Irreversible intestinal failure
Child; Humans; Parenteral Nutrition; Adaptation; infant; Physiological; Gastrointestinal Motility; Intestinal Absorption; Digestive Physiology; Hirschsprung Disease/complications; Intestinal Pseudo-Obstruction/complications/surgery; Intestines/transplantation; Liver Diseases/prevention & control; Malabsorption Syndromes/etiology/therapy; Short Bowel Syndrome/complications/surgery
Intestinal failure (IF) can be defined as the reduction of functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for maintenance in adults or growth in children. In developed countries, IF mainly includes individuals with the congenital or early onset of conditions requiring protracted or indefinite parenteral nutrition (PN). Short bowel syndrome was the first commonly recognized cause of protracted IF. The normal physiologic process of intestinal adaptation after extensive resection usually allows for recovery of sufficient intestinal function within weeks to months. During this time, patients can be sustained on parenteral nutrition. Only a few children have permanent intestinal insufficiency and life-long dependency on PN. Non-transplant surgery including small bowel tapering and lengthening may allow weaning from PN in some cases. Hormonal therapy with recombinant human growth hormone has produced poor results while therapy with glucagon-like peptide-2 holds promise. Congenital diseases of enterocyte development such as microvillus inclusion disease or intestinal epithelial dysplasia cause permanent IF for which no curative medical treatment is currently available. Severe and extensive motility disorders such as total or subtotal intestinal aganglionosis (long segment Hirschsprung disease) or chronic intestinal pseudo-obstruction syndrome may also cause permanent IF. PN and home-PN remain are the mainstays of therapy regardless of the cause of IF. Some patients develop complications while receiving long-term PN for IF especially catheter related complications (thrombosis, sepsis) and liver disease. These patients may be candidates for intestinal transplantation. This review discusses the causes of irreversible IF and emphasizes the specific medico-surgical strategies for prevention and treatment of these conditions at several stages of IF.
2004
Goulet O; Ruemmele F; Lacaille F; Colomb V
Journal Of Pediatric Gastroenterology And Nutrition
2004
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1097/00005176-200403000-00006" target="_blank" rel="noreferrer">10.1097/00005176-200403000-00006</a>
Cellular oxygen sensing need in CNS function: physiological and pathological implications.
Humans; Regional Blood Flow; Physiological; Adaptation; Brain/me [Metabolism]; Hypoxia; Central Nervous System/bs [Blood Supply]; Central Nervous System/me [Metabolism]; Homeostasis/ph [Physiology]; Hypoxia; Oxygen/me [Metabolism]; Transcription Factors/me [Metabolism]; alpha Subunit; Apoptosis/ph [Physiology]; Brain/pp [Physiopathology]; Cell Hypoxia/ph [Physiology]; Gene Expression Regulation; Hypoxia-Inducible Factor 1; NADPH Oxidase/me [Metabolism]; Neovascularization; Pathologic/pp [Physiopathology]; Signal Transduction/ph [Physiology]; Transcription Factors/ph [Physiology]
Structural and functional integrity of brain function profoundly depends on a regular oxygen and glucose supply. Any disturbance of this supply becomes life threatening and may result in severe loss of brain function. In particular, reductions in oxygen availability (hypoxia) caused by systemic or local blood circulation irregularities cannot be tolerated for longer periods due to an insufficient energy supply to the brain by anaerobic glycolysis. Hypoxia has been implicated in central nervous system pathology in a number of disorders including stroke, head trauma, neoplasia and neurodegenerative disease. Complex cellular oxygen sensing systems have evolved for tight regulation of oxygen homeostasis in the brain. In response to variations in oxygen partial pressure (P(O(2))) these induce adaptive mechanisms to avoid or at least minimize brain damage. A significant advance in our understanding of the hypoxia response stems from the discovery of the hypoxia inducible factors (HIF), which act as key regulators of hypoxia-induced gene expression. Depending on the duration and severity of the oxygen deprivation, cellular oxygen-sensor responses activate a variety of short- and long-term energy saving and cellular protection mechanisms. Hypoxic adaptation encompasses an immediate depolarization block by changing potassium, sodium and chloride ion fluxes across the cellular membrane, a general inhibition of protein synthesis, and HIF-mediated upregulation of gene expression of enzymes or growth factors inducing angiogenesis, anaerobic glycolysis, cell survival or neural stem cell growth. However, sustained and prolonged activation of the HIF pathway may lead to a transition from neuroprotective to cell death responses. This is reflected by the dual features of the HIF system that include both anti- and proapoptotic components. These various responses might be based on a range of oxygen-sensing signal cascades, including an isoform of the neutrophil NADPH oxidase, different electron carrier units of the mitochondrial chain such as a specialized mitochondrial, low P(O(2)) affinity cytochrome c oxidase (aa(3)) and a subfamily of 2-oxoglutarate dependent dioxygenases termed HIF prolyl-hydroxylase (PHD) and HIF asparaginyl hydroxylase, known as factor-inhibiting HIF (FIH-1). Thus specific oxygen-sensing cascades, by means of their different oxygen sensitivities, cell-specific and subcellular localization, may help to tailor various adaptive responses according to differences in tissue oxygen availability.
2004
Acker Till; Acker H
The Journal Of Experimental Biology
2004
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1242/jeb.01075" target="_blank" rel="noreferrer">10.1242/jeb.01075</a>
The metabolic events of starvation
Humans; Adaptation; Body Weight; Body Composition; Homeostasis; Alanine/me [Metabolism]; Brain/metabolism; Carbohydrate Metabolism; Fasting; Gluconeogenesis; Ketones/metabolism; Kidney/metabolism; Liver/metabolism; Physiological; Proteins/metabolism; Sodium/metabolism; Starvation/metabolism; Triglycerides/metabolism; Water-Electrolyte Balance
1976
Saudek CD; Felig P
The American Journal Of Medicine
1976
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1016/0002-9343(76)90540-4" target="_blank" rel="noreferrer">10.1016/0002-9343(76)90540-4</a>
Starvation in man
Time Factors; Adaptation; Body Weight; Human; Homeostasis; Fasting; Gluconeogenesis; Physiological; Fatty Acids; Starvation/me [Metabolism]; Triglycerides/me [Metabolism]; Adrenal Cortex Hormones/ph [Physiology]; Alanine/me [Metabolism]; Brain/me [Metabolism]; Glucagon/me [Metabolism]; Glucose/metabolism; Glucose/pd [Pharmacology]; Insulin/me [Metabolism]; Insulin/ph [Physiology]; Kidney/me [Metabolism]; Liver/me [Metabolism]; Nitrogen/me [Metabolism]; Nonesterified/me [Metabolism]; Proteins/me [Metabolism]
1970
Cahill GF
New England Journal Of Medicine
1970
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1016/s0300-595x(76)80028-x" target="_blank" rel="noreferrer">10.1016/s0300-595x(76)80028-x</a>
Hunger disease
Humans; Nutritional Support; Health Status; Time Factors; Adaptation; Energy Metabolism; Body Composition; Physiological; Starvation/metabolism; Adipose Tissue/metabolism; Appetite/physiology; Eating/physiology; Hunger/physiology; Nutrition Disorders/metabolism; Obesity/metabolism/physiopathology; Weight Loss/physiology
This paper examines three aspects of hunger disease: the effect of initial fat stores on macronutrient fuel selection during total starvation (no energy) and how it influences survival; the effects of different rates of weight loss on tissue and body function; and the importance of appetite sensations, including hunger, during malnutrition and during enteral and parenteral nutritional support. Long-term starvation studies in humans reveal major differences in fat carbohydrate and protein metabolism between lean and obese subjects, including a 2-4-fold lower contribution of protein oxidation to energy expenditure in obese subjects, which ensures that more of the excess body fat is oxidized. The rate of weight loss, determined by recent dietary intake, can have major effects on tissue and body function, including wound healing, the acute phase protein response, muscle fatigue and psychological/behavioural function in both clinical and non-clinical settings. In depleted states uncomplicated by disease, changes in appetite sensations can result in energy intakes as high as 6000 to 10,000 kcal/day ( 25-42 MJ/day). Long-term enteral tube feeding and parenteral nutrition are associated with frequent disturbances in appetite sensations, and in those able to eat normally they tend to add rather than replace oral intake to an extent that appears to depend on the regimen. It is concluded that 1) differences between lean and obese subjects in macronutrient fuel selection during starvation are adaptive because they optimize survival in both groups of subjects; 2) the rate of weight loss in health and disease has a major effect on certain tissue and body functions, independently of the magnitude of weight loss; and 3) clinically relevant disturbances in appetite sensations are common subjects receiving long-term enteral and parenteral nutrition. The clinical modulation of all these variables would be aided by greater knowledge of the mechanisms involved.
2000
Elia M
Clinical Nutrition (edinburgh, Scotland)
2000
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1054/clnu.2000.0157" target="_blank" rel="noreferrer">10.1054/clnu.2000.0157</a>
Protective and damaging effects of stress mediators
Humans; Adaptation; Biomarkers of Pain; Brain/physiology; Immune System/physiology; Homeostasis; Physiological; Stress/physiopathology; Cardiovascular Physiology
1998
McEwen BS
The New England Journal Of Medicine
1998
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1056/nejm199801153380307" target="_blank" rel="noreferrer">10.1056/nejm199801153380307</a>