Coronary Revascularization of Giant Aneurysms in Children With Kawasaki Disease: A Report of Two Cases.

In recent years, >100 cases of coronary artery stenotic lesions due to Kawasaki disease were treated with coronary artery bypass grafting (CABG). Surgical indications include stenosis of >75%, myocardial infarction history, electrocardiographic changes, and ischemia, as detected by myocardial scintigraphy and electrocardiography, due to drugs or exercise. Some centers have reported good patency rates, even in infants. The advantages of CABG in younger patients are minimal loss of left ventricular function, early elimination of post-operative ischemia risk, and improved quality of life. However, the disadvantage of performing CABG in younger patients is the small coronary artery diameter and the thin vessel wall, which can lead to post-operative occlusion, especially when performed by inexperienced surgeons. The optimal timing of CABG varies by institution and case, which depends on the presence or absence of complications, such as left ventricular dysfunction or valve regurgitation, and surgeon's experience. Importantly, unlike adult surgery, childhood CABG needs to be kept open for the very longest possible period of time to determine the optimal conditions for surgery. We report two pediatric cases of giant coronary artery aneurysms diagnosed in infancy. During school age, the patients had a mild decline of left ventricular ejection function. In one case, there were no clinical symptoms because of the development of collateral vessels and limitation of exercise. Both patients underwent surgery with good results. The gastric gastroepiploic artery could be anastomosed owing to the development of collateral blood vessels, although it was obstructed. At 1 year after surgery, both patients had a good post-operative course without complications of anastomotic stenosis or myocardial damage due to aneurysm resection. If conditions are favorable, bypass surgery can be postponed to several years until the coronary arteries are sufficiently large to warrant a delay in coronary artery stenosis in cases of infantile Kawasaki disease.

Rotationally Resolved Excitation Spectra Measured by Slow Electron Detachment from Si2.

Laser-induced delayed electron detachment from Si2- stored in an electrostatic ion storage ring was observed on the 10 microsecond time scale. The excitation spectra for photon energies near threshold show well-resolved multipeak structures, which are attributed to rovibronic transitions to the electronic excited state. This structure appears only in the signal measured with the delay. The occurrence of delayed detachment on such a long time scale is unusual for diatomic molecules, suggesting that both the autodetachment and fluorescence are slow.

"Weariness" and "unpleasantness" reduce adherence to branched-chain amino acid granules among Japanese patients with liver cirrhosis: results of a single-center cross-sectional survey.

AIM: Branched-chain amino acids (BCAA) are valuable in the treatment of liver cirrhosis because they increase serum albumin levels. Poor adherence to BCAA may adversely affect prognosis, but little is known about factors predicting adherence. We undertook a survey of patients prescribed BCAA for the treatment of cirrhosis. METHODS: Pharmacists carried out face-to-face interviews with patients (or their representatives) prescribed any of nine BCAA formulations. Question categories included patient characteristics, prescription of BCAA granules, and perceptions of BCAA administration, including adherence and possible factors that might impact adherence. "Poor adherence" was defined as "not taking the medication appropriately" or "forgetting to take the medication". RESULTS: Overall, 253 patients (or representatives) completed the survey, of whom 135 were men, 114 were women, and 148 were ≥70 years old. Most patients (163) were prescribed BCAA for ≥2 years and were using three packs per day. Thirty-two patients did not take their medication appropriately and 69 sometimes forgot to administer it. Weariness of taking the medication (P < 0.001) and the perceived unpleasantness (P = 0.023) of the medication in terms of its taste and volume were significantly associated with poor adherence. The patients reported that the most influential educators were general practitioners, followed by certified hepatologists, then pharmacists. CONCLUSION: Most patients had good adherence to BCAA in clinical practice. Poor adherence was associated with weariness with taking medication, and the unpleasantness of the medication itself. Patient education from general practitioners and hepatologists combined with adherence counseling from pharmacists may help improve adherence.

Detection of Recurrent Fluorescence Photons.

We have detected visible photons emitted from the thermally populated electronic excited state, namely recurrent fluorescence (RF), of C_{6}^{-} stored in an electrostatic ion storage ring. Clear evidence is provided to distinguish RF from normal fluorescence, based on the temporal profile of detected photons synchronized with the revolution of C_{6}^{-} in the ring, for which the time scale is far longer than the lifetime of the intact photoexcited state. The relaxation (cooling) process via RF is likely to be commonplace for isolated molecular systems and crucial to the stabilization of molecules in interstellar environments.

Inverse internal conversion in C4(-) below the electron detachment threshold.

Inverse internal conversion followed by recurrent fluorescence was observed as a fast decay (10 µs range) in the time profile of neutral yields from photo-excited C4(-) molecular ions. We also elucidated the contribution of such electronic radiative cooling to the C4(-) ions with internal energy far below the detachment threshold by an alternative novel approach, observing the laser wavelength and storage time dependence (ms range) of the total yield of the photo-induced neutrals.

[Unresectable Gallbladder Cancer Successfully Treated with Gemcitabine Chemotherapy].

We report a case of unresectable gallbladder cancer successfully treated with gemcitabine (GEM). A 77-year-old man was admitted to our hospital in April 2010 with jaundice. He was diagnosed as having gallbladder cancer that had invaded the liver and hepatic artery, along with lymph node metastasis. The tumor was considered unresectable, and he received chemotherapy with GEM. Each course of treatment consisted of 1,000 mg/m2 GEM administered once a week for 3 weeks followed by a week of no treatment. After the 3rd course, computed tomography revealed reduction in liver invasion and disappearance of lymph node metastasis. After the 18th course, the tumor had completely disappeared, and the patient achieved a complete response. GEM monotherapy was continued for 5 years, even though the tumor had disappeared. The patient is alive and has been disease-free for more than 5 years.

[Palliative Care for Rectal Cancer Complicated with Gastric Cancer].

Medical advancements have led to an increase in the number of elderly people. However, standard treatments may sometimes be difficult to use in elderly people. Here, we report the case of an elderly patient with rectal and gastric cancer who refused radical surgery. The patient was an 83-year-old man who had type-2 diabetes, hypertension, hyperuricemia, mitral valve regurgitation, and mild dementia. Furthermore, he was blind in both eyes owing to glaucoma. He first visited our hospital in 2005. In 2010, he was diagnosed with anemia, but he refused a thorough examination; however, he did consent to take iron supplements. In July 2011, he consulted our hospital for symptoms of frequent diarrhea, and agreed to an examination. After colonoscopy, he was diagnosed with rectal cancer that was becoming obstructive. There were no metastases to other organs, but he was also diagnosed with gastric cancer. As he and his family refused radical surgery, a stoma was constructed. After the operation, he received palliative care but died in September 2013.

Outcome of univentricular repair in patients with Down syndrome.

OBJECTIVE: Total cavopulmonary connection (TCPC) is rarely performed for a functional single ventricle in children with Down syndrome; therefore, the postsurgical outcomes are not well known. We evaluated mortality and related factors after TCPC in children with Down syndrome. METHODS: Between January 2004 and March 2010 we identified 8 patients with Down syndrome among 235 patients who had undergone TCPC. The preoperative clinical course, preoperative data, and postoperative clinical course were evaluated. In addition, clinical parameters and postoperative clinical course were compared between children with Down syndrome (n = 8) and a non-Down syndrome group (n = 227). RESULTS: The median age at the time of TCPC was 4.1 years (range, 3.4-5.5 years), and the preoperative mean pulmonary artery pressure was 13.9 ± 1.81 mm Hg. We observed respiratory complications in 2 patients, surgical site infection in 3 patients, and chylothorax in 2 patients. No significant difference was observed in preoperative data and mortality rate (1 of 8 patients [12.5%] in the Down syndrome group; 5 of 227 patients [2.2%] in the non-Down syndrome group) between the groups of children with and without Down syndrome. However, when the postoperative clinical course was examined, the durations of intensive care unit stay (P = .009) and hospital stay (P = .007) were found to be significantly prolonged in the Down syndrome group. CONCLUSIONS: Patients with Down syndrome tend to show prolonged recovery after TCPC. However, as opposed to previous reports, the mortality rate of patients undergoing TCPC is lower with no significant difference from that of children without Down syndrome.

Analysis of arterial function in adults with a history of Kawasaki disease.

BACKGROUND AND PURPOSE: It remains controversial whether Kawasaki disease (KD) is a risk factor for the early onset of atherosclerosis. The purpose of the present study was to assess endothelial function and arterial stiffness as markers of the early onset of atherosclerosis in adult patients with a history of KD. METHODS AND SUBJECTS: We compared 14 adult patients with a history of KD with 41 healthy controls. To assess arterial endothelial function, we measured the reactive hyperemia-peripheral arterial tonometry (RH-PAT) index and augmentation index adjusted to 75 bpm (AIx@75) using the Endo-PAT 2000 (Itamar Medical, Caesarea, Israel). In addition, we analyzed medical history, blood pressure, lifestyle habits, and atherosclerosis-related serum biochemical markers [asymmetric dimethylarginine, adiponectin, lipoprotein (a), cholesterols, atherogenic index of plasma]. RESULTS: There was no difference between the KD and control groups with regard to the RH-PAT index values (2.10 ± 0.43 and 1.84 ± 0.49, respectively; p=0.19). However, in the KD group, the RH-PAT index values were negatively correlated with the febrile period in the acute phase of disease (r(2)=0.458, p=0.048). In addition, the AIx@75 values were higher in KD patients compared to healthy controls (-7.69 ± 11.86% and -15.87 ± 8.72%, respectively; p=0.01). No significant differences existed between the KD and control groups with regard to the serum biomarkers of atherosclerosis. CONCLUSIONS: We speculate that endothelial dysfunction in former KD patients is affected by the febrile period of the acute phase, and antiplatelet drugs may improve endothelial function. The increased arterial stiffness of patients caused by post-inflammatory fibrotic changes in the arterial wall indicates that adults with a history of KD have an increased risk of developing atherosclerosis.

Improvement in non-tachycardia-induced cardiac failure after radiofrequency catheter ablation in a child with a right-sided accessory pathway.

A 6-year-old boy was referred for an evaluation of intolerance to physical activity at his elementary school. The patient had no episodes of palpitations. He was diagnosed as Wolff-Parkinson-White syndrome with a right-sided accessory pathway (AP) and dilated cardiomyopathy (DCM). Ventricular dyskinesis was detected mostly in the ventricular septum. Because the asynchronous septal motion caused by pre-excitation through a right-sided AP might deteriorate his cardiac function, he underwent an AP ablation, after which the asynchronous ventricular wall motion disappeared and the wall thickness improved. We suggest that an AP ablation may be the treatment of first priority in patients who have DCM-like dyskinesis even without sustained tachyarrhythmias.

Non-invasive assessment of liver fibrosis in patients after the Fontan operation.

BACKGROUND: This study analyzed the change in liver fibrosis markers after the Fontan operation and investigated their clinical usefulness as an index of congestive liver fibrosis. METHODS: The study enrolled 24 patients who underwent the Fontan operation between January 1994 and December 2008. We subdivided the postoperative period into four intervals and then compared the hepatological markers during each. Eighteen patients underwent postoperative cardiac catheterization and the correlation between hepatological markers and the inferior vena cava (IVC) pressure was analyzed. RESULTS: The mean age of the patients was 138.6 months and the mean interval between the Fontan operation and the examination was 97.8 months. Type IV collagen was extremely high in every interval (I, 286 ± 93; II, 265 ± 93; III, 305 ± 143; IV, 206 ± 70), while none of the laboratory variables changed significantly in each interval. A significant positive correlation was observed between type IV collagen and the IVC pressure, but no significant correlation with any other hepatological marker was detected. CONCLUSIONS: No specific parameter that reflects the progress in liver fibrosis was identified in this study. The possibility exists that type IV collagen reflects the degree of hepatic congestion.

Left ventricular torsion and strain in patients with repaired tetralogy of Fallot assessed by speckle tracking imaging.

BACKGROUND: In patients with tetralogy of Fallot (TOF), left ventricular (LV) dysfunction is an important factor associated with poor clinical outcome. OBJECTIVE: The purpose of this study was to investigate the torsion and multidirectional strain of the LV in patients with TOF. METHODS: Echocardiographic images were prospectively acquired in 29 patients who underwent TOF repair (age range, 5-25 years) and in 29 normal controls. Torsion and circumferential and longitudinal strain of the LV were assessed using speckle tracking imaging. RESULTS: The torsion in patients was smaller compared to that in the controls due to small apical rotation and/or inverse basal rotation (P < 0.01). Torsion and untwisting rates decreased with increasing age (R = 0.37, P < 0.05). Basal circumferential strain and strain rate (SR) at systole and diastole decreased with age (R = 0.58; R = 0.57; R = 0.57, all P < 0.001) and were smaller in patients compared to those in the controls (all P < 0.01). Septal longitudinal strain and SR at systole and diastole decreased with age (R = 0.52; R = 0.62; R = 0.71, all P < 0.001) and were smaller than those of the controls (P < 0.01), although lateral longitudinal strain and SR were relatively maintained. CONCLUSION: Abnormal torsion and strain pattern of the LV were observed in patients without symptoms of cardiac failure. Assessment of torsion and strain is a very sensitive tool to detect the early deterioration of LV function in patients with TOF.

Fatal hemorrhage induced by subtilase cytotoxin from Shiga-toxigenic Escherichia coli.

Subtilase cytotoxin (SubAB) is an AB(5) type toxin produced by a subset of Shiga-toxigenic Escherichia coli. The A subunit is a subtilase-like serine protease and cleaves an endoplasmic reticulum chaperone BiP. The B subunit binds to a receptor on the cell surface. Although SubAB is lethal for mice, the cause of death is not clear. In this study, we demonstrate in mice that SubAB induced small bowel hemorrhage and a coagulopathy characterized by thrombocytopenia, prolonged prothrombin time and activated partial thromboplastin time. SubAB also induced inflammatory changes in the small intestine as detected by ¹8F-fluoro-2-deoxy-d-glucose positron emission tomography imaging and histochemical analysis. Using RT-PCR and ELISA, SubAB was shown to increase interleukin-6 in a time-dependent manner. Thus, our results indicate that death in SubAB-treated mice may be associated with severe inflammatory response and hemorrhage of the small intestine, accompanied by coagulopathy and IL6 production.

A causal relationship between right paraduodenal hernia and superior mesenteric artery syndrome: a case report.

INTRODUCTION: Cases of right paraduodenal hernia and superior mesenteric artery syndrome have been reported separately, but their occurrence in combination has not been reported. CASE PRESENTATION: A 46-year-old Japanese man who had never undergone laparotomy was admitted to our hospital due to an acute abdomen. An enhanced multidetector-row computed tomography scan of our patient showed a cluster of small intestines with ischemic change in his right lateral abdominal cavity. Emergency surgery was subsequently performed, and strangulation of the distal jejunum along with incidental right paraduodenal hernia was found. His necrotic ileum was resected, and the jejunum encapsulated by the sac was repaired manually without reduction.Three days after the operation, however, our patient developed vomiting. An upper gastrointestinal series revealed a straight line cut-off sign on the third portion of his duodenum. A second enhanced multidetector-row computed tomography scan showed that he had a lower aortomesenteric angle and a shorter aortomesenteric distance compared to his condition before his right paraduodenal hernia was surgically repaired. We strongly suspected that the right paraduodenal hernia repair may have induced superior mesenteric artery syndrome. On the 21st post-operative day, duodenojejunostomy was performed because conservative management had failed. CONCLUSIONS: In this case, enhanced multidetector-row computed tomography, which permits reconstructed multiplanar imaging, helped us to visually identify these diseases easily. It is important to recognize that surgical repair of a right paraduodenal hernia may cause superior mesenteric artery syndrome.

Drug-eluting stents ameliorate pulmonary vein stenotic changes in pigs in vivo.

The outcome of stent implantation for pulmonary vein stenosis (PVS) in children remains poor. Several reports describe placing drug-eluting stents to treat PVS, but their effectiveness remains unknown. In this study, three bare-metal stents (BMSs) and three sirolimus-eluting stents (SESs) were implanted in 1-month-old pigs. The pigs were killed 8 weeks later to compare in-stent stenosis rates. The extent of neointimal thickness, as measured by injury score, was significantly less in the SES group than in the BMS group (injury score 1: BMS 0.351 + or - 0.033 vs SES 0.226 + or - 0.031 mm; P < 0.01; injury score 2: BMS 1.232 + or - 0.244 vs SES 0.609 + or - 0.208 mm; P < 0.01). The pathologic findings showed confluence of inflammatory cells around the stent wires in BMS-treated areas and granuloma formation. Granuloma formation was not seen with SES. The degree of in-stent stenosis was significantly reduced in the SES group, suggesting that the use of drug-eluting stents is an effective treatment for PVS. Because of the small sample size and the considerable variation in injury scores and balloon-to-vein ratios, future studies with larger samples are necessary.

The effect of pretreatment with different doses of GnRH to synchronize follicular wave on superstimulation of follicular growth in dairy cattle.

The objective of this study was to evaluate the efficiency of gonadotropin releasing hormone (GnRH) and GnRH doses in synchronizing follicular wave emergence as a pretreatment for superovulation in cattle. Fourteen Holstein-Friesian cows 6 days from estrus were randomly assigned to receive 100 microg (n=4), 50 microg (n=5), or 25 microg (n=5) of GnRH. Superovulation was induced with injections of porcine FSH (pFSH) twice daily, decreasing the dose (total 42 AU) over 5 days beginning 2.5 days after receiving GnRH. On the 7th and 8th injections of pFSH, 750 microg of PGF(2alpha) was also given. With the exception of one cow that was given 50 microg of GnRH, ovulation was induced in all cows from the three groups and the new follicular wave emergence was observed. The total number of follicles for the 25 microg GnRH group was less than that observed for the 100 microg GnRH group (P<0.05), although there were no differences between the 100 microg, 50 microg and 25 microg GnRH groups with respect to the number of preovulatory follicles (>or=10 mm) and CL. The numbers of normal embryos were greater for the 25 microg GnRH group than the 100 or 50 microg GnRH groups (P<0.01); however, the numbers of ova/embryos did not differ significantly between the three groups. These results suggest that 25 microg of GnRH was sufficient to induce ovulation and follicular wave emergence. On day 6 of the estrous cycle, a reduction of the dose of GnRH to synchronize follicular wave emergence as a pretreatment for superstimulation promotes transferable embryos.