Objective The presence of deep venous thrombosis (DVT) in a cryptogenic stroke (CS) patient with a right-to-left shunt (RLS) may lead to the development of paradoxical embolism. The aim of the present was to investigate the prevalence of DVT and pulmonary embolism (PE) in CS patients and the clinical features of CS in relation to DVT location and the presence of PE.
Methods The medical records of 903 patients with cerebral infarction were retrospectively reviewed. For patients with a diagnosis of CS, contrast saline transcranial color-coded sonography was performed to identify an RLS. DVT and PE were assessed by duplex ultrasonography and/or contrast-enhanced computed tomography. Proximal DVT (P-DVT) was defined as DVT in the popliteal, femoral, or iliac veins, and distal DVT (D-DVT) was defined as DVT at other locations. The patients were divided into three groups: CS with P-DVT and/or PE (P-DVT/PE) group; CS with D-DVT (D-DVT) group; and CS without DVT (no DVT) group.
Results Seventy-two (37%) of 194 patients with CS had an RLS. The median time to first DVT examination from stroke onset was three days. Twenty-nine percent of CS patients with an RLS had DVT. The P-DVT/PE group comprised 8.3% of the CS patients with an RLS and included a larger number of patients with multi-territory infarction than the D-DVT group. The D-DVT and P-DVT/PE groups tended to be female and older, while the P-DVT/PE group tended to have pre-stroke disability.
Conclusion CS patients, especially those with multi-territory lesions, should be immediately examined for DVT and PE.
We herein report two cases of eosinophilic granulomatosis with polyangiitis (EGPA) initially diagnosed as eosinophilic gastroenteritis (EGE) based solely on endoscopic biopsy results. One year after the EGE diagnosis, one patient presented with multiple purpura, and skin biopsy findings resulted in a change of the diagnosis to EGPA. In another patient, multiple skin and colonic ulcerations emerged eight years after the diagnosis of EGE, at which time histological examinations of endoscopic biopsy specimens revealed vasculitis, and the diagnosis was changed to EGPA. Physicians should be aware of the possible existence of EGPA in cases diagnosed as EGE.
Hepatitis-associated aplastic anemia (HAAA) is a variant of acquired aplastic anemia and characterized by bone marrow failure that follows the development of acute hepatitis. We herein report a rare case of HAAA with rapid progression of liver fibrosis due to repeated hepatitis. A pathological examination of liver specimens revealed liver fibrosis progression over a short period. Immunosuppressive therapy with cyclosporine effectively cured both the pancytopenia and hepatitis. Our case suggests that the pathological examination of the liver tissue is useful for determining a treatment plan and that immunosuppressive therapy is a promising treatment for both aplastic anemia and persistent hepatitis.
A 69-year-old woman with a family history of pancreatic cancer was referred because of imaging changes of a pancreas cyst. Magnetic resonance cholangiopancreatography showed a faintly dilated main pancreatic duct and a pancreas body cyst that had changed rapidly over the past year. Computed tomography demonstrated an emerging enhancing lesion in the pancreatic cyst. Endoscopic ultrasonography revealed an irregular-margined, heterogeneous-echoic pancreatic mass, without findings of early chronic pancreatitis. She underwent distal pancreatectomy. A histologic examination of the resected specimen revealed invasive adenocarcinoma with numerous multicentric foci of pancreatic intraepithelial neoplasia (PanIN), including high-grade PanIN, apparently separate from the main cancer.
Hepatic encephalopathy (HE) is a significant symptom of decompensated liver cirrhosis. Occlusion of portosystemic shunts is used to treat refractory HE. Nevertheless, these treatments often cause adverse events, such as ascites and esophageal varices. We treated a 57-year-old man with refractory HE using shunt-preserving disconnection of the portal and systemic circulation (SPDPS). After SPDPS, there were no obvious complications, and the patient's ammonia level significantly decreased. To date, the patient has not experienced recurrent HE. SPDPS appears to be a safe and effective treatment method for portosystemic encephalopathy.
Transcatheter aortic valve implantation (TAVI) is widely accepted as the treatment for patient with severe aortic stenosis (AS) whose prognosis may be over one year; however, there is no consensus concerning extremely high-risk patients whose prognosis may not exceed one year. We herein report a highly frail patient with severe AS complicated with transthyretin-type cardiac amyloidosis who had a very poor prognosis. Given his condition, we treated him by percutaneous antegrade balloon aortic valvuloplasty (A-BAV) instead of TAVI. A-BAV may be a beneficial option for treating extremely high-risk severe AS patients, including those with cardiac amyloidosis.
A 92-year-old man was referred to our hospital with decompensated heart failure. He was treated with diuretics and inotropic agents, but the clinical response was unsatisfactory. Echocardiography incidentally revealed an atrial septal defect (ASD) with a significant left-to-right shunt and pulmonary-to-systemic-blood-flow ratio of 2.36. Because the ASD was considered to be the primary cause of his treatment-resistant heart failure, the patient underwent transcatheter ASD closure. Shortly after shunt closure, his hemodynamics significantly improved, and he was discharged nine days later. This is an extremely rare case of a nonagenarian who showed an excellent clinical course after transcatheter ASD closure.
We herein report a 62-year-old man with idiopathic pulmonary fibrosis who developed remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome during follow-up. Pulmonary infiltrations were detected concomitantly with the development of RS3PE syndrome, and prednisolone improved both the pulmonary and extrapulmonary lesions. Recognizing the pulmonary manifestations of RS3PE syndrome is necessary to provide an appropriate diagnosis and disease management.
A 77-year-old man with anemia who had undergone 2 abdominal surgeries for colon and gastric cancer experienced dyspnea after swallowing a patency capsule before endoscopy for investigating the cause of anemia. Chest radiography and computed tomography revealed that the patency capsule was located within the bronchus intermedius. It was successfully removed by flexible bronchoscopy. The balloon was placed over the capsule and inflated. Subsequently, the catheter was pulled, while thus dragging the capsule with it and preventing its destruction. In cases of patency capsule aspiration, the capsule must be removed without deformity, before it causes inflammation by releasing barium into the airway.
Immune checkpoint inhibitors have changed the landscape of classic cancer treatment. However, their use is associated with the emergence of new adverse events. An elderly man with rheumatoid arthritis was started on pembrolizumab for newly diagnosed advanced lung cancer. He subsequently developed hemophagocytic lymphohistiocytosis (HLH), which is potentially fatal but has not been properly established as an immune checkpoint inhibition-induced event. We herein report the case of a patient with pembrolizumab-induced HLH.
Secondary pulmonary alveolar proteinosis (sPAP) is a complication of myelodysplastic syndrome (MDS). A 60-year-old woman was diagnosed with MDS with excess blasts-1. Fifty-four months after the initial diagnosis, treatment with azacitidine was initiated. Seventy-three months after the diagnosis, a bone marrow examination revealed increased myeloblasts, at which time computed tomography showed diffuse ground-glass opacities and interlobular septal thickening in the bilateral lower lung fields. A lung biopsy revealed the presence of PAP; therefore, the clinical diagnosis of MDS/sPAP was confirmed. Careful attention should be paid to the development of sPAP in MDS patients with pulmonary lesions during azacitidine treatment.
Aortic arch aneurysm (AAA) is a rare involvement in Behçet disease (BD). It is often life-threatening, yet few reports related to the treatment of AAA have been published. We herein report a 27-year-old woman with AAA caused by vascular BD. She was initially treated with prednisolone 1 mg/kg/d. However, the inflammation had not subsided after three weeks, so infliximab (IFX) was added for relief. After IFX administration, the C-reactive protein level normalized, and computed tomography at three months after therapeutic intervention revealed that the aneurysm had disappeared. This case suggests that early induction of IFX might be effective for aortic aneurysm in BD.
A 25-year-old woman had convulsions and disturbance of consciousness. Head magnetic resonance imaging (MRI) showed punctate areas in the occipital lobes with increased signals on T2-weighted imaging. The MRI abnormalities responded well to steroid pulse therapy, so we made a diagnosis of posterior reversible encephalopathy syndrome (PRES). Three months later, she developed a fever and dyspnea. Chest computed tomography revealed marked thickness of the tracheal and bronchial wall, and bronchoscopy showed a cobble-stone appearance of the tracheal mucosa, indicative of relapsing polychondritis (RPC). We consider that PRES had developed due to autoimmune vasculitis in the brain with RPC.
A 58-year-old Japanese woman with herpes zoster developed Behçet's disease (BD) with symptoms including orthostatic intolerance as an autonomic disorder. Multiple immune-suppressive therapies and a β-blocker successfully controlled both the disease activity of BD and the autonomic disorders. A cytokine multiplex analysis of her serum revealed the elevation of proinflammatory cytokines [interleukin (IL)-1, IL-6, IL-12, tumor necrosis factor alfa (TNFα), and interferon gamma (IFN-γ)] and a low IL-10 concentration. IL-10 production is reported to be important for defense against herpes zoster virus (VZV). Insufficient IL-10 production is reported in BD. The reactivation of VZV with this cytokine profile suggests that BD will develop with various symptoms, including severe autonomic disorders.
Nivolumab, an antibody against human programmed cell death 1 (PD-1), enhances pre-existing immune responses and has antitumor activity. However, it may also cause undesirable immune-related adverse events (irAEs), such as anti-PD-1-related colitis. In addition, Nivolumab can worsen pre-existing autoimmune diseases. Ulcerative colitis (UC) is a chronic inflammatory disease of the colon. Its exact cause is unknown, but it may involve the dysregulation of the mucosal immune response. Thus, it is of great interest whether nivolumab can affect UC activity. This is the first report of a patient with epipharyngeal carcinoma and ulcerative colitis who was confirmed to have been safely treated with nivolumab based on autopsy findings.