Video‐assisted thoracoscopic surgery can help enable the complete resection of a mediastinal tumor caused by immunoglobulin G4‐related disease and avoid the need for postoperative medication: A case report

Immunoglobulin (Ig) G4‐related disease has various clinical signs and symptoms, and steroidal therapy with corticosteroids has been found to be effective for treatment. Few cases of IgG4‐related disease associated with paravertebral tumor have been reported, and there have been no reports on complete resection of such a tumor. Here, we report a case of IgG4‐related disease associated with a paravertebral tumor that was successfully resected without the need for postoperative medication. An 84‐year‐old woman was admitted to our hospital with a paravertebral tumor. She underwent thoracoscopic surgery, and pathological examination of the tumor specimen revealed that the tumor resulted from IgG4‐related disease. After resection, there was no need for postoperative medication. Our case indicates the rare possibility of a paravertebral tumor associated with IgG4‐related disease and the potential for complete resection as a treatment for such a tumor.     

Triglyceride Deposit Cardiomyovasculopathy with Massive Myocardial Triglyceride which Was Proven Using Proton-magnetic Resonance Spectroscopy

The patient was a 73-year-old man with a history of hypertension, diabetes mellitus, dyslipidemia, rheumatoid arthritis, repeated percutaneous coronary intervention and percutaneous peripheral intervention procedures. He was frequently admitted to our hospital for congestive heart failure with orthopnea. The myocardial washout rate of iodine-123-β-methyl iodophenyl-pentadecanoic acid was defective on scintigraphy. He was diagnosed with triglyceride deposit cardiomyovasculopathy (TGCV). Proton magnetic resonance spectroscopy (¹H-MRS) indicated the level of myocardial triglyceride (TG) content to be extremely high (4.92%). This is the first report to confirm a massive accumulation of TG in the myocardium of a patient with TGCV using ¹H-MRS noninvasively.     

Confusion and prospects for carcinogenesis of gastric adenoma and dysplasia: What is the correct answer currently?

There are differences in the diagnoses of superficial gastric lesions between Japan and other countries. In Japan, superficial gastric lesions are classified as adenoma or cancer. Conversely, outside Japan, the same lesion is classified as low-grade dysplasia (LGD), high-grade dysplasia, or invasive neoplasia. Gastric carcinogenesis occurs mostly de novo, and the adenoma-carcinoma sequence does not appear to be the main pathway of carcinogenesis. Superficial gastric tumors can be roughly divided into the APC mutation type and the TP53 mutation type, which are mutually exclusive. APC-type tumors have low malignancy and develop into LGD, whereas TP53-type tumors have high malignancy and are considered cancerous even if small. For lesions diagnosed as category 3 or 4 in the Vienna classification, it is desirable to perform complete en bloc resection by endoscopic submucosal dissection followed by staging. If there is lymphovascular or submucosal invasion after mucosal resection, additional surgical treatment of gastrectomy with lymph node dissection is required. In such cases, function-preserving curative gastrectomy guided by sentinel lymph node biopsy may be a good alternative.     

Role of radial strain and displacement imaging to quantify wall motion dyssynchrony in patients with left ventricular mechanical dyssynchrony and chronic right ventricular pressure overload

Left ventricular (LV) deformation with ventricular septal shift is one of the most distinctive echocardiographic observations in patients with chronic right ventricular (RV) pressure overload (PO). However, little is known about the effects of RVPO on LV performance and regional synchrony. Accordingly, our objective was to test the hypothesis that chronic RVPO affects regional wall motion, synchronicity, and global LV function using a novel speckle-tracking approach to quantify and characterize regional LV wall motion dyssynchrony. Displacement and strain imaging echocardiographic studies were performed in 20 patients with RVPO from pulmonary arterial hypertension or pulmonic stenosis (mean age 53 +/- 16 years, New York Heart Association class 2.6 +/- 0.7, and peak RV systolic pressure 73 +/- 28 mm Hg) and 20 age-matched normal subjects (mean age 47 +/- 16 years). Segmental signals from 6 segments around the mid-LV short axis were defined as dyssynchronous if their changes were opposite to that of the global LV signal at each time frame, and overall LV dyssynchrony was calculated as the percentage of dyssynchrony in all 6 segments within the specified time interval from onset of QRS to the end of isovolumic relaxation. RVPO was associated with a large degree of regional dyssynchrony with paradoxical ventricular septal motion observed by displacement imaging (21 +/- 6%, p <0.05 vs control group), which was closely associated with LV eccentricity index (r = 0.79, p <0.05) and LV myocardial performance index with linear regression (r = 0.76, p <0.05). In contrast, strain imaging showed uniform segmental radial thickening in the RVPO group, which was similar to the control group, and suggests that there was no intrinsic LV contractile dyssynchrony. In conclusion, LV wall motion dyssynchrony assessed by displacement imaging, not intrinsic contractile dyssynchrony by strain imaging, coexists with LV chamber deformation with ventricular septal shift and is closely associated with impairment of LV performance.     

Metformin,but not pioglitazone,decreases postchallenge plasma ghrelin levels in type 2 diabetic patients: a possible role in weight stability?

Aim: Effects of metformin and pioglitazone on body weight are clearly different. Recently, the role of ghrelin, an orexigenic peptide derived from stomach, has been appreciated. Plasma ghrelin levels display a preprandial peak and postprandial suppression, suggesting its physiological role. We hypothesized that metformin or pioglitazone may modulate circulating ghrelin levels and this modulation may be related to differential effects on body weight with these agents. Methods: Thirty‐five Japanese type 2 diabetic patients [21 men and 14 women, age 62 ± 2 years, body mass index (BMI) 26.6 ± 0.5 kg/m² and haemoglobin A1c (HbA1c) 8.2 ± 0.1%, mean ± s.e.] were randomly assigned to groups for the addition of metformin or pioglitazone. At baseline and 4 months later, a 75‐g oral glucose tolerance test (OGTT) was performed to measure plasma ghrelin levels. Results: In 33 subjects who completed the study, the overall decrease in HbA1c (～1%) was comparable between the two groups. As expected, BMI increased in the pioglitazone group but not in the metformin group. After the treatment, plasma ghrelin levels at each point of OGTT remained unchanged in the pioglitazone group. In the metformin group, fasting ghrelin levels were unaltered, whereas the absolute levels at 30, 60 and 120 min decreased significantly. The area under the curve for the 2‐h ghrelin profile also decreased significantly. Conclusions: Metformin, but not pioglitazone, decreased plasma ghrelin levels after the glucose load. This decrease may in part account for weight stability in type 2 diabetic patients treated with metformin.     

Detection of the earliest ventricular contraction site in patients with Wolff-Parkinson-White syndrome using two-dimensional guided M-mode tissue Doppler echocardiography

OBJECTIVE: The purpose of this study was to examine the feasibility of M-mode tissue Doppler imaging for localizing the accessory pathway in patients with Wolff-Parkinson-White (WPW) syndrome. METHODS: Two-dimensional guided tissue Doppler M-mode was recorded at the mitral and tricuspid annular levels in 13 WPW patients. Time intervals were measured from the onset of the delta wave or the R wave to the beginning of the ventricular systolic motion. The earliest contraction site was defined as the site demonstrating the shortest time interval, and compared with the earliest activated site determined by body surface mapping and the successful ablation site. RESULTS: In 6 patients with a left-sided pathway, tissue Doppler localization was identical to the ablation site. In 3 with a left-sided pathway and 3 with a right-sided pathway, localization was judged as an adjacent region of the ablation site. In 1 patient with a right lateral pathway, the pathway location was misdiagnosed. The tissue Doppler diagnosis for the left-sided pathways correlated well with the ablation site, in contrast to the right-sided pathways (p = 0.05). Prediction of the accessory pathway localization by tissue Doppler M-mode was equivalent to localization based on body surface mapping. CONCLUSIONS: In WPW syndrome, tissue Doppler M-mode can detect the earliest contraction sites and seems helpful in localizing the left-sided accessory pathways, but is of limited use for right-sided pathways.     

Association of CTLA-4 polymorphism with positive anti-GAD antibody in Japanese subjects with type 1 diabetes mellitus

OBJECTIVE: CTLA-4, expressed on activated T cells, is thought to be a negative regulator of T cell function. Its gene (2q33) may confer genetic susceptibility to type 1 diabetes mellitus (IDDM12). The present study was undertaken to clarify the role of CTLA-4 gene polymorphism in Japanese subjects with type 1 diabetes and its effect on their clinical features. SUBJECTS AND METHODS: In 117 Japanese subjects with type 1 diabetes, the CTLA-4 exon 1 polymorphism (49 A/G) was defined by PCR-RFLP analysis. Anti-GAD antibodies (GAD-Ab) and fasting serum C-peptide were also determined. 141 healthy age- and sex-matched subjects served as controls. RESULTS: The frequency of each polymorphism was not different between the type 1 diabetic subjects and the controls; AA 21, AG 42 and GG 54 for the diabetic subjects, and AA 22, AG 47 and GG 72 for the controls. The frequency of the GG genotype was higher in the diabetic subjects with positive GAD-Ab (greater than 8 U/ml) (67%) than in the GAD-Ab negative subjects (39%) (P < 0.05). The prevalence of positive GAD-Ab declined with the duration of diabetes. In the diabetic subjects with disease duration of less than 5 years (n = 40), the frequency of the GG genotype was also higher in the GAD-Ab positive subjects (71%) (P < 0.05). In the analysis of all the diabetic subjects, there was a strong association between positive GAD-Ab and beta cell function (P < 0.0001). CONCLUSIONS: There was no evidence that the CTLA-4 exon 1 polymorphism (49 A/G) confers genetic susceptibility to type 1 diabetes mellitus in our case-control study in Japanese subjects. However, the frequency of positive GAD-Ab was higher in the GG subjects. CTLA-4 polymorphism might contribute to the clinical heterogeneity of type 1 diabetes mellitus in Japanese subjects.     

Mediastinal neurilemmoma originating in the right phrenic nerve: a case report]

We report on a 31-year-old man with a mediastinal neurilemmoma originating in the right phrenic nerve. The patient was admitted because of abnormal chest X-ray shadows observed during a routine checkup. A preoperative diagnosis of bronchial cyst in the mediastinum was made. The tumor was resected by video-assisted thoracic surgery. It was 3 x 4 x 3 cm in size, weighted 15 g, and originated in the right phrenic nerve. The histopathologic diagnosis was mediastinal neurilemmoma. Only 14 cases have been reported in the Japanese literature.     

Gln27Glu and Arg16Gly polymorphisms of the beta2-adrenergic receptor gene are not associated with obesity in Japanese men

The beta2-adrenergic receptor (beta2AR), beta3AR, or uncoupling protein 1 (UCP1) may play a pathogenic role in obesity. In Swedish Caucasians, a polymorphism at codon 27 (Gln27Glu) of the beta2AR gene was shown to be associated with obesity, but no such association was shown for a polymorphism of codon 16 (Arg16Gly). Thus, we investigated whether these polymorphisms contribute to obesity in 210 Japanese men. The frequencies of the Gln27Glu and Arg16Gly polymorphisms were 0.05 and 0.48, respectively, and there was no association with obesity. A strong linkage disequilibrium between the Gln27Glu and Arg16Gly polymorphisms was shown, but there was no apparent additive effect on the clinical or metabolic characteristics. Our results suggest that the Gln27Glu and Arg16Gly polymorphisms of the beta2AR gene are not a major contributing factor to obesity in Japanese men.     

Prognostic impact of cascade screening for familial hypercholesterolemia on cardiovascular events

BackgroundFamilial hypercholesterolemia (FH) is an autosomal dominant disorder mainly caused by mutations in the low-density lipoprotein (LDL) receptor or associated genes, resulting in elevated serum cholesterol levels and an increased risk of premature atherosclerotic cardiovascular disease (ASCVD).ObjectiveWe aimed to evaluate the prognostic impact of cascade screening for FH.MethodsWe retrospectively investigated the health records of 1050 patients with clinically diagnosed FH, including probands and their relatives who were cascade-screened, who were referred to our institute. We used Cox models that were adjusted for established ASCVD risk factors to assess the association between cascade screening and major adverse cardiac events (MACE). The median period of follow-up evaluating MACE was 12.3 years (interquartile ranges [IQR] = 9.1–17.5 years), and MACE included death associated with ASCVD, or acute coronary syndrome.ResultsDuring the observation period, 113 participants experienced MACE. The mean age of patients identified through cascade screening was 18-years younger than that of the probands (38.7 yr vs. 57.0 yr, P < 0.0001), with a lower proportion of ASCVD risk factors. Interestingly, patients identified through cascade screening under milder lipid-lowering therapies were at reduced risk for MACE (hazard ratio [HR] = 0.67; 95%CI = 0.44 to 0.90; P = 0.0044) when compared with the probands, even after adjusting for those known risk factors, including age, and prior ASCVD.ConclusionsThe identification of patients with FH via cascade screening appeared to result in better prognosis.