A case of graft replacement of the ascending aorta to the aortic arch and the partial descending aorta in a single stage for thrombosed aortic dissection (debakey type II + IIIB)

We report here a case of graft replacement of the ascending aorta to the aortic arch and the middle portion of the descending aorta in a single stage for thrombosed aortic dissection. The patient was a 53-year-old male who was transfered to our hospital with a diagnosis of thrombosed aortic dissection. Conservative therapy was continued but three weeks after the onset, chest enhanced CT scan and digital subtraction angiography revealed an opacified false lumen in the ascending aorta and a ulcer like projection in the middle portion of the descending aorta. He was therefore diagnosed as having redissection in DeBakey type II + IIIb thrombosed aortic dissec- tion. Graft replacement of the ascending aorta, the aortic arch, and a part of the descending aorta was performed in a single stage via median stenotomy with the aid of extracorporeal circulation and selective cerebral perfusion. Postoperative digital subtraction angiography showd satisfac- tory reconstruction of the thoracic aorta. The patient is still leading a normal life two years after the operation.     

Ependymosarcoma with eosinophilic granular cells

Ependymosarcoma is a new entity of malignant gliomas composed of ependymal and sarcomatous components. We report a rare case of ependymosarcoma with eosinophlic cells which occurred to the right trigon of the lateral ventricle. A 62‐year‐old man complained of headaches over a 2‐month period. A hard, gray mass was found in the right trigon of the lateral ventricle during the operation. Although he received radiation and chemotherapy, the patient died due to tumor disseminating through the whole brain within 7 months after the operation. The histological examination revealed that the anaplastic glial components intermingled with the sarcomatous components. Immunohistochemically, sarcomatous cells were positive for α smooth muscle actin and desmin. However, anaplastic glial cells were not positive for these markers. In addition, Masson trichrome stain showed a plethora of collagen fibers between sarcomatous cells, but no collagen fibers were produced by the glial tumor cells. Solid focal papillary lesions of the glial tumor showed dot‐like epithelial membrane antigen and diffuse cytoplasmic D2‐40 immunoreactivity. Based on the above findings, these anaplastic glial tumor cells should show focal ependymal differentiation, and sarcomatous cells show myofibroblastic differentiation. In addition, almost 10% of the tumor cells in the neoplasm showed bright eosinophilic granules in the cytoplasm. These cytoplasmic eosinophilic granules and bundles were negative on PAS staining. Intracytoplasmic eosinophilic granules of tumor cells were strongly positive for αB‐crystallin, HSP 27 and GFAP, respectively. These findings suggest that the clinicopathological characteristics of the present case should be consistent with the criterion of ependymosarcoma by Rodriguez et al.     

EGCG inhibits activation of the insulin-like growth factor (IGF)/IGF-1 receptor axis in human hepatocellular carcinoma cells

The receptor tyrosine kinase (RTK) insulin like growth factor-1 (IGF-1)/IGF-1 receptor (IGF-1R) axis plays an important role in the development of hepatocellular carcinoma (HCC). EGCG inhibits activation of the various types of RTKs and that this is associated with inhibition of multiple downstream signaling pathways. In this study we examined the effects of EGCG on activity of the IGF/IGF-1R axis in HepG2 human HCC cells which express constitutive activation of this axis. The level of phosphorylated (i.e. activated) form of the IGF-1R protein (p-IGF-1R) was increased in a series of human HCC cell lines when compared with the Hc normal human hepatocytes. EGCG preferentially inhibited growth of HepG2 cells when compared with Hc cells. Treatment of HepG2 cells with EGCG induced apoptosis and caused a decrease in the p-IGF-1R protein and its downstream signaling molecules including the p-ERK, p-Akt, p-Stat-3, and p-GSK-3β proteins, both in the absence or presence of ligand stimulation. EGCG also decreased the levels of both IGF-1 and IGF-2 proteins and mRNAs, but increased the levels of the IGFBP-3 protein. These findings suggest that EGCG can overcome the stimulatory effects of IGFs on the IGF-1R dependent signaling pathway, thus expanding the roles of EGCG as an inhibitor of critical RTKs involved in HCC cell proliferation. These results provide further evidence that EGCG may be useful in the chemoprevention or treatment of liver cancer.     

Clinicl Significance of Mesangial IgM Deposits in Minimal Change Nephrotic Syndrome

The clinicopathologic features and the response to corticosteroid therapy were compared in 9 patients with minimal change nephrotic syndrome (MCNS) and diffuse mesangial IgM deposits (Group I) and in 32 of those without IgM deposits (Group II). However, serum IgM levels in Group I in both relapse and remission were significantly higher than those of Group II and controls (p<0.001). In Group I mesangial IgM deposits were diffuse in 9 (100%), mesangial C_1q was present in 4, IgA and fibrinogen were each observed in 1, respectively. Electron dense deposits in the mesangium were also present in 2 to 5 patients in Group I. No significant differences were found between the two groups in age of onset, sex ratio, laboratory data except for serum IgM level, duration before biopsy, follow-up periods, outcome, and response to steroid therapy. Our data suggest that a more severe degree of either impairment of mesangial clearance of IgM or overproduction of IgM may be involved in patients with MCNS and mesangial IgM deposits but that these patients could not be considered a distinct group of patients.     

Macular nerve fibers temporal to fovea may have a greater potential to recover function in patients with Leber's hereditary optic neuropathy

PURPOSE: It is known that even after visual loss, younger patients with Leber's hereditary optic neuropathy (LHON) can recover vision. The purpose of this study was to determine the mean age at onset for LHON patients with and without visual recovery who carried the 11778 mutation, and to determine the pattern of central vision recovery. METHODS: Thirty-five LHON patients with the 11778 mutation of mitochondrial DNA who had visited the Keio University Hospital between 1980 and 1999 and were followed for 2 to 20 years, were the subjects of this retrospective study. The patients who had recovered vision were tested by Goldmann perimetry, Humphrey perimetry, and landmark-driven fundus microperimetry with a scanning laser ophthalmoscope (SLO). The fixation status was assessed by SLO microperimetry. RESULTS: Nine of the 35 patients (14 of 70 eyes) demonstrated a recovery of visual acuity to better than 0.3 in at least one eye. The mean age of disease onset was 15.9 +/- 4.6 years in patients with visual recovery and 25.5 +/- 8.9 years in patients without visual recovery. This difference in the mean age at onset was significant (P =.0001; Welch t-test). These 9 patients (14 eyes) showed fenestrated central scotomas in testing by Humphrey 10-2 threshold and SLO microperimetry. The nasal side of the central visual fields had a higher sensitivity than the temporal side in 7 of the 9 patients in Humphrey 10-2 threshold testing. Areas insensitive to 0 dB were detected on the nasal side of the central retina in these patients by SLO microperimetry, and fixation stability was related to the degree of clinical visual acuity. CONCLUSION: The LHON patients with the 11778 mutation and a younger age of onset were more likely to show visual recovery. The findings made by perimetry suggest that the nerve fiber bundles in the nasal field (retina temporal to the fovea) may have a greater potential to recover function in LHON patients.     

Different effects of phencyclidine and methamphetamine on firing activity of medial prefrontal cortex neurons in freely moving rats

Immunohistochemistry detected calcium-binding proteins (CaBPs) in corpuscular and Merkel nerve endings of the rat vibrissa. CaBP-immunoreactive (ir) corpuscular endings were divided into two types: ramified and unramified endings. Ramified endings were subdivided into reticular and Ruffini endings. Unramified endings were identical to longitudinal lanceolate endings which have been described previously. Reticular and unramified endings as well as Merkel endings co-expressed neurocalcin (NC)- and parvalbumin (PV)-immunoreactivity (ir). However, such endings were devoid of peptide 19 (PEP19)-ir. PV-ir Ruffini endings were immunoreactive for PEP19 but not NC. The retrograde tracing method revealed that 34, 21 and 18% of trigeminal neurons which project to the infraorbital nerve exhibited NC-, PEP19- and PV-ir, respectively. In addition, 73 and 36% of the PV-ir neurons showed NC- and PEP19-ir, respectively. The content and co-expression of CaBPs in vibrissal low-threshold mechanoreceptors may depend on their terminal morphology.     

Prediction of relapse by plasma lipoprotein(a) concentration in children with steroid-sensitive nephrotic syndrome

AIM: To clarify whether plasma lipoproteins, including Lp(a), can predict relapse pattern in the first years after diagnosis of nephrotic syndrome (NS), we evaluated them in patients with steroid-sensitive NS. METHODS: We analyzed the medical records of 35 patients with steroid-sensitive NS who were seen by us from January 1992 to December 1999 followed for at least 1 year. These patients were divided into two groups. Group 1 consisted of 20 patients who infrequently relapse (IR: <2 in 6 months or <3 in a year), group 2 consisted of 15 patients who frequently relapse (FR: > or =2 in 6 months or > or =3 in a year). Clinical and laboratory findings such as age at onset, gender, urinalysis, serum level of total protein, albumin, and concentrations of serum lipid including lipoprotein(a) (Lp(a)) were investigated between group 1 and group 2. RESULTS: The concentration of plasma Lp(a) in group 2 was higher than that in group 1 (81.0 +/- 35.2 vs. 35.9 +/- 26.5 mg/dl, p < 0.01). On multivariate analysis using logistic regression model, the concentration of plasma Lp(a) was an independent risk factor for relapse of NS. CONCLUSIONS: Our findings suggest that of all the laboratory data examined, high values of Lp(a) can predict future relapse of NS and should be well documented.     

Non-blood contacting biventricular support for severe heart failure

BACKGROUND: Direct mechanical ventricular actuation (DMVA) is a non-blood contacting method of biventricular support. DMVA employs a vacuum attached, pneumatically regulated, flexible membrane to transfer both systolic and diastolic forces to the ventricular myocardium. The purpose of this study was to determine if DMVA effectively restores pump performance when applied to the severely failing heart. METHODS: Bovines (n = 10) underwent thoracotomy and were instrumented for continuous hemodynamic monitoring. Cardiac failure was induced by beta1-blockade to achieve a cardiac index of < 1.5 l/min/m2 for 1 hour. Heart rate was maintained at 100 bpm by atrioventricular sequential pacing. Synchronous DMVA support was then applied for 3 hours. RESULTS: Eight animals achieved significant reductions in cardiac index and mean arterial pressures (35%* and 43%* control, respectively; *p < 0.05). DMVA restored cardiac index to baseline and significantly increased arterial pressures (p < 0.05; DMVA versus cardiac failure). Pulmonary flow and mean pulmonary artery pressures were similar to baseline during DMVA (p = NS). Pathologic exam did not demonstrate evidence of significant device trauma. CONCLUSIONS: DMVA support can effectively restore pump performance of the acutely failing heart. Synchronization may be inherent to the stimulus of cardiac compression. These data further substantiate DMVA's potential as an adjunct to the field of circulatory support.     

Malignant fibrous histiocytoma of perirenal tissue: a case report

A 75-year-old woman was admitted to our hospital with an extremely large retroperitoneal tumor that had been detected with ultrasound on a routine health check. She had no complaint except lumbar pain. Computed tomography revealed a heterogenous tumor located outside the right kidney which was enhanced gradually. Doppler ultrasound showed mild vascularity in the tumor. We performed tumorectomy and right nephrectomy because the tumor was adherent to the right kidney. The tumor was 16 x 11 x 7 cm in size and weighed 621 g. The histopathological diagnosis was malignant fibrous histiocytoma. The tumor was considered to have arisen from perirenal tissue.