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

The carotid bodies from 71 patients ranging in age from 28 weeks' gestation to 30 years were obtained at autopsy. Patients were divided into two groups based on the presence or absence of chronic hypoxemia. There was a high correlation between the weight of individual carotid bodies in each case. Among the 12 patients with chronic hypoxemia, eight patients had carotid bodies heavier than predicted by statistical analysis. Of these eight patients, six had cystic fibrosis and two had cyanotic heart disease. Morphometric and cell population analyses of the carotid bodies from these eight patients and from those of the control population indicated that enlargement of the carotid bodies during normal or abnormal growth results from proportionate increases in lobule parenchyma and stroma. There was also an increase in the width and length of the lobules without an increase in the diameter of the cell cords or a change in the size or proportion of the chief cells. Growth and development of the carotid bodies were studied in a control group of 59 patients without chronic hypoxemia. There were no sex related differences in carotid body weights. The combined weight of the carotid bodies correlated most strongly with body weight, although there was some correlation with age and body length. A regression equation reflecting the data relating to body weight (BW) is: Combined weight of carotid bodies (in mg.) = 0.29 BW (in kg.) +3.0. Leukemic infiltrates were present in two patients with acute lymphocytic leukemia, and diffuse lymphocytic infiltration with nodule formation was present in one patient with mental retardation. Metaplastic cartilage was present in a carotid body of one patient.
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PMID:Carotid body hypertrophy in patients with cystic fibrosis and cyanotic congenital heart disease. 84 53

Simultaneous red blood cell (RBC) and plasma volume determinations were obtained in 16 patients with cystic fibrosis (CF) and moderately severe pulmonary involvement. Hypervolemia with an increase in both RBC and plasma volumes was observed. Changes in blood volume were marked when values were indexed by weight but less significant when indexed by height. Decreasing systemic arterial oxygen saturation was associated with a progressive increase in RBC mass, hematocrit value, and hemoglobin level and a decrease in mean corpuscular hemoglobin concentration. RBC and total blood volumes were highest in patients with cor pulmonale and congestive heart failure. However, the compensatory polycythemic response in patients with CF was inadequate when compared with the response to hypoxemia in patients with cyanotic congenital heart disease. The insufficient oxygen-carrying capacity may compromise tissue oxygen delivery and necessitate treatment.
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PMID:Blood volume changes in patients with cystic fibrosis. 85 May 98

Thirty-seven (1-6%) tracheas from 2170 children showed squamous metaplasia. (Cases with tracheo-oesophageal fistula and congenital heart disease were excluded). The metaplasia extended into the bronchi in 15 cases. Features of pulmonary retention were present in seven cases. Respiratory infection, probably viral, seemed to be the most significant causative factor in 20 children, including those with cystic fibrosis. Tracheal instrumentation was a possible factor in 11 cases but oxygen therapy alone did not seem important. The metaplasia was almost certainly congenital in one child and probably in two others but no stillborn infants showed metaplasia. In many children the metaplasia seemed to be due to a combination of factors.
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PMID:Squamous metaplasia of the tracheal epithelium in children. 94 Nov 6

Initial experience of home enteral nutrition (HEN) was gained from malnourished patients with Crohn's disease. The rationale for HEN was to improve the patients' lifestyle by reducing the need for repeated admissions for nutritional support: this method is extremely useful in correcting nutritional problems. Over the past ten years the use of HEN has expanded to cover other clinical areas including correction of growth retardation secondary to gastrointestinal disease, cystic fibrosis, inborn errors of metabolism, congenital heart disease, and chronic renal failure, in addition to many types of neoplasia and chronic neurological diseases. At the present time, approximately 150 patients receive HEN within the catchment area of the Greater Glasgow Health Board (population 940,000). Despite the increasing availability of HEN many clinicians and dietitians are still reluctant to consider HEN as a 'routine adjunct' to clinical management, claiming that it is too dangerous or complicated. The aims of this article are to explain our method of running a HEN service, offer advice on practical problems and discuss further developments and potential difficulties.
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PMID:Home sweet HEN--a guide to home enteral nutrition. 145 96

Heart-lung transplantation was, for many years, conceptualized as a possible treatment for patients with combined end-stage cardiac and pulmonary disease. As experience grew with heart transplantation, particularly in the 1970s, the difficulties of performing the orthotopic operation in patients with fixed pulmonary hypertension became apparent. This further impetus for combined heart-lung transplantation led to successful animal experiments in the late 1970s, and the first successful heart-lung transplant operation was performed in 1981. There has been significant evolution in the operative technique for the recipient operation, with emphasis on preservation of the phrenic, vagal, and recurrent laryngeal nerves and on meticulous hemostasis, with particular attention to the bronchial vessels of the posterior mediastinum. Donor procurement is of critical importance to the success of the operation, and criteria for donor selection have been well established. Lung preservation remained, for many years, a significant limitation, but current techniques involving the use of prostaglandin E1 have led to safe distant procurement with ischemic times up to 6 hours. The heart-lung transplant operation remains an effective modality for the treatment of patients with congenital heart disease, primary pulmonary hypertension, chronic obstructive pulmonary disease, and cystic fibrosis.
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PMID:Technique of combined heart-lung transplantation. 148 24

Hypertrophic osteoarthropathy should be considered in any child who presents with a non-inflammatory synovitis of the knees, ankles and wrists and particularly if there is finger clubbing or soft tissue hypertrophy of the fingers. It is rare to get the typical facial appearances until after adolescence. Alteration in epiphyseal growth is associated with the periosteal reaction, as is change in the shape of the patella; whether this leads to premature osteoarthrosis is not yet certain. Secondary disease is common in cyanotic congenital heart disease, but its frequency appears to be decreasing with early treatment of such children. Chest infections are also better controlled so again it is less common, but does still occur, particularly in cystic fibrosis in the older age group who have intractable chest infections. It is seen but rarely in disorders such as inflammatory bowel disease or pulmonary metastases, particularly from bone tumours.
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PMID:Hypertrophic osteoarthropathy in the paediatric age. 162 66

Because cystic fibrosis (CF) epithelia have ion transport abnormalities that may in part be regulated by intracellular calcium metabolism, and the kidney is actively involved in both ion transport and calcium homeostasis, we have investigated renal calcium handling in CF. Twenty-four-hour urine collections were analyzed in 34 CF patients (age 5 to 35 years) and kidney ultrasound studies were performed in 17 CF patients (age 6 months to 23 years). Renal histologic findings at postmortem examination of 14 CF patients (age 4 months to 23 years) were compared with those of 12 patients (age 11 months to 17 years) with other chronic illnesses (6 congenital heart disease, 6 malignancy). In 30 of the 34 CF patients urinary calcium excretion was normal (less than 4 mg (0.1 mmol)/kg/24 hr). Four CF patients had hypercalciuria (calcium excretion 4.4 to 8.8 mg (0.11 to 0.22 mmol)/kg/24 hr). However, these patients had other possible explanations for hypercalciuria, such as immobilization (n = 2), increased dietary sodium load (n = 1), and glucocorticoid therapy (n = 1). None of the 17 patients examined by renal ultrasonography had nephrocalcinosis. Five CF patients had histologic evidence of sparse nephrocalcinosis at autopsy. However, 6 of 12 autopsy kidney specimens from patients with other chronic illnesses and similar preterminal events also showed nephrocalcinosis. The hypercalciuria and nephrocalcinosis in CF and other chronic debilitating diseases may be explained by factors known to affect calcium handling. Our evidence does not support a primary renal defect as the basis of hypercalciuria and nephrocalcinosis in CF.
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PMID:Renal calcium handling in cystic fibrosis: lack of evidence for a primary renal defect. 169 Jul 95

Two hundred and twenty-one disabled children from seven diagnostic groups have been examined with respect to height, weight and prevalence of four different feeding problems. Retarded growth and feeding problems were common in children with cerebral palsy, mental retardation, congenital heart disease and deaf-blindness, but rare in children with esophagus atresia, cystic fibrosis and epilepsy. Mean relative height and weight were significantly lower (p much less than 0.01) in children with mechanical feeding problems, such as impairment of self-feeding skills and oral-motor dysfunction, than in children without these problems, regardless of diagnostic group. Mean relative weight was also significantly lower in children with poor appetite than in children with good appetite. Feeding problems contribute to short stature and underweight in severely disabled children.
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PMID:Feeding problems, height and weight in different groups of disabled children. 183 17

We have performed heart-lung transplantation in 10 children for the preoperative diagnoses of primary pulmonary hypertension (4), complex congenital heart disease with pulmonary hypertension (4), pulmonary atresia (1), and cystic fibrosis (1). Ages ranged from 4 months to 18 years. There were 15 episodes of pulmonary rejection, with an occurrence rate of 1.67 episodes per patient. Pulmonary infections occurred frequently, with an occurrence rate of 3.3 episodes per patient. The actuarial survival rate at 1 and 2 years was 78% and 47%, respectively. Patient attrition between 1 and 2 years was attributable to the complications of obliterative bronchiolitis, which has effected 71% (5/7) of the long-term survivors. Four of the 5 surviving children have minimal physical limitation and are in functional class I. These data support continued investigation into heart-lung transplantation in children and set the stage for further program development into single-lung transplantation in children.
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PMID:Heart-lung transplantation in infants, children, and adolescents. 205 4

As part of a longitudinal study, children in three groups (healthy, n = 33; cystic fibrosis (CF) n = 28; congenital heart disease (CHD) n = 23) were seen between the ages of 12 and 18 months to assess infant-mother attachment and at two years to observe parent-child negotiation of autonomy/dependence in a puzzle task. Although the healthy group included the highest proportion of securely attached infants and the CHD group the lowest, the distribution of attachment patterns did not differ significantly between groups, or between any of the groups and established norms. At two years the healthy children had the most positive experience in the puzzle task while the children in the CF group had the least positive experience. When diagnostic groups were divided according to prior attachment status, the data for the healthy group were consistent with the prediction that securely attached children would have the most positive mother-child interaction at age two. This was not the case in either of the medically diagnosed groups. These data suggest that the influence of the early parent-child relationship may be altered by a child's health status. We may need different models to account for development in healthy and medically compromised children.
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PMID:Early diagnosed chronic illness and mother-child relationships in the first two years. 228 26


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