The ERA Registry Annual Report 2021: a summary.

Background: The European Renal Association (ERA) Registry collects data on kidney replacement therapy (KRT) in patients with end-stage kidney disease (ESKD). This paper is a summary of the ERA Registry Annual Report 2021, including a comparison across treatment modalities. Methods: Data was collected from 54 national and regional registries from 36 countries, of which 35 registries from 18 countries contributed individual patient data and 19 registries from 19 countries contributed aggregated data. Using this data, incidence and prevalence of KRT, kidney transplantation rates, survival probabilities and expected remaining lifetimes were calculated. Result: In 2021, 533.2 million people in the general population were covered by the ERA Registry. The incidence of KRT was 145 per million population (pmp). In incident patients, 55% were 65 years or older, 64% were male, and the most common primary renal disease (PRD) was diabetes (22%). The prevalence of KRT was 1040 pmp. In prevalent patients, 47% were 65 years or older, 62% were male, and the most common PRDs were diabetes and glomerulonephritis/sclerosis (both 16%). On 31 December 2021, 56% of patients received haemodialysis, 5% received peritoneal dialysis, and 39% were living with a functioning graft. The kidney transplantation rate in 2021 was 37 pmp, a majority coming from deceased donors (66%). For patients initiating KRT between 2012-2016, 5-year survival probability was 52%. Compared to the general population, life expectancy was 65% and 68% shorter for males and females receiving dialysis, and 40% and 43% shorter for males and females living with a functioning graft.

Sodium and water dynamics in the progression of chronic kidney disease: mechanisms and clinical significance.

AIM: Lifestyle modifications can postpone the progression of chronic kidney disease toward its terminal stage. This mini-review aims to explore the impact of salt and water intake on the progression of chronic kidney disease (CKD) and provide insights into the optimal consumption levels to preserve the glomerular filtration rate. METHODS: We reviewed relevant literature to examine the association between salt and water consumption and CKD progression. Our analysis includes discussions on the pathophysiology, findings from clinical trials, and recommended intake guidelines. RESULTS: Sodium intake, often linked to cardiovascular risk and CKD progression, has shown a complex J-shaped association in some studies, leading to uncertainty about the ideal salt intake level. Sodium and fluid retention are key factors contributing to hypertension, a well-established risk factor for CKD progression. Low-sodium diets have demonstrated promise in reducing blood pressure and enhancing the effects of renin-angiotensin-aldosterone system inhibitors in non-dialysis CKD patients. However, a debate persists regarding the independent effect of salt restriction on CKD progression. Despite medical recommendations, salt consumption remains high among CKD patients. Additionally, the role of water consumption in CKD remains controversial despite its established benefits for CKD prevention in the general population. CONCLUSION: Lifestyle modifications involving salt and water intake can influence the progression of CKD. While low-sodium diets have shown potential for mitigating hypertension and proteinuria in non-dialysis CKD patients, their independent impact on CKD progression warrants further investigation. The role of water consumption in CKD remains uncertain, and there is a need for additional research in this area. Clinicians should consider individualized dietary recommendations for CKD patients to help preserve the glomerular filtration rate and improve overall outcomes.

A Case of Polymyositis Associated with Cytomegalovirus Infection in a Patient with Hashimoto's Thyroiditis.

Polymyositis is a rare condition with an unknown etiology occurring more frequently in adult women. There is a lack of evidence on the coexistence of PM and CMV infection in a patient with hypothyroidism due to Hashimoto's Thyroiditis. However, the growing occurrence of both CMV infection and the simultaneous occurrence of autoimmune diseases points out a relationship, while the association direction remains unclear. Case outline: A 32-year-old woman recently treated for HT hypothyroidism was admitted to the hospital two weeks after being treated for common flu by the family doctor, complaining about a worsening condition with muscle pain, weakness, frequent falls, and fatigue. The first tests showed a normalized thyroid function, with elevated values of troponin and serum creatinine kinase (CK). The immunological tests revealed the presence of a high titer of CMV IgG antibodies and raised levels of CMV DNA. Pelvis MRI images demonstrated markedly elevated signals on the STIR sequences in the pelvis, thighs, and calves, indicating active and severe multifocal myositis. The diagnosis of PM was confirmed with the muscle biopsy on day 7 of hospitalization. The patient showed significant improvements within two weeks after the medical therapy and physiotherapy.

The ERA Registry Annual Report 2020: a summary.

Background: The European Renal Association (ERA) Registry collects data on kidney replacement therapy (KRT) in patients with ESKD. This paper is a summary of the ERA Registry Annual Report 2020, also including comparisons among primary renal disease (PRD) groups. Methods: Data were collected from 52 national and regional registries from 34 European countries and countries bordering the Mediterranean Sea: 35 registries from 18 countries providing individual level data and 17 registries from 17 countries providing aggregated data. Using this data, KRT incidence and prevalence, kidney transplantation rates, expected remaining lifetimes and survival probabilities were calculated. Results: A general population of 654.9 million people was covered by the ERA Registry in 2020. The overall incidence of KRT was 128 per million population (p.m.p.). In incident KRT patients, 54% were older than 65 years, 63% were men and the most common PRD was diabetes mellitus (21%). Regarding initial treatment modality in incident patients, 85% received haemodialysis (HD), 11% received peritoneal dialysis (PD) and 4% received a pre-emptive kidney transplant. On 31 December 2020, the prevalence of KRT was 931 p.m.p. In prevalent patients, 45% were older than 65 years, 60% were men and glomerulonephritis was the most common PRD (18%). Of these patients, 58% were on HD, 5% on PD and 37% were living with a kidney transplant. The overall kidney transplantation rate in 2020 was 28 p.m.p., with a majority of kidney grafts from deceased donors (71%). The unadjusted 5-year survival, based on incident dialysis patient from 2011-15, was 41.8%. For patients having received a deceased donor transplant, the unadjusted 5-year survival probability was 86.2% and for patients having received a living donor transplant it was 94.4%. When comparing data by PRD group, differences were found regarding the distribution of age groups, sex and treatment modality received.

Trends in kidney transplantation rate across Europe: study from the ERA Registry.

BACKGROUND: The aim of this study was to identify trends in total, deceased donor (DD) and living donor (LD) kidney transplantation (KT) rates in European countries. METHODS: The European Renal Association (ERA) Registry and the Global Observatory on Donation and Transplantation (GODT) databases were used to obtain the number of KTs in individual European countries between 2010 and 2018. General population counts were obtained from Eurostat or the national bureaus of statistics. The KT rate per million population (p.m.p.) and the average annual percentage change (APC) were calculated. RESULTS: The total KT rate in the 40 participating countries increased with 1.9% annually  [95%  confidence  interval  (CI) 1.5, 2.2] from 29.6 p.m.p. in 2010 to 34.7 p.m.p. in 2018, reflecting an increase of 3.4 p.m.p. in the DD-KT rate (from 21.6 p.m.p. to 25.0 p.m.p.; APC 1.9%; 95% CI 1.3, 2.4) and of 1.5 p.m.p. in the LD-KT rate (from 8.1 p.m.p. to 9.6 p.m.p.; APC 1.6%; 95% CI 1.0, 2.3). The trends in KT rate varied widely across European countries. An East-West gradient was observed for DD-KT rate, with Western European countries performing more KTs. In addition, most countries performed fewer LD-KTs. In 2018, Spain had the highest DD-KT rate (64.6 p.m.p.) and Turkey the highest LD-KT rate (37.0 p.m.p.). CONCLUSIONS: The total KT rate increased due to a rise in the KT rate from DDs and to a lesser extent from LDs, with large differences between individual European countries.

Association of multiple retinal nodular hamartomas and "confetti" skin lesions with end-stage renal disease in patients with tuberous sclerosis.

PURPOSE: The main purpose of this study is to explore characteristics of patients with chronic kidney disease in tuberous sclerosis (TSC) and to underline differences in clinical characteristics between end-stage renal disease (ESRD) patients and patients in earlier stages of chronic kidney disease. METHODS: This multicentric, retrospective study included data for 48 patients from seven South-Eastern European countries (Albania, Bosnia and Herzegovina, Croatia, Greece, Montenegro, Serbia, Slovenia) in the period from February to August 2020. Researchers collected data from local and national nephrological and neurological registries and offered clinical and laboratory results from medical histories in follow-up periods. RESULTS: This study enrolled 48 patients with a median age of 32.3 years (range, 18-46 years), and predominant female gender (60.45%). The percentage of patients with chronic kidney disease (CKD) diagnosis of the total number of patients was 66.90%, with end-stage renal disease development in 39.6%. The most prevalent renal lesions leading to chronic kidney disease were angiomyolipomas (AMLs) in 76.6%, while multiple renal cysts were present in 42.6% of patients. Nephrectomy was performed in 43% of patients, while the mTOR inhibitors were used in 18 patients (37.5%). The majority of patients had cutaneous manifestations of tuberous sclerosis-83.30% had hypomelanotic cutaneous lesions, and 68.80% had angiofibromas. Multiple retinal nodular hamartomas and "confetti" skin lesions were more frequent in end-stage renal disease (ESRD) than in patients with earlier stages of chronic kidney disease (p-0.033 and 0.03, respectively). CONCLUSION: Our study has also shown that retinal hamartomas and "confetti" skin lesions are more frequent in end-stage renal diseases (ESRD) patients than in other chronic kidney disease (CKD) patients. Usage of mTOR inhibitors can also reduce the number of complications and associated with tuberous sclerosis, such as dermatological manifestations and retinal hamartoma, which are more common in the terminal stage of chronic kidney disease.

The ERA Registry Annual Report 2019: summary and age comparisons.

BACKGROUND: Data on renal replacement therapy (RRT) for end-stage renal disease were collected by the European Renal Association (ERA) Registry via national and regional renal registries in Europe and countries bordering the Mediterranean Sea. This article provides a summary of the 2019 ERA Registry Annual Report, including data from 34 countries and additional age comparisons. METHODS: Individual patient data for 2019 were provided by 35 registries and aggregated data by 17 registries. Using these data, the incidence and prevalence of RRT, the kidney transplantation activity and the survival probabilities were calculated. RESULTS: In 2019, a general population of 680.8 million people was covered by the ERA Registry. Overall, the incidence of RRT was 132 per million population (p.m.p.). Of these patients, 62% were men, 54% were ≥65 years of age and 21% had diabetes mellitus as primary renal disease (PRD), and 84% had haemodialysis (HD), 11% had peritoneal dialysis (PD) and 5% had pre-emptive kidney transplantation as an initial treatment modality. The overall prevalence of RRT on 31 December 2019 was 893 p.m.p., with 58% of patients on HD, 5% on PD and 37% living with a kidney transplant. The overall kidney transplant rate was 35 p.m.p. and 29% of the kidney grafts were from a living donor. The unadjusted 5-year survival probability was 42.3% for patients commencing dialysis, 86.6% for recipients of deceased donor grafts and 94.4% for recipients of living donor grafts in the period 2010-14. When comparing age categories, there were substantial differences in the distribution of PRD, treatment modality and kidney donor type, and in the survival probabilities.

The ERA-EDTA Registry Annual Report 2018: a summary.

BACKGROUND: The European Renal Association - European Dialysis and Transplant Association (ERA-EDTA) Registry collects data on kidney replacement therapy (KRT) via national and regional renal registries in Europe and countries bordering the Mediterranean Sea. This article summarizes the 2018 ERA-EDTA Registry Annual Report, and describes the epidemiology of KRT for kidney failure in 34 countries. METHODS: Individual patient data on patients undergoing KRT in 2018 were provided by 34 national or regional renal registries and aggregated data by 17 registries. The incidence and prevalence of KRT, the kidney transplantation activity and the survival probabilities of these patients were calculated. RESULTS: In 2018, the ERA-EDTA Registry covered a general population of 636 million people. Overall, the incidence of KRT for kidney failure was 129 per million population (p.m.p.), 62% of patients were men, 51% were ≥65 years of age and 20% had diabetes mellitus as cause of kidney failure. Treatment modality at the onset of KRT was haemodialysis (HD) for 84%, peritoneal dialysis (PD) for 11% and pre-emptive kidney transplantation for 5% of patients. On 31 December 2018, the prevalence of KRT was 897 p.m.p., with 57% of patients on HD, 5% on PD and 38% living with a kidney transplant. The transplant rate in 2018 was 35 p.m.p.: 68% received a kidney from a deceased donor, 30% from a living donor and for 2% the donor source was unknown. For patients commencing dialysis during 2009-13, the unadjusted 5-year survival probability was 42.6%. For patients receiving a kidney transplant within this period, the unadjusted 5-year survival probability was 86.6% for recipients of deceased donor grafts and 93.9% for recipients of living donor grafts.

Therapeutic monitoring of mycophenolic acid in renal transplanted patients by a validated HPLC method.

INTRODUCTION: Mycophenolate mofetil and its active metabolite mycophenolic acid are routinely used as immunosuppressant drugs in solid organ transplantation in a fixed daily dose regimen in association with cyclosporine, tacrolimus and steroids. Therapeutic drug monitoring for mycophenolic acid concentration has been suggested to optimize outcomes by reducing rejection and drug related toxicities in clinical renal transplantation. AIM: To determine the predose concentration of mycophenolic acid in renal transplanted patients by a validated proposed high-performance liquid chromatography (HPLC) method and to estimate the interindividual variability based on the therapeutic target. MATERIALS AND METHODS: An HPLC method combined with protein precipitation has been validated for mycophenolic acid determination in the human plasma obtained from 21 renal transplant recipients. HPLC analysis was carried out using the chromatographic system Agilent Technologies 1200 DAD. Samples were injected manually, and the compounds were separated on a LiChrosphere® select B C18 analytical column. The mobile phase was 45:55 (v/v) acetonitrile-buffer phosphate, pH 2.5, flow rate of 1.0 mL/min and column temperature of 30°C. Detection was performed at 215 nm. Whole blood samples were collected into vacutainers containing EDTA and separated at 6000 g for 10 minutes. A 200-µL aliquot of patient plasma was transferred to a tube, followed by addition of 10 µL of naproxen as internal standard and 400 µL of acetonitrile (v/v) as a protein precipitating agent. Each tube was vortex-mixed for 30 sec and then centrifuged for 10 min at 10000 rpm. 20 µL of the supernatant was injected into the HPLC system for analysis. RESULTS: The method showed appropriate linearity for MPA with correlation coefficient greater than 0.999. High inter-patient variability is observed with 18% of patients within the target trough concentration range, 27% of patients below the target trough concentration range and 54% over the range with risk of toxicity. CONCLUSIONS: Therapeutic monitoring of MPA might contribute to a better management of renal transplant recipient with the goal of optimizing therapeutic regimens in order to reduce the risk of rejection and MPA-related toxicity.

The ERA-EDTA Registry Annual Report 2017: a summary.

BACKGROUND: This article presents a summary of the 2017 Annual Report of the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry and describes the epidemiology of renal replacement therapy (RRT) for end-stage renal disease (ESRD) in 37 countries. METHODS: The ERA-EDTA Registry received individual patient data on patients undergoing RRT for ESRD in 2017 from 32 national or regional renal registries and aggregated data from 21 registries. The incidence and prevalence of RRT, kidney transplantation activity and survival probabilities of these patients were calculated. RESULTS: In 2017, the ERA-EDTA Registry covered a general population of 694 million people. The incidence of RRT for ESRD was 127 per million population (pmp), ranging from 37 pmp in Ukraine to 252 pmp in Greece. A total of 62% of patients were men, 52% were ≥65 years of age and 23% had diabetes mellitus as the primary renal disease. The treatment modality at the onset of RRT was haemodialysis for 85% of patients. On 31 December 2017, the prevalence of RRT was 854 pmp, ranging from 210 pmp in Ukraine to 1965 pmp in Portugal. The transplant rate in 2017 was 33 pmp, ranging from 3 pmp in Ukraine to 103 pmp in the Spanish region of Catalonia. For patients commencing RRT during 2008-12, the unadjusted 5-year patient survival probability for all RRT modalities combined was 50.8%.

Familial hemolytic uremic syndrome with occurrence in the postpartum period.

The hemolytic uremic syndrome (HUS) is a heterogeneous group of similar entities characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure (ARF) and is an important cause of ARF in childhood. Mutations have been reported in the complement regulatory protein factor H in both sporadic and familial HUS and have been identified in 10-20% of cases. Inherited HUS is unusual. We report the occurrence of HUS in two siblings after delivery, complicated with ARF and with a good outcome.

The European Renal Association - European Dialysis and Transplant Association Registry Annual Report 2014: a summary.

Background: This article summarizes the European Renal Association - European Dialysis and Transplant Association Registry's 2014 annual report. It describes the epidemiology of renal replacement therapy (RRT) for end-stage renal disease (ESRD) in 2014 within 35 countries. Methods: In 2016, the ERA-EDTA Registry received data on patients who in 2014 where undergoing RRT for ESRD, from 51 national or regional renal registries. Thirty-two registries provided individual patient level data and 19 provided aggregated patient level data. The incidence, prevalence and survival probabilities of these patients were determined. Results: In 2014, 70 953 individuals commenced RRT for ESRD, equating to an overall unadjusted incidence rate of 133 per million population (pmp). The incidence ranged by 10-fold; from 23 pmp in the Ukraine to 237 pmp in Portugal. Of the patients commencing RRT, almost two-thirds were men, over half were aged ≥65 years and a quarter had diabetes mellitus as their primary renal diagnosis. By day 91 of commencing RRT, 81% of patients were receiving haemodialysis. On 31 December 2014, 490 743 individuals were receiving RRT for ESRD, equating to an unadjusted prevalence of 924 pmp. This ranged throughout Europe by more than 10-fold, from 157 pmp in the Ukraine to 1794 pmp in Portugal. In 2014, 19 406 kidney transplantations were performed, equating to an overall unadjusted transplant rate of 36 pmp. Again this varied considerably throughout Europe. For patients commencing RRT during 2005-09, the 5-year-adjusted patient survival probabilities on all RRT modalities was 63.3% (95% confidence interval 63.0-63.6). The expected remaining lifetime of a 20- to 24-year-old patient with ESRD receiving dialysis or living with a kidney transplant was 21.9 and 44.0 years, respectively. This was substantially lower than the 61.8 years of expected remaining lifetime of a 20-year-old patient without ESRD.

Inflammation, Left Ventricular Hypertrophy, and Mortality in End-stage Renal Disease.

INTRODUCTION: The aim of this study was to evaluate ventricular geometry, its relationship with the inflammatory markers, and mortality of patients with end-stage renal disease on peritoneal and hemodialysis treatment. MATERIALS AND METHODS: We enrolled adult patients on long-term dialysis (hemodialysis and peritoneal dialysis) for more than 3 months. Two-dimensional echocardiography was performed by an experienced cardiologist who was blinded to all clinical details of patients. Cardiovascular mortality was assessed during a 2-year follow-up period. RESULTS: There were 129 participants, of whom 86 (66%) were on hemodialysis. Left ventricular hypertrophy was present in 86.7%; concentric hypertrophy was found in 64 (49.1%) and eccentric hypertrophy in 48 patients (37.2%). Patients with left ventricular hypertrophy were further divided into tertiles according to their left ventricular mass index. Logistic regression found pulse pressure as an independent risk factor associated with left ventricular mass index (odds ratio [OR], 1.04; 95% confidence interval (CI), 1.01 to 1.19; P = .047). Cardiovascular mortality rate was 15.5%. Multivariable analysis showed that C-reactive protein (OR, 1.06; 95% CI, 1.01 to 1.10; P = .01), pulse pressure (OR, 1.01; 95% CI, 1.0 to 1.26; P = .046), and left ventricular mass index (OR, 1.03; 95% CI, 1.01 to 1.21; P = .03) were independent risk factors for cardiovascular mortality. CONCLUSIONS: Concentric hypertrophy is the most frequent left ventricular geometry model in patients with chronic kidney disease. Inflammation, pulse pressure, and  left ventricular hypertrophy are interrelated and all contribute to mortality and cardiovascular death risk among dialysis patients.

Association between Nephrolithiasis, Hypertension and Obesity in Polycystic Kidney Disease.

AIM: We aim to define the correlations between nephrolithiasis, hypertension, age and obesity in patients with autosomal dominant polycystic kidney disease (ADPKD) in Albania. MATERIAL AND METHODS: We included 100 patients with autosomal dominant polycystic kidney from 2011 to 2014. The patients underwent X-ray and renal ultrasonography. We performed the metabolic evaluation of blood and urine. RESULTS: The patients with renal stones had a higher level of mean systolic and diastolic blood pressure compared with patients without stones (155 ± 12 mmHg vs. 145 ± 8 mmHg, and 105 ± 0.9 mmHg vs. 92 ± 1.28 mmHg, respectively). Patients with renal stones were older (47 ± 15 vs. 38 ± 5 years), had a higher prevalence of obesity [body mass index (BMI): 28 ± 2.4 vs. 25.7 ± 0.6], had higher levels of total cholesterol level (220 ± 5 mg/dl vs. 203 ± 4 mg/dl) as well as triglyceride levels (160 ± 9 mg/dl vs. 126 ± 4 mg/dl), compared with no renal stone individuals. CONCLUSION: ADPKD patients with renal stones in our study had a higher mean level of systolic and diastolic blood pressure, BMI and cholesterol and triglycerides levels compared with individuals without renal stones.

Histocompatibility testing for organ transplantation purposes in Albania: a single center experience.

BACKGROUND: Histocompatibility testing (HT) which includes donor-recipient human leukocyte antigen (HLA) matching, cross-match testing (XMT) and anti-HLA antibody searching are crucial examinations in solid organ transplantation aiming to avoid the hyperacute graft rejection and also to predict the immunological outcome of the graft. AIMS: The aim of this study was to analyse the tissue typing data collected at the Laboratory of Immunology and Histocompatibility of the University Hospital Center of Tirana, Albania, in order to define those actions that should be taken for improvements in the situation of kidney transplantation in Albania. STUDY DESIGN: Descriptive study. METHODS: The donor/recipient cross-match testing was performed through a standard complement-dependent cytotoxicity (CDC) assay using separated donor T and B cells that were tested in parallel with the recipient serum sample. All recipient sera were screened for anti-Class I and anti-Class II HLA antibodies using a bead based Luminex anti-HLA antibody screening test. In the case of detected positivity, an allele-specific anti-HLA antibody determination was conducted with the respective Luminex anti-Class I and Class II HLA antibody determination kits. RESULTS: A total of 174 recipients and 202 donors were typed for the purpose of living donor kidney transplantation at our laboratory between January 2006 and December 2012. The mean age and female gender proportion of patients were 34.9 years and 34.5%, respectively, and 48.0 years and 65.3% for the donors, respectively. Here, 25.9% of the patients reported a positive complement-dependent cytotoxicity cross-match test and/or a positive anti-HLA antibody testing result. Eighteen patients that were negative for the complement-dependent cytotoxicity cross-match test were positive for anti-HLA antibodies. CONCLUSION: The predominant causes of end-stage renal disease (ESRD) in our patient population are chronic pyelonephritis and glomerulonephritis. The female gender is significantly more frequent among donors, which emphasises the need for more gender equity as far as the altruistic willingness for organ donation is concerned. The significant number of patients with Luminex anti-HLA antibody positivity combined with complement-dependent cytotoxicity cross-match negative results underlines the necessity of using additional methods like cell-based flow cytometry or bead-based Luminex anti-HLA antibody assays for the detection of anti-donor-specific antibodies. We also suggest that the number of kidney transplantations in Albania needs to be increased significantly by expanding it with paired exchange living donation and also by implementing an efficient deceased donor kidney transplantation program.

Evaluation of thrombocytopenia in systemic lupus erythematosus and correlation with different organs damages.

INTRODUCTION: Thrombocytopenia is highly prevalent among patients with Systemic Lupus Erythematous(SLE) and at the same time it has been reported that a correlation exists between Thrombocytopenia and organ damage. The aim of this study is to highlight the correlation between Thrombocytopenia and the clinical manifestations of SLE. OBJECTIVES: The objective is to show the clinical manifestations and organ damage of Systemic Lupus Erythematous (SLE) patients who have been found to have Thrombocytopenia. METHODS: A retrospective study was conducted examining all patient charts diagnosed and treated for SLE at the Rheumatology Service of Mother Teresa Hospital Centre. All the data were collected from discharged patient charts. The data included were Anti DNA,AAN,C3 , thrombocytopenia, leucopenia, and organ damage. Data were taken from 2009 to 2013. The classification criteria of the American College of Rheumatology was used for all patients regarding the diagnosis. RESULTS: Out of 330 patients, 12 (3.64%) are men and 318 (96.3%) women. 73 of all patients have thrombocytopenia as cases and 257 patients had SLE without thrombocytopenia, which was considered as the control group. AAN 68(93.1%), Anti DNA 50 (64.3%) , low value of C3 46 (63%), and leucopenia were higher in thrombocytopenic patients compared with control group (p<0.05) 48 (65.7%) of thrombocytopenic patients develop lupus nephritis, 10 (13.6%) were with pulmonary involvement, and 42 (57.5%) had leukopenia. CONCLUSION: Thrombocytopenia is not directly associated with any disease activity, organ damage and mortality, but it should be considered as a prognostic factor which may help identifying a category of patients whose disease course can be aggravated.

Exhausting multiple hemodialysis access failures.

INTRODUCTION: Vascular access is often considered the Achilles heel the of hemodialysis because of its impact on morbidity, all cause mortality and finally costs of these patients. The most common complication of permanent hemodialysis (HD) vascular access is thrombosis, with some cases being related to hypercoagulability states. Antiphospholipid antibody syndrome (APAS) is a cause of increased thrombotic tendency, and this may complicate the management of such patients on HD. CASE REPORT: We describe a 41-year-old woman with end stage renal disease (ESRD) from Adult Polycystic Kidney Disease who was referred to our tertiary care center for treatment and selection of renal replacement therapy form. It was thought to initiate with peritoneal dialysis considering her actual conditions. She was putted on hemodialysis for several sessions, and a subclavian cathether was her first vascular access. The surgeon created an arterio-venous fistula which did not mature. After the implantation of the peritoneal cathether she started peritoneal dialysis and continued living with that for 2 years. She felt exhausted and because of a grave peritonitis episode accompanied with procedure failure and a long hospitalization she was transferred to hemodialysis. Renal transplantation was not possible because she didn't have a kidney donation. She was maintained on regular HD, but her dialysis care was complicated by recurrent vascular access failures. She had multiple interventions for arterio-venous fistulas and grafts but almost all of them failed due to thrombosis to the extent that only one access site was available for her routine renal replacement treatment. A thorough thrombophilia screen confirmed the presence of antiphospholipid antibodies. A diagnosis of APAS was made and she was anticoagulated with warfarin. The AVG made in this last available site is still working from 18 months. If it fails we have no answers and solutions for her. CONCLUSION: The presence of APAS can complicate HD management by causing recurrent vascular access thrombosis and failure, and nephrologist must remain alert to this possibility. Checking and treating as soon as possible it's our future challenge.

Metformin-induced lactic acidosis associated with multiorganic failure.

Lactic acidosis is a rare but severe complication in patients with type 2 diabetes treated with metformin. Patients with lactic acidosis show commonly signs of shock, tissue hypoxia, acute hepatic or renal failure and the link between metformin therapy and lactic acidosis may be coincidental, associated or causal. Excessive plasma metformin concentrations show that lactic acidosis is due to a toxicological mechanism. We report a case of severe multiorganic failure in a subject after treatment with high doses of metformin.

Pompe disease with heterogeneous presentations within a family.

Pompe disease is an acid maltase deficiency being part of glycogen storage diseases that affects all age groups. In both childhood and adult forms, the classic clinical picture is that of a progressive myopathy. Respiratory muscle involvement is common, may occur early in the course of the disease, and is the most frequent cause of mortality from acid maltase deficiency. Its association with rhabdomyolysis is rare and with a fatal prognosis. We describe the cases of a family with Pompe disease with a clinical spectrum extending throughout different ages of onset, degrees of organ involvement, and rates of progression. The twin patients with adult form of Pompe disease presented episodes of acute renal failure and respiratory insufficiency with a good outcome.

Urinary tract infections in polycystic kidney disease.

AIM: The aim of this study was to evaluate the bacteriological findings and the frequency of urinary tract infections in autosomal dominant polycystic kidney disease and their impact on renal function. METHODS: One hundred eighty patients with autosomal dominant polycystic kidney disease were studied from 2003 to 2008. Subjects were considered as having urinary tract infections if they had had one or more episodes of urinary infection. The antibiotic therapy for the treatment has been adapted according to the bacteriological findings. RESULTS: Urinary tract infections were observed in 60% of our patients (108 patients), and were more frequent in women than in men. The infections were typically caused by gram negative enteric organisms. Blood culture was positive in 10%, while urine culture was negative in 40%. The episodes of isolated cyst infections (negative urine culture and absence of white blood cell casts in urinary sediment) were more frequent than those of acute or chronic pyelonephritis (urinary sediment was positive for white blood cell casts). CONCLUSION: We conclude that urinary tract infections are frequent in our patients with autosomal dominant polycystic kidney disease. Distinguishing between cyst infection and acute or chronic pyelonephritis is often a challenge, and the diagnosis relies mainly on clinical and bacteriological findings.