Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis which mortality can reach 50%. The usual treatment involves suspension of the technique, medical treatment, and surgical enterolysis. We report the case of a 36-year-old female who undergone continuous ambulatory peritoneal dialysis without complications for 10 years. She presented at the emergency department with abdominal pain and a diagnosis of peritonitis was assumed. The patient developed a systemic inflammatory response, and her abdominal computer tomography (CT) scan showed multiple parietal calcifications. After multiple broad-spectrum antibiotics, an undetermined febrile syndrome persisted, and parenteral nutrition was started. An EPS diagnosis was established and treatment with tamoxifen was proposed. Exploratory laparoscopy was decided, and the intraoperative findings were consistent with EPS. The patient was submitted to extensive enterolysis. A clinical improvement led to a discharge after 60 days of hospitalization. After more than 30 months with tamoxifen, she remains on hemodialysis and doing well. There are no randomized controlled trials to guide EPS management and the following procedures are more difficult to decide, who makes these reports important to the reflection. We describe a case of successful treatment of EPS, with a very severe presentation. The management with enterolysis and tamoxifen contributed decisively to this outcome.

INTRODUCTION AND AIMS: Atypical hemolytic-uremic syndrome has ill-defined ethyopathogenesis, and may affect multiple organs. It results from complement alternative pathway dysregulation and its consequences can be catastrophic if not timely detected, as it can be life-threatening. Discovery of new pathogenic mutations which can help to detect this disease sooner is of utmost importance. Here we present one of these new pathogenic mutations of CFI gene.

INTRODUCTION AND AIMS: Atypical hemolytic-uremic syndrome has ill-defined ethyopathogenesis, and may affect multiple organs.It results from complement alternative pathway dysregulation and its consequences can be catastrophic if not timely detected, as it can be life-threatening.Discovery of new pathogenic mutations which can help to detect this disease sooner is of utmost importance.Here we present one of these new pathogenic mutations of CFI gene.METHODS: F, 44 years old, currently on peritoneal dialysis.Catastrophic presentation of kidney disease, with need for urgent start of dialysis (Urea 306 mg/dL and SCr 25 mg/dL).Past smoking habits, obesity, controlled hypertension, and one living birth.Without family or personal history of kidney disease.Severe hemolytic anemia (Hb 3,9 g/dL and LDH 1200) was noted at time of admission in our hospital.Additional laboratory evaluation revealed a low C3 complement level of 70 mg/dL (normal, 90-200 mg/ dL), a normal C4 complement level and negative antinuclear antibody.Plasmapheresis was initiated with no clinical response.Kidney biopsy revealed crescentic glomerulonephritis and vasculitis, and she was started on IV cyclophosphamide.She maintained no clinical response and terminal chronic kidney disease was assumed.She began peritoneal dialysis after a period of 30 days on hemodialysis.Later, genetic testing for mutation in the alternative complement pathway (CFH, MCP, CFI, CFB, C3, THBD) by gene sequencing, revealed the patient to be heterozygous for a new variant c.1071T>G (p.IIe357Met) at CFI gene.This was a variant within a conservative domain.She was referred to a genetic counselor.Genetic analysis was performed on her family members.RESULTS: The same mutation was detected in her mother (heterozygous for variant c.1071T>G (p.IIe357Met) but not in her father.Her mother is 82 years old and has no history of chronic kidney disease.Taken together with the available clinical information and the above analysis, the c.1071T>G was designated as potentially disease-causing.Althought this mutation has been reported in other countries with association to this disease(1,2), its significance is still considered to be unknown.This is, as far as we know, the first case ever described of this apparently disease-causing mutation in our country.CONCLUSIONS: Atypical hemolytic-uremic syndrome results from complement dysregulation, which can arise from mutations within complement factor H, B and I.This patient's genetic analysis was negative for other previously known mutations.Here we describe a new mutation in CFI gene in a patient affected by aSHU.This mutation was not previously described in our country and is still very much unknown.While detection of this new variant in an unaffected parent could suggest the variant is benign, it could also reflect incomplete penetrance.As it is located in a conservative domain, the hypothesis that it can be pathogenic remains strong.This case illustrates the importance of genetic referral and genetic study in cases of atypical-hemolytic uremic syndrome1.

 ABSTRACT Introduction: The population ageing has conditioned a progressive increase of very old patients on haemo- dialysis. These patients present multiple comorbidities that worsen the prognosis in dialysis and have an impact on their quality of life. Material and Methods: A retrospective observational study was performed, analysing all patients over 80 years of age that started regular haemodialysis between January 2004 and December 2011. The comorbidities were stratified using the Charlson score and correlated with mortality. Survival analysis was performed with Kaplan-Meier curves. Results: Fifty-nine patients were included, 35 females and 24 males, with a mean age of 84 ± 3 years. At the start of dialysis, the estimated glomerular filtration rate calculated using the modification of diet in renal disease (MDRD) formula was 10.8 ± 2.5 mL/min/1.73 m 2 . The initial Charlson score was 8 ± 3 and became higher at the end of follow-up (11.5 ± 2.9; p < 0.01). A Charl- son score ≥ 8 was associated with more hospitalization days (878 vs. 360; p < 0.005). The survival rate was 56 % at 12 months and 31% at 24 months. The mortality rate at the end of follow-up (20.4 ± 15.7 months) was 55.9%, with a mortality rate of 27.3% at 3 months. The patients with early death presented a higher Charlson score (13.0 ± 1.7 vs. 6.4 ± 1.2; p < 0.01) at the beginning of dialysis. Conclusion: The benefits of dialysis in survival and quality of life in very elderly patients have been questioned. In our series, more than 1/4 of the patients died in the first 3 months of dialysis, corresponding to higher comorbidity scores. The use of comorbidity scores like the Charlson's may assist in the assessment of the short-term prognosis, but the individualized decision should prevail in all cases. Key‑Words: Charlson score; chronic renal disease; comorbidities; elderly patients; haemodialysis; survival.

management of patients with human immunodeficiency virus (HIV) in intensive care, and would like to report our experience in the era of improved life expectancy for HIV patients treated with highly-active antiretroviral therapy (HAART). With the dramatically improved prognosis for these patients, more are being admitted to intensive care units (ICU) for non-HIV-associated conditions.2,3 We reviewed all HIVpositive patients admitted to the ICU of the Hospital Center of Tras-os-Montes and Alto Douro in Portugal between 1st January 1997 and 31st December 2008. Results