A 64-Year-Old Woman with Chest Pain, Limb Weakness, and Endometrial Cancer

Necrotizing autoimmune myopathy (NAM) is a rare subgroup of idiopathic inflammatory myopathies (IIM). This pathology usually affects proximal limb muscles and in some cases the myocardium. Patients usually display proximal limb weakness. Muscular biopsy is required to confirm the diagnosis. We report the case of a 64-year-old woman with an atypical first presentation of NAM, manifested by chest pain in the context of metastatic endometrial cancer. The diagnosis of NAM was however made when she returned a second time with proximal limb weakness. A treatment with prednisone was then initiated, to which rituximab was rapidly associated, beside a specific chemotherapy

Disseminated tuberculosis presenting with polymorphonuclear effusion and septic shock in an HIV-seropositive patient: a case report

<p>Abstract</p> <p>Introduction</p> <p>Because a substantial number of patients present with few or atypical symptoms, the recognition of tuberculosis remains challenging. Disseminated tuberculosis presenting with septic shock has already been described in some case reports, but, to the best of our knowledge, it has never been associated with polymorphonuclear effusion.</p> <p>Case presentation</p> <p>We describe the case of a 27-year-old man from western Africa who was seropositive for human immunodeficiency virus. He presented with pleural and abdominal polymorphonuclear effusions and quickly developed septic shock due to disseminated <it>Mycobacterium tuberculosis </it>infection leading to multiple organ failure and death.</p> <p>Conclusion</p> <p>In high-risk patients, <it>Mycobacterium tuberculosis </it>infection should be considered even in exceptional clinical presentations, such as septic shock and polymorphonuclear effusions.</p

Establishing correlations between normal pancreatic and submandibular gland ducts

Background The objectives of this study were to evaluate the relationship between ductal morphometry and ramification patterns in the submandibular gland and pancreas in order to validate their common fractal dimension. Methods X-ray ductography with software-aided morphometry were obtained by injecting barium sulphate in the ducts of post-mortem submandibular gland and pancreas specimens harvested from 42 adult individuals. Results Three cases were excluded from the study because of underlying pathology. There was a significant correlation between the length of the main pancreatic duct (MPD) and the intraglandular portion of the right submandibular duct (SMD) (r = 0.3616; p = 0.028), and left SMD (r = 0.595; p  0.05). Both dimensions of the SMD showed a significant right-left correlation (p < 0.05). The number of MPD side branches (mean = 37) correlated with the number of side branches of left SMD, but not with the right one (mean = 9). Tortuosity was observed in 54% of the MPD, 32% of the right SMD, and 24% of the left SMD, with mutual association only between the two salivary glands. Conclusions Although the length of intraglandular SMD and MPD correlate, other morphometric ductal features do not, thus suggesting a more complex relationship between the two digestive glands

Ex vivo evaluation of an atherosclerotic human coronary artery via histology and high-resolution hard X-ray tomography

Atherosclerotic arteries exhibit characteristic constrictions and substantial deviations from cylindrical shape. Therefore, determining the artery’s cross-section along the centerline is challenging, although high-resolution isotropic three-dimensional data are available. Herein, we apply high-resolution computed tomography in absorption and phase to a plaque-containing human artery post-mortem, through the course of the preparation stages for histology. We identify the impact of paraffin embedding and decalcification on the artery lumen. For automatic extraction of lumen’s cross-section along centerline we present a dedicated pipeline. Comparing fixated tissue before and after paraffin embedding gives rise to shape changes with lumen reduction to 50–80%. The histological slicing induces further deformations with respect to tomography. Data acquired after decalcification show debris unintentionally distributed within the vessel preventing the reliable automatic lumen segmentation. Comparing tomography of laboratory- and synchrotron-radiation-based X rays by means of joint histogram analysis leads us to conclude that advanced desktop tomography is capable of quantifying the artery’s lumen as an essential input for blood flow simulations. The results indicate that the most reliable lumen quantification is achieved by imaging the non-decalcified specimen fixed in formalin, using phase contrast modality and a dedicated processing pipeline. This study focusses on a methodology to quantitatively evaluate diseased artery segments post-mortem and provides unique structural parameters on the treatment-induced local shrinkage, which will be the basis of future studies on the flow in vessels affected by constrictions

Fatal subarachnoid hemorrhage following ischemia in vertebrobasilar dolichoectasia

Vertebrobasilar dolichoectasia (VBD) is a chronic disorder with various cerebrovascular and compressive manifestations, involving subarachnoid hemorrhage (SAH). Occurrence of SAH shortly after worsening of clinical VBD symptoms has occasionally been reported. The goal of the study was to examine this association, in particular its pathophysiology, clinical precursor signs, time course, and outcome. To this end, in a retrospective multicenter study, we analyzed 20 patients with VBD and SAH in regard to preceding clinical symptoms, presence of vertebrobasilar thrombosis and ischemia, outcome and neuropathological correlates. Median age of the 7 female and 13 male patients was 70 years (interquartile range [IQR] 18.3 years). Fourteen patients (70%) presented with new or acutely worsening posterior fossa signs at a median of 3 days prior to SAH (IQR 2, range 0.5-14). A thrombus within the VBD was detected in 12 patients (60%). Thrombus formation was associated with clinical deterioration (χ 2 = 4.38, P = 0.04) and ponto-cerebellar ischemia (χ 2 = 8.09, P = 0.005). During follow-up after SAH, 13 patients (65%) died, after a median survival time of 24 hours (IQR 66.2, range 2-264 hours), with a significant association between proven ponto-cerebellar ischemia and case fatality (χ 2 = 6.24, P = 0.01). The data establish an association between clinical deterioration in patients with VBD, vertebrobasilar ischemia, and subsequent SAH. Antithrombotic treatment after deterioration appears controversial and SAH outcome is frequently fatal. Our data also indicate a short window of 3 days that may allow for evaluating interventional treatment, preferably within randomized trials

L'interniste face aux maladies rares : quand y penser ? L'exemple des maladies mitochondriales

Rare Diseases, defined by a prevalence of less than 1 per 2000 persons, affect 36 million people in Europe, 500 000 in Switzerland, corresponding to 6-8% of the general population. 7000 rare diseases are currently recorded.Mitochondrial diseases are a heterogeneous group of genetic diseases. They are characterized by intracellular failure of energy production and affect predominantly energy-dependent tissues. The clinical presentation is not always suggestive, particularly in adulthood. In order to reach the diagnosis, a prerequisite is to think of them. In this article, we will focus on the clinical aspects of mitochondrial disorders in order to give the internist simple tools on how not to miss those rare diseases in his daily practice

Complementary X-ray tomography techniques for histology-validated 3D imaging of soft and hard tissues using plaque-containing blood vessels as examples

A key problem in X-ray computed tomography is choosing photon energies for postmortem specimens containing both soft and hard tissues. Increasing X-ray energy reduces image artifacts from highly absorbing hard tissues including plaque, but it simultaneously decreases contrast in soft tissues including the endothelium. Therefore, identifying the lumen within plaque-containing vessels is challenging. Destructive histology, the gold standard for tissue evaluation, reaches submicron resolution in two dimensions, whereas slice thickness limits spatial resolution in the third. We present a protocol to systematically analyze heterogeneous tissues containing weakly and highly absorbing components in the original wet state, postmortem. Taking the example of atherosclerotic human coronary arteries, the successively acquired 3D data of benchtop and synchrotron radiation-based tomography are validated by histology. The entire protocol requires ∼20 working days, enables differentiation between plaque, muscle and fat tissues without using contrast agents and permits blood flow simulations in vessels with plaque-induced constrictions

Acute disseminated fatal toxoplasmosis after haploidentical stem cell transplantation despite atovaquone prophylaxis in a young man

We describe a case of fatal acute disseminated breakthrough toxoplasmosis in a 19-year-old adolescent after haploidentical hematopoietic stem cell transplantation for acute lymphoblastic leukemia despite continued atovaquone prophylaxis. Diagnosis was at necropsy, and confirmed by postmortem polymerase chain reaction analysis in plasma. This report illustrates the need for protozoal monitoring despite atovaquone prophylaxis, in severely immunocompromised patients with intolerance to standard treatment

ChatGPT in glioma adjuvant therapy decision making: ready to assume the role of a doctor in the tumour board?

Objective To evaluate ChatGPT‘s performance in brain glioma adjuvant therapy decision-making.Methods We randomly selected 10 patients with brain gliomas discussed at our institution’s central nervous system tumour board (CNS TB). Patients’ clinical status, surgical outcome, textual imaging information and immuno-pathology results were provided to ChatGPT V.3.5 and seven CNS tumour experts. The chatbot was asked to give the adjuvant treatment choice, and the regimen while considering the patient’s functional status. The experts rated the artificial intelligence-based recommendations from 0 (complete disagreement) to 10 (complete agreement). An intraclass correlation coefficient agreement (ICC) was used to measure the inter-rater agreement.Results Eight patients (80%) met the criteria for glioblastoma and two (20%) were low-grade gliomas. The experts rated the quality of ChatGPT recommendations as poor for diagnosis (median 3, IQR 1–7.8, ICC 0.9, 95% CI 0.7 to 1.0), good for treatment recommendation (7, IQR 6–8, ICC 0.8, 95% CI 0.4 to 0.9), good for therapy regimen (7, IQR 4–8, ICC 0.8, 95% CI 0.5 to 0.9), moderate for functional status consideration (6, IQR 1–7, ICC 0.7, 95% CI 0.3 to 0.9) and moderate for overall agreement with the recommendations (5, IQR 3–7, ICC 0.7, 95% CI 0.3 to 0.9). No differences were observed between the glioblastomas and low-grade glioma ratings.Conclusions ChatGPT performed poorly in classifying glioma types but was good for adjuvant treatment recommendations as evaluated by CNS TB experts. Even though the ChatGPT lacks the precision to replace expert opinion, it may serve as a promising supplemental tool within a human-in-the-loop approach