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Tolerance of unusually low mixed venous oxygen saturation. Adaptations in the chronic low cardiac output syndrome.

Introduction of a reliable method for continuous monitoring of mixed venous oxygen saturation has focused attention on the therapeutic and prognostic implications of this value. It is generally agreed that mixed venous oxygen saturation in the 40 to 60 percent range is associated with substantial patient morbidity and mortality if not rapidly corrected. However, several patients with chronic low cardiac output syndrome who have had mixed venous oxygen saturation of less than 40 percent for prolonged periods of time and who have not had decompensation were observed. Three representative patients with these findings are described and mechanisms put forth that may account for the observed relations between mixed venous oxygen saturation and mortality in these and other patients.

Adaptation, Physiological↗

Syndromic retinitis pigmentosa: ERG and phenotypic changes.

PURPOSE: Our aim was to review the phenotype and extent of ERG changes in syndromic RP (SRP). PATIENTS AND METHODS: A retrospective review of charts of 82 patients seen over the last 20 years with SRP was carried out. Clinical data were compared with changes in ERG. Full-field ERGs comprised selective rod-driven, maximal dark-adapted mixed responses, and isolated cone-driven signals. Occasionally, ERGs were recorded under brief general anaesthesia. ERG changes were classified as normal, reduced or extinguished. RESULTS: Syndromic RP was diagnosed for the following entities: Usher (45 patients), Laurence-Moon-Bardet-Biedl (LMBB, 17 patients), Kearns-Sayre (10), Batten (6), Refsum (3), Senior-Loken (1). ERG changes varied in every subgroup and were therefore not specific for the syndromes. CONCLUSION: There was wide variation of clinical presentation in SRP, much as seen in isolated RP, often without obvious ophthalmoscopic changes. ERG testing is a prerequisite for differential diagnosis as well as for early detection of multiple handicaps.

Electroretinography↗

The management of irritable bowel syndrome: a European, primary and secondary care collaboration.

OBJECTIVE: To develop recommendations for the diagnosis and management of irritable bowel syndrome for European doctors delivering primary care. These recommendations can be adapted by local medical groups according to their language, custom and health-care systems. METHODS: Twenty-one general practitioners and gastroenterologists from Europe attended a workshop planned by a steering committee. After a state-of-the-art symposium, four working groups considered the following aspects of irritable bowel syndrome management: what to tell the patient, diagnosis, non-medical treatment and psychosocial management. Current and future drug management was reviewed by the steering committee. The resulting recommendations were considered at two plenary sessions during the meeting, and by circulation of the material during development of the manuscript. RESULTS: The process permitted a unique dialogue between general practitioners and gastroenterologists, in which it was necessary to reconcile the specialists' emphasis on thoroughness with the practical, epidemiological and economic realities of primary care. Despite this dichotomy, consensus was achieved. CONCLUSIONS: European general practitioners and gastroenterologists have produced recommendations that emphasize education of the patient, a positive symptom-based diagnosis, diet and lifestyle advice, psychological support and a critical analysis of current specific psychological and pharmacological treatments.

Colonic Diseases, Functional↗

Social support and pain behavior.

Research has shown that social support is generally associated with better adaptation to chronic disease. However, the role that social support plays in adjustment to chronic pain syndromes has received less research attention. The present study was designed to examine the relation of high versus low levels of social support to pain behavior in chronic pain patients. Fifty-one patients with chronic pain completed the Social Support Questionnaire (SSQ) and the McGill Pain Questionnaire. Patients were also observed using a standard observation system to assess pain behaviors. In order to compare pain behaviors displayed by subjects with high versus low levels of social support, a median split was performed on the 2 dimensions of the SSQ: satisfaction with support, and availability of people for support. The results indicated that pain behavior varied as a function of level of satisfaction with social support. Individuals reporting high satisfaction with social support exhibited significantly higher levels of total pain behavior and higher levels of individual pain behaviors such as guarding, rubbing, and bracing. There was no significant difference in total pain behaviors between those high versus low in terms of level of availability of support. There also were no differences between high versus low social support groups in total pain ratings as measured by the McGill Pain Questionnaire. The results are discussed in terms of an operant conditioning perspective on social support. Individuals who are satisfied with the quality of their social support may be satisfied because they receive positive reinforcement from the social environment when they engage in pain behavior.

Adaptation, Psychological↗

Neurobehavioral and psychosocial functioning of children with opsoclonus-myoclonus syndrome.

Childhood opsoclonus-myoclonus syndrome (OMS) is a movement disorder which typically strikes children in the early preschool years, seriously affecting intellectual, social-emotional and general adaptive development. This series of 13 cases with well-documented neurological histories, aged 1.7 to 16.3 years, provides an initial systematic evaluation of these children's neuropsychological, psychosocial and adaptive status. As expected, children with OMS had significantly reduced intelligence and severe speech and motor output problems; however, most of them also demonstrated a range of preserved neurocognitive abilities and impressive goal-directedness and communicative effort. Psychosocial problems included mild behavioral impairment on the Achenbach Child Behavior Checklist, and severe adaptive limitations on the Vineland Adaptive Behavior Scales despite relatively strong social skills. Developmental factors and likelihood of subcortical localization are discussed, and practical guidelines are provided for behavioral and educational management of these children.

Adolescent↗

Chronic fatigue syndrome. A practical guide to assessment and management.

Chronic fatigue and chronic fatigue syndrome (CFS) have become increasingly recognized as a common clinical problem, yet one that physicians often find difficult to manage. In this review we suggest a practical, pragmatic, evidence-based approach to the assessment and initial management of the patient whose presentation suggests this diagnosis. The basic principles are simple and for each aspect of management we point out both potential pitfalls and strategies to overcome them. The first, and most important task is to develop mutual trust and collaboration. The second is to complete an adequate assessment, the aim of which is either to make a diagnosis of CFS or to identify an alternative cause for the patient's symptoms. The history is most important and should include a detailed account of the symptoms, the associated disability, the choice of coping strategies, and importantly, the patient's own understanding of his/her illness. The assessment of possible comorbid psychiatric disorders such as depression or anxiety is mandatory. When the physician is satisfied that no alternative physical or psychiatric disorder can be found to explain symptoms, we suggest that a firm and positive diagnosis of CFS be made. The treatment of CFS requires that the patient is given a positive explanation of the cause of his symptoms, emphasizing the distinction among factors that may have predisposed them to develop the illness (lifestyle, work stress, personality), triggered the illness (viral infection, life events) and perpetuated the illness (cerebral dysfunction, sleep disorder, depression, inconsistent activity, and misunderstanding of the illness and fear of making it worse). Interventions are then aimed to overcoming these illness-perpetuating factors. The role of antidepressants remains uncertain but may be tried on a pragmatic basis. Other medications should be avoided. The only treatment strategies of proven efficacy are cognitive behavioral ones. The most important starting point is to promote a consistent pattern of activity, rest, and sleep, followed by a gradual return to normal activity; ongoing review of any 'catastrophic' misinterpretation of symptoms and the problem solving of current life difficulties. We regard chronic fatigue syndrome as important not only because it represents potentially treatable disability and suffering but also because it provides an example for the positive management of medically unexplained illness in general.

Adaptation, Psychological↗

[Anaesthesia for a non cardiac surgery in a patient with an Eisenmenger syndrome. Interest of a non invasive haemodynamic monitoring].

Patients with an Eisenmenger syndrome have an instuble hemodynamic status. During a general anaesthesia, the intracardiac shunt has to maintain the correct orientation and volume, adapted to each patient, in such a condition, to avoid the risk of hypoxemia and cardiac failure. The haemodynamic monitoring with a Swan Ganz catheter could be useful. But it is necessary to evaluate the advantage and the risks when the technique is used in these pathological circumstances. Moreover, when the cardiac output is measured with the thermodilution technique, the right-left intra cardiac shunt volume, is not taking into account. The continuous haemodynamic monitoring, with a simplified transoesophageal echo-Doppler system, as it was done in this case, allows appreciate the real quantitative variations of the shunt. In this way the more adequate calculation of some others haemodynamic parameters, over all the total systemic vascular resistances, allows a more precise therapeutic approach.

Anesthesia↗

[Pelvic actinomycosis: the value of preoperative diagnosis. Apropos of a case].

Pelvic actinomycosis is a chronic suppurative granulomatous disease caused by an anaerobic Gram positive germ, Actinomyces. This rare disease can be severe and life-threatening. A intra pelvic bridge becomes chronic and produces a pseudo-tumoural syndrome of the pelvis with retroperitoneal infiltration or extension to neighbouring organs. Initially, neoplasia is usually diagnosed, leading to mutilating surgical exeresis before the pathology results reveal the presence of pelvic actinomycosis. The most difficult task is to entertain the diagnosis in a patient with an intrauterine device and poor general health, signs of infection and a pelvic syndrome. We observed such a case where the diagnosis was suspected before surgery. Adapted first intention antibiotic therapy led to spectacular recovery and allowed limited surgery without unnecessary ablation of neighbouring organs.

Abscess↗

Physical activity, muscle, and the HSP70 response.

Selye (1936) described how organisms react to various external stimuli (i.e., stressors). These reactions generally follow a programmed series of events and help the organism adapt to the imposed stress. The heat shock response is a common cellular reaction to external stressors, including physical activity. A characteristic set of proteins is synthesised shortly after the organism is exposed to stress. Researchers have not determined how heat shock proteins affect the exercise response. However, their role in adaptation to exercise and training might be inferred, since the synthetic patterns correlate well with the stress adaptation syndrome that Selye described. This review addresses the 70 kilodalton heat shock protein family (HSP70), the most strongly induced heat shock proteins. This paper provides an overview of the general heat shock response and a brief review of literature on HSP70 function, structure, regulation, and potential applications. Potential applications in health, exercise, and medicine are provided.

Amino Acid Sequence↗

[Clinico-demographic characteristics and features of the social and occupational adaptation of schizophrenics in 3 population groups living in the northeastern region of the European portion of the USSR (clinico-epidemiologic study)].

The authors conducted a clinico-epidemiological study of schizophrenics among the indigenous population, including that living in isolated areas and newcomers, taking into consideration forms of the disease course and employing a uniform syndromal assessment of the mental state. The authors have established general pathogenetic regularities of the course and their relationship with the age and sex. Patients from the indigenous population, particularly of geographically isolated areas, showed a higher incidence of schizophrenia, a more severe clinical picture of the disease and a lower level of social and occupational adaptation as compared to the migrated population.

Adult↗

[Evaluation of therapeutic measures in toxicoseptic states].

Based on observations made in 112 cases of toxico-septic conditions that had developed in surgical and obstetrical conjunctures, the authors propose a unified codification of the major therapeutical means (etiologic therapy, general, non-specific and specific therapy, adapted for functions and organs). The removal, or surgical draining of the septic focus is the most efficient therapy of toxicoseptic conditions of surgical or obstetrical origin. Compared to this method all the other therapeutical methods are secondary or adjuvant. The authors have acquired a vast experience in the use of a device for peritoneal washing-draining in cases with toxico-septic syndrome of surgical and obstetrical origin. General, non-specific therapeutic measures for quantitative and qualitative maintenance of the internal environment of the organism are achieved with relatively large amounts of non-colloidal crystalloid isotonic solutions, administered over the entire duration of the toxico-septic condition. The importance of the specific therapy is stressed, adapted for functions and organs, stressing amost particularly the therapeutical measures in which the authors have acquired extensive experience.

Abscess↗

[Problem of adaptation of patients with the syndrome of early childhood autism].

Sixty-seven children with early childhood autism received therapeutic and corrective treatment in a day-time clinic. In 37 cases socialization of children proved possible, and following treatment, they were transferred to specialized, logopedic, and general kindergartens and schools. The authors advocate the advisability of setting up day-time clinics in the framework of childhood psychiatric hospitals and of organization of separate groups for children with early childhood autism in the framework of specialized kindergartens.

Adaptation, Psychological↗

Biological basis of drug-induced tolerance, rebound, and dependence. Contribution of recent research on benzodiazepines.

It is proposed that the general biological basis of acquired drug tolerance, of rebound phenomena induced by drugs, and of physiological dependence is a drug-induced adaptive syndrome. Several examples of the compensatory molecular, cellular and system responses are presented that may be induced by the primary drug-induced perturbation in the base-line of various neuronal and non-neuronal activities. Some form of adaptive syndrome is the inevitable consequence of the reciprocal interaction between most or all major classes of drugs and the organism. Knowledge of the molecular and cellular targets of drugs provides an understanding of the various phenomena of the drug-induced adaptive syndrome as well as of the means to avoid or attenuate their potential danger for subjects chronically exposed to drugs. Psychological dependence is discussed as a further factor which, in combination with drug-induced adaptive changes, facilitates drug abuse and, in particular, addiction or drug-seeking behavior. The phenomena of the adaptive syndrome induced by benzodiazepines are discussed against the background of medical science's present advanced knowledge of the molecular and synaptic mechanisms of action of this class of drugs.

Animals↗

[Altitude and the autonomic nervous system].

Ascent to high altitudes arouses the sympathetic nervous system in non-acclimatized healthy humans. Such activation is provoked by hypobaric hypoxia combined with other stressors. While this is an adaptive response, it also contributes to the general physical deterioration consequent to prolonged exposure to high altitudes, and is even implied in specific syndromes: acute mountain sickness, high altitude pulmonary edema (HAPE), and high altitude cerebral edema. Muscular sympathetic nervous activity increases at simulated high altitudes (hypoxic mixture or hypobaric chamber), and HAPE-susceptible subjects show an exaggerated response. Increases in plasma and urinary catecholamines take several days to appear: they are observed rarely at simulated altitudes but frequently on the field. Heart rate variability decreases at high altitudes, particularly in subjects suffering from acute mountain sickness. The low frequency component has been described in healthy subjects as increased or decreased, but is reduced in sympatomatic subjects; increases in the low frequency/high frequency ratio are reported in all studies. Few and discordant data are available regarding baroreflex sensitivity at high altitudes. Some (past and present) authors regard parasympathetic fitness as a defense against illness at high altitudes. Current guidelines advise against high altitude exposure for cardiac patients in whom sympathetic arousal would be too risky.

Altitude↗

Drug administration in chronic liver disease.

Cirrhosis encompasses a range of pathophysiological changes that may alter drug disposition. Drugs that are dependent primarily on the liver for their systemic clearance are more likely to be subject to reduced elimination and subsequent accumulation. Drug accumulation may lead to excessive plasma drug concentrations and adverse effects, if the adverse effects of the drug are concentration-dependent. The effects of hepatic insufficiency on the pharmacokinetics of drugs are not consistent or predictable. Furthermore, the influence of hepatic disease on the disposition of various drugs can vary, even though those drugs may share the same apparent metabolic pathway. Problems in forecasting drug kinetic behaviour are further enhanced by the additional impairment of kidney function (frequently encountered in patients with advanced liver disease) and by the unpredictability of the glomerular filtration rate using customary methods in patients with cirrhosis. Accordingly, dosages are generally adapted empirically, with the help of serum drug concentrations, when applicable. However, drugs eliminated predominantly by hepatic metabolism are not among those most commonly inducing adverse drug reactions or causing severe complications in patients with cirrhosis. Electrolyte disturbances and the hepatorenal syndrome produced by furosemide (frusemide)-the disposition of which is not substantially modified in liver disease-appear to be the most frequent adverse drug reactions in patients with liver disease. Furthermore, clinically significant alterations in the action of medications at concentrations generally considered to be in the normal therapeutic range are not uncommon. Tissue responsiveness to the pharmacological action of some drugs may be modified, as evidenced by the increased susceptibility of the brain in patients with cirrhosis to the action of many psychoactive agents. Another example is the greater susceptibility of such patients to the nephrotoxic potential of aminogly-cosides, which should not be used in this patient group. Drugs may also interfere with adaptive physiological processes induced by liver disease. ACE inhibitors and nonsteroidal anti-inflammatory drugs counteract the enhanced activity of the renin-angiotensin system in advanced liver disease, thereby generating a high risk of excessive hypotension or acute renal failure, respectively. These drugs are best avoided in patients with cirrhosis. Finally, there may be pharmacological effects that overlap with some pathophysiological modifications related to the process of liver disease, such as increased portal pressure produced by some calcium antagonists, or hypoprothrombinaemia related to the inhibition of synthesis of vitamin K-dependent clotting factors by some beta-lactam antibacterials (especially moxalactam and cefamandole). Accordingly, the use of these drugs should be avoided in advanced liver disease. It is noteworthy that reduced drug metabolism in patients with liver disease does not seem to have a significant impact on the frequency of hepatotoxicity. Special caution should be exercised, however, in patients with alcoholic liver disease because excessive alcohol intake may potentiate the hepatotoxic effect of paracetamol (acetaminophen).

Adverse Drug Reaction Reporting Systems↗

[The study of adaptation syndrome in mixed-infection of tick-borne encephalitis and borreliosis in children].

Twenty two children with mixed-infection of tick-borne encephalitis (TBE) and ixodic tick borreliosis (ITB) were studied. Blood hydrocortisone level was changed in 94.5% of the cases. The most significant activation of hydrocortisone secretion in combination with the most pronounced and prolonged general brain manifestations, was detected in infants. Blood hydrocortisone level correlated with clinical symptoms of combined TBE and ITB infections. Along with higher hydrocortisone level, down-regulation of production of antibodies both to B. burgdorferi and to TBE virus was specific for all children studied.

Adaptation, Physiological↗

Aspects of digestive tract tumors in Down syndrome: a literature review.

The purpose of this study was to describe the digestive neoplasms found in persons with Down syndrome. Due to intellectual disability, persons with Down syndrome do not convey their symptoms and pain, leading to delayed diagnosis and potentially worse outcome. It is thus important to know which organs are at risk for tumors and possible tumor risk factors. In a review of the literature, we found 13 benign tumors and 127 cancers in 1 fetus, 8 children, and 131 adults with Down syndrome. The review suggests a decreased incidence of digestive cancer, however, with a possible increased incidence of neoplasms of the pancreas and gallbladder. The distribution of cancers is distinct from that in the general population and that in persons with other intellectual disabilities who share the same life conditions, suggesting that constitutional protective factors exist. This review may allow a more specific, adapted medical follow-up for persons with Down syndrome and could help to elucidate the oncogenesis of digestive neoplasms.

Colorectal Neoplasms↗