Light-chain deposition disease (LCDD) is a rare monoclonal gammopathy that involves the deposition of light chains (LC) in multiple organs, leading to progressive dysfunction. The kidney is usually the most affected organ and responsible for the initial clinical manifestations. We present the case of a patient with LCDD with prominent liver involvement (marked cholestasis, hepatomegaly and portal hypertension) but with no evidence of coexisting lymphoproliferative disorder. LEARNING POINTS Light-chain deposition disease (LCDD) is rare, and diagnosis can be challenging, especially in the absence of a coexistent lymphoproliferative disorder. The presence of light chains in blood or urine can be intermittent, and immunohistochemistry results of biopsy can be difficult to interpret. Hence, there is a need for a high level of suspicion in this diagnosis. Although renal involvement is the most classic presentation, LCDD should be suspected when there is multiple organ involvement, as in this case, even when renal manifestations do not dominate the clinical picture. Prognosis depends on the affected organs, and treatment should be started as rapidly as possible to suppress production of the clonal light-chain and halt organ damage.
<b><i>Background and Aims:</i></b> Liver cirrhosis (LC) is the end stage of multiple processes that lead to hepatic failure and is the 10th most common cause of death in the Western world. Bacterial infections are one of the most important clinical problems in patients with LC, as their underlying immune status is compromised. Approximately 60% of bacterial infections in cirrhotic patients are community acquired (CA) and 40% are nosocomial. The most common infections in cirrhotic patients are spontaneous bacterial peritonitis (SBP) (25%), urinary tract infection (UTI) (20%), pneumonia (15%), bacteremia (12%), and cellulitis (2–11%). The aim of this study was to evaluate the most frequent infections in patients with LC and describe the evolution of the microbiology in these patients. <b><i>Material and Methods:</i></b> This is a retrospective analysis of 4 interspersed years (2008, 2010, 2012, and 2014) that included 372 patients. Demographic characteristics were evaluated, including gender, age, type of infection, bacteria resistance profile, antibiotic use, Child-Pugh-Turcotte<i></i> and Model for End-Stage Liver Disease scores, and mortality rate. <b><i>Results:</i></b> The mean age of all patients enrolled in this study was 64.5 ± 12.2 years. Male patients were significantly more prevalent than female ones (72 vs. 28%). In the 4 analyzed years, the following numbers of infections occurred: 71 infections (49% CA and 51% nosocomial) in 2008; 86 infections (60.5% CA and 39.5% nosocomial) in 2010; 99 infections (56.6% CA and 43.4% nosocomial) in 2012; and 116 infections (70.7% CA and 29.3% nosocomial) in 2014. The most frequent infections were UTI (32.5%), respiratory tract infection (29.3%), SBP (26.1%), and cellulitis (6.2%). A microbiological agent was identified in 32.4, 59.3, 53.5, and 61.2% of infections in 2008, 2010, 2012, and 2014, respectively, with a predominance of gram-negative bacilli. In all series, a third-generation cephalosporin was the most frequent antibiotic used empirically. The majority of patients was in stage B (42.7%) of the Child-Pugh-Turcotte<i></i> score, followed by stage C (39.3%) and stage A (18%). Mortality increased significantly over the years, from 7% in 2008 to 25% in 2014 <i>(p = 0.016)</i>. <b><i>Conclusions:</i></b> The present study showed a high prevalence of bacterial infections in patients with LC. A high rate of suspicion is needed for an infectious process in these patients, and an appropriate antibiotic treatment can decrease the morbidity and mortality in cirrhotic patients.
Disseminated intravascular coagulation (DIC) is an uncommon clinical entity, characterized by widespread activation of the coagulation process, which results in intravascular deposition of fibrin in small and medium-sized vessels. Important hemodynamic trouble, multiorganic dysfunction and severe hemorrhagic diathesis can follow. The most common non-traumatic situations associated with DIC are sepsis or disseminated serious infections and neoplastic diseases. Aortic aneurysms (AA) are rarely associated with DIC. Sometimes they are first recognized in the investigation of this coagulopathy. Surgical repair is usually required for the resolution of concomitant DIC. We present in this case-report a 96-years-old patient with chronic DIC with an underlying inoperable AA. Resumo A coagulação intravascular disseminada (CID) é uma entidade clínica relativamente incomum, caraterizada por extensa ativação da coagulação que resulta na deposição intravascular de fibrina em vasos de pequeno e médio calibre. Podem resultar importantes perturbações hemodinâmicas, disfunção de múltiplos órgãos e grave diátese hemorrágica. As situações não traumáticas que mais vezes se complicam de CID são as infeções graves com sepsis e diversas doenças de foro oncológico. Os aneurismas aórticos (AA) associam-se raramente a CID. Podem ser reconhecidos pela primeira vez na investigação desta coagulopatia. Em regra só a reparação cirúrgica do AA permite a resolução da CID concomitante. Apresentamos neste artigo o caso clínico de um homem de 96 anos com CID crónica associada a AA inoperável. CASO CLÍNICOquais o fator tecidular que desperta o processo está mais localizado e/ou existe em menor quantidade 3 . O passo fundamental no tratamento da CID é o tratamento eficaz da doença ou condição subjacente 1,2 . Embora rara, é conhecida a associação de CID com aneurisma da aorta 1,3 . Em regra a resolução da CID exige a reparação cirúrgica do aneurisma 4 , além do tratamento de suporte conforme necessário. Apresentamos o caso clínico de um ancião com aneurismas aórticos sem possibilidade de resolução cirúrgica, cuja apresentação clínica foi uma forma crónica de CID, e que veio a falecer ao fim de 13 meses. A propósito deste caso fazemos uma breve revisão da literatura. Descrição do casoEm junho de 2013 um homem de 96 anos, residente num lar, recorreu ao Serviço de Urgência (SU) do nosso hospital por equimoses espontâneas com início dez dias antes. Referia episódios de expetoração hemoptóica no último mês, além de disfonia. Em observação por ORL, fora identificada paresia da corda vocal esquerda. Negava história de traumatismos, epistaxis, hematemeses, hematúria, retorragias ou melenas. Não tinha antecedentes patológicos de relevo nem tomava qualquer medicamento. Apresentava equimose e edema na mão esquerda, equimose extensa na face interna do braço direito e equimoses na face posterior de ambas as coxas. A pele e as mucosas estavam ligeiramente descoradas; não apresentava adeno- IntroduçãoA coagulação intravascular disseminada (CID) é uma entida...
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