Progressive muscular dystrophy (PMD) is a primary muscle disease characterized by progressive muscle weakness and wasting, which is inherited by an X-linked recessive pattern and occurs mainly in males. There are several types of muscular dystrophies classified according to the distribution of predominant muscle weakness including Duchenne and Becker, Emery-Dreifuss, facioscapulohumeral, oculopharyngeal, and limb-girdle type. Clinical manifestations of PMD are clumsy, unsteady gait, pneumonia, heart failure, pulmonary edema, hydropericardium, hydrothorax, aspiration, syncopal attacks, and sudden cardiac death. The deceased was a 34-year-old man, and the onset of the first clinical symptom, gait disturbance, was in his late teens. His elder brother had the same disease and experienced brain death after a head trauma and died after mechanical ventilation was discontinued. After an autopsy, we found contracture of the joints, pseudohypertrophy of the calf, wasting and fat replacement of the thigh muscle, pericardial effusion (80 mL), fibrosis and fat replacement of the cardiac ventricular wall, pulmonary edema, and froth in the bronchus. The cause of death was heart failure and dyspnea due to muscular dystrophy. There was no sign or suspicion of foul play in his death.
Adolescent ; Adult ; Autopsy ; Brain Death ; Bronchi ; Cause of Death ; Contracture ; Craniocerebral Trauma ; Death, Sudden, Cardiac ; Dyspnea ; Fibrosis ; Gait ; Gait Disorders, Neurologic ; Heart Failure ; Humans ; Hydrothorax ; Joints ; Male ; Muscle Weakness ; Muscular Dystrophies ; Pericardial Effusion ; Pneumonia ; Pulmonary Edema ; Respiration, Artificial ; Siblings ; Thigh

PURPOSE: Minimal change esophagitis (MCE) is a reflux disease without mucosal breaks, known to be partially associated with abnormal gastric motor function. Electrogastrography (EGG) is commonly applied to assess gastric motor function in a noninvasive fashion. We aimed to determine the relationship between MCE and gastric myoelectrical activity (GME) recorded on EGG in children. METHODS: We retrospectively assessed the records of 157 children without underlying disease who underwent both EGG and upper gastrointestinal endoscopy at Gachon University Gil Medical Center between January 2010 and June 2015. The children were stratified according to the appearance of the esophagus (normal vs. MCE). Between-group differences in EGG parameters and their correlation with each MCE finding were statistically analyzed. RESULTS: Only the power ratio, one of the EGG parameters analyzed, differed significantly between the two groups (MCE, 1.68±3.37 vs. normal, 0.76±1.06; p < 0.05), whereas the other parameters, such as dominant frequency, dominant power, and the ratio of abnormal rhythm, showed no differences. Among children with MCE, significant correlations were noted between erythema and power ratio (p < 0.05), friability and postprandial dominant frequency (p < 0.05), and edema and/or accentuation of mucosal folds and pre-prandial frequency (p < 0.05). Helicobacter pylori infection correlated with postprandial arrhythmia (MCE, 33.59±15.52 vs. normal, 28.10±17.23; p < 0.05). EGG parameters did not differ between children with normal esophagus and those with biopsy-proven chronic esophagitis. CONCLUSION: In children with MCE, gastric dysmotility may affect the development of MCE, manifesting as EGG abnormalities. H. pylori infection may also affect GME. However, larger prospective investigations are needed to confirm these findings.
Arrhythmias, Cardiac ; Child ; Edema ; Endoscopy ; Endoscopy, Gastrointestinal ; Erythema ; Esophagitis ; Esophagus ; Helicobacter pylori ; Humans ; Ovum ; Prospective Studies ; Retrospective Studies

OBJECTIVE: The beneficial effect of hypothermia after hemicraniectomy in malignant middle cerebral artery (MCA) infarction has been controversial. We aim to investigate the safety and clinical efficacy of hypothermia after hemicraniectomy in malignant MCA infarction.METHODS: From October 2012 to February 2016, 20 patients underwent hypothermia (Blanketrol III, Cincinnati Sub-Zero, Cincinnati, OH, USA) at 34°C after hemicraniectomy in malignant MCA infarction (hypothermia group). The indication of hypothermia included acute cerebral infarction >2/3 of MCA territory and a Glasgow coma scale (GCS) score < 11 with a midline shift >10 mm or transtentorial herniation sign (a fixed and dilated pupil). We retrospectively collected 27 patients, as the control group, who had undergone hemicraniectomy alone and simultaneously met the inclusion criteria of hypothermia between January 2010 and September 2012, before hypothermia was implemented as a treatment strategy in Dong-A University Hospital. We compared the mortality rate between the two groups and investigated hypothermia-related complications, such as postoperative bleeding, pneumonia, sepsis and arrhythmia.RESULTS: The age, preoperative infarct volume, GCS score, National institutes of Health Stroke Scale score, and degree of midline shift were not significantly different between the two groups. Of the 20 patients in the hypothermia group, 11 patients were induced with hypothermia immediately after hemicraniectomy and hypothermia was initiated in 9 patients after the decision of hypothermia during postoperative care. The duration of hypothermia was 4±2 days (range, 1 to 7 days). The side effects of hypothermia included two patients with arrhythmia, one with sepsis, one with pneumonia, and one with hypotension. Three cases of hypothermia were discontinued due to these side effects (one sepsis, one hypotension, and one bradycardia). The mortality rate of the hypothermia group was 15.0% and that of the control group was 40.7% (p=0.056). On the basis of the logistic regression analysis, hypothermia was considered to contribute to the decrease in mortality rate (odds ratio, 6.21; 95% confidence interval, 1.04 to 37.05; p=0.045).CONCLUSION: This study suggests that hypothermia after hemicraniectomy is a viable option when the progression of patients with malignant MCA infarction indicate poor prognosis.
Arrhythmias, Cardiac ; Brain Edema ; Cerebral Infarction ; Glasgow Coma Scale ; Hemorrhage ; Humans ; Hypotension ; Hypothermia ; Infarction ; Infarction, Middle Cerebral Artery ; Logistic Models ; Middle Cerebral Artery ; Mortality ; National Institutes of Health (U.S.) ; Pneumonia ; Postoperative Care ; Prognosis ; Retrospective Studies ; Sepsis ; Stroke ; Treatment Outcome

Epinephrine is used with lidocaine to provide bloodless surgical field and to prolong the action of local anesthetics. But epinephrine also has many adverse reactions, such as agitation, restlessness, headache, tachycardia, hypertension, and some significantly more dangerous conditions including myocardial ischemia, ventricular arrhythmia, cerebral hemorrhage and pulmonary edema, cardiac arrest, etc. We have experienced epinephrine-induced acute pulmonary edema due to submucosal intranasal application of epinephrine soaked gauze during septoplasty. The patient was successfully treated in intensive care unit with positive pressure ventilation, diuretics, and inotropic support. He was extubated after 5 hours and discharged after 7 days without any complications.
Anesthetics, Local ; Arrhythmias, Cardiac ; Cerebral Hemorrhage ; Dihydroergotamine ; Diuretics ; Epinephrine ; Headache ; Heart Arrest ; Humans ; Hypertension ; Intensive Care Units ; Lidocaine ; Myocardial Ischemia ; Positive-Pressure Respiration ; Psychomotor Agitation ; Pulmonary Edema* ; Tachycardia

OBJECTIVE: To show the effect of dual monitoring including cardiac output (CO) and intracranial pressure (ICP) monitoring for severe traumatic brain injury (TBI) patiens. We hypothesized that meticulous treatment using dual monitoring is effective to sustain maintain minimal intensive care unit (ICU) complications and maintain optimal ICP and cerebral perfusion pressure (CPP) for severe TBI patiens. METHODS: We included severe TBI, below Glasgow Coma Scale (GCS) 8 and head abbreviation injury scale (AIS) >4 and performed decompressive craniectomy at trauma ICU of our hospital. We collected the demographic data, head AIS, injury severity score (ISS), initial GCS, ICU stay, sedation duration, fluid therapy related complications, Glasgow Outcome Scale (GOS) at 3 months and variable parameters of ICP and CO monitor. RESULTS: Thirty patients with severe TBI were initially selected. Thirteen patients were excluded because 10 patients had fixed pupillary reflexes and 3 patients had uncontrolled ICP due to severe brain edema. Overall 17 patients had head AIS 5 except 2 patients and 10 patients (58.8%) had multiple traumas as mean ISS 29.1. Overall complication rate of the patients was 64.7%. Among the parameters of CO monitoring, high stroke volume variation is associated with fluid therapy related complications (p=0.043) and low cardiac contractibility is associated with these complications (p=0.009) statistically. CONCLUSION: Combined use of CO and ICP monitors in severe TBI patients who could be necessary to decompressive craniectomy and postoperative sedation is good alternative methods to maintain an adequate ICP and CPP and reduce fluid therapy related complications during postoperative ICU care.
Brain Edema ; Brain Injuries* ; Cardiac Output* ; Cerebrovascular Circulation* ; Decompressive Craniectomy ; Fluid Therapy ; Glasgow Coma Scale ; Glasgow Outcome Scale ; Head ; Humans ; Injury Severity Score ; Intensive Care Units ; Intracranial Pressure* ; Monitoring, Physiologic ; Multiple Trauma ; Reflex, Pupillary ; Stroke Volume

Laryngospasm, an occlusion of the glottis, can occur at any time during anesthesia, and is associated with serious perioperative complications such as hypoxia, hypercabia, aspiration, bronchospasm, arrhythmia, prolonged recovery, cardiac collapse, and eventually catastrophic death. Importantly, postoperative negative pressure pulmonary edema (NPPE) is a rare, but well described life-threatening complication related to acute and chronic upper airway obstruction. Sugammadex well known for affirmatively reducing the postoperative pulmonary complications associated with residual neuromuscular blockade may have an indirect role in triggering the negative intrathoracic pressure by raising a rapid and efficacious respiratory muscle strength in acute upper airway obstruction. Herein, we report a case of postoperative NPPE following repetitive laryngospasm even after reversal of rocuronium-induced neuromuscular blockade using sugammadex.
Airway Obstruction ; Anesthesia ; Anoxia ; Arrhythmias, Cardiac ; Bronchial Spasm ; Delayed Emergence from Anesthesia ; Glottis ; Laryngismus* ; Neuromuscular Blockade* ; Pulmonary Edema* ; Respiratory Muscles

Small-breed dogs (n = 168; weight < 15 kg) diagnosed with myxomatous mitral valve degeneration based on a routine clinical examination, radiology, electrocardiography, and echocardiography at the Seoul National University Veterinary Medical Teaching Hospital were included in this study. Survival periods were determined, and there were significant differences in survival rates among the three International Small Animal Cardiac Health Council classes. The mean follow-up period was 14.3 ± 12.1 months. Univariate analysis revealed that dyspnea, pulmonary edema, and vertebral heart score were significantly associated with survival time (p < 0.05). Additionally, age, left atrial-to-aortic root ratio, ejection fraction, and left ventricular end diastolic volume were associated with an increased risk of death (p < 0.1), while body weight, body condition score, systolic blood pressure, arrhythmia, syncope, fractional shortening, and end systolic volume were not associated with an increased risk of death. These results suggest that among the assessed variables dyspnea, pulmonary edema, and vertebral heart score could be useful prognostic factors for providing patient information to owners.
Animals ; Arrhythmias, Cardiac ; Blood Pressure ; Body Weight ; Dogs* ; Dyspnea ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Hospitals, Teaching ; Humans ; Mitral Valve* ; Prognosis ; Pulmonary Edema ; Retrospective Studies* ; Seoul ; Stroke Volume ; Survival Rate ; Syncope

BACKGROUND AND OBJECTIVES: Dilated cardiomyopathy can be the end-stage of severe cardiac disorders and directly affects the cardiac muscle, inducing cardiomegaly and heart failure (HF). Although a wide variety of animal models are available to study dilated cardiomyopathy, there is no model to assess dilated cardiomyopathy with non-invasive, simple, and large screening methods. METHODS: We developed a dilated cardiomyopathy model in zebrafish larvae using short duration terfenadine, a known cardiotoxic drug that induces ventricular size dilation. Fractional shortening of zebrafish hearts was calculated. RESULTS: We treated zebrafish with 5 to 10 µM terfenadine for 24 hours. In terfenadine-treated zebrafish, blood frequently pooled and clotted in the chamber, and circulation was remarkably reduced. Atria and ventricles were swollen, and fluid was deposited around the heart, mimicking edema. Cardiac contractility was significantly reduced, and ventricular area was significantly enlarged. Heart rate was markedly reduced even after terfenadine withdrawal. Acridine orange staining also showed that terfenadine increased cardiomyocyte apoptosis. A significant increase of natriuretic peptide B (NPPB) mRNA was found in terfenadine-treated zebrafish. A low dose of terfenadine (5–10 µM) did not show mortality in short-term treatment (24 hours). However, moderate dose (35–45 µM) terfenadine treatment reduced zebrafish survival within 1 hour. CONCLUSION: With advantages of rapid sample preparation procedure and transparent observation of the live heart, this model can potentially be applied to large-scale drug screening and toxicity assays for non-ischemic HF.
Acridine Orange ; Apoptosis ; Cardiomegaly ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Drug Evaluation, Preclinical ; Edema ; Heart ; Heart Failure ; Heart Rate ; Larva* ; Mass Screening ; Models, Animal ; Mortality ; Myocardium ; Myocytes, Cardiac ; RNA, Messenger ; Terfenadine* ; Zebrafish*

Acute myocardial infarction (AMI) is very rare in young adults. Nephrotic syndrome is an uncommon cause of AMI in young adults and is characterized by excessive proteinuria, hypoalbuminemia, hyperlipidemia, and generalized edema. The hypercoagulable state and accelerated atherosclerosis in patients with nephrotic syndrome may be associated with the development of AMI in young adults. We report herein a case of ST-segment elevation myocardial infarction with sudden cardiac arrest in a young patient with minimal change disease.
Atherosclerosis ; Death, Sudden, Cardiac ; Edema ; Humans ; Hyperlipidemias ; Hypoalbuminemia ; Myocardial Infarction* ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Proteinuria ; Shock, Cardiogenic* ; Young Adult*

A 75-year-old man was admitted to our hospital with sudden onset of vomiting and abdominal distension. The patient was taking medication for arrhythmia. Computed tomography showed stenosis of the ileum and a small bowel dilatation on the oral side from the region of stenosis. A transnasal ileus tube was placed. Enteroclysis using contrast medium revealed an approximately 6-cm afferent tubular stenosis 10 cm from the terminal ileum and thumbprinting in the proximal bowel. Transanal double-balloon enteroscopy showed a circumferential shallow ulcer with a smooth margin and edema of the surrounding mucosa. The stenosis was so extensive that we could not perform endoscopic balloon dilation therapy. During hospitalization, the patient's nutritional status deteriorated. In response, we surgically resected the region of stenosis. Histologic examination revealed disappearance of the mucosal layer and transmural ulceration with marked fibrosis, especially in the submucosal layer. Hemosiderin staining revealed sideroferous cells in the submucosal layers. Based on the pathologic findings, the patient was diagnosed with ischemic enteritis. The patient's postoperative course was uneventful.
Aged ; Arrhythmias, Cardiac ; Constriction, Pathologic* ; Dilatation ; Double-Balloon Enteroscopy ; Edema ; Enteritis* ; Fibrosis ; Hemosiderin ; Hospitalization ; Humans ; Ileum ; Ileus ; Intestines ; Ischemia ; Mucous Membrane ; Nutritional Status ; Ulcer ; Vomiting