Abdominal surgery in patients with chronic noncirrhotic extra hepatic portal vein obstruction: A multicenter retrospective study.

BACKGROUND AIMS: In patients with noncirrhotic chronic extra-hepatic portal vein obstruction (EHPVO), data on morbimortality of abdominal surgery are scarce. APPROACH RESULTS: We retrospectively analyzed the charts of 76 patients (78 interventions) with EHPVO undergoing abdominal surgery within the VALDIG network. Fourteen percent of the patients had ≥1 major bleeding (unrelated to portal hypertension) and 21% had ≥1 Dindo-Clavien grade ≥3 postoperative complication within 1 month after surgery. Fifteen percent had ≥1 portal hypertension related complication within 3 months after surgery. Three patients died within 12 months after surgery. An unfavorable outcome (i.e. ≥1 above-mentioned complications or death) occurred in 37% of the patients and was associated with a history of ascites and with non-wall, non-cholecystectomy surgical intervention: 17% of the patients with none of these features had an unfavorable outcome, versus 48% and 100% when one or both features were present, respectively. We then compared 63/76 EHPVO patients with 126 matched (2:1) control patients without EHPVO but with similar surgical interventions. As compared with control patients, incidence of major bleeding (p<0.001) and portal-hypertension related complication (p<0.001) was significantly higher in patients with EHPVO, but not that of grade ≥3 postoperative complication nor of death. The incidence of unfavorable post-operative outcome was significantly higher in patients with EHPVO than in those without (33% vs. 18%, p=0.01). CONCLUSION: Patients with EHPVO are at high-risk of major peri- or postoperative bleeding and postoperative complications, especially in those with ascites or undergoing surgery other than wall surgery or cholecystectomy.

Atrial fibrillation as a new prognosis factor in chronic patients after hospitalization: the CHRONIBERIA index.

A collaborative project in different areas of Spain and Portugal was designed to find out the variables that influence the mortality after discharge and develop a prognostic model adapted to the current healthcare needs of chronic patients in an internal medicine ward. Inclusion criteria were being admitted to an Internal Medicine department and at least one chronic disease. Patients' physical dependence was measured through Barthel index (BI). Pfeiffer test (PT) was used to establish cognitive status. We conducted logistic regression and Cox proportional hazard models to analyze the influence of those variables on one-year mortality. We also developed an external validation once decided the variables included in the index. We enrolled 1406 patients. Mean age was 79.5 (SD = 11.5) and females were 56.5%. After the follow-up period, 514 patients (36.6%) died. Five variables were identified as significantly associated with 1 year mortality: age, being male, lower BI punctuation, neoplasia and atrial fibrillation. A model with such variables was created to estimate one-year mortality risk, leading to the CHRONIBERIA. A ROC curve was made to determine the reliability of this index when applied to the global sample. An AUC of 0.72 (0.7-0.75) was obtained. The external validation of the index was successful and showed an AUC of 0.73 (0.67-0.79). Atrial fibrillation along with an advanced age, being male, low BI score, or an active neoplasia in chronic patients could be critical to identify high risk multiple chronic conditions patients. Together, these variables constitute the new CHRONIBERIA index.

Fungal Endocarditis: A Rare Case of Multiple Arterial Embolization.

Fungal endocarditis is a rare and fatal condition, with a mortality of up to 75%, affecting immunocompromised hosts with a predisposing condition, namely, a history of previous cardiac or noncardiac surgery. Embolization is frequent, accounting for 44% of cases, and as the most common site is the brain, it can cause leptomeningitis, parenchymal granulomas, or abscesses. This case report describes a man with aortic valve replacement one year ago and a recent carotid endarterectomy who was admitted with fever and neurological deficits. The workup permitted a diagnosis of fungal endocarditis, and the patient underwent a combined and aggressive treatment approach with antifungal therapy and surgery, with a successful replacement of the aortic valve. During hospitalization, the patient's neurological status deteriorated, and a cerebral abscess was discovered on the left frontal lobe. Despite the poor prognosis, the patient recovered slowly and was discharged from the hospital three months later. The present case highlights the high index of suspicion needed for the diagnosis and the need for a multidisciplinary team to approach these patients to achieve a positive outcome.

Hemophagocytic lymphohistiocytosis following an acute HIV infection, in a known immunosuppressed patient: A case report.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory condition with a wide range of causes, being frequently associated with infections in adults. The association between HLH and acute HIV infection has been rarely described. Case presentation: A 62-year-old male, with a past medical history of Henoch-Schönlein purpura under immunosuppressive treatment, presented with a two-week history of fever, asthenia, anorexia, cough and purpuric rash. Initial blood tests showed pancytopenia, elevated C-reactive protein and renal failure. Microbiological investigations were negative, but persistent fever and pancytopenia led to HLH suspicion. This diagnosis was supported by hyperferritinemia, hypertriglyceridemia, high soluble-interleukin-2 receptor levels and hepatosplenomegaly, fulfilling 5/8 diagnostic criteria of the Histiocyte Society-2004. Further investigation revealed a positive HIV-1 antibody and the patient reported recent sexual risks, with TCD4 + lymphocytes below 100/mL and HIV-1 viremia above 10 million copies/mL, confirming an acute HIV infection. Antiretroviral therapy (ART) and glucocorticoids were started with full clinical recovery. Conclusion: HLH occurrence can be obscured by the features of a primary disease and can mimic other clinic conditions. In this patient, the prompt identification of an acute HIV infection as the cause of HLH allowed the early initiation of antiretroviral treatment and corticosteroids, with an efficient control of the viral replication and inflammatory response, preventing a potentially fatal evolution.

Invasive Pulmonary Aspergillosis after COVID-19 Pneumonia.

COVID-19 emerged in China in late 2019 and quickly spread worldwide. The severe immunomodulation and depletion of lymphocytes caused by the virus and its therapy led to an increase in the incidence of superinfections. COVID-19-associated pulmonary aspergillosis (CAPA) is a new entity with increasing incidence and high associated mortality. We present the case of a 68-year-old patient admitted to our ward after recovering from severe COVID-19 pneumonia. Due to worsening of her clinical condition, chest computed tomography was performed and a lung abscess was documented with the identification of Aspergillus niger. Despite therapy with voriconazole, the patient's condition deteriorated, culminating in her death. LEARNING POINTS: COVID-19-associated pulmonary aspergillosis (CAPA) is a new entity with an increasing incidence.It is a serious and life-threatening complication in patients with severe COVID-19 even in the absence of the classic risk factors for invasive pulmonary aspergillosis.Clinical suspicion is crucial since a timely diagnosis and treatment have a major impact on prognosis.

Berardinelli-Seip Congenital Lipodystrophy Discovered Following a STEMI Event.

A 60-year-old man, with a history of familial lipodystrophy, hypertriglyceridaemia, hepatic steatosis and bone cysts, was admitted due an acute coronary event. Coronary angiography showed significant stenosis in the left anterior descending artery, which was treated. Transthoracic echocardiography showed a slightly dilated left ventricle with diffuse and heterogeneous thickening of its walls, slightly decreased left ventricular function and reduced global longitudinal strain. Due to these echocardiographic findings, cardiac magnetic resonance imaging was requested, which identified intramyocardial diffuse fibrosis of the basal septum and points of insertion of the left and right ventricles, without oedema, microvascular obstruction or myocardial infarction. Owing to the constellation of symptoms and distinctive features on cardiac imaging, a diagnosis of Berardinelli-Seip congenital lipodystrophy (BSCL) was suspected, which was confirmed through genetic testing of the pathogenic variants in BSCL2 and AGPAT2. BSCL is a rare autosomal recessive syndrome characterized by the congenital absence of adipose tissue and triglyceride deposition in other tissues, such as muscle, liver and heart. LEARNING POINTS: Berardinelli-Seip congenital lipodystrophy (BSCL) is a rare congenital lipodystrophy, with an incidence of 1-9 per million population, which is usually diagnosed at birth and is associated with pathogenic variants of the BSCL2 and AGPAT2 genes.Due to the absence of functional adipocytes, lipid storage occurs in other tissues, including skeletal muscle and liver.Diagnosis is based on the presence of three major or two major and two minor characteristics.

Legionella and SARS-CoV-2 Coinfection in a Patient With Pneumonia - An Outbreak in Northern Portugal.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has plagued virtually every continent and country, and Portugal is no exception. The high number of cases has caused a major burden on health services and obvious economic consequences, forcing an important reformulation in the health sectors' organization. In the past weeks, counties in the country's northern coastal region have reported an increasing number of Legionella cases, whose origin is yet to be determined. This exacerbates the already important pressure on the region's health facilities. We present a case of a patient diagnosed with Legionella pneumonia and concomitant coronavirus disease 2019 (COVID-19) pneumonia, highlighting the need for etiological investigation not only for common community agents but also for pandemic pathogens and regional outbreaks.