We describe the case of a 1-year and 7-month-old girl who was born at 36 weeks and 6 days of pregnancy weighing 1,351 g. In addition to the diagnosis of Cornelia de Lange syndrome and Tetralogy of Fallot, we confirmed shunt blood flow from the lesser curvature of the aortic arch to the main pulmonary artery. Thus, we additionally diagnosed ectopic patent ductus arteriosus (PDA). Ultrasonography showed interruption and retrograde flow of the diastolic blood flow in the anterior cerebral artery. Therefore, we made a diagnosis of blood stealing due to an ectopic PDA, and we performed surgical intervention via a median sternotomy at 25 days. When we ligated the shunt blood vessel, the percutaneous oxygen saturation decreased from the high 90% range to the low 70% range, thus we temporarily released the ligation. We narrowed the ectopic PDA so that the percutaneous oxygen saturation could be maintained the high 80% range. Postoperative ultrasonography showed improvement of the pressure waveform in the anterior cerebral artery. After discharge, oxygen demand increased gradually with weight gain, and we performed intracardiac repair using a monocusp valve patch at 1 year and 7 months. We report a rare case of Cornelia de Lange syndrome wherein we achieved intracardiac repair of Tetralogy of Fallot after ectopic PDA banding in the neonatal period.

Objective: To assess the noncontrast two-dimensional single-shot balanced turbo-field-echo magnetic resonance angiography (b-TFE MRA) features of the abdominal aortic aneurysm (AAA) status following endovascular aneurysm repair (EVAR) and evaluate to detect endoleaks (ELs). Materials and Methods: We examined four aortic stent-grafts in a phantom study to assess the degree of metallic artifacts. We enrolled 46 EVAR-treated patients with AAA and/or common iliac artery aneurysm who underwent both computed tomography angiography (CTA) and b-TFE MRA after EVAR. Vascular measurements on CTA and b-TFE MRA were compared, and signal intensity ratios (SIRs) of the aneurysmal sac were correlated with the size changes in the AAA after EVAR (AAA prognoses). Furthermore, we examined six feasible b-TFE MRA features for the assessment of ELs. Results: There were robust intermodality (r = 0.92–0.99) correlations and interobserver (intraclass correlation coefficient = 0.97–0.99) agreement. No significant differences were noted between SIRs and aneurysm prognoses. Moreover, “mottled high-intensity” and “creeping high-intensity with the low-band rim” were recognized as significant imaging findings suspicious for the presence of ELs (p < 0.001), whereas “no signal black spot” and “layered high-intensity area” were determined as significant for the absence of ELs (p < 0.03). Based on the two positive features, sensitivity, specificity, and accuracy for the detection of ELs were 77.3%, 91.7%, and 84.8%, respectively. Furthermore, the k values (0.40–0.88) displayed moderate-toalmost perfect agreement. Conclusion Noncontrast MRA could be a promising imaging modality for ascertaining patient follow-up after EVAR.

Objective: To assess the noncontrast two-dimensional single-shot balanced turbo-field-echo magnetic resonance angiography (b-TFE MRA) features of the abdominal aortic aneurysm (AAA) status following endovascular aneurysm repair (EVAR) and evaluate to detect endoleaks (ELs). Materials and Methods: We examined four aortic stent-grafts in a phantom study to assess the degree of metallic artifacts. We enrolled 46 EVAR-treated patients with AAA and/or common iliac artery aneurysm who underwent both computed tomography angiography (CTA) and b-TFE MRA after EVAR. Vascular measurements on CTA and b-TFE MRA were compared, and signal intensity ratios (SIRs) of the aneurysmal sac were correlated with the size changes in the AAA after EVAR (AAA prognoses). Furthermore, we examined six feasible b-TFE MRA features for the assessment of ELs. Results: There were robust intermodality (r = 0.92–0.99) correlations and interobserver (intraclass correlation coefficient = 0.97–0.99) agreement. No significant differences were noted between SIRs and aneurysm prognoses. Moreover, “mottled high-intensity” and “creeping high-intensity with the low-band rim” were recognized as significant imaging findings suspicious for the presence of ELs (p < 0.001), whereas “no signal black spot” and “layered high-intensity area” were determined as significant for the absence of ELs (p < 0.03). Based on the two positive features, sensitivity, specificity, and accuracy for the detection of ELs were 77.3%, 91.7%, and 84.8%, respectively. Furthermore, the k values (0.40–0.88) displayed moderate-toalmost perfect agreement. Conclusion Noncontrast MRA could be a promising imaging modality for ascertaining patient follow-up after EVAR.

BACKGROUND: Specific IgE antibodies against the low-molecular-weight carbohydrate antigen that does not bridge IgE molecules on mast cells are not associated with clinical symptoms. Cross reactivity can be determined in allergen-specific IgE detection assays when the carbohydrate structures between pollen allergens and plant derived food allergens are similar; in such cases, false positive results for grain or legume allergens can be reported for pollen allergic patients who are not sensitized to those allergens. This phenomenon arises owing to the presence of cross-reactive carbohydrate determinants (CCDs). OBJECTIVE: This study aimed to assess the impact of CCD interference on the results for pollen allergen-specific IgE antibodies in the general adult population and to perform CCD inhibition tests evaluating the involvement of CCD on samples positive to pollen allergens. METHODS: Serum samples from 322 subjects were tested for IgE antibodies to pollens and CCD. The research subjects were given questionnaires about pollen allergic symptoms to help assess the presence of allergies. Allergen IgE antibodies for Japanese cedar, Japanese cypress, orchard grass, ragweed, MUXF, bromelain, horseradish peroxidase (HRP), and ascorbate oxidase (ASOD) were analyzed. RESULTS: It was observed that among individuals who tested positive to any of the pollen allergens, the positive ratio of CCD-specific IgE antibody was the highest for HRP (13.5%–50.0%). The results from the inhibition tests revealed that CCD was marginally present. Although IgE antibodies for cedar pollen did not react with CCD, IgE antibodies for Japanese cypress, orchard grass, and ragweed might be detected by the presence of CCD. CONCLUSION: The results of the inhibition tests revealed the obvious presence of CCD suggesting its involvement. Considering these findings, careful evaluation of patient IgE results should be performed for Japanese cypress, orchard grass, and ragweed.
Adult ; Allergens ; Ambrosia ; Antibodies ; Ascorbate Oxidase ; Asian Continental Ancestry Group ; Bromelains ; Cryptomeria ; Cupressus ; Dactylis ; Fabaceae ; False Positive Reactions ; Horseradish Peroxidase ; Humans ; Hypersensitivity ; Immunoglobulin E ; Mast Cells ; Plants ; Pollen ; Research Subjects ; Rhinitis, Allergic ; Rhinitis, Allergic, Seasonal

BACKGROUND/AIMS: Twenty-four-hour esophageal impedance and pH monitoring allows detection of all types of reflux episodes and is considered the best technique for identifying gastroesophageal refluxes. However, normative data for the Japanese population are lacking. This multicenter study aimed to establish the normal range of 24-hour esophageal impedance and pH data both in the distal and the proximal esophagus in Japanese subjects. METHODS: Forty-two healthy volunteers (25 men and 17 women) with a mean ± standard deviation age of 33.3 ± 12.4 years (range: 22–72 years) underwent a combined 24-hour esophageal impedance and pH monitoring. According to the physical and pH properties, distal or proximal esophageal reflux events were categorized. RESULTS: Median 45 reflux events occurred in 24 hours, and the 95th percentile was 85 events. Unlike previous reports, liquid-containing reflux events are median 25/24 hours with the 95th percentile of 62/24 hours. Acidic reflux events were median 11/24 hours with the 95th percentile of 39/24 hours. Non-acidic gas reflux events were median 15/24 hours with the 95th percentile of 39/24 hours. Proximal reflux events accounted for 80% of the total reflux events and were mainly non-acidic gas refluxes. About 19% of liquid and mixed refluxes reached the proximal esophagus. CONCLUSIONS: Unlike previous studies, liquid-containing and acidic reflux events may be less frequent in the Japanese population. Non-acidic gas reflux events may be frequent and a cause of frequent proximal reflux events. This study provides important normative data for 24-hour impedance and pH monitoring in both the distal and the proximal esophagus in the Japanese population.
Asian Continental Ancestry Group* ; Electric Impedance* ; Esophagus ; Gastroesophageal Reflux ; Healthy Volunteers ; Humans ; Hydrogen-Ion Concentration* ; Male ; Reference Values*

OBJECTIVE: To evaluate the multiphase contrast-enhanced magnetic resonance (MR) imaging features of Bacillus Calmette-Guerin (BCG)-induced granulomatous prostatitis (GP). MATERIALS AND METHODS: Magnetic resonance images obtained from five patients with histopathologically proven BCG-induced GP were retrospectively analyzed for tumor location, size, signal intensity on T2-weighted images (T2WI) and diffusion-weighted images (DWI), apparent diffusion coefficient (ADC) value, and appearance on gadolinium-enhanced multiphase images. MR imaging findings were compared with histopathological findings. RESULTS: Bacillus Calmette-Guerin-induced GP (size range, 9-40 mm; mean, 21.2 mm) were identified in the peripheral zone in all patients. The T2WI showed lower signal intensity compared with the normal peripheral zone. The DWIs demonstrated high signal intensity and low ADC values (range, 0.44-0.68 x 10(-3) mm2/sec; mean, 0.56 x 10(-3) mm2/sec), which corresponded to GP. Gadolinium-enhanced multiphase MR imaging performed in five patients showed early and prolonged ring enhancement in all cases of GP. Granulomatous tissues with central caseation necrosis were identified histologically, which corresponded to ring enhancement and a central low intensity area on gadolinium-enhanced MR imaging. The findings on T2WI, DWI, and gadolinium-enhanced images became gradually obscured with time. CONCLUSION: Bacillus Calmette-Guerin-induced GP demonstrates early and prolonged ring enhancement on gadolinium-enhanced MR imaging which might be a key finding to differentiate it from prostate cancer.
Aged ; Gadolinium/*diagnostic use ; Humans ; Image Enhancement ; Immunotherapy/*adverse effects ; Magnetic Resonance Imaging/*methods ; Magnetic Resonance Spectroscopy ; Male ; Middle Aged ; Mycobacterium bovis/*pathogenicity ; Prostate-Specific Antigen/blood ; Prostatic Neoplasms/diagnosis ; Prostatitis/*diagnosis ; Retrospective Studies ; Urinary Bladder Neoplasms/drug therapy

We present a case of Marfan's syndrome with acute aortic dissection during the trimester of her pregnancy, who underwent a Bentall operation 2 days after emergency cesarean section. A 24-year-old woman during the 31st week of pregnancy visited our emergency room due to sudden onset of chest and back pain, though she had no abnormality until this event. Because of her tall height, spider fingers, positive wrist sign, visual disorder and scoliosis, she was given a diagnosis of Marfan's syndrome. Enhanced CT and cardiac ultrasonography revealed that she was suffering from acute aortic dissection with annulo-aortic ectasia. Since it was difficult for her to continue with her pregnancy, she underwent emergency cesarean section and gave birth to a male baby weighted 1, 706g. Although there was little likelifood of early thrombus formation in the false lumen or significant aortic regurgitation indicating an emergency operation, fear of massive bleeding from her uterus and the exfoliated surface of the placenta after cesarean section required an observation period of 2 days. We performed a Bentall operation successfully after careful sedation, ventilation and blood pressure control for 2 days.

Between February 1999 and November 1999, 33 patients (age 67.0±7.6 years old) underwent off-pump CABG using coronary shunt tubes. The number of graft anastomoses per patient was 2.8±0.8. The operative mortality was 0%. There was no incidence of on-pump conversion, low cardiac output syndrome, IABP insertion, mediastinitis or stroke. The maximum CPK-MB during the perioperative period was 25.9±18.8IU/l. One patient had perioperative myocardial infarction probably due to native coronary artery spasm. In patients with off-pump CABG, the intubation time, the ICU stay and the hospital stay were shorter. The number of patients who were extubated in the operating room was higher and the cost was lower than those with on-pump CABG. An early phase study revealed patency ratios of 85% (the previous term) and 97% (the latter term). Off-pump CABG is a safe and effective means of revascularization with no mortality, minimal morbidity and good short-term patency.

Combined monitoring of rSO₂ and SSEP is routinely performed during cardiopulmonary bypass (CPB), but it is not sensitive enough to detect focal lesions of the brain. Thus, we assessed whether simultaneous measurement of S-100β is able to enhance diagnostic sensitivity or not. Between September 1999 and February 2000, serial measurement of plasma levels of S-100β and SSEP and rSO₂ monitoring during CPB were simultaneously performed in 26 consecutive patients (19 men and 7 women). Ages ranged from 46 to 85 (mean 67±10 years). Neurological complications developed in 5 (19.2%). Among those patients, hemiplegia developed in 2, and dementia, temporary convulsion, and deep coma in 1 each. Three of them showed abnormally low rSO₂ levels during surgery, but no patient showed abnormal change in SSEP waves after surgery. There was no significant difference in S-100β level 1h after CPB between patients associated with or without neurological complications (1.98±0.48 vs. 1.89±1.65), however, its level 24h after CPB remained significantly higher in patients with neurological complications (1.01±1.14 vs. 0.22±0.24). S-100β level 24h after CPB appears to improve diagnostic sensitivity for detecting such focal brain damage lesions as those in which SSEP or rSO₂ are not efficient enough to make a diagnosis. However, further study is required to evaluate how fast it can differentiate patients with and without brain damage.

The earlier the diagnosis of acute type A aortic dissection is made, the more frequent the complications of aortic root destruction and/or a compromised coronary artery are encountered. Only aortography is diagnostic in these lesions, however, recently this modality tends to be avoided in order to try to improve the survival rate of the patients by obtaining diagnosis by noninvasive modalities. Therefore, contrast-enhanced CT scans in 49 patients with aortic dissection were analyzed in order to detect the slightest signs suggesting aortic root lesions. In 4 of the 6 cases in which intimal flap was detected in the aortic root by CT and in 2 of the 14 cases with an aortic root more than 35mm in diameter, aortic root reconstruction and/or concomitant CABG were neccessary for the repair of the destroyed aortic root. The aortic root diameter was more than 40mm in 8 of 9 patients with aortic root destruction, with a mean value of 45.6±3.6mm (p<0.01). In summary, detection of a septum in the aortic root and/or an aortic root dilated more than 40mm on CT were important signs suggesting the dissection extending to the aortic sinus combined with aortic root destruction. In such cases aortic root reconstruction and/or concomitant CABG may be necessary.