Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022116 (ischemia)
 91,303 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Unilateral lung transplantation has become a successful method for the treatment of end-stage pulmonary disease, whereas double-lung transplantation has provided benefit to patients with nonfibrotic lung disease such as emphysema and cystic fibrosis. In the past 5 years, 16 single-lung and 13 double-lung transplantations have been performed by the Toronto Lung Transplant Group in patients with end-stage lung disease. Seven perioperative and two late deaths have been recorded so far. Since the introduction of heart-lung transplantation at Stanford in 1981 and at Pittsburgh in 1982 for the treatment of Eisenmenger's syndrome and terminal pulmonary vascular disease, more than 350 combined heart-lung transplantations have been carried out throughout the world. Presently, the 2-year actuarial survival is about 62%. The long-term results have not yet reached the same level of success as those of cardiac transplantation alone. Although several factors have played a role in this difference, a prominent cause has been the lack of a reliable and simple method for pulmonary protection against prolonged ischemia. Most of the techniques proposed against ischemia can be classified as normothermic or static hypothermic cardiopulmonary preservation. The use of the normothermic method has not always been successful. For this reason, interest has now been directed toward the potential for hypothermic preservation of the heart-lung bloc and the use of free-radical scavenger therapy in the reduction of reperfusion injury.
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PMID:Lung and heart-lung transplantation. 218 26

In a 2-year-old boy with untreated cystic fibrosis, an acute deterioration of his chronic respiratory insufficiency developed due to bilateral pneumonia. This condition caused acute right-sided heart failure and nontransmural myocardial infarction of the inferior wall. In concordance with this diagnosis, a marked increase of CPK-MB levels combined with transient severe ischemia on the ECG and the absence of myocardial injury at echocardiography was seen. At 3 years follow-up, he was in good clinical condition.
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PMID:Nontransmural myocardial infarction as a complication of untreated cystic fibrosis. 232 73

We previously described a technique for en bloc double-lung transplantation that was initially applied to select patients with cystic fibrosis and emphysema. This procedure is quite complex and associated with several limitations, including a substantial incidence of airway ischemia, postoperative myocardial depression, and cardiac denervation. To address these problems we have developed a simpler procedure for replacing both lungs. The operation is done through a transverse thoracosternotomy and involves sequential replacement of the two lungs. Positive features include separate bronchial anastomoses to reduce ischemic airway complications, elimination of the need for total cardiopulmonary bypass and a period of ischemic cardiac arrest, improved exposure to reduce intraoperative and postoperative hemorrhage, and maintenance of cardiac innervation. Additionally, the technique can be more easily mastered and widely applied. Details of the procedure and its initial clinical application in 3 patients having emphysema, cystic fibrosis, and bronchiolitis obliterans following previous double-lung transplantation, respectively, are described. All 3 patients recovered without complication. Postoperative function was excellent in spite of lung ischemic times ranging up to 91/2 hours.
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PMID:Improved technique for bilateral lung transplantation: rationale and initial clinical experience. 233 34

Primary care physicians have been very cooperative in referring screened patients to the two designated CF centers in Wisconsin--the University of Wisconsin Cystic Fibrosis Center, and the center at the Medical College of Wisconsin in Milwaukee--and their help has made this study possible. By 1990, we anticipate that meaningful clinical comparisons between the screened and control groups will be possible, and at that time we can begin to obtain some definitive answers concerning the benefits and potential risks of neonatal screening for cystic fibrosis. At this time, it would be premature to make a decision concerning the efficacy of screening for cystic fibrosis for the State of Wisconsin. It is very important that the study go to completion before making conclusive recommendations. We are eager to meticulously document the natural history of CF by following study patients for a long time. Answers to questions concerning rate of decline of the IRT value in true positives, psychosocial risks of screening to true positives, effect on future reproductive plans, and the cost effectiveness of the screening program will not be available for at least two more years. False positive IRT results seem to be related to perinatal asphyxia. We postulate the mechanism is ischemia in the pancreas related to hypoxia during the perinatal period leading to transient release of trypsin from the pancreas into the bloodstream. Decline of the IRT result over time is of great interest because a repeat blood sampling approach would hopefully eliminate several false positives.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Neonatal screening for cystic fibrosis in Wisconsin. 272 72

Vasoactive intestinal peptide (VIP), first isolated from the gut, was originally considered a candidate gastrointestinal hormone. Since about 1975, however, it has become increasingly clear that it is primarily a neurotransmitter or neuromodulator and that it exerts its functions mainly by local release from nerve endings. VIP plays a hormonal role only when it is released in large amounts from a tumor, with a consequent overflow into the circulation and grossly elevated plasma concentrations of the peptide. Moderately increased VIP plasma and tissue concentrations that cause mainly local effects are found in intestinal ischemia. Crohn's disease and some other chronic inflammatory diseases of the bowel. VIP is also measured in increased amounts in the normal fetus and neonate, where it may play an important physiological role. Such an increase of VIP levels in the circulation could enhance perfusion and metabolic activity of tissues during their rapid-growth period. On the other hand, disorders with a disturbed VIP function such as achalasia and Hirschsprung's disease and possibly also asthma and cystic fibrosis seem to be characterized mainly by a derangement of smooth muscle activity and/or exocrine secretion. Considering this list of disorders where VIP has either a proven or suspected role, it is easy to imagine the significance of this peptide in pediatric pathophysiology.
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PMID:[Vasoactive intestinal polypeptide (VIP)--possible importance in diseases of childhood]. 299 82

The pancreases of 17 patients who had cystic fibrosis with and without diabetes mellitus were evaluated at autopsy by routine staining and immunohistochemical methods for insulin, glucagon, somatostatin, and pancreatic polypeptide. Qualitative assessment of the number of islets of Langerhans and the degrees of exocrine pancreatic atrophy, fibrosis, and fat replacement was made for each pancreas. Quantitative assessment of islet composition was performed in 15 of the 17 based on the immunochemical reactivity of each cell type. Nondiabetic patients with cystic fibrosis in the latter part of the first decade of life have classic fibrocystic changes of the pancreas, with some persisting exocrine tissue, islets that appear normal, and prominent nesidioblastosis. The latter process may protect these patients from glucose intolerance. Young adult diabetic patients with cystic fibrosis have total loss of exocrine pancreas with fat replacement, lack of nesidioblastosis, a qualitative decrease in the number of islets, fibrosis of and amyloid deposits in islets, decreased numbers of insulin-containing cells in each islet, and atrophy of islet cells, probably resulting from progressive ischemia. Although the potential exists for an increasing incidence of diabetes mellitus in patients with cystic fibrosis as their life spans increase, individual variation occurs in this disease.
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PMID:Endocrine pancreas in cystic fibrosis: an immunohistochemical study. 636 38

Iatrogenic pathology of the optic nerve is examined according to a framework which distinguishes direct and indirect effects on the optic nerve. Direct effects due to toxic drugs should be suspected when unexplained, usually bilateral loss of visual acuity occurs. The 3 clinical stages of classical optic toxic neuropathy are 1) anomalies of color vision, 2) loss of visual acuity and narrowing field of vision, and 3) papillary palor corresponding to irreversible optic atrophy. Usually only the 1st stages are reversible, but the reversibility may be incomplete. The list of drugs which can cause such effects is lengthy and includes antiinfectious drugs such as sulfamides and derivatives of hydroxyquinoleins, chloramphenicol especially when used to treat cystic fibrosis of the pancreas in children, the antituberculins ethambutol in high doses and isoniazide, which occasion particular risks when combined; antiparasitics such as quinine and its derivatives chloroquine and hydroxychloroquine, which cause optic neuropathy through their effect on the retina; arsenic pentavalents such as tryparsamide, quinacrine, trecator and mystatin; drugs affecting the central nervous system such as monoamineoxydase inhibitors, laroxyl, phenothiazine and the barbituates; anticonvulsants such as phenytoin; antimitotics such as vincristine; digitalics, disulfiram; penicillamines, and pexid. The action of lasers on the optic nerve can have a similar effect. The optic nerve may be indirectly damaged during surgical procedures leading to hypotonia, acute ischemia of the head of the optic nerve or embolic accident after a local or regional injection. Damage may also be caused by radiotherapy of intracranial tumors and certain drugs which cause isolated papillary edema or edema associated with headaches, such as Tetracycline, large doses of vitamin A or D, corticoids, and oral contraceptive (OC) pills, which may cause papillary edema through cerebral pseudo-tumors that regress with discontinuation of treatment. This condition has been observed in women with uncontrolled hyperlipidemia. It is probable that an alteration ofaxonal transport is at the basis of the neuropathic mechanisms. The 1st step in therapy is the suppression of the toxin, or at least its discontinuation. Some success has been obtained with vitamin B therapy, corticotherapy, zinc, or isaxonine, depending on the specific condition.
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PMID:[Iatrogenic pathology of the optic nerve]. 676 92

The development of the patch electrode by Neher and Sakmann (1976) has revolutionized electrophysiology. The technique not only provided the possibility to expand the electrophysiological studies to most cells, but brought electrophysiology to the molecular level: it is now possible to measure the electrical current through one single ionic channel. The happy marriage between electrophysiology and molecular biology has resulted in a structure-function analysis revealing the molecular substructures of the ionic channel responsible for the processes of permeation and selectivity of activation and inactivation and different types of block. In the future it may become possible to cure diseases by genetic manipulation and to design better drugs to treat disorders, based on the malfunction of ionic channels (e.g. cystic fibrosis, Parkinson, epilepsy, cardiac arrhythmias, ischemia, hypertension).
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PMID:Electrophysiology on the molecular way. 768 63

Right ventricular cardiac function is altered by abnormalities affecting primarily the left-sided cardiac structures, the lungs, or the right-sided cardiac structures themselves. The most common cardiac causes for right ventricular dysfunction are chronic left ventricular ischemia and rheumatic mitral valvular disease. Pulmonary diseases that result in right ventricular dysfunction include pulmonary air-space disease, including emphysema, and pulmonary interstitial and parenchymal diseases, including idiopathic pulmonary fibrosis and cystic fibrosis. Chronic pulmonary vascular disease, including chronic thromboembolism and PPH have a significant effect on right ventricular performance. Common to all of these diseases is elevation of pulmonary vascular resistance with a commensurate increase in right ventricular pressure, resulting in right ventricular hypertrophy. The limited ability of right ventricular myocardium to function in the face of increased pulmonary resistance results in right ventricular dilatation, tricuspid regurgitation, and ultimately right ventricular failure. MR imaging provides direct, noninvasive visualization of the right ventricular chamber as well as the myocardium itself, allowing reliable demonstration of morphologic changes in the size and shape of the ventricle, thickness of the myocardium, and presence of abnormal infiltration by fat or edema. Furthermore, because MR imaging techniques do not depend upon geometric assumptions about the complex shape of the right ventricle, they may be used for accurate and reproducible quantitation of right ventricular volume and myocardial mass.
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PMID:MR imaging of pulmonary hypertension and right ventricular dysfunction. 872 68

Cystic fibrosis (CF) is a disease characterized mainly by altered exocrine gland function that eventually produces irreversible dysfunction of the pancreas and lungs. The respiratory insufficiency that develops in CF patients in the advanced stages of disease can only be corrected at this time by lung or heart-lung transplantation. We describe our experience with 6 terminal phase CF patients who underwent sequential double lung transplantation (SDLT). Anesthesia was intravenous, with exhaustive hemodynamic and respiratory monitoring. During surgery the most frequently encountered hemodynamic complications were low minute volume, arterial hypotension and irregular heart rate. The main respiratory complications were hypoxemia, hypercapnia and pulmonary edema of the implanted lung, which developed in all cases to varying degrees related to the organ's state of preservation and duration of ischemia. Other complications were the need for extracorporeal circulation in 1 case, oliguria and blood loss requiring multiple transfusions. The most critical moments were at the time of clamping the pulmonary artery, the period after revascularization of the donated lung, and at the start of patient ventilation through the first implanted lung so that the second could be implanted. Although our series is small, it is of interest given the limited Spanish experience with lung transplantation in CF patients, and the good early results obtained, which are similar to those reported for other diseases.
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PMID:[Anesthetic complications in sequential bipulmonary transplantation in patients with cystic fibrosis. Apropos of 6 cases]. 884 34


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