Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The main manifestations of neuromuscular disease in the newborn period are hypotonia and weakness. Infants with severe hypotonia but only marginal weakness usually do not have a disorder of the lower motor unit. These infants may have genetic conditions, metabolic disturbances, congenital heart disease, hypothyroidism, sepsis, or other systemic disorders. Early on, neonates with central nervous system pathology may present with profound hypotonia, decreased reflexes, and moderate to severe but transient weakness. However, they also tend to have seizures, obtundation, cranial nerve signs, or history of perinatal asphyxia.
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PMID:Neuromuscular disorders in the newborn. 939 65

Focal deficits, seizures and epilepsy, altered consciousness, and disturbed behaviours can complicate heart diseases and their medical treatment as well as cardiological procedures and cardiac surgery. Neurological complications of common cardiac conditions are discussed. These cardiac conditions are acute myocardial infarction and ischaemic heart disease, atrial fibrillation and cardiac arrhythmias, congestive heart failure, valvular heart diseases, infective endocarditis, congenital heart disease, invasive cardiological procedures and cardiac surgery. As transient ischaemic attack, stroke, seizures and epilepsy are the most common neurological complications, their management is also reviewed. Precautions should be taken to prevent neurological complications of heart disease. Regular surveillance for these complications would allow early diagnosis and initiation of appropriate management.
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PMID:Neurological complications of heart disease. 948 97

Long QT syndrome (LQTS), is an inherited cardiac disorder in which ventricular tachyarrhythmias predispose affected individuals to syncope, seizures, and sudden death. Characteristic electrocardiographic findings include a prolonged QT interval, T wave alternans, and notched T waves. We have screened LQTS patients from 89 families for mutations in the pore region of HERG , the K+ channel gene previously associated with chromosome 7-linked LQT2. In six unrelated LQTS kindreds, single-strand conformation polymorphism analyses identified aberrant conformers in all affected family members. These conformers were not seen in over 100 unaffected, unrelated control individuals, suggesting that they represent pathogenic LQTS mutations. DNA sequence analyses of the aberrant conformers demonstrated that they reflect five different missense mutations: V612L, A614V, N629D, N629S, and N633S. The missense mutation A614V was found in two unrelated families. Further functional studies will be required to determine what effect each of these changes may have on HERG channel function.
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PMID:Multiple different missense mutations in the pore region of HERG in patients with long QT syndrome. 954 37

The management of the behavior of mentally challenged adults when providing required dental care is often a problem, whether in the dental office or in a hospital setting. Our institution has a designated program to provide required dental care to this group of patients. Because of the high incidence of poor cooperation, which may include aggressive antagonistic behavior, many of these patients are scheduled for dental care under general anesthesia with an incomplete preoperative medical assessment. The purpose of this study was to determine the impact and limitations that an incomplete medical assessment may present in the delivery of dental care under general anesthesia to these adults with developmental disability. After approval from the institutional review board, the medical records of 139 patients treated in this program between 1992 and 1994 were reviewed to determine the patient profiles, anesthesia management, and complications. The charts of these patients, who underwent dental and radiographic examination, scaling and prophylaxis, and restoration and extraction of teeth under general anesthesia, were reviewed. There were 149 procedures performed on these patients, some more than once. The mean age was 29.5 yr. Males predominated females by a ratio of 2:1. All had multiple diagnoses, medical problems, and medications. Twenty-three patients had Down's Syndrome, four had schizophrenia disorders, 42 had seizure disorders, 11 had hypothyroidism, seven had heart disease, and 14 had central nervous system and neuromuscular disorders. The remainder had a variety of diagnoses, including rare syndromes. One hundred had intravenous (i.v.), 25 had mask inhalation, and 24 had intramuscular ketamine (Ketalar) induction. Nasotracheal intubation was uneventful in 139 patients, five had difficult visualization of the larynx and intubation. Ten patients experienced intraoperative complications, including nonfatal ventricular arrhythmia, slight fall in blood pressure and hypertension (greater than 20% of preoperative value), and four individuals developed laryngospasm. In the Post Anesthetic Care Unit, five patients experienced minor airway problems resulting in a desaturation of oxygen to a level below 85%. Adults with developmental disabilities can be safely managed under general anesthesia for dental treatment in a hospital setting with minimal morbidity and without extensive preoperative investigations.
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PMID:General anesthesia for the provision of dental treatment to adults with developmental disability. 979 4

The authors review the management of brain abscesses (BAbs) in 59 pediatric cases. The major surgical procedure used to treat them was repeated puncture and aspiration (51 cases, or 86.44%), excision (8 cases - 13.56%) being of secondary importance. The main etiology was metastatic lesions (24 cases - 40.68%), those due to cyanotic congenital heart disease being the most frequent. Single lesions dominated (41 cases - 69.49%). Gram-positive cocci were the main bacteria involved (31 cases, 52.5%). CT scan represented the main tool in the diagnosis and follow-up. Puncture and aspiration in BAbs led to a significant decrease in mortality (7 cases - 11.86%), and the incidence of seizures and neurological deficits was also reduced. Mortality was significantly correlated with the following factors: consciousness status, multiple BAbs location and hematogenous dissemination. There were recurrences in 11 cases (18.64%), all observed after aspiration procedures. The current concepts for complex management of BAbs, as reflected in recent literature data, are reviewed, with particular emphasis on the use of real-time CT or ultrasound-guided operative techniques.
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PMID:Neurosurgical management of brain abscesses in children. 1046 79

Target hematocrit/hemoglobin values in dialysis patients are still controversial. The Spanish Cooperative Renal Patients Quality of Life Study Group (including 34 hemodialysis units) conducted a prospective, 6-mo study of the effect on patient functional status and quality of life of using epoetin to achieve normal hematocrit in hemodialysis patients with anemia. The possible adverse effects of increased hematocrit, patient hospitalization, and epoetin requirements were also studied. The study included 156 patients (age range, 18 to 65 yr). Given the minimal experience in the safety of increasing hematocrit in dialysis patients to normal levels with epoetin, stable patients on hemodialysis who had received epoetin treatment for at least 3 mo and had a stable hemoglobin level of > or = 9 g/dl were included in the study. Patients with antecedents of congestive cardiac failure, ischemic cardiopathy, diabetes mellitus, uncontrolled hypertension, cerebrovascular accident or seizures, malfunction of the vascular access or severe comorbidity (defined by a comorbidity index), and those over 65 yr of age were excluded from the study. Quality of life was measured with the Sickness Impact Profile (SIP) and Karnofsky scale. Patients completed questionnaires at home at onset and conclusion of the 6-mo study. Mean hematocrit increased from 30.9 to 38.4% and hemoglobin from 10.2 to 12.5 g/dl during the study. Health indicator scores improved significantly: mean Physical Dimension (SIP) from 5.38 to 4.1 (P < 0.005); mean Psychosocial Dimension from 9.2 to 7 (P < 0.001); mean global SIP from 8.9 to 7.25 (P < 0.001); mean Karnofsky scale score from 75.6 to 78.4 (P < 0.01). (SIP is scaled so that lower scores represent better functional status, and vice versa for the Karnofsky scale). Therefore, functional status and quality of life improved with increased hematocrit. No deaths occurred. Three patients (2%) were censored for hypertension and nine (5.7%) for thrombosis of the vascular access. The cumulative probability of thrombosis of the vascular access was 0.067. The average epoetin dose rose from 93 +/- 62 U/kg per wk at onset to 141 +/- 80 U/kg per wk at conclusion, a 51% increase. The number of patients hospitalized decreased and hospital lengths of stay were shorter during the study period than in the same patients in the 6-mo period preceding the study (P < 0.05). Nine patients (5.7%) had thrombosis of the vascular access. There were no changes in the prevalence of arterial hypertension, but three patients (2%) showed hypertension that was difficult to control. It is concluded that normalization of hematocrit in selected hemodialysis patients, i.e., nondiabetic patients without severe cardiovascular or cerebrovascular comorbidities, improves quality of life and decreases morbidity without significant adverse effects.
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PMID:Increasing the hematocrit has a beneficial effect on quality of life and is safe in selected hemodialysis patients. Spanish Cooperative Renal Patients Quality of Life Study Group of the Spanish Society of Nephrology. 1066 41

E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore can be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decades in western world, it has been introduced in India only since April, 1995. Various cardiac defects where PGE1 is useful include (a) lesions with ductus dependent pulmonary blood flow e.g. pulmonary atresia with or without ventricular septal defect, critical valvular pulmonic stenosis etc, (b) lesions with ductus dependent systemic blood flow e.g. critical aortic stenosis, coarctation of aorta, interruption of aortic arch etc, and (c) admixture lesions like transposition of great arteries. The drug is given as a continuous intravenous infusion. The initial dose is 0.05 to 0.4 ug/kg/min, infusion rate must be decreased to 0.01 ug/kg/min as soon as the desired effect is achieved as incidence of side effects is more at higher doses. Serious side effects include apnoea, hypotension, hyperthermia, seizures etc. We have used this drug in 43 infants ranging in age from one to forty five days. Beneficial response was seen in 41 of 43 infants and the major side effect was apnoea (seen in 5 of 32 spontaneously breathing infants). Unfortunately the high cost of the drug prohibits its wide spread and long term use. PGE1 is a life saving drug for infants born with ductus dependent congenital cardiac malformations. It helps in stabilizing these patients prior to further surgical palliation or correction.
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PMID:Prostaglandin E1: first stage palliation in neonates with congenital cardiac defects. 1077 66

1. Long QT syndrome (LQTS) is a cardiac disorder characterized by syncope, seizures and sudden death; it can be congenital, idiopathic, or iatrogenic. 2. Long QT syndrome is so-named because of the connection observed between the distinctive polymorphic ventricular tachycardia torsade de pointes and prolongation of the QT interval of the electrocardiogram, reflecting abnormally slowed ventricular action potential (AP) repolarization. Acquired LQTS has many similar clinical features to congenital LQTS, but typically affects older individuals and is often associated with specific pharmacological agents. 3. A growing number of drugs associated with QT prolongation and its concomitant risks of arrhythmia and sudden death have been shown to block the 'rapid' cardiac delayed rectifier potassium current (IKr) or cloned channels encoded by the human ether-a-go-go-related gene (HERG; the gene believed to encode native IKr). Because IKr plays an important role in ventricular AP repolarization, its inhibition would be expected to result in prolongation of both the AP and QT interval of the electrocardiogram. 4. The drugs that produce acquired LQTS are structurally heterogeneous, including anti-arrhythmics, such as quinidine, non-sedating antihistamines, such as terfenadine, and psychiatric drugs, such as haloperidol. In addition to heterogeneity in their structure, the electrophysiological characteristics of HERG/IKr inhibition differ between agents. 5. Here, clinical observations are associated with cellular data to correlate acquired LQTS with the IKr/HERG potassium (K+) channel. One strategy for developing improved compounds in those drug classes that are currently associated with LQTS could be to design drug structures that preserve clinical efficacy but are modified to avoid pharmacological interactions with IKr. Until such time, awareness of the QT-prolongation risk of particular agents is important for the clinician.
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PMID:Familial and acquired long qt syndrome and the cardiac rapid delayed rectifier potassium current. 1102 66

Surgical infections of the central nervous system are still attended by high rates of morbidity and mortality, although substantial progress has been made since the advent of computed tomography (CT) scanning technology. In this retrospective review of 25 surgically treated patients with either brain abscess or subdural empyema at the University Hospital of the West Indies, the majority of patients were male and between the ages of 10 and 30 years with a mean age of 16.9 years. Almost half the patients had a hemiparesis on presentation while 60% had fever. Subdural empyema was more common than a localized intracerebral abscess which was most frequently located in the frontal lobe. The most common predisposing factors were sinusitis and congenital heart disease. Streptococci spp and Staphylococci spp were the most frequently isolated organisms. All patients underwent CT scanning and surgical intervention. The mortality rate was 20%, and 21% of the survivors had late seizures. There must be a high index of clinical suspicion and early referral to specialist centres where neuroradiological investigation and prompt neurosurgical intervention can be carried out to minimize morbidity and mortality.
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PMID:Brain abscess at the University Hospital of the West Indies. 1107 12

Pediatric cardiac catheterization may be indicated under certain conditions, but is associated with some risk. The purpose of the study was to evaluate the complications associated with diagnostic and interventional catheterization procedures done over an 18-month period in our laboratory. Of the 230 cardiac catheterizations, 204 were solely diagnostic in nature. Eleven percent were interventional catheterizations including aortic and pulmonary valvuloplasties and balloon atrial septostomy. Six percent of the patients constituted grown-up congenital heart disease (GUCH). The median age was 34 months excluding the GUCH group. There was one death below one year of age (0.4% mortality) occurring six hours after the diagnostic catheterization; it was attributed to the underlying disease. There were eight complications (3.4%) that we would consider serious, including atrial flutter, ventricular tachycardia, severe hypercyanotic spell, seizure, transient complete heart block, peripheral vascular injury which resulted in pseudoaneurysm formation of the femoral artery requiring surgical intervention, and transient pulse loss. When catheterization is necessary, it should be carried out as efficiently as possible with awareness of conditions that probably increase the risk of a clinically important event. Although patients undergoing cardiac catheterization are now younger and have more complex cardiac abnormalities, the procedure seems to have become safer when compared to previous literature.
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PMID:Complications of pediatric cardiac catheterization: 18-month study. 1119 45


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