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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Failure to thrive is a common feature of children with congenital heart disease. Whether this is the result of poor nutrition or an abnormally high basal metabolic rate is unknown, yet the state of nutrition has a profound effect on the metabolic response to injury and strongly influences the outcome of surgical treatment. The aim of this study was therefore to measure the preoperative and postoperative energy requirements of children with congenital heart disease. Eighteen children (aged 4 to 33 months) were given two oral doses of doubly labeled water (H2(18)O and 2H2O), the first 1 week before operation and the second 6 hours after the end of cardiac surgery. By measuring the relative loss of each isotope from the body water pool, we were able to calculate the rate of carbon dioxide production and therefore total energy expenditure. In five patients, energy expenditure was clearly elevated, suggesting that a raised basal metabolic rate is an important factor in the observed failure to thrive in at least a proportion of such children. Postoperatively, energy expenditure fell to values below normal for healthy children (not having an operation), which suggests that the stress of surgery leads to smaller energy requirements than have previously been thought.
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PMID:Energy expenditure in children with congenital heart disease, before and after cardiac surgery. 830 56

Children with congenital heart disease may have significant growth retardation, which in part may be caused by insufficient dietary intake. Data on energy and nutrient intake were collected using a 14-day dietary record by weighing, in 22 children with congenital heart disease (mean age 39 months, range 12-126 months), prior to corrective operation. When viewed in relation to actual weight, energy intake averaged 88% (SD 17%) of that recommended by the FAO/WHO/UNU. Energy intakes and weight SD scores were significantly correlated (r = 0.55, p < 0.01). Protein intake was generally high, and even sufficient to allow catch-up growth. The majority of the children did not meet the recommendations for iron, zinc, calcium, or vitamins D, E, C, B1 or B6. Parents should be advised to give their children vitamin/mineral supplements, and to supply extra energy to children with failure to thrive.
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PMID:Energy and nutrient intakes in congenital heart disease. 847 62

Smith-Lemli-Opitz syndrome (SLOS) is a common autosomal recessive disorder. Children with SLOS present with specific facial dysmorphism and have multiple congenital anomalies including cleft palate, congenital heart disease, genitourinary anomalies, and limb abnormalities. They also manifest severe failure to thrive and mental retardation. A metabolic defect at the final step in the cholesterol biosynthetic pathway has been described in SLOS patients. This defect results in markedly reduced cholesterol levels and abnormal accumulation of cholesterol precursors, particularly 7-dehydrocholesterol. This newly described metabolic defect in humans is one of only a few metabolic errors known to cause multiple birth defects. The biochemical profile of reduced plasma cholesterol levels in association with markedly elevated levels of the cholesterol precursor 7-dehydrocholesterol is now used to confirm the diagnosis of SLOS, which was formerly made on purely clinical grounds. This biochemical abnormality has been confirmed in dozens of patients with SLOS in both the United States and Europe. The severe cholesterol deficiency seen in these patients has multiple effects on health and early childhood development, because cholesterol is an essential component of many cell functions, which explains many of the clinical findings seen in SLOS.
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PMID:Abnormal cholesterol metabolism in Smith-Lemli-Opitz syndrome. 877 24

Ribavirin was approved in early 1986 for treatment of illness associated with respiratory syncytial virus (RSV) infection in hospitalized children; however, the value of its use remains controversial. This investigation was undertaken to describe ribavirin utilization and to evaluate the effectiveness of ribavirin in reducing the period of hospitalization. All children with laboratory-confirmed RSV infection, hospitalized over seven epidemic periods at a single institution, were identified. Characteristics from the medical records of the 768 children and their course of hospitalization were evaluated as determinants of treatment with ribavirin. Very young age as well as diagnoses of congenital heart disease, certain pulmonary conditions, and failure to thrive were all significantly related to increased likelihood of treatment. Children requiring intensive care were likely to be treated; however, the most critically ill children, those who required mechanical ventilation, were not treated. Although these results were in keeping with treatment guidelines for the use of ribavirin, actual utilization declined significantly over the course of the evaluation. The effect of ribavirin treatment on the duration of hospitalization among children with community-acquired infection was evaluated in a multivariate model, which controlled for the effects of underlying conditions, measures of illness severity, and epidemic year. This analysis failed to demonstrate a benefit for ribavirin in reducing length of stay; in fact, ribavirin treatment was significantly associated with prolonged hospitalization. It was not likely that the explanation for ribavirin's association with prolonged hospitalization was that the most seriously ill children were treated; however, it was not possible to determine whether this association was due to an adverse effect of treatment or resulted from a perceived need to complete the course of therapy.
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PMID:Ribavirin utilization and clinical effectiveness in children hospitalized with respiratory syncytial virus infection. 878 Jun 3

Aside from congenital heart disease, anomalies associated with unilateral hypoplasia of the depressor anguli oris muscle have not been well-documented in large series. We evaluated the associated anomalies in 50 infants or children with this disorder (male:female = 2:1) and found accompanying anomalies in 35 (70%) of 50 cases. They included anomalies of the head and neck (48%), heart (44%), skeleton (22%), genitourinary tract (24%), central nervous system (10%), gastrointestinal tract (6%), and miscellaneous minor anomalies (8%). Nearly half of our cases (22/50) had at least 2 associated systemic anomalies. Failure to thrive and psychomotor retardation were found in 5 (10%) and 3 (6%) patients, respectively, on follow-up. Three infants died neonatally of severe heart disorders, and the other one died of central nervous system anomalies. The above findings indicate that a thorough search for associated anomalies, particularly in the cardiovascular system, should be performed in all newborns with asymmetric crying face.
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PMID:Frequency of associated anomalies in congenital hypoplasia of depressor anguli oris muscle: a study of 50 patients. 921 25

Enteral feeding, the provision of liquid nutrients into the gastrointestinal tract, is an important component of pediatric care. For the infant or child with a functioning or even a partially-functioning GI tract, the use of the enteral route provides a safe and efficient means of delivering nutrition at a time of life when requirements are extremely high. In addition to high nutrient requirements in the early years of life, there are a number of specific pediatric conditions, such as failure to thrive, short bowel syndrome, and congenital heart disease, which place further demands on the growing child. These demands can be met through the careful use of enteral feeds. This article reviews the physiology and practical application of enteral feeding to the pediatric age group.
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PMID:Enteral nutrition in the pediatric population. 965 75

Disturbances in growth are often a consequence of congenital heart disease during infancy and childhood. The magnitude of the growth disturbance is generally related to the anatomical lesion and is most severe in infants and children with congestive heart failure. Presently, surgical repair in this population is often delayed in order to permit increased weight gain. Surgery is preformed when a patient reaches an ideal weight and age, or failure to thrive precludes further waiting. The available data indicate that caloric intake in these infants and children may be nearly adequate for age, but is inadequate to permit normal growth rates. Energy expenditure appears to be significantly elevated in this population relative to that of age-matched infants and children. Therefore, while caloric intake may be appropriate for age, increased energy expenditure leaves the infant or child with congenital heart disease with little energy available for growth. More information is needed on energy intakes and expenditures of specific patient populations, and especially of patients with congestive heart failure, before accurate predictions of their metabolic needs are possible. This knowledge may allow us to better meet the nutritional needs of these populations and decrease the risk of malnutrition and failure to thrive, in turn decreasing surgical risk for these patients.
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PMID:Growth, nutrition and energy expenditure in pediatric heart failure. 1097 12

Central ventilation disorders(1) and airway obstruction(2) with chronic hypoxemia are causally related to cor pulmonale. Pulmonary vascular resistance is often reversible, and hypoxic pulmonary hypertension often responds to treatment with supplemental oxygen. Oxygen therapy during sleep may be useful as a temporary palliative treatment in children with obstructive sleep apnea syndrome (3) and Cheyne-Stokes respiration (CSR) in congestive heart failure(4). This type of sleep-related breathing disorder is characterized by periodic crescendo-decrescendo alterations in tidal volume. Proposed mechanism include an increased central nervous system sensitivity to changes in arterial PCO2 and PO2, a decrease in total body stores of CO2 and O2 with resulting instability in arterial blood gas tensions in response to changes in ventilation, and an increased circulatory time. Clinical features of obstructive and central sleep-related breathing disorders include daytime somnolence, unusual breathing patterns, failure to thrive, and cyanosis masquerading as cyanotic congenital heart disease(2). Down syndrome is often associated with cardiac malformations, left to right shunt, and the development of pulmonary hypertension(5). However, this may be exacerbated by sleep-related breathing disorders, as illustrated in the following case report.
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PMID:Cheyne-Stokes respiration as an additional risk factor for pulmonary hypertension in a boy with trisomy 21 and atrioventricular septal defect. 1127 40

In order to study the functions of simian immunodeficiency virus (SIV) Nef in vivo in a small-animal model, we constructed transgenic (Tg) mice expressing the SIV(mac)239 nef gene in the natural target cells of the virus under the control of the human CD4 gene promoter (CD4C). These CD4C/SHIV-nef(SIV) Tg mice develop a severe AIDS-like disease, with manifestations including premature death, failure to thrive or weight loss, wasting, thymic atrophy, an especially low number of peripheral CD8+ T cells as well as a low number of peripheral CD4+ T cells, diarrhea, splenomegaly, and kidney (interstitial nephritis, segmental glomerulosclerosis), lung (lymphocytic interstitial pneumonitis), and heart disease. In addition, these Tg mice fail to mount a class-switched antibody response after immunization with ovalbumin, they produce anti-DNA autoantibodies, and some of them develop Pneumocystis carinii lung infections. All these results suggest a generalized Nef-induced immunodeficiency. The low numbers of peripheral CD8+ and CD4+ T cells are likely to reflect a thymic defect and may be similar to the DiGeorge-like "thymic defect" immunophenotype described for a subgroup of human immunodeficiency virus type 1-infected children. Therefore, it appears that SIV Nef alone expressed in mice, in appropriate cell types and at sufficient levels, can elicit many of the phenotypes of simian and human AIDS. These Tg mice should be instrumental in studying the pathogenesis of SIV Nef-induced phenotypes.
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PMID:Expression of simian immunodeficiency virus nef in immune cells of transgenic mice leads to a severe AIDS-like disease. 1190 38

Identification of variables that predict duration of RSV-associated hospitalization may be useful in the identification of preventive and therapeutic strategies. A recently published prediction model (Michigan model) for the duration of hospitalization in RSV infection demonstrated good discrimination between children with and without an increased likelihood of a hospital stay >or= 7 days, based on variables such as log weight, congenital heart disease, failure to thrive, premature birth, bronchopulmonary dysplasia, other pulmonary diseases, miscellaneous conditions, early mechanical ventilation, and early ribavirin treatment (receiver operating characteristic (ROC) area, 0.89). Validation of this model is of particular interest for Europe, since the mean duration of hospitalization in The Netherlands is approximately twice that in the USA. The objective of the current study was 1) to validate the Michigan model for RSV hospitalized patients in a large university hospital in The Netherlands, and 2) to develop our own prediction model for a prolonged hospital stay. Data from 177 children younger than 12 months of age admitted with confirmed RSV infection to the Sophia Children's Hospital Rotterdam between 1992-1995, were used for valiation of the Michigan model and derivation of the Rotterdam model. Mean duration of hospitalization for the Rotterdam database was 10.3 (+/-6.3) days, with a median of 9 days; 138 (78%) patients had a hospital stay >or= 7 days. The Michigan model performed poorly when applied to the Rotterdam database, with an ROC area of 0.65 (95% CI, 0.57-0.73). The Rotterdam prediction model (hospital stay >or= 9 days, the median in our database) considered weight and need for oxygen supplementation. The ROC area was 0.65 (95% CI, 0.57-0.73). When using data from patients for the 1995-1996 season, the ROC area was 0.52 (95% CI, 0.34-0.72). The Michigan and the Rotterdam models failed to identify a considerable number of patients who had a prolonged hospital stay, with a low false-positive rate. We conclude that neither the Michigan, nor the Rotterdam model reliably predicted the duration of hospitalization based on demographic and clinical variables.
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PMID:Prediction of duration of hospitalization in respiratory syncytial virus infection. 1200 Dec 79


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