[Development of severe aplastic anemia during steroid therapy for autoimmune hemolytic anemia].

A 50-year-old male patient was admitted for close monitoring of anemia (hemoglobin level, 5.0 g/dl). Autoimmune hemolytic anemia (AIHA) of warm type was diagnosed based on the elevated reticulocyte and bone marrow erythroblast counts, elevated indirect bilirubin level, serum haptoglobin level below the detection limit, and positive direct Coombs test result. The patient responded to prednisolone 60 mg/day (1.0 mg/kg); however, pancytopenia was observed during gradual dose tapering and maintenance therapy. The bone marrow showed remarkable hypoplastic findings, and magnetic resonance imaging scans of the thoracolumbar spinal cord showed an overgrowth of the adipose tissue. Thus, the patient was diagnosed with aplastic anemia (AA) stage 4. He was successfully treated with a combination of immunosuppressive therapy (anti-thymocyte globulin +cyclosporine), which allowed him to reduce his dependence on transfusions. However, the direct Coombs test result remained positive even after hematopoietic recovery. Aplastic anemia following AIHA treatment is extremely rare and has not been reported previously.

Environmental reservoir dynamics predict global infection patterns and population impacts for the fungal disease white-nose syndrome.

Disease outbreaks and pathogen introductions can have significant effects on host populations, and the ability of pathogens to persist in the environment can exacerbate disease impacts by fueling sustained transmission, seasonal epidemics, and repeated spillover events. While theory suggests that the presence of an environmental reservoir increases the risk of host declines and threat of extinction, the influence of reservoir dynamics on transmission and population impacts remains poorly described. Here we show that the extent of the environmental reservoir explains broad patterns of host infection and the severity of disease impacts of a virulent pathogen. We examined reservoir and host infection dynamics and the resulting impacts of Pseudogymnoascus destructans, the fungal pathogen that causes white-nose syndrome, in 39 species of bats at 101 sites across the globe. Lower levels of pathogen in the environment consistently corresponded to delayed infection of hosts, fewer and less severe infections, and reduced population impacts. In contrast, an extensive and persistent environmental reservoir led to early and widespread infections and severe population declines. These results suggest that continental differences in the persistence or decay of P. destructans in the environment altered infection patterns in bats and influenced whether host populations were stable or experienced severe declines from this disease. Quantifying the impact of the environmental reservoir on disease dynamics can provide specific targets for reducing pathogen levels in the environment to prevent or control future epidemics.

[Acquired hemophilia A accompanied by immune thrombocytopenia].

A 72-year-old man was hospitalized because of thrombocytopenia (0.5×104/µl) and anemia. The bone marrow test result revealed excessive numbers of megakaryocytes and no platelet adhesion. Furthermore, platelet-associated immunoglobulin G levels were high, and he was tested positive for Helicobacter pylori antibody. On the basis of these findings, immune thrombocytopenia was diagnosed. The patient was initially treated with eradication therapy; prednisolone, 20 mg/day (0.5 mg/kg) and a thrombopoietin receptor agonist 12.5 mg/day. During the course of treatment, the anemia worsened. Detailed examination revealed marked prolongation of activated partial thromboplastin time and inhibition of factor VIII activity. Therefore, he was diagnosed with acquired hemophilia A. Although extensive muscle hemorrhage had occurred, hemostatic therapy comprising intensification of the immunosuppressive therapy and administration of recombinant activated factor VII resulted in successful hemostasis. As the treatment progressed, inhibition of factor VIII recurred temporarily; however, immunosuppressive therapy was continued. No recurrence was observed even after 1 year of the onset of both diseases.

[Easy screening of outpatients for atrial fibrillation by analyzing fingertip pulse wave variation].

AIM: Atrial fibrillation (AF), which can lead to cardioembolic stroke, is often not properly diagnosed in hospital outpatient departments or medical clinics. We therefore used a pulse analysis to screen patients for AF, and examined the benefits of using this method in screening. METHODS: We performed screening of the hospital's first-visit and ambulatory patients during the afternoon in 2014 (total number, 50,875; true number, 16,356), mainly targeting patients older than 65 years of age. Among the true number of outpatients, the device was used on 5,013 patients, 8,656 times. We independently developed a pulse analysis software application which analyzed the pulse interval variation. We assessed the accuracy of this analytical method in the detection of AF. RESULTS: AF was detected in 56 patients, who were considered for or introduced to anticoagulation treatment. In their cases, the method was considered useful for detecting undiagnosed or untreated AF. This figure amounts to 0.34% of all outpatients and 1.1% of the patients who were screened in 2014. The average age was 76.9±7.7 years, 67.9% of the patients had a CHADS2 score of more than 2, half had a history of arrhythmia in the past, and 37.5% were first-visit patients. The sensitivity of the device used was 89.7%. CONCLUSIONS: Using the method described in this study, we detected asymptomatic AF in numerous patients, and demonstrated that this method is potentially useful in screening outpatients for asymptomatic AF.

Systemic and rapidly progressive light-chain deposition disease initially presenting as tubulointerstitial nephritis.

A 42-year-old woman was admitted to a hospital after first-time detection of proteinuria and hematuria during a routine medical check-up. Because her serum creatinine level had rapidly increased from 0.9 to 3.2 mg/dl since measurement 3 months earlier, she was referred to our hospital. Renal biopsy revealed extensive tubular atrophy and interstitial fibrosis with mild leukocyte infiltration. Glomeruli showed minimal changes, and no immunoglobulin or complement deposition was observed by immunofluorescence. Oral prednisolone was commenced under the diagnosis of chronic tubulointerstitial nephritis, and she discharged once. However, its effects were transient; her renal function deteriorated rapidly and hemodialysis was initiated 5 months after her initial check-up. On readmission, urinary Bence-Jones protein κ-type was detected, and examination of bone marrow led to a diagnosis of Bence-Jones κ-type multiple myeloma. Light-chain staining using a renal biopsy specimen obtained 2 months earlier showed κ-light-chain deposition on tubular basement membranes but not glomeruli. Despite undergoing chemotherapy with vincristine, doxirubicin, and dexamethasone, the patient died suddenly from a cardiac arrhythmia. Autopsy showed κ-light-chain deposition in the heart, thyroid, liver, lungs, spleen, and ovaries. Congo red staining yielded negative results. Typical light-chain deposition disease (LCDD) characterized by nodular glomerulosclerosis was observed in the kidneys. This case demonstrates that tubulointerstitial nephritis can be an early pathological variant of LCDD, which may be followed by accelerated and massive light-chain deposition in glomeruli.

[Prospects of CyberKnife stereotactic radiation therapy for cerebral vascular malformations and functional diseases].

Minimally invasive stereotactic radiation therapy has become one of the promising options for treating patients with intracranial diseases. Among the currently available instruments, the CyberKnife system (Accuray Inc., Sunnyvale, CA) is a unique apparatus equipped with image-guided target locating system. Because this system does not rely on rigid frame immobilization with screws, it has several distinct advantages over frame-based systems, including improved patient comfort and increased treatment degrees of freedom. In particular, this system has enabled selection of single-session and multi-session stereotactic radiation therapies, depending on the size, shape, and position of the lesions. Furthermore, this device is expected to aid stereotactic radiation therapy for large lesions that cannot be treated because of the size; moreover, it causes less damage to normal structures. In this article, we describe the advantages of the Cyberknife system for the management of vascular malformations and functional diseases.

Acoustic emission measurement using a strain-insensitive fiber Bragg grating sensor under varying load conditions.

Acoustic emission (AE) during a pressure test of a carbon fiber-reinforced plastic tank was measured using a fiber Bragg grating (FBG) sensor and, for comparison, using a conventional piezoelectric sensor. The FBG-inscribed optical fiber was used not only as an optical transmission line but also as an ultrasonic transmission line. The FBG sensor showed resonant characteristics and could detect AE continuously during the test. AE detected with the FBG sensor exhibited a cumulative behavior similar to that detected with the piezoelectric sensor. The FBG sensor was demonstrated to have comparable AE detection capability to piezoelectric sensors.