Eiji Hiraoka

Activated RhoA Stimulates c-fos Gene Expression in Myocardial Cells

Rho regulates various cell functions, including cell morphology and motility. However, the functional role of Rho on the signaling pathway in myocardial cells (MCs) is unknown. In the present study, we attempted to explore the mode of Rho action for c-fos gene expression in MCs. Expression of the c-fos promoter/enhancer linked to the luciferase reporter gene (c-fos luciferase) was stimulated by the wild type of RhoA and the point-mutated active form of RhoA (RhoA Val14) but not the biologically inactive effector domain mutant of RhoA. Rho GDP dissociation inhibitor inhibited the action of RhoA on c-fos luciferase expression. The deletion analysis revealed that the c-fos serum response element (SRE) and the 12-O-tetradecanoylphorbol-13-acetate response element (TRE) mainly account for c-fos luciferase expression by RhoA Val14. The c-fos SRE mutant, which contains an intact binding site for the serum response factor but lacks the ternary complex factor binding site, was activated by RhoA Val14. The action of RhoA Val14 on c-fos luciferase expression was not inhibited by downregulation of protein kinase C, protein kinase C inhibitors, or tyrosine kinase inhibitors. These results indicate that activated RhoA stimulates c-fos gene expression through the c-fos SRE and TRE and that the signaling pathway from activated RhoA to the c-fos promoter/enhancer is independent of these inhibitor-sensitive pathways in MCs.

Marked effect and steroid-sparing ability of anakinra on a patient with refractory adult-onset Still’s disease

We report a case of refractory adult-onset Still’s disease (AOSD) successfully controlled with anakinra, an interleukin-1 (IL-1) receptor antagonist. The patient was a 23-year-old Japanese woman with AOSD who could not be induced into remission despite of two courses of pulsed methylprednisolone followed by high-dose glucocorticoid administration in conjunction with high-dose intravenously administered γ-globulin and methotrexate. To the best of our knowledge, this is the first case report in Japan of AOSD remission induced with anakinra.

Severe Sepsis due to Otogenic Pneumococcal Meningitis with Pneumocephalus without Meningeal Symptoms.

The absence of meningeal signs and symptoms is rare in patients with bacterial meningitis and may lead to a delay in diagnosis and treatment. Furthermore, the onset of bacterial meningitis associated with pneumocephalus is a rare complication of ear infections. We herein report a rare case of otogenic meningitis complicated by pneumocephalus that was initially missed due to the absence of typical meningeal signs and symptoms and later diagnosed correctly based on a thorough review of the patients systems.

Progressive Ischemic Stroke due to Thyroid Storm-Associated Cerebral Venous Thrombosis

Patient: Female, 49 Final Diagnosis: Cerebral venous thrombosis Symptoms: Altered mental state • weakness in limbs Medication: — Clinical Procedure: — Specialty: Endocrinology and Metabolic Objective: Rare co-existance of disease or pathology Background: Cerebral venous thrombosis (CVT) is a rare but fatal complication of hyperthyroidism that is induced by the hypercoagulable state of thyrotoxicosis. Although it is frequently difficult to diagnose CVT promptly, it is important to consider it in the differential diagnosis when a hyperthyroid patient presents with atypical neurologic symptoms. Care Report: A 49-year-old Japanese female with unremarkable medical history came in with thyroid storm and multiple progressive ischemic stroke identified at another hospital. Treatment for thyroid storm with beta-blocker, glucocorticoid, and potassium iodide-iodine was started and MR venography was performed on hospital day 3 for further evaluation of her progressive ischemic stroke. The MRI showed CVT, and anticoagulation therapy, in addition to the anti-thyroid agents, was initiated. The patient’s thyroid function was successfully stabilized by hospital day 10 and further progression of CVT was prevented. Conclusions: Physicians should consider CVT when a patient presents with atypical course of stroke or with atypical MRI findings such as high intensity area in apparent diffusion coefficient (ADC) mapping. Not only is an early diagnosis and initiation of anticoagulation important, but identifying and treating the underlying disease is essential to avoid the progression of CVT.

Postpartum vertebral artery dissection with posterior reversible encephalopathy syndrome

We diagnosed postpartum eclampsia with posterior reversible encephalopathy syndrome (PRES) in a 35-year-old woman who began experiencing headaches after delivery. Cervical MR angiography (MRA) suggested concomitant vertebral artery (VA) dissection. Antiplatelet therapy was not indicated. Each episode resolved spontaneously. The patient subsequently developed sudden onset of thunderclap headache. She experienced a tonicoclonic seizure in the outpatient clinic. On the basis of clinical course and MRI, we diagnosed postpartum eclampsia with PRES. Antiplatelet therapy was not indicated, as there were no signs of stroke. Antiepileptic and antihypertensive medicines were used for a short term to control seizure and blood pressure. Subsequent MRI 17 days after discharge showed resolution of the abnormal signals, but the intramural heme signals became apparent on the dissecting segment of VA. Subsequent MRI after 3 months indicated resolution of arterial dissection. To the best of our knowledge, this represents the third report of postpartum cervicocephalic artery dissection with PRES in the literature.

Amoebiasis masquerading as inflammatory bowel disease

A 60-year-old Japanese man presented with bloody diarrhoea. He stated that he had been diagnosed with ulcerative colitis (UC) 3 years prior, but discontinued follow-up care as treatment was ineffective. One year later, he came to our hospital with anorexia and weight loss. The abdomen was soft and flat without tenderness. Laboratory tests were unremarkable; faecal culture and Clostridium difficile toxin were negative. Findings and biopsy from a subsequent colonoscopy reconfirmed his diagnosis of UC. Neither mesalazine, which was initially prescribed, nor additional treatments improved his symptoms. Repeat colonoscopy, performed 5 months later, demonstrated similar findings in the same area. Although the pathology remained consistent with UC, multiple treatment failures suggested ongoing occult infection. Additional testing revealed positive Entamoeba histolytica antibody. 14 days of metronidazole dramatically improved his symptoms. He has remained asymptomatic after 2 years.

A case of lower leg skin rash and severe arthralgia

A 23-year-old previously healthy man was in his usual state of good health until 5 days prior to admission when he noted fever of 38.0°C, cough, rhinorrhoea and sore throat. Two days prior to admission, he developed a symmetrical painful rash over the lower extremities, and bilateral arthralgia in his knees and ankles. Over the ensuing 48 h, palpable purpura developed over the bilateral buttocks, lower legs and thighs (figure 1). He subsequently presented to our outpatient clinic and was admitted for inability to walk due to severe bilateral knee and ankle pain. Swelling in the knees and ankles was not noted. Owing to the history of prior …

Constrictive Pericarditis as a Long-term Undetermined Etiology of Ascites and Edema

Constrictive pericarditis (CP) is defined as impedance to diastolic filling caused by a fibrotic pericardium. The diagnosis of CP is a clinical challenge and requires a high index of clinical suspicion. The signs and symptoms of CP include fatigue, edema, ascites, and liver dysfunction. These can be mistakenly diagnosed as primary liver disease. We present the case of a 69-year-old woman with a 7-year history of leg edema and a 2-year history of ascites who was initially diagnosed with cryptogenic liver cirrhosis and was finally diagnosed with CP.

Can folate replacement induce lymphoma progression

A 43-year-old Japanese man with a low haemoglobin level of 1.3 g/dL and multiorgan dysfunction syndrome (MODS) was admitted to our hospital. He was diagnosed with folate deficiency, which was initially attributed to his malnutrition. He was transfused with several units of packed red blood cells and treated with folate, thiamine and vitamin B12 supplements; he showed a prompt haematological response and recovery from MODS. However, 3 weeks after the initial recovery, he had a relapse of pancytopenia and developed high-grade fever along with rapidly enlarging, generalised lymphadenopathy. Bone marrow biopsy revealed hemophagocytosis, and lymph node biopsy revealed peripheral T-cell lymphoma, not otherwise specified. Folate supplementation may have promoted lymphoma progression.

Myocarditis Associated with Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome: A Case Report and Review of the Literature

Patient: Male, 33 Final Diagnosis: Perimyocarditis associated with drug reaction with eosinophilia and systemic symptoms syndrome Symptoms: Skin rash Medication: — Clinical Procedure: — Specialty: Dermatology Objective: Rare disease Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a clinical syndrome that can be life-threatening, depending on the organs involved. Although DRESS commonly presents with skin lesions, myocarditis and pericarditis associated with DRESS, although rare, can be fatal. A case of DRESS associated with myocarditis is presented with a review of the literature of 43 reported cases of DRESS associated with myocarditis that included the present case, to evaluate the effectiveness of treatment of DRESS with corticosteroids. Case Report: A 33-year-old man presented with fever, diarrhea, and a diffuse maculopapular rash, four weeks after being treated with antibiotics and a nonsteroidal anti-inflammatory drug (NSAID). He developed renal failure, liver dysfunction, and profound hypotension with severe left ventricular dysfunction that required mechanical cardiac support. A diagnosis was made of myocarditis associated with DRESS syndrome. After treatment began with prednisolone, the skin rash, multi-organ dysfunction, and cardiogenic dysfunction resolved. Conclusions: Myocarditis is a rare complication associated with DRESS, but when it is suspected, urgent echocardiography should be performed, particularly when hemodynamic instability occurs. Early diagnosis, removal of the causative agent, and treatment with corticosteroids are important to reduce mortality from cardiac involvement in patients with DRESS.