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)

From 1958 to March 1987 we corrected 704 patients with pectus excavatum. The condition occurred more frequently in boys (544 patients) than girls (160 patients). In the majority of patients (86%), the defect was evident at birth or within the first year of life. Musculoskeletal abnormalities were identified in 133 patients (scoliosis, 107; kyphosis, 4; myopathy, 3; Poland's syndrome, 3; Marfan's syndrome, 2; Pierre Robin syndrome, 2; prune belly syndrome, 2; neurofibromatosis, 3; cerebral palsy, 4; tuberous sclerosis, 1; and congenital diaphragmatic hernia, 2). Sixteen patients had associated congenital heart disease. A family history of chest wall deformity was present in 37% of the cases and a history of scoliosis in 11%. Surgical correction was performed using a uniform technique for bilateral subperichondrial resection of the deformed costal cartilages and sternal osteotomy resecting a wedge of the anterior cortex and fracturing the posterior cortex. Anterior displacement was maintained with silk sutures closing the osteotomy defect. In 28 early cases, the sternum was secured by intramedullary fixation with a Steinman pin. All repairs were completed with a low complication rate (4.4%; pneumothorax, 11; wound infection, 5; wound hematoma, 3; wound dehiscence, 5; pneumonia, 3; seroma, 1; hemoptysis, 1; hemopericardium, 1). Six complications were associated with Steinman pin fixation (hemoptysis, seroma, hemopericardium, pneumothorax, 3). Major recurrence occurred in 17 patients (2.7%) and led to revision in 12. Satisfactory long-term results were achieved in the remaining 687 patients, with follow-up ranging from 2 weeks to 27 years. Mean follow-up was 4.3 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical repair of pectus excavatum. 320 60

The syndrome of the rigid spine is described in two patients aged 15 and 12 years. A total of 8 patients with this syndrome were studied. The disease was characterized by the myopathic syndrome, scoliosis, failure to bend the head and body, cardiopathy and occasional contractures of the proximal joints. The clinical, biochemical, roentgenological and morphological findings are presented. A multimodality treatment suggested should be carried out by a team comprising an orthopedist, a neuropathologist, and a pediatrician.
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PMID:[Rigid spine syndrome in children and adolescents]. 370 42

The case records of eight children with an acute spinal cord lesion after cardiac surgery were reviewed. All had been operated on for congenital heart disease over the past 10 years. The prognosis for neurological recovery was poor and scoliosis and bladder dysfunction were common complications. Ischaemic cord injury is usually associated with coarctation of the aorta but may occur when surgery is performed for other cardiovascular defects.
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PMID:Ischaemic spinal cord injury after cardiac surgery. 401 68

Of fifty patients with the diagnosis of Klippel-Feil syndrome, less than half had the classic clinical triad of findings, while more than half had scoliosis, and a third had renal anomalies. All patients were at risk of having other serious, but less apparent, anomalies, including: Sprengel's deformity (twenty-one patients), impairment of hearing (fifteen patients), synkinesia (nine patients), and congenital heart disease (seven patients). The discovery of one of these lesions should, therefore, stimulate a careful search for associated anomalies.
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PMID:Klippel-Feil syndrome; a constellation of associated anomalies. 443 58

Electrocardiograms of 802 patients with isolated scoliosis followed and/or operated at Columbia Presbyterian Medical Center were reviewed. There were 586 patients younger than 18 years and 216 patients older than 18 years of age. There were 86 males and 716 females. Eleven patients had associated congenital heart disease. Effects of age, site of scoliosis, side of convexity and severity of curvature on multiple electrocardiographic variables were analysed. Electrocardiographic variables such as heart rate, Q-Tc, P wave amplitude, P-R interval, amplitude of R and S waves in V1 and V6 were similar to data reported in the normal population without scoliosis. The effect of age on these electrocardiographic variables was similar to changes reported in the normal population and were not affected by site of scoliosis or side of curvature. The frontal QRS axis was within normal limits of 94% in patients under 18 years of age with right-sided thoracic scoliosis and in 87% of patients over 18 years. Evidence of right axis deviation (5%) and right ventricular hypertrophy (2%) were present equally in patients younger and older than 18 years of age and 8% of patients over 18 years old. Since in our patient population the degree of scoliosis severity increased with age it is likely that the increasing incidence of left axis deviation found in patients over 18 years of age with scoliosis is related to scoliosis severity and the altered intrathoracic cardiac orientation. Because right axis deviation and right ventricular hypertrophy are noted occasionally in patients with scoliosis and because congenital heart disease and pulmonary disease are more prevalent in this patient population, it is recommended that the presence of right axis deviation and right ventricular hypertrophy on the ECG should lead to a complete cardiopulmonary evaluation and exclusion of possible additional cardiac or pulmonary anomalies.
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PMID:The electrocardiogram in patients with scoliosis. 706 29

A Somali female baby with right upper limb triplication, polythelia, left sided hemihypertrophy, congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis is described. It seems that the above described pattern of anomalies has not been reported before. The possible developmental genetic mechanism responsible for this phenotype is briefly discussed.
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PMID:Right upper limb bud triplication and polythelia, left sided hemihypertrophy and congenital hip dislocation, facial dysmorphism, congenital heart disease, and scoliosis: disorganisation-like spectrum or patterning gene defect? 756 71

We report a growth-retarded infant with congenital heart disease and maternal isodisomy for chromosome 16. Non-mosaic trisomy 16 was detected at mid-trimester chorionic villus sampling, performed because biochemical screening indicated an increased Down's syndrome risk. Further karyotyping analysis of the placenta, after delivery, showed a 50 per cent mosaic trisomy 16. The infant had an atrioventricular (A-V) canal defect, scoliosis, and several minor dysmorphic features. Although uniparental disomy for chromosome 16 has been reported previously, to our knowledge this is the first case of uniparental isodisomy for chromosome 16 which has been investigated with multiple DNA probes.
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PMID:Uniparental isodisomy for chromosome 16 in a growth-retarded infant with congenital heart disease. 765 92

Scoliosis surgery in the adolescent is prolonged, painful and haemorrhagic. There are neurological risks and surveillance of the medulla is necessary throughout surgery. An anterior (Dwyer, Zielke) or posterior (Harrington, Cotrel-Dubousset, Luque) approach to the spinal column cas used. Surgery using a posterior approach is the more haemorrhagic. The haemorrhage is increased by poor positioning of the patient, by the duration of surgery and by taking the bone graft. Constant care should be given to blood economy, using controlled hypotension, haemodilution and peroperative autotransfusion of lost blood (Cell-Saver). The anaesthetic should provide excellent analgesic effects and must be compatible with regain of consciousness during surgery and/or or the use of evoked potential techniques. Complications at that time are those of the circulation and those of neurological origin and linked with hypothermia. In the presence of haemorrhage, the maintenance of total blood volume is difficult when there is cardiopathy (myopathy). Neurological complications should be detected sufficiently early for them to be reversible (sensori-motor evoked potentials and/or "wake-up test"). Hypothermia is constant and requires the systematic use of a heated mattress, a heated humidifier and the heating of infusions. The postoperative complications are respiratory in origin and are especially associated with neuro-muscular disease (postoperative artificial ventilation). The per- and postoperative difficulties demonstrate the importance of the preoperative examination and of the preparation of the operation (respiratory preparation). Finally, staged autotransfusion should be used, when possible, and should be part of the techniques of blood economy in a true transfusion strategy.
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PMID:[Anesthetic problems and postoperative care in the surgery for scoliosis]. 781 11

Ten of 32 patients with primary ciliary dyskinesia syndrome (PCDS) also had other conditions. Five esophageal problems, 4 congenital heart disease, 2 scoliosis, and 4 miscellaneous and probably coincidental conditions were discovered. Additionally, a patient in this series, who has a normal heart, had a brother who died after surgery for complex congenital heart disease. In retrospect, he too probably had PCDS. The association of severe esophageal and cardiac disease with primary ciliary dyskinesia has not been described before. The diagnosis of PCDS may carry more implications than previously recognized.
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PMID:New associations of primary ciliary dyskinesia syndrome. 841 46

The first palliative operations of congenital heart disease in the 1940s and 1950s, and then open heart surgery in the 1960s, have resulted in survival of patients whose follow-up is now over 30 years. Problems of patients with congenital heart disease are cardiac (pulmonary hypertension, systemic hypertension, dysrhythmias, endocarditis, cerebral insults, etc.), and noncardiac (scoliosis, intrathoracic adhesions, cosmetic problems, pregnancy, physical activity, employment, etc.). A pediatric cardiologist usually follows up such patients until adolescence, however, after that time they remain without physician's care who followed them in their childhood, being transferred for further controls and follow-up to internists-cardiologists. Many difficulties arise since internists-cardiologists are not properly trained in this particular sense, and pediatricians are not trained to follow-up adult patients. The care for a patient should be coordinated. To care for an adolescent, who was the child with congenital heart disease, pediatrician and cardiologist have to work at the same medical centre. The follow-up should continue as a team work, in which, besides pediatrician and cardiologists, cardiosurgeon, psychologist, psychiatrist, obstetrician, specialist for physical medicine and social worker should be included. A correction of educational plan is mandatory.
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PMID:[Problems in adolescents and adults with congenital heart defects]. 853 60


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