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

The course of the left recurrent laryngeal nerve through the chest brings it in close proximity with the heart and great cardiac vessels. Diseases of the heart and the great vessels are known to cause vocal cord paralysis, probably by mechanical injury to the recurrent laryngeal nerve. Pulmonary artery hypertension and dilation occur in up to 80% of patients with cystic fibrosis. We report a case of a 23-year-old woman with cystic fibrosis and left vocal cord paralysis. We believe that sudden pulmonary artery expansion produced recurrent laryngeal nerve injury and vocal cord paralysis. This is only the second association of unilateral vocal cord paralysis and cystic fibrosis in the medical literature. The pathophysiology of the cardiovocal syndrome is discussed.
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PMID:Vocal cord paralysis associated with cystic fibrosis. 368 57

A combined morphometric, ultrastructural, and biochemical study was done on carotid bodies (CBs) obtained at autopsy from 213 patients in a pediatric and young adult population. The objective was to determine whether this group had statistically significant differences in sudden infant death syndrome (SIDS, n = 38), cystic fibrosis (CF, n = 30) and cyanotic heart disease (CHD, n = 17), compared with an age-matched control population (n = 128). Average combined weights of CBs in CF and CHD were significantly greater than those of controls in most age intervals (Student t test, P less than 0.05), and computerized planimetry showed an increase in both total surface area and area of "functional" parenchyma. There was diminished chief cell argyrophilia in 72% of CF CBs, and in 8 cases studied ultrastructurally there was moderate to marked depletion of dense-core neurosecretory granules. Most CBs from patients with CHD showed intense cytoplasmic argyrophilia similar to that of controls. Quantitative analysis for tissue catecholamines showed that dopamine was present in greatest concentration in each group of patients but was significantly elevated in CHD. There were no significant differences in morphometry, ultrastructure, or catecholamine content of CBs from SIDS victims, compared with age-related controls. These data add further support to CBs having a chemoreceptor role in humans with compensatory hypertrophy and hyperplasia occurring in most patients with chronic hypoxia due to CF and CHD. There were no significant findings to indicate that CBs play a direct role in the etiopathogenesis of SIDS.
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PMID:Carotid body hyperplasia in cystic fibrosis and cyanotic heart disease. A combined morphometric, ultrastructural, and biochemical study. 399 43

Cardiomyopathy in cystic fibrosis (CF) is an unusual heart disease, mainly characterized by a multifocal fibrosis of the left ventricle. The disorder chiefly occurs in the age group of 1-2 years and leads to fatal cardiac failure. The causal pathogenesis of the disease has not been discovered up to now. In two cases of CF-associated cardiomyopathy we found an oedema (mainly lymphoedema) of the myo- and epicardium and a lymph stasis in lymph vessels and lymph nodes of the heart. Based on a comparative study using animal models we speculate that a) CF may be complicated by a disorder of cardiac lymph circulation, and b) chronic cardiac lymphoedema of the heart in CF can cause focal myocardial damage with fibrosis.
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PMID:Cardiomyopathy in cystic fibrosis: lymphoedema of the heart with focal myocardial fibrosis. 621 64

An increase in hemoglobin concentration characterizes the normal compensatory response to chronic tissue hypoxia. We observed no such increase in 42 chronically hypoxic patients with cystic fibrosis, in whom the mean concentration was 12.6 gm/dl; one third of the patients were anemic. Compared with patients with cyanotic heart disease, patients with cystic fibrosis did not have a compensatory increase in P50 or 2,3-diphosphoglycerate. Despite anemia, erythropoietin levels in patients with cystic fibrosis were not significantly different from normal control values. The growth of colony-forming units-erythroid in patients with cystic fibrosis was similar to that in control subjects, and there was no inhibition of growth with the addition of autologous serum. Erythropoietin sensitivity, determined by measuring the CFUe dose response curve, was normal in both patients and controls. Results of iron studies were consistent with iron deficiency in the majority of patients. Impaired absorption of iron was observed in six of 13 iron-deficient patients with cystic fibrosis. An inverse correlation between erythrocyte sedimentation rate and peak serum iron was obtained during the iron absorption study. Eight patients who underwent a therapeutic trial of iron demonstrated a 1.8 gm/dl rise in hemoglobin concentration. Two patients with previously documented iron malabsorption responded to parenteral iron therapy after failure to respond to oral supplementation. These studies demonstrate that patients with cystic fibrosis not only have an impaired erythroid response to hypoxia, but are frequently anemic. Their inadequate erythroid response to hypoxia results in part from disturbances in erythropoietin regulation and iron availability.
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PMID:Inadequate erythroid response to hypoxia in cystic fibrosis. 673 32

We reviewed 94 consecutive episodes of pyogenic brain abscess seen at Children's Hospital Medical Center, Boston, between 1945 and 1980. After 1970, the mortality as reduced from 36% to 14%. Predisposing factors included congenital heart disease, otitic and sinus infections, closed head injuries, and cystic fibrosis. There were seven patients younger than 5 months of age. In one patient with Fallot's tetralogy, an abscess recurred at the site of retained thorium dioxide (Thorotrast) after an 11-year interval. The continuing substantial mortality is attributed to the presence of coma at the time of treatment, hemorrhagic complications of tapping abscesses, and the location of abscesses in deep brain structures. The early detection and successful treatment of brain abscesses in children remains a clinical challenge.
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PMID:Cerebral abscess in children. 727 May 21

Is an acute bronchial, obstructive disease of the infant caused mainly by the respiratory syncytial virus. It appears epidemically preceded by infections of the upper respiratory ducts, followed by coughing, dysnea, expiratory sibilants, suprasternal and subcostal during inspiration and radiologic evidences of choneking. In the differential diagnosis the physician must consider pulmonary dysgenesis, diaphragmatic hernia, congenital lobar emphysema, congenital cardiopathy, pneumothorax, obstruction due to foreign body, asthmatic crisis and fibrocystic disease. Fundamentally, two diagnoses should be discarded: 1) dyspenic bacterial bronchopneumonic syndrome; 2) prime infection T. B. bronchopneumonia with bronchiolitic syndrome.
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PMID:[Bronchiolitis]. 742 29

The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplantation program, which began in 1982. Twenty pediatric patients (age range 3 to 18 years) have had heart-lung (n = 11), double lung (n = 8), and single lung (n = 1) transplantation procedures. The causes of end-stage lung disease were primary pulmonary hypertension (n = 7), congenital heart disease (n = 5), cystic fibrosis (n = 4), pulmonary arteriovenous malformation (n = 2), graft-versus-host disease (n = 1), and desquamative interstitial pneumonitis (n = 1). Four (20%) patients had thoracic surgical procedures before the transplantation operation. The survival was 80% at a mean follow-up of 2 years. Immunosuppressive drugs included cyclosporine (n = 9) or FK 506 (n = 11) based therapy with azathioprine and steroids. Children were followed up by means of spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection was 1.4 at 30 days, 0.5 at 30 to 90 days, and 1.4 at more than 90 days, and the first treated rejection episode occurred on average 28 days after the operation. Obliterative bronchiolitis developed in four (25%) of 16 patients surviving more than 100 days. Results of pulmonary function tests have remained good in almost all recipients. The greatest infectious risk was that of cytomegalovirus: one death and one case of pneumonia. Posttransplantation lymphoproliferative disease was diagnosed in two (12.5%) patients; both recovered. The most common complications were hypertension (25%) and postoperative bleeding (15%). Early results indicate that lung transplantation is a most promising therapy for children with severe vascular and parenchymal lung disease.
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PMID:Pediatric lung transplantation. The years 1985 to 1992 and the clinical trial of FK 506. 767 72

Heart-lung transplantation is a complex, expensive, and resource-intensive procedure. It is performed more often where there is coexistence of end-stage pulmonary disease with advanced cardiac disease that is not secondary to pulmonary hypertension (e.g., some forms of congenital heart disease, some situations with Eisenmenger's syndrome, and possibly the coexistence of end-stage heart and end-stage lung disease). Available data indicate that some disease states are associated with a higher probability of successful outcome after HLT than are others. Objectively validated and reliable patient selection criteria cannot be obtained from the current medical literature. Expert opinion provided to OHTA contained varying degrees of detail and uniformity with respect to patient selection (Tables 11 and 12). While most agree that "irreversible cardiopulmonary disease" is an indication for this procedure, there are no published data that have provided details as to how patients so designated were selected as candidates for HLT. For example, for chronic obstructive pulmonary disease (emphysema), neither expert opinion nor published studies of HLT provided data indicating the stage of the disease at which a decision to transplant is reasonable and objectively justifiable. A similar lack of detail for cystic fibrosis, bronchiectasis, pulmonary fibrosis, sarcoidosis, asbestosis, and other diseases was noted. Thus, there are insufficient data regarding transplant recipients to permit the development of objective criteria for a threshold for HLT in the various disease states. The circumstances are more problematic regarding suggested contraindications for HLT. While objective survival rates obtained from registry data indicated that survival bore little relationship to recipient age, various opinions provided to OHTA specified a maximum age for HLT candidates, ranging from under 45 years to 60 years. Adverse factors and contraindications, such as "other significant disease," "recurrent pulmonary emboli," "hepatic disease," "infection," and "insulin-dependent diabetes" were nonspecific and unsupported by published, objective evidence. More ambiguous contraindications included "psychiatric illness," "poor family support structure," "history of noncompliance with medical regimens," and lack of "emotional stability." While psychiatric, psychologic, or sociologic evaluations of potential HLT recipients undeniably have relevance to patient selection, use of such criteria should be supported at least by evidence of generally consistent application across the transplant community. Of note was the recommended requirement that the recipient possess "adequate financial resources," which implies that the benefits of HLT are not expected to be equitably provided to the public.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Institutional and patient criteria for heart-lung transplantation. 771 18

The peak velocity for fetal weight gain occurs in the last few weeks of pregnancy. As the fetus matures, it swallows and absorbs an increasing amount of amniotic fluid, which contributes to the growth of the fetus. The authors studied cases of small intestinal atresia (IA) treated over 9 years to determine whether amniotic fluid has any nutritive role in the development of human fetus, and if it does, at what stage of gestation is the contribution of amniotic fluid significant to fetal nutrition. Fifty-nine newborns had IA (24 jejunal, 35 ileal). Ten of the patients had associated anomalies (3 cystic fibrosis, 2 congenital heart disease, 1 neural tube defect, 1 microcephaly, 2 malrotation, 1 vesicoureteric reflux). Among the 24 babies with jejunal atresia, one was a twin, and birth weight was not recorded for another. These two patients were excluded from the study. Of the remaining 22 patients with jejunal atresia, 10 were born before 36 weeks' gestation; only five of 35 patients with ileal atresia were born before 36 weeks' gestation. Fourteen patients were below the 10th percentile for birth weight after correction for gestational age, one was born before 36 weeks, and 13 were born after 36 weeks. Five (41.7%) of the 12 patients with jejunal atresia who were born after 36 weeks' gestation were underweight, as were eight (26.7%) of the 30 patients with ileal atresia. Thus, it appears that amniotic fluid contributes to the fetal growth in the last few weeks of gestation, and the higher the obstruction in the small intestine, the more pronounced the effect on the nutrition of the fetus.
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PMID:Small intestinal atresia: effect on fetal nutrition. 780 60

Forty children (aged 1 to 18 years, 27 female and 13 male) have undergone heart-lung (21), double lung (17), and single lung (2) transplant procedures at our center from 1985 through April 1994. The indications for transplantation have been diverse, primary pulmonary hypertension (10), cystic fibrosis (11), congenital heart disease (10), arteriovenous malformation (3), emphysema (1), graft-versus-host disease (1), rheumatoid lung (1), cardiomyopathy (1), desquamative interstitial pneumonitis (1), and Proteus syndrome (1). The actuarial 1-year survival was 73% (mean follow-up 2 years). One-year actuarial survival for disease groups ranged from 60% for cystic fibrosis to 90% for congenital heart disease. We have identified six issues critical to the patient and programatic survival of pediatric lung transplantation. Our experience and management strategies in these areas are reviewed. Cytomegalovirus: Cytomegalovirus disease developed in six of eight patients with cytomegalovirus mismatching (donor +/recipient-) and in seven of 32 patients who survived more than 30 days (23%). All but cytomegalovirus donor -/recipient- patients were treated with ganciclovir for 4 weeks after transplantation. Obliterative bronchiolitis: Obliterative bronchiolitis developed in seven of 32 (25%) patients who survived more than 30 days. Obliterative bronchiolitis was manifest within the first posttransplantation year as a rapid decline in small airway function. Aggressive augmentation of immunosuppression has been used with little success. Posttransplantation lymphoproliferative disease: Posttransplantation lymphoproliferative disease developed in five of 32 (15%) patients who survived more than 30 days developed. One patient died (17% mortality) despite retransplantation. In four patients the disease resolved with reduction in immunosuppression alone, and one required the addition of interferon alfa. Cystic fibrosis: We have changed our management strategies to avoid triple drug immunosuppression, perioperative blood and bronchial cultures, aggressive antimicrobial therapy, and exclusion of patients with panresistant organisms; this has resulted in elimination of infectious mortalities thus far in the pediatric cystic fibrosis group. Airways: In 21 heart-lung recipients with tracheal anastomoses we have had no airway complications. The double and single lung transplant recipients accounted for 34 bronchial and one tracheal anastomoses. Three (9%) bronchial stenoses developed. Two were treated with silicone stents and one with balloon dilation.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Critical issues in pediatric lung transplantation. 781 8


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