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Kidney and urinary tract development disorder are two of the most prevalent congenital malformations and the main cause of chronic kidney disease in pediatric age patients. As such, it is very important that the neph‑ rologist understands these pathologies to improve transition and ensure a good continuity between pediatric and adult nephrological care. The purpose of this article is to present a brief review of congenital anomalies of the kidney and urinary tract (CAKUT). Kidney malformations are classified according to macroscopic and microscopic anatomic features, and are the result of the following abnormal renal developmental processes: malformations of the renal parenchyma, abnormalities of the embryonic migration of the kidneys and abnormalities of the developing urinary collecting system. info:eu-repo/semantics/publishedVersion

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 Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2021-09-08T12:02:01Z No. of bitstreams: 1 Port J Nephrol Hypert 2018_389.pdf: 667433 bytes, checksum: 92f7e8602f492f364fe7fd1354e22766 (MD5) Made available in DSpace on 2021-09-08T12:02:01Z (GMT). No. of bitstreams: 1 Port J Nephrol Hypert 2018_389.pdf: 667433 bytes, checksum: 92f7e8602f492f364fe7fd1354e22766 (MD5) Previous issue date: 2018 info:eu-repo/semantics/publishedVersion

Background: Rheumatoid arthritis (RA) is a systemic inflammatory disorder characterized by joint inflammation, associated with autoantibody production. Renal involvement arises as a complication of treatment or can be related to the disease itself. Methods: 53 biopsies from patients with RA from 1989 to 2015 were reviewed. Histologic diagnosis, age, gender, duration of RA, drug therapy, renal function, proteinuria and haematuria were analyzed. Results: Amyloidosis was the most common renal histologic pattern (21 patients). Membranous Nephropathy (MN) was found in 12 patients, followed by Mesangial Proliferative Glomerulonephritis (n=4) and Focal and Segmental Glomerulosclerosis (n=4), IgA Nephropathy (n=3), Necrotizing Glomerulonephritis (n=3), Chronic Interstitial Nephritis (n=3), Endocapillary Proliferative Glomerulonephritis (n=2) and Minimal Change Disease (n=1). Amyloidosis correlated with long duration RA (14.9±6.66 years vs 8.84±6.37 years; p

none 24 si In a 24-month, multicenter, open-label, randomized trial, 715 de novo kidney transplant recipients were randomized at 10-14 weeks to convert to everolimus (n = 359) or remain on standard calcineurin inhibitor (CNI) therapy (n = 356; 231 tacrolimus; 125 cyclosporine), all with mycophenolic acid and steroids. The primary endpoint, change in estimated glomerular filtration rate (eGFR) from randomization to month 12, was similar for everolimus versus CNI: mean (standard error) 0.3(1.5) mL/min/1.732 versus -1.5(1.5) mL/min/1.732 (p = 0.116). Biopsy-proven acute rejection (BPAR) at month 12 was more frequent under everolimus versus CNI overall (9.7% vs. 4.8%, p = 0.014) and versus tacrolimus-treated patients (2.6%, p < 0.001) but similar to cyclosporine-treated patients (8.8%, p = 0.755). Reporting on de novo donor-specific antibodies (DSA) was limited but suggested more frequent anti-HLA Class I DSA under everolimus. Change in left ventricular mass index was similar. Discontinuation due to adverse events was more frequent with everolimus (23.6%) versus CNI (8.4%). In conclusion, conversion to everolimus at 10-14 weeks posttransplant was associated with renal function similar to that with standard therapy overall. Rates of BPAR were low in all groups, but lower with tacrolimus than everolimus open de Fijter JW, Holdaas H, Øyen O, Sanders JS, Sundar S, Bemelman FJ, Sommerer C, Pascual J, Avihingsanon Y, Pongskul C, Oppenheimer F, Toselli L, Russ G, Wang Z, Lopez P, Kochuparampil J, Cruzado JM, van der Giet M; ELEVATE Study Group: [...; Guido Garosi; Paolo Rigotti; Giuseppe Tisone; Paola Todeschini; ...] de Fijter JW, Holdaas H, Øyen O, Sanders JS, Sundar S, Bemelman FJ, Sommerer C, Pascual J, Avihingsanon Y, Pongskul C, Oppenheimer F, Toselli L, Russ G, Wang Z, Lopez P, Kochuparampil J, Cruzado JM, van der Giet M; ELEVATE Study Group: [..; Guido Garosi; Paolo Rigotti; Giuseppe Tisone; Paola Todeschini; ..]

Submitted by Dulce Barreto (mdulce.barreto@chlc.min-saude.pt) on 2018-12-04T12:23:07Z No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_239.pdf: 2246548 bytes, checksum: aa0bf757455735d40ae58520adaca94b (MD5) Made available in DSpace on 2018-12-04T12:23:07Z (GMT). No. of bitstreams: 1 Port J Nephrol Hypert 2016_30_239.pdf: 2246548 bytes, checksum: aa0bf757455735d40ae58520adaca94b (MD5) Previous issue date: 2016 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

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2021-03-10T16:16:04Z No. of bitstreams: 1 AJT 2016.pdf: 182376 bytes, checksum: 39db2b830e5e396350eb70455ea58502 (MD5) Made available in DSpace on 2021-03-10T16:16:04Z (GMT). No. of bitstreams: 1 AJT 2016.pdf: 182376 bytes, checksum: 39db2b830e5e396350eb70455ea58502 (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

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.

Severe chronic kidney disease may lead to disturbances, such as hyperphosphatemia, increased secretion of fibroblast growth factor -23 (FGF -23) and vitamin D deficiency. These may increase plasmatic levels of parathyroid hormone, and decrease plasmatic levels of calcium. Altogether, these may contribute to the development of secondary hyperparathyroidism, and to abnormalities in mineral metabolism. Kidney transplantation is the best option to improve longevity and quality of life in end -stage chronic kidney disease patients. Vitamin D deficiency has been associated with cardiovascular disease, which is the leading cause of death in chronic kidney disease. Therefore, diagnosing this deficiency may be pivotal for minimizing mortality in chronic kidney disease, because pharmacological treatments for this deficiency may be prescribed. Calcitriol is indicated for the treatment of vitamin D deficiency, both in chronic kidney disease and in kidney transplanted patients. However, calcitriol may increase the plasmatic levels of calcium and phosphorous, which can lead to vascular calcifications, that have been associated with cardiovascular mortality. Selective vitamin D receptor activators are indicated for the treatment of vitamin D deficiency in chronic kidney disease. These have the advantage of being associated with lower increases of plasmatic levels of calcium and phosphorous. These drugs also seem to have additional effects that may minimise patient morbidity and mortality, especially due to potentially reducing cardiovascular events. Unfortunately, there are few studies about the use of these drugs in kidney transplanted patients. Here we present a review about the physiology of vitamin D, the consequences of its deficiency in chronic kidney disease and in kidney transplanted patients, and about the diagnosis and treatment of this deficiency. Finally, we discuss the new line of research about the efficacy and safety of selective vitamin D receptor activators in kidney transplanted patients.

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

Calciphylaxis is a rare and devastating obliterative vasculopathy, leading to ischemia and subcutaneous necrosis. In most cases it affects patients with renal disease and is associated with high morbidity and mortality. We present two case reports followed recently in our department, and a literature review on this topic. Case one refers to an 80 -year -old Caucasian woman with chronic kidney disease stage 5 and primary hyperparathyroidism with secondary brown tumour and calciphylaxis. Case two refers to a 59 -year -old Caucasian woman admitted with severe nephrotic syndrome associated with amyloidosis, that developed a catastrophic picture of calciphylaxis, ending in the patient’s death. There is a critical need to understand the pathogenesis of calciphylaxis. Its comprehension is the only way to improve the survival of these patients, and may help to elucidate the pathophysiology of vascular calcification in general. Educating physicians in the prevention and early detection of calciphylaxis is crucial. Only by increasing the knowledge about risk factors, pathophysiology, response to treatment and outcome, will we be able to improve prophylaxis and therapy of patients with calciphylaxis, decreasing the high mortality of this entity.

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

Background: Economic evaluations help health authorities facing budget constraints. This study compares the health-related quality of life (HRQOL) and costs in patient subgroups on haemodialysis (HD) and renal transplantation (KT). Methods: In a prospective study with follow-up of 1-3 years, we performed a costutility analysis of KT vs. HD, adopting a lifetime horizon. A societal perspective was taken. Costs for organ procurement, KT eligibility, transplant surgery and follow-up of living donors were included. Key clinical events were recorded. HRQOL was assessed using the EuroQol instrument. Results: The HRQOL remained stable on HD patients. After KT, mean utility score improved at 3 months while mean EQ-VAS scores showed a sustained improvement. Mean annual cost for HD was 32,567.57€. Mean annual costs for KT in the year-1 and in subsequent years were, 60,210.09€ and 12,956.77€ respectively. Cost for initial hospitalization averaged 18,740.74€. HLA-mismatches increased costs by 75% for initial hospitalization (p < 0.001) and 41% in the year-1 (p < 0.05), and duplicate the risk of readmission in the year-1 (p < 0.05). The incremental costutility ratio was 5,534.46€/QALY, increasing 35% when costs for organ procurement were added. KT costs were 41,541.63€ more but provided additional 7.51 QALY. Conclusions: The KT is cost-effective compared with HD. Public funding should reflect the value created by the intervention and adapt to the organ demand.

Introdução: Foram descritas várias doenças cutâneas em doentes transplantados, em relação com a terapêutica imunossupressora instituída. Pretendemos caracterizar o espectro clínico das patologias dermatológicas e comparar os diagnósticos entre os doentes transplantados hepáticos e os doentes transplantados renais. Material e Métodos: Estudo descritivo e retrospectivo através da consulta de processos clínicos de todos os doentes submetidos a transplante hepático ou renal entre 2000 - 2010 referenciados à Consulta de Dermatologia e Venereologia. Resultados: Observámos 319 doentes transplantados (23,5%) e apurámos 410 diagnósticos (230 na sub-população com transplante hepático e 180 na sub-população com transplante renal), divididos em quatro grupos: 1) infecções cutâneas; 2) cancro cutâneo ou lesões precursoras; 3) manifestações cutâneas relacionadas com efeitos secundários de fármacos; 4) outras patologias dermatológicas não iatrogénicas. As infecções cutâneas foram as mais observadas (42,2%), em média 32,7 meses após o transplante. Este grupo incluiu 20,5% de infecções fúngicas, 12,7% virais e 8,5% bacterianas. Identificámos patologia tumoral e lesões precursoras em 11,7% dos casos, em média 44,8 meses após o transplante e assumindo maior importância na sub-população com transplante renal (20,6% vs 4,8% nos transplantados hepáticos; P < 0,001). Os transplantados renais apresentaram predomínio de carcinomas espinocelulares (CEC) sobre os casos de carcinomas basocelulares (CBC), numa razão CEC:CBC de 1,3:1 mas nos transplantados hepáticos verificou-se uma razão CBC: carcinomas de 3,5:1. Ocorreram efeitos secundários de fármacos em 10,5% dos casos e outras patologias dermatológicas não iatrogénicas em 35,6%. Discussão: Apesar da patologia tumoral ser a mais referida na literatura, as infecções cutâneas foram as mais observadas na nossa amostra. As diferenças significativas entre as duas sub-populações estudadas podem estar relacionadas com o maior grau de imunossupressão a que os doentes transplantados renais estão sujeitos. Conclusão: Dada a elevada frequência de patologia cutânea nestes doentes é essencial incluir o acesso a consultas de Dermatologia e Venereologia nos cuidados multi-disciplinares pós-transplante.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2013-10-16T11:57:22Z No. of bitstreams: 1 RPNH 2013 209.pdf: 512308 bytes, checksum: 35f0152a1d38e36d45e1002ba48daea9 (MD5) Made available in DSpace on 2013-10-16T11:57:22Z (GMT). No. of bitstreams: 1 RPNH 2013 209.pdf: 512308 bytes, checksum: 35f0152a1d38e36d45e1002ba48daea9 (MD5) Previous issue date: 2013

Background: Although vascular access is essential for adequate haemodialysis delivery, the systematic use of a patient's venous patrimony may eventually lead to exhaustion of suitable sites for placement of a new vascular access. Case Report: We present two cases of such patients. In the first one we inserted a 55cm catheter through the left external iliac vein, and a 40cm translumbar catheter was placed in the second one. Both interventions were performed percutaneously under radiological guidance. Both patients were anticoagulated after the procedure. Conclusion: Unusual sites for haemodialysis catheter placement may be life saving in selected situations and offer safe and viable alternatives for adequate haemodialysis delivery.

Familial renal glucosuria (FRG) is a rare co -dominantly inherited benign phenotype characterized by the presence of glucose in the urine. It is caused by mutations in the SLC5A2 gene that encodes SGLT2, a Na+ -glucose co -transporter. The purpose of our current work was twofold: to characterize the molecular and phenotype findings of an FRG cohort and, in addition, to detail the SGLT2 expression in the adult human kidney. The phenotype of FRG pedigrees was evaluated using direct sequencing for the identification of sequence variations in the SLC5A2 gene. The expression of SGLT2 in the adult human kidney was studied by immunofluorescence on kidney biopsy specimens. In the absence of renal biopsies from FRG individuals, and in order to evaluate the potential disruption of SGLT2 expression in a glucosuric nephropathy, we have selected cases of nucleoside analogues induced proximal tubular toxicity. We identified six novel SLC5A2 mutations in six FRG pedigrees and described the occurrence of hyperuricosuria associated with hypouricaemia in the two probands with the most severe phenotypes. Histopathological studies proved that SGLT2 is localized to the brush -border of the proximal tubular epithelia cell and that this normal pattern was found to be disrupted in cases of nucleoside analogues induced tubulopathy. We present six novel SLC5A2 mutations, further contributing to the allelic heterogeneity in FRG, and identified hyperuricosuria and hypouricaemia as part of the FRG phenotype. SGLT2 is localized to the brush -border of the proximal tubule in the adult human normal kidney, and aberrant expression of the co -transporter may underlie the glucosuria seen with the use of nucleoside analogues.

Introduction. Peritubular capillary complement 4d staining is one of the criteria for the diagnosis of antibody-mediated rejection, and research into this is essential to kidney allograft evaluation. The immunofluorescence technique applied to frozen sections is the present gold-standard method for complement 4d staining and is used routinely in our laboratory. The immunohistochemistry technique applied to paraffin-embedded tissue may be used when no frozen tissue is available. Material and Methods. The aim of this study is to evaluate the sensitivity and specificity of immunohistochemistry compared with immunofluorescence. We describe the advantages and disadvantages of the immunohistochemistry vs. the immunofluorescence technique. For this purpose complement 4d staining was performed retrospectively by the two methods in indication biopsies (n=143) and graded using the Banff 07 classification. Results. There was total classification agreement between methods in 87.4% (125/143) of cases. However, immunohistochemistry staining caused more difficulties in interpretation, due to nonspecific staining in tubular cells and surrounding interstitium. All cases negative by immunofluorescence were also negative by immunohistochemistry. The biopsies were classified as positive in 44.7% (64/143) of cases performed by immunofluorescence vs. 36.4% (52/143) performed by immunohistochemistry. Fewer biopsies were classified as positive diffuse in the immunohistochemistry group(25.1% vs. 31.4%) and more as positive focal (13.2% vs. 11.1%). More cases were classified as negative by immunohistochemistry (63.6% vs. 55.2%). Study by ROC curve showed immunohistochemistry has a specificity of 100% and a sensitivity of 81.2% in relation to immunofluorescence (AUC: 0.906; 95% confidence interval: 0.846-0.949; p=0.0001). Conclusions. The immunohistochemistry method presents an excellent specificity but lower sensitivity to C4d detection in allograft dysfunction. The evaluation is more difficult, requiring a more experienced observer than the immunofluorescence method. Based on these results, we conclude that the immunohistochemistry technique can safely be used when immunofluorescence is not available.

HIV-infected patients may be affected by a variety of renal disorders. Portugal has a high incidence of HIV2 infection and a low prevalence of HIV-infected patients under dialysis treatment. The aim of this study was to characterise the type of renal disease in Portuguese HIV-infected patients and to determine if HIV2 infection is associated to renal pathology. Only 60 of the 5158 HIV-infected patients followed in our hospital underwent renal biopsy. Clinical and laboratory data and the type of renal disease were reviewed. Male gender was predominant (76.7%), as was Caucasian race (78.3%). Mean age was 37.9±10.6 years. The majority had criteria for AIDS, 66% were on combined antiretroviral therapy and 18.3% were on dialysis. The predominant lesions were immunecomplex glomerulonephritis (n=19), tubulointerstitial nephropathy (n=12), focal segmental glomerulosclerosis(n=11), followed by HIVAN (n=8). Other patterns(amyloidosis, vasculitis, minimal change lesion) were observed. Only three patients were HIV2 infected, and presented diabetic nephropathy, acute tubular necrosis and tubulointerstitial nephritis. No correlations between clinical findings and renal pathology were found. In conclusion, renal disease in HIV patients has a broad spectrum, and renal biopsy remains the gold standard for establishing the diagnosis and guide treatment. Renal disease is not frequent in HIV2-infected patients, and, when present, is probably not directly associated with HIV infection.

Background: Acute kidney injury in the pandemic swine origin influenza A virus (H1N1) infection has been reported as coursing with severe illness, although renal pathogenic mechanisms and histologic features are still being characterised. Case Report: We present two patients admitted with H1N1 pneumonia, sepsis, acute respiratory distress syndrome and need for invasive mechanical ventilation who developed acute kidney injury and became dialysis-dependent. In both cases a kidney biopsy was performed to establish a definitive diagnosis. Severe acute tubular necrosis was identified, with no further abnormalities. Conclusion: This report seems to confirm that the acute kidney injury in H1N1 infection is focused on the tubular cells. Our cases corroborate the renal histopathologic findings of other studies, highlighting the central role of the tubular cell. We bring new evidence of the histopathology of AKI in H1N1 infection since our data were collected in living patients and not via post-mortem studies.

Renal dysfunction often complicates the course of orthotopic liver transplant recipients and is associated with increased morbid -mortality. The aims of this study were to determine the incidence of chronic renal disease and its impact on patient survival. Clinical data included age, gender and weight,aetiology of hepatic failure, presence of diabetes,hypertension, hepatitis B and C infection, renal dysfunction pretransplant and immunosuppression. Laboratory data included serum creatinine at days 1, 7, 21, month 6, 12 and yearly. The glomerular filtration rate was determined by Cockcroft-Gault equation. We studied retrospectively from September 1992 to March 2007 708 orthotopic liver transplant recipients. Mean age 44±12.6 years, 64% males, 17% diabetic, 18.8% hypertensive, 19.9% with hepatitis C and 3.8% hepatitis B. Renal dysfunction pretransplant was known in 21.6%. Mean follow-up was 3.6 years. Mean transplant survival 75% at 12 months. 154 patients died. Univariate and multivariate analyses were performed and a p

Acute renal failure (ARF) is common after orthotopic liver transplantation (OLT). The aim of this study was to evaluate the prognostic value of RIFLE classification in the development of CKD, hemodialysis requirement, and mortality. Patients were categorized as risk (R), injury (I) or failure (F) according to renal function at day 1, 7 and 21. Final renal function was classified according to K/DIGO guidelines. We studied 708 OLT recipients, transplanted between September 1992 and March 2007; mean age 44 +/- 12.6 yr, mean follow-up 3.6 yr (28.8% > or = 5 yr). Renal dysfunction before OLT was known in 21.6%. According to the RIFLE classification, ARF occurred in 33.2%: 16.8% were R class, 8.5% I class and 7.9% F class. CKD developed in 45.6%, with stages 4 or 5d in 11.3%. Mortality for R, I and F classes were, respectively, 10.9%, 13.3% and 39.3%. Severity of ARF correlated with development of CKD: stage 3 was associated with all classes of ARF, stages 4 and 5d only with severe ARF. Hemodialysis requirement (23%) and mortality were only correlated with the most severe form of ARF (F class). In conclusion, RIFLE classification is a useful tool to stratify the severity of early ARF providing a prognostic indicator for the risk of CKD occurrence and death.

Renal transplant in highly sensitised patients is associated with increased morbidity. The aim of this retrospective study was to evaluate the clinical evolution of 30 highly sensitised deceased donor kidney transplants and the influence of different timing of B cell directed treatment and its importance in the outcome of these patients. All recipients had negative complement dependent lymphocytotoxicity cytotoxic T cell crossmatch and no identified anti human leucocyte antigen class I donor specific antibodies. T cell flow crossmatch was performed within 24h of transplantation with serum obtained pretransplant (historic, recent or baseline). Posttransplant flow crossmatch were performed prospectively starting on the 3rd posttransplantation day. The immunosuppressive regime included thymoglobulin, tacrolimus, mycofenolate mofetil and steroids. Positive flow crossmatch occurred in 20/29 patients by the 3rd posttransplantation day, and in 17/27 patients after the 3rd posttransplantation day. All patients were started on intravenous immunoglobulin before transplantation: in nine patients (group A) at 400mg/kg/day for five days; in the remaining 21 patients (group B), as a continued infusion of 2g/kg during 48h. In group A, Rituximab was added only in the presence of antibody mediated rejection; in group B, introduced on the 3rd posttransplantation day whenever a positive flow crossmatch (with serum obtained pre or posttransplant) was reported. Antibody mediated rejection was observed in 44.4% of patients in group A, and 19% of those in group B. Mean follow-up was 12.2±5.5 months. Overall allograft survival was 76.6%, 81% in group B, and 66.6% in group A. At last follow up, mean serum creatinine was 1.3±0.6 mg/dl. Renal transplantation with pretransplant positive flow crossmatch is highly associated with antibody mediated rejection, despite introduction of intravenous immunoglobulin pretransplantation. However high dose intravenous immunoglobulin for 48h plus Rituximab by the 3rd posttransplantation day reduce the incidence of antibody mediated rejection by more than 50% and allowed for allograft survival of 81% at one year, with an excellent renal function.

Polyomavirus nephropathy is a major complication in renal transplantation, associated with renal allograft loss in 14 to 80% of cases. There is no established treatment, although improvement has been reported with a variety of approaches. The authors report two cases of polyomavirus infection in renal allograft recipients. In the first case, a stable patient presented with deterioration of renal function, worsening hypertension and weight gain following removal of ureteral stent placed routinely at the time of surgery. Ultrasound examination and radiology studies revealed hydronephrosis due to ureteral stenosis. A new ureteral stent was placed, but renal function did not improve. Urinary cytology revealed the presence of decoy cells and polyomavirus was detected in blood and urine by qualitative polymerase chain reaction. Renal biopsy findings were consistent with polyomavirus -associated nephropathy. In the second case, leucopaenia was detected in an asymptomatic patient 6 months after transplantation. Mycophenolate mophetil dosage was reduced but renal allograft function deteriorated, and a kidney biopsy revealed polyomavirus -associated nephropathy, also with SV40 positive cells. In both patients immunosuppression with tacrolimus was reduced, mycophenolate mophetil stopped and intravenous immune globulin plus ciprofloxacin started. As renal function continued to deteriorate, therapy with leflunomide (40 mg/day) was associated and maintained during 5 and 3 months respectively. In the first patient, renal function stabilised within one month of starting leflunomide and polymerase chain reaction was negative for polyomavirus after 5 months. A repeated allograft biopsy 6 months later showed no evidence of polyomavirus nephropathy. In the second patient, polyomavirus was undetectable in blood and urine by polymerase chain reaction after 3 months of leflunomide treatment, with no evidence of polyomavirus infection in a repeated biopsy 6 months after beginning treatment.