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We attempted to deliver adriamycin (ADM) to lymph nodes by using sulfatide-inserted liposomes, which consist of phosphatidylcholine, cholesterol, and sulfatide (molar ratio, 5 : 4 : 1) (Lip-ADM). We measured the concentrations of ADM in regional lymph nodes of the stomach after the injection of Lip-ADM or free ADM into the gastric mucosa of dogs, and found that in the case of Lip-ADM, ADM levels in all regional nodes were significantly higher than those in the case of free ADM and these high levels were maintained for 48h. Then, we transplanted a mammary tumor into foot pads of mice to make inguinal lymph node metastases. Upon subcutaneous injection into the femur, Lip-ADM brought about a higher concentration of the drug in the metastasized cells at 24 hours than did the free ADM. These data suggest that liposomes entrapping ADM are effective for the treatment of lymph node metastases of gastric cancers.

Effect of moxisylyte on the lower urinary tracts was studied with the urethral pressure profile (UPP) and balloon method in anesthetized female dogs. In the UPP, moxisylyte produced relaxation in both the proximal and distal urethras. The relaxation effects of prazosin and bunazosin on the distal urethra was weaker than on the proximal urethra. In the balloon method, moxisylyte, prazosin, bunazosin and phentolamine caused a decrease in urethral pressure dose-dependently. The ID25 values of moxisylyte, prazosin, bunazosin and phentolamine were 23.4, 0.43, 0.76 and 33.1 micrograms/kg, respectively. In the balloon method, moxisylyte noncompetitively antagonized phenylephrine induced contraction of the urethra at high doses, whereas prazosin, bunazosin, phentolamine and yohimbine competitively antagonized phenylephrine induced contraction of the urethra. These results suggest that moxisylyte relaxes both the proximal and distal urethras due to alpha 1-adrenoceptor antagonistic and direct urethral smooth muscle relaxant actions. Therefore, moxisylyte is useful for the therapeutic treatment of micturitional disorder.

We report here the effect of activated vitamin D3 (Vit. D3) in postoperative hypoparathyroidism. We performed 42 total thyroidectomies and 43 sub-total thyroidectomies from 1978 to 1989.Hypoparathyroidism occurred in 36 patients (85.7%) after total thyroidectomy and 5 (11.6%) after sub-total thyroidectomy. We treated 18 of these patients with Vit. D3 only (43.9%) and 21 with Vit. D3+calcium (51.2%) orally. Treatment with Vit. D3 only is easier and more useful than that with Vit. D3 + calcium in controlling the serum calcium level of patients with hypoparathyroidism.We should monitor not only the serum calcium level but also the serum albumin to determine the real serum calcium level.With Vit. D3, which we prescribed in doses of 0.5-6.0μg (mean: 2.47±1.45μg) per day, we could control the serum calcium level well in patients with postoperative hypoparatyroidism without a high calcium level in the urine.It is probably concluded that Vit. D3 treatment alone is adequate for postoperative hypoparathyroidism patients, who have normal calcium uptake from the digestive tract, and it is the easiest and safest way to control the serum calcium level.

In order to rule out low-grade osteoporosis in postoperative hypoparathyroidism patients treated with activated vitamin D3, we examined (1) the midportion of parathyroid hormone (M-PTH, relatively stable compared to other PTH), (2) osteocalcin (osteoblast producing Gla protein), (3) multiple scanning X-ray photodensitometry (MD/MS method, using X-ray film of secondary metacarpal bone with alminium steps).Sixteen patients were examined: 13 of a series of 45 who had had total thyroidectomy and 3 of a series of 49 who had had subtotal thyroidectomy. In spite of normal serum calcium levels, 9 patients were diagnosed as osteoporosis by the MD/MS methods. Increasing of the vitamin D3 dose reduced the lower-grade osteoporosis in these patients within 3-4 months.It is concluded that osteocalcin and the MD/MS methods are useful in demonstrating mild osteoporosis in patients with postoperative hypoparathyroidism, in spite of normal serum calcium levels. We can monitor the doses of vitamin D3 during long term therapy with the use of these examinations.

We previously constructed novel liposomes for efficient transfection of an entrapped gene into mammalian cells [Koshizaka, T., Hayashi, Y., and Yagi, K. (1990) J. Clin. Biochem. Nutr., 7, 185-192]. By encapsulation of histone, a nuclear protein, into the above liposomes at a histone/DNA ratio (w/w) of 0.2, the expression in COS-1 cells of β-galactosidase activity by the plasmid pCH110, which contains the lacZ gene under the control of the SV40 early promoter, was found to be increased by -50%. The increased expression efficiency seems to be due to the efficient transfer of the plasmid to the nucleus after its incorporation into the cells, probably through the protection of the DNA by its binding with histone and/or accelerated transfer of the DNA to the nucleus by this protien.

Familial primary pulmonary hypertension was found in two siblings (sister and brother). The woman noted exertional dyspnea at the age of 28 yr. The younger brother noticed exertional dyspnea, cough with bloody sputum and pretibial edema at the age of 38 yr. We diagnosed them as primary pulmonary hypertension as based on hemodynamic and histopathological findings. Regardless of the treatment, both patients died of right-sided heart failure with a short time course. We examined their family members, but there were no other members with primary pulmonary hypertension.

Objective Meningiomas are the most common neoplasms of the central nervous system and are more frequent in women than in men. Many studies have been conducted to determine whether the progesterone receptor (PR) and estrogen receptor (ER) are present or absent in meningiomas. No previous studies, however, have investigated the status (presence or absence) of gonadotropin-releasing hormone (GnRH) and its receptor (GnRH-R), two major factors related to PR and ER, in meningiomas. This study aims to determine the status of GnRH and GnRH-R and to elucidate the correlations of GnRH and GnRH-R with PR, ER, and clinical features in meningiomas. Methods Eighty-two specimens of human meningiomas were obtained for immunohistochemical analysis with anti-GnRH, anti-GnRH-R, anti-PR, anti-ER, and anti-Ki-67 (MIB-1) antibodies, and for RT-PCR analysis of the mRNA expressions of GnRH and GnRH-R. Correlations of GnRH and GnRH-R with PR, ER, Ki-67, and clinical features such as age, sex, tumor grade, and tumor histology were assessed. Results Seventy-eight (95.1%) of the 82 meningiomas reacted positively in the cytoplasm for the GnRH-R. Forty-nine (59.8%) of the 82 cases reacted positively in the cytoplasm for the GnRH. The positive immunoreactivity for GnRH-R and GnRH was confirmed by the RT-PCR analyses of mRNA. Forty-seven (96%) of the 49 cases with positive immunoreactivity for GnRH-R also had positive immunoreactivity for GnRH. PR expression was higher in the tumors positive for GnRH-R (p = 0.002), and a significantly higher proportion of tumors from male patients exhibited positive immunoreactivity for GnRH (p = 0.02). No significant correlations were found between the status of GnRH-R or GnRH with other clinicopathological features. Conclusion Over half of meningiomas may be regulated by GnRH–GnRH-R expression in an autocrine fashion. This unique expression profile of GnRH and GnRH-R may open the way to the development of GnRH analogs as a treatment tool in the future.

Objectives Hemangiopericytomas (HPCs) are rare neoplasms with relatively high rates of recurrence and extracranial metastasis. Though the differential diagnoses from angiomatous meningiomas and from solitary fibrous tumors (SFTs) are both important, the latter diagnosis is somewhat more important in light of the benign prognosis of SFTs and the difficulties in distinguishing SFTs from HPCs. Newly developed immunohistochemical methods reveal differences in the specific immunohistochemical features of HPCs and SFTs. To elucidate whether SFTs have been misdiagnosed as HPCs in the past, our group used recent immunohistochemical methods to re-evaluate tissues that had been originally diagnosed as HPCs. We also compared the clinical features of these cases. Patients and methods Thirteen sequential cases of HPC diagnosed in Kanazawa University Hospital and Kumamoto University Hospital between 1970 and 2006 were retrospectively analyzed by immunohistochemical staining for CD34, Bcl-2, epithelial membrane antigen (EMA), vimentin, and S100 protein, and by measurement of the MIB-1 labeling index (LI). The cases were then re-evaluated and newly diagnosed based on the results of the immunohistochemical stainings. The clinical course of each case was also evaluated. Results Four of the 13 cases were newly diagnosed as SFTs and eight were reconfirmed as HPCs, based on the immunohistochemical studies for CD34, Bcl-2, and reticulin staining. One case was newly diagnosed as meningioma on the basis of a strong EMA positivity. The MIB-1 LI was less than 1% in 12 of the cases. In two cases, one case of HPC and the other of meningioma, the MIB-1 LI was relatively high, 8% and 4% respectively. All eight of the HPCs recurred, and 5 of the HPC patients died of the disease. Only one case of the SFTs recurred. Conclusion Our study suggests that a relatively high percentage of the tumors diagnosed as HPCs in the past may have in fact been intracranial SFTs. Immunohistochemical examinations of CD34, Bcl-2, and reticulin stains are keys for the differential diagnosis. Given that SFTs have a considerably better prognosis than HPCs, it is important to carry out meticulous immunohistochemical examinations for the primary diagnosis.

The aim of this study was to evaluate the relevance of anal endosonography (AES) during Georgeson laparoscopy-assisted anorectoplasty (GLA) for high/intermediate type imperforate anus (HIIA) using a continence evaluation questionnaire (CEQ) and postoperative AES (post-AES).Fifteen HIIA patients who underwent GLA were enrolled in this prospective study. Six had intraoperative AES (op-AES) to confirm the accuracy of the positioning of the pull-through (PT) canal and 9 did not (no-AES). All subjects were reviewed prospectively by one surgeon and all had post-AES to measure the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock to confirm that the PT colon was central. A CEQ (5 parameters: frequency of motions, severity of staining, severity of perianal erosions, anal shape, and requirement for medications; maximum score: 10) was administered to 9 subjects (4 from op-AES and 5 from no-AES) followed-up for over 3 years to assess continence.Mean age at GLA for both groups was not statistically different. There were no statistical differences between the two groups for thickness of the ES and PR on post-AES. Mean annual CEQ scores for the op-AES group 1, 2, 3, 4, and 5 years after GLA were 5.3, 6.3, 7.4, 8.4, and 8.5, respectively, while for the no-AES group were 5.9, 7.1, 8.1, 8.2, and 8.2, respectively; all differences were not statistically significant.Our results suggest that op-AES is not necessary during GLA because there is no difference in clinical and functional status that can be attributed to op-AES.

The rice genome contains two ent-copalyl diphosphate synthase genes: OsCPS1 acts in gibberellin (phytohormone) biosynthesis, and OsCPS2/OsCyc2 acts in the synthesis of oryzalexins A-F and phytocassanes A-E (phytoalexins). We characterized the enzymatic properties of recombinant OsCPS2/OsCyc2 fused with a tag-protein at the N-terminus, and compared them to those of OsCPS1. Several enzymatic properties of OsCPS2/OsCyc2, including the optimal pH, optimal temperature, divalent cation requirement, and kinetic values for the geranylgeranyl diphosphate (GGDP) substrate, were almost the same as those of OsCPS1. However, OsCPS2/OsCyc2 activity was not inhibited by 50-60 muM GGDP substrate, by which the OsCPS1 activity was inhibited. Furthermore, the OsCPS1 activity exhibited approximately 70% inhibition by 100 muM Amo-1618 (a gibberellin biosynthetic inhibitor), whereas the OsCPS2/OsCyc2 activity exhibited approximately 10% inhibition. These results indicate that the properties of OsCPS2/OsCyc2 were partially different from those of OsCPS1, although OsCPS2/OsCyc2 catalyzes the same reaction step as OsCPS1.

Glioblastoma multiforme (GBM) can be divided into genetic subsets: approximately one-third of GBM, primarily in older adults, have EGFR amplification; another one-third, primarily in younger adults, have TP53 mutation. The majority of GBM also have homozygous deletions of the CDKN2 (p16/MTS1) gene, resulting in cell cycle deregulation and elevated proliferation indices. We evaluated the relationship between CDKN2 deletions and the GBM subsets as defined by EGFR amplification or TP53 mutation in 70 GBM. Twenty-eight cases (40%) had EGFR amplification, 21 (30%) had TP53 mutation, and 21 (30%) had neither change. CDKN2 deletions were present in 36 (51%) GBM. Of the 28 GBM with EGFR amplification, 20 (71%) had CDKN2 deletion (p = 0.0078). The remaining 16 cases with CDKN2 loss were divided between GBM with TP53 mutations (6 cases) and GBM with neither EGFR amplification nor TP53 mutation (10 cases). Thus, CDKN2 deletions occur twice as commonly in GBM with EGFR amplification (71%) than in GBM with TP53 mutation (29%). CDKN2 deletions occurred in GBM from patients somewhat older than those patients with GBM lacking CDKN2 deletion (mean age 53 vs. 48 years). Specifically among GBM with EGFR amplification, those with CDKN2 deletions also occurred in patients slightly older than those few GBM without CDKN2 deletions (mean age 55 vs. 51 years). The presence of CDKN2 deletions in most GBM with EGFR amplification and in generally older patients may provide one explanation for the potentially more aggressive nature of such tumors.

An 8-year-old girl experienced five repeated hemorrhagic episodes around the cerebellar vermis. Surgical specimens histologically showed abnormal vascular components invaded by tumor cells. Autopsy disclosed medulloblastoma disseminating in the subependymal zone of the ventricles. An atypical clinical course is reported with special attention to the cause of hemorrhage.

The surgical treatment of 100 cases with congenital dilatation of bile duct with special reference to late complications was analyzed. There were no deaths nor occurrences of malignancy. Among 91 patients who had undergone the standard operation, namely total excision of the dilated extrahepatic bile duct and reconstruction after Roux-en-Y hepaticojejunostomy, there were one early complication (pancreatic juice leakage) and five late complications (four intrahepatic gallstones and one liver abscess). The cause of intrahepatic gallstone formation after a total excisional operation was attributed to the remaining intrahepatic bile duct dilatation and the stenosis located between the intrahepatic bile duct dilatation and the common hepatic duct. Accordingly, these results support the total excisional procedure for this condition; however, with regard to the cases associated with cystic dilatation of intrahepatic bile ducts, completely free bile drainage from the dilated intrahepatic biliary system should be performed at the radical operation.

Properties of hydrogenated amorphous silicon (a‐Si:H) prepared by glow discharge decomposition of silane with visible light illumination on a growing surface have been described. The visible light illumination during growth has resulted in smaller light‐induced changes, slightly lower photoconductivity before the prolonged illumination, lower content of bonded hydrogen, and lower ratio of the number of silicon dihydrides to that of total silicon‐hydrogen bonds. Formation of Si network and consequent structural disorder of SiSi bonds, on the other hand, has not been affected by the illumination during the growth. The improved stability against the long exposure to light can be ascribed to the lower bonded hydrogen content and the reduced density of SiH2 bonds, while the structural disorder of the Si network does not have a direct relationship with the light‐induced changes.

The effect of gate oxide(insulator) thickness on device parameters of a thin-gate-oxide MOSFET relating intrinsic circuit performance has been analyzed through a 1-D numerical simulation with a long-channel approximation. The intrinsic device operation speed in the form of transconductance/gate input capacitance has been found to increase reciprocally with gate-oxide thickness down to less than 5 nm with scaled channel length and width.

From 1976 to 1989, 51 patients with squamous cell carcinomas of the head and neck underwent simultaneous bilateral neck dissections, 19 had secondary contralateral neck dissections, and 169 had unilateral neck dissections at the Department of Otolaryngology, Asahikawa Medical School.The indications for simultaneous bilateral neck dissection were categorized into six groups: I, clinically palpable bilateral nodes; II, primary lesions large enough to suggest bilateral neck metastasis; III, development of bilateral neck masses during preoperative treatment; IV, recurrence of primary lesions with doubtful neck metastasis; V, large ipsilateral lymphnodes suspicious of contralateral metastasis; VI, technical convenience for reconstructive procedure and/or the patient's general condition.According to the TNM classification, the coincidence of N and pN was 25.5% in the simultaneously operated group, although 39.2% of them had bilateral neck metastase. The most common cause of death was distant metastasis to lungs or bones. Gontralateral neck dissection was performed secondarily in 19 of the 188 patients treated with unilateral neck dissection.Postoperative complications were mainly venous stagnation and neurological dysfunction. Bilateral ligation of the internal jugular veins was not related to these complications.The results suggest that more than two-thirds of patients with squamous cell carcinoma of the head and neck can be treated with unilateral neck dissection and that simultaneous bilateral neck dissection is justified in selected cases.

日本肺癌学会組織分類規約にもとついて, 類表皮癌, 腺癌, 大細胞癌と診断された311例の手術別出標本について, それらの組織亜型, 分化度と患者の3年および5年生存率との対比をおこなった.その結果, 腺癌, 類表皮癌, 大細胞癌症例の順に, さらに類表皮癌, 腺癌症例においては高分化, 中分化, 低分化型の順に3年および5年生存率は低下する傾向が認められた.これらの結果から日本肺癌学会組織分類規約にもとついた組織亜型分類あるいは分化度分類と患者の術後生存率との間に関連性の存在することが示唆された.

Subcutaneous administration of Z-100 twice a week starting immediately after supralethal whole-body irradiation of mice produced a prolongation of survival time. The effect of Z-100 on the hematopoietic system was thought to have contributed to the prolongation and was thus investigated. A single subcutaneous dose of Z-100 immediately after irradiation inhibited reduction of the total number of nucleated cells in the femoral bone marrow of the treated mice, although the inhibition was not by promotion of the proliferation of specific cells but by promotion of the recovery of multiple cell lines. Treatment with Z-100 promoted colony formation in the spleen of the treated mice and CFU-S formation in the femoral bone marrow, indicating that the drug accelerated the recovery of hematopoietic stem cells. The recovery of CFU-C count was also promoted by Z-100, which suggested that the drug has a restoring effect on the recovery of granulocytic and macrophagic precursor cells. Furthermore, Z-100 produced a greater increase in the CSF activity in the serum of irradiated mice, leading to the presumption that CSF induced by Z-100 was greatly involved in promoting the recovery of the above-mentioned hematopoietic stem cells. We conclude that Z-100 prolonged survival time of irradiated mice by promoting recovery of hematopoiesis of the mice.

Purpose: The aim of the study was to investigate posttraumatic stress disorder (PTSD) in mothers of children who have undergone surgery for congenital disease at a pediatric surgery department. Methods: A questionnaire survey was carried out in 145 mothers of children who had undergone surgery and were still alive. For comparison, the mothers were categorized into 3 groups according to the severity of their child's disease. Results: Of the 145 mothers, 29 (20%) were likely to be diagnosed as having developed PTSD at the time of the survey. Posttraumatic stress disorder symptoms correlated with factors such as anxiety and condition of the child. In terms of the disease severity of the child, factors such as anxiety tended to be observed more frequently in the higher disease severity group, whereas the proportion of mothers likely to be diagnosed as having developed PTSD was smallest in the moderate-severity group. Conclusions: Twenty percent of the mothers of children had probably developed PTSD. In the moderate-severity group, there seemed to be a factor that alleviated PTSD symptoms. Because mothers provided effective care for the symptoms of children in the moderate-severity group, this observation suggests that participation of the mother in their child's treatment might prevent them from developing PTSD symptoms.

Background/Purpose Advances in the management for biliary atresia (BA) have improved the prognosis and has greatly increased the number of long-term survivors. Even the long-term survivors, however, still face some problems. This retrospective review was performed to assess pregnancy-associated issues in long-term survivors after surgery for BA. Materials and Methods Of 55 patients with BA surviving for 16 years or more without liver transplantation, 9 patients have experienced pregnancy and delivery. Clinical courses, the outcome of pregnancy and delivery, and current statuses were retrospectively evaluated from their clinical records. Results The study revealed 14 pregnancies and 11 deliveries. Before pregnancy, no patient showed visible jaundice, but 6 patients had some complications such as episodes of cholangitis and portal hypertension. Two of the patients had both conditions and 2 others developed visible jaundice after pregnancy. One intrauterine fetal death occurred. Conclusions Our retrospective review suggests that the previously mentioned conditions can be risk factor for cholangitis and gastrointestinal bleeding during or after pregnancy but are not considered to be contraindications for pregnancy and delivery. Complications can occur with pregnancy even during the normal course. Thus, careful observation is recommended.

Huge subperiosteal hematomas extending beyond the cranial sutures are rare and clinically, their distinction from subgaleal hematomas may be difficult. We report a subperiosteal hematoma that involved the entire scalp including the orbit and recurred within 6 months. We discuss our neuroradiologic and histopathologic findings and our management strategy.

Hepatoblastoma is one of the common pediatric solid tumors with frequent mutation of the beta-catenin gene which might be an early event of its carcinogenesis. However, the detailed molecular mechanism is still unknown. We studied the expression levels of CCAAT/enhancer binding protein alpha (C/EBPalpha) and C/EBPbeta, which regulate differentiation and growth of embryonic hepatocytes, to establish whether or not they were involved in affecting the clinical behavior of hepatoblastoma. The quantitative real-time reverse transcriptase-PCR revealed that expression of C/EBPalpha mRNA was significantly up-regulated in tumors 223% (p=0.013) as compared with that in adjacent normal livers, while expression of C/EBPbeta was down-regulated to 27% (p=0.002). Of interest, the immunohistochemical analysis showed that expression of C/EBPalpha was higher and that of C/EBPbeta lower in the poorly differentiated tumor cells than in the well-differentiated cells within the same tumor. Furthermore, high expression of C/EBPalpha (p=0.047) as well as low expression of C/EBPbeta (p=0.025) was significantly associated with poor prognosis of the patients. Cox hazard model suggested that expression of C/EBPalpha and that of C/EBPbeta were independent indicators to predict the prognosis from age but not from histology. Thus, expression of C/EBP proteins may play an important role in the genesis and clinical behavior of hepatoblastoma probably by inducing different stages of arrest of differentiation.

Hepatoblastoma is one of the common pediatric solid tumors with frequent mutation of the beta-catenin gene which might be an early event of its carcinogenesis. However, the detailed molecular mechanism is still unknown. We studied the expression levels of CCAAT/enhancer binding protein alpha (C/EBPalpha) and C/EBPbeta, which regulate differentiation and growth of embryonic hepatocytes, to establish whether or not they were involved in affecting the clinical behavior of hepatoblastoma. The quantitative real-time reverse transcriptase-PCR revealed that expression of C/EBPalpha mRNA was significantly up-regulated in tumors 223% (p=0.013) as compared with that in adjacent normal livers, while expression of C/EBPbeta was down-regulated to 27% (p=0.002). Of interest, the immunohistochemical analysis showed that expression of C/EBPalpha was higher and that of C/EBPbeta lower in the poorly differentiated tumor cells than in the well-differentiated cells within the same tumor. Furthermore, high expression of C/EBPalpha (p=0.047) as well as low expression of C/EBPbeta (p=0.025) was significantly associated with poor prognosis of the patients. Cox hazard model suggested that expression of C/EBPalpha and that of C/EBPbeta were independent indicators to predict the prognosis from age but not from histology. Thus, expression of C/EBP proteins may play an important role in the genesis and clinical behavior of hepatoblastoma probably by inducing different stages of arrest of differentiation.

A simulation of interdigitated back-contact silicon heterojunction (IBC-SHJ) solar cells was performed using a quantum transport model to consider the quantum effect at the crystalline/amorphous (c/a) heterojunction interface. It was found that the impact of the quantum effect on the open-circuit voltage is comparable to that of the interface defect density at the c/a interface, indicating the importance of implementation of the quantum model. The optimal back-contact design was also discussed from the viewpoint of mass production, in which the design rule is relaxed. The degradation of the conversion efficiency by widening the gap between the p- and n-aSi:H layers can be compensated by improving passivation quality at the c/a interface.

Sine-wave modulated rf plasma has been used to control particle generation and growth in a plasma-enhanced chemical vapor deposition of silicon dioxide thin films using TEOS/O2. The density and the size of particles generated in the plasma are greatly reduced when the plasma is modulated with sine-wave modulation at low modulation frequency (

A 14-yr-old boy with total parenteral nutrition-dependent short-bowel syndrome associated with hypoganglionosis underwent the LR-IT by using a 150 cm segment of distal ileum taken from a healthy donor. The graft vessels were connected to infrarenal aorta and inferior vena cava. The immunosuppressive regimen consisted of daclizumab, tacrolimus, and steroid. The graft surveillance for ACR was accomplished using zoom endoscopy and mucosal biopsy. The blood trough level of tacrolimus was maintained between 20 and 25 ng/mL for the first 2 months, followed by 15-20 ng/mL thereafter. The 50 mg of daclizumab was administered on the day of operation, and same dosage was repeated at 2-wk intervals. The first ACR occurred on POD-9 and was progressive, and required a 14-day course of OKT-3 injection. After the treatment with OKT-3, the graft recovered from the ACR, and began to function well enough to discontinue the intravenous nutrition on POD-55. No infectious complication has occurred. The patient was discharged in POD-112, and currently tolerates full oral intake without requiring intravenous nutritional or fluid supplementation. The donor was discharged without any complications. The LR-IT could successfully be performed with minimal risk to the donor, and it can be a treatment of choice for patients with short-gut syndrome associated with hypoganglionosis.

Furaquinocin (FQ) A, produced by Streptomyces sp. strain KO-3988, is a natural polyketide-isoprenoid hybrid compound that exhibits a potent antitumor activity. As a first step toward understanding the biosynthetic machinery of this unique and pharmaceutically useful compound, we have cloned an FQ A biosynthetic gene cluster by taking advantage of the fact that an isoprenoid biosynthetic gene cluster generally exists in flanking regions of the mevalonate (MV) pathway gene cluster in actinomycetes. Interestingly, Streptomyces sp. strain KO-3988 was the first example of a microorganism equipped with two distinct mevalonate pathway gene clusters. We were able to localize a 25-kb DNA region that harbored FQ A biosynthetic genes ( fur genes) in both the upstream and downstream regions of one of the MV pathway gene clusters (MV2) by using heterologous expression in Streptomyces lividans TK23. This was the first example of a gene cluster responsible for the biosynthesis of a polyketide-isoprenoid hybrid compound. We have also confirmed that four genes responsible for viguiepinol [3-hydroxypimara-9(11),15-diene] biosynthesis exist in the upstream region of the other MV pathway gene cluster (MV1), which had previously been cloned from strain KO-3988. This was the first example of prokaryotic enzymes with these biosynthetic functions. By phylogenetic analysis, these two MV pathway clusters were identified as probably being independently distributed in strain KO-3988 (orthologs), rather than one cluster being generated by the duplication of the other cluster (paralogs).

We report a 45-year-old woman with a meningioma which was in contact with only the pituitary stalk on MRI. As the pituitary stalk has no dura mater, we suggest this tumour may have originated from the arachnoid membrane of the pituitary stalk. Though some reports have shown that meningiomas can arise from sites lacking a dural component, this is the first report of a meningioma originating from the pituitary stalk.

Gastrointestinal stromal tumors (GIST) are c-KIT positive neoplasm that occur predominantly in adults. A 13-year-old boy who presented with massive gastrointestinal hemorrhage was subsequently diagnosed with GIST of the stomach. The tumor originated from the lesser curvature with a narrow base and no infiltration into surrounding organs. Two metastatic lesions in the liver were also found. Total excision of the gastric lesion was performed followed by adjuvant imatinib mesylate chemotherapy. He has had no signs of recurrence during 8 months of follow-up. GIST in children are rare and their behavior is notoriously difficult to predict. We report our experience and review the literature.

Objective The aim of this study was to investigate the psychological status of Japanese children with congenital anorectal malformation and their mothers to develop appropriate psychiatric interventions. Method The subjects comprised 50 children with congenital anorectal malformation aged 0 to 16 years and their mothers. The psychology of children aged 7 to 16 years was investigated by Kovacs' Children's Depression Inventory (CDI). The psychology of their mothers was assessed by Spielberger's State-Trait Anxiety Index (STAI) and Zung's Self-rating Depression Scale (SDS). Results Depression was more marked in the children aged 12 to 16 years than in those aged 7 to 11 years according to the CDI. The mothers of preschool children showed higher levels of anxiety and depression than those of school-aged children, according to the STAI and SDS. Significant correlations between the CDI score of the children and the STAI or SDS score of the mothers were observed only among children aged 7 to 11 years. Conclusions The frequency of depression and anxiety among children with congenital anorectal malformation and that among their mothers was associated with the age of the child. Long-term postoperative psychosocial support for the children and their mothers may be required, taking into account the age of the child.

A 44-year-old man presented with a rare subfrontal schwannoma with headache. He had undergone radiation therapy for a pineal lesion 30 years previously, but the histology was not verified. On admission, neurological examination revealed no focal deficits including hyposmia. Computed tomography demonstrated a mass measuring 5 x 3 cm in the right anterior cranial fossa. Magnetic resonance imaging revealed a heterogeneously enhanced mass with multiple cysts. The tumor, which was clearly separable from the surrounding normal brain, was totally resected through a right frontal craniotomy. There was no adhesion between the tumor and the olfactory groove or cribriform plate. The frontal base dura was suspected to be the tumor attachment. The patient was discharged without hyposmia. Histological examination revealed a typical pattern of schwannoma consisting of Antoni type A and type B. Immunohistochemical staining was positive for S-100 protein but negative for epithelial membrane antigen. Subfrontal schwannoma not associated with the olfactory groove or cribriform plate is extremely rare. The operative findings and absence of hyposmia suggest that the meningeal branch of the trigeminal nerve was the origin of the tumor. However, the possibility of radiation-induced reactive Schwann cells cannot be excluded.

By regarding intense electron beams (∼340 keV, ∼2 kA, ∼10 ns) of a single pulse mode as a magnetic field source, we observed diffusion velocities of self‐magnetic fields of the beams through a 1.5 mm thick wall of a sintered superconducting Bi(2223) tube. Contrary to our prediction, fields of about 900 G out of the self‐magnetic fields diffused through the wall with an average diffusion velocity of 4×104 m/s, almost independently of the operation temperatures for the range from Tc(∼103 K) to 80 K. Such a slow, viscous diffusion process accounts for effective functioning of superconducting tubes as lenses (Supertrons) for the short electron beams.

✓ Solitary fibrous tumor (SFT) is a benign and rare neoplasm. To date, only 37 patients with intracranial SFTs have been reported. Although a number of the tumors were recurrent and some later underwent malignant transformation, none of these lesions progressed to cerebrospinal fluid (CSF) dissemination. In this paper the authors report a case of SFT in which the lesion recurred several times and ultimately was disseminated by the CSF. The patient was a 63-year-old woman with multiple intracranial and spinal tumors. Fifteen years before this presentation, at the age of 48 she had been hospitalized for resection of a falcotentorial tumor. During the ensuing 15 years she underwent multiple surgeries and sessions of radiation therapy for recurrent lesions. The exclusive location of her tumors in the subarachnoid space at the end of this 15-year period indicate CSF dissemination of the tumor. The tumor that was resected when the patient was 48 years old and the latest resected lesion were analyzed by performing immunohistological CD34, epithelial membrane antigen, vimentin, S100 protein, and reticulin staining, and determining the MIB-1 labeling index (LI). Most of the results were identical, and both tumors were diagnosed as SFT according to a staining pattern that showed a strong and diffuse positive reaction for CD34. Nevertheless, the authors noted that the MIB-1 LI increased from less than 1% in the original tumor to 13% in the latest tumor. The increased proliferation of MIB-1 indicates that the malignant transformation could have occurred during tumor recurrence with CSF dissemination.

An 86-year-old woman suffered sudden onset chest discomfort and dyspnea during sleep in the early morning of August 1, 2003. On admission, electrocardiography demonstrated complete atrioventricular block. Echocardiography showed severe hypokinesis in the left ventricular apex and hyperkinesis at the base. Serum cardiac deviant enzymes were slightly elevated. Based on these findings, acute myocardial infarction accompanied by complete atrioventricular block was suspected. Emergency coronary angiography performed under temporary pacing showed no significant stenosis in the coronary arteries. However, ventriculography demonstrated the presence of a foxhole-shaped left ventricular abnormality. A VDD type permanent pacemaker was implanted 5 days after the onset of the symptoms because the atrioventricular block had not improved. Following the implantation, pleural effusion and a temporary elevation of the body temperature occurred. The myocardial scintigraphy using 123I-metaiodobenzylguanidine (123I-MIBG) in the acute phase demonstrated extensive cardiac sympathetic nerve damage in the left ventricular apex. This condition was managed by medication including diuretics, angiotensin II receptor blocker and beta-blocker. Three months later, echocardiography and 123I-MIBG myocardial scintigraphy showed slight recovery of wall motion and sympathetic activity. However, mild pleural effusion persisted. A complete atrioventricular block was also present at this stage. In view of these findings, this case suggested that the foxhole-shaped left ventricular abnormality induced complete atrioventricular block with delayed recovery of wall motion.

Current management of congenital esophageal atresia (CEA) is described on the basis of our experience and recent literatures. Primary repair for Gross C type CEA is performed as modern standard treatment in infants without high-risk factors such as associated severe cardiac anomaly and respiratory insufficiency. Surgical strategy depends on preoperative condition of the infant therefore preoperative full assessment of the infant is very important. In general, delayed primary repair or staged repair on CEA is selected for premature infants weighing less than 1,500 g and high-risk infants. Recently, primary repair has become an effective option in premature infants without high-risk factors. In long-gap CEA, gastrostomy and/or closure of tracheoesophageal fistula is performed initially. Esophagoesophagostomy is carried out after attempts to decrease gap length. Intraoperative esophageal elongation is required in some infants. However esophageal replacement should be selected if esophageal elongation fails is impossible due to hypogenesis of lower esophagus. Thoracoscopic primary repair was recently reported as a new optional treatment. This treatment will be able to decrease the damage on the thoracic wall. However this procedure should be adopted after very careful discussion because it is difficult to accomplish without very skillful endoscopic surgical technique.

There are no data concerning the incidence of provoked coronary arterial spasms via intracoronary administration of ergonovine (ER). This study sought to establish the incidence of spasms due to intracoronary injection of ER in Japanese patients who underwent coronary angiography. The subjects were 596 consecutive patients (369 men, mean age 64.2 ±10.3 years) who were studied with a selective ER test. ER was administered in total doses of 40 µg into the right coronary artery and 64 µg into the left coronary artery. A positive spasm was defined as a total or subtotal occlusion. Coronary vasospasms were determined in 173 patients (29.0%). Spasms occurred often in patients with ischemic heart disease (43.3%); during effort and rest in patients with angina (46.3%), exertional angina (27.7%), recent myocardial infarction (36.7%), healed myocardial infarction (34.1%), and especially in patients with rest angina (55.5%), but were relatively uncommon in patients with nonischemic heart disease (3.7%). The incidence of provoked coronary spasms in this study was 2.2-2.6 times higher than in previous reports with intravenous ER administration. More spasms were superimposed on significant atherosclerotic lesions than on nonfixed atherosclerotic lesions (42.8% vs 24.0%, p

Hepatoblastoma is one of the most common malignant liver tumors in young children. Recent evidences have suggested that the abnormalities in Wnt signaling pathway, as seen in frequent mutation of the beta-catenin gene, may play a role in the genesis of hepatoblastoma. However, the precise mechanism to cause the tumor has been elusive. To identify novel hepatoblastoma-related genes for unveiling the molecular mechanism of the tumorigenesis, a large-scale cloning of cDNAs and differential screening of their expression between hepatoblastomas and the corresponding normal livers were performed. We constructed four full-length-enriched cDNA libraries using an oligo-capping method from the primary tissues which included two hepatoblastomas with high levels of alpha-fetoprotein (AFP), a hepatoblastoma without production of AFP, and a normal liver tissue corresponded to the tumor. Among the 10,431 cDNAs randomly picked up and successfully sequenced, 847 (8.1%) were the genes with unknown function. Of interest, the expression profile among the two subsets of hepatoblastoma and a normal liver was extremely different. A semiquantitative RT-PCR analysis showed that 86 out of 1188 genes tested were differentially expressed between hepatoblastomas and the corresponding normal livers, but that only 11 of those were expressed at high levels in the tumors. Notably, PLK1 oncogene was expressed at very high levels in hepatoblastomas as compared to the normal infant's livers. Quantitative real-time RT-PCR analysis for the PLK1 mRNA levels in 74 primary hepatoblastomas and 29 corresponding nontumorous livers indicated that the patients with hepatoblastoma with high expression of PLK1 represented significantly poorer outcome than those with its low expression (5-year survival rate: 55.9 vs 87.0%, respectively, p=0.042), suggesting that the level of PLK1 expression is a novel marker to predict the prognosis of hepatoblastoma. Thus, the differentially expressed genes we have identified may become a useful tool to develop new diagnostic as well as therapeutic strategies of hepatoblastoma.

We report on a girl with biliary atresia (BA) who also suffered with anorectal agenesis without fistula and complicated urogenital malformation. The outcome of patients with these severe anomalies is poor, but she has survived without liver and/or renal transplantation for more than 3 years. A careful treatment plan for each anomaly in addition to prevention of cholangitis and urinary tract infection is indispensable for managing these complicated anomalies.

We report herein the cases of two infants who developed right pneumonectomy syndrome, both of whom were born with gross C-type esophageal atresia (EA/TEF), and a hypoplastic right lung arising from the lower esophagus, being a bronchopulmonary foregut malformation (BPFM). Appropriate and well-timed treatments for a variety of sequelae primarily caused by the mediastinal shift must be considered after right pneumonectomy in early childhood.

Background/purpose The authors created a new artificial anal sphincter using a shape memory alloy (AS-SMA) to treat fecal incontinence and evaluated its validity. Methods AS-SMA consists of 2 Ti-Ni plates to sandwich the intestine and generates a pressure of 55 mm Hg at its resting position. With the electric power supply, the 2 metals bend to form an almondlike shape making a maximum gap of 33 mm between each other at the temperature of 55°C. The device was attached to the colostomy in a piglet and was operated several times a day for 1 month. Fecal continence, bowel movements, and general condition of the piglet were recorded. After the experiment, tissue damage around the device was investigated. Results Fecal continence was obtained while the device was in the resting position. When it was operated, bowel movements were observed. The bowel movements to operations ratio was 82:105 (78%). During the experiment, the animal had neither abdominal distension nor vomiting. At the autopsy, there was mild inflammation and shallow burns around the device. No compression injury was detected. Conclusions AS-SMA achieved fecal continence of the colostomy. With reduction of the associated side effects, it would be a potential substitute for an impaired anal sphincter.

Glioblastoma (GB) often has loss of heterozygosity on the chromosomes, 1p, 10p, 10q, 11p, 17p, 19q, 22q, and several others. In the case of chromosome 12q; however, it remains to be seen whether LOH occurs. Apaf‐1, the apoptotic protease activating factor‐1, located at chromosome 12q22‐23, is a major effecter of the p53 mediated apoptosis pathway, and Apaf‐1 inactivation due to chromosome 12q22‐23 LOH and hypermethylation may be involved in some of the neoplasms in malignancy. However, little is known about the frequency of the 12q22‐23 LOH or the state of Apaf‐1 in GB. To elucidate their involvement in GB, we analyzed a series of 33 GBs for chromosome 12q22‐23 LOH, Apaf‐1 mRNA expression, and Apaf‐1 protein expression, using microsatellite analysis, reverse transcription (RT) ‐PCR analysis, and immunohistochemical (IHC) analysis, respectively. We also evaluated if and how the 12q22‐23 LOH correlated with the p53 gene mutation and EGFR gene amplification. Chromosome 12q22‐23 LOH was detected in 14 (42%) of 33 cases. Among the examined cases with LOH at 12q22‐23, a low expression of Apaf‐1 mRNA was detected in 9 (69%) of 13 cases, and a low expression of Apaf‐1 protein was detected in 12 (86%) of 14 cases. The 12q22‐23 LOH was significantly correlated with low expression of mRNA and protein (p