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Query: UMLS:C0235394 (wasting)
 8,040 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pseudohypoaldosteronism is an uncommon disorder characterized by urinary sodium wasting and is attributed to a defect in distal renal tubular sodium handling with failure to respond to endogenous aldosterone. Sweat electrolyte values in other reported patients, when measured, have been normal. A 3.5-year-old girl developed repeated episodes of dehydration, hyponatremia, and hyperkalemia during the first 19 months of life. Serum sodium was as low as 113 mEq/liter and potassium as high as 11.1 mEq/liter. Her plasma and urinary aldosterone levels were persistently elevated (Figs. 1-4). Unlike patients with classic pseudohypoaldosteronism she demonstrated no urinary sodium wasting (Figs. 2 and 3). During episodes of hyponatremia and reduced sodium intake her urinary sodium was less than 5 mEq/liter. In addition, her sweat sodium concentration was consistently above 125 mEq/liter and salivary sodium concentration above 58 mEq/liter. Her chest x-ray, 72-hr fecal fat excretion, serum and urinary pancreatic amylase (amy-2) were normal, providing no evidence for cystic fibrosis. It is proposed that this patient represents a new variant of pseudohypoaldosteronism with excessive loss of sodium from the sweat and salivary glands instead of the kidneys.
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PMID:Pseudohypoaldosteronism due to sweat gland dysfunction. 93 33

A high energy intake, compensating for malabsorption, and the energy cost of lung disease, lung infections, and the underlying metabolic abnormality, is required to ensure normal growth in patients with cystic fibrosis. This goal can be readily achieved by adherence to a high quantity, normally balanced diet (with 40% of the energy as triglycerides of long-chain fatty acids). In contrast, this goal cannot be reached in the majority of patients adhering to low-fat and thus low-energy-containing diets, which almost inevitably lead to malnutrition with wasting and stunting. The prevention of malnutrition may well have considerably enhanced the prognosis of patients at one clinic. Further work is needed to define the interrelationship of nutrition and lung disease, and to define the appropriate nutrient requirements induced by the lung disease per se, recurrent infections, and the underlying disease process.
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PMID:The impact of nutrition in cystic fibrosis: a review. 304 37

A knowledge of the relationship between nutrition and the respiratory system applied in chronic airflow obstruction (BCO) enables a better understanding of the increased frequency (30 to 50%) of protein-energy malnutrition (MEP) in this population. The physiology of the wasting in chronic airflow obstruction seems to relate to hypermetabolism (HMB) which is not compensated by an increased alimentary intake. The HMB is linked to a rise in the work of the respiratory muscles whose efficiency is altered by intrathoracic hyperinfiltration and also the consequences of MEP on the mass and function of the respiratory muscles. In the animal MEP induced by alimentary restrictions leads to a model of pulmonary emphysema and to the diminution of the synthesis of surfactant. This emphysema seems to be principally due to an alteration of the process of protein synthesis and to a diminution of lysyl-oxydase activity. The nutrients (utilised notably by the venous route) have their own pharmacological role, and in addition they have an effect on the natural equilibrium of the energy and nitrogen balance. Lipids (rich in polyunsaturated fatty acids) intervene in the synthesis of prostaglandins, and exercise some effects on the inflammatory process and the activity in the bronchial and vascular smooth muscles. Based on this fact they have been used for their anti-inflammatory role at the pulmonary level in the treatment of mucoviscidosis. The administration of amino acids changes ventilation by acting on the central neuro-muscular command mechanism (VT/TI). The perfusion of amino acids enables a restoration of the chemo-sensitivity to oxygen and to CO2 abolished by the prolonged restricted diet. Finally the partial pressure of oxygen ought to be interpreted with respect to meal times because an oral dose of glucose can provoke an increase in the PaO2 of around 10 mmHg for healthy subjects and those with BCO. A preventive and therapeutic attitude vis a vis BCO should take account of the relationship between nutrition and the respiratory system in parallel with a correction of hypoxaemia in order to avoid the development of wasting.
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PMID:[Malnutrition and chronic obstructive bronchopathies]. 314 Mar 15

We evaluated nutritional status, pulmonary impairment, nutritional intake, and fat absorption in 73 cystic fibrosis (CF) patients to identify the primary factor(s) influencing growth. In general, the growth pattern in our patients was satisfactory since 60/73 were not underweight. When caloric intake is greater than or equal to 95% of RDA, wasting does not occur regardless of the degree of malabsorption, dietary fat content, or lung involvement. In the group of patients who consume less than the RDA, underweight is related to the severity of pulmonary disease; indeed, 11/13 underweight patients have a chest x-ray score over 15. Steatorrhea is well controlled in most patients; only 11 of 73 show a fat excretion greater than 25% of fat intake. The daily number of capsules of Pancrease varies from 4 to 57. The amount of Pancrease to be given was individualized to meet each patient's requirements using fat balance studies to determine the necessary daily Pancrease dose, then distributing the total dose in proportion to the fat content of each meal.
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PMID:The management of enzymatic therapy in cystic fibrosis patients by an individualized approach. 340 60

The energy needs, nutritional status and body composition of 6 undernourished adolescents and young adults with cystic fibrosis (3 male and 3 female) were studied prior to and following a 12 day period of refeeding. Nutritional rehabilitation was attempted with continuous naso-gastric feeding of an elemental diet. The energy needs of the patients were shown to be 25-80% higher than in healthy individuals of the same age, sex and size. Lean body mass as judged by measurement of total body potassium and nitrogen was relatively well preserved, but there was significant wasting of adipose tissue. During the short period of refeeding, body weight, fat and potassium all increased significantly, while fat free body mass and total body nitrogen did not change. A model is proposed for the aetiology of undernutrition in the adolescent and young adult cystic with deteriorating lung function. Based on this model and the finding of the short-term N/G refeeding program described in this paper, we have embarked on a long-term supplementation study in malnourished CF patients who do not respond to dietary counselling and oral supplementation.
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PMID:Energy needs and nutritional rehabilitation in undernourished adolescents and young adult patients with cystic fibrosis. 643 97

The achievement and maintenance of energy balance in cystic fibrosis (CF) is one of the central aims of management. Growth retardation in affected children and wasting in CF adults remain major clinical problems. We consider the basis for the energy deficit, examine the spectrum of nutrient imbalance and review the current guidelines for dietary management.
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PMID:Nutritional aspects in cystic fibrosis. 886 68

Cystic fibrosis (CF) is an exocrine disease affecting multiple organ systems. Patients with CF usually present with respiratory or gastrointestinal abnormalities. This study presents a case of a previously healthy 17-yr-old man who was diagnosed with CF after presenting with metabolic alkalosis and hypokalemia. The defect associated with CF is in the cystic fibrosis transmembrane regulator (CFTR), which acts primarily as a chloride channel. Partially functional CFTR may be associated with less severe pulmonary and gastrointestinal manifestations, as in the case presented. Dysfunctional CFTR in the sweat ducts of CF patients are responsible for excessive chloride and sodium losses, especially in warm weather. Hypokalemia seen with heat stress is secondary to sweat as well as renal potassium wasting. Metabolic alkalosis is maintained by the excessive sweat sodium chloride losses which leads to extracellular fluid (ECF) volume contraction and chloride depletion. Generation of alkalosis may be related to dysfunctional CFTR in the kidney, but is most likely secondary to hypokalemia with ECF volume contraction. Finally, one must consider CF when confronted with hypokalemia and alkalosis in a previously healthy patient.
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PMID:Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent. 904 54

Current international recommendations for energy requirements are based on >9000 measurements of energy intake in both breast- and formula-fed infants. The measurement of energy intake in babies is far from straightforward and the possibility of significant error is great. The opportunity now exists, however, to compare current recommendations with measurements of total energy expenditure (TEE) obtained via the doubly labeled water technique. Approximately 300 measurements of TEE in the first year of life have been made in normal, healthy babies. These data show that estimates of energy intake derived from the measurements of TEE are considerably below the current international recommendations. The same technique has also allowed the energy requirements of sick infants to be evaluated. Two examples are highlighted of infants born small for gestational age and infants born with cystic fibrosis. First, data collected from babies born small for gestational age suggest that such infants have a TEE and hence requirement approximately 20% above that found in infants born with a weight appropriate for their gestational age. This information will be relevant to those professionals attempting to supply optimum nutrition to babies born small for gestational age. Second, in cystic fibrosis it has been suggested that, concurrent with the basic features of the disease, there is an energy-wasting lesion that will increase TEE and hence energy requirement. Recent data collected from babies with cystic fibrosis strongly suggest that this is not the case, and previous data were confounded by subclinical disease status.
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PMID:Energy requirements for growth and development in infancy. 977 76

Cystic fibrosis is commonly associated with energy deficiency in children and adults. Chronic undernutrition will lead to failure to thrive, wasting, and stunting of linear growth; nutrition and survival are intimately related in cystic fibrosis. These problems can simply be considered as energy imbalance, and management centers on restoration of energy balance. Specific nutrient deficiencies, such as fat soluble vitamins, are common in cystic fibrosis. Recent work has highlighted problems with bone density and also the prevalence of vitamin K deficiency. Management of nutritional problems can be complex, and injudicious treatment can further worsen the situation, as the relationship between high daily doses of pancreatic enzymes and the development of fibrosing colonopathy illustrates.
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PMID:Nutrition and cystic fibrosis. 978 64

Children with chronic illness live with the specific consequences of their illness, as well as secondary endocrine abnormalities that further compromise growth and pubertal development. These secondary abnormalities may significantly add to their physiologic and psychological burden. Although these endocrine abnormalities theoretically arise as adaptations to the chronic illness, they may have deleterious effects if they persist untreated. Children with HIV infection and other wasting disorders, for example, show growth suppression out of proportion to the severity of their primary illness as a result of growth hormone resistance and enhanced cortisol secretion. In hematologic conditions such as sickle cell anemia, thalassemia, or bone marrow transplant, damage to the hypothalamus and/or pituitary may lead to growth hormone deficiency, gonadal insufficiency, and hypothyroidism. Growth and pubertal delay are also common among children with cystic fibrosis, along with insulin-dependent diabetes mellitus caused by pancreatic fibrosis. Similarly, children receiving long-term steroid therapy have delays in growth and pubertal development, accompanied by risk for osteoporosis, whereas chronic renal disease is associated with growth and pubertal delay, as well as secondary hyperparathyroidism. Recognition of potential endocrinopathies in children with chronic illness is an important aspect of the care of these children because the disturbances are frequently amenable to treatment, permitting full or partial restoration of normal growth and development in these children. In this chapter, the endocrine consequences of common chronic conditions of childhood are reviewed, as well as the etiology of the endocrine disturbance, the clinical consequences, and recommendations for treatment.
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PMID:Advances in the recognition and treatment of endocrine complications in children with chronic illness. 1064 63


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