[Ruptured Stanford Type A Acute Aortic Dissection Accompanying Persistent Sciatic Artery Aneurysm: Report of a Case].

Persistent sciatic artery( PSA) is a rare congenital anomaly and often results in aneurismal or occlusive changes. A 82-year-old woman was reffered with diagnosis of Stanford type A acute aortic dissection complicated cardiac tamponade. Emergent aortic arch replacement was performed. Cardiopulmonary bypass was established by central cannulation into true lumen of aortic arch because of asceding aortic rupture during the operation, axillary arteries dissection and hypoplastic femoral arteries. Post-operative course was uneventful. She was transferred to the local hospital 32 days after the operation for the purpose of rehabilitation.

A novel frameshift variant of GATA3 (p.Ala17ProfsTer178) responsible for HDR syndrome in a Japanese family.

HDR syndrome is an autosomal dominant disorder characterized by hypoparathyroidism (H), deafness (D), and renal dysplasia (R) caused by genetic variants of the GATA3 gene. We present the case of a 38-year-old Japanese man with HDR syndrome who exhibited hypoparathyroidism, sensorineural deafness, renal dysfunction, severe symptomatic hypocalcemia with Chvostek's and Trousseau's signs, and QT prolongation on electrocardiography. He had a family history of deafness and hypocalcemia. Genetic testing revealed a novel GATA3 gene variant at exon 2 (c.48delC), which induces a frameshift resulting in termination at codon 178, causing HDR syndrome. We summarized 45 Japanese cases of HDR syndrome with regard to the mode of onset (familial or sporadic) and the age at diagnosis. In addition, we summarized all previous cases of HDR syndrome with GATA3 gene variants. Mapping of previously reported genetic variants in HDR syndrome revealed that most missense variants were observed at exons 4 and 5 regions in the GATA3 gene. These two regions contain zinc finger domains, demonstrating their functional importance in GATA3 transcription. This review of literature provides a useful reference for diagnosing HDR syndrome and predicting the related future manifestations.

Lower extremity artery thromboembolism during removal of Impella after repair for ventricular septal rupture: A case report.

Although the anticoagulant complications of Impella are well known, the timing of heparin administration when using Impella immediately after open heart surgery has not been established. We report a case of a 59-year-old man with Impella-assisted repair of a ventricular septal perforation after acute myocardial infarction who developed thromboembolism of the lower extremity arteries after removal of Impella.

[Right Atrial Myxoma After Catheter Ablation:Report of a Case].

An 81-year-old man underwent total arch replacement for thoracic aortic aneurysm 8 years ago and catheter ablation for paroxysmal atrial fibrillation 1 year ago. Transthoracic echocardiography revealed a mass in the right atrium, and the patient was admitted for close examination and treatment. Transesophageal echocardiography revealed a 23×17 mm large well-defined mass above the cavotricuspid isthmus. Two venous drainage cannulas were inserted directly to the superior vena cava and to the inferior vena cava via the right femoral vein, in order to avoid the direct contact with the right atrium prior to institution of cardiopulmonary bypass. The right atrial tumor was found attached to the cavotricuspid isthmus, and was resected together with the right atrial wall. Pathological examination showed myxomatous tissue. Postoperative course was uneventful. He was discharged 23 days after the operation.

[Surgical Treatment for Ruptured Thoracoabdominal Aneurysm Following Descending Aortic Replacement with Double Barrel Anastomosis].

A 75-year-old woman was diagnosed with type B acute aortic dissection 14 years ago and 3-channeled aortic dissection 7 years ago. She received total arch replacement 6 years ago and descending aortic replacement with double barrel anastomosis technique for distal anastomosis 5 years ago. Computed tomography( CT) revealed giant thyroid tumor and thoracoabdominal aortic aneurysm( 58 mm in diameter). She suffered from back pain during her follow-up period. CT revealed ruptured thoracoabdominal aortic aneurysm. First, the false lumen of descending aorta was closed by thoracic endovascular aortic repair, and then thoracoabdominal aortic replacement was performed uneventfully.

Japanese 17q12 Deletion Syndrome with Complex Clinical Manifestations.

17q12 deletion syndrome is a rare chromosomal anomaly with variable phenotypes, caused by the heterozygous deletion of chromosome 17q12. We herein report a 35-year-old Japanese patient with chromosomal 17q12 deletion syndrome identified by de novo deletion of the 1.46 Mb segment at the 17q12 band by genetic analyses. He exhibited a wide range of phenotypes, such as maturity-onset diabetes of the young (MODY) type 5, structural or functional abnormalities of the kidney, liver, and pancreas; facial dysmorphic features, electrolyte disorders; keratoconus, and acquired perforating dermatosis. This case report provides valuable resources concerning the clinical spectrum of rare 17q12 deletion syndrome.

A newly identified compound activating UCP1 inhibits obesity and its related metabolic disorders.

OBJECTIVE: Promoting thermogenesis in adipose tissue has been a promising strategy against obesity and related metabolic complications. We aimed to identify compounds that promote thermogenesis in adipocytes and to elucidate their functions and roles in metabolism. METHODS: To identify compounds that directly promote thermogenesis from a structurally diverse set of 4800 compounds, we utilized a cell-based platform for high-throughput screening that induces uncoupling protein 1 (Ucp1) expression in adipocytes. RESULTS: We identified one candidate compound that activates UCP1. Additional characterization of this compound revealed that it induced cellular thermogenesis in adipocytes with negligible cytotoxicity. In a subsequent diet-induced obesity model, mice treated with this compound exhibited a slower rate of weight gain, improved insulin sensitivity, and increased energy expenditure. Mechanistic studies have revealed that this compound increases mitochondrial biogenesis by elevating maximal respiration, which is partly mediated by the protein kinase A (PKA)-p38 mitogen-activated protein kinase (MAPK) signaling pathway. A further comprehensive genetic analysis of adipocytes treated with these compounds identified two novel UCP1-dependent thermogenic genes, potassium voltage-gated channel subfamily C member 2 (Kcnc2) and predicted gene 5627 (Gm5627). CONCLUSIONS: The identified compound can serve as a potential therapeutic drug for the treatment of obesity and its related metabolic disorders. Furthermore, our newly clarified thermogenic genes play an important role in UCP1-dependent thermogenesis in adipocytes.

[Surgical Treatment of the Perforation of the Right Ventricle and Diaphragm by a Permanent Pacing Lead:Report of a Case].

An 97-year-old woman was diagnosed with complete atrioventricular block and underwent pacemaker implantation( PMI). Three days after the PMI, computed tomography revealed cardiac perforation and migration of the lead to the abdominal cavity. Surgical procedure through median sternotomy was performed, and the penetrated lead was removed. The holes of the right ventricle and diaphragm were repaired. Abdominal organ was not injured. She was discharged 14 days after the surgical procedure.

[Delayed Type â ¢b Endoleak After Thoracic Endovascular Aortic Repair for Thoracic Aortic Aneurysm: Report of a Case].

An 85-year-old woman underwent aortic arch replacement and thoracic endovascular aortic repair (TEVAR) 5 years ago. She suffered from chest and back pain. Computed tomography (CT) demonstrated enlargement of the aortic aneurysm by a type Ⅲb endoleak. TEVAR was performed to close a type Ⅲb endoleak with a relining technique uneventfully. Intraoperative completion aortography and postoperative CT confirmed the disappearance of a type Ⅲb endoleak. She was discharged 27 days after the treatment.

[Acute Type A Aortic Dissection Complicated by Cerebral Malperfusion and Leg Ischemia:Report of a Case].

An 82-year-old woman suddenly developed chest pain and apoplexy. Computed tomography (CT) showed acute type A aortic dissection, the true lumen in the brachicephalic artery was severely compressed by the faulse lumen. Pulsation in the either leg was not detected during induction of anesthesia. We evaluated the cerebral blood flow and lower extremity blood flow using near infrared spectroscopy (NIRS) during the operation, tissue oxygenation index (TOI) was continuously monitored during the operation. Cardiopulmonary bypass( CPB) was established by puncturing the true lumen in the ascending aorta and bicaval venous drainage. TOI was returned to normal range by CPB. Although the central repair (ascending aorta replacement) was performed, leg ischemia persisted. We performed ascending aorta-bifemoral bypass. After the operation, leg ischemia disappeared and CT revealed patency of the bypass graft. Postoperative course was uneventful without deterioration of neurological function. She was discharged 49 days after the operation.

Relationship between the changes in hepatokine levels and metabolic effects after laparoscopic sleeve gastrectomy in severely obese patients.

PURPOSE: To clarify the relationships between the changes in hepatokines and weight loss, and between these changes and the metabolic effects, and the roles played by these changes, after laparoscopic sleeve gastrectomy (LSG). METHODS: We recruited 25 Japanese patients with severe obesity, who underwent LSG. We measured two hepatokines: selenoprotein P (SeP) and leukocyte cell-derived chemotaxin 2 (LECT2), at the baseline, and then 6 months and 1 year after LSG. Finally, we compared the changes in the hepatokines with the parameters of type 2 diabetes (T2D) and non-alcoholic steatohepatitis (NASH). RESULTS: Changes in LECT2 were correlated with the percentage of total weight loss (ρ = - 0.499, P = 0.024) and the decrease in total fat area (ρ = 0.559, P = 0.003). The changes in SeP were correlated with those in hemoglobin A1c (ρ = 0.526, P = 0.043) and the insulinogenic index (ρ = 0.638, P = 0.010) in T2D patients. In patients with NASH, the LECT2 levels were correlated with liver steatosis (ρ = 0.601). CONCLUSIONS: SeP levels decrease in association with HbA1c reduction, whereas LECT2 levels are associated with reductions in fat mass and NASH scores after LSG. Hepatokines may be involved in the pathology of obesity and its complications.

Association of plasminogen activator inhibitor-1 and fibroblastic growth factor 21 in 3 groups of type 2 diabetes: Without overweight/obesity, free of insulin resistance, and without hepatosteatosis.

The physiological effects of fibroblast growth factor 21 (FGF21), leading to beneficial metabolic outcomes, have been extensively revealed in recent decades. Significantly elevated serum levels of FGF21 in obesity and type 2 diabetes mellitus (T2DM) are referred to as FGF21 resistance. However, Asian population tend to develop metabolic disorders at a lesser degree of obesity than those of Western. This study aimed to explore factors potentially related to serum FGF21 according to the severity of metabolic disorders in patients with T2DM. This cross-sectional study included 176 T2DM patients. The patients were categorized according to whether they had hepatic steatosis (fatty liver index [FLI] ≥ 60), insulin resistance (homeostasis model assessment of insulin resistance [HOMA-R] ≥ median), and/or overweight/obesity (body mass index [BMI] ≥ 25.0 kg/m2). Independent predictors of serum FGF21 were determined using multiple linear regression analysis in these 3 groups of T2DM patients. Circulating FGF21 levels were correlated positively with BMI, abdominal fat areas, leptin, and plasminogen activator inhibitor-1 (PAI-1). After adjustment for potential confounders, multiple linear regression analysis identified leptin as a factor strongly associated with serum FGF21 levels in all patients. Moreover, PAI-1 was a significant predictor of FGF21 in those with FLI < 60, BMI < 25.0 kg/m2, and HOMA-R < median, while leptin was the only independent factor in each of their counterparts. The factors related to serum FGF21 differ according to the severity of metabolic disorders. FGF21 appears to be independently associated with PAI-1 in T2DM patients: without overweight/obesity, those free of insulin resistance, and those without hepatic steatosis.

[Compartment Syndrome After Stanford Type A Acute Aortic Dissection Surgery:Report of a Case].

A 53-year-old man presented to the emergency department with chest and back pain. Contrast-enhanced computed tomography( CT) revealed a Stanford type A acute aortic dissection with a pseudo-lumen occlusion. On the same day, the patient underwent emergent aortic arch replacement with frozen elephant trunk. When introducing cardiopulmonary bypass, arterial cannula was inserted into the right femoral artery. The day after surgery, swelling of the right lower leg appeared with CK and intramuscular compartment pressure elevation. Thus, the patient was diagnosed with compartment syndrome and decompressive fasciotomy was performed. Although there was no preoperative blood flow disturbance in the lower extremities on preoperative CT, lower limbs ischemia happened. Necrotic muscles in his right leg required debridement, but amputation was not needed. The patient was discharged unaided utilising orthotics on the day 120. In muscular, young male patients, care should be taken in the method of blood delivery.

[Amyloidosis Diagnosed Following Aortic Valve Replacement:Report of a Case].

A 76-year-old man had been under observation for nephrotic syndrome. He suffered from hypotension and transient loss of consciousness. He was diagnosed with thoracic aortic aneurysm and severe aortic valve stenosis. Ascending aorta replacement concomitant with aortic valve replacement was performed uneventfully. He was diagnosed with light chain amyloidosis by pathological examination of the resected ascending aorta. He was received referral treatment for amyloidosis.

[Stanford Type A Acute Aortic Dissection Complicated by Right Lung Hemorrhage:Report of a Case].

A 59-year-old woman was transferred to our hospital because of a sudden onset of chest and back pain. Computed tomography (CT) demonstrated Stanford type A acute aortic dissection with cardiac tamponade and right airway bleeding. Hemorrhage from ruptured false lumen extended along the pulmonary artery (PA), compression of the right PA were recognized due to hematoma surrounding the PA. An emergency operation was performed. The primary tear was located at the distal aortic arch, and total arch replacement with frozen elephant trunk was performed. During the operation, she had airway bleeding. The bleeding was thought to be due to the hematoma extending along the pulmonary artery. She was extubated 7th postopratively. She was discharged 44 days after the operation.

[Very Late Stent Thrombosis during Perioperative Period of Aortic Valve Replacement:Report of a Case].

A 73-year-old man underwent drug eluting stent (DES) implantation in the left anterior descending artery (LAD) 11 years ago and in the right coronary artery (RCA) 8 years ago. He suffered from chest tightness and was diagnosed with severe aortic valve stenosis. Perioperative coronary angiography revealed no significant stenosis and thrombotic occlusion of the DES. Five days before operation, antiplatelet therapy was discontinued. Aortic valve replacement was performed uneventfully. But he developed chest pain and transient loss of consciousness, electrocardiographic changes were observed on the 8th postoperative day. Emergency coronary angiography revealed thrombotic occlusion of the drug eluting stent in the RCA, despite the postoperative oral adoministration of warfarin and aspirin. Percutaneous catheter intervention (PCI) restored the stent patency. Dual antiplatelet therapy (DAPT) was initiated immediately after the PCI, and anticoagulation therapy with warfarin was continued. Clinical symptons of stent thrombosis disappeared immediately after the PCI. He was discharged 7 days after the PCI.

Evaluation of High Intracranial Plaque Prevalence in Type 2 Diabetes Using Vessel Wall Imaging on 7 T Magnetic Resonance Imaging.

BACKGROUND: While type 2 diabetes (T2D) is a major risk for ischemic stroke, the associated vessel wall characteristics remain essentially unknown. This study aimed to clarify intracranial vascular changes on three-dimensional vessel wall imaging (3D-VWI) using fast spin echo by employing 7Tesla (7T) magnetic resonance imaging (MRI) in T2D patients without advanced atherosclerosis as compared to healthy controls. METHODS: In 48 T2D patients and 35 healthy controls, the prevalence of cerebral small vessel diseases and intracranial plaques were evaluated by 3D-VWI with 7T MRI. RESULTS: The prevalence rate of lacunar infarction was significantly higher in T2D than in controls (n = 8 in T2D vs. n = 0 in control, p = 0.011). The mean number of intracranial plaques in both anterior and posterior circulation of each subject was significantly larger in T2D than in controls (2.23 in T2D vs. 0.94 in control, p < 0.01). In T2D patients, gender was associated with the presence of intracranial plaques. CONCLUSION: This is the first study to demonstrate the high prevalence of intracranial plaque in T2D patients with neither confirmed atherosclerotic disease nor symptoms by performing intracranial 3D-VWI employing 7TMRI. Investigation of intracranial VWI with 7T MRI is expected to provide novel insights allowing early intensive risk management for prevention of ischemic stroke in T2D patients.

Adiponectin Paradox More Evident in Non-Obese Than in Obese Patients with Diabetic Microvascular Complications.

Aims/Introduction: Adiponectin is generally regarded as a beneficial molecule, protecting against insulin resistance and atherosclerosis, and its serum levels are low in individuals with obesity as well as in those with type 2 diabetes (T2DM). However, several clinical studies have shown associations between high adiponectin values and major health concerns. These conflicting findings are termed the "adiponectin paradox". Similarly, these paradoxical adiponectin elevations were observed in patients with diabetic microvascular complications. This cross-sectional study aimed to identify differences in factors, including adiponectin, related to diabetic vascular complications between non-obese and obese patients. Materials and Methods: Study patients with T2DM were non-obese (n=197) or obese (n=197), matched by a propensity score model adjusted with age and gender. Independent factors for each of the microvascular complications were determined using multivariate logistic regression analyses. Results: The prevalence of nephropathy was high in obese T2DM patients. In addition to long diabetes duration, elevated adiponectin was a common characteristic of patients with microvascular complications. Logistic regression analyses for microvascular complications revealed adiponectin to be highly related to retinopathy (odds ratio [OR], 1.138; 95%confidence intervals [CI], 1.004-1.289, p<0.001), nephropathy (OR, 1.192; CI, 1.077-1.319, p<0.001) and neuropathy (OR, 1.217; CI, 1.071-1.384, p<0.001), in non-obese patients. In contrast, the association between adiponectin values and complications was modest in obese patients. Conclusion: Adiponectin regulation in response to vascular damage differed between non-obese and obese patients, suggesting that adiponectin regulation is compromised by fat accumulation. Assuming that paradoxical elevation of adiponectin in vascular damage is a compensatory response, we speculate that responsive upregulation might be insufficient in obese patients. These newly-recognized differences in adiponectin values might lead to novel insights into adiponectin regulation and our understanding of the adiponectin paradox.

[Gallbladder Necrosis after Surgery for Stanford Type A Aortic Dissection in an Elderly Patient:Report of a Case].

An 86-year-old woman who suffered from cardiac tamponade due to acute Stanford type A aortic dissection was admitted to our hospital. An emergency operation was performed uneventfully. She suffered from abdominal pain 13 days after the operation. Computed tomography( CT) scan revealed pericholecystic fluid and unclear gallbladder wall, revealing acalculous necrotizing cholecystitis. We performed open cholecystectomy and abdominal cavity drainage. No gallstones were observed. She underwent intensive treatment. She was discharged without complications 44 days after the cholecystectomy.

[Hybrid Treatment of the Intrathoracic Right Subclavian Artery Aneurysm:Report of a Case].

A 78-years-old woman was referred to our institution for the treatment of right subclavian artery (SCA) aneurysm. She previously underwent total arch replacement via median sternotomy approach. Preoperative computed tomography revealed a 55 mm sized SCA aneurysm. Stent graft was inserted from brachiocephalic artery to right common carotid artery via the graft anastomosed. The orifice of the right SCA was covered with stent graft inserted into the right common carotid artery-brachiocephalic artery and the right SCA was occluded with coils distal to the aneurysm, carotid-SCA bypass was performed with 8 mm ePTFE graft. Postoperative examination confirmed complete exclusion of the aneurysm and patency of the bypass graft. We thought that hybrid treatment for this patient was a less invasive alternative to conventional surgical procedure.