Fatigue in incident peritoneal dialysis and mortality: A real-world side-by-side study in Brazil and the United States.

BACKGROUND: We tested if fatigue in incident Peritoneal Dialysis associated with an increased risk for mortality, independently from main confounders. METHODS: We conducted a side-by-side study from two of incident PD patients in Brazil and the United States. We used the same code to independently analyze data in both countries during 2004 to 2011. We included data from adults who completed KDQOL-SF vitality subscale within 90 days after starting PD. Vitality score was categorized in four groups: >50 (high vitality), ≥40 to ≤50 (moderate vitality), >35 to <40 (moderate fatigue), ≤35 (high fatigue; reference group). In each country's cohort, we built four distinct models to estimate the associations between vitality (exposure) and all-cause mortality (outcome): (i) Cox regression model; (ii) competing risk model accounting for technique failure events; (iii) multilevel survival model of clinic-level clusters; (iv) multivariate regression model with smoothing splines treating vitality as a continuous measure. Analyses were adjusted for age, comorbidities, PD modality, hemoglobin, and albumin. A mixed-effects meta-analysis was used to pool hazard ratios (HRs) from both cohorts to model mortality risk for each 10-unit increase in vitality. RESULTS: We used data from 4,285 PD patients (Brazil n = 1,388 and United States n = 2,897). Model estimates showed lower vitality levels within 90 days of starting PD were associated with a higher risk of mortality, which was consistent in Brazil and the United States cohorts. In the multivariate survival model, each 10-unit increase in vitality score was associated with lower risk of all-cause mortality in both cohorts (Brazil HR = 0.79 [95%CI 0.70 to 0.90] and United States HR = 0.90 [95%CI 0.88 to 0.93], pooled HR = 0.86 [95%CI 0.75 to 0.98]). Results for all models provided consistent effect estimates. CONCLUSIONS: Among patients in Brazil and the United States, lower vitality score in the initial months of PD was independently associated with all-cause mortality.

Falência hepática fulminante por hepatite C aguda em uma paciente portadora de lúpus eritematoso sistêmico e deficiência de fator V de Leiden: um relato de caso / Fulminant liver failure due to acute hepatitis C in a patient with systhemic erithematosis lupus and defficiency of Leiden factor V: a case report

A falência hepática fulminante (FHF) é uma doença rara com tendência a um desfecho trágico. As hepatites virais são uma causa incomum e ainda há controvérsias se o vírus da hepatite C (HCV) poderia ser um fator etiológico. Relatamos o caso de uma paciente feminina de 50 anos, hipertensa, diabética, lúpica e com deficiência de fator V de Leiden, que foi a óbito por FHF por hepatite aguda por HCV comprovada por sorologias e PCR-quantitativo. Outros casos de FHF por HCV em pacientes com comorbidades hepáticas ou imuno-mediadas serão revisados, bem como evidências imunogenéticas que levaram a interpretação da FHF como tendo um fundo auto-imune que ainda precisa ser mais estudado.

Fulminant Hepatic Failure (FHF) is a rare condition that tends to have a tragical disclosure. Viral hepatitis are an uncommon cause and it is still controversial if hepatitis C virus (HCV) could be an etiological factor. We report the case of a 50-year-old female patient who was diabetic, hipertense, lupic and had Leiden´s factor V deficiency, who died of FHF caused by acute hepatitis C proven by serology and quantitative-PCR. Other cases of FHF caused by HCV in patients with hepatic comorbidities or imunne-mediated diseases will be reviewed, as well as immunogenetic evidences that can lead to interpretation of FHF a shaving an auto-immune background that still needs more studying.

Mannose binding lectin deposition in skin of lupus erythematosus patients: a case series.

INTRODUCTION: Mannose binding lectin (MBL) has been linked to predisposition to systemic lupus erythematosus (SLE) and to disease activity. Some studies found deposits of MBL in glomerular tissue of patients with lupus nephritis. There is no research about the deposition of MBL in skin. MATERIALS AND METHODS: Skin biopsies from lesional and non lesional skin of 4 discoid lupus erythematosus (DLE) and 10 SLE patients were submitted to immunofluorescence staining for IgG, IgA, IgM, C3, C4, C1q, C5b-9 and MBL. Charts were reviewed for demographic, clinical and serological data. Patients with SLE had disease activity measured by SLEDAI. RESULTS: MBL was found only in SLE lesional skin and its presence showed an association trend towards higher disease activity. Deposition of C5b-9 occurred in vessels only in patients with SLE (70%) and in the two patients with kidney involvement. CONCLUSIONS: MBL deposition was found in the lesional skin of SLE patients but not in SLE non lesional skin nor in DLE patients, and it seems to be less frequent and less strong than observed in the kidneys biopsies, suggesting that the complement participation in the pathophysiology of SLE process may not be the same in these two clinical manifestations.

Gluteoplastia unilateral para o tratamento de sequela de esclerodermia / Unilateral gluteoplasty for the treatment of scleroderma sequelae

A esclerodermia é uma doença autoimune com incidência de 0,4-2,7 para cada 100.000 pessoas com predominância pelo sexo feminino. Ela se apresenta de diversas formas, sendo a linear a forma mais prevalente em crianças, afetando 60% dos casos. Acomete membros e tronco, causando atrofia muscular e do subcutâneo, discrepância no comprimento dos membros, contraturas articulares, podendo levar inclusive à incapacidade física. Relatar uma indicação incomum para a gluteoplastia com resultado satisfatório usando- -se técnicas sedimentadas. J.B., feminino, 26 anos, branca, tratada de esclerodermia cutânea dos 3 aos 10 anos com remissão completa da atividade da doença. Apresentava sequela com atrofia cutânea, subcutânea e muscular somente no lado direito, localizada principalmente no sulco mamário, glúteo e face interna da coxa. A cirurgia foirealizada em dois tempos. O primeiro tempo consistiu em gluteoplastia unilateral de aumento com implante de silicone no plano intramuscular, lipoenxertia glútea e crural associados à mamoplastia de aumento bilateral. Num segundo momento uma nova lipoenxertia atenuou as assimetrias remanescentes. Devido à versatilidade e segurança da gluteoplastia associando-se inclusão de implante de silicone com a lipoenxertia é possível, em situações inusitadas, como esta paciente com uma sequela de esclerodermia unilateral, reestabelecer o contorno corporal com harmonia e resultados naturais

Scleroderma is an autoimmune disease with an incidence of 0.4 - 2.7 for every 100.000 people with preference by women. It shows itself in many ways, the linear form is more prevalent in children, affecting 60% of the cases. Affects limbs and trunk, causing muscle and subcutaneous atrophy, discrepancy in the length of the members, joint contractures, and may lead to physical disability. To report a rare indication for gluteoplasty with satisfactory results using known techniques. J. B., female, 26 years old, white, treated in cutaneous scleroderma from 3 to 10 years old with complete remission of the disease activity. She developed sequelae with cutaneous, subcutaneous and muscular atrophy only in the right side, located mainly in the inframammary fold, buttocks and inner thigh. The surgery was performed in two stages. The first time was a unilateral augmentation gluteoplasty with silicone gel implant positioned in the muscular plane, gluteal and crural lipofilling associated with bilateral augmentation mammoplasty. In a second moment a new lipofilling attenuated the remaining asymmetries. Due to the versatility and safety of gluteoplasty associating the inclusion of silicone gel implant with the lipofilling is possible, in unusual situations, such as this patient with a sequelae of unilateral scleroderma, reestablish the body contour with harmony and natural results

Lesões palpebrais no lúpus eritematoso: [relato de caso] / Lid lesions in lupus erythematosus: [case report]

O envolvimento cutâneo do lúpus eritematoso, quando aparece de maneira isolada em pálpebra, pode ser de difícil diagnóstico. Diagnósticos errôneos são comuns, principalmente o de blefarite resistente a tratamento. Todavia o diagnóstico precoce é importante no sentido de evitar a cicatrização e possíveis seqüelas nas delicadas estruturas locais. Descrevem-se três casos de lesões palpebrais em pacientes com lúpus eritematoso, e, em cada uma das situações, essa lesão teve um significado clínico diferente. Nas duas primeiras pacientes, firmou-se o diagnóstico de lesão discóide pela biópsia. Na terceira paciente encontrou-se um carcinoma basocelular.

The cutaneous involvement of lupus erythematosus is difficult to diagnose when it appears isolated in the eyelid. Misdiagnosis is common, confusions arising mainly with chronic resistant blepharitis. Yet the early diagnosis is important to avoid scarring and damage to the delicate local structures. We present three patients with lupus and eyelid cutaneous lesions, each of them with a different clinical significance. In the first two patients it was possible to diagnose discoid lesion through skin biopsy. In the third, a basocelular carcinoma was found.