Background Kyphosis is a risk factor for the presence of hiatal hernia, which is a strong predictor of Barrett's esophagus. However, the association between kyphosis and Barrett's esophagus has not yet been clarified. To investigate this relationship, the Cobb angle, a marker of kyphosis, was measured in patients with and without Barrett's esophagus.
Methods From January 2006 to December 2010, 26 patients with long-segment Barrett's esophagus (LSBE) were retrospectively enrolled. As the comparative groups, 100 consecutive patients with short-segment Barrett's esophagus (SSBE) and 100 consecutive control patients without Barrett's esophagus were also enrolled in this study. Cobb angles were measured on lateral chest radiographs, and kyphosis was defined as a Cobb angle of greater than 50°. Kyphosis, along with other patient characteristics, were evaluated as possible predictors for SSBE and LSBE.
Results The mean Cobb angles in the non-BE, SSBE, and LSBE groups were 31.6° (95% CIs, 29.3°-33.9°), 34.8° (32.1°-37.4°) and 49.4° (44.9°-53.9°), respectively. Statistically significant differences were found between the LSBE and the other 2 groups (p<0.001). The mean Cobb angles were 33.3°±12.4° and 37.0°±14.1°, respectively in patients without and with hiatal hernia (p=0.039). Predictors for Barrett's esophagus of any length were erosive esophagitis and hiatal hernia. Kyphosis had the highest odds ratio for the presence of LSBE (OR, 1.50; 95% CI, 1.05-1.94; p=0.033). Other predictors were hiatal hernia and the absence of Helicobacter pylori infection.
Conclusion Kyphosis is a risk factor for the presence of LSBE in Japanese patients.

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Objective Platelet counts before starting the treatment affect the discontinuation and dose reduction of peginterferon in chronic hepatitis C. Thrombocytopenia leads to failure to achieve sustained virological response. This study was undertaken to evaluate the efficacy of partial splenic embolization (PSE) prior to starting peginterferon therapy combined with ribavirin in chronic hepatitis C patients showing thrombocytopenia.
Patients and Methods We compared the clinical features of 11 patients receiving PSE (PSE group) prior to starting the combined therapy with those of 13 patients not receiving PSE (non-PSE group). All of the patients showed platelet counts ≤12×10⁴ /mm³ and serum hepatitis C virus-RNA levels ≥100 KIU/mL at baseline. The end-point of PSE was a volume of splenic infarction over 75%. Peginterferon alpha-2b at a dose of 1.2 μg/kg was administered by subcutaneous injection once a week. The dose of ribavirin was weight adjusted.
Results PSE was successfully performed without serious adverse events. The period from PSE to starting the combined therapy was 14 (6-27) days. After PSE, platelet counts were significantly increased. In PSE group, platelet counts during the combined therapy were maintained above those at baseline. In non-PSE group, platelet counts at the 2nd week after the start of the combined therapy significantly decreased to less than those at baseline. Overall, 80% adherence to expected peginterferon dose was not achieved in 5 patients (45%) of PSE group and in 11 (85%) of non-PSE group (p=0.043).
Conclusion Increased platelet counts after PSE facilitates the adherence to peginterferon therapy in chronic hepatitis C patients with thrombocytopenia.

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Introduction Rice cake is a traditional but very popular food in Asia including Japan and has never been known as a cause of ileus. Rice cake is now becoming widespread in the United States and European countries along with other Japanese foods. We may encounter rice cake ileus all over the world. This study was aimed at characterizing the rice cake ileus.
Methods We consecutively encountered 14 patients with rice cake ileus from April 2003 to October 2010 in our hospital. All of the characteristics of the cases were reviewed and analyzed.
Results All patients had ingested rice cake by swallowing without chewing. It has most frequently occurred in January (57.1%). The main symptoms were abdominal colicky pain (100%) and nausea (85.7%) and physical findings included abdominal tenderness (100%) and muscular defense (28.6%). All patients improved by conservative therapy including fluid supply (100%), naso-gastric tube (28.6%) and long tube (28.6%) insertion. No patient needed emergency open surgery.
Conclusion Rice cake ileus which is caused by swallowing the rice cake without chewing, frequently occurs in January, has previous history of abdominal surgery and shows high density intestinal contents on CT and only needs conservative therapy. Globalism in food culture may provide a new disease entity. Therefore, we should be aware of this type of ileus and be prepared to manage it appropriately.

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Background The blood pressure response to the rapid removal of fluid during hemodialysis is complex and the pathophysiological mechanisms underlying intradialytic hypotension are not clear and sometimes these mechanisms render dialysis difficult to continue.
Purpose We analyzed the changes in blood pressure and sympathetic nerve tone and attempted to clarify whether the dynamic pattern of this relationship reflects cardiovascular dysfunction.
Methods The dynamic pattern of sympathetic nerve activity throughout dialysis was analyzed by frequency analysis of RR intervals recorded by 24 hours Holter electrocardiography in 64 patients and 3 minutes ECG every 15 minutes during dialysis in 121 stable end-stage renal failure patients who underwent maintenance hemodialysis. Blood pressure and fluid volume removed was measured every 15-30 minutes during dialysis and the average value of the ratio of low to high frequency components (LF/HF) was calculated as an index of sympathetic nerve tone. The relationship between removed fluid volume, systolic blood pressure (Bp) and LF/HF was analyzed.
Results The patients were classified into 3 groups based on the correlation between the LF/HF and Bp as follows: positive (52 cases), inverse (54 cases), and not significant (NS; 61 cases). Eighteen patients who showed multiple arrhythmias, atrial fibrillation and other artifacts or noises were eliminated as they were inadequate for frequency analysis of RR intervals. The positive group was characterized by a hypotension-resistant response with a low LF/HF, whereas the inverse group was characterized by a hypotension-prone response with high LF/HF. These results suggest that cardiovascular dysfunction is responsible for the inverse correlation.
Conclusion Analysis of the relationship between sympathetic nerve tone and Bp is effective in predicting the existing of cardiovascular dysfunction.

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Objective We aimed to clarify the prevalence of preexisting Metabolic Syndrome (MetS) defined by the Japanese original criteria among patients with non-fatal myocardial infarction (MI).
Methods This is a retrospective cohort study using the computer database obtained by the preliminary health checkup from April 2003 to December 2008. We extracted the subjects with newly developed non-fatal MI from the study population. The newly non-fatal MI was diagnosed by the history of coronary heart disease (CHD) and new appearance of abnormal Q wave on electrocardiograms. MetS was diagnosed by using the Japanese original criteria. If waist circumference was not available, BMI was used alternatively. We evaluated the prevalence of preexisting MetS and other risk factors of CHD among the subjects. We compared the prevalence of preexisting risk factors between MetS group and non-MetS group.
Results From a study population of 298,455 subjects, 446 subjects with a history of CHD were found. Among the 446, 92 subjects (85 men and 7 women) with abnormal Q wave on electrocardiogram were found. The prevalence of preexisting MetS with non-fatal MI was 19.6% (95% CI; 15.5-23.7%). The prevalence of other preexisting risk factors were 60.0% with smoking history, 55.6% with over-work, 53.3% with stressful life and 36.1% with impaired glucose tolerance. These prevalence rates were not significantly different between MetS group and non-MetS group. Only the prevalence (22.3%) of elevated LDL-cholesterol in the non-MetS group was significantly higher than in the MetS group (14.4%).
Conclusion Preexisting MetS may be able to predict only 20% of future MI. To prevent future myocardial infarction, precaution guidance may be required for people with not only preexisting MetS but also other preexisting risk factors of CHD.

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Objective Increased hemoglobin (Hb) levels are known to be associated with increased cardiovascular events and mortality in hypertensive patients, but the underlying mechanism remains unclear. However, an increased Ambulatory Arterial Stiffness Index (AASI), the surrogate maker of arterial stiffness, has been proven to be an independent predictor of cardiovascular disease. This pilot study evaluated the association between Hb and AASI in untreated essential hypertensive patients without anemia.
Methods A total of 566 untreated essential hypertensive patients without anemia were divided into Normal-Hb and High-Hb groups according to their Hb levels. The AASI and its symmetric calculation (Sym_AASI) were derived from 24h-Ambulatory Blood Pressure Monitoring (24h-ABPM). A multivariable linear regression analysis was performed to determine the relationship between Hb and AASI, Sym_AASI.
Results High-Hb group (n=127) showed higher AASI and Sym_AASI (0.51±0.11 vs 0.43±0.12, p<0.001; 0.33±0.10 vs 0.27±0.08, p<0.001) compared to Normal-Hb group (n=439). Univariate correlation analysis showed that Hb levels were positively related to AASI and Sym_AASI values (r=0.459, p<0.001; r=0.353, p<0.001). After adjustment for age, sex, BMI, current smoker, eGFR, uric acid, total cholesterol, high-density lipoprotein, 24h-SBP, 24h-PP and dipper status, Hb persisted as a independent determinant of AASI and Sym_AASI (β=0.402, p<0.001 and β=0.298, p<0.001, respectively).
Conclusion High hemoglobin seems be to associated with increased AASI in untreated essential hypertensive patients without anemia.

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Objective High-sensitivity C-reactive protein (hs-CRP) is a marker for low-grade inflammation, as well as atherosclerosis, obesity, hyperglycemia and hypertension. Because the factor showing the strongest association with inflammation is currently unknown, we investigated the associations between hs-CRP and clinical and biochemical characteristics in Japanese subjects with mild obesity or impaired glucose tolerance.
Methods Subjects aged <65 years old, attending the Seirei Medical Examination Center, underwent complete physical and laboratory examinations. A total of 112 subjects (mean age 59.9±5.9 years old, males/females: n=50/62) with a waist circumference of >85 cm in males and >90 cm in females, homeostasis model assessment-insulin resistance (HOMA-IR) ≥1.7, or impaired glucose tolerance were eligible for this study. All subjects had normal albuminuria.
Results Log-transformed hs-CRP concentrations were significantly correlated with BMI (r=0.278, p<0.01), HOMA-IR (r=0.296, p<0.005), 2-h post-challenge IRI during an oral glucose tolerance test (r=0.218, p<0.05), maximum intima-media thickness (r=0.240, p<0.05), visceral fat area evaluated by computed tomography (r=0.423, p<0.0001) and subcutaneous fat area (r=0.231, p<0.05). Multiple linear regression analysis showed that visceral fat was the most significantly correlated factor with hs-CRP.
Conclusion Visceral fat mass was a significant and independent predictor for serum hs-CRP levels in Japanese subjects with mild obesity and/or impaired glucose tolerance.

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Objective Because it is often difficult to precisely diagnose and distinguish progressive supranuclear palsy (PSP) from corticobasal degeneration (CBD), multiple system atrophy-parkinsonism (MSA-P) and Parkinson's disease (PD) at the onset of the disease, we compared the patients and clarified the features of these diseases.
Methods We compared 77 PSP, 26 CBD, 26 MSA-P and 166 PD patients from clinical and imaging points of view including cerebral blood flow (CBF) in the frontal eye field.
Results The clinical characteristics of PSP were supranuclear gaze disturbance, optokinetic nystagmus (OKN) impairment and falls at the first visit. On head MRI, midbrain tegmentum atrophy was much more frequently detected in PSP than in all of the other groups. Heart-to-mediastinum average count ratio (H/M) in iodine-123 meta-iodobenzyl guanidine (¹²³I-MIBG) myocardial scintigraphy was not decreased in PSP, CBD, MSA-P and PD-Yahr 1 (-1), but patients of PD-2, 3, 4 and 5 showed a significant decrease compared with the PSP group. The CBF in the left frontal eye field of PD-3 group and that in right frontal eye field of PD-3 and PD-4 groups were lower than that of PSP group, although other groups showed a tendency without a significant decrease compared with PSP group.
Conclusion PSP is distinguishable from CBD, MSA-P and PD even at the early stage with extra-ocular movement (EOM) disturbance, falls, atrophy of the midbrain tegmentum, and H/M in ¹²³I-MIBG myocardial scintigraphy, and the reduction of CBF in area 8 could serve as a supplemental diagnostic method for distinguishing PSP from PD-3 or PD-4.

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Background The (1→3)-β-D-Glucan (BG) assay has been approved for diagnosing invasive fungal disease (IFD). However, the test performance has been variable. We conducted a meta-analysis to determine the overall accuracy of BG assay for diagnosing IFD.
Methods The sensitivity, specificity, and positive and negative likelihood ratios (PLR and NLR, respectively) of BG for diagnosing IFD were pooled using a bivariate meta-analysis. We also performed subgroup analyses.
Results Twelve reports, including 15 studies, were included for the analysis (proven and probable IFD vs possible or no IFD). The sensitivity, specificity, PLR and NLR were 0.76 (95% CI, 0.67-0.83), 0.85 (95% CI, 0.73-0.92), 5.05 (95% CI, 2.71-9.43), and 0.28 (95% CI, 0.20-0.39), respectively. Subgroup analyses showed that the BG assay had higher specificities for patients with hematological disorders and a positive BG result with two consecutive samples. The combination of galactomannan and BG increased the specificity value to 0.98 (95% CI, 0.95-0.99) for diagnosing invasive aspergillosis.
Conclusion Serum BG determination is clinically useful for diagnosing IFD in at-risk patients, especially for hematology patients. The combination of galactomannan and BG was sufficient for diagnosing invasive aspergillosis. Since the BG assay is not absolutely sensitive and specific for IFD, the BG results should be interpreted in parallel with clinical findings.

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Objective The aim of this study was to evaluate the adherence of guidelines for the management and treatment of glucocorticoid-induced osteoporosis, and to investigate whether it is associated with factors such as age, gender, glucocorticoid dose, physician specialty, and size of facility.
Methods This was a cross-sectional study utilizing administrative data from a database of health insurance claims (2004-2007); 2,368 patients who received glucocorticoid treatment for≥90 days were extracted. The guideline adherence was determined by evaluations based on glucocorticoid prescription dose, prescription of anti-osteoporosis drugs, and whether or not bone mineral density was measured.
Results Overall proportion of guideline adherence was 23.3%. In cases in which the equivalent dose of prednisolone was<5 mg/d and≥5 mg/d, the adherence was 8.3% and 30.5% respectively. Factors correlating with low adherence included young age, male gender, and lower glucocorticoid doses. Surgery and otolaryngology specialties had lower adherence than internal medicine. Smaller clinical facilities had lower adherence than larger facilities.
Conclusion The adherence of guidelines for the management and treatment of glucocorticoid-induced osteoporosis is still low, and improvements in treatment quality can be expected through education of patient groups and medical care providers with large deviations from the guidelines.

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We describe a 48-year-old man with thyroid storm presenting with heart failure. He presented severely impaired left ventricular wall motion and a marked increase in the liver enzymes. He developed disseminated intravascular coagulation on day 2. Due to elevated serum thyroid hormone level, anti-thyroid hormone receptor antibody positivity, and his clinical symptoms, he was diagnosed as thyroid storm due to untreated Graves' disease. His condition did not improve even after 6 days of conventional therapy including steroids. After therapeutic plasma exchange was carried out, his thyroid hormone level decreased markedly. Consequently, his condition recovered gradually, and he was discharged at day 43.

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Pulmonary tumor thrombotic microangiopathy (PTTM) is an uncommon cancer-related complication that has been most frequently reported to be associated with adenocarcinoma. We present a case of PTTM which developed in a 60-year-old man with esophageal carcinoma. One year after definitive treatment of the tumor, he developed pulmonary hypertension. Transbronchial lung biopsy (TBLB) specimens showed fibrocellular intimal proliferation and luminal stenosis of the small pulmonary vessels, which contained squamous cell carcinoma cells. Thus, PTTM associated with esophageal carcinoma was diagnosed. This is the first reported case of PTTM associated with esophageal squamous cell carcinoma. TBLB seemed to be useful for obtaining a definitive diagnosis.

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An 82-year-old man had a severe stenosis in the proximal left anterior descending artery (LAD) and an intermediate stenosis in the distal right coronary artery (RCA). The territory of mid to distal LAD was perfused via an angiographically well-developed collateral circulation from the distal RCA. Fractional flow reserve (FFR) in the distal RCA was 0.84. After successful coronary intervention for the proximal LAD, repeat FFR in the distal RCA was 0.96. In this case, the severity of the stenosis in the donor artery was overestimated by using FFR due to the presence of well-developed collateral circulation.

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A 60-year-old man with arrhythmogenic right ventricular cardiomyopathy was readmitted for the battery exchange of his implantable cardioverter-defibrillator (ICD). Since (i) he had been treated with a dual-coil shock lead (Sprint Fidelis, Medtronic) and (ii) pre-operative venography showed mild collateral flow to the left subclavian vein, a single-coil lead was additionally implanted. However, the single-coil defibrillation system was unable to terminate the induced ventricular fibrillation (VF), thus dual defibrillation shock pathways were created using the connection to the superior vena cava coil of the Fidelis lead. The combined connections of the two shock leads provided an appropriate margin of the defibrillation threshold.

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Patients rarely consult physicians before developing coagulopathy or bleeding in most reported cases of superwarfarin intoxication. A 57-year-old woman ingested red-dyed pellets of anticoagulant rodenticide containing difethialone and warfarin as well as tablets of nitrazepam. Although she presented to the hospital in a comatose state, notable pink-colored excreta hinted at the consumption of anticoagulant rodenticide, which led to the early diagnosis of superwarfarin intoxication. Supplementation of large doses of intravenous and oral vitamin K successfully prevented coagulopathy and bleeding. On the other hand, temporary and reversible myocardial suppression was extremely severe, and required the introduction of percutaneous cardiopulmonary support.

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We report a case of Stevens-Johnson syndrome (SJS) in which the patient had been diagnosed with severe obliterative bronchitis. A 29-year-old woman was admitted with a high fever and a widespread vesicular rash. She was diagnosed with SJS and betamethasone administration was started. After one month, her vesicular skin rash improved; however, she developed respiratory failure and was assisted with mechanical ventilation. Computed tomography of the chest demonstrated a hyperlucent lung with narrowing of the peripheral vessels. Bronchoscopy revealed an occlusion of the bronchus when the patient exhaled. The flow-volume curve revealed a severe obstructive pattern. The patient was diagnosed with obliterative bronchitis following SJS. She was treated with a bronchodilator and steroids, but could not breathe adequately without the ventilator. During the following year, her PaCO₂ increased to 100 torr and her heart function also continued to worsen. Despite intensive treatment, she died one year and seven months after the onset of SJS. In SJS and toxic epidermal necrolysis (TEN) patients, chronic pulmonary complications are rare, but there is no effective therapy for obliterative bronchitis following SJS/TEN. Therefore, early awareness of this condition is needed and lung transplantation must be considered at an early stage of this disease.

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We report cases of Japanese sisters with neuromyelitis optica (NMO). The elder sister was 25, when she was diagnosed with right optic neuritis. After 3 months, she developed left optic neuritis and myelitis. At age 27, she had the second relapse, but she has been free from episodes thereafter. The younger sister was 26, when she was diagnosed with optic neuritis. Thus far, she has 9 relapses, comprising both myelitis and optic neuritis. Both sisters had normal brain MRI scans, longitudinally extensive transverse myelitis over 3 vertebral segments, and positive results for anti-aquaporin-4 antibody (AQAP4Ab). They fulfilled the Wingerchuk criteria for definite NMO. Both sisters shared some immunogenetic factors, but they were not exposed to the same environmental factors after their early twenties. The final disability status was almost the same in both cases, and both showed a very benign course. These data suggest that genetic factors affect the age at onset and environmental factors may affect the frequency of relapse.

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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy is a cerebrovasuclar disease caused by NOTCH3 mutations, usually localized to exons 3 and 4. This report describes the clinical and neuroradiological findings of 2 subjects of two unrelated Japanese families who shared a common p.Arg332Cys mutation. The subject from family A presented syncope attacks as the sole clinical presentation at the beginning of his disease course. The subject from family B showed recurrent ischemic attacks, followed by a large intracranial hemorrhage. This is the first report to describe the detailed phenotypes of patients with a rare p.Arg332Cys mutation in Japan.

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A 38-year-old Japanese man with Nasu-Hakola disease (NHD) had repeated pathological fractures and frontal lobe symptoms which developed when he was 18 and 26 years old, respectively. Neuropsychological testing showed memory impairment, and in particular, visuo-spatial memory at the age of 35. Furthermore, single-photon emission computed tomography revealed precuneus hypoperfusion. The patient later suffered prolonged convulsive seizures, which left him in a persistent vegetative state. Genetic testing confirmed a heterozygous mutation in the DAP12 gene (a single-base deletion of 141 G in exon 3) specific to NHD. Precuneus dysfunction might contribute to characteristic memory impairment of NHD.

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Sclerosing mesenteritis is a rare, benign disorder characterized by non-specific and chronic inflammation of the mesenteric adipose tissue. The disease usually presents with gastrointestinal symptoms and abdominal masses. The long-term prognosis is favorable, but it often becomes severe. In the present report we describe a 77-year-old man who presented with diarrhea, massive ascites and an abdominal mass. The rapid deterioration of the general condition of the patient limited invasive examinations and left the primary disease unclear. Despite symptomatic therapy, malnutrition and hypovolemia were prolonged, and he died. The definitive diagnosis of sclerosing mesenteritis and the cause of the fatal outcome were disclosed at autopsy. This case indicates that sclerosing mesenteritis is a potentially-fatal disease and the need for aggressive treatment should be discussed.

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We present a case of fulminant amebic colitis in a human immunodeficiency virus (HIV)-infected homosexual man. The patient developed colonic perforation over a short time despite empirical therapy with metronidazole, and underwent right hemicolectomy. Amebic colitis was pathologically diagnosed by identifying invasive trophozoites of Entamoeba in a surgical specimen. Amebic colitis is one of the important differential diagnoses of acute abdomen in HIV-infected patients and/or homosexual men, especially in East Asia. Although fulminant amebic colitis is a rare manifestation of amebiasis, early diagnosis and treatment are thought to be important to improve the outcome of this highly fatal complication.

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Visceral leishmaniasis (VL) has increased as a complicating infection in subjects with human immunodeficiency virus (HIV) in developing countries. Both infections tend to lower the cell-mediated immunity resulting in poor drug response. In HIV-positive subjects the clinical course as well as organ involvement of VL simulates tuberculosis, another very common tropical infection. We present a case of VL/HIV co-infection where the individual failed to respond to first and second line antiretroviral therapy with persistently low CD4 counts. This patient was also subjected empirically to antitubercular therapy with no clinical improvement; he was finally diagnosed as a case of VL in HIV upon revelation of amastigotes in bone marrow despite the initial negative serology on two occasions. He showed dramatic improvement in CD4 counts and clinical status on Amphotericin B therapy. In endemic areas and in HIV positive subjects a systemic and careful parasitology follow-up is necessary to ensure that no clinical form of leishmaniasis is overlooked.

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