Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of the 355 patients with tuberous sclerosis complex (TSC) examined at the Mayo Clinic, 49 had died (9 of causes other than TSC). We attempted to determine what pattern of organ involvement occurred most often in the 40 patients who died of TSC. One baby died of cardiac failure due to cardiac rhabdomyomas, and one child died of rupture of an aneurysm of the thoracic aorta. Eleven patients died of renal disease, which was the commonest cause of death. Ten patients died as a result of brain tumors, and four patients (who were 40 years of age or older) died of lymphangiomyomatosis of the lung. Thirteen patients with severe mental handicaps died of either status epilepticus or bronchopneumonia; in all but one of these patients, the only source of information was the death certificate. Survival curves show a decreased survival for patients with TSC in comparison with that for the general population. Patients with TSC need lifelong follow-up for early detection of potentially life-threatening complications.
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PMID:Causes of death in patients with tuberous sclerosis. 186 50

A 17-year-old boy was admitted to hospital in acute cardiac failure and psychosis. The clinical course, EEG records and tissue diagnosis, including biopsies of brain, skin, skeletal muscle, peripheral nerve and liver were compatible with Lafora-body disease (LBD). Unusual features were those of optic atrophy and macular degeneration, signs generally regarded as negative criteria for the diagnosis of this disease. We also present the findings on endomyocardial biopsy which was performed because cardiac failure as an early symptom of LBD has not been previously described. The patient died in status epilepticus a few months after discharge from hospital.
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PMID:Lafora-body disease with optic atrophy, macular degeneration and cardiac failure. 250 38

Patients with mitochondrial disease may present to the Intensive Care Unit (ICU) with a variety of neurological and general medical disorders. Eleven patients were admitted to a neurological ICU between 1970 and 1992 because of respiratory insufficiency, status epilepticus and/or metabolic encephalopathy associated with mitochondrial disease. Respiratory impairment occurred in eight patients and was associated with nocturnal hypoventilation due to respiratory muscle weakness, aspiration due to bulbar weakness and abnormalities of central control leading to a reduced CO2 drive, irregular respiratory patterns and sleep apnoea. Seven patients received continuous respiratory support during the acute illness; three were subsequently weaned to domiciliary ventilation, and four died. Five patients had stroke-like episodes, which in two were recurrent. Four patients developed tonic-clonic grand mal epilepsy associated with myoclonic fits (2 patients), absences (2), focal fits (1) and status epilepticus (2). Encephalopathy was associated with recurrent lactic acidosis (2 patients), cardiac failure (2), hyponatraemia (2), renal abnormalities (3) and complete heart block (1). Although rare, mitochondrial disease should be considered in any patient with unexplained respiratory failure, intractable epilepsy, lactic acidosis or recurrent stroke.
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PMID:Management of mitochondrial disease on an intensive care unit. 776 70

So far propofol has only been used in clinical settings for sedation and induction of anaesthesia. This study describes several indications in preclinical and emergency events. All users were anaesthetists, so that experience of administration and dosage was extremely helpful. Since the drug met the expected criteria it is now regularly used for the sedation of ventilated patients during transport. The most important indications for preclinical induction of anesthesia with propofol are patients with isolated head injury and patients with respiratory insufficiency due to status asthmaticus resistant to therapy. After repeated unsuccessful attempts at therapeutic intervention with benzodiazepines and other antiepileptics we were able to interrupt status epilepticus in 11 patients by means of propofol, thereby preventing the patient from being intubated as a consequence of iatrogenic respiratory failure. However, emergency doctors must always be aware of the severe cardiocirculatory side effects of the drug, and must, hence, ensure that hypovolaemia or cardiac failure is excluded or corrected prior to propofol administration.
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PMID:[Propofol in emergency care--areas of application and initial experiences]. 781 Jan 46

The etiology of sudden death in patients with epilepsy remains unclear. Previous studies in a well-established sheep model of status epilepticus showed that more than one-third of the unsedated animals died within 5 minutes of seizure onset due to hypoventilation. The relative contributions of airway obstruction and central hypoventilation could not be determined because airway flow and respiratory effort were not monitored. In this study, status epilepticus was induced in unsedated sheep with tracheostomies monitored by electrocardiography, electroencephalography, arterial line, serial blood gases, and airway flowmeter. All 8 animals demonstrated central apnea and hypoventilation, which resulted in the death of 1 and contributed to the death of another. A third animal died of acute heart failure within 2 minutes of seizure onset, accompanied by a large septal myocardial hemorrhage, contraction bands, and signs of global cardiac ischemia. More subtle contraction bands, subendocardial hemorrhage, and signs of acute myocardial ischemia were seen in other animals as well, none of which died of cardiac causes. Malignant arrhythmia was not seen in any of the sheep. Central hypoventilation and apnea accompany generalized status epilepticus and may be an important cause of sudden death in epileptics. Acute cardiac failure may also be a cause of epileptic sudden death.
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PMID:Central apnea and acute cardiac ischemia in a sheep model of epileptic sudden death. 938 70

Although midazolam has been proposed for the treatment of a variety of conditions such as anxiety, dyspnoea, hiccups and status epilepticus, terminal agitation is the only condition where its use is based on a reasonably large number of published clinical studies. A causal approach is generally recommended. Whenever possible, the aetiological condition (pain, fever, constipation, etc.) should be corrected. Such general measures as ensuring a peaceful, familiar environment, and the use of a night light, fluid therapy to counteract dehydration, and antipyretics for fever are beneficial. When symptomatic treatment is needed, drugs with little anticholinergic effect are to be recommended. The use of benzodiazepines as single drug treatment may exacerbate the condition. Haloperidol or risperidone (which has fewer side effects) are recommended. If the agitation is marked, a common strategy is to add lorazepam. Chlormethiazole is an alternative. Subcutaneous midazolam should be reserved for refractory cases. Attention should be paid to dosage, reduced doses being given to the elderly, patients on opioid medication, and patients with impaired liver or renal function. Overdosage may induce deep sedation, and result in carbon dioxide retention and subsequently heart failure and pulmonary oedema which may be fatal.
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PMID:[Midazolam (Dormicum) in terminal anxiety and agitation. The last choice alternative in palliative care]. 1035 70

Cerebrovascular disease has different acute, ischemic and hemorrhagic presentations and may be associated with epileptic crises during the acute phase, or a later epileptic syndrome may develop. Status epilepticus is an infrequent complication which may appear at any time during the course of the illness, sometimes as the first and only sign of epilepsy. The risk of acute crises or of an epileptic syndrome varies depending on the nature of the vascular accident: its occurrence is more likely in hemorrhagic lesions and in those involving the cerebral cortex. The acute crises may be treated with benzodiazepines or with fast acting antiepileptic drugs; parenteral administration may sometimes be necessary. The need for prolonged prophylactic antiepileptic treatment is still under discussion, since there is no evidence that this prevents later development of an epileptic syndrome. The management of status epilepticus is the same whatever the etiology, although one has to take account of the risk of side-effects related to the age and general health of the patient. When deciding on treatment for vascular epilepsy consideration should be given not only to which drugs are to be used, but also their pharmacokinetic characteristics and interactions with any treatment required by the patient for coexisting conditions such as arterial hypertension, heart failure, anticoagulation, diabetes, etc.
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PMID:[Overall treatment of vascular epilepsy]. 1071 3

Propofol is used for the treatment of refractory status epilepticus. When given as a long-term infusion propofol may cause a rare but frequently fatal complication, the propofol infusion syndrome. The hallmarks are metabolic acidosis, lipemia, rhabdomyolysis and myocardial failure. Propofol infusion syndrome is caused by impaired fatty acid oxidation. Beside anticonvulsants the ketogenic diet, a high-fat, low-carbohydrate, adequate-protein diet, is an effective treatment for difficult-to-control seizures. We report a 10-year-old boy with catastrophic epilepsy, who developed fatal propofol infusion syndrome when a ketogenic diet was initiated. Substances like propofol which impair fatty acid oxidation may pose an increased risk if combined with ketogenic diet.
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PMID:Fatal propofol infusion syndrome in association with ketogenic diet. 1532 67

In critically ill patients, adequate sedation increases comfort, minimizes stress response and facilitates diagnostic and therapeutic procedures. Propofol (2-, 6-diisopropylphenol) is an intravenous sedative-hypnotic agent popular for sedation in the Intensive Care Unit. The favorable propofol pharmacokinetic, characterized by a three compartment linear model, allows rapid onset and short duration of action. The emergence time from sedation with propofol varies with the depth and the duration of sedation and the patient's bodyweight. Propofol causes hypotension, particularly in volume depleted patients, decreases cerebral oxygen consumption, reduces intracranial pressure and has potent anti-convulsant properties. It is a potent antioxidant, has anti-inflammatory properties and is a bronchodilator. As a consequence of these properties, propofol is being increasingly used in the management of traumatic head injury, status epilepticus, delirium tremens, status asthmaticus and in septic patients. Prolonged use (>48 h) of high doses of propofol (>66 mcg/Kg/min) has been associated with lactic acidosis, bradycardia, and lipidemia in pediatric patients. A rare complication firstly reported in pediatrics patients and also observed in adults is known as "propofol syndrome" characterized by myocardial failure, metabolic acidosis and rhabdomiolysis. Hyperkalemia and renal failure have also been associated with this syndrome. Hypertriglyceridemia and pancreatitis are uncommon complications. A large number of trials have compared the use of propofol with midazolam. Sedation with propofol is associated with adequate sedation in ICU patients, shorter weaning time and earlier tracheal extubation compared to midazolam, but not before ICU discharge.
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PMID:Sedation in PACU: the role of propofol. 1630 51

Propofol is increasingly used for the treatment of status epilepticus due to the ease of use and tolerability, even if safety data from randomized clinical trials are lacking. An association of high infusion rates of propofol (>5 mg/kg/h) for more than 48 h and constellation of acidosis, rhabdomyolysis, and cardiovascular collapse has been reported in children, but has only been described in a few adult cases. We report a case and autopsy findings of an adult who developed rhabdomyolysis and cardiac failure after receiving propofol for status epilepticus. The patient became symptomatic within 55 h after initiation of propofol infusion. The maximal infusion rate did not exceed 7.2 mg/kg/h, and propofol in excess of 5mg/kg/h was infused for less than 20 h. Preexisting antiepileptic medication may have exacerbated acidosis. Propofol infusion for the treatment of status epilepticus should be carefully weighted against its real risk to develop propofol infusion syndrome, and alternative agents such as benzodiazepines or barbiturates should be considered for first line therapy. If necessary, prolonged propofol infusion at high doses for the treatment of status epilepticus should be used with caution, and in all cases careful monitoring for rhabdomyolysis and acidosis must be performed.
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PMID:Propofol-associated fatal myocardial failure and rhabdomyolysis in an adult with status epilepticus. 1738 34


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