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Study objectives Niemann-Pick type C (NPC) is an autosomal recessive and congenital neurological disorder characterized by the accumulation of cholesterol and glycosphingolipids. Symptoms include hepatosplenomegaly, vertical supranuclear saccadic palsy, ataxia, dystonia, and dementia. Some cases frequently display narcolepsy-like symptoms, including cataplexy which was reported in 26% of all NPC patients and was more often recorded among late-infantile onset (50%) and juvenile onset (38%) patients. In this current study, we examined CSF orexin levels in the 10 patients of NPC with and without cataplexy, which supports previous findings. Methods Ten patients with NPC were included in the study (5 males and 5 females). NPC diagnosis was biochemically confirmed in all 10 patients, from which 8 patients with NPC1 gene were identified. We compared CSF orexin levels among NPC, narcoleptic and idiopathic hypersomnia patients. Results Six NPC patients with cataplexy had low or intermediate orexin levels. In 4 cases without cataplexy, their orexin levels were normal. In 5 cases with Miglustat treatment, their symptoms stabilized or improved. For cases without Miglustat treatment, their conditions worsened generally. The CSF orexin levels of NPC patients were significantly higher than those of patients with narcolepsy-cataplexy and lower than those of patients with idiopathic hypersomnia, which was considered as the control group with normal CSF orexin levels. Discussion Our study indicates that orexin level measurements can be an early alert of potential NPC. Low or intermediate orexin levels could further decrease due to reduction in the neuronal function in the orexin system, accelerating the patients’ NPC pathophysiology. However with Miglustat treatment, the orexin levels stabilized or improved, along with other general symptoms. Although the circuitry is unclear, this supports that orexin system is indeed involved in narcolepsy-cataplexy in NPC patients. Conclusion The NPC patients with cataplexy had low or intermediate orexin levels. In the cases without cataplexy, their orexin levels were normal. Our study suggests that orexin measurements can serve as an early alert for potential NPC; furthermore, they could be a marker of therapy monitoring during a treatment.

The aim of this study was to determine whether l-methyl-[

The OSIRIS study investigated efficacy and safety of simeprevir plus sofosbuvir for eight or 12 weeks in hepatitis C virus (HCV) genotype 4-infected patients with METAVIR F0-F4 fibrosis. Sixty-three patients (33 treatment-naive and 30 peg-interferon/ribavirin (Peg-IFN/RBV)-experienced) enrolled in a partly randomized, open-label, multicentre, phase IIa study. Patients with F0-F3 fibrosis were randomized (1:1) into two groups (A1 and A2), stratified according to treatment experience and METAVIR score, to receive either eight weeks (Group A1, n=20) or 12 weeks (Group A2, n=20) of treatment. Patients with compensated cirrhosis (METAVIR F4) received 12 weeks of treatment (Group B, n=23). Treatment comprised simeprevir 150 mg and sofosbuvir 400 mg daily. The primary efficacy endpoint was sustained virologic response 12 weeks after planned end of treatment (SVR12). Safety and tolerability were assessed throughout. Overall, 92% (95% CI: 82-97) of patients achieved SVR12; 75% (15/20) in Group A1 and 100% in groups A2 and B. Patients who did not achieve SVR12 (n=5) experienced viral relapse during the first 32 days following treatment and were all prior Peg-IFN/RBV null responders. The most commonly reported treatment-emergent adverse events (TEAEs) were asymptomatic lipase increase (14%), pruritus (14%), headache (13%) and hyperbilirubinaemia (11%). No patients discontinued due to TEAEs. In conclusion, simeprevir plus sofosbuvir for 12 weeks achieved a 100% SVR rate in HCV genotype 4-infected patients with or without compensated cirrhosis (ClinicalTrials.gov: NCT02278419). The AE and laboratory profile were favourable and consistent with previous data for simeprevir plus sofosbuvir in eight- and 12-week regimens.

Wnt signalling plays a critical role in the development of cancer. Recent studies indicate that Wnt signalling is negatively regulated by secreted Wnt antagonists such as secreted frizzled related proteins (sFRPs) and Dickkopfs (Dkks). We compared Dkk family expression levels in normal prostate and prostate cancer cells and found a reduction in Dkk-3 expression in cancer cells. Ectopic expression of Dkk-3 inhibited colony formation in LNCaP and PC3 prostate cancer cell lines and inducible expression of Dkk-3 reduced LNCaP cell proliferation. Moreover, small interfering RNA-mediated downregulation of Dkk-3 enhanced cell cycle progression in untransformed RWPE-1 prostate epithelial cells. Immunohistochemical analysis revealed that Dkk-3 is expressed in a subset of normal prostate gland acini and that Dkk-3 expression is reduced in prostate tumours, particularly those with a high Gleason grade, suggesting a role for Dkk-3 in postmitotic differentiation. Consistent with this, depletion of Dkk-3 disrupted acinar morphogenesis of RWPE-1 cells in a three-dimensional cell culture model. Our results are consistent with the loss of Dkk-3 expression resulting in impairment of glandular structure and uncontrolled prostate epithelial cell (PrEC) proliferation, both of which are crucial for prostate cancer progression.

Objective To assess the clinical negative predictive value (NPV) of multiparametric MRI (mp-MRI) for prostate cancer in a 5-year follow-up. Materials and methods One hundred ninety-three men suspected of harboring prostate cancer with negative MRI findings were included. Patients with positive transrectal ultrasound (TRUS)-guided biopsy findings were defined as false-negative. Patients with negative initial TRUS-guided biopsy findings were followed up and only patients with negative findings by digital rectal examination, MRI, and repeat biopsy and no increase in PSA at 5-year follow-up were defined as “clinically negative”. The clinical NPV of mp-MRI was calculated. For quantitative analysis, mean signal intensity on T2-weighted images and the mean apparent diffusion coefficient value on ADC maps of the initial MRI studies were compared between peripheral-zone (PZ) cancer and the normal PZ based on pathologic maps of patients who had undergone radical prostatectomy. Results The clinical NPV of mp-MRI was 89.6% for significant prostate cancer. Small cancers, prostatitis, and benign prostatic hypertrophy masking prostate cancer returned false-negative results. Quantitative analysis showed that there was no significant difference between PZ cancer and the normal PZ. Conclusion The mp-MRI revealed a high clinical NPV and is a useful tool to rule out clinically significant prostate cancer before biopsy.

To clarify the mechanisms of impaired insulin secretion in Nagoya-Shibata-Yasuda (NSY) mice, an inbred strain of mice with spontaneous development of type 2 (non-insulin-dependent) diabetes mellitus, the insulin response to glucose (5.5 to 27.8 mmol/L) and nonglucose stimuli (glibenclamide, arginine, and BayK8644, a Ca-channel opener) was studied in vitro using isolated islets from male NSY and control C3H/He mice at 36 weeks of age by the batch incubation method. Insulin response to 5.5 mmol/L glucose was not significantly different between NSY and C3H/He mice, but insulin response to a high concentration of glucose (> or = 11.1 mmol/L) was significantly smaller in NSY mice than in control C3H/He mice. The dose-response curve of insulin secretion showed a markedly reduced maximum response, but almost normal glucose sensitivity in NSY islets. Insulin responses to glibenclamide (1 mmol/L), arginine (20 mmol/L), and BayK8644 (0.1 mmol/L) were also significantly smaller in NSY mice than in C3H/He mice. Insulin content of islets, in contrast, was significantly higher in NSY mice than in C3H/He mice. The impaired insulin response to glucose and nonglucose stimuli together with higher insulin content in islets in the NSY mouse suggest that a defect in voltage-dependent Ca(2+)-channel or thereafter in the cascade of insulin secretion may be responsible for impaired insulin secretion in NSY mice. NSY mice, therefore, could be a novel animal model of type 2 diabetes with a defect in insulin secretion at a different site from that in previously known animal models.

The ultrastructures of the upper surface layer of rat articular cartilage were studied with our "in vivo cryotechnique" followed by freeze-substitution method for scanning electron microscopy (SEM) or transmission electron microscopy (TEM). Rat hip or knee articular cartilage was quickly frozen by the in vivo cryotechnique with liquid isopentane-propane cryogen (-193 degrees C), and surface areas of some frozen specimens were freeze-fractured with a scalpel in liquid nitrogen. They were freeze-substituted and freeze-dried, ion-sputtered, and then observed in SEM. Other frozen specimens were routinely freeze-substituted and embedded in epoxy resin for TEM. Many globular structures were detected in the thick upper surface layer that had not been revealed by the conventional fixation methods. Their sizes were reduced by Triton X-100 treatment, and their localization was also detected in synovial fluid, as revealed by SEM. Such globular lipid-like structures in the upper surface layer of hip or knee articular cartilage might contribute to joint lubrication.

OBJECTIVE To clarify the contribution of the Asp905Tyr polymorphism of the musclespecific glycogen-targeting subunit of protein phosphatase 1 (PP1G) to insulin resistance and related diseases. RESEARCH DESIGN AND METHODS We investigated the Asp905Tyr polymorphism of the PPP1R3 gene, which encodes the muscle-specific glycogen-targeting subunit of PP1G, in 259 Japanese patients with NIDDM and 194 healthy control subjects. RESULTS No significant difference was found in the genotype distribution between NIDDM patients (n = 259; Asp/Asp = 0.10, Asp/Tyr = 0.44, Tyr/Tyr = 0.46) and healthy control subjects (n = 194; Asp/Asp = 0.13, Asp/Tyr = 0.37, Tyr/Tyr = 0.50) or between patient groups subdivided by the mode of treatment: NIDDM patients with insulin therapy (Asp/Asp = 0.14, Asp/Tyr = 0.46, Tyr/Tyr = 0.40) and those without insulin therapy (Asp/Asp = 0.07, Asp/Tyr = 0.43, Tyr/Tyr = 0.50). However, NIDDM patients with the Tyr allele, which was previously reported to be associated with insulin resistance, tended to have lower BMIs than those without this allele (Asp/Asp: 24.5 ±1.1 kg/m2, Asp/Tyr: 22.6 ± 0.4 kg/m2, Tyr/Tyr: 22.8 + 0.3 kg/m2, P = 0.06 by analysis of variance). CONCLUSIONS These data suggest that the Asp905Tyr polymorphism of the PPP1R3 gene is not associated with NIDDM or high BMI, both of which are known to be insulin-resistant states, in the Japanese population.

An insertion/deletion (I/D) polymorphism in the angiotensin-converting enzyme (ACE) gene has repeatedly been shown to be associated with ischaemic heart disease, but the association of this genetic marker with diabetic microangiopathy is controversial. To assess the association of the genotypes with the development of diabetic nephropathy or retinopathy, we performed a meta-analysis of data from the literature, using Mantel-Haenszel method followed by the Breslow-Day test for assessing homogeneity among data. In a total of 4773 diabetic patients from 18 studies with (n = 2495) and without (n = 2278) renal complications, the D allele was significantly associated with diabetic nephropathy (p < 0.0001) in a dominant model (summary odds ratio 1.32, 95 % confidence interval: 1.15 to 1.51). There was no significant evidence against homogeneity of the odds ratios (χ2 = 18.9, 20 df; p = 0.53). The association was significant both in non-insulin-dependent (p < 0.005) and in insulin-dependent diabetes mellitus (p < 0.05). Likewise, in a total of 2010 diabetic patients with (n = 1008) and without (n = 1002) retinopathy, there was no association of the I/D polymorphism with diabetic retinopathy. These data suggest that the ACE I/D polymorphism affects the risk for diabetic nephropathy, but not for diabetic retinopathy. [Diabetologia (1998) 41: 47–53]

The mouse ob gene and its human homologue OB have recently been cloned. The mutations in the ob gene are known to be associated with extreme obesity. The relationship between the human OB gene and disease, however, is largely unknown due to the lack of suitable markers within or adjacent to the OB gene. To obtain informative markers, we searched for simple tandem repeat polymorphisms in the genomic sequence of the human OB gene and identified a novel tetranucleotide repeat in the 3′ flanking region. Fifteen alleles were detected in this marker with a heterozygosity of 0.85 and polymorphism information content of 0.83, indicating a highly informative nature of this marker. Two-point linkage mapping in two Centre Etude Polymorphisme Humaine (CEPH) reference families suggested that this marker is located in the interval between D7S514 and D7S530, the same interval where the OB gene is located (recombination fractions with D7S514 and D7S530 were 0.026 and 0.034, respectively). Although allele frequency distributions of this marker did not differ between 84 control subjects and 69 NIDDM patients, there was a tendency to higher body weight in control subjects with class I/class I genotype than in those without this genotype (68.8 ± 11.1 vs 60.8 ± 10.3 kg, p = 0.05). The highly polymorphic nature of this marker and its location in the OB gene makes this marker useful for linkage studies of the OB gene with a number of phenotypes, such as obesity, non-insulin-dependent diabetes mellitus, hypertension and the insulin resistance syndrome. [Diabetologia (1996) 36: 1398–1401]

To determine the effect of an aldose reductase inhibitor, SNK-860, on the worsening of the electroretinogram (ERG) during a diabetic state, rats with streptozotocin-induced diabetes were administered SNK-860 (1 or 4 mg kg-1 orally) daily for 4 weeks. The effectiveness of SNK-860 in prolonging the peak latencies of oscillatory potentials in the b-wave of the electroretinogram of diabetic rats varied between these different waveform components (designated O1, O2 and O3). SNK-860 (4 mg kg-1 day-1) either completely or partially prevented the prolonged peak latencies at O1 and sigma(O1 + O2 + O3). The drug failed to shorten the latency of the O2 and O3 components, and produced only a modest reduction in retinal levels of sorbitol and fructose, with no increase in myo-inositol. There was a significant correlation between the state of the ERG (components O1 and sigma(O1 + O2 + O3)) and the retinal levels of sorbitol and fructose (P < 0.01), but not of myo-inositol. It is concluded that a better understanding of the mechanism by which SNK-860 acts may provide new insight into the pathogenesis of hyperglycaemic retinal dysfunction and help to establish effective therapy for diabetic retinopathy.

Male breast cancer is a rare disease with an incidence between 0.5% and 2.4% of that in women. We report a case of intracystic papillary carcinoma of the breast in a 75-year-old Japanese man. The macroscopic features of the carcinoma could be accurately demonstrated by pneumocystography and ultrasonography preoperatively.

M-SHRSP rats appear to be a useful animal model for studying juvenile human malignant hypertension. Using M-SHRSP, the present study was conducted. Drugs selected for use were captopril, SQ 29,852 and hydralazine hydrochloride. The rats were also fed a 33% fish meal diet. When given separately, all three drugs were shown to be anti-hypertensive. However, using the fish meal diet combined with hydralazine was more effective than were any of the drugs given separately, while the effect of hydralazine combined with captopril or SQ 29,852 was even greater than that of the combined fish meal diet and hydralazine treatment. Some rats treated with captopril or SQ 29,852 separately were resistant to treatment, however even for these rats life spans were significantly prolonged and hypertensive vascular lesion incidence rates were drastically lowered. It was also found that even such vascular lesions as angionecrosis seemed to disappear with captopril or SQ 29,852 treatment.

We report a rare case of lung cancer associated with the right aortic arch. A 72-year-old male was admitted to our hospital for surgical treatment of squamous cell carcinoma arising from left B3. The patient had a right aortic arch with the type of mirror-image branching. He underwent a left upper lobectomy and lymph node dissection. We easily resected the lymph nodes in the left side of the upper mediastinum without rotating aortic arch because the aortic arch was positioned on the other side.

Background/Aims: Filtration coefficients (Lp) and plasma volume were estimated in order to investigate whether suppressed Lp associates with intradialytic hypotension and/or diabetic nephropathy. Methods: Twenty-one patients were evaluated. Nine patients were diabetic (DM) and 12 were nondiabetic (non-DM). Three of DM and 4 of non-DM were prone to dialysis-induced hypotension (hypo(+)) and others (hypo(–)) were not. Changes in hematocrit (Ht) were measured for 60 min after the start of ultrafiltration. Lp and plasma volume at the start of ultrafiltration (Vp0) were estimated to fit calculating values of Ht based on Schneditz’s open two compartment model to actual value. Results: There was no significant difference in the mean values of Lp/Vp0 either between hypo(+) and hypo(–) (0.87 ± 0.37 vs. 1.24 ± 0.48 ml/mm Hg·min·liter; n.s.) or between DM and non-DM (1.04 ± 0.32 vs. 1.17 ± 0.56 ml/mm Hg·min· liter; n.s.). However, the comparisons of Lp/Vp0 among the four groups (hypo(+)/DM, hypo(–)/DM, hypo(+)/non-DM and hypo(–)/non-DM) showed significant differences between hypo(+)/non-DM and hypo(–)/non-DM (1.08 ± 0.40, 1.02 ± 0.32, 0.71 ± 0.29*, 1.40 ± 0.53* ml/mm Hg·min·liter; *p < 0.05). Differences in the percentage of Vp0 to body weight (Vp0/BW) among four groups and correlation between Lp/Vp0 and Vp0/BW were not significant. Conclusion: These data indicated that reduction of Lp/Vp0 was not simply caused by decreased circulating plasma volume (Vp0/BW) and that the suppressed filtration coefficients may have substantial association with dialysis-induced hypotension in non-DM. The estimation of Lp using in-line measurement of Ht was a useful method for analyzing intradialytic hypotension.

An emergency coronary artery bypass graft (CABG) was given to a 66 year-old patient due to acute myocardial infarction (AMI). Circulating blood volume (BV) was measured to study in the perioperative period. Three coronary artery bypasses were made under cardiopulmonary bypass, being managed by ultrafiltration when the pump-oxygenator was in action and by peritoneal dialysis in the early postoperative period. Preoperative BV reduced immediately after the operation. It showed an increasing trend 4 hours after the operation, but after that BV reduced from that before the operation while water balance was kept positive. Cardiac output after the operation was higher than before. It suggested that in this patient using hemodialysis BV levels turned to be lower compared with that before the operation, as excessive water leaked out of the blood vessel, although water balance was kept positive due to improved cardiac functions after the operation.

Postoperative respiratory management following cardiac surgery is sometimes troublesome in patients with pulmonary hypertension. We retrospectively studied the relationship between the etiology of preoperative pulmonary hypertension and the postoperative decline of blood oxygenation capacity by focusing on the postoperative intravascular fluid volume.Sixteen adult patients with an atrial septal defect (preload group) and 17 patients with solitary mitral valve disease (afterload group) were studied. The mean age of the patients in the preload and afterload group was 51 and 52 years old, respectively.Preoperative pulmonary-systemic pressure ratio and pulmonary artery resistance index were significantly higher in the preload group than in the afterload group. The respiratory index (RI) and the pulmonary shunt ratio (Qs/Qt) measured immediately after the operation was larger in the afterload group than in the preload group. Postoperative RI and Qs/Qt remained high until postoperative day 3 in both groups. In the preload group RI on postoperative day 1 had a reverse correlation with the central venous pressure (CVP). Meanwhile, the RI in the afterload group on postoperative day 1 was slightly larger in patients with a high CVP and pulmonary capillary wedged pressure. Similar relationships were seen in the relations between pulmonary capillary wedged pressure and RI in the afterload group. CONCLUSIONS In conclusion, a preoperative pulmonary vascular change and a postoperative precipitous decrease of pulmonary blood flow may have caused postoperative lung oxygenation impairment in the preload group. An extended period of extracorporeal circulation associated with cardiac arrest and postoperative volume overload may have caused lung impairment in the afterload group. In aspect of postoperative management, low CVP is beneficial to the patients in the afterload group, however, hypovolemia should be avoided in patients of the preload group.

A 16-year-old boy was diagnosed as having severe aortic regurgitation and moderate aortic stenosis due to congenital aortic bicuspid valve. A chest X-ray film showed the cardiothoracic ratio (CTR) of 64% and echocardiography revealed severe dilation of the left ventricular dimension with severe wall thickness. An electrocardiogram showed multiple ventricular arrhythmias. The patient underwent the Ross operation with the reconstruction between the right ventricle and the pulmonary arteries using a three-valved conduit which was made by an expanded polytetrafuloroethylene vessel graft. Ventricular arrhythmia disappeared just after the operation and left ventricular dimensions improved with the CTR of 53% except mild regurgitation of the neo-aortic valve three months later.

Since the MEG inverse problem is ill-posed and admits many possible solutions, it is not possible to give it a single "true" answer. Therefore, we propose here to use a specific probabilistic algorithm to map the full probability distribution of the MEG sources with Markov Chain Monte Carlo methods. Using a Bayesian approach, the probability of the MEG solutions is expressed as the product of the likelihood by the prior probability. To compute the prior and constrain the MEG inverse problem resolution, MRI data are also acquired and automatically processed to determine the brain position and volume. We then use Parallel Tempering algorithm to estimate the full posterior probability and determine the likely solutions of the inverse problem. We illustrate the method with results obtained from the analysis of somatosensory data. This illustrates both the MRI processing for the prior computation, and how the knowledge of the full posterior probability distribution can be used to estimate the position of the sources, as well as their likely extension.

To investigate that blood transfusion under cardiopulmonary bypass is a possible inducer for inflammation, a retrospective study was made with 20 adult patients who underwent coronary artery bypass grafting. The subjects were divided into two groups; transfusion group (group T) including 9 patients who received blood transfusion during cardiopulmonary bypass and the control group (group C) including 11 patients who did not undergo perioperative transfusion. Respiratory index as an indicator of respiratory functions was determined before and immediately after cardiopulmonary bypass, at the end of surgery and 4 hours thereafter. Cardiac index and arterial pressure were determined as the indicator of cardiac function. Moreover, interleukin 6 and 8 (IL-6 and IL-8), inflammatory cytokines were measured and compared between the two groups. The mean amount of blood transfusion was 2.1 units per individual of group T. The minimum value of hematocrit during cardiopulmonary bypass was significantly lower in group T (15.8 +/- 1.8%) than group C (19.1 +/- 1.4%), but the difference became not significant after cardiopulmonary bypass. There were no significant differences either in aortic pressure or cardiac index between two groups. The respiratory index at the end of surgery was higher in group T but the difference was not significant. Meanwhile IL-8 level at the end of cardiopulmonary bypass was significantly higher in group T (67.9 +/- 36 pg/ml) than group C (35.1 +/- 21 pg/ml). However, there was no difference in IL-6 level between the two. These results suggested that inflammation might be aggravated by an increase of IL-8 induced by blood transfusion.

This study was designed to evaluate the effects of an Na+/H+ exchange inhibitor, SM-20550, on ischemia-reperfusion injury in the skeletal muscle. Male Sprague-Dawley rats were exposed to ischemia and reperfusion by clamping and releasing clamps both at the abdominal aorta and the bilateral femoral arteries. Rats were divided into three groups; the sham, the SM-20550 treated (SM), and the untreated control (Control) groups. In the SM and control groups, rats were exposed to 5-hr ischemia and 5-hr reperfusion. In the sham group, vessel isolation only was performed. SM-20550 (2.8 mg/kg/hr) in the SM group or vehicle in the sham and control group was continuously administered during ischemia and reperfusion periods. The wall thickness of the vessels were significantly (p0.01) decreased in the control group than any other group. The internal diameter was significantly (p0.01) higher in the control and SM group than in the sham group. The wall thickness/internal diameter ratio was significantly (p0.05) lower in the control group than in the SM group. Thus, an Na+/H+ exchange inhibitor, SM-20550, ameliorated the morphological change due to ischemia reperfusion. These findings provide a clue into the mechanism of ischemia reperfusion injury.

We present autopsy findings in a patient with a dissecting aneurysm of the vertebral artery causing subarachnoid haemorrhage. We sectioned the artery longitudinally and compared it with the angiogram, which showed the “pearl-and-string”. Histological examination showed a pseudoaneurysm covered by adventitia alone, forming the “pearl”. Internal elastic lamina and media were destroyed, and haematoma extended in the subadventitial space of the wall of the pseudoaneurysm. Media thickened by haematoma caused the “string”, narrowing the parent artery. Alcian blue staining showed that stratified internal elastic lamina in the aneurysm and the parent artery, had undergone marked mucoid degeneration, which may have been responsible for the dissection.

Screening for abdominal aortic aneurysm (AAA) has not yet been established in Japan. We therefore report the characteristics of a screened population and discuss the implications of screening using ultrasound in Japan.The subjects in our screening group were composed of 4428 participants who were 60 years of age or older. Aneurysm was detected in 16 cases, 15 males and 1 female, the detection rate being 0.4% in total and 0.9% in the males. We compare the characteristics of screened patients (n = 16) with non-screened patients operated on for abdominal aortic aneurysm (n = 166).There were no significant differences in the mean age or in the female ratio between the screened and non-screened groups (71 vs 70 y/o, 6% vs 13%, respectively). Solitary iliac aneurysms were significantly (p0.05) more frequent in the screened than in the non-screened group (19% vs 3%). The size of aneurysm in the screened group was significantly (p0.05) smaller compared with the non-screened group. Sixty-three per cent of the screened group and only 8% of the non-screened group had an aneurysm less than 40 mm in size. Aneurysm was palpable in only 31% of those of the screened group. There were no significant differences between the groups in the frequency of arteriosclerotic risk factors such as hypertension, ischaemic heart disease, diabetes mellitus, peripheral vascular disease and smoking habits. Surgical treatment was selected in 7 out of 16 screened patients. The remaining 9 patients with small-sized abdominal aortic aneurysms have been carefully followed up.Screening for abdominal aortic aneurysm using ultrasound is advisable especially for male participants and for the detection of iliac aneurysms. This screening procedure is useful for early detection because the screened aneurysm is generally small-sized and impalpable.

The purpose of this study was to investigate the influence of coronary artery bypass grafting on the degree of stenosis of the native coronary artery.retrospective data analysis.University hospital.consecutive patients undergoing coronary artery bypass grafting (n=52). Bypasses using internal thoracic artery grafts (n=26) and saphenous vein grafts (n=37) to incompletely occluded coronary arteries were studied.coronary artery bypass grafting using internal thoracic artery or saphenous vein grafts.stenosis of the native coronary artery on angiography.Three recipient coronary arteries bypassed with internal thoracic artery grafts (12%) and 14 recipient coronary arteries bypassed with saphenous vein grafts (38%) showed progression of narrowing (p=0.024). Two recipient coronary arteries bypassed with internal thoracic artery grafts (8%) and 13 recipient coronary arteries bypassed with saphenous vein grafts (35%) showed total occlusion (p=0.016). Hypertension, hyperlipidemia, diabetes mellitus, and smoking history did not correlate with progression of stenosis of the native coronary arteries. Graft flow measured during surgery in the saphenous vein grafts was not significantly different between the group that exhibited progression of the native stenosis and the group that did not.Coronary artery bypass grafting with saphenous vein grafts may result in progression of stenosis of the recipient coronary artery. This is less likely after coronary artery bypass grafting with internal thoracic artery grafts. This difference may be due to the ability of the pedicled internal thoracic artery graft to regulate flow. Thus competitive flow in the native coronary artery is minimized. This has significant clinical implications.

Seven patients with pulmonary carcinoid including three with typical carcinoid and four with atypical carcinoid were reported. There were 3 males and 4 females, with an age range of 37 to 80 years. All patients with typical carcinoid had pathological stage I. Of the patients with atypical carcinoid, one patient had stage I, two stage IIIA and one stage IIIB. All but two patients underwent lobectomy and mediastinal lymph node dissection. Bronchoplastic operation or right pneumonectomy were performed in the two patients. All but one patient underwent absolute or relative curative operation. Only one patient died on the 4th postoperative day. One patient with atypical carcinoid died of cancer recurrence 4 years after surgery. One patient died of breast cancer. One patient underwent surgery for metachronous multiple lung cancer, and he is doing well without recurrence. In conclusion, a limited operation is thought to be acceptable only in patients with typical carcinoid in a peripheral lesion without lymph node metastasis. Lobectomy and mediastinal lymph node dissection is necessary for the atypical carcinoid case.

CD40 is a member of the tumor necrosis factor receptor super-family expressed by B cells, monocytes, dendritic cells, epithelial cells and hematopoietic progenitor cells. CD40 has recently been reported to be expressed on several epidermal tumors as well. CD40 on epidermal tumor cells interacts with lymphocytes expressing ligand for CD40 (CD40L) or monoclonal antibodies against CD40 with a significant decrease in proliferation. In this study, we examined the expression of CD40 and CD40L in Bowen's disease and squamous cell carcinoma (SCC). CD40 immunoreactivity was observed in a significantly lower proportion of tumor cells from SCC than from Bowen's disease. CD40L mRNA expression was detected in Bowen's disease and SCC by reverse transcriptase polymerase chain reaction (RT-PCR). CD40-CD40L interactions in epidermal tumors may play a role in the proliferation, and the lack of CD40 in tumor cells from SCC might be involved in the mechanisms of escape from the growth inhibitory effect.

The zona pellucida is a good target antigen for contraceptive vaccines due to its strong immunogenicity and high tissue specificity. However, this contraceptive effect is inevitably associated with ovarian failure. Therefore, it is necessary to define an epitope of the zona antigen to which the antibody produced inhibits fertilization without any undesirable side effects.The DNA fragment coding for the NH2-terminal region of porcine zona pellucida proteins (ZPA) (1-198 amino acids) and human ZPA (1-206 amino acids) was prepared to produce recombinant porcine ZPA (r-ZPA), r-pZPA1-198 and r-hZPA1-206. Using Freund's complete adjuvant. antisera against these proteins were raised in rabbits.The resultant antisera to r-pZPA1-198 and r-hZPA1-206 were cross-reacted with each other on enzyme-linked immunosorbent assay and immunofluorescent staining. The antiserum to r-pZPA1-198 inhibited in vitro fertilization in pigs, but not human sperm binding to the zona pellucida, while the antiserum to r-hZPA1-206 inhibited the human sperm-binding assay.Antiserum to r-ZPA inhibited fertilization in the animal species possessing a homologous amino-acid sequence as an immunogen. The recombinant protein, r-hZPA1-206 seems to be a feasible candidate for the development of contraceptive vaccines for humans.

We report a case of 59-year-old man of descending necrotizing mediastinitis (DNM) secondary to peritonsillar abscess. A 59-year-old man with diabetes mellitus was admitted to a local hospital because of cervical swelling related to a peritonsillar abscess. Despite administration of antibiotics, swelling of the neck, dysphagia and dyspnea deteriorated. Therefore he was urgently undergone a tracheotomy and transferred to our hospital by an ambulance. The surgery consisted with neck and anterior mediastinal drainage through neck and cervical collar incision. Culture of drainage fluid showed clostridium difficile. On postoperative day 5, we started hyperbaric oxygen therapy (HBOT). After lavage and HBOT, the patient improved by degrees, and discharged on postoperative day 82. DNM is a rare but serious complication of otopharyngeal and deep neck infection that spreads down to the mediastinum through the cervical-facial planes. Its mortality rate remains high even with aggressive surgical drainage and appropriate antibiotics. Our patient was successfully treated with urgent surgical drainage, antibiotics and HBOT. HBOT might be of great value as an adjunctive management to control this fatal infection.

Controversy remains regarding the optimal nutrition after hepatic resection. We studied the feasibility and efficacy of an intravenous nutrition with high-dose fat emulsion and amino acids without glucose provision by comparing a glucose-based intravenous nutrition.Twenty-eight patients received either glucose-intravenous nutrition (glucose-IVN group: glucose, 4.2 g; amino acids, 0.8 g/Kg/day) or high-dose fat emulsion and amino acids without glucose provision (HFHA-IVN group: lipids, 2.2 g; amino acids, 1.6 g/Kg/day) for 7 days after hepatic resection (14 patients in each group). Postoperative changes in biochemical tests and plasma levels and arterial-venous concentration differences of amino acids and total ketone bodies across the leg were compared between the two.The 2 groups were comparable regarding perioperative patients' characteristics. None of the patients from either group developed any complications. Postoperative glucose levels showed normal in the HFHA-IVN group, but elevated in the glucose-IVN group. Seven of the glucose-IVN group patients required exogenous insulin administration. Lipid levels were decreased in the glucose-IVN group, but remained normal in the HFHA-IVN group. The HFHA-IVN group showed higher amino acid levels, higher amino acid release, and hyperketonemia and vigorous uptake of ketones by skeletal muscle.These results indicate that dextrose provision is not essential and the HFHA-IVN provides an alternative to glucose-based intravenous nutrition in patients developing glucose intolerance after hepatic resection.

This study was designed to investigate the effect of a newly synthesised sodium/hydrogen ion exchange inhibitor, SM-20550, on ischaemia-reperfusion induced injury in a rat hind limb model.In order to induce ischaemia of the hind limbs, the abdominal aorta just distal to the renal arteries and the bilateral femoral arteries were clamped. Nineteen rats were divided into three groups. In the sham group (n=5), the vessels were only dissected and a vehicle solution was administered. In the control group (n=7), the vessels were clamped for five hours, and a vehicle solution was administered 10 minutes prior to clamping and continued for five hours after reperfusion. In the SM group (n=7), clamping was maintained for five hours with a bolus injection of SM-20550 and continuous infusion of the solution for five hours after reperfusion. Water content of the left gastrocnemius muscle was calculated. The right gastrocnemius was fixed in 10% formalin. A transverse thin section was stained with antimyoglobin antibody. Stained cells of the right gastrocnemius were counted and the myoglobin staining index was calculated.Water content was significantly (p0.002) lower in the SM group than in the control group. The myoglobin staining index was significantly (p0.01) higher in the SM group than in the control group. There was no significant difference between the control and the SM groups in creatinine phosphokinase (CPK) and lactate dehydrogenase (LDH).Our results indicate that the sodium/hydrogen ion exchange inhibitor, SM-20550, ameliorates oedema formation and ischaemia-reperfusion induced injury of the skeletal muscle.

A 62-year-old man with synchronous multiple primary cancers involving the lung, stomach, and thyroid was admitted. Initially the patient's chest X-ray showed an abnormal shadow in the right middle-lobe indicating lung cancer. During preoperative examination, gastric cancer of the antrum and angle were detected. Excisional biopsy of the lymph node in the neck after chest surgery revealed thyroid cancer. A middle lobectomy with mediastinal lymph node dissection was performed for lung cancer and the histological diagnosis was moderately differentiated adenocarcinoma, pT4N2M0, stage IIIB. Gastric cancer was treated by endoscopic mucosal resection. Considering the relatively better prognosis of papillary thyroid cancer, we concluded that no further treatment to the thyroid lesion was necessary. In Japan, according to autopsy reports, triple primary cancers are gradually increasing. During the periods 1994 to 1996, the incidence of triple cancers was 0.81% of all autopsy cases reported.

A 74-year-old man had an previous antero-septal and inferior myocardial infarction and an abdominal aortic aneurysm (AAA) 48 mm in diameter. Coronary angiography showed obstruction of the left anterior descending artery and of the right coronary artery, and 95% stenosis of the circumflex artery. The value of an ejection fraction of the left ventricle was 33%, measured by left venticulography. CABG and replacement of the aneurysm were performed simultaneously, because of the necessity of an intra-aortic balloon pumping (IABP) due to the impaired left ventricular function. First, CABG was performed under cardiac arrest. After declamping the ascending aorta, subsequently, replacement of AAA was performed while extracorporeal circulation (ECC) assisted heart beating. Weaning from ECC was smooth, and the operation was successful without using IABP. The patient was discharged 32 days after the operation. Consequently, cardiopulmonary bypass during AAA operation could decrease heart loads when hemodynamic states change in aortic clamping or after declamping. A simultaneous operation of CABG and AAA using ECC is safe and effective for impaired left ventricular function.

Intracytoplasmic sperm injection (ICSI) has been validated as a useful treatment in severe male-factor patients who could not achieve fertilization and live births by conventional in vitro fertilization treatment. To examine the impact of male factors on ICSI outcome, clinical laboratory data were retrospectively analyzed.One hundred two cycles of ICSI treatment indicated by severe male-factor infertility were entered into this study. Sperm parameters including sperm motility, sperm concentration, and sperm motility index assessed by the Sperm Quality Analyzer were evaluated.Five hundred seventy-six metaphase II oocytes retrieved were manipulated. The normal fertilization (2 PN) rate per oocyte was 64.9 +/- 26.0% (mean +/- SD). Of the 99 transfers, 31 clinical pregnancies were obtained, yielding an average pregnancy rate of 31.3% per transfer. The mean sperm motility, sperm concentration, and sperm motility index were 20.3 +/- 16.1% (range, 0 to 50%), 18.2 +/- 25.1 x 10(6)/ml (range,1 to 150 x 10(6)/ml), and 31.2 +/- 45.0 (range, 0 to 220), respectively. Sperm concentration did not have a significant impact on fertilization rate by ICSI. In four cases, ICSI was performed using totally immotile sperm and the fertilization rate was 43.5%, which was significantly lower than that of some of the other sperm motility groups, and no pregnancy could be achieved. In 14 cases in which the sperm motility index assessed by the Sperm Quality Analyzer was 0, the fertilization rate (50.0%) was significantly lower than in most of the other sperm motility index groups.These findings suggest that in severe male-factor cases with totally immotile sperm or a sperm motility index of 0, the selection of good-quality sperm should be verified before injection.

To investigate the relationship between polyol pathway hyperactivity and altered carnitine metabolism in the pathogenesis of diabetic neuropathy, the effects of an aldose reductase inhibitor, [5-(3-thienyl) tetrazol-1-yl]acetic acid (TAT), and a carnitine analog, acetyl-L-carnitine (ALC), on neural functions and biochemistry and hemodynamic factors were compared in streptozotocin-diabetic rats. Significantly delayed motor nerve conduction velocity, decreased R-R interval variation, reduced sciatic nerve blood flow and decreased erythrocyte 2, 3-diphosphoglycerate concentrations in diabetic rats were all ameliorated by treatment with TAT (administered with rat chow containing 0.05% TAT, approximately 50 mg/kg/day) or ALC (by gavage, 300 mg/kg/day) for 4 weeks. Platelet hyperaggregation activity in diabetic rats was diminished by TAT but not by ALC. TAT decreased sorbitol accumulation and prevented not only myo-inositol depletion but also free-carnitine deficiency in diabetic nerves. On the other hand, ALC also increased the myo-inositol as well as the free-carnitine content without affecting the sorbitol content. These observations suggest that there is a close relationship between increased polyol pathway activity and carnitine deficiency in the development of diabetic neuropathy and that an aldose reductase inhibitor, TAT, and a carnitine analog, ALC, have therapeutic potential for the treatment of diabetic neuropathy.

We report 11 subjects, consisting of sibling cases from 5 unrelated families, with paroxysmal kinesigenic choreoathetosis (PKC). In these subjects the PKC attacks started between the ages of 5 and 17 years and were well controlled with anti-epileptic drugs or subsided spontaneously. In 8 cases from 4 families with PKC, infantile convulsions occurred between the ages of 3 and 8 months and an excellent prognosis was obtained in all but one male. Four of the 8 subjects had complex partial seizures which were characterized by staring, eye deviation, apnea, or loss of consciousness. The nature of these convulsions shared some of the clinical features of benign infantile epilepsies, which have been recently advocated as a new category of epilepsy. There were no differences in the clinical between the cases with and without infantile convulsions.

The interactions of neurotrophins with the Trk family of tyrosine kinase receptors result in growth and maturational changes in neuronal cells. Although the histogenesis of brain tumors composed of mature neuronal cells is still not completely understood, neurotrophins and Trk receptors may be involved in the evolution, maturation, and persistence of these tumors. The clinical and anatomic pathological features of 8 primary neuronal cell tumors (ganglioglioma: 3 cases, cerebral neurocytoma: 3 cases, intraventricular neurocytoma: 2 cases) occurring in the central nervous system (CNS) have been examined. In addition to routine histological examinations, immunohistochemistry was used to evaluate the expression of neurotrophin receptors (TrkA, TrkB) and of neuronal differentiation markers such as neuron-specific enolase, neurofilament, synaptophysin, and chromogranin A. While neither TrkA nor TrkB expression was demonstrated in 2 intraventricular neurocytomas, the remaining 6 tumors did show positive immunohistochemical staining for TrkA and/or TrkB proteins; for TrkA protein, ganglionic cells showed membraneous or cytoplasmic staining, while small non-ganglionic neuronal cells with scant cytoplasm occasionally showed positive cytoplasmic immunoreactivity. For TrkB protein, small non-ganglionic neuronal cells showed a more intense immunoreaction than ganglionic cells. Gangliogliomas with high TrkA and TrkB expression showed higher levels of neuronal differentiation, as demonstrated by the neuron-specific enolase and neurofilament immunoreactivity. The existence of neurotrophin receptors in the tumor cells thus suggests that neurotrophic influence are involved in the evolution and subsequent cellular maturation in neuronal cell tumors of the CNS.

Abstract A possible pathogenic polymorphism in the gene for the G subunit of the glycogen-associated regulatory form of protein phosphatase 1 (PP1 G subunit), causing an Asp-to-Tyr substitution at codon 905 (Asp905Tyr), has been reported to be associated with insulin resistance and hypersecretion of insulin in the white population. Since marked heterogeneity has been reported in the association of mutations of candidate genes with essential hypertension between Japanese and other ethnic groups, we investigated the association of Asp905Tyr with essential hypertension in Japanese subjects. The frequency of the Tyr allele in Japanese control subjects (0.70) was much higher than that in the Danish population (0.10, P −8 ), indicating that the Tyr allele, previously reported as a rare variant in white subjects, is a common allele in our population. The genotype distribution in Japanese hypertensive patients (n=109; Asp/Asp=0.09, Asp/Tyr=0.39, Tyr/Tyr=0.52) was not significantly different (χ 2 =0.7, df =2, P >.6) from that in normotensive control subjects (n=148; Asp/Asp=0.12, Asp/Tyr=0.36, Tyr/Tyr=0.52). Among subjects with different PP1 G subunit genotypes, there was no difference in blood pressure, serum cholesterol, plasma glucose and insulin levels, and glucose disposal rate estimated by the euglycemic hyperinsulinemic clamp test. These data indicate that the Asp905Tyr polymorphism of the PP1 G subunit is not associated with essential hypertension, nor with insulin resistance and/or hyperinsulinemia in Japanese patients with essential hypertension, suggesting that the polymorphism plays little if any role in susceptibility to insulin resistance or hypertension.

A 52-year-old man with reduced left ventricular function (ejection fraction 0.27) due to myocardial infarction, underwent coronary artery bypass grafting (CABG; LITA-LAD, free RITA-4PD) for angina pectoris. He had suffered from recurrent sustained ventricular tachycardia (VT) since 5 hours after CABG. This arrhythmia was resistant to various antiarrhythmic agents such as Lidocaine, Mexiletine, Disopyramide, Procainamide and Propafenone. He required mechanical circulatory supports (intra-aortic balloon pumping and percutaneous cardiopulmonary support) for the maintenance of hemodynamics during repeated VT that required cardioversions of a total of 441 times during 18 days. Following the administration of Amiodarone, the VT was successfully suppressed. However, he had repeated episodes of VT on exercise, thus, he underwent insertion of the implantable cardioverter-defibrillator at the 98 post-operative day, and he was successfully discharged at the 134 post-operative day after CABG. The instrument was verified to be normal in function after the VT induction test.

A putative pathogenic mutation in the beta3-adrenergic receptor gene (Trp64Arg) has been reported to be associated with higher diastolic blood pressure as well as clinical features of the insulin resistance syndrome and an earlier onset of non-insulin-dependent diabetes mellitus (NIDDM) in Pima Indians and Finns. Because essential hypertension is reported to be associated with insulin resistance, we studied the mutation in Japanese patients with essential hypertension to clarify associations of this mutation with hypertension, insulin resistance, and basal adrenergic state in hypertensive subjects. The allele frequency of the mutation (Arg) in patients with essential hypertension was similar to that in control subjects (35 of 202 alleles [17.3%] v 27 of 146 [18.5%], respectively, P.7). Insulin sensitivity measured by hyperinsulinemic euglycemic glucose clamp and plasma norepinephrine and epinephrine levels were also similar in hypertensive subjects with and without the mutation. These data suggest that Trp64Arg mutation in the beta3-adrenergic receptor gene does not play a major role in susceptibility to essential hypertension or in insulin resistance and basal adrenergic state in hypertension.

A case of primary transitional cell carcinoma of the prostatic urethra is reported. A 60 year-old male, with a chief complaint of difficult urination consulted our clinic on March, 1994. Urethrography and endoscopic examination revealed the solitary tumor of the prostatic urethra and transurethral resection was performed. Histologically, the tumor was Grade 2 transitional cell carcinoma without submucosal invasion. The post-operative course was uneventful and no recurrence has been seen. To our knowledge, including our case, 10 cases of primary transitional cell carcinoma of the prostatic urethra have been reported in the Japanese literature.

To evaluate the long-term results of surgical patients with coronary artery bypass grafting (CABG), we comparatively analyzed the 10-year survival and cardiac event-free rates between 713 patients group with at least one internal thoracic artery to the left anterior descending artery (LAD), (ITA-CABG) and 241 patients group revascularized with vein grafts alone (SVG-CABG). ITA-CABG patients had more progressed diseases with a higher incidence of risk factors than SVG-CABG patients: number of vessel diseased 2.5 +/- 0.7 vs 2.3 +/- 0.7, LMTD 20.2% vs 14.1%, diabetes mellitus 37.3% vs 27.0% and hyperlipidemia 38.0% vs 30.7%. The 10-year cumulative LAD graft patency and severe disease-free rate was 90.3 and 67.0% for ITAs and vein grafts in this series. The 10-year overall actuarial survival, cardiac death-free and cardiac event-free rates for ITA and SVG groups were 88.8 vs 79.5%, 97.4 vs 92.6% and 84.1 vs 73.1%, all with a statistical significance (generalized Wilcoxon or logrank method). For the patients with reduced ventricular systolic function (EFor = 0.4), ITA-CABG offered a significantly better 10-year cardiac death free rate. Also, for the diabetic patients, ITA-LAD offered a significantly better 10-year cardiac event-free rate. In conclusion, the use of ITA graft can reduce postoperative cardiac events and enhance the long-term survival in Japanese patients. The ITA should be utilized at least for LAD in all CABG patients whenever feasible.

Parasagittal meningiomas often invade the superior sagittal sinus (SSS), but rarely grow inside the SSS as a primary tumor. The authors report a case of parasagittal meningioma growing mainly inside the SSS and presenting papilledema. The SSS is invisible behind the tumor end on the right carotid angiogram but still patent on the left carotid angiogram. The superficial cortical vein on the opposite side works as a collateral pathway. The tumor may have originated from the right wall of the SSS, grew inside the sinus and covered the entrances of the right ascending cerebral veins. V-P shunt was performed after removal of the mass outside the sinus for resolving headache and visual symptoms.

This case report describes a case of carcinomatous peritonitis which showed a good response to the combination therapy of 5-FU, leucovorin and cisplatin. A 55-year-old man was admitted with complaints of meteorism due to ascites fluid. Ascitic examination showed a high concentration of CEA and CA19-9, and adenocarcinoma cells. Laboratory and radiological examination did not reveal the origin of the carcinoma, and laparotomy suggested an appendiceal origin. The combination chemotherapy with 5-FU, leucovorin and cisplatin was conducted for a total of 11 courses for 2 years with good QOL until his death. This therapy was suggested to be useful for inoperable carcinomatous peritonitis.

The effects of propionyl-L-carnitine (PCAL) on caudal motor nerve conduction velocity, the coefficient of variation of the R-R interval on the electrocardiogram, and sciatic nerve blood flow were compared with those of [5-(3-thienyl)tetrazol-1-yl] acetic acid monohydrate, an aldose reductase inhibitor, in rats with streptozotocin-induced diabetes. Diabetic control rats showed significantly delayed nerve conduction (P.05), decreased R-R variability (P.05) and reduced sciatic nerve blood flow (P.05). Oral administration of PCAL (0.5 g/kg/day) and [5-(3-thienyl)tetrazol-1-yl] acetic acid monohydrate (0.05% in the diet: 60 mg/kg/day) for 8 weeks significantly improved both nerve conduction (P.05) and R-R variability (P.05) in diabetic rats, along with the normalization of sciatic nerve blood flow. PCAL treatment increased the nerve tissue levels of carnitine and myo-inositol and reduced the serum triglyceride level in diabetic rats. Our results suggests that PCAL could have therapeutic potential for the treatment of diabetic neuropathy.

To investigate the relationship between metabolic and vascular factors, especially polyol pathway and platelet aggregation, in the pathogenesis of diabetic neuropathy, the effects of a novel potent aldose reductase inhibitor, TAT ((5-(3-thienyl) tetrazol-1-yl) acetic acid monohydrate) on adenosine diphosphate-induced platelet aggregation, polyol contents in platelets, motor nerve conduction velocity (MNCV), and sciatic nerve blood flow (SNBF) were examined in streptozotocin-induced diabetic rats. Diabetic rats demonstrated hyperaggregation in response to adenosine diphosphate, accompanied by sorbitol and fructose accumulation and myoinositol depletion in platelets. Treatment with TAT improved these abnormalities in diabetic rats. A delayed MNCV and a reduced SNBF in diabetic rats were normalized by the administration of TAT. These observations suggest that increased polyol pathway activity plays an important role in platelet aggregation in the development of diabetic neuropathy and that aldose reductase inhibitor is useful for the treatment of diabetic neuropathy from the viewpoint not only of metabolic factors but also of vascular factors.

A 42-year-old man with giant bronchogenic cyst occupied from the middle and posterior mediastinum was treated surgically. The tumor was stoutly adhered to anterior aspect of the esophagus and membranous portion of the right bronchus. In dissecting the tumor, muscular coat of the esophagus was partly severed, which was repaired interruptedly with absorbable suture material. However, esophageal perforation was detected by barium swallow on the 3rd postoperative day. Conservative therapy included intrathoracic drainage and antibiotics was successfully undertaken. It is imperative to dissect the tumor very carefully if the giant bronchogenic cyst adheres to the esophagus or other surrounding organs.