[Anesthetic management in a patient with arrhythmogenic right ventricular cardiomyopathy and an implantable cardioverter defibrillator: a case report]. / Anestesia para paciente com cardiomiopatia arritmogênica do ventrículo direito portador de cardioversor desfibrilador implantável: descrição de caso.

BACKGROUND AND OBJECTIVES: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic cardiomyopathy characterized by potentially lethal ventricular tachycardia. Here we describe a patient with ARVC and an Implantable Cardioverter Defibrillator (ICD) in whom maxillary sinus surgery was performed under general anesthesia. CASE REPORT: The patient was a 59 year-old man who was scheduled to undergo maxillary sinus surgery under general anesthesia. He had been diagnosed as having ARVC 15 years earlier and had undergone implantation of an ICD in the same year. Electrocardiography showed an epsilon wave in leads II, aVR, and V1-V3. Cardiac function was within normal range on transthoracic echocardiography. The ICD was temporarily deactivated after the patient arrived in the operating room and an intravenous line was secured. An external defibrillator was kept on hand for immediate defibrillation if any electrocardiographic abnormality was detected. Remifentanil 0.3 µg/kg/min, fentanyl 0.1 mg, propofol 154 mg, and rocuronium 46 mg were administered for induction of anesthesia. Tracheal intubation was performed orally. Anesthesia was maintained oxygen 1.0 L.min-1, air 2.0 L.min-1, propofol 5.0-7.0 mg.kg-1.h-1, and remifentanil 0.1-0.25 µg.kg-1.min-1. The surgery was completed as scheduled and the ICD was reactivated. The patient was then extubated after administration of sugammadex 200 mg. CONCLUSION: We report the successful management of anesthesia without lethal arrhythmia in a patient with ARVC and an ICD. An adequate amount of analgesia should be administered during general anesthesia to maintain adequate anesthetic depth and to avoid stress and pain.

Anesthetic management in a patient with arrhythmogenic right ventricular cardiomyopathy and an implantable cardioverter defibrillator: a case report / Anestesia para paciente com cardiomiopatia arritmogênica do ventrículo direito portador de cardioversor desfibrilador implantável: descrição de caso

Abstract Background and objectives: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic cardiomyopathy characterized by potentially lethal ventricular tachycardia. Here we describe a patient with ARVC and an Implantable Cardioverter Defibrillator (ICD) in whom maxillary sinus surgery was performed under general anesthesia. Case report: The patient was a 59 year-old man who was scheduled to undergo maxillary sinus surgery under general anesthesia. He had been diagnosed as having ARVC 15 years earlier and had undergone implantation of an ICD in the same year. Electrocardiography showed an epsilon wave in leads II, aVR, and V1-V3. Cardiac function was within normal range on transthoracic echocardiography. The ICD was temporarily deactivated after the patient arrived in the operating room and an intravenous line was secured. An external defibrillator was kept on hand for immediate defibrillation if any electrocardiographic abnormality was detected. Remifentanil 0.3 µg/kg/min, fentanyl 0.1 mg, propofol 154 mg, and rocuronium 46 mg were administered for induction of anesthesia. Tracheal intubation was performed orally. Anesthesia was maintained oxygen 1.0 L.min−1, air 2.0 L.min−1, propofol 5.0-7.0 mg.kg−1.h−1, and remifentanil 0.1-0.25 µg.kg−1.min−1. The surgery was completed as scheduled and the ICD was reactivated. The patient was then extubated after administration of sugammadex 200 mg. Conclusion: We report the successful management of anesthesia without lethal arrhythmia in a patient with ARVC and an ICD. An adequate amount of analgesia should be administered during general anesthesia to maintain adequate anesthetic depth and to avoid stress and pain.

Resumo Introdução e objetivo: A Cardiomiopatia Arritmogênica do Ventrículo Direito (CAVD) é uma cardiomiopatia genética caracterizada por taquicardia ventricular potencialmente letal. Descrevemos um paciente com CAVD com Cardioversor Desfibrilador Implantável (CDI) submetido a anestesia geral para cirurgia de seio maxilar. Relato do caso: Paciente masculino, 59 anos, a ser submetido a anestesia geral para cirurgia de seio maxilar. O paciente foi diagnosticado com CAVD há 15 anos, momento em que foi submetido a implante de CDI. A eletrocardiografia mostrou onda épsilon nas derivações II, aVR e V1-V3. O ecocardiograma transtorácico revelou função cardíaca normal. Após a entrada do paciente na sala de cirurgia, o CDI foi temporariamente desativado e uma via intravenosa foi instalada. Um desfibrilador externo foi mantido próximo ao paciente caso fosse detectada alguma anormalidade eletrocardiográfica que indicasse desfibrilação do paciente. Foram administrados 0,3 mg/kg/min de remifentanil, 0,1 mg de fentanil, 154 mg de propofol e 46 mg de rocurônio para indução da anestesia. A intubação traqueal foi realizada por via oral. A anestesia foi mantida com 1 L/min de oxigênio, 2 L/min de ar, 5-7 mg/kg/h de propofol e 0,1-0,25 µg/kg/min de remifentanil. O procedimento cirúrgico proposto foi concluído e o CDI foi reativado. O tubo traqueal foi retirado após administração de 200 mg de sugamadex. Conclusão: Descrevemos técnica de anestesia bem sucedida sem arritmia letal em paciente com CAVD e CDI. Analgesia adequada deve ser administrada durante a anestesia geral para manter profundidade anestésica correta e evitar estresse e dor.

LDL uptake-dependent phosphatidylethanolamine translocation to the cell surface promotes fusion of osteoclast-like cells.

Osteoporosis is associated with vessel diseases attributed to hyperlipidemia, and bone resorption by multinucleated osteoclasts is related to lipid metabolism. In this study, we generated low-density lipoprotein receptor (LDLR)/lectin-like oxidized LDL receptor-1 (LOX-1, also known as Olr1) double knockout (dKO) mice. We found that, like LDLR single KO (sKO), LDLR/LOX-1 dKO impaired cell-cell fusion of osteoclast-like cells (OCLs). LDLR/LOX-1 dKO and LDLR sKO preosteoclasts exhibited decreased uptake of LDL. The cell surface cholesterol levels of both LDLR/LOX-1 dKO and LDLR sKO osteoclasts were lower than the levels of wild-type OCLs. Additionally, the amount of phosphatidylethanolamine (PE) on the cell surface was attenuated in LDLR/LOX-1 dKO and LDLR sKO preosteoclasts, whereas the PE distribution in wild-type OCLs was concentrated on the filopodia in contact with neighboring cells. Abrogation of the ATP binding cassette G1 (ABCG1) transporter, which transfers PE to the cell surface, caused decreased PE translocation to the cell surface and subsequent cell-cell fusion. The findings of this study indicate the involvement of a novel cascade (LDLR∼ABCG1∼PE translocation to cell surface∼cell-cell fusion) in multinucleation of OCLs.

Range-ambiguity artifact in abdominal ultrasound.

Range-ambiguity artifacts (RAAs) are an erroneous mapping of returning echoes into a composite picture. The purpose of this review was to illustrate the mechanism of RAAs and to present the diagnostic problems caused by RAAs. RAA features differ slightly from organ to organ. At the level of the urinary bladder, RAAs take the form of a cloud-like, ill-demarcated, immobile, echogenic area, and the depth of the echogenic area differs depending on the pulse repetition frequency (PRF). This form is referred to as "static RAA" in this review. There are two key ultrasound characteristics of RAAs at the level of the liver: (a) the depth of RAAs change according to the PRF, and (b) RAAs move in accordance with the cardiac cycle. This form is referred to as "mobile RAA" in this review. At the level of the gallbladder, RAAs take the form of fine echogenic lines in the gallbladder. This phenomenon is actually a combination of two phenomena: a ring-down artifact and RAA. This form is referred to as "complex RAA (searchlight phenomenon)" in this review. The easiest way to reduce RAAs is to change the image depth. Sufficient knowledge of RAAs can prevent misdiagnosis of erroneously displayed returning echoes as real pathologic changes.

Multinodular fatty change in the liver in three patients with chronic hepatic porphyria: Contribution of sonography to the diagnosis.

We present three cases of chronic hepatic porphyria (CHP) in alcoholic patients, in which grayscale ultrasound (US) revealed multiple echogenic masses in the liver, mimicking multinodular hepatocellular carcinoma on alcoholic liver injury. In all cases, contrast-enhanced US (CEUS) showed iso-enhancement of the mass lesions throughout all vascular phases. Additionally, two-dimensional shear wave elastography (2DSWE) (performed in two cases) revealed the mass to have almost the same SWE value as the surrounding parenchyma. When encountering alcoholic patients with multiple echogenic masses in the liver, CHP must be included in the differential diagnosis. CEUS and 2DSWE allow us to increase our diagnostic confidence of CHP.

Hepatocellular carcinoma in otherwise sonographically normal liver.

PURPOSE: Hepatocellular carcinoma (HCC) on normal liver is very rare. The goal of this study was to determine the clinical manifestations and the role of ultrasonography (US) in the diagnosis of HCC arising in normal liver. METHODS: The clinical data and US findings in 12 cases of surgically resected HCC in normal liver were retrospectively analyzed. RESULTS: The patients were asymptomatic, had no hepatocarcinogenic factor, and hepatic function tests were almost normal in most cases. HCCs were large, encapsulated, and solitary, and there were predominantly well-differentiated or moderately differentiated in most cases. US showed a hypoechoic rim and lateral shadowing, suggestive of peritumoral capsule formation, and on contrast-enhanced US (CEUS), the tumor was hyperenhanced in arterial phase and washed out in postvascular phase, revealing typical HCC findings. CONCLUSIONS: US raises suspicion of HCC by showing lateral shadowing on grayscale ultrasound and hypervascularity on CEUS of the lesion.

The polymethoxy flavonoid sudachitin suppresses inflammatory bone destruction by directly inhibiting osteoclastogenesis due to reduced ROS production and MAPK activation in osteoclast precursors.

Inflammatory bone diseases, including rheumatoid arthritis, periodontitis and peri-implantitis, are associated not only with the production of inflammatory cytokines but also with local oxidative status, which is defined by intracellular reactive oxygen species (ROS). Osteoclast differentiation has been reported to be related to increased intracellular ROS levels in osteoclast lineage cells. Sudachitin, which is a polymethoxyflavone derived from Citrus sudachi, possesses antioxidant properties and regulates various functions in mammalian cells. However, the effects of sudachitin on inflammatory bone destruction and osteoclastogenesis remain unknown. In calvaria inflamed by a local lipopolysaccharide (LPS) injection, inflammation-induced bone destruction and the accompanying elevated expression of osteoclastogenesis-related genes were reduced by the co-administration of sudachitin and LPS. Moreover, sudachitin inhibited osteoclast formation in cultures of isolated osteoblasts and osteoclast precursors. However, sudachitin rather increased the expression of receptor activator of NF-κB ligand (RANKL), which is an important molecule triggering osteoclast differentiation, and the mRNA ratio of RANKL/osteoprotegerin that is a decoy receptor for RANKL, in the isolated osteoblasts, suggesting the presence of additional target cells. When osteoclast formation was induced from osteoclast precursors derived from bone marrow cells in the presence of soluble RANKL and macrophage colony-stimulating factor, sudachitin inhibited osteoclastogenesis without influencing cell viability. Consistently, the expression of osteoclast differentiation-related molecules including c-fos, NFATc1, cathepsin K and osteoclast fusion proteins such as DC-STAMP and Atp6v0d2 was reduced by sudachitin. In addition, sudachitin decreased activation of MAPKs such as Erk and JNK and the ROS production evoked by RANKL in osteoclast lineage cells. Our findings suggest that sudachitin is a useful agent for the treatment of anti-inflammatory bone destruction.

Focal nodular hyperplasia: our experience of 53 Japanese cases.

PURPOSE: Contrast-enhanced ultrasound (CEUS) is a very sensitive diagnostic tool in characterizing liver tumors. It is especially useful in the diagnosis of focal nodular hyperplasia (FNH) of the liver. According to the previous reports, FNH is common in young women, and it is usually diagnosed by MRI. The majority of the previous reports come from European countries, and a very few studies of large series designed to describe the clinical features in Japanese patients have been reported. The aim of this study was to (a) describe the clinical features in 53 patients (59 lesions) diagnosed with CEUS and (b) compare the data with those from the previous reports. METHODS: The medical data from 53 patients diagnosed on the basis of typical CEUS findings at our institution and affiliated hospitals were reviewed, and their clinical data were analyzed. RESULTS: (1) The medical data from 53 cases showed a slight male predilection, with 30 male cases (57 %) and 23 female cases (43 %), although the occurrence in both sexes was equal. FNH cases were distributed throughout all generations in both sexes, mostly concentrated in the age of 30-60 years old, and metabolic cases were more common in men than in women (4 vs 0). (2) The lesions were small (mean: 23 mm) and distributed throughout the whole liver. (3) Lesion size was not influenced by age in either sex. (4) A rapid draining to the hepatic vein was recognized in five out of 59 lesions (8 %). CONCLUSIONS: Our data indicate that FNH occurs slightly more frequently in men than in women in Japan. It occurs also at any age in both sexes, but the mean lesion size was smaller in our series than in the previous reports. Metabolic disease was seen only in male FNH patients. A direct communication between the FNH lesion and the hepatic vein is diagnostically worth noting.

Lectin-like oxidized low-density lipoprotein receptor-1 abrogation causes resistance to inflammatory bone destruction in mice, despite promoting osteoclastogenesis in the steady state.

Inflammatory bone diseases have been attributed to increased bone resorption by augmented and activated bone-resorbing osteoclasts in response to inflammation. Although the production of diverse proinflammatory cytokines is induced at the inflamed sites, the inflammation also generates reactive oxygen species that modify many biological compounds, including lipids. Among the oxidized low-density lipoprotein (LDL) receptors, lectin-like oxidized LDL receptor-1 (LOX-1), which is a key molecule in the pathogenesis of multifactorial inflammatory atherosclerosis, was downregulated with osteoclast differentiation. Here, we demonstrate that LOX-1 negatively regulates osteoclast differentiation by basically suppressing the cell-cell fusion of preosteoclasts. The LOX-1-deleted (LOX-1(-/-)) mice consistently decreased the trabecular bone mass because of elevated bone resorption during the growing phase. In contrast, when the calvaria was inflamed by a local lipopolysaccharide-injection, the inflammation-induced bone destruction accompanied by the elevated expression of osteoclastogenesis-related genes was reduced by LOX-1 deficiency. Moreover, the expression of receptor activator of NF-κB ligand (RANKL), a trigger molecule for osteoclast differentiation, evoked by the inflammation was also abrogated in the LOX-1(-/-) mice. Osteoblasts, the major producers of RANKL, also expressed LOX-1 in response to proinflammatory agents, interleukin-1ß and prostaglandin E2. In the co-culture of LOX-1(-/-) osteoblasts and wild-type osteoclast precursors, the osteoclastogenesis induced by interleukin-1ß and prostaglandin E2 decreased; this process occurred in parallel with the downregulation of osteoblastic RANKL expression. Collectively, LOX-1 abrogation results in resistance to inflammatory bone destruction, despite promoting osteoclastogenesis in the steady state. Our findings indicate the novel involvement of LOX-1 in physiological bone homeostasis and inflammatory bone diseases.

Osteocytes produce interferon-ß as a negative regulator of osteoclastogenesis.

Osteoclastogenesis is controlled by osteocytes; osteocytic osteoclastogenesis regulatory molecules are largely unknown. We searched for such factors using newly developed culture methods. Our culture system mimics the three-dimensional cellular structure of bone, consisting of collagen gel-embedded osteocytic MLO-Y4 cells, stromal ST2 cells on the gel as bone lining cells, and bone marrow cells. The gel-embedded MLO-Y4 cells inhibited the osteoclastogenesis induced by 1,25(OH)2D3 without modulating receptor activator of NF-κB ligand (RANKL) and osteoprotegerin (OPG) production by ST2 cells, despite MLO-Y4 cells supported osteoclastogenesis in the absence of ST2 cells. In the bone marrow cell culture, the conditioned medium from MLO-Y4 cells decreased the capability of osteoclastic differentiation from the cells induced by macrophage colony-stimulating factor. This decreased capability was concomitant with an increase in protein kinase R mRNA expression and an inhibition of c-Fos translation. These changes were partially normalized by the simultaneous addition of an anti-interferon (IFN)-ß neutralizing antibody to MLO-Y4 cell conditioned medium. To study primary osteocytes, we prepared non-osteocytic cell-free osteocyte-enriched bone fragments (OEBFs). When osteoclast precursors were induced by macrophage colony-stimulating factor in the presence of OEBFs, the generated cells exhibited a diminished capacity for osteoclastogenesis. OEBFs prepared from OPG-knock-out mice exhibited a similar effect, indicating OPG-independent inhibition. The addition of anti-IFN-ß neutralizing antibody during the co-culture with OEBFs partially recovered the osteoclastogenic potential of the generated cells. The MLO-Y4 cells and OEBFs expressed IFN-ß mRNA. Although osteocytic RANKL is known to be important for osteoclastogenesis, our data suggest that osteocytes also produce IFN-ß as an inhibitor of osteoclastogenesis.

Pseudoaneurysm in a chronic pancreatitis patient: report of a case, with emphasis on contrast-enhanced sonograms.

The association between pseudoaneurysm of the splenic artery and pancreatitis is now established. Rupture of an aneurysm is a lethal condition, and early diagnosis and treatment are required to prevent this hazardous life-threatening complication. In our case, early detection of pseudoaneurysm of the splenic artery enabled us to start prompt embolization, which yielded good results. Splenic infarction is known to be an important and frequent complication of transarterial embolization of splenic artery aneurysms. Thus, when performing transarterial embolization of a splenic artery aneurysm, this complication must be kept in mind and it is absolutely necessary to confirm the presence or absence of this complication after embolization of the aneurysm. In our case of pseudoaneurysm of the splenic artery due to acute aggravation of chronic pancreatitis, contrast-enhanced ultrasonography confirmed the spleen to be free from infarction. Thus, this technique is strongly recommended in such instances.

Multiple anechoic hepatic nodules in a patient with malignant lymphoma: report of a case with an emphasis on B-mode sonograms.

We present a case of hepatic malignant lymphoma (ML) in which the hepatic ML nodules were imaged as round anechoic nodules with posterior echo enhancement, mimicking hepatic cysts on B-mode ultrasonography (US). However, the boundary echo between the nodules and the surrounding hepatic tissue seemed to be less distinct than that of a hepatic cyst. Contrast-enhanced US showed the nodules to be hypervascular, which ruled out the possibility of hepatic cysts. Our observation stresses the importance of boundary echo for the diagnosis of hepatic ML on B-mode US. We also offer a possible explanation for this phenomenon.

Portal tumor thrombus secondary to gastric carcinoma: report of a case with an emphasis on color Doppler ultrasound findings.

We report the case of a 67-year-old woman with portal tumor thrombus (PTT) associated with gastric carcinoma. Abdominal ultrasound (US) revealed a marked thickening of the gastric wall and a mass lesion in the splenic vein. Color Doppler US showed the intraportal mass to be blood flow-poor and revealed a gastroepiploic vein extending from the splenic hilum to the portal confluence, passing behind the peritoneum. These findings corresponded well with gastric carcinoma with PTT. Endoscopy confirmed the presence of an advanced type II carcinoma predominantly located in the upper body, which yielded histological results consistent with a well-differentiated adenocarcinoma. The patient's general condition deteriorated rapidly, and she died 2 months later. To the best of our knowledge, this is the first report describing the presence of a large gastroepiploic vein secondary to gastric carcinoma-associated PTT.