Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0032290 (aspiration pneumonia)
 2,291 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myotonic dystrophy or Steinert's disease may be discovered during acute respiratory failure, sometimes caused by a general anaesthetic. It complicates chronic respiratory failure which is present in almost all cases, both restrictive and obstructive, the clinical signs of which progress with the myopathy. Apart from myotonic degeneration of the respiratory muscles, a hypoventilation syndrome of central origin has been described, but the etiology of this respiratory failure is dominated by repeated aspiration pneumonia favoured by constant dysphagia and passage of food into the trachea and poor cough reflex. One should attempt to correct this.
 ...
PMID:[Myotonic dystrophy and acute respiratory insufficiency]. 19 96

Cardiopulmonary abnormalities are frequently encountered in myotonic dystrophy. We present five patients with myotonic dystrophy who entered the intensive care unit in acute respiratory failure. The possible etiologic factors of pulmonary complications are reviewed. The most important is probably aspiration pneumonia. The difficulties in the treatment of the respiratory failure are emphasezed. Myotonia of the chest muscles and diaphragm make artificial ventilation difficult. Recovery is delayed chiefly by swallowing disturbances. General anaesthesia is hazardous. Four patients presented cardiac arrhythmias and/or conduction abnormalities which were transient.
 ...
PMID:Respiratory failure and cardiac disturbances in myotonic dystrophy. 96 70

Myotonic dystrophy (MyD) involves a variety of systems. Respiratory disorders are common, namely elevation of diaphragm, alveolar hypoventilation, aspiration pneumonia and sleep apnea. We evaluated respiratory involvement. The subjects were 11 patients with MyD. Also 6 patients with limb girdle muscular dystrophy (LG) were examined to be compared with MyD. Both groups had the similar activities of daily living. All of them never complained of dyspnea. Arterial blood gas studies were performed in supine position and standing position. A new evidence was found that hypoxemia was aggravated and alveolar-arterial oxygen pressure difference was increased in supine position in MyD. Next, pulmonary function tests were done in supine position and sitting position. Functional residual capacity (FRC) were more reduced in supine position in MyD compared with LG. The value to subtract closing capacity from FRC was negative in supine position in MyD, showing closing phenomenon. We propose the mechanism of the aggravation of hypoxemia may be the following. The reduction of FRC caused by respiratory muscle involvement brings out the closing phenomenon. Abnormal uneven distribution of ventilation-perfusion ratio happens and then hypoxemia is worsened in supine position in MyD.
 ...
PMID:[Aggravation of hypoxemia in supine position in myotonic dystrophy]. 129 47

We report a case of congenital myotonic dystrophy (CMD) in which not only the mother but also the paternal family is affected by myotonic dystrophy (DM). Clinical symptoms consisted of poor spontaneous movements, typical facial appearance, respiratory insufficiency attributable to diaphragmatic weakness, feeding difficulties due to impaired gastrointestinal tract motility and poor sucking, joint contractures and thin ribs. She died at 7 month of age, still ventilated, from aspiration pneumonia. By employing molecular genetic methods we were able to show that the affected child was not homozygous for the DM gene.
 ...
PMID:[Direct genotype analysis in congenital myotonic dystrophy with an unusual family anamnesis]. 841 5

Although the genetic basis of myotonic dystrophy (MD) has recently been clarified, data on prognosis and causes of death are few. To assess the relation between the age of onset and the degree to which activities of daily living (ADL) were affected, we studied statistically 586 patients with MD, who were registered in the database in hospitals of Japan in 1985. Many of the young onset patients had a long duration of illness, and many of the long-term patients showed the more severely affected ADL. When we adjusted the duration of illness, using the Mantel extension method, many patients whose age of onset was older had poorer ADL than the younger onset ones. To clarify the causes of death in MD, we collected data on 72 patients who succumbed to it from answers to questionnaires sent to all councilors of the Japanese Society of Neurology and the Japanese Society of Child Neurology in 1994. Before their deaths, among 32 MD cases unable to sit up, 16 (50%) died from respiratory involvement (respiratory failure and pneumonia), 8 (25%) from dysphagia (i.e., aspiration pneumonia and choking) and 2 (6%) from cardiac involvement. Moreover, among 25 cases that could sit up, 9 cases (36%) died from dysphagia, 4 (15%) from respiratory involvement and 2 (8%) from cardiac involvement. Dysphagia and cardiac involvement are often the causes of death in the early stage. To avoid these complications, management of swallowing and careful cardiac control is essential in patients with MD.
 ...
PMID:[Prognosis for myotonic dystrophy]. 875 40

Myotonic dystrophy is an autosomal dominant inherited disease of the skeletal and cardiac musculature that involves the pharyngeal and gastrointestinal smooth and striated muscles, resulting in velopharyngeal insufficiency, Swallowing difficulties, gastrointestinal motility disorders and anal incontinence. Gastrointestinal symptoms are found in a large proportion of patients suffering from this disease and may herald the onset of muscular disorders, in rare cases they are even the predominant feature of the disorder. We report on a 31-years-old patient with formerly undiagnosed myotonic dystrophy in combination with a non-rotation of the intestinal tract, an association of disorders that to our knowledge never has been reported before. Our patient was admitted as an emergency with signs of an acute abdomen with ileus, associated with acute aspiration pneumonia. Surgical intervention was avoided once the diagnosis of myotonic dystrophy had been confirmed and the patient was treated successfully by conservative therapy. A review of the literature indicates that conservative treatment of motility disorders of the bowel in patients with myotonic dystrophy is to be recommended.
 ...
PMID:Intestinal non-rotation and pseudoobstruction in myotonic dystrophy: case report and review of the literature. 891 34

A 56-year-old woman with a 23 year-history of myotonic dystrophy was admitted to our hospital because of dysphagia and aspiration pneumonia. On admission, patient's ECG showed Ist degree of atrioventricular block and elongation of QRS (133 msec.). On the third hospital day, abnormal Q wave abruptly appeared in aVL associated with elevation of ST segment in V2 and V3, reverse T wave in I, II, III, aVF and V 2-6 leads on ECG. Echo cardiogram demonstrated asynergy at the apex and septal wall. Coronary angiography was normal. Left ventricular ejection fraction determined by left ventriculography decreased to 47.4%. Based on these findings, we thought "stunned myocardium" had developed. Biopsied myocardium from the apex, septum, and free wall showed non-specific findings including mild fibrosis and lymphocytic infiltration. Although overt myocardial disease is rare in myotonic dystrophy, myocardiac disease such as "stunned myocardium" not due to arrhythmia or coronary angiopathy may develop under severe infection, stress, and/or respiratory distress.
 ...
PMID:["Stunned myocardium" on echo cardiogram in myotonic dystrophy: a case report]. 1235 86

The object of this review is to provide the definitions and criteria for diabetic ketoacidosis (DKA) and the hyperglycemic hyperosmolar state (HHS), and convey current knowledge of the causes of permanent disability or mortality from complications of these conditions, of the risk factors for DKA and HHS, and of early indicators and contemporary treatment of suspected cerebral edema. The frequency of DKA at onset of type 1 diabetes mellitus (DM1) varies from 10-70%, depending on availability of health care and frequency of diabetes. At the onset of type 2 diabetes (DM2), DKA occurs in 5-52%. One study reported HHS in approximately 4% of new patients with DM2. Recurrent DKA rates are equally dependent on variability in medical services and socio-economic circumstances, and are estimated to be eight episodes per 100 patient years, with 20% of patients accounting for 80% of the episodes. Mortality for each episode of DKA internationally varies from 0.15-0.31%, with idiopathic cerebral edema accounting for two-thirds or more of this mortality. Other causes of death or disability include untreated DKA or HHS, hypokalemia, hypophosphatemia, hypoglycemia, other intracerebral complications, peripheral venous thrombosis, mucormycosis, rhabdomyolysis, acute pancreatitis, acute renal failure, sepsis, aspiration pneumonia, and other pulmonary complications. Population-based studies from the UK, Australia, the USA, and Canada report cerebral edema incidence in DKA of 0.5-2.0%. Published information does not support the notion that treatment factors are causal in cerebral edema. Younger age, greater severity of acidosis, degree of hypocapnia, and severity of dehydration have been suggested as risk factors in several studies. Bimodal distribution of the time of onset of cerebral edema and wide variation in brain imaging findings suggest the variability and likely multiple causation of the clinical picture. Functional brain scanning has indicated that DKA is accompanied by increased cerebral blood flow suggesting that the predominant mechanism of edema formation is a vasogenic process. A method of monitoring for diagnostic and major and minor signs of cerebral edema has been proposed and tested which indicates that intervention will be required in five individuals to provide early intervention for a single case of cerebral edema. The preferred intervention of mannitol infusion has typically been accompanied by intubation and hyperventilation, but recent evidence indicates outcome is adversely affected by aggressive hyperventilation. The prevention of DKA and HHS at the onset of diabetes mellitus requires a high degree of awareness and suspicion by primary care providers; prevention of recurrent DKA necessitates a diligent team effort.
 ...
PMID:Hyperglycemic crises and their complications in children. 1731 23

The phenotype of myotonic dystrophy type 2 (DM2) shows similarities as well as differences to that of myotonic dystrophy type 1 (DM1). Dysphagia, a predominant feature in DM1, has not yet been examined in DM2. In a recent nationwide questionnaire survey of gastrointestinal symptoms in DM2, 12 out of 29 DM2 patients reported to have difficulty in swallowing for solid food. The aim of the study was to investigate the presence of dysphagia in patients with genetically proven DM2 who reported difficulty in swallowing for solid food at the questionnaire survey. Swallowing function and fiberoptic endoscopic evaluation of swallowing (FEES) were examined by a speech therapist and otorhinolaryngologist, respectively. In DM2 patients who reported difficulty in swallowing the presence of dysphagia could be confirmed (clinically in 100%, by FEES in 88%). A correlation exists between Dysphagia Outcome and Severity Score (DOSS) and age (p=0.05). None of the patients was underweight, and none of the patients had suffered aspiration pneumonia in the past. Dysphagia is present among DM2 patients and is more severe in older patients. However, dysphagia is generally mild, and do not lead to weight loss, or aspiration pneumonia.
 ...
PMID:Dysphagia is present but mild in myotonic dystrophy type 2. 1916 24

Oropharyngeal dysphagia and esophageal motility disorders were found to be the most important causes of aspiration pneumonia in patients with myotonic dystrophy. The purpose of this report was to evaluate clinical characteristics of the oral motor movements and swallowing of individuals with myotonic dystrophy type 1 (DM1) using a standardized clinical protocol and surface electromyography (sEMG). Participants were 40 individuals divided in two groups: G1 composed of 20 adults with DM1 and G2 composed of 20 healthy volunteers paired by age and gender to the individuals in G1. Statistical analysis included one-way ANOVA with two factors for within- and between-group comparisons and Bonferroni correction for multiple comparisons. Patients with DM1 presented deficits in posture, position, and mobility of the oral motor structures, as well as compromised mastication and deglutition. The sEMG data indicated that these patients had longer muscle activations during swallowing events. The longer duration of sEMG in the group of patients with DM1 is possibly related to myotonia and/or incoordination of the muscles involved in the swallowing process or could reflect a physiological adaptation for safe swallowing.
 ...
PMID:Oral motor movements and swallowing in patients with myotonic dystrophy type 1. 2346 Mar 43


1