Amplatzer Vascular Plug With Endologix AFX Lining for Visceral Re-Entry Closure in a Patient With Chronic Type B Aortic Dissection.

PURPOSE: To demonstrate the efficacy of a combination of Amplatzer vascular plug (AVP2) and Endologix AFX for a visceral entry closure. CASE REPORT: A 70-year-old woman with treated chronic type B aortic dissection was seen to have sac enlargement. An isolated residual tear was observed at the origin of the celiac artery. A 12 mm AVP2 was successfully deployed for the entry closure. Angiography after the plug deployment still demonstrated residual flow to the false lumen. Hence, an Endologix AFX VELA proximal endograft was deployed, covering the AVP membrane and leaving no endoleak. CONCLUSION: Deployment of a vascular plug with Endologix AFX lining was efficacious for securing complete closure of an entry tear at the origin of the celiac artery in our patient. CLINICAL IMPACT: Vascular plugs are reported to be effective for closing small entries, although their effectiveness is limited when the entry site is not located on the flat intima, or in a high-flow situation. In the presented case, an isolated entry tear at the origin of the celiac artery was successfully closed with a vascular plug following Endologix AFX stent-graft lining of the true lumen.

Directing a dispersion cannula tip toward the aortic root during thoracic aortic arch surgery does not adversely affect cardiac function.

INTRODUCTION: Neurologic complications of open thoracic aortic surgery are devastating problems in patients with severely diseased aortas. This study aimed to clarify whether directing the aortic cannula tip toward the aortic root affects the postoperative cardiac function in patients undergoing open thoracic aortic surgery. METHODS: A total of 16 patients who underwent total or partial arch replacement between January 2014 and April 2019 were enrolled and divided into two groups. Ascending aorta perfusion was performed by placing the cannula tip toward the aortic root (reversed direction group, seven patients) or toward the aortic arch (standard direction group, nine patients). Intraoperative and perioperative data, including mortality, morbidity, and postoperative cardiac function, were compared between the groups. RESULTS: There were no hospital deaths or stroke events in either group. The aortic cross-clamping time was 102.4 ± 20.3 minutes in the reversed direction group and 87.1 ± 9.9 minutes in the standard direction group (p = 0.049). Furthermore, the intubation time was 28.4 ± 12.9 hours in the reversed direction group and 12.4 ± 6.8 hours in the standard direction group (p = 0.022). Both times were significantly longer in the reverse direction group. Postoperative serum creatine kinase-MB levels were significantly lower in the reversed direction group (6.2 ± 3.3 U/L vs 13.3 ± 4.8 U/L, respectively, p = 0.006). The cardiac output and cardiac index did not significantly differ. CONCLUSIONS: Directing the aortic cannula tip toward the aortic root does not adversely affect the postoperative cardiac function after aortic arch surgery.

MACF1 Mutations Encoding Highly Conserved Zinc-Binding Residues of the GAR Domain Cause Defects in Neuronal Migration and Axon Guidance.

To date, mutations in 15 actin- or microtubule-associated genes have been associated with the cortical malformation lissencephaly and variable brainstem hypoplasia. During a multicenter review, we recognized a rare lissencephaly variant with a complex brainstem malformation in three unrelated children. We searched our large brain-malformation databases and found another five children with this malformation (as well as one with a less severe variant), analyzed available whole-exome or -genome sequencing data, and tested ciliogenesis in two affected individuals. The brain malformation comprised posterior predominant lissencephaly and midline crossing defects consisting of absent anterior commissure and a striking W-shaped brainstem malformation caused by small or absent pontine crossing fibers. We discovered heterozygous de novo missense variants or an in-frame deletion involving highly conserved zinc-binding residues within the GAR domain of MACF1 in the first eight subjects. We studied cilium formation and found a higher proportion of mutant cells with short cilia than of control cells with short cilia. A ninth child had similar lissencephaly but only subtle brainstem dysplasia associated with a heterozygous de novo missense variant in the spectrin repeat domain of MACF1. Thus, we report variants of the microtubule-binding GAR domain of MACF1 as the cause of a distinctive and most likely pathognomonic brain malformation. A gain-of-function or dominant-negative mechanism appears likely given that many heterozygous mutations leading to protein truncation are included in the ExAC Browser. However, three de novo variants in MACF1 have been observed in large schizophrenia cohorts.

Fully hydrogenated canola oil extends lifespan in stroke-prone spontaneously hypertensive rats.

BACKGROUND: Canola oil (Can) and several vegetable oils shorten the lifespan of stroke-prone spontaneously hypertensive rats (SHRSP). Although similar lifespan shortening has been reported for partially hydrogenated Can, the efficacy of fully hydrogenated oils on the lifespan remains unknown. The present study aimed to investigate the lifespan of SHRSP fed diets containing 10 % (w/w) of fully hydrogenated Can (FHCO) or other oils. METHODS: Survival test: Upon weaning, male SHRSP were fed a basal diet for rodents mixed with one of the test oils -i.e., FHCO, Can, lard (Lrd), and palm oil (Plm) throughout the experiment. The animals could freely access the diet and drinking water (water containing 1 % NaCl), and their body weight, food intake, and lifespan were recorded. Biochemical analysis test: Male SHRSP were fed a test diet with either FHCO, Can, or soybean oil (Soy) under the same condition, except to emphasize effects of fat, that no NaCl loading was applied. Soy was used as a fat source in the basal diet and was set the control group. Blood pressures was checked every 2 weeks, and serum fat levels and histological analyses of the brain and kidney were examined after 7 or 12 weeks of feeding. RESULTS: During the survival study period, the food consumption of FHCO-fed rats significantly increased (15-20 % w/w) compared with that of rats fed any other oil. However, the body weight gain in the FHCO group was significantly less (10-12 %) than that in the control group at 9-11 weeks old. The FHCO (> 180 days) intervention had the greatest effect on lifespan, followed by the Lrd (115 ± 6 days), Plm (101 ± 2 days), and Can (94 ± 3 days) diets. FHCO remarkably decreased the serum cholesterol level compared with Can and the systolic blood pressure from 12 to 16 weeks of age. In addition, while some rats in the Can group exhibited brain hemorrhaging and renal dysfunction at 16 weeks old, no symptoms were observed in the FHCO group. CONCLUSION: This current study suggests that complete hydrogenation decreases the toxicity of Can and even prolongs the lifespan in SHRSP.

Novel toxicity of tris(1,3-dichloro-2-propyl) phosphate in adult male rats.

The widespread use of tris(1,3-dichloro-2-propyl) phosphate (TDCIPP) as a flame retardant has led to its release to the environment. Thus, the toxicological effects of TDCIPP on humans and animals are of importance. For better understanding of its potential toxicities, TDCIPP (250, 500, or 650 mg/kg/day) or vehicle control was administrated orally to adult male Wistar-Imamichi rats for 7 days. After the final administration of compounds, organ weights, histopathology, blood biochemistry, and hematology were examined. Hepatic toxicity was observed at doses ≥ 500 mg/kg/day of TDCIPP, and renal toxicity was observed at 650 mg/kg/day. The anti-androgenic activity of TDCIPP was previously confirmed in vitro and in vivo, but weights of epididymis, an androgen-dependent organ, were not affected by TDCIPP treatment in adults. Serum alkaline phosphatase activity was significantly decreased in all TDCIPP-treated rats independent of dose. Hemoglobin concentration, hematocrit, red blood cell count, and reticulocyte count were decreased in all TDCIPP-treated rats, but mean corpuscular volume, total iron-binding capacity, and serum iron were normal, suggesting that renal anemia was caused by TDCIPP. Together with previous reports on effects of anti-androgenic substances on red blood cell indices, anemia caused by TDCIPP could be due to its anti-androgenic activity. These considerations will contribute to further assessment of the toxicity of the compound.

Method for Combined Observation of Serial Sections of Stented Arteries Embedded in Resin by Light Microscopy and Transmission Electron Microscopy.

We have developed a new method for obtaining information on whole tissues by light microscopy (LM) and ultrastructural features by transmission electron microscopy (TEM). This method uses serial sections of a stented artery embedded in resin. Stents were implanted in porcine coronary arteries in this study. The heart was perfusion fixed in a 2% paraformaldehyde and 1.25% glutaraldehyde mixed solution. The stented artery was then removed, fixed in 1% osmium, embedded in Quetol 651 resin, and sectioned serially. For LM, the black color of osmium was removed from the section by immersion in periodic acid and hydrogen peroxide after deplasticization. These sections were stained with hematoxylin and eosin and Elastica-Masson trichrome stain. For TEM, thin sections were re-embedded in Quetol 812 resin by the resupinate method and cut into ultrathin sections. A clear, fine structure was obtained, and organelles, microvilli, and cell junctions in the endothelium were easily observed. The combined observation of adjacent specimens by LM and TEM enabled us to relate histopathological changes in the millimeter scale to those in the nanometer scale.

Long-term prognosis of epilepsy in patients with cerebral palsy.

AIM: To assess the long-term natural course and prognosis of epilepsy in patients with cerebral palsy (CP). METHOD: We retrospectively collected data for 72 patients (36 males, 36 females) with CP who had epilepsy who visited our institutions between 1980 and 2015. The data from medical records, electroencephalography (EEG), and neuroimaging findings were reviewed. Time-to-event statistical analyses were performed to analyse the remission outcome and the Cox regression model was used for multivariate analyses. RESULTS: Median age at onset of epilepsy was 2 years 0 months, and 17 years 0 months at the latest follow-up. In total, 34 patients (47%, 0.043 per person-year) achieved seizure remission at a median age of 11 years 0 months. Favourable factors for seizure remission included older age, motor disability being able to roll over/crawl but not able to sit, intellectual disability with an IQ between 36 and 70, normal findings on neuroimaging, and CP type other than spastic quadriplegia. In multivariate analysis, spastic quadriplegia was found to be associated with continued seizure activity. Antiepileptic drugs could be discontinued without relapse in 10 patients at a median age of 16 years 6 months, occurring 11 years 6 months after the onset of epilepsy. The drugs were terminated if the patient was aged at least 10 years and had perinatal causative aetiology and normalization or amelioration of epileptiform discharges on EEG. INTERPRETATION: The remission rate of epilepsy in CP increases up to young adulthood, and termination of antiepileptic drugs can be considered in selected cases at older ages. WHAT THIS PAPER ADDS: The remission rate of epilepsy in cerebral palsy increases up to 20 years after onset. In some cases, antiepileptic drugs (AEDs) can be terminated without relapse. Older age, perinatal aetiology, and improvement on electroencephalography are favourable factors for terminating AEDs.

PRONÓSTICO A LARGO PLAZO DE LA EPILEPSIA EN PACIENTES CON PARÁLISIS CEREBRAL: OBJETIVO: Evaluar el curso natural y pronóstico a largo plazo de la epilepsia en niños con parálisis cerebral (PC). MÉTODO: Recopilamos retrospectivamente los datos de 72 pacientes (36 varones, 36 mujeres) con PC que habían tenido epilepsia y concurrieron a nuestras instituciones entre 1980 y 2015. Los datos de los registros médicos, electroencefalográficos (EEG) y los hallazgos de neuroimágenes fueron revisados. Se realizaron análisis estadísticos de tiempo - evento para analizar los resultados de la remisión y se utilizó el modelo de regresión de Cox para los análisis multivariados. RESULTADOS: La edad media del comienzo de la epilepsia fue a los 2 años, y el seguimiento más tardío fue a los 17 años. En total, 34 pacientes (47%, 0,0043 por persona / año) alcanzaron la remisión de las convulsiones a la edad media de los 11 años. Los factores favorables para la remisión de las convulsiones incluyeron mayor edad, la discapacidad motora que sea capaz de rolar, gatear aunque no sentarse, discapacidad intelectual con un CI entre 36 y 70, hallazgos normales en neuroimágenes y otro tipo de PC diferente de la cuadriparesia espástica. En el análisis multivariado, se encontró que la cuadriparesia espástica se asociaba con una actividad convulsiva constante. Las drogas antiepilépticas pudieron ser discontinuadas sin recaídas en 10 pacientes a la edad media de 16 años y 6 meses, 11 años y 6 meses después del comienzo de la epilepsia. Las drogas fueron suspendidas si el paciente tenía por lo menos10 años de edad, etiología perinatal y normalización y disminución de las descargas epilépticas en el EEG. INTERPRETACIÓN: La tasa de remisión de la epilepsia en la PC aumenta hasta la edad adulta temprana, y la terminación de los fármacos antiepilépticos puede considerarse en casos seleccionados a edades más avanzadas.

PROGNÓSTICO EM LONGO PRAZO DA EPILEPSIA EM PACIENTES COM PARALISIA CEREBRAL: OBJETIVO: Avaliar o curso natural em longo prazo e prognóstico da epilepsia em pacientes com paralisia cerebral (PC). MÉTODO: Coletamos retrospectivamente dados coletados para 72 pacientes (36 do sexo masculino, 36 do sexo feminino) com PC que tinham epilepsia e visitaram nossas instituições entre 1980 e 2015. Os dados dos prontuários médicos, eletroencefalografia (EEG), e achados de neuroimagem foram revisados. Análises estatísticas de tempo para o evento foram realizadas para o resultado de remissão e o modelo de regressão de Cox foi usado para análise multivariada. RESULTADOS: A idade mediana do início da epilepsia foi 2 anos e 0 meses, e 17 anos e 0 meses no último acompanhamento. No total, 34 pacientes (47%, 0,043 por pessoa-ano) apresentaram remissão das convulsões em uma idade mediana de 11 anos 0 meses. Fatores favoráveis para a remissão incluíram maior idade, deficiência motora sendo capaz de rolar/arrastar, mas não de sentar, deficiência intelectual com entre 36 e 70, achados normais de neuroimagem, e outros tipos de PC que não quadriplegia espástica. Na análise multivariada, a quadriplegia espástica foi associada com atividade convulsiva continuada. Drogas antiepilépticas puderam ser descontinuadas sem recidiva em 10 pacientes, em uma idade mediana de 16 anos e 6 meses, ocorrendo 11 anos e 6 meses após o início da epilepsia. Os medicamentos foram cessados se o paciente tinha ao menos 10 anos de idade e teve etiologia causadora perinatal e normalização ou melhora das descargas epileptiformes ao EEG. INTERPRETAÇÃO: A taxa de remissão da epilepsia em PC aumenta até a idade adulta jovem, e a cessação das drogas antiepilépticas pode ser considerada em casos selecionados de maior idade.

[Re-operation for Recurrent Constrictive Pericarditis with Multiple Organ Dysfunction].

The patient was a 64-year-old man with recurrent constrictive pericarditis which developed 12 years after the initial pericardiectomy. He had bilateral heart failure with severe left ventricular diastolic dysfunction, massive ascites, renal failure, and coagulopathy. Computed tomography showed a heavily calcified pericardium around the right atrium, the phrenic side of the right ventricle, and the left ventricle. He underwent pericardiectomy via median sternal re-entry. The calcified pericardium was safely decorticated with an ultrasonic surgical knife. The pericardium around the left ventricular side was safely decorticated under cardiopulmonary bypass and use of a heart positioner. Although permanent hemodialysis was necessary after the operation, he has been well for 6 years since the operation.

Endocrine disrupting chemicals, 4-nonylphenol, bisphenol A and butyl benzyl phthalate, impair metabolism of estradiol in male and female rats as assessed by levels of 15α-hydroxyestrogens and catechol estrogens in urine.

Bisphenol A (BPA), 4-nonylphenol (NP) and butyl benzyl phthalate (BBP), termed endocrine-disrupting chemicals, are known to mimic estrogen activity. The effects of these chemicals on 17ß-estradiol (E2 ) metabolism in vivo in rats were examined. Male and female rats were given NP (250 mg kg-1  day-1 ), BPA (250 µg kg-1  day-1 ) or BBP (500 mg kg-1  day-1 ) by gavage for 14 days, followed by a single intraperitoneal injection of E2 (5 mg kg-1 ) on the final day. The urinary excretion over 72 hours of 2-hydroxyestrone 1-N-acetylcysteine thioether, 2-hydroxyestrone 4-N-acetylcysteine thioether, 4-hydroxyestrone 2-N-acetylcysteine thioether, 2-hydroxy-17ß-estradiol (2-OHE2 ), 2-hydroxyestrone (2-OHE1 ), 4-hydroxy-17ß-estradiol, 4-hydroxyestrone, 15α-hydroxyestriol (E4 ), 15α-hydroxy-17ß-estradiol and 15α-hydroxyestrone was measured. Increases in urinary excretion of 2-OHE1 and decreases in E4 were observed in males treated with NP or BBP. Decreases in urinary excretion of 2-OHE2 and E4 were observed in males treated with BPA. Decreases in urinary excretion of 2-OHE1 and 2-OHE2 were observed in females treated with BBP. Normalized liver and weights were increased in both sexes treated with NP or BBP. Histologic observations revealed marked changes in the distal tubules and collecting ducts in the kidneys of rats exposed to NP and BBP, and hypertrophy in the hepatocytes of the centrilobular zone of the liver. No BPA-related effects on organ weight and on liver or kidney histopathology were found. These results suggest that the 14 day oral dosing of NP and BBP disrupted E2 metabolism, resulting from marked morphological and functional alterations in the liver and kidneys. In addition, BPA could induce metabolic and endocrine disruption.

Intravenous injection of artificial red cells and subsequent dye laser irradiation causes deep vessel impairment in an animal model of port-wine stain.

Our previous study proposed using artificial blood cells (hemoglobin vesicles, Hb-Vs) as photosensitizers in dye laser treatment for port-wine stains (PWSs). Dye laser photons are absorbed by red blood cells (RBCs) and hemoglobin (Hb) mixture, which potentially produce more heat and photocoagulation and effectively destroy endothelial cells. Hb-Vs combination therapy will improve clinical outcomes of dye laser treatment for PWSs because very small vessels do not contain sufficient RBCs and they are poor absorbers/heaters of lasers. In the present study, we analyzed the relationship between vessel depth from the skin surface and vessel distraction through dye laser irradiation following intravenous Hb-Vs injection using a chicken wattle model. Hb-Vs were administered and chicken wattles underwent high-energy irradiation at energy higher than in the previous experiments. Hb-Vs location in the vessel lumen was identified to explain its photosensitizer effect using human Hb immunostaining of the irradiated wattles. Laser irradiation with Hb-Vs can effectively destroy deep vessels in animal models. Hb-Vs tend to flow in the marginal zone of both small and large vessels. Increasing laser power combined with Hb-Vs injection contributed for deep vessel impairment because of the synergetic effect of both methods. Newly added Hb tended to flow near the target endothelial cells of the laser treatment. In Hb-Vs and RBC mixture, heat transfer to endothelial cells from absorbers/heater may increase. Hb-Vs function as photosensitizers to destroy deep vessels within a restricted distance that the photon can reach.

Effect of inflow cannula side-hole number on drainage flow characteristics: flow dynamic analysis using numerical simulation.

BACKGROUND: Venous drainage in cardiopulmonary bypass is a very important factor for safe cardiac surgery. However, the ideal shape of venous drainage cannula has not been determined. In the present study, we evaluated the effect of side-hole number under fixed total area and venous drainage flow to elucidate the effect of increasing the side-hole numbers. METHOD: Computed simulation of venous drainage was performed. Cannulas were divided into six models: an end-hole model (EH) and models containing four (4SH), six (6SH), eight (8SH), 10 (10SH) or 12 side-holes (12SH). Total orifice area of the side-holes was fixed to 120 mm2 on each side-hole cannula. The end-hole orifice area was 36.3 mm2. The total area of the side-holes was kept constant when the number of side-holes was increased. RESULT: The mean venous drainage flow rate of the EH, 4SH, 6SH, 8SH, 10SH and 12SH was 2.57, 2.52, 2.51, 2.50, 2.49, 2.41 L/min, respectively. The mean flow rate decreased in accordance with the increased number of side-holes. CONCLUSION: We speculate that flow separation at the most proximal site of the side-hole induces stagnation of flow and induces energy loss. This flow separation may hamper the main stream from the end-hole inlet, which is most effective with low shear stress. The EH cannula was associated with the best flow rate and flow profile. However, by increasing side-hole numbers, flow separation occurs on each side-hole, resulting in more energy loss than the EH cannula and flow rate reduction.

Treatment of self-injurious behaviors in Lesch-Nyhan syndrome with S-adenosylmethionine.

An 11-year-old boy with Lesch-Nyhan syndrome (LNS) had persistently injured himself by biting his lips and buccal mucosa since infancy. Risperidone was only partially effective in suppressing this behavior. Oral administration of S-adenosylmethionine (SAMe), involving increasing the dose from 400 mg to 1 g, resulted in the amelioration of self-injurious behavior and anxiety as well as marked improvement in his self-esteem, performance at school, and friendships. No adverse effects were noted. SAMe may have a favorable effect on symptoms of LNS by activating monoaminergic pathways and/or increasing the adenosine pool in the salvage pathway of guanosine monophosphate synthesis. Defects in these pathways have been essentially implicated in the neurological pathophysiology of LNS.

[Clinical characteristics of early juvenile GM2 gangliosidosis: a case report].

We describe the case of a 15-year-old male with early juvenile type GM2 gangliosidosis. He first manifested with progressive clumsiness in his extremities at the age of 1.5 years, followed by motor regression. Intellectual disability became evident as late as age 6 years. This discrepancy along with rapid motor deterioration after varicella infection, lack of startle response or macrocephaly, and paucity of myoclonus were thought to be characteristic of juvenile GM2 gangliosidosis. In contrast to the cerebellar atrophy as the initial finding in usual juvenile GM2 gangliosidosis, magnetic resonance imaging revealed initially cerebral, and subsequently cerebellar, progressive atrophy. Autistic behavioral problems, including phonophobia, during intellectual regression in this patient was also unusual in juvenile GM2 gangliosidosis. Thus, recognition of these features would prompt proper diagnosis and insights into the pathomechanisms of GM2 gangliosidosis.

Stress-reactive rats (high-avoidance female rats) have a shorter lifespan than stress-nonreactive rats (low-avoidance female rats).

Although Hatano high-avoidance and low-avoidance rats (HAA and LAA, respectively) have been selectively bred for good versus poor avoidance learning, HAA rats are known to be more reactive to stress than LAA rats. In this study, HAA and LAA female rats were compared during reproductive aging by observing estrous cycles from 8 to 11 months of age. Furthermore, these rats were allowed to live out their natural lifespans, that is, until 24 months of age, in order to compare their survival and to clarify the relationship between reproductive aging and tumor development. At eight months of age, 2 of 35 HAA rats and 20 of 35 LAA rats had abnormal estrous cycles. The median lifespan of the HAA rats (673 days) was shorter than that of the LAA rats (733 days). The incidence of pituitary neoplasia was higher in the HAA rats than in the LAA rats. These results suggest that HAA female rats (i.e., stress-reactive rats) have a shorter lifespan than LAA female rats (i.e., stress-nonreactive rats) and develop pituitary neoplasia, which was one of the causal factors in their accelerated mortality. However, the onset of an age-matched abnormal cycle did not correspond with their lifespan.

A family of distal arthrogryposis type 5 due to a novel PIEZO2 mutation.

Distal arthrogryposis (DA) encompasses a heterogeneous group of hereditary disorders with multiple congenital contractures predominant in the distal extremities. A total of 10 subtypes are proposed based on the pattern of contractures and association with extraarticular symptoms. DA5 is defined as a subtype with ptosis/oculomotor limitation. However, affected individuals have a variety of non-ocular features as well. We report on a two-generation family, including four affected individuals who all had congenital contractures of the distal joints, ptosis, restricted ocular movements, distinct facial appearance with deep-set eyes, and shortening of the 1st and 5th toes. The proband and her affected mother had restrictive lung disease, a recently recognized syndromic component of DA5, while younger patients did not. The proband had metacarpal and metatarsal synostosis, and the mother showed excavation of the optic disk. Whole-exome sequencing revealed a novel heterozygous mutation c.4456G>C (p.A1486P) of PIEZO2. PIEZO2 encodes a mechanosensitive ion channel, malfunction of which provides pleiotropic effects on joints, ocular muscles, lung function, and bone development.

Cardiac supporting device using artificial rubber muscle: preliminary study to active dynamic cardiomyoplasty.

Dynamic cardiomyoplasty is a surgical treatment that utilizes the patient's skeletal muscle to support circulation. To overcome the limitations of autologous skeletal muscles in dynamic cardiomyoplasty, we studied the use of a wrapped-type cardiac supporting device using pneumatic muscles. Four straight rubber muscles (Fluidic Muscle, FESTO, Esslingen, Germany) were used and connected to pressure sensors, solenoid valves, a controller and an air compressor. The driving force was compressed air. A proportional-integral-derivative system was employed to control the device movement. An overflow-type mock circulation system was used to analyze the power and the controllability of this new device. The device worked powerfully with pumped flow against afterload of 88 mmHg, and the beating rate and contraction/dilatation time were properly controlled using simple software. Maximum pressure inside the ventricle and maximum output were 187 mmHg and 546.5 ml/min, respectively, in the setting of 50 beats per minute, a contraction/dilatation ratio of 1:2, a preload of 18 mmHg, and an afterload of 88 mmHg. By changing proportional gain, contraction speed could be modulated. This study showed the efficacy and feasibility of a pneumatic muscle for use in a cardiac supporting device.

Short-lasting unilateral neuralgiform headache attacks with ispilateral facial flushing is a new variant of paroxysmal extreme pain disorder.

BACKGROUND: We encountered a 5-year-old girl who had short-lasting, severe, unilateral temporal headaches with ipsilateral lacrimation, nasal congestion and rhinorrhoea, and facial flushing after severe attacks. Family history revealed similar short-lasting, severe headaches in an older brother, younger sister, mother, maternal aunt, and maternal grandfather's brother. METHODS: We performed routine laboratory examinations and electrophysiological and radiological studies for three children, and whole-exome sequencing to determine the genetic causality in this family. RESULTS: Focal hyperperfusion of the right trigeminal root entry zone was seen during a right-sided attack in one child, while left-sided temporal headache attacks were provoked by bilateral electrical stimulation of the upper extremities in another. We identified a novel SCN9A mutation (NM_002977: c.5218G>C, p.Val1740Leu) in all affected family members, but not in any of the unaffected members. SCN9A encodes the voltage-gated sodium-channel type IX alpha subunit known as Na(v)1.7. CONCLUSIONS: Gain-of-function mutations in Na(v)1.7 are well known to cause paroxysmal extreme pain disorder (PEPD), a painful Na-channelopathy characterized by attacks of excruciating deep burning pain in the rectal, ocular, or jaw areas. The SCN9A mutation suggests that our patients had a phenotype of PEPD with a predominant symptom of short-lasting, severe, unilateral headache.

Trehalose solution protects mesothelium and reduces bowel adhesions.

BACKGROUND: Preventing interbowel adhesions still remains a challenge. Peritoneal mesothelial damage can induce postoperative adhesions. Our study evaluated the effects of 3% trehalose solution on mesothelial protection and adhesion prevention. Also, we compared this novel solution with Seprafilm regarding efficacy. METHODS: Mesothelial damage was induced on the cultured human mesothelial cell (Met-5A) and rabbit cecum-serosal surface by air-drying for 60 min, and trehalose solution was applied. Cell integrity was tested by measuring lactate dehydrogenase, and serosal-morphologic changes were analyzed using scanning electron microscopy. Intra-abdominal adhesions were induced in rabbits by the combination of abrasion and air-drying procedures. Animals were divided into four groups: control, 3% trehalose solution, Seprafilm, and 3% trehalose solution with Seprafilm. Adhesions were evaluated blindly 7 d later. RESULTS: Lactate dehydrogenase release from the Met-5A cells was reduced dose-dependently by trehalose (P < 0.05). Morphologic studies clearly showed that mesothelial cells on the serosal surface were kept intact by 3% trehalose solution. In a rabbit adhesion model, 3% trehalose solution reduced adhesions between bowel and bowel or bowel and surrounding structures (P < 0.01 versus control and Seprafilm). Seprafilm reduced adhesions between abdominal wall and underlying viscera (P < 0.01 versus control and 3% trehalose solution). Three-percent trehalose solution with Seprafilm showed additive effects of adhesion prevention, reducing adhesion formation at the previously mentioned sites. CONCLUSIONS: Three-percent trehalose solution protects mesothelial cells and leads to reduced adhesions between bowel and bowel or bowel and surrounding structures. This effect seems to be resulted from the characteristics of the solution covering most areas that potentially develop adhesions.

Bilateral axillary arterial aneurysms in siblings with Marfan syndrome.

Axillary arterial aneurysm associated with Marfan syndrome is an extremely rare and dangerous disease because of its potential risk for rupture and impairment of neurologic function in the upper extremities. Treatment of large axillary aneurysms can be surgically challenging because the axillary artery is anatomically located under the clavicle bones and the brachial plexus runs dorsal to the aneurysm. We report 2 cases of bilateral axillary arterial aneurysms in brothers with Marfan syndrome. Revival of a novel approach and cautious attempts during the procedures are described in both cases.

Hybrid operation for combined aortic arch aneurysm and aortic root dilation in a Jehovah's witness patient.

Aortic surgery for Jehovah's Witness patients with extended aneurysms is challenging. The present report describes a case of a 65-year-old male Jehovah's Witness with aortic aneurysm that extended from the ascending to the transverse aortic arch. Two-stage hybrid operation was performed as follows: ascending aortic replacement with debranching of brachiocephalic artery, followed by extra-anatomic bypass of cervical arteries, and thoracic aortic stent-grafting. The patient fully recovered without complication, and the lowest hemoglobin concentration during the hospital admission was 9.5 g/dL. We conclude that the hybrid procedure was effective in securing a margin of safety for bloodless aortic surgery.