Successful treatment with oral ribavirin of adenovirus-associated hemophagocytic syndrome in a stem cell transplantation recipient

A 51-year-old woman with severe aplastic anemia underwent allogeneic peripheral blood stem cell transplantation using blood stem cells from an HLA-identical sibling. Adenovirus type 11 hemorrhagic cystitis developed and progressed to nephritis and hemophagocytic syndrome. Oral ribavirin was effective not only for the hemorrhagic cystitis and nephritis but also for the hemophagocytic syndrome. Since therapeutic strategies for adenovirus infection after hematopoietic stem cell transplantation have not been established, we present our case and discuss the therapeutic approach.     

Clinical significance of anti-phosphatidylserine-prothrombin complex antibodies in antiphospholipid syndrome

We clarified the clinical significance of IgG anti-phosphatidylserine-prothrombin complex (PS-PT) antibodies in the antiphospholipid syndrome (APS). The study population consisted of 122 patients with SLE and lupus-like disease. IgG anti-PS-PT antibodies were detected in 44% of 59 patients according to the diagnostic criteria by Harris and Hughes. This frequency was significantly (p < 0.005) higher than the 14% seen in patients without APS. IgG anti-PS-PT antibodies were strongly (p < 0.005) associated with thrombosis. In addition, IgG anti-PS-PT antibodies were positive in 64% of IgG beta2-GPI dependent anti-cardiolipin antibody negative APS patients under the Sapporo criteria. The above findings indicate that IgG anti-PS-PT antibodies as well as beta2,-GPI dependent anti-cardiolipin antibodies should be examined in the diagnosis of APS.     

Successful catheter ablation for incessant ventricular tachycardia in a patient with hypertrophic cardiomyopathy.

A 35-year-old man was referred to Nihon University Hospital because of repetitive ventricular tachycardia (VT) at 180-200 beats/min. QRS morphology of the VT was right bundle branch block with a northwest axis. Transthoracic echocardiography showed hypertrophic cardiomyopathy. Coronary angiography was normal and left ventriculography showed neither obstruction in the left ventricle (LV) nor any pressure gradients within the LV or between the LV and aorta. Hemodynamic deterioration occurred during VT. Intracardiac mapping showed that the VT originated from the posteroseptal portion of the LV near the apex and Purkinje potentials that preceded the onset of the QRS complex by 58-70 ms were documented. Radiofrequency ablation at these sites terminated the VT, which has not recurred for 25 months.     

Influence of nasal resistance on initial acceptance of continuous positive airway pressure in treatment for obstructive sleep apnea syndrome

BACKGROUND: Continuous positive airway pressure (CPAP) is considered as the standard therapy for obstructive sleep apnea syndrome (OSAS), but some patients with OSAS are unable to accept CPAP due to nasal obstruction and poor nasal airflow. OBJECTIVES: We assessed the influence of nasal resistance before beginning CPAP treatment on the initial acceptance of CPAP in OSAS patients. METHODS: The study subjects comprised 77 patients (74 males, 3 females) with primary OSAS, all of whom received CPAP treatment with nasal masks. Before trials, all subjects underwent overnight polysomnography, and nasal resistance was measured with active anterior rhinomanometry in the seated position on the first day of CPAP trial. RESULTS: The CPAP treatment was accepted by 56 patients after the initial trials with overnight polysomnography. Body mass index, the number of apnea/hypopnea episodes per hour (apnea/hypopnea index; AHI), and the number of episodes per hour with an oxygen desaturation of >3% (oxygen desaturation index) were significantly higher (p<0.01) and nasal resistance was lower (p=0.003) in patients who accepted CPAP than in those who did not. Logistic regression analysis, with patient age, body mass index, Epworth sleepiness scale score, AHI, oxygen desaturation index, and nasal resistance before CPAP treatment as explanatory variables, showed that nasal resistance (OR+0.1 Pa/cm3/s: 1.48; p=0.002) and AHI (OR+1 event/h: 0.93; p=0.003) were significant factors for CPAP non-acceptance. CONCLUSIONS: Nasal resistance before the beginning of CPAP treatment has a significant effect on the acceptance of CPAP in OSAS patients, and hence, could be a predictive parameter for the initial acceptance of CPAP.     

The effect of lifestyle factors on osteoporotic fractures

Osteoporotic fracture is one of the biggest causes of bed-ridden conditions. Lifestyle factors such as dietary habits, smoking habits, alcohol consumption, and exercise habit are well associated with bone mineral densities. This review summarized the improved custom behavior is beneficial to prevent osteoporotic fractures in elderly.     

Two distinct activity patterns of fast-spiking interneurons during neocortical UP states

During sleep, neocortical neuronal networks oscillate slowly (<1 Hz) between periods of activity (UP states) and silence (DOWN states). UP states favor the interaction between thalamic-generated spindles (7-14 Hz) and cortically generated gamma (30-80 Hz) waves. We studied how these three nested oscillations modulate fast-spiking interneuron (FSi) activity in vivo in VGAT-Venus transgenic rats. Our data describe a population of FSi that discharge "early" within UP states and another population that discharge "late." Early FSi tended to be silent during epochs of desynchronization, whereas late FSi were active. We hypothesize that late FSi may be responsible for generating the gamma oscillations associated with cognitive processing during wakefulness. Remarkably, FSi populations were differently modulated by spindle and gamma rhythms. Early FSi were robustly coupled to spindles and always discharged earlier than late FSi within spindle and gamma cycles. The preferred firing phase during spindle and gamma waves was strongly correlated in each cell, suggesting a cross-frequency coupling between oscillations. Our results suggest a precise spatiotemporal pattern of FSi activity during UP states, whereby information rapidly flows between early and late cells, initially promoted by spindles and efficiently extended by local gamma oscillations.     

Histological diagnosis of spinal chondrosarcoma by endobronchial ultrasound-guided transbronchial needle aspiration

Rare neoplastic diseases including sarcomas can occur in the middle mediastinum. Obtaining a histological specimen is crucial for diagnosis and for establishing a treatment plan. Our research group has recently reported a minimal invasive method of tissue sampling of the mediastinum using direct real-time endobronchial ultrasound-guided transbronchial needle aspiration. By the use of this new modality, histological cores can be obtained for pathological diagnosis that can be used for immunohistochemistry as well as genetic analysis. A very rare case of spinal chondrosarcoma due to hereditary multiple exostoses successfully diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration and by immunohistochemistry with genetic analysis is reported.     

Hepatic veno-occlusive disease after tranexamic acid administration in patients undergoing allogeneic hematopoietic stem cell transplantation

Tranexamic acid is one of the widely used antifibrinolytic agents. In spite of its effective inhibitory activity against plasminogen, thromboembolic adverse events caused by tranexamic acid are rare. We encountered three recipients of allogeneic hematopoietic stem cell transplantation (HSCT) who developed hepatic veno-occlusive disease (VOD) shortly after the administration of tranexamic acid. Hepatic VOD was resolved completely in all patients with the discontinuation of the drug, and with supportive measures with or without intravenous tissue plasminogen activator administration. These findings suggest that administration of tranexamic acid could be one of the possible risk factors for developing hepatic VOD in HSCT recipients.     

Does hypoplasia of one pulmonary artery preclude a definitive repair in pulmonary atresia,intact ventricular septum,and hypoplastic right ventricle?

Summary Twins with pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle associated with underdeveloped and discontinuous left pulmonary artery are described. Operations to connect the left pulmonary artery to the main pulmonary trunk, with subsequent biventricular repair assisted by bidirectional cavopulmonary anastomosis and atrial fenestration were performed. Follow-up catheterization proved spontaneous closure of the fenestration in one patient. Both patients have been in a satisfactory condition for 3 years since operation.     

Mesenteric fibromatosis successfully resected with duodeno-jejunectomy and nephrectomy

We report a rare case of intra-abdominal fibromatosis of the jejunal mesentery. A 71-year-old man, who was incidentally diagnosed with left hydronephrosis, was found to have a tumor near the duodeno-jejunal junction. The tumor appeared to invade the intestinal wall and was obstructing the left ureter. The tumor was indefinite in diagnosis preoperatively. The patient underwent an en-bloc excision of the tumor by a partial duodeno-jejunectomy combined with a left nephrectomy. Microscopic examination of the tumor showed that non-dysplastic fibroblasts proliferating in the jejunal mesentery had infiltrated into the adjacent small intestines and ureter, resulting in a diagnosis of intra-abdominal fibromatosis. This diagnosis was supported by findings of immunohistochemical analyses showing positive staining for vimentin and smooth muscle actin and negativity for keratin, CD34, C-kit and S-100. To our knowledge, this is the first documented case of intra-abdominal fibromatosis of the jejunal mesentery completely resected with the operative procedure described herein. In cases of a preoperatively undiagnosed retroperitoneal or mesenteric tumor that invades adjacent structures, it is important to consider intra-abdominal fibromatosis as a possible differential diagnosis. This may be helpful in planning the appropriate therapeutic strategies including extended multi-organ resection in selected patients.