Hypercalcemia Heralding Pneumocystis jirovecii Pneumonia in an HIV-Seronegative Patient with Diffuse Cutaneous Systemic Sclerosis.

Pneumocystis pneumonia (PCP) is a life-threatening fungal infection occurring in immunocompromised patients such as HIV-positive patients with low CD4 cell count or patients under heavy immunosuppressive therapy. We report the case of a 59-year-old male with severe diffuse cutaneous systemic sclerosis presenting with asthenia, dry cough and worsening shortness of breath for the last 15 days. Biological studies were remarkable for PTH-independent severe hypercalcemia with low 25-hydroxyvitamin D and a paradoxically elevated 1,25-dihydroxyvitamin D. Early bronchoalveolar lavage allowed for PCP diagnosis and targeted treatment. We discuss the underlying physiopathology and difficulties regarding prophylaxis and treatment.

A Cross-sectional Real-life Study of the Prevalence, Severity, and Determinants of Metabolic Dysfunction-associated Fatty Liver Disease in Type 2 Diabetes Patients.

Background and Aims: Most data on liver assessment in type 2 diabetes mellitus (T2DM) patients are from retrospective cohorts with selection bias. We aimed at appraising the feasibility, results, and benefits of an outpatient systematic noninvasive screening for metabolic dysfunction-associated fatty liver disease (MAFLD) severity and determinants in T2DM patients. Methods: We conducted a 50-week cross-sectional study enrolling adult T2DM outpatients from a diabetes clinic. An algorithm based on guidelines was applied using simple bioclinical scores and, if applicable, ultrasound and/or elastometry. Results: Two hundred and thirteen patients were included. Mean age and body mass index were 62 years and 31 kg/m2 and 29% of patients had abnormal transaminase levels. The acceptance rate of additional liver examinations was 92%. The prevalence of MAFLD, advanced fibrosis and cirrhosis was 87%, 11%, and 4%, respectively. More than half of the cases of advanced fibrosis had not been suspected and were detected by this screening. MAFLD was associated with poor glycemic control, elevated transaminases, low HDL-C and the absence of peripheral arterial disease. Advanced fibrosis was linked to high waist circumference and excessive alcohol consumption, which should be interpreted with caution owing to the small number of patients reporting excessive consumption. Conclusions: Simple bioclinical tools allowed routine triage of T2DM patients for MAFLD severity, with high adherence of high-risk patients to subsequent noninvasive exams.

Nonbacterial thrombotic endocarditis in a patient with gastric cancer and SARS-CoV-2 infection.

Nonbacterial thrombotic endocarditis, formerly known as marantic endocarditis, is a very rare complication of advanced malignancy and other hypercoagulable states in which sterile, fibrin vegetations develop on heart valve leaflets. The most common malignancies associated with this entity are lung, pancreatic and gastric cancer. It has also been described as a presentation of COVID-19, which is known to be frequently complicated with coagulopathy and thromboembolic events. We report the case of a 62 year-old female patient newly diagnosed with stage IV gastric cancer and acute SARS-CoV-2 infection, presenting with confusion and homonymous hemianopsia in the setting of multiple acute ischemic strokes complicating a nonbacterial thrombotic mitral endocarditis. Herein, we discuss the underlying pathophysiology and make the hypothesis that SARS-CoV-2 infection could have participated in the pathogenesis of nonbacterial thrombotic endocarditis in our patient suffering from a gastric cancer.

Lessons for the clinical nephrologist: an uncommon cause of pulmonary-renal syndrome.

Pulmonary-renal syndrome refers to the combination of elevated plasma creatinine concentration and/or abnormal urinalysis with diffuse alveolar hemorrhage, and involves both an urgent diagnostic approach and care. We report the case of a 24-year-old man presenting with diffuse alveolar hemorrhage as well as a nephritic syndrome associating kidney failure, moderate hypertension, hematuria and selective glomerular proteinuria. The initial high suspicion of anti-glomerular basement membrane (GBM) disease or ANCA-associated vasculitis justified intravenous pulse-corticotherapy in association with plasma exchange. Renal biopsy was remarkable for an IgA nephropathy, lesions of active thrombotic microangiopathy (TMA) and a positive staining for complement factor C4d. Because anti-GBM and ANCA antibodies returned negative, plasma exchange was discontinued, but oral corticosteroids were maintained to prevent alveolar hemorrhage recurrence. In the absence of renal function recovery, hemodialysis was initiated. TMA lesions are frequently seen in IgA nephropathy and are associated with a poorer prognosis. Complement activation seems to be involved in the development of those lesions and contributes to disease progression. Conversely, alveolar hemorrhage in the setting of IgA nephropathy is uncommon. It is thought to result from non-specific mucosal hemorrhage, an immune complex mediated basement membrane damage and an IgA-mediated capillaritis against basement membrane antigens.

Dabigatran etexilate in the treatment of localized intravascular coagulopathy associated with venous malformations.

INTRODUCTION: Venous malformations (VM) are congenital slow-flow vascular malformations. A common complication is localized intravascular coagulopathy (LIC), which results from chronic, localized, intraluminal activation and consumption of clotting factors and is characterized by elevated D-dimer and, in severe cases, decreased fibrinogen levels. Therapeutic options include anti-coagulation by Low Molecular Weight Heparin (LMWH) which has been reported to improve pain, decrease thrombosis, improve laboratory parameters and reduce hemorrhagic complications. However, the use of subcutaneous injections of LMWH has several limitations and the indications for therapy remain controversial. MATERIALS AND METHODS: We present the first small case series of 3 patients with VM-LIC in whom a long-term treatment by dabigatran etexilate, a direct thrombin inhibitor, was initiated in the setting of ambulatory hemostasis consultations. RESULTS: Clinically, drug tolerance was excellent in all 3 patients. Moderate hemorrhagic complaints occurred in 2 out of 3 patients but there was no thromboembolic event under dabigatran therapy. Dabigatran provided symptomatic relief on pain, swelling and heaviness feeling. Biologically, dabigatran treatment showed various modifications in D-dimers level but the fibrinogen level and platelet count remained unchanged. CONCLUSION: In conclusion, dabigatran etexilate may provide a viable and more convenient alternative to LMWH in the long-term treatment of VM-LIC. The dose has to be tailored in each patient in function of the bleeding tendency and laboratory hemostasis parameters.

Systemic thrombolysis and endovascular thrombectomy in severe acute ischemic stroke after dabigatran reversal with idarucizumab.

Patients presenting with an acute ischemic stroke despite dabigatran therapy (last intake <24 h or unknown) should be evaluated for reversal by idarucizumab, making them eligible for safe and effective intravenous thrombolysis. It has been shown to be feasible, well-tolerated, and easy to manage in an emergency room or stroke unit.

The second case of a young man with L-arginine-induced acute pancreatitis.

BACKGROUND: Dietary supplementation of arginine has been used by numerous world-class athletes and professional bodybuilders over the past 30 years. L-Arginine indeed enhances muscular power and general performance via maintaining ATP level. However, L-arginine is also known to induce acute pancreatitis in murine models. CASE REPORT: We report the case of young man presenting with upper abdominal pain and increased serum lipase levels. Contrast-enhanced computed tomography confirms a mild acute pancreatitis. Common etiologies have been ruled out and toxicological anamnestic screening reveals the intake of protein powder. This is, to the best of our knowledge, the second case in human of arginine-induced acute pancreatitis. CONCLUSION: This case report suggests that every patient presenting with acute pancreatitis without obvious etiology should be evaluated for the intake of toxics other than alcohol, including L-arginine.

Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature.

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled. Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.