönlein purpura (HSP) is an immunoglobulin A (IgA)-mediated smallvessel systemic vasculitis, rare in adults. The association with solid tumours has been described, especially with lung cancer. Case Report: We present the case of a 60-year-old Caucasian male, diagnosed with lung adenocarcinoma that underwent surgical resection without (neo)adjuvant theraphy. Two months latter he was admitted for abdominal pain, purpuric rash on his lower extremities and acute kidney injury, with serum creatinine (Scr) of 2 mg/dl. Urinalysis revealed haematuria and 24h proteinuria (P24h) of 1.5 g. The serum protein electrophoresis, complement components C3 and C4, circulating immune complexes, cryoglobulins, ANCA, ANA, anti-dsDNA and the remaining immunologic study as screening for viral infections (HCV, HBV and HIV) were negative. Renal ultrasound was normal and kidney biopsy revealed mild mesangial proliferation; 2 cellular glomerular crescents and 1 fibrinoid necrosis lesion; large amounts of red blood cell casts; lymphocytic infiltration in the intertubular interstitial capillaries; moderate arteriolar hyalinosis. Immunofluorescence demonstrated mesangial and parietal deposits of IgA. The diagnosis of HSP was assumed, and the patient started prednisolone 1 mg/kg/day. Ten months after diagnosis the patient’s baseline Scr is 1.4 mg/dl with P24h of 0.18g, without haematuria. Conclusion: Although this is a rare association and the exact mechanism behind the disease is yet unknown, physicians should be aware of it. The early recognition and treatment may prevent renal disease progression.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2016-03-09T15:36:09Z No. of bitstreams: 1 RPNH 2015 248.pdf: 450396 bytes, checksum: e9826b6a897ebdcffbfcc9d99b5ddb9b (MD5) Made available in DSpace on 2016-03-09T15:36:09Z (GMT). No. of bitstreams: 1 RPNH 2015 248.pdf: 450396 bytes, checksum: e9826b6a897ebdcffbfcc9d99b5ddb9b (MD5) Previous issue date: 2015

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2016-01-26T16:41:58Z No. of bitstreams: 1 RPNH 2015 173.pdf: 336979 bytes, checksum: 14bc5d994cbaf22c5feb5647fe3c5a47 (MD5) Made available in DSpace on 2016-01-26T16:41:58Z (GMT). No. of bitstreams: 1 RPNH 2015 173.pdf: 336979 bytes, checksum: 14bc5d994cbaf22c5feb5647fe3c5a47 (MD5) Previous issue date: 2015

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2015-04-29T16:18:35Z No. of bitstreams: 1 RPNH 2014 353.pdf: 1830201 bytes, checksum: f82c77d74a5615cdd8590964181c68ed (MD5) Made available in DSpace on 2015-04-29T16:18:35Z (GMT). No. of bitstreams: 1 RPNH 2014 353.pdf: 1830201 bytes, checksum: f82c77d74a5615cdd8590964181c68ed (MD5) Previous issue date: 2014

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2015-04-29T09:55:44Z No. of bitstreams: 1 RPNH 2014 168.pdf: 330492 bytes, checksum: a8e3272656187900d032746b1e6c1c79 (MD5) Made available in DSpace on 2015-04-29T09:55:44Z (GMT). No. of bitstreams: 1 RPNH 2014 168.pdf: 330492 bytes, checksum: a8e3272656187900d032746b1e6c1c79 (MD5) Previous issue date: 2014

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2015-04-29T16:10:29Z No. of bitstreams: 1 RPNH 2014 84.pdf: 2726889 bytes, checksum: 92751fc2b3125c82d5ff0ee097e441d3 (MD5) Made available in DSpace on 2015-04-29T16:10:29Z (GMT). No. of bitstreams: 1 RPNH 2014 84.pdf: 2726889 bytes, checksum: 92751fc2b3125c82d5ff0ee097e441d3 (MD5) Previous issue date: 2014

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2015-04-29T16:33:22Z No. of bitstreams: 1 RPNH 2013 309.pdf: 371116 bytes, checksum: f1726fd9aa6318de3426a29ce8b0893d (MD5) Made available in DSpace on 2015-04-29T16:33:22Z (GMT). No. of bitstreams: 1 RPNH 2013 309.pdf: 371116 bytes, checksum: f1726fd9aa6318de3426a29ce8b0893d (MD5) Previous issue date: 2013

Background: In the haemodynamically unstable patient the method of treatment of acute renal failure is still largely controversial. The purpose of our study was to compare slow extended dialysis with continuous haemodiafiltration in the critical patient with indication for renal replacement therapy and haemodynamic instability. Patients and Methods: This is a cohort study comparing in 63 ventilated critical patients a 12 month period when only continuous haemodiafiltration was used (n=25) with an equal period of slow extended dialysis (n=38). Our primary objective was to evaluate the impact of the dialytic procedure on cardiovascular stability in those patients. As secondary aims we considered system coagulation/thrombosis and predictors of mortality. In the two groups we analysed the first session performed, the second session performed and the average of all the sessions performed in each patient. Results: In these patients, mortality in the intensive care unit was high (68% in the continuous haemodiafiltration group and 63% in the slow extended dialysis group). We did not find any association between the dialytic technique used and death; only the APACHE score was a predictor of death. Slow extended dialysis was a predictor of haemodynamic stability, a negative predictor of sessions that had to be interrupted for haemodynamic instability, and a predictor of achieving the volume removal initially sought. Slow extended dialysis was also associated with less coagulation of the system. Conclusions: Our data suggested that slow extended dialysis use was not inferior to continuous haemodiafiltration use in terms of cardiovascular tolerability.

Introduction: The clinical importance of humoral-mediated acute rejection has been progressively recognised. Early recognition and treatment with plasmapheresis and intravenous immunoglobulin have recently improved short term prognosis. Case report: In this report we describe the clinical features of three 2nd transplant patients developing severe acute humoral rejection during the first week post-transplant while on anti-thymocyte globulin therapy. Treatment with plasmapheresis/ intravenous immunoglobulin/rituximab resulted in rapid reversal of oliguria,and recovery of renal function within the 1st week of treatment in 2/3 patients. Diagnosis was confirmed by graft biopsies revealing peritubular neutrophiles and C4d deposits. Sequential graft biopsies in all three patients revealed complete histological recovery within two weeks. One patient never recovered renal function, and one patient lost his graft at three months following hemorrhagic shock. After 2 years follow up, the remaining patient maintains a serum creatinine of 1.1mg/dl. Conclusion: The regimen using plasmapheresis plus intravenous immunoglobulin and rituximab was effective in rapidly reversing severe acute humoral rejection.