Iliofemoral Aneurysm in Patients with Type 1 Neurofibromatosis: A Case Report and a Literature Review.

Vascular involvement, especially in the iliofemoral segment, is rare in type 1 neurofibromatosis. We herein report a case involving a 49-year-old male diagnosed with type 1 neurofibromatosis who presented with right inguinal pain and swelling. CT angiography revealed a 50-mm aneurysm extending from the right external artery to the common femoral artery. Although surgical reconstruction was performed successfully, the patient required an additional operation 6 years later for aneurysm enlargement in the deep femoral artery. Histopathological examination confirmed the proliferation of neurofibromatosis cells in the aneurysm wall.

[Simultaneous Resection of Mediastinal and Liver Hydatid Disease].

Cystic echinococcosis is an uncommon zoonosis in Japan. Typical location of the cyst is the liver and the lung. Mediastinal hydatidosis is found in less than 0.1% of all hydatid disease patients. We report a rare case of 68 years old male with mediastinal and liver hydatid cysts. Serological test confirmed the diagnosis of hydatidosis. The patient underwent complete resection of mediastinal and liver cysts successfully. Postoperative follow-up for two years has not shown any signs of recurrence. Simultaneous resection of the cysts in different sites including mediastinum is rare and has not been reported in Japan. Hydatid disease is an important differential diagnosis for patient who has cryptogenic liver cyst and history of contact to wildlife or unsterile water.

[Usefulness of Thoracic Endovascular Aortic Repair for the Treatment of Massive Hemoptysis Due to Bronchiectasis;Report of Two Cases].

Massive hemoptysis caused by bronchiectasis threatens life, so early bronchial arterial embolization (BAE) is needed. In case 1, a 80-year-old female complained of hemoptysis and back pain caused by bronchiectasis and Stanford type B aortic dissection. In case 2, a 78-year-old male had history of the surgery of descending thoracic aortic aneurysm and bronchiectasis combined with aortopulmonary fistula. Both cases in bronchiectasis with massive hemoptysis were difficult to perform BAE due to complicated with acute aortic dissection and aortopulmonary fistula. We applied Thoracic endovascular aortic repair (TEVAR) to these cases by its occlusive effects of the orifice of bronchial artery and collateral flow from intercostal arteries. Their postoperative courses were satisfactory without hemoptysis. We suggest that TEVAR can become the one of the option for the treatment of massive hemoptysis that is not indicated to BAE.

[Systemic Lupus Erithematosus Accompanied with Acute Type A and Chronic Type B Aortic Dissection].

A 62-year-old female complained of easy fatigue and sore throat and chest computed tomography revealed acute type A aortic dissection with pericardial effusion and chronic type B aortic dissection that was on set in 2012.She had past history of systemic lupus erythematosus (SLE) 23 years ago and received steroid therapy since then. Blood examination showed inflammation reaction and slight disseminated intravascular coagulopathy. We underwent 2 stage operation safely. In 1st stage we performed ascending graft replacement to type A aortic dissection and in 2nd stage we carried out thoracic endovascular arterial replacement to type B aortic dissection. She had no complication like cerebral infarction and paraplegia postoperatively. It seemed to be rare for the patient in SLE complicated with acute type A and chronic type B aortic dissection and the cause of double aortic dissection was considered long steroid therapy. We carried out 2 stage operation by conventional surgery and endovascular treatment safely without any postoperative complication. We need medical follow up continuously to residual aneurysm.

[Thoracic Endovascular Aortic Repair for Residual Aneurysm after Operation of Acute Stanford type B Aortic Dissection;Report of Two Cases].

We performed thoracic endovascular aortic repair (TEVAR) for residual aortic aneurysm after operation of acute Stanford type B aortic dissection in 2 cases. In case 1, a 53-year-old male who had undergone operation for acute type III a aortic dissection in 2005, underwent TEVAR for ulcer-like projection at distal re-entry site. In case 2, a 55-year-old male who had undergone graft replacement for acute type III b aortic dissection in 2007, underwent TEVAR because the size of the anastomotic aneurysm was increased to 7 cm. Their postoperative courses were satisfactory. We think TEVAR is useful for residual aneurysm after type B aortic dissection surgery because it is less invasive and brings better results.

Correlation between renal function and common risk factors for chronic kidney disease in a healthy middle-aged population: a prospective observational 2-year study.

BACKGROUND/AIMS: Age, proteinuria, metabolic syndrome, and hyperuricemia are the reported risk factors for chronic kidney disease (CKD) and cardiovascular disease (CVD). However, the best predictor of changes in renal function in the early stages of renal disease in a healthy middle-aged population is still unknown. Our study evaluated the correlation between changes in renal function and common risk factors to determine such a predictor. METHODS: In total, 2,853 healthy persons aged ≤50 years participated in the study. They had no proteinuria and were not on medications for hypertension, diabetes mellitus, hyperlipidemia, or hyperuricemia. Over 2 years, participants underwent annual health screening. The relationship between changes in estimated glomerular filtration rate (eGFR) and changes in risk factors for CKD was evaluated using univariate and multivariate linear regression analyses. RESULTS: Over 2 years, eGFR showed a significant decrease. Univariate regression analysis revealed that changes in fasting plasma glucose (FPG), total cholesterol, LDL-cholesterol, serum uric acid levels, and hemoglobin showed a significant negative correlation with changes in eGFR. Multiple regression analysis confirmed that changes in FPG, serum uric acid levels, in particular, and hemoglobin had a significant negative correlation with changes in eGFR. CONCLUSION: The changes in eGFR and other variables over 2 years were small and could be within expected biologic variation. A longer observational study is needed to elucidate whether FPG, serum uric acid and hemoglobin represent the earliest markers of eGFR decline.

[An ascending graft replacement surgery in acute aortic dissection with preoperative cerebral infarction responding to postoperative external and internal decompression; report of a case].

We report a case of a 71-year-old woman who had extensive cerebral infarction associated with acute type A aortic dissection. We urgently performed ascending aortic graft replacement. Postoperative computed tomography of the brain taken immediately after the aortic surgery showed further aggravation of the right cerebral edema and a midline shift. The patient underwent emergent internal and external decompression of the brain. Eventually, consciousness recovered to normal level though preoperative left paraplegia persisted. She was discharged 150 days after the operation. We conclude that immediate internal and external cerebral decompression after surgery for acute type A dissection with preoperative cerebral malperfusion can prevent postoperative higher brain dysfunction.

Longer HSD11B2 CA-repeat in impaired glucose tolerance and type 2 diabetes.

Type 2 11ß-hydroxysteroid dehydrogenase encoded by the HSD11B2 gene converts cortisol to inactive cortisone, and alteration in this enzymatic activity might affect glucose homeostasis by affecting circulating levels or tissue availability of glucocorticoids. We investigated the association of HSD11B2 variant with glucose homeostasis. Subjects with normal glucose tolerance (n=585), impaired glucose tolerance (n=202) and type 2 diabetes (n=355) were genotyped for a highly polymorphic CA-repeat polymorphism in the first intron of HSD11B2. Allele and genotype frequencies differed between normal and impaired glucose tolerance (P = 0.0014 and 0.0407, respectively; 4 degree of freedom) or type 2 diabetes (P = 0.0053 and 0.0078), with significant linear trends between the repeat length and the phenotype fraction. In normal subjects, total CA-repeat length was negatively correlated with fasting insulin and HOMA-ß. Thus, subjects having more CA repeats are susceptible to developing abnormal glucose tolerance, whereas normal subjects carrying more CA repeats appeared to have frugal characteristics in insulin secretion.

Delayed closure of postinfarction ventricular septal rupture.

We present the case of a patient with postinfarction ventricular septal rupture (VSR) who underwent delayed repair using a modified infarction exclusion technique. The patient was taken to the operating room 21 days after the first incidence of acute myocardial infarction because the intra-aortic balloon pump maintained a stable circulatory condition without cardiogenic shock. In our procedure, a Dacron patch was sutured to the healthy endocardium to exclude the infarcted septum, and the VSR was subsequently closed with another Dacron patch. After three years of postoperation, the patient's condition remains normal with good ventricular kinesis and no residual shunt. We describe herein a novel procedure for repairing postinfarction VSR by using two Dacron patches.

[Infected left atrial myxoma; report of a case].

We report a rare case of infected left atrial myxoma. A 69-year-old male was admitted to our hospital due to cerebral infarction accompanied by lower limb ischemia. Transesophageal echocardiography showed a mobile left atrial tumor. On the 16th hospital day, he sufferd from high fever and Klebsiella pneumoniae was positive by blood culture. We excised the left atrial tumor, preventing systemic embolism and progression of sepsis. Histological examination showed a typical myxoma and organized thrombus with Gram-positive bacterial colonies, which disagreed with those in blood culture. After he recovered from sepsis, the 3rd toe of the right foot was amputated and then right femoro-popliteal bypass was done because of failure of wound healing. He was discharged from the hospital on the 74th postoperative day in good condition.

[Successful treatment of a 97-year-old female with acute aortic dissection (DeBakey type II); report of a case].

We operated on an advanced aged patient who presented with acute aortic dissection (DeBakey type II). The patient was a 97-year-old female who was admitted to our hospital due to chest pain. We initially administered conservative medical therapy as far as possible. However, due to the continuing chest pain and the fact that the diameter of the aneurysm was quite large and the risk of a rupture was high, we therefore decided to operate on the ascending graft replacement using retrograde cerebral perfusion on the 8th day from onset. Sufficiently controlling the bleeding proved to be difficult because the site of cannulation on the right atrium was fragile and it therefore tended to split easily. The postoperative course was uneventful except for the fact that it took the patient longer than usual to wake up from anesthesia. As far as could be determined based on an extensive search of the pertinent literature, this appears to be the most advanced aged case of an operation for acute aortic dissection ever reported in Japan.

Case report of familial Carney complex due to novel frameshift mutation c.597del C (p.Phe200LeufsX6) in PRKAR1A.

Carney complex is an autosomal dominantly inherited disease characterized by skin pigmentation, myxoma, primary pigmented nodular adrenocortical disease (PPNAD), and acromegaly. However, only a few incidences of PPNAD combined with acromegaly are observed in patients. The type 1alpha regulatory subunit of cAMP-dependent protein kinase (PRKAR1A) has been identified in patients as a causative gene for Carney complex by a positional cloning approach. Here, we report a female patient diagnosed with Cushing's syndrome and a GH-producing pituitary adenoma without otherwise evident acromegaly that could be diagnosed only by specialized endocrinological tests. Based on family history of acromegaly (mother and sister) and the fact that the combination of both diseases is very rare, genetic diagnosis involving Carney complex was considered to be appropriate. The 10 exons and flanking regions of PRKAR1A were screened for mutations by direct DNA sequencing. The patient and her mother and sister were found to have the same, novel frameshift mutation resulting from a single base deletion in exon 6 coding cAMP-binding domain A, denoted c.597delC in PRKAR1A. This single base deletion generated an immature stop codon at the sixth codon (p.Phe200LeufsX6). Even family members with the same mutation can show distinct phenotypes, suggesting that Carney complex is a multifactorial disorder comprising various genetic and environmental factors. Genetic diagnosis makes it possible to prepare more effective therapeutic strategies for patients and gene carriers and to avoid unnecessary tests for non-carriers in the family of the patient.

Risk factors for heatstroke among Japanese forestry workers.

We examined the risk factors for heatstroke among forestry workers in Japan during the summer. We distributed a questionnaire to 124 forestry workers to determine heatstroke symptoms, degree of sweating and hydration, as well as perceived hotness and amount of sunlight at work sites. Forty of the workers (32.3%) reported experiencing heatstroke symptoms. Thirteen and 21 of them reported such symptoms during July and August, respectively. Eleven workers experienced heatstroke at around 14:00; 5 and 4 developed symptoms at around 11:00 and 10:00, respectively. Groups with and without heatstroke symptoms significantly differed in terms of perceived hotness (p<0.05), sunlight (p<0.05), degree of sweating (p<0.01) and frequency of hydration (p<0.05) while working. Heatstroke symptoms developed in 60.6% of workers aged up to 50 yr, but in only 22.0% of those over the age of 51 (p<0.01). Multiple regression analysis selected the following key variables associated with the development of heatstroke symptoms (R(2)=0.236 and p=0.006): frequency of urination, hotness, BMI and years of forestry work (standard coefficients: +0.229, +0.194, +0.280 and -0.162, respectively). The results of the present study showed that one third of forestry workers developed some symptoms of early heatstroke during summer forestry work. Furthermore, the results indicate that a short duration of forestry service was one of the risk factors contributing to the onset of heatstroke, in addition to heat stress, loss of body water and electrolytes, and obesity.

Transition of nanostructure in DNA-cationic surfactant complexes with the added salt.

Nanostructures of complexes of DNA with single-chain surfactant of octadecyltrimethylammonium (OTA) and double-chain surfactant of didodecyldimethylammonium (DDA) in aqueous NaCl solution at concentration, Cs, from 0 to 500 mM were studied using small-angle-scattering techniques (SAXS). SAXS profiles of the DNA-OTA complex show two SAXS peaks with a spacing ratio of 1:3(1/2) in the solution at Cs below 150 mM and three peaks with a spacing ratio of 1:3(1/2):4(1/2) at Cs above 250 mM. Contents of Na+ and Cl- ions in the complexes evaluated from the atomic absorbance for Na+ and the potentiometry for Cl- revealed charge molar ratios of OTA/DNA = 1 and DDA/DNA = 1.25. Contents of Na+ and Cl- ions per ionic unit of DNA molecule in the DNA-OTA complex equilibrating with the solution at Cs below 100 mM were much less than 0.1, while they increased with NaCl concentration at Cs above 200 mM. The DNA-OTA complex in the solution at Cs above 260 mM exhibited an endothermic peak in the DSC measurements, and the others did not. On the basis of the experimental results, the salt concentration dependent nanostructures are discussed.

Protonation-induced structural change of lyotropic liquid crystals in oley- and alkyldimethylamine oxides/water systems.

Lyotropic phase behavior of the nonionic and the half-ionized oleyldimethylamine oxide (OlDMAO)/water systems was investigated using polarized light microscopy, small-angle X-ray diffraction, and differential scanning calorimetry. Nonionic OlDMAO formed isotropic micellar solution, nematic, hexagonal, cubic, and lamellar liquid crystalline phases as the surfactant concentration increased. In contrast, half-ionized OlDMAO (i.e., 1:1 mixture of the nonionic and the protonated species) had a greater tendency to form bilayer structures, and the phase diagram became quite similar to those of double-chained ionic surfactants rather than single-chained ones, despite the introduction of positive charges to the nonionic one. The preference of the bilayer structures in the half-ionized OlDMAO was interpreted in terms of the dimers stabilized by the hydrogen bond between the nonionic and protonated species. For alkyldimethylamine oxides with a saturated hydrocarbon chain (CnDMAO, chain length: n = 14, 16, and 18), the phase sequence of lyotropic liquid crystals was hardly affected by the protonation, but an elongation of the cylinders of the hexagonal phase was observed for the half-ionized C14DMAO. Consequently, it can be considered that the dominant bilayer formation of the half-ionized OlDMAO is attributed to the combined effect of the hydrogen-bonded dimer formation and the cis-double-bond configuration of the alkyl chain.

Circulating insulin-like growth factor-1 and insulin-like growth factor binding protein-3 are associated with early carotid atherosclerosis.

OBJECTIVE: Growth hormone (GH)-insulin-like growth factor (IGF)-1 axis regulates growth and survival of vascular cells and cardiomyocytes. The role of GH-IGF-1 axis in cardiovascular disease is controversial. METHODS AND RESULTS: We assessed the association of circulating levels of IGF-1 and IGF binding protein-3 (IGFBP-3) with early carotid atherosclerosis and atherosclerotic risk factors in 330 Japanese men (age 51.6+/-8.6 years, range 29 to 77, body mass index [BMI] 23.6+/-2.9 kg/m2). Intima-media thickness (IMT) of the common carotid artery was measured by ultrasound. Abdominal visceral adipose and subcutaneous adipose tissue area by computer-assisted tomographic scan were determined. Correlation coefficients were calculated by partial correlation analysis. BMI and plasma insulin showed positive associations with circulating IGF-1 and IGFBP-3. Subcutaneous adipose tissue was correlated with IGF-1. High-density lipoprotein cholesterol was inversely associated with IGF-1. Blood pressure, total cholesterol, triglyceride, and visceral adipose tissue were positively associated with IGFBP-3. IGF-1 and IGFBP-3 were associated with carotid IMT independent of age, BMI, blood pressure, and insulin. Insulin was associated with carotid IMT in univariate analysis. However, it was not correlated with carotid IMT in the multivariate analyses which included IGF-1 or IGFBP-3 as a covariate. CONCLUSIONS: Increased circulating IGF-1 and IGFBP-3 may be stimulators of atherosclerosis.

Effect of shift work on mental state of factory workers.

This paper examines the effects of shift work on the mental state of factory workers. As an indicator of the workers' mental condition, the authors used a scoring system (referred to below as the 'depression tendency score') based on the SRQ-D investigative report. The depression tendency score of the men was higher among the shift worker group than among the regular day worker group (p < 0.01). The depression tendency score of the male back-to-back shift workers was higher than that of the male regular day workers among skilled workers (p < 0.05). Among the women, no notable difference in depression tendency score was observed between the regular day worker group and the shift worker group. However, the depression tendency score of the female two-shift workers was higher than that of the female regular day workers among skilled workers (p < 0.05). We conclude that the mental health of men is easily affected by back-to-back shift work and that of women is affected by two-shift work because of the difference in modern societal/home role between man and woman.