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)

As the practice of lung transplantation in children evolves, the indications are expanding. The major diagnostic groups for which transplantation is offered are similar to those used in adult lung transplantation with the notable absence of obstructive lung disease. Of all children under the age of 18 years undergoing lung transplantation, cystic fibrosis accounts for approximately 35%, pulmonary vascular disease, with or without associated congenital heart disease, accounts for 25-30%, and interstitial lung diseases comprise about 10%. The other categories included retransplantation and a variety of diagnoses which by themselves describe an unusual form of end-stage pulmonary disease. The selection of candidates with transplantable diagnoses is crucial, and as this specialty evolves the contraindications have as well. Colonization of the airway with resistant bacterial or fungal organisms, history of multiple prior thoracic procedures, need for mechanical ventilation, diabetes mellitus, and the presence of other organ failure constitute some relative contraindications for transplantation. Retransplantation is a controversial issue that has not yet been resolved.
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PMID:Pediatric lung transplantation: indications and contraindications. 884 20

Mature cardiomyocytes have been shown to possess a cyclic AMP-mediated chloride channel (I(Cl)) which is the product of the cystic fibrosis transmembrane regulator (CFTR) gene. Species variability has been demonstrated for other ion channels. This study was designed to evaluate human I(Cl) regulation using the whole-cell patch-clamp bioassay. Atrial tissue obtained from children undergoing congenital heart surgery was enzymatically dispersed into isolated myocytes. The patients ranged in age from 1 day to 11 years (mean 2 years). Isoproterenol was used to activate the cAMP second-messenger system in a potassium-free environment. Membrane calcium and sodium channels were pharmacologically blocked. Of 20 human atrial myocytes obtained from 13 pediatric patients, 80% had a small basal chloride current. The current could be inhibited by the anion transport blocker, 9-anthracene carboxylic acid. In 4 of 20 otherwise viable myocytes, no I(Cl) could be elicited, either at baseline or with beta-adrenergic stimulation. Of the 16 myocytes with a basal I(Cl), the current was unaffected by cAMP stimulation in 15 (94%) cells. There were no significant differences in age, gender or clinical status of patients whose cells conducted Cl- current compared with patients whose myocytes had no measurable I(Cl). Ten mature guinea pig ventricular myocytes were evaluated using the same whole-cell patch-clamp technique. Seven of 10 cells showed a reversible increase in I(Cl) with isoproterenol exposure. Despite presence of the CFTR gene in human cardiomyocytes, functional expression of the cAMP-activated I(Cl) does not appear evident in isolated pediatric atrial myocytes. Whether the pathophysiology of congenital heart disease may influence chloride current modulation via alterations in adrenergic tone, intracellular Ca2+ regulation, and cellular osmotic conditions remains to be established.
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PMID:Lack of cystic fibrosis transmembrane regulator-type chloride current in pediatric human atrial myocytes. 900 Jun 43

Bronchial casts are characterized by the formation of obstructive airway plugs that may be large enough to fill the branching pattern of an entire lung. The condition is rare but can occur at any age. Casts may be secondary to underlying diseases such as asthma and cystic fibrosis, but there are often no predisposing factors. There is no accepted classification system for bronchial casts; but only a confusion of descriptive terms such as mucoid impaction, fibrinous bronchitis, and pseudomembranous bronchitis. Based on a review of nine well-documented cases and the available literature, we have separated bronchial casts into two well-defined groups: Type 1 (inflammatory), consisting of casts composed mainly of fibrin with a dense eosinophilic inflammatory infiltrate; and Type 2 (acellular), consisting of casts composed mainly of mucin with little or no cellular infiltrate and occurring only in children with congenital cyanotic heart disease. Acute mortality was high in both groups. Survivors of Type 1 casts seem to be well controlled with inhaled steroids. Optimal therapy for patients with Type 2 casts is not clear; the prognosis probably depends on underlying cardiac status. We hope that this simple classification will provide a framework for further study of this obscure condition.
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PMID:Bronchial casts in children: a proposed classification based on nine cases and a review of the literature. 900 37

Enteral nutrition is increasingly used in the management of sick children. Malnutrition may frequently complicate chronic illness in children and nutritional support may be a primary treatment. Particularly children with malnutrition due to certain chronic disorders may need long term enteral nutrition not only in hospital but also at home. We report our experience on home enteral nutrition of 14 patients, aged from 3 months to 15 years. Eleven had a serious weight deficit (< 3 degrees percentile), but none had severe alteration of the biohumoral nutritional parameters. The indication for EN were: in 7 patients cerebral palsy, in 2 cystic fibrosis, and in the other 5 patients congenital heart disease, multiple food intolerance, oesophageal athresia, Werding-Hoffman disease, Costello's Syndrome. Nutritional support was given by nasogastric tube in 6 patients, through gastrostomy in 3 patients and by mouth in 5 patients. We did not see any severe complication due to home enteral nutrition. The practicalities of this nutritional approach are discussed. Although home enteral nutrition was evaluated in a low number of patients, it may have a role in promoting growth of children with inadequate dietary intake or malnutrition and in improving their quality of life.
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PMID:[Home enteral nutrition in pediatric age. Based on the Torino experience]. 905 21

Compared participation levels in a longitudinal study of parent-child relationships from infancy to 4 years in families of children with cystic fibrosis, congenital heart disease, and with no chronic illness. Demographic (parent's age, education) and child, parent, and family variables (medical status, family environment) were investigated for their predictive value of families' participation. 34% of families (71/209) were lost to the study at a later date. Families of children with cystic fibrosis were the least likely to be lost. Parents' age and/or education predicted participation in all groups. Families in both pediatric samples participated less when parental well-being was less optimal, and the level of mother-infant attachment organization was lower. Unlike demographic factors, family factors have differential impact on participation in families in pediatric and nonpediatric samples.
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PMID:Factors influencing family participation in a longitudinal study: comparison of pediatric and healthy samples. 911 46

The response of bronchiolitis to bronchodilator drugs is controversial. The present study was designed to evaluate the efficacy of oral or metered dose inhaler (MDI) salbutamol using a coffee cup as a spacer device in bronchiolitis. In the trial, 31 hospitalized patients between 6 and 24 months of age, who exhibited the first episode of acute bronchiolitis without any other predisposing illness such as cystic fibrosis, congenital heart disease etc., were randomly assigned to receive oral salbutamol (n = 11, 0.1 mg/kg per dose, four times a day), or MDI salbutamol (n = 12, 200 micrograms per dose, every 3 h) or formed the control group without any bronchodilator therapy (n = 8). All of the patients were given supplemental oxygen as needed and adequate hydration was maintained. The patients were evaluated with clinical symptom scores. There were no differences in the beneficial or side effects of salbutamol, or the number of days in hospital between the treatment groups and the control group. It was concluded that there is no beneficial effect in using bronchodilators in infants with bronchiolitis. Supplemental oxygen and maintenance of normal hydration may be adequate.
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PMID:Effect of oral and inhaled salbutamol in infants with bronchiolitis. 912 56

When infants with recurrent wheezing have a clinical course inconsistent with asthma, an extensive list of alternative diagnoses needs to be considered. Anatomic malformations, such as congenital heart disease, laryngotracheomalacia, and diaphragmatic hernia, should be considered for immediate medical stabilization and early surgical correction. Life-threatening infections such as bacterial epiglottitis, retropharyngeal cellulitis, and viral myocarditis require prompt intervention. A careful history and physical examination reveal important diagnostic clues that, in this case, prompted a directed evaluation to rule out common masqueraders of asthma such as foreign body aspiration, cystic fibrosis, gastroesophageal reflux, viral pneumonitis, or pulmonary tuberculosis. On occasion, such a search is unrevealing and a diagnostic challenge remains. In those situations, judicious use of modern technology to scrutinize anatomic (high-resolution computed tomography) and functional (infant pulmonary function tests) pathology, and justifiable invasive procedures such as bronchoscopy and lung biopsy, uncover the true diagnosis, allowing for optimal management.
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PMID:A wheezy infant unresponsive to bronchodilators. 916 57

Pediatric lung transplantation is becoming more common, and with increasing experience there is increasing success. The most common indications for considering lung transplantation are cystic fibrosis, pulmonary vascular disease (usually due to congenital heart disease), and fibrotic lung disease. The contraindications and complications are similar to adult transplant patients, although post-transplant lymphoproliferative disease and airway complications may occur more frequently. The patients with cystic fibrosis face additional obstacles to the success of transplantation: airway colonization with Gram-negative organisms, pancreatic insufficiency, glucose intolerance, and osteoporosis. The survival for children is comparable to adults, reaching about 65% at 1 year, and 69% at 2 years.
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PMID:Pediatric lung transplantation. 918 31

In a prospective study of 137 children (47 with cystic fibrosis, 48 with congenital heart disease, 42 with no chronic illness), four domains were examined as predictors of parent-reported behavioral problems, particularly internalizing problems, at 4 years of age: child health, child temperament, parent-child relationships, and family environment. Family environment, as measured by the Parenting Stress Index at 1,2, and 3 years, was the most powerful predictor. This suggests that this index is useful as an early screen for children at risk for behavioral problems and that a reduction of parenting stress is an appropriate target of preventive interventions.
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PMID:Prediction of preschool behavioral problems in healthy and pediatric samples. 934 73

To identify Chinese geneticists' views of ethical issues in genetic testing and screening, a national survey was conducted. Of 402 Chinese geneticists asked to participate, 255 (63%) returned by mail anonymous questionnaires. The majority of respondents thought that genetic testing should be offered in the workplace for alpha-antitrypsin deficiency (95%) and the predisposition of executives to heart disease, cancer, and diabetes (94%); that genetic testing should be included in preemployment physical examinations (86%); that governments should require premarital carrier tests (86%), newborn screening for sickle cell (77%), and Duchenne muscular dystrophy (71%); and that children should be tested for genes for late-onset disorders such as Huntington disease (85%), susceptibility to cancers (85%), familial hypercholesterolemia (84%), alcoholism (69%), and Alzheimer disease (61%). Most believed that partners should know each other's genetic status before marriage (92%), that carriers of the same defective gene should not mate with each other (91%), and that women should have a prenatal diagnosis if medically indicated (91%). The majority said that in China decisions about family planning were shared by the couple (82%). More than half had views that, in China, there were no laws to prohibit disability discrimination (64%), particularly to protect people with adult polycystic kidney disease (57%), cystic fibrosis (56%), or genetic predisposition to other diseases (50%). To some extent, these results might provide a basis for a discussion of eugenics in China, particularly about China's Maternal and Infant Health Care Law (1994).
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PMID:Chinese geneticists' views of ethical issues in genetic testing and screening: evidence for eugenics in China. 1048 40


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