Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020505 (hyperphagia)
 6,116 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Exercise training has been considered suitable only in cystic fibrosis (CF) patients with mild to moderate pulmonary dysfunction without progressive hypoxaemia during exercise. We trained 16 CF patients, all with advanced lung disease (mean standardized forced expiratory volume in 1 s (FEV1), 30% pred.), with a ventilatory limitation to exercise and a progressive hypoxaemia and hypercapnia at low maximal exercise capacity, Wmax (mean Wmax, 50% pred). Exercise training was performed on a cycle ergometer twice a day for 20 min at approximately 75% of the maximal predicted heart rate for at least 3 weeks. Supplemental oxygen was administered to reach a haemoglobin oxygen saturation of 90% during training. Patients considered malnourished because of a Quetelet Index of less than 20 kg m-2 received hyperalimentation orally or by duodenal tube (total 3500-4000 kcal day-1). Evaluation directly after the training period showed a statistically significant improvement in Wmax, maximal oxygen consumption, maximal minute ventilation, pulse, PaCO2 at rest, FEV1 and body weight. None of the pretraining variables was able to predict the outcome of the training programme in the individual patient. We detected no adverse effects of the programme. This study shows that oxygen-assisted exercise training in combination with correction of the nutritional status is safe and beneficial in CF patients with severe lung disease.
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PMID:Oxygen-assisted exercise training in adult cystic fibrosis patients with pulmonary limitation to exercise. 193 21

An 81-yr-old male with pulmonary emphysema was hospitalized because of malnutrition and hypoglycemia. This patient developed ventilatory failure requiring mechanical assistance 12 h after initiation of iv hyperalimentation. Severe hypophosphatemia (0.1 mg/dl), mild hypocalcemia, hypomagnesemia, and hypokalemia were subsequently and concomitantly documented. Repeated attempts to wean him from the respirator failed until hypophosphatemia was corrected. When difficulties are encountered in weaning patients from mechanical ventilation, attention should be directed toward detection of hypophosphatemia. This may be crucial in the presence of chronic lung disease.
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PMID:Hypophosphatemia as a reversible cause of refractory ventilatory failure. 662 64

In view of contradicting results concerning alteration in lung function during hyperalimentation and the administration of intravenous lipid emulsion, a study was planned to determine possible changes in pulmonary blood perfusion during administration of total nutrient admixture (TNA) and the effect of filtration. Sixteen patients (8 men, 8 women; mean age 65.6 yr) with no previous lung disease received TNA at the rate of 4-5 g lipid/h as preoperative treatment for 5-9 days (mean 6.7 days). The 5-microns filter was randomly used in eight patients. The lung blood perfusion parameters measured repeatedly during this period included blood gases, percentage of the predicted value of vital capacity (VC), and pulmonary diffusing capacity for carbon monoxide (DLCO), as well as the value of the dead space volume and the tidal volume ratio (VD/VT) and the calculated shunt (QS/QT). TNA infusion appears to cause only a slight decrease in PaO2 and DLCO and a similar increase in VC and QS/QT, whereas VD/VT decreased significantly. The latter effect was abolished by filtration of TNA. There were no significant differences between the two groups. We therefore conclude that short-term preoperative administration of TNA at the rate of 4-5 g lipid/h in patients without preexisting lung disease does not jeopardize lung functions. Filtration of the TNA is, however, recommended to avoid the potential hazardous effects of long-term treatment of TNA on lung function.
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PMID:Alteration of pulmonary function by filtration of intravenous nutrient mixture. 848 28

This retrospective study was designed to investigate the related factors and outcome of systemic fungal infection in very low-birth-weight (VLBW) infants. Medical records of infants admitted to the neonatal intensive care unit of National Cheng Kung University Hospital between January 1990 and June 1994 were reviewed. Of the 262 VLBW infants, 15 (5.7%) had fungemia (14 Candida 1 Cryptococcus) during the study period. Among the fungemic infants, 60% also had urinary tract infection; 18% had central nervous system infection. Their mean birth weight was 1079 +/- 78 g (504-1474 g), and the gestational age was 28.6 +/- 0.6 weeks (23-32 weeks). Thirteen of them (87%) had respiratory distress syndrome and patent ductus arteriosus, while 60% had chronic lung disease. The percentage of antibiotic usage, parenteral hyperalimentation, endotracheal intubation, placement of central venous line and steroid therapy were 100%, 100%, 73%, 67% and 36% respectively. The mean age at diagnosis of fungemia was 40.5 +/- 4.8 days (10-76 days). Common clinical manifestations were respiratory deterioration (93%), poor feeding (58%) and fever (53%). The frequency of side effects of amphotericin B in decreasing order were: hypokalemia (54%), hyponatremia (31%) and decreased urine amount (23%). The mortality rate was 40%. It was concluded that systemic fungal infection in VLBW infants might result in high mortality and the side effects were high in the treated infants.
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PMID:Systemic fungal infection in very low-birth-weight infants. 885 49

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.
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PMID:Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report. 2650 47

Negative energy balance is a prevalent feature of cystic fibrosis (CF). Pancreatic insufficiency, elevated energy expenditure, lung disease, and malnutrition, all characteristic of CF, contribute to the negative energy balance causing low body-growth phenotype. As low body weight and body mass index strongly correlate with poor lung health and survival of patients with CF, improving energy balance is an important clinical goal (e.g., high-fat diet). CF mouse models also exhibit negative energy balance (growth retardation and high energy expenditure), independent from exocrine pancreatic insufficiency, lung disease, and malnutrition. To improve energy balance through increased caloric intake and reduced energy expenditure, we disrupted leptin signaling by crossing the db/db leptin receptor allele with mice carrying the R117H Cftr mutation. Compared with db/db mice, absence of leptin signaling in CF mice (CF db/db) resulted in delayed and moderate hyperphagia with lower de novo lipogenesis and lipid deposition, producing only moderately obese CF mice. Greater body length was found in db/db mice but not in CF db/db, suggesting CF-dependent effect on bone growth. The db/db genotype resulted in lower energy expenditure regardless of Cftr genotype leading to obesity. Despite the db/db genotype, the CF genotype exhibited high respiratory quotient indicating elevated carbohydrate oxidation, thus limiting carbohydrates for lipogenesis. In summary, db/db-linked hyperphagia, elevated lipogenesis, and morbid obesity were partially suppressed by reduced CFTR activity. CF mice still accrued large amounts of adipose tissue in contrast to mice fed a high-fat diet, thus highlighting the importance of dietary carbohydrates and not simply fat for energy balance in CF. NEW & NOTEWORTHY We show that cystic fibrosis (CF) mice are able to accrue fat under conditions of carbohydrate overfeeding, increased lipogenesis, and decreased energy expenditure, although length was unaffected. High-fat diet feeding failed to improve growth in CF mice. Morbid db/db-like obesity was reduced in CF double-mutant mice by reduced CFTR activity.
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PMID:Absence of leptin signaling allows fat accretion in cystic fibrosis mice. 3011 52