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 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Compared responses of parents of infants with cystic fibrosis, those with congenital heart disease, and those with healthy babies on the Parenting Stress Index. Diagnostic group differences were found mainly in the Child Domain with parents of ill infants reporting greater stress. Differences between mothers and fathers were found mainly in the Parent Domain. The groups did not differ in reports of stress arising from life events other than the target child's illness.
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PMID:Chronic illness in infancy and parenting stress: a comparison of three groups of parents. 238 Aug 77

Combined heart-lung transplantation is now being carried out in many centers around the world. Single-lung and double-lung transplantation are being done in only a few centers and are still considered to be experimental procedures. Combined heart-lung transplantation has been performed successfully in adults and in small children and offers a definite therapeutic alternative to such diseases as severe congenital heart disease with pulmonary hypertension, cystic fibrosis, and primary pulmonary hypertension. At present, the survival statistics for this procedure are similar to those for heart transplantation 15 years ago; it is hoped that, as with heart transplantation, survival rates will improve with better management and more reliable diagnosis of acute rejection, in order to prevent the insidious and relentless onset of obliterative bronchiolitis, which is now the most significant limiting factor to the success of long-term survival in heart-lung transplantation.
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PMID:Pathology of the transplanted heart and lung. 240 6

Taurine (2-aminoethanesulfonic acid), well known for its role in bile salt synthesis, is also involved in a number of crucial physiological processes including modulation of calcium flux and neuronal excitability, osmoregulation, detoxification, and membrane stabilization. With the exception of cow's milk, taurine is widely distributed in foods from many animal, but not plant, sources. Although taurine is synthesized from sulfur-containing amino acids, concern has been expressed about the adequacy of endogenous sources, especially in neonates. Accordingly, proprietary milk formulas are now supplemented with taurine. Retinal dysfunction occurs in taurine-deficient animals. A milder form of this condition has been observed in children on long-term total parenteral nutrition. Preliminary evidence suggests a possible role for taurine administration in congestive heart disease, acute hepatitis, cystic fibrosis, and myotonia. Further studies are required before taurine can be routinely advocated for use in these and other disorders. Recent discoveries concerning taurine's role in cellular proliferation and membrane protection underscore its physiological significance. In this context, taurine's interaction with other nutrients, biochemicals, and xenobiotics warrants extensive exploration. As a conditionally essential nutrient, taurine has several important preventive medical applications.
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PMID:Taurine: an overview of its role in preventive medicine. 249 6

Clinical chemistry is going through an identity crisis, squeezed between automation (de-skilling) on the service side and molecular genetics in research. Automated routine estimations are now carried out and interpreted by machines; the skilled staff members required are more likely to have degrees in electronics than medicine or biochemistry. The role of molecular genetics is more ambiguous; it is inherently reductionist, in that it attempts to explain most clinical phenomena in terms of DNA sequence alone. This has been remarkably successful for single-gene defects (such as those causing Duchenne muscular dystrophy, hemoglobinopathies, cystic fibrosis, and ataxias) and may well prove equally so for Alzheimer's disease, cancer, heart disease, and schizophrenia. DNA diagnosis is not yet routine, but because of technical advances such as gene amplification ("PCR") and high-sensitivity gene-detection assays, it may soon become so, not only in major centers but also in local pathology laboratories and general practice. Clinical chemists must decide whether they wish to respond to this new and stimulating challenge by retooling and retraining. Should anyone be permitted into clinical chemistry during the 1990s without knowledge of both electronics and molecular genetics? Will there be a clinical chemistry in the twenty-first century other than through molecular genetics? This article is a personal response to these questions.
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PMID:Molecular genetics and the transformation of clinical chemistry. 233 3

The parents of 436 disabled pre-school children were interviewed about habits and problems relevant to dental health. The children, who represented 10 different disabling conditions, were examined and dmft registered. The purpose was to study the relationship between different background variables and caries experience. The dmft score was analyzed in accordance with several sociocultural, medical, and habitual variables, using a multiple classification analysis (MCA). The number of daily carbohydrate intakes, duration of use of nursing bottle, family income, and diagnosis were the variables with the strongest association with dmft. Children with congenital heart disease, asthma, and cystic fibrosis had a considerably higher adjusted dmft than the other diagnostic groups. The proportion of children with caries experience was higher in the present survey than in groups of Norwegian children of corresponding age.
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PMID:Caries experience in disabled pre-school children. 293 82

While many chronic illnesses are diagnosed in infancy, there is little systematic study of the impact of chronic illness on early social development and how parental adjustment to a chronically ill infant contributes to subsequent outcomes. This paper outlines some of the factors which affect parents' ability to care for chronically ill infants and preschoolers. It draws upon studies of infants with other medical problems, ongoing longitudinal study of infants with cystic fibrosis and congenital heart disease by the authors; and the authors' clinical experience.
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PMID:Chronic illness and early development. The parent's perspective. 305 Sep 16

In primary pulmonary hypertension of recent clinical onset, pulmonary endothelial cells show injury. To characterize this phenomenon, we measured plasma von Willebrand factor (vWF) by immunologic and ristocetin cofactor assays in 6 patients with primary pulmonary hypertension, 17 patients with secondary pulmonary artery hypertension associated with congenital heart disease or cystic fibrosis, and 13 patients with congenital heart disease and normal pulmonary artery pressure. In selected cases, we also determined the vWF multimer pattern. In all 6 cases of primary pulmonary hypertension, the ristocetin cofactor activity was increased relative to the vWF antigen (vWF:Ag) concentration (a ratio of 2.55 +/- 0.36; normal range, 0.8 to 1.4); 4 of the 6 also had a similar and abnormal vWF multimer pattern--an increased proportion of the fastest moving bands. In the other 2, the multimer pattern was normal. Of the other 30 patients, a mild increase in ristocetin cofactor/vWF:Ag was seen in only 2 with secondary pulmonary hypertension and 1 with normal pulmonary artery pressure: these also had an abnormal vWF multimer pattern that was different from that observed in patients with primary pulmonary hypertension. The vWF abnormalities we describe in primary pulmonary hypertension offer a marker of the disease and could be helpful in understanding its pathogenesis.
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PMID:von Willebrand factor abnormalities in primary pulmonary hypertension. 310 58

Airway reactivity is associated with rapid decline in pulmonary function with age or packyears of smoking in otherwise normal subjects. Because heterozygotes for cystic fibrosis (CFHZ) have a higher prevalence of airway reactivity than do controls, we tested the hypothesis that CFHZ have a more rapid decline in pulmonary function with age or packyears of smoking than do controls. We performed spirometry on 280 parents of children with CF and 280 age-matched control parents of children with heart disease. The ALA-DLD-78 questionnaire was also administered to these volunteers. Although the CF parents report wheezing apart from a cold more frequently than control parents, they show no greater decline of pulmonary function (FEV1, FEF25-75%, or FEV1/ht3) with age or packyears of smoking than do controls. The complaint of wheezing apart from a cold contributes significantly to the variance of FEV1 and FEF25-75% even when the effects of height, age, packyears of smoking, sex, and CF heterozygosity are controlled. The hypothesis that obligate heterozygotes for CF have increased age- or smoking-related decline in airflow is not supported by these data. Instead, the results suggest that any pulmonary function abnormalities associated with CF heterozygosity are small and not clinically significant and that CF heterozygosity per se is not a major risk factor for development and progression of obstructive airways disease.
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PMID:Pulmonary function in obligate heterozygotes for cystic fibrosis. 319 30

Parents of children with cystic fibrosis have been reported to have a high prevalence of increased airway reactivity, but these studies were done in a select young, healthy, symptomless population. In the present study respiratory symptoms were examined in 315 unselected parents of children with cystic fibrosis and 162 parents of children with congenital heart disease (controls). The cardinal symptom of airway reactivity, wheezing, was somewhat more prevalent in cystic fibrosis parents than in controls, but for most subgroups this increased prevalence did not reach statistical significance. Among those who had never smoked, 38% of obligate heterozygotes for cystic fibrosis but only 25% of the controls reported wheezing (p less than 0.05). The cystic fibrosis parents who had never smoked but reported wheezing had lower FEV1 and FEF25-75, expressed as a percentage of the predicted value, than control parents; and an appreciable portion of the variance in pulmonary function was contributed by the interaction of heterozygosity for cystic fibrosis with wheezing. For cystic fibrosis parents, but not controls, the complaint of wheezing significantly contributed to the prediction of pulmonary function (FEV1 and FEF25-75). In addition, parents of children with cystic fibrosis reported having lung disease before the age of 16 more than twice as frequently as control parents. Other respiratory complaints, including dyspnoea, cough, bronchitis, and hay fever, were as common in controls as in cystic fibrosis heterozygotes. These data are consistent with the hypothesis that heterozygosity for cystic fibrosis is associated with increased airway reactivity and its symptoms, and that the cystic fibrosis heterozygotes who manifest airway reactivity and its symptoms may be at risk for poor pulmonary function.
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PMID:Pulmonary abnormalities in obligate heterozygotes for cystic fibrosis. 343 35

Twenty patients with massive or recurrent hemoptysis underwent percutaneous transcatheter embolotherapy between 1979 and 1986 for the following diseases: cavitary aspergillosis (n = 4); cystic fibrosis (n = 4); tuberculosis (n = 3); bronchogenic carcinoma (n = 3); bronchiectasis (n = 3); small cell lung carcinoma 6 years after irradiation (n = 1); congenital heart disease, after Glenn and Blalock anastomoses (n = 1); and unknown interstitial disease (n = 1). Bronchial arteries were embolized in all but one patient. In nine patients (45%) nonbronchial systemic collateral arteries contributed significantly to areas of pathologic pulmonary tissue and frequently were the major arterial supply. These nonbronchial systemic collaterals included branches of the subclavian and axillary arteries (n = 7), intercostal arteries (n = 5), and phrenic arteries (n = 3) and accounted for 59.5% of the total number of arteries embolized. Recognition and occlusion of nonbronchial systemic collaterals providing blood to hypervascular pulmonary lesions is essential for successful percutaneous embolotherapy of hemoptysis.
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PMID:Nonbronchial systemic collateral arteries: significance in percutaneous embolotherapy for hemoptysis. 361 66


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