Hepatitis fulminante en paciente con amiloidosis primaria con respuesta hematológica completa

Primary amyloidosis is a systemic disease that results from abnormal kappa or lambda protein misfolding. The prognosis is poor, and the treatment includes chemotherapy. Hepatic disorders related to primary amyloidosis are found in at least 70% of autopsies. They have a bad prognosis factor, but their clinical manifestations are rare. We report a patient aged 41 with an initial diagnosis of nephrotic syndrome. Renal biopsy showed amyloid deposition. The treatment included quimiotherapy, reaching a complete hematological response. Even though the patient developed a hepatic failure dying.La amiloidosis primaria es una enfermedad multisistémica debida al mal plegamiento de proteínas kappa o lambda. El pronóstico es infausto y el tratamiento emplea quimioterapia. La afectación hepática se describe en el 70% de las autopsias de pacientes con amiloidosis sistémica y se asocia a peor pronóstico. Presentamos el caso de un varón de 41 años de edad que debuta con edemas en miembros inferiores y bolsa escrotal y síndrome nefrótico clínico y bioquímico. La biopsia renal es compatible con una amiloidosis primaria. Inicia tratamiento quimioterápico logrando respuesta hematológica completa, pero, a pesar de ello, sufre un fallo hepático fulminante de origen colestásico, y fallece

Are Reduced Levels of Coagulation Proteins Upon Admission Linked to COVID-19 Severity and Mortality?

Background: The link between coagulation system disorders and COVID-19 has not yet been fully elucidated. Aim: Evaluating the association of non-previously reported coagulation proteins with COVID-19 severity and mortality. Design: Cross-sectional study of 134 COVID-19 patients recruited at admission and classified according to the highest COVID-19 severity reached (asymptomatic/mild, moderate, or severe) and 16 healthy control individuals. Methods: Coagulation proteins levels (antithrombin, prothrombin, factor_XI, factor_XII, and factor_XIII) and CRP were measured in plasma by the ProcartaPlex Panel (Invitrogen) multiplex immunoassay upon diagnosis. Results: We found higher levels of antithrombin, prothrombin, factor XI, factor XII, and factor XIII in asymptomatic/mild and moderate COVID-19 patients compared to healthy individuals. Interestingly, decreased levels of antithrombin and factors XI, XII, and XIII were observed in those patients who eventually developed severe illness. Additionally, survival models showed us that patients with lower levels of these coagulation proteins had an increased risk of death. Conclusion: COVID-19 provokes early increments of some specific coagulation proteins in most patients. However, lower levels of these proteins at diagnosis might "paradoxically" imply a higher risk of progression to severe disease and COVID-19-related mortality.This study was supported by grants from Instituto de Salud Carlos III [ISCIII; Grant Number COV20/1144 (MPY224/20) to AF-R/MJ-S]. AF-R, MJ-S, and MR are Miguel Servet researchers supported and funded by ISCIII (Grant Numbers: CP14CIII/00010 to AF-R, CP17CIII/00007 to MJ-S, and CP19CIII/00002 to MR).S

Characteristics, complications and outcomes among 1549 patients hospitalised with COVID-19 in a secondary hospital in Madrid, Spain: a retrospective case series study

Objectives To describe demographic, clinical, radiological and laboratory characteristics, as well as outcomes, of patients admitted for COVID-19 in a secondary hospital.Design and setting Retrospective case series of sequentially hospitalised patients with confirmed SARS-CoV-2, at Infanta Leonor University Hospital (ILUH) in Madrid, Spain.Participants All patients attended at ILUH testing positive to reverse transcriptase-PCR on nasopharyngeal swabs and diagnosed with COVID-19 between 1 March 2020 and 28 May 2020.Results A total of 1549 COVID-19 cases were included (median age 69 years (IQR 55.0–81.0), 57.5% men). 78.2% had at least one underlying comorbidity, the most frequent was hypertension (55.8%). Most frequent symptoms at presentation were fever (75.3%), cough (65.7%) and dyspnoea (58.1%). 81 (5.8%) patients were admitted to the intensive care unit (ICU) (median age 62 years (IQR 51–71); 74.1% men; median length of stay 9 days (IQR 5–19)) 82.7% of them needed invasive ventilation support. 1393 patients had an outcome at the end of the study period (case fatality ratio: 21.2% (296/1393)). The independent factors associated with fatality (OR; 95% CI): age (1.07; 1.06 to 1.09), male sex (2.86; 1.85 to 4.50), neurological disease (1.93; 1.19 to 3.13), chronic kidney disease (2.83; 1.40 to 5.71) and neoplasia (4.29; 2.40 to 7.67). The percentage of hospital beds occupied with COVID-19 almost doubled (702/361), with the number of patients in ICU quadrupling its capacity (32/8). Median length of stay was 9 days (IQR 6–14).Conclusions This study provides clinical characteristics, complications and outcomes of patients with COVID-19 admitted to a European secondary hospital. Fatal outcomes were similar to those reported by hospitals with a higher level of complexity