Nephrotic syndrome due to primary systemic AL amyloidosis, successfully treated with VAD (vincristine, doxorubicin and dexamethasone) alone

We report 3 patients with nephrotic syndrome ascribed to primary systemic AL amyloidosis that were successfully treated with VAD (vincristine, doxorubicin and dexamethasone) alone. M-protein in serum disappeared soon after VAD, and nephrotic syndrome gradually improved in parallel with a decrease in daily protein excretion in urine. Long-term follow-up of these patients showed neither relapse of nephrotic syndrome nor reappearance of M-protein. High-dose melphalan followed by autologous stem cell support is a standard therapy for primary systemic AL amyloidosis, but in high-risk cases for this treatment, such as elderly patients and those with multiple organ involvement, VAD might be a therapeutic option.     

Microbial community in a sediment-hosted CO2 lake of the southern Okinawa Trough hydrothermal system

Increasing levels of CO2 in the atmosphere are expected to cause climatic change with negative effects on the earth's ecosystems and human society. Consequently, a variety of CO2 disposal options are discussed, including injection into the deep ocean. Because the dissolution of CO2 in seawater will decrease ambient pH considerably, negative consequences for deep-water ecosystems have been predicted. Hence, ecosystems associated with natural CO2 reservoirs in the deep sea, and the dynamics of gaseous, liquid, and solid CO2 in such environments, are of great interest to science and society. We report here a biogeochemical and microbiological characterization of a microbial community inhabiting deep-sea sediments overlying a natural CO2 lake at the Yonaguni Knoll IV hydrothermal field, southern Okinawa Trough. We found high abundances (>10(9) cm(-3)) of microbial cells in sediment pavements above the CO2 lake, decreasing to strikingly low cell numbers (10(7) cm(-3)) at the liquid CO2/CO2-hydrate interface. The key groups in these sediments were as follows: (i) the anaerobic methanotrophic archaea ANME-2c and the Eel-2 group of Deltaproteobacteria and (ii) sulfur-metabolizing chemolithotrophs within the Gamma- and Epsilonproteobacteria. The detection of functional genes related to one-carbon assimilation and the presence of highly 13C-depleted archaeal and bacterial lipid biomarkers suggest that microorganisms assimilating CO2 and/or CH4 dominate the liquid CO2 and CO2-hydrate-bearing sediments. Clearly, the Yonaguni Knoll is an exceptional natural laboratory for the study of consequences of CO2 disposal as well as of natural CO2 reservoirs as potential microbial habitats on early Earth and other celestial bodies.     

Effects of endoscopic transthoracic sympathicotomy on hemodynamic and neurohumoral responses to exercise in humans.

Endoscopic transthoracic sympathicotomy (ETS) is a minimal invasive procedure of thoracic sympathetic block and has been used successfully in the treatment of primary palmar hyperhidrosis. To examine the effect of Th 2-3 ETS on hemodynamic responses to submaximal upright treadmill exercise in humans, cardiac output, plasma noradrenaline and adrenaline at rest and during the last 40s of stage 2 in a modified Bruce protocol were measured before and after ETS in 21 patients with primary palmar hyperhidrosis. Heart rate, mean arterial pressure, rate-pressure product, and noradrenaline decreased at rest and at submaximal exercise after ETS. Cardiac index at rest did not change either before or after ETS, but decreased (8.9 +/- 0.6 vs 6.8 +/- 0.4L x min(-1) m-2; p<0.01, mean +/- SEM) at submaximal exercise after ETS. Stroke index and systemic vascular resistance were similar both at rest and at submaximal exercise before and after ETS. Thus, ETS reduces myocardial oxygen demand and plasma noradrenaline levels both at rest and during exercise without significantly depressing cardiac function in terms of stroke volume.     

Histopathological regression of systemic AA amyloidosis after surgical treatment of a localized Castleman's disease.

Previously, we reported a case of localized plasma cell type Castleman's disease with severe hepatomegaly and reactive systemic AA amyloidosis. The amyloid deposits were demonstrated in both the hepatic tissue and in the gastric mucosa. Surgical resection of an isolated extra-hepatic tumor was performed. The laboratory findings, including SAA and IL-6, remained within normal limits and the patient's hepatomegaly subsequently showed regression. Nine years after the operation, no amyloid deposition was seen in the gastric mucosa and the patient's liver was of normal size. Our findings with long-term follow up in this case indicated that the cessation of SAA production was the probable cause of histopathological regression of AA amyloid deposits in this patient.     

Development of beta-tricalcium phosphate/collagen sponge composite for bone regeneration

Synthetic biomaterials have been developed and used for bone grafting. Here, we developed a biodegradable sponge composite for bone tissue engineering by combining beta-tricalcium phosphate (beta-TCP) and collagen. In addition, we sought to determine the optimal beta-TCP granules/collagen ratio by evaluating and bone formation in vivo. Porous beta-TCP granules were mixed with atelocollagen hydrochloride solution at various ratios--0.02, 0.05, 0.1, and 0.2 g/mL. The resultant mixtures were freeze-dried and subjected to dehydrothermal treatment in vacuo. The final composites obtained were designated beta-TCP/collagen sponge composites (beta-TCP/CS). Through compression testing, it was found that the stress values for beta-TCP/CS (0.2 g/mL) were higher than those of the other three composites over the whole strain range. Histological evaluation at four weeks after implantation revealed that the collagen sponge had degraded and newly formed bone was present on the surface of the beta-TCP granules. At 12 weeks, the beta-TCP granules were completely degraded and remodeling of the lamellar bone was observed.     

De novo appearance of cerebellar cavernous malformation in a patient with moyamoya disease: case report and review of the literature

The authors report a case of cerebellar cavernous malformation associated with moyamoya disease. An adolescent male with moyamoya disease had undergone bilateral direct and indirect extracranial-intracranial anastomosis at 11 years of age, and the course had been uneventful until MRI detected the appearance of a cavernous malformation in the cerebellum 3 years later. The lesion had grown, bled, and caused headache and disturbance of consciousness 2 years after the initial detection. The cavernous malformation was removed surgically and pathologically verified. The patient has recovered without any neurological deficits. This is a quite rare case with cavernous malformation which appeared in a moyamoya disease patient. The association of the two different vascular disorders in a young patient may suggest the existence of some interaction in the pathogenesis of these diseases. Since cavernous malformations with a de novo appearance may grow and become clinically significant, careful observation is necessary.     

Successful treatment with infliximab and low-dose methotrexate in a Japanese patient with familial Mediterranean fever

We report a Japanese patient with familial Mediterranean fever (FMF) who was successfully treated with the anti-tumor necrosis factor (TNF)-alpha monoclonal antibody, infliximab, and low-dose methotrexate. This patient was diagnosed as having FMF based on periodic fever with polyarthralgia typical of this disease and heterozygous mutations in the MEFV gene. Conventional treatment, such as colchicine and reserpine, failed to sufficiently control the FMF attacks. After starting infliximab (3 mg/kg) and low-dose methotrexate (6 mg/week), the frequency of the FMF attacks dramatically decreased and the clinical effect has remained unchanged for longer than 1 year. Combination therapy with infliximab and low-dose methotrexate may be a potent therapeutic option for FMF patients, particularly when conventional treatment is ineffective or cannot be employed because of adverse events.     

Visible light is able to regulate neurite outgrowth

The pheochromocytoma cell line PC12 displays neuronal characteristics. PC12 cells differentiate their phenotype from a proliferating cell to a neurite-bearing neuron upon treatment with nerve growth factor (NGF). The neurite outgrowth of PC12 cells on polystyrene tissue culture flasks and extracellular matrix protein-adsorbed glass plates was reversibly controlled using visible light. The percentage of cells with neurites decreased with increasing light intensity. Furthermore, neurite outgrowth was dramatically suppressed with light intensities over 300 Lux (approximately 130 microW). Neurite outgrowth occurred in the absence of irradiation by visible light, but did not occur or was limited with irradiation, depending on the membranes on which PC12 cells were cultured. These results hold promise for the creation of patterned neuronal networks corresponding to patterned irradiation of visible light on nerve cells.