From Ultrafast Photoinduced Small Polarons to Cooperative and Macroscopic Charge-Transfer Phase Transition.

We study by femtosecond infrared spectroscopy the ultrafast and persistent photoinduced phase transition of the Rb0.94Mn0.94Co0.06[Fe(CN)6]0.98 ⋅ 0.2H2O material, induced at room temperature by a single laser shot. This system exhibits a charge-transfer based phase transition with a 75 K wide thermal hysteresis, centred at room temperature, from the low temperature Mn3+-N-C-Fe2+ tetragonal phase to the high temperature Mn2+-N-C-Fe3+ cubic phase. At room temperature, the photoinduced phase transition is persistent. However, the out-of-equilibrium dynamics leading to this phase is multi-scale. Femtosecond infrared spectroscopy, particularly sensitive to local reorganizations through the evolution of the frequency of the N-C vibration modes with the different characteristic electronic states, reveals that at low laser fluence and on short time scale, the photoexcitation of the Mn3+-N-C-Fe2+ phase creates small charge-transfer polarons [Mn2+-N-C-Fe3+]* within ≃250 fs. The local trapping of photoinduced intermetallic charge-transfer is characterized by the appearance of a polaronic infrared band, due to the surrounding Mn2+-N-C-Fe2+ species. Above a threshold fluence, when a critical fraction of small CT-polarons is reached, the macroscopic phase transition to the persistent Mn2+-N-C-Fe3+ cubic phase occurs within ≃ 100 ps. This non-linear photo-response results from elastic cooperativity, intrinsic to a switchable lattice and reminiscent of a feedback mechanism.

The frequency of Fabry disease with the E66Q variant in the α-galactosidase A gene in Japanese dialysis patients: a case report and a literature review.

Fabry disease (FD) is an Xlinked disorder resulting in a deficiency in α-galactosidase A (α-Gal) activity. FD is one of the causes of progressive renal dysfunction, but its diagnosis is often delayed or missed completely. We herein report the case of a 70-year-old male who had been receiving hemodialysis (HD) for 23 y who was diagnosed with FD after his participation in a screening program for plasma α-Gal activity for 892 HD patients. He had a low plasma α-Gal activity level and was demonstrated to have an E66Q mutation in exon 2 of the α-Gal gene. One of his daughters had the same mutation. The proband died due to aspiration pneumonia before receiving enzyme replacement therapy. We reviewed previous studies and found E66Q mutation in 36% of Japanese FD patients on HD including the present case. The clinical characteristics of E66Q variant are also discussed.

Distribution of protein disulfide isomerase in rat hepatocytes.

We investigated quantitatively the distribution of protein disulfide isomerase (PDI) in rat hepatocytes by immunocytochemistry using a post-embedding protein A-gold technique. In hepatocytes, gold particles were mainly localized in the intracisternal space of the rough and smooth endoplasmic reticulum (ER) and nuclear envelopes. Autolysosomes engulfing ER were occasionally densely labeled, especially in rat hepatocytes previously treated with leupeptin in vivo, suggesting that the autophagosome-autolysosome system may be an important route for degradation of PDI. A few gold particles were also found on the plasma membranes. Localization of gold particles on the other subcellular organelles, such as Golgi apparatus, peroxisomes, and nuclear matrix, was sparse and at the control level. The predominant localization of PDI on the intracisternal surface of the ER and nuclear envelope supports a potential role of PDI in the formation of disulfide bonds of nascent polypeptides, thus accelerating formation of the higher-order structure of secretory and membrane proteins and rendering the translocation process irreversible.

Distribution of protein disulfide isomerase in rat epiphyseal chondrocytes.

We investigated the intracellular distribution of protein disulfide isomerase (PDI) in rat epiphyseal chondrocytes by immunocytochemistry, using a post-embedding protein A-gold technique. Gold particles were localized primarily in the cisternal space of the rough endoplasmic reticulum (ER) and nuclear envelopes. The ER cisternae of the chondrocytes in all the differentiating epiphyseal zones--resting, proliferative, pre-hypertrophic, and hypertrophic--were equally and highly labeled. The labeling density of the cisternal space of the dilated ER, probably reflecting marked accumulation of secretory proteins such as procollagen, was always higher than that of the non-dilated ER. In the dilated cisternal space, gold particles were freely and evenly distributed, without preferential binding to the luminal surface of the ER membranes. We suggest that PDI catalyzes the formation of disulfide bonds of various secretory proteins, perhaps type II procollagen, in the cisternal space of the ER in epiphyseal chondrocytes. The exclusive localization of gold particles in the cisternal space of the ER and nuclear envelopes and the lack of gold particles in the Golgi apparatus, including cis-Golgi cisternae, indicate that PDI is an ER-soluble protein in the chondrocytes and is presumably sorted out in some pre-Golgi compartment and not transported to the Golgi apparatus.

Localization of protein disulfide isomerase on plasma membranes of rat exocrine pancreatic cells.

We investigated immunocytochemically the ultrastructural localization of protein disulfide isomerase (PDI) in rat pancreatic exocrine cells by use of the post-embedding protein A-gold technique. We found that not only the endoplasmic reticulum (ER) and nuclear envelope but also the trans-Golgi cisternae, secretory granules, and plasma membranes were heavily labeled with gold particles. Labeling density of the gold particles in the rough ER and plasma membranes of the exocrine pancreatic cells was twofold and twentyfold greater, respectively, than that of hepatocytes. In the acinar lumen, amorphous material presumably corresponding to the secreted zymogens was also labeled with gold particles. These results suggest that in rat exocrine pancreatic cells a significant amount of PDI is transported to the plasma membrane and secreted to the acinar lumen.

Sclerosing cholangitis and systemic lymphadenopathy.

A 48-year-old man was admitted to our hospital with jaundice and systemic lymphadenopathy. Cholangiographic findings and liver histology disclosed the presence of sclerosing cholangitis. The patient also had a marked polyclonal increase in IgG levels. The cholangiographic findings, the systemic lymphadenopathy, and the increase in IgG levels resolved completely after treatment with prednisolone. This case suggests that there is an association between sclerosing cholangitis and immunologic abnormalities, and that corticosteroid treatment is useful for this disorder.

Expression rate of cytokine mRNA in the liver of chronic hepatitis C: comparison with chronic hepatitis B.

This study was carried out to test the hypothesis that, in chronic hepatitis (CH), inflammatory processes, including viral replication, host immune response, and hepatocyte destruction, are regulated by a cytokine network in the liver. Expression of the mRNA of the cytokines IL1-beta, IL2, IL4, IL5, IL6, TNF-alpha, and IFN-gamma, the lymphocyte markers CD4 and CD8, and the HLA class I molecule, beta 2-microglobulin (B2MG) in the liver tissue of 20 CH(C) cases and 9 CH(B) patients was investigated by the reverse transcription polymerase chain reaction (RT-PCR) method. TNF-alpha, CD4, and B2MG mRNA were detected in 100% of cases of in both CH(B) and CH(C). The expression rates of IL1-beta, IL2, IL4, IFN-gamma, and CD8 mRNA were 80%, 40%, 25%, 40%, and 80% in CH(C) and 88.9%, 44.5%, 30%, 55.6%, and 100% in CH(B). IL6 mRNA was detected only in CH(B), in 22.2% of cases, IL5 mRNA was not detected in either CH(B) or CH(C). IL2, IL4, and IFN-gamma mRNA were expressed significantly more frequently in patients who had high serum ALT and a high histological activity index (HAI) score. There was no difference in cytokine expression between CH(B) and CH(C), except in IL6, suggesting the existence of a common immunopathogenesis for CH(B) and CH(C). In chronic viral hepatitis, IL1-beta and TNF-alpha appear to play a major role in immune responses and IL2, IL4, and IFN-gamma seem to be associated with increased cytotoxic T cell response.(ABSTRACT TRUNCATED AT 250 WORDS)

Expression of interferon-alpha receptor mRNA in the liver in chronic liver diseases associated with hepatitis C virus: relation to effectiveness of interferon therapy.

To investigate whether interferon-alpha receptor (IFN-alpha Rc) expression was related to the effectiveness of interferon therapy in hepatitis C virus (HCV)-associated chronic liver disease (CLD). IFN-alpha Rc mRNA was investigated by reverse transcription polymerase chain reaction (RT-PCR) in liver biopsies and peripheral blood mononuclear cells (PBMCs) from 40 patients with HCV-associated CLD who subsequently received IFN-alpha therapy. IFN-alpha Rc mRNA in the liver was detected in 18 of 20 (90%) responders to IFN and in 5 of 20 (25%) non-responders (P < 0.01). In PBMCs, IFN-alpha Rc mRNA was detected in all patients regardless of response to IFN. Increased histological hepatitis activity and liver fibrosis were significantly related to the absence of IFN-alpha Rc mRNA. The HCV-RNA genotype showed no significant relationship to IFN-alpha Rc mRNA expression. Our results suggest that IFN-alpha Rc mRNA expression in the liver, but not in PBMCs, is closely associated with the effectiveness of IFN-alpha therapy in HCV-associated CLD.

Strictureplasty for short duodenal stenosis in Crohn's disease.

Involvement of the gastroduodenum is extremely rare in Crohn's disease. For obstructing duodenal Crohn's disease, bypass procedures have traditionally been selected. However, more recently, strictureplasty has become an acceptable surgical option. We treated two Crohn's disease patients with short proximal duodenal stenosis, using Finney-type strictureplasty. Their postoperative courses were uneventful and they have remained asymptomatic during follow-up periods of more than 5 years, and 4 months, respectively. Owing to the good clinical results of our two patients, we consider strictureplasty to be indicated for short proximal duodenal stenosis in Crohn's disease.

Interleukin-10 expression in intestine of Crohn disease.

Inflammatory bowel diseases are considered to be related to dysregulation of pro- and anti-inflammatory cytokines in the intestinal wall. We investigated the levels of TNFalpha, IFNgamma, and IL-10 mRNA expression in intestinal tissues resected from the patients with Crohn disease (CD) (n=29), ulcerative colitis (UC) (n=8), and controls (n=8) using reverse transcription-polymerase chain reaction (RT-PCR). In addition, we examined the relationship between the expression of these cytokine mRNA and their clinical conditions using CD activity index (CDAI) and Nutritional Surgical Risk Index (NSRI). Compared with controls, tissues in CD showed high levels of TNFalpha and IFNgamma mRNA expression both in inflamed and non-inflamed tissues, and showed high levels of IL-10 mRNA expression in inflamed tissues. In UC, high levels of IL-10 mRNA expression were detected both in inflamed and non-inflamed UC tissues, while those of TNFalpha and IFNgamma were not. In 80% of CD tissues (n=23), levels of IL-10 and TNFalpha expression were interrelated. While the remaining tissues (n=6) showed low levels of IL-10 expression despite high levels of TNFalpha expression in inflamed CD tissues, and 4 of these 6 patients had high CDAI and low NSRI. Furthermore, in low nutritional CD patients (NSRI <40, n=13), the levels of IL-10 mRNA to inhibit pro-inflammatory cytokines were poorer than in good nutritional patients (NSRI >/=40, n=16). These findings suggest the overexpressions of TNFalpha and IFNgamma in CD, and less producibility of IL-10 against these cytokine might lead to development of severe CD.

Maffucci's syndrome involving hemangioma in the cervical spine.

STUDY DESIGN: This is a case report of a patient with Maffucci's syndrome, with hemangioma located in the cervical spine causing paraplegia. OBJECTIVE: To present a rare case of Maffucci's syndrome with hemangioma located in the cervical spine, and to discuss the incidence and treatment of hemangioma located in the central nerve system in Maffucci's syndrome. SUMMARY OF BACKGROUND DATA: Maffucci's syndrome is characterized by enchondromatosis and soft tissue hemangioma. Hemangioma usually is located in the subcutaneous tissue and rarely in the mucosa and visceral organs. There have been no reports in the literature on the incidence of hemangioma in the cervical spine occurring with Maffucci's syndrome. METHODS: The patient was an 11-year-old girl, who complained of muscle weakness of the upper extremity and gait disturbance. After clinical and radiologic evaluation, the patient was diagnosed as having dumbbell tumor of the cervical spine associated with Maffucci's syndrome. RESULTS: The tumor was successfully excised after selective angiography and embolization of the feeding artery from the ascending cervical artery and costocervical trunk, the result being excellent neurologic recovery for the patient. Histologic study showed the tumor was hemangioma similar to the red-blue tissue tumor in the hand. CONCLUSION: The possibility of hemangioma being localized in the spinal column, as well as in the subcutaneous tissue, mucosa, and visceral organ, should be considered in cases of Maffucci's syndrome.

External iliac artery occlusion in posterior spinal surgery.

STUDY DESIGN: Report of a case of external iliac artery occlusion occurring as a rare complication of lumbar posterior spinal surgery. OBJECTIVE: To clarify the cause of this rare complication and recommend methods for preventing its recurrence. SUMMARY OF BACKGROUND DATA: Several cases of central retinal artery occlusion after posterior spinal surgery have been reported, but there has been no reported case of external iliac artery occlusion after posterior spinal surgery. METHODS: A 65-year-old woman who had a 32-year history of systemic lupus erythematosus underwent posterior decompression and spinal fusion for degenerative scoliosis. She was supported on a Hall frame during the operation. Three hours after surgery, paralysis and sensory impairment of the left leg and cyanosis of the toes of the left foot were noted. The popliteal, dorsalis pedis, and posterior tibial pulses were not palpable. Angiography showed complete occlusion of the external iliac artery, and emergency removal of the thrombus was performed. RESULTS: One year later, neurologic symptoms were absent, and the leg vessels were normal. CONCLUSIONS: Prolonged direct pressure on the inguinal region during posterior spinal surgery on a Hall frame may cause external iliac artery occlusion. Early recognition and adequate treatment can prevent serious sequelae.

The long-term results of ankle joint synovectomy for rheumatoid arthritis.

The clinical and radiological results of synovectomy for rheumatoid arthritis in the ankle joint were investigated in 20 ankles of 15 patients. The average follow-up period after synovectomy was 15 years, ranging from 10 to 25 years. The clinical evaluation at the time of follow-up, found that only two ankles showed recurrence of synovitis, and no patient complained of severe ankle pain disturbing the activities of daily life. During the period between the synovectomy and our investigation, no patients required further surgical procedures for their ankle joints. The radiological evaluation found that in approximately two-thirds of the cases, deterioration of the radiological grade, evaluated with Larsen's criteria, had continued after synovectomy. There was no considerable radiological deterioration in the less-erosive subset patients, classified according to Ochi's criteria (1). In the unilateral synovectomized cases, using the non-operated ankles as the natural-course control, osteoarthritic changes were predominant in the operated ankle joint, and the non-operated ankle demonstrated inflammatory disease changes. These results indicate that: (1) synovectomy for a rheumatoid ankle is still a preferred treatment, lessening the clinical symptom of persistent, marked synovial proliferation resistant to medical treatment. (2) Radiological deterioration continues after synovectomy in many cases. However, a radiogram demonstrates predominant osteoarthritic destruction, which indicates the natural course of rheumatoid destruction in the operated site could be altered by synovectomy.

Multiple small lesions of hepatocellular carcinoma controlled by percutaneous and laparoscopic ethanol injection--a case report.

A 61-year-old man was admitted to our clinic for a liver examination. Ultrasonography revealed multiple echo-rich lesions in both lobes. A laparoscopy showed a liver with an irregular surface, and a 3-mm-sized dark reddish lesion on the inferior surface of the right lobe. alpha-Fetoprotein and plasma protein induced by vitamin K absence or antagonist were normal. A liver biopsy specimen obtained from the small lesion by laparoscopy-guide showed a well-differentiated hepatocellular carcinoma with bile formation. Biopsy specimens obtained later from the 2 echo-rich lesions by ultrasonographic-guide were histologically similar to the lesion laparoscopically observed. Laparoscopic ethanol injection and percutaneous ethanol injection were performed as therapeutic procedures. Recurrence of hepatocellular carcinoma at the treated sites was not observed during the 6-year observation period. Thus, laparoscopy might play an important role in the early detection and treatment of hepatocellular carcinomas on the surface of the liver.

Ovarian response and FSH profile in cows following injection of various doses of inhibin antiserum.

Dose effect of inhibin antiserum on ovarian response and hormonal profiles were investigated. On day 12 of the estrous cycle (day 0 = estrus), 14 of 19 cows were given a single i.v. injection of 25 ml (n = 4), 37.5 ml (n = 5) or 50 ml (n = 5) antiserum against inhibin produced in a castrated male goat. The other 5 animals were given 50 ml castrated male goat serum (control serum). The animals in each group received a single i.m. injection of 0.5 mg prostaglandin F2 alpha analogue (PG) 48 hr following the serum injection. The population of follicles and ovulation rate (estimated by the number of corpora lutea) were examined by ultrasonography. Administration of inhibin antiserum consistently resulted in a significant (p < 0.01) increase in plasma concentrations of follicle-stimulating hormone (FSH) in inhibin-neutralized groups, although the increased FSH levels were sustained longer in 50-ml group than in the 25- and 37.5- ml groups. Levels in the circulating inhibin antibody titer were positively correlated with dosage of inhibin antiserum. A large number of antral follicles (> or = 4 mm in diameter) developed similarly after hypersecretion of FSH in all neutralized groups, coupled with a rise in plasma estradiol levels, while the number of large follicles (> or = 10 mm in diameter) on estrus showed a dose-dependent increase. Multiple ovulation (2 to 4) was recorded in all animals after injection of 50 ml inhibin antiserum, however all cows in the 25-ml group experienced only one ovulation and injection of 37.5 ml resulted in a variable number of ovulations (1 to 5). These results demonstrated that administration of inhibin antiserum on day 12, followed by injection of PG, was able to induce hypersecretion of FSH and subsequently multiple ovulations. The number of large follicles on estrus day and ovulations were affected by dosage of inhibin antiserum and were correlated with persistence of increased FSH levels or circulating antibody levels.

Clinical and pathologic characteristics of lumbar disk herniation in the elderly.

The clinical and pathologic characteristics of lumbar disk herniation in 23 elderly patients (15 men and 8 women) who required surgical treatment were investigated. Average age at surgery was 67.3 years, and average follow-up was 23 months. Preoperatively, the predominant symptom was severe unilateral leg pain, and 13 patients were nonambulatory because of leg pain. Operative treatment included wide laminectomy in 8, hemilaminectomy in 3, fenestration in 10, and osteoplastic laminectomy in 2 patients. Four (17%) patients had disk herniation at the L2-L3 or L3-L4 level. Sequestered herniation with or without migration was observed in 15 (65%) patients. Of 14 sequestered disk fragments examined histologically, 8 (57%) specimens contained cartilaginous or bony end plate with anulus fibrosus or nucleus pulposus. Postoperatively, results were rated as excellent in 11 patients and good in 12; no patient was rated as fair or poor. Severe leg pain affecting activities of daily life is a predominant symptom of disk herniation in the elderly. For patients in whom conservative treatment has failed, operative treatment should be considered. In lumbar disk herniation in the elderly, the incidence of cephalad and lateral herniation is higher than in younger patients. In addition, sequestered and migrated herniations including end plate are frequent.

Prediction for the development of postoperative infections in the operation of esophageal cancer compared with gastric surgery.

The purpose of this study was to assess the point at which the postoperative infection has occurred in order to decide upon the proper duration of prophylactic antibiotic use. Another goal of this study was to determine whether prediction for the development of postoperative infections in major surgery such as esophagectomy should be the same as that in routine gastroenterological surgery. Twenty-five patients who underwent transthoracic esophagectomy and 127 patients who underwent gastrectomy were studied. On the third day after gastric surgery, the body temperature of patients who developed an infection was higher than that of the patients who did not develop an infection. The relative changes in peripheral white blood count (WBC), and C-reactive protein (CRP) concentration on the third and fourth days were more predictive of the development of infection than the absolute values. Almost all patients with systemic inflammatory response syndrome (SIRS) on the third day after gastric surgery developed an infection. On the other hand, the incidence of SIRS in patients who did not develop an infection was high on both the third and fourth days after esophageal surgery. It was nearly impossible to predict who would develop an infection in esophageal surgery. The high incidence of postoperative infections, and their significant consequences justify planned successive postoperative antibiotic use in esophageal surgery.

[The effects of Ara-A on viral markers in duck hepatitis B virus carrier ducks].

Duck hepatitis B virus (DHBV) carrier ducks of one week old were injected with Ara-A (adenine arabinoside) of different dose including 2.5 (11 ducks), 5.0 (11), 10.0 (10) and 20.0 (10) mg/kg for 14 days. This antiviral effect showed dose-dependence up to 5.0 mg/kg and this dose seemed effective to obtain significant antiviral effect. Viral DNA and DNA polymerase activity were reduced significantly from the 1st week after starting the administration of Ara-A. This antiviral effect was maintained even at the 1st week after discontinuation of the drug. These findings were quite similar to those observed in HBV carriers. With the increasing necessity of Ara-A treatment in patients who will not respond to interferon therapy, DHBV seemed a suitable model for the investigation of the dose and antiviral effect of Ara-A treatment in humans.

Cytoskeletal abnormalities in relation with meiotic competence and ageing in porcine and bovine oocytes during in vitro maturation.

We investigated the frequencies of cytoskeletal anomalies in metaphase-II (M-II) and incompetent [arrested at an immature metaphase (IM) stage] porcine and bovine oocytes during in vitro maturation (IVM) in relation with ageing by immunostaining and confocal microscopy. In porcine oocytes, meiotic arrest at the IM stage was associated with abnormalities of cortical actin but not with abnormal spindles. Prolongation of IVM culture to 52 h did not affect microfilament and spindle abnormalities, but reduced the microfilament-rich area overlaying the spindle. Meiotic arrest of bovine oocytes at the IM stage was associated with degenerations of microfilaments, and the frequencies of abnormal spindles were also higher than those of M-II oocytes. Ageing of bovine oocytes (IVM for 30 h) did not affect cortical microfilaments but increased the frequency of spindle alterations in both M-II and IM bovine oocytes. These results suggest that, in both species, altered ability of oocytes to polymerize F-actin might be a possible reason for the failure of polar body extrusion during IVM. Also, there seem to be differences between the two species in the sensitivity of oocytes to suffer ageing-related spindle damages.

Development of single blastomeres derived from two-cell embryos produced in vitro in pigs.

The objective was to investigate development of single blastomeres derived from IVP two-cell porcine embryos. There was no difference (P > 0.05) in blastocyst rates among intact two-cell embryos (IN), zona-free two-cell embryos (ZF), and single blastomere (SB) groups (50.0 ± 9.7, 57.4 ± 5.7, and 45.1 ± 7.2%, respectively; mean ± SEM). However, blastocyst yield for the SB group (90.2 ± 14.4%, based on the original number of two-cell embryos before blastomere separation) was higher (P < 0.05) than those of IN and ZF groups. Although the number of inner cell mass (ICM) and trophectoderm (TE) cells in SB blastocysts (6.2 ± 0.8 and 15.5 ± 1.1, respectively) was lower (P < 0.05) than those in IN (12.4 ± 1.3 and 26.0 ± 3.8) and ZF blastocysts (10.7 ± 1.6 and 26.4 ± 3.4), ICM:TE ratios did not differ significantly among groups. Expressions of transcripts associated with cellular organization (TUBA1 and TUBB) were reduced (P < 0.05) in SB versus IN blastocysts. However, there was no significant difference among groups for expression of transcripts associated with responses to stress (HSPE1, HSPD1, and HSPCA) or glucose catabolism (ENO1, COX6C, COX7B, NDUFA4, NDUFA13, UCRC, and UQCRFS1) in blastocysts. The percentage of the sister blastomere pairs in which both cells developed to blastocysts (36.6 ± 5.3%) or both degenerated (46.3 ± 10.3%) were higher (P < 0.05) than that of the pairs in which one developed to blastocyst while the other degenerated (17.1 ± 7.8%). When both pairs developed to blastocysts, one blastocyst had more (P < 0.05) ICM and TE cells (8.2 ± 1.2 and 20.2 ± 2.1, respectively) than the other (5.2 ± 0.9 and 13.5 ± 1.1), although ICM:TE cell ratios were not significantly different. In conclusion, blastomere separation at the two-cell stage significantly increased blastocyst yield from IVP porcine embryos. This might be a useful approach for conservation of rare pig breeds, in which low numbers of embryos limited the success of embryo transfer.