Eisenmenger's syndrome is defined as pulmonary hypertension with right-to-left or bidirectional shunting of blood through an intracardiac or aorto-pulmonary commumication. It can occur with complex congenital cardiac malformations, such as septal defect and patent ductus arteriosus. Parturients with Eisenmenger's syndrome are at high risk for peripartum morbidity and mortality. We experienced a case of parturient for cesarean section with Eisenmenger's syndrome and performed epidural anesthesia with fractionated doses of 2% lidocaine and fentanyl. The sensory block reached to T10 level and blood pressure was maintained with intravenous phenylephrine. After baby out, sudden hypotension with severe bradycardia developed and arterial oxygen saturation dropped. Immediate intubation and resuscitation was done, but cardiac rhythm disturbance, hypoxemia, and acidosis did not corrected. Cardiac standstill developed and stopped resuscitation. The neonate's Apgar score was 7 and he was transferred to pediatric ICU.
Acidosis ; Anesthesia, Epidural* ; Anoxia ; Apgar Score ; Blood Pressure ; Bradycardia ; Cesarean Section* ; Ductus Arteriosus, Patent ; Eisenmenger Complex* ; Female ; Fentanyl ; Hypertension, Pulmonary ; Hypotension ; Intubation ; Lidocaine ; Mortality ; Oxygen ; Peripartum Period ; Phenylephrine ; Pregnancy ; Resuscitation

No abstract available.
Niemann-Pick Disease, Type A*

Hallervorden-Spatz disease (HSD) is a rare autosomal-recessive hereditary disorder characterized by the early onset of progressive movement alterations, including dystonia, rigidity, choreoathetosis, and mental deterioration. HSD is also associated with a variety of psychiatric symptoms, primarily depression and mental deterioration. However, psychosis has rarely been reported as a major symptom of HSD. We report two siblings who presented psychiatric symptoms as major clinical presentations, accompanied by ataxic and spastic gait, dysarthria, and typical neuroimaging findings of HSD. A 14-year-old girl presented complex motor tics, stereotypic behavior and anxiety symptoms. Her older brother, a 16-year-old boy, presented prominent auditory hallucinations, persecutory delusions and social withdrawal symptoms. Psychiatric symptoms were improved after atypical antipsychotic treatment. HSD is a rare disease but should be carefully considered in the diagnosis of patients with both motor disorder and various psychiatric symptoms.
Adolescent ; Anxiety ; Delusions ; Depression ; Dysarthria ; Dystonia ; Gait Disorders, Neurologic ; Hallucinations ; Humans ; Neuroimaging ; Pantothenate Kinase-Associated Neurodegeneration ; Psychotic Disorders ; Rare Diseases ; Siblings ; Substance Withdrawal Syndrome ; Tics

BACKGROUND: Reactive oxygen species (ROS) induce lipid peroxidation and tissue damage in the endothelium. We tested the antioxidant effect of lidocaine and procaine on ROS-induced endothelial damage in the rabbit aorta. METHODS: Aortic rings isolated from rabbits were suspended in an organ bath filled with Krebs-Henseleit (K-H) solution bubbled with 5% CO2 and 95% O2 at 37.5degrees C. After precontraction with phenylephrine (PE, 10(-6) M), changes in tension were recorded following a cumulative administration of acetylcholine (ACh 3 x 10(-8) to 10(-6) M). Differences were measured as percentages of ACh-induced relaxation of aortic rings before and after exposure to ROS as generated by electrolysis of the K-H solution. The aortic rings were pretreated with lidocaine or procaine (10(-5) M to 3 x 10(-3) M) to compare their effects, as well as ROS scavengers, catalase, mannitol, sodium salicylate, and deferoxamine, and a catalase inhibitor, 3-amino-1,2,4-triazole (3AT). RESULTS: Lidocaine and procaine dose-dependently maintained endothelium-dependent relaxation induced by ACh despite ROS activity (P < 0.05 vs control value). The 3AT pretreated procaine (3 x 10(-3) M) group decreased more significantly than the un-pretreated procaine group (P < 0.05). CONCLUSIONS: These findings suggest that lidocaine and procaine dose-dependently preserve endothelium-dependent vasorelaxation against ROS attack, potentially via hydrogen peroxide scavenging.
Acetylcholine ; Amitrole ; Antioxidants ; Aorta ; Aorta, Abdominal ; Baths ; Catalase ; Deferoxamine ; Electrolysis ; Endothelium ; Hydrogen Peroxide ; Lidocaine ; Lipid Peroxidation ; Mannitol ; Oxygen ; Phenylephrine ; Procaine ; Rabbits ; Reactive Oxygen Species ; Relaxation ; Sodium Salicylate ; Vasodilation

The hypokalemic periodic paralysis is characterized by intermittent falccid paralysis of extremities with spontaneous recovery. It is rarely accompanied by cardiac arrhythmia, especially fatal ventricular tachycardia or torsades de pointes. We observed a 29 year old man, who had suffered from intermittent periodic paralysis and fatal ventricular tachyarrhythmia. He had the first episode of muscle weakness in his low grade of elementary school, which lasted for 20 -30 hours. Similar episodes of muscle weakness occurred 1 -7 times per year, especially after carbohydrate rich food. On admission to emergency room, his chief complaints were generalized weakness and chest tightness, serum potassium level was 1.6mEq/l, and four extremities showed Grade 0 motor weakness. His electrocardiography(ECG) showed Atrioventricular dissociation due to sinus tachycardia and accelerated junctional rhythm, intraventricular conduction distrubance. During intravenous potassium administration, ECG showed sustained ventricular tachycardia and cardiovascular collapse occurred. So we carried out resuscitation and cardioversion. After resuscitation, he recovered from cardovascular collapse and ECG showed sinus tachycardia. But during continuous monitoring ECG showed torsades de pointes with cardiovascular collapse. We carried out resuscitation and defibrillation repeatedly. Serum potassium level was 1.7 - 1.8mEq/L at that time. After successful resuscitation, ECG showed sinus rhythm, and his mental status was fully recovered. After he admitted to intensive care unit, paralytic attack and cardiac arrhythmia did not occurred any more. Serum potassium level was maintained between 3.9 -6.1lmEq/L during his hospital days. He was fully recovered but could not take any medications(e.g. acetazolamide, potassium supplying agent and antiarrhythmic drugs) due to severe gastrointestinal disturbances. During the 30 months of postdischarge period, he experienced three mild paralysis attacks, but they were not accompanied by chest tightness, palpitation or syncope.
Acetazolamide ; Adult ; Arrhythmias, Cardiac* ; Electric Countershock ; Electrocardiography ; Emergency Service, Hospital ; Extremities ; Heart Block ; Humans ; Hypokalemia ; Hypokalemic Periodic Paralysis* ; Intensive Care Units ; Muscle Weakness ; Paralysis ; Potassium ; Resuscitation ; Syncope ; Tachycardia ; Tachycardia, Sinus ; Tachycardia, Ventricular ; Thorax ; Torsades de Pointes

Cancer is the tissue complex consisted with heterogeneous cellular compositions, and microenvironmental cues. During the various stages of cancer initiation, development, and metastasis, cell–cell interactions as well as cell-extracellular matrix play major roles. Conventional cancer models both 2-dimensional and 3-dimensional (3D) present numerous limitations, which restrict their use as biomimetic models for drug screening and fundamental cancer biology studies. Recently, bioprinting biofabrication platform enables the creation of high-resolution 3D structures. Moreover this platform has been extensively used to model multiple organs and diseases, and this versatile technique has further found its creation of accurate models that figure out the complexity of the cancer microenvironment. In this review we will focus on cancer biology and limitations with current cancer models and we discuss vascular structures bioprinting that are critical to the construction of complex 3D cancer organoids. We finally conclude with current literature on bioprinting cancer models and propose future perspectives.
Biology ; Biomimetics ; Bioprinting* ; Cues ; Drug Evaluation, Preclinical ; Neoplasm Metastasis ; Organoids ; Tumor Microenvironment*

Tissue engineering is limited by our inability to adequately vascularize tissues post implantation because all tissue-engineered substitutes (with the exception of cornea and cartilage) require a vascular network to provide the nutrient and oxygen supply needed for their survival. This review gives a brief overview of the processes and factors involved in the vascularization and angiogenesis and summarizes the different strategies to overcome the issue of slow vascularization and angiogenesis in a range of tissue-engineered substitutes. Moreover, we will announce some potential future plans.
Cornea ; Methods* ; Oxygen ; Tissue Engineering

Mechanical complications of acute myocardial infarction which may lead to heart failure or shock include ruptute of left ventricular free wall, ventricular septum and papillary muscle. The clinical characteristics of these lesions vary conservative management alone has high mortality rate, for which reason surgical repair of these defects are essential. Structural defects including rupture of the left ventricular free wall, ventricular septum, and papillary muscle, accout for 5% to 20% of all deaths from acute myocardial infarction. Among these, ventricular septal defects occur in approximately 1% of all myocardial infarction, and account for up to 2% of deaths subsequent to myocardial infarction. Rupture of the ventricular septum following acute myocardial infarction(AMI) is associated with high mortality rate, as 54% of the patients succumb within two weeks, 87% within two months and 92.5% during the first year. We experienced two cases of postinfarct ventricular septal defects(VSD) which had been repaired within 1 week after AMI due to progressive deterioration of patients` conditions, and were to be reoperated because of repeated septal ruptures in postoperation period and development of cardiogenic shock.
Heart Failure ; Heart Septal Defects, Ventricular ; Humans ; Infarction* ; Mortality ; Myocardial Infarction ; Papillary Muscles ; Rupture ; Shock ; Shock, Cardiogenic ; Ventricular Septum

The aconite root has been used in oriental medicine to improve metabolism of debilitated patient and to cure acute dysuria, cardiac weakness, gout, neuralgias and rheumatism. The crude drug "bu-shi" or "cho-oh", which is obtained from the Aconitum roots, contains the potent poisons aconitine, mesaconitine, jesaconitine, and hypaconitine, which are C
Aconitine ; Aconitum* ; Adult ; Alkaloids ; Animal Experimentation ; Arrhythmias, Cardiac* ; Dizziness ; Dysuria ; Gout ; Hemodynamics ; Humans ; Medicine, East Asian Traditional ; Metabolism ; Nausea ; Neuralgia ; Poisons ; Rheumatic Diseases ; Shock* ; Tachycardia ; Tachycardia, Ventricular ; Torsades de Pointes ; Ventricular Fibrillation ; Vomiting

Three-dimensional (3D) printing is an additive manufacturing process by which precursor materials are deposited layer by layer to form complex 3D geometries from computer-aided designs, and bioprinting offers the ability to create 3D architecture living cells. Bioprinting methods have been developed rapidly pattern living cells, biological macromolecules, and biomaterials, and an advantage of the 3D microenviroment over traditional 2-dimensional cell culture is the ability to obtain more accurate and reliable data from model about tumor formation, progression, and response to anticancer therapies. This review focuses on recent advances in the use of biopriniting technologies for cancer research, bioprinting physiologically relevant testing platforms for anticancer drug development, and computational modeling for improvement bioprinting technique.
Biocompatible Materials ; Bioprinting ; Cell Culture Techniques ; Computer-Aided Design