Cerebral venous thrombosis.

Cerebral venous thrombosis is part of the so-called thrombosis in unusual sites. It is defined as an occlusion in the cerebral venous territory. Its incidence is progressively increasing, especially in developing countries. It is more frequently observed in young women, with hormonal factors such as pregnancy or hormonal contraception being significant risk factors in the development of this condition. The clinical presentation will depend fundamentally on the topography of the thrombosis, with a confirmatory diagnosis based mainly on imaging tests. The treatment generally consists of anticoagulation, and other options may be considered depending on the severity of the case. Overall, the prognosis is better than that of other intracranial vascular disorders. This review describes the current evidence available regarding cerebral venous thrombosis.

Cerebral venous thrombosis in adults: a case series of 35 patients from a tertiary hospital.

OBJECTIVE: To describe the baseline characteristics, clinical presentation, imaging tests and outcomes, and identify potential prognostic factors in a cohort of patients diagnosed with cerebral venous thrombosis (CVT). PATIENTS AND METHODS: This retrospective, single-center, observational study included adult patients diagnosed with CVT from January 2016 to December 2020. The variables were reviewed using electronic medical records. RESULTS: A total of 35 patients were included, with a median age at diagnosis of 50.3 (+/- 17.8) years, and the majority being women (74.4%). Nearly 95% of the patients presented at least one risk factor for the development of CVT. Heparins were used for the acute phase in 97.1% of cases, with 75% of those being low molecular weight heparins.During the first two weeks, a compound event (death, intensive care unit admission, National Institute of Health Stroke Scale at discharge >3, CVT recurrence, major bleeding, or the presence of complications) occurred in 28.6% of patients (10 patients).Over the mean follow-up period of 3.3 years, 14.3% of the patients died (with only one death attributed to CVT), one patient experienced major bleeding, and no patients had a recurrence of CVT. CONCLUSIONS: In our cohort, CVT predominantly affected young women with at least one risk factor for its development. The presence of edema on CT and corticosteroid treatment were associated with a poor short-term prognosis. However, we observed a favorable long-term prognosis in terms of mortality, recurrence, and bleeding.

[Risk of thrombosis recurrence among patients with COVID-19- and surgery-associated venous thromboembolism]. / Riesgo de recurrencia de la trombosis en pacientes con tromboembolia venosa asociada a COVID-19 y a cirugía.

Introduction: Recent surgery is a well-known major transient risk factor for venous thromboembolism (VTE) due to the low risk of VTE recurrence after anticoagulation is discontinued. On the other hand, the risk of VTE recurrence among patients with COVID-19-associated VTE is unknown. This study aimed to compare the risk of VTE recurrence between patients with COVID-19- and surgery-associated VTE. Methods: A prospective observational single-center study was performed including consecutive patients diagnosed with VTE in a tertiary hospital from January 2020 to May 2022 and followed up for at least 90 days. Baseline characteristics, clinical presentation, and outcomes were assessed. The incidence of VTE recurrence, bleeding, and death was compared between both groups. Results: A total of 344 patients were included in the study: 111 patients with surgery-associated VTE and 233 patients with COVID-19-associated VTE. Patients with COVID-19-associated VTE were more frequently men (65.7% vs 48.6%, p = 0.003). VTE recurrence was 3% among COVID-19 patients and 5.4% among surgical patients, with no significant differences (p = 0.364). The incidence rate of recurrent VTE was 1.25 per 1000 person-months in COVID-19 patients and 2.29 person-months in surgical patients, without significant differences (p = 0.29). In the multivariate analysis, COVID-19 was associated with higher mortality (HR 2.34; 95% CI 1.19-4.58), but not with a higher risk of recurrence (HR 0.52; 95% CI 0.17-1.61). No differences were found in recurrence in the multivariate competing risk analysis (SHR 0.82; 95% CI 0.40 - 2.05). Conclusions: In patients with COVID-19 and surgery-associated VTE, the risk of recurrence was low, with no differences between both groups.

Risk of thrombosis recurrence among patients with COVID-19 and surgery-associated venous thromboembolism.

INTRODUCTION: Recent surgery is a well-known major transient risk factor for venous thromboembolism (VTE) due to the low risk of VTE recurrence after anticoagulation is discontinued. On the other hand, the risk of VTE recurrence among patients with COVID-19-associated VTE is unknown. This study aimed to compare the risk of VTE recurrence between patients with COVID-19- and surgery-associated VTE. METHODS: A prospective observational single-center study was performed including consecutive patients diagnosed with VTE in a tertiary hospital from January 2020 to May 2022 and followed up for at least 90 days. Baseline characteristics, clinical presentation, and outcomes were assessed. The incidence of VTE recurrence, bleeding, and death was compared between both groups. RESULTS: A total of 344 patients were included in the study: 111 patients with surgery-associated VTE and 233 patients with COVID-19-associated VTE. Patients with COVID-19-associated VTE were more frequently men (65.7% vs 48.6%, p =  0.003). VTE recurrence was 3% among COVID-19 patients and 5.4% among surgical patients, with no significant differences (p =  0.364). The incidence rate of recurrent VTE was 1.25 per 1000 person-months in COVID-19 patients and 2.29 person-months in surgical patients, without significant differences (p =  0.29). In the multivariate analysis, COVID-19 was associated with higher mortality (HR 2.34; 95% CI 1.19-4.58), but not with a higher risk of recurrence (HR 0.52; 95% CI 0.17-1.61). No differences were found in recurrence in the multivariate competing risk analysis (SHR 0.82; 95% CI 0.40-2.05). CONCLUSIONS: In patients with COVID-19 and surgery-associated VTE, the risk of recurrence was low, with no differences between both groups.

Niemann-Pick disease type-B: a unique case report with compound heterozygosity and complicated lipid management.

BACKGROUND: Niemann-Pick disease (NPD) is a rare autosomal recessive hereditary disease characterized by deficient activity of acid sphingomyelinase. CASE PRESENTATION: We present a case of NPD type B with a unique compound heterozygosity for SMPD1 (NM_000543.4:c.[84delC];[96G > A]) in which both mutations that induce an early stop codon are located before the second in-frame initiation codon. The clinical presentation of the patient is compatible with NPD type B. She was initially diagnosed of Gaucher Disease, but her altered lipid profile led to a clinical suspicion of NPD. Combined high doses of atorvastatin and ezetimibe were given to treat the severe hypercholesterolemia. CONCLUSIONS: The pharmacological management of the lipid profile in these patients is important. A unique compound mutation in SMPD1 gene is described.

Incidence of asymptomatic deep vein thrombosis in patients with COVID-19 pneumonia and elevated D-dimer levels.

AIM: An increased risk of venous thromboembolism (VTE) in patients with COVID-19 pneumonia admitted to intensive care unit (ICU) has been reported. Whether COVID-19 increases the risk of VTE in non-ICU wards remains unknown. We aimed to evaluate the burden of asymptomatic deep vein thrombosis (DVT) in COVID-19 patients with elevated D-dimer levels. METHOD: In this prospective study consecutive patients hospitalized in non-intensive care units with diagnosis of COVID-19 pneumonia and D-dimer > 1000 ng/ml were screened for asymptomatic DVT with complete compression doppler ultrasound (CCUS). The study was approved by the Institutional Ethics Committee. RESULTS: The study comprised 156 patients (65.4% male). All but three patients received standard doses of thromboprophylaxis. Median days of hospitalization until CCUS was 9 (IQR 5-17). CCUS was positive for DVT in 23 patients (14.7%), of whom only one was proximal DVT. Seven patients (4.5%) had bilateral distal DVT. Patients with DVT had higher median D-dimer levels: 4527 (IQR 1925-9144) ng/ml vs 2050 (IQR 1428-3235) ng/ml; p < 0.001. D-dimer levels > 1570 ng/ml were associated with asymptomatic DVT (OR 9.1; CI 95% 1.1-70.1). D-dimer showed an acceptable discriminative capacity (area under the ROC curve 0.72, 95% CI 0.61-0.84). CONCLUSION: In patients admitted with COVID-19 pneumonia and elevated D-dimer levels, the incidence of asymptomatic DVT is similar to that described in other series. Higher cut-off levels for D-dimer might be necessary for the diagnosis of DVT in COVID-19 patients.

Predictive value of D-dimer testing for the diagnosis of venous thrombosis in unusual locations: A systematic review.

BACKGROUND: The value of D-dimer testing for the diagnosis of thrombosis in unusual sites is not properly established and evidence is scarce. We performed a systematic review of the literature. METHODS: The search was conducted in MEDLINE and Cochrane Library for papers published in the last 10 years including different presentations of thrombosis in unusual sites. Twenty-three articles were included, from January 1, 2008, to December 31, 2018, comprising 3378 patients with thrombosis in unusual sites (upper extremity deep vein thrombosis, cerebral vein thrombosis and splanchnic vein thrombosis). The Newcastle-Ottawa scale was used to assess the quality of the studies. RESULTS: Two articles were related to upper extremity thrombosis, showing a high sensitivity and negative predictive value for D-dimer testing. Twelve articles concerned cerebral vein thrombosis, concluding that the timing of D-dimer testing was important, and that patients with a shorter duration of symptoms showed higher D-dimer levels. Sensitivity and specificity in these patients ranged from 58% to 97% and from 77% to 97.5%, respectively. Nine articles were related to splanchnic vein thrombosis. One described a population of patients with mesenteric venous thrombosis, and the rest included patients with portal vein thrombosis. The D-dimer testing methods and the proposed cut-off levels were remarkably different among the included studies. CONCLUSION: D-dimer testing should not be currently recommended for the diagnosis of thrombosis in unusual sites as a first line diagnostic tool. The development of algorithms combining biomarkers such as D-dimer and clinical decision tools could improve the diagnosis.

Monitoring anti-Xa levels in patients with cancer-associated venous thromboembolism treated with bemiparin.

OBJECTIVE: To analyze the relationship between therapeutic (weight-adjusted) dose of bemiparin and anti-Xa activity in patients with venous thromboembolism (VTE) and cancer in comparison with a cohort of patients with VTE without cancer, and its relationship with outcomes. MATERIALS AND METHODS: This is a prospective cohort study that comprised a cohort of patients with cancer-associated VTE and a cohort of non-cancer patients with VTE, all of them treated with bemiparin. The ethics committee approved the study and informed consent was obtained from the patients. RESULTS: One hundred patients were included (52 with cancer and 48 without cancer), with a median follow-up of 9.8 months. Mean anti-Xa activity was 0.89 (± 0.33) UI/mL in oncological patients and 0.83 (± 0.30) UI/mL in non-cancer patients (mean difference - 0.05 95% CI - 0.18; 0.06). A multiple linear regression model showed that anti-Xa peak was associated with the dose/kg independently of possible confounding variables (presence of cancer, age, sex and eGFR-estimated Glomerular Filtration Rate), in a way that for every 1 UI of dose/kg increase, the anti-Xa peak activity increased 0.006 UI/mL (95% CI 0.003; 0.009) (p < 0.001). The predictive capacity of anti-Xa peak in the oncology cohort showed an area under the ROC curve of 0.46 (95% CI 0.24-0.68), 0.70 (95% CI 0.49-0.91) and 0.74 (95% CI 0.44-0.94) for death, first bleeding and recurrence of VTE, respectively, and none was statistically significant. CONCLUSION: In patients with venous thromboembolism treated with bemiparin, anti-Xa levels were not influenced by the presence of cancer.

Massive generalized crystal-storing histiocytosis associated with extracellular crystalline nephropathy: clinical, immunohistochemical, and ultrastructural studies of a unique disorder and review of the literature.

Crystal-storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of nonneoplastic histiocytes containing intracytoplasmic crystallized immunoglobulins. In most cases, there is an associated underlying lymphoplasmacytic neoplasm expressing Ig kappa light chain. About 131 cases of CSH have been identified. There is a localized and a generalized form of CSH and it can involve several sites including bone marrow, lungs, lymph nodes, liver, spleen, gastrointestinal tract, and kidney. Generalized CSH is less frequent and involves multiple organs and tends to have a worst prognosis than localized CSH. Around 20 cases of renal involvement in CSH have been reported so far. Paraprotein-induced crystalline nephropathy can be divided into two categories based on whether the crystals in the kidney are intracellular (including light chain proximal tubulopathy with crystals and CSH) or extracellular (including the crystalline variant of myeloma cast nephropathy and crystalglobulin-induced nephropathy). The former tends to present with slowly worsening kidney dysfunction and generally has a good prognosis, whereas the latter usually presents with rapidly progressive renal failure and is associated with poor renal outcome. We present a case of generalized CSH associated with extracellular crystalline nephropathy with a fulminant and fatal clinical course. Kappa light-chain crystals were found exclusively extracellularly within the tubular lumen, not within the tubular epithelial cells nor the histiocytes, and the massive presence of those precipitates led to the acute renal failure. Consequently, we review the renal involvement in CSH in the literature and discuss the unique mechanism of renal injury in this case.

Fifth-year internal medicine residents in Spain: A survey on education, assessment of competencies and job perspectives. / Residentes de medicina interna de quinto año en España: encuesta sobre educación, evaluación de competencias y perspectivas de empleo.

BACKGROUND: Specialist training is based on the gradual acquisition of expertise, skills and responsibilities. The aim of this study is to determine the opinion of residents regarding their training. MATERIAL AND METHODS: This was a cross-sectional descriptive study based on an online survey of 5th-year residents during February and March 2017. RESULTS: A total of 194 residents (62.8% of the total) responded to the survey, 62.9% of whom were women and 50% of whom were younger than 30years, representing hospitals from all levels and from the 17 autonomous communities. More than 80% of the residents choose the specialty once again and believed that the duration of the residence was appropriate; however, 76.3% would eliminate some of their rotations. Most of the residents did not know the objectives of each rotation, and 37.1% felt they were not adequately supervised. Some 82.5% would change the evaluation system, and 68.0% would favour performing an excellence test. Most of the residents had published at least one article or performed one presentation at a congress; however, only 27.8% had completed a doctoral thesis. Although 74.7% of the internists believed they would find employment, only 28.4% had an offer 1month after completing their residence. CONCLUSIONS: The residents are satisfied with their training, although there is still a deficit in supervision and dissatisfaction with the method of assessing their knowledge and the precarious job market during the first year for specialists.

Getting to know internal medicine mentors: new training requirements. / Conociendo a los tutores de Medicina Interna: nuevas necesidades para la formación.

INTRODUCTION AND OBJECTIVES: Mentors are responsible for planning the residents' learning. The aim of this study was to determine the situation of internal medicine mentors in Spain and detect areas of improvement that can facilitate their work. MATERIAL AND METHODS: Online surveys were sent to internal medicine mentors from May to July 2017, the results of which were subsequently analysed. RESULTS: A total of 110 mentors from 13 autonomous communities and from hospitals of all levels with courses in internal medicine responded to the survey. Of these mentors, 63 were men (57.3%), and the mean age was 48 years. The mean experience as mentors was 8.5 years. Some 88.2% of the cases had a ratio of 5 residents to 1 mentor; 46% of the mentors believed this ratio should be decreased to optimize their work. A third of the mentors were chosen by the heads of the department, and 30% had not previously taken courses on training. The mentor-resident interview was used by most mentors (96.4%) as a communication tool. A quarter of the rotations were not planned by the mentors, and only half had contact with the centres where the residents performed the external rotations. Sixty-one percent of the mentors were of the opinion that resident assessments were not conducted properly, with very little use of the new assessment tools. CONCLUSIONS: Reducing the mentor-resident ratio and adding training in assessment techniques and learning development could improve the quality of the mentoring.