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1/43. Severe anorexia nervosa associated with osteoporotic-linked femural neck fracture and pulmonary tuberculosis: a case report.

    We report a case study of a 38-year-old woman who had been suffering from anorexia nervosa (AN) since the age of 26. Before admittance to our clinic, she weighed 23.8 kg (at a height of 164 cm, 8.8 body mass index [BMI]) but still carried out strenuous physical activities. After good psychotherapeutic response and weight gain (34.4 kg), she accidentally fell and broke her femoral neck-favored as it was by osteoporosis. The X-ray taken before dynamic hip screw implantation coincidentally showed signs of pulmonary tuberculosis (TB), which could then be proven by computed tomography (CT) scans and cultures from a bronchoscopy. Other than lack of appetite and loss of weight, which we attributed to AN, there were no other clinical or biochemical indicators which could have pointed to an earlier TB diagnosis. As a result, the need for screening procedures is discussed. The manifestation of TB during the first weight gain after 12 years of severe malnutrition, during which there were no serious infections, seems to endorse former observations that AN patients appear to be "resistant" to some extent against infectious diseases, a "protection" which may be lost with convalescence and weight gain.
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2/43. parenteral nutrition in severely burned children.

    In adults supplemental parenteral nutrition (PN) is advisable in burns over 40% especially when weight loss exceeds 10% of body weight. In children with smaller reserves and higher requirement of proteins and energy no rigid scheme for parenteral supplementation is used at our unit. In a young infant it may be added already at a 20-30% deep burn, especially with connected gastrointestinal tract problems, infection etc. Metabolic and protein requirements are estimated 50-100% in addition to their normal needs. Hypertonic glucose (gradually increased from 20-40%), covered with insulin in the early phase, is used as source of carbohydrates. L-amino acid mixture containing the "pediatric essential amino acids" histidine and cysteine is given as a nitrogen source. 20% Intralipid is given in a gradually increased amount of 2-4 g/kg per day to provide calories and essential fatty acids. Among electrolytes K, Ca, P and Mg must be added. Increased amounts of vitamin C and folate are needed by burned children. Vitamin E is also required during prolonged lipid administration. trace elements (Zn. Fe, etc.) are supplied orally or i.v. with special solutions or fresh plasma infusions. Our experience with parenteral nutrition in severely burned children will be presented. There were no severe metabolic side-effects but sepsis represented the major problem. The concomitant heat preservation by warming the room and use of infra-red heaters is emphasized.
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keywords = nutrition
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3/43. Growth and nutrition in rett syndrome.

    PURPOSE/METHOD: In this paper we review the existing literature on factors that contribute to growth failure in rett syndrome (RS) with particular emphasis on the extent and nature of the feeding difficulties that arise. Data on growth and feeding related problems, collected over four years in a specialized clinic for females with rett syndrome are presented and management protocols developed in the clinic discussed. RESULTS/CONCLUSION: Feeding related problems and growth failure occur commonly in rett syndrome yet our understanding of the mechanisms causing growth failure are poorly understood. Both nutritional and non-nutritional factors are thought to contribute and it has not been possible to develop efficacious intervention strategies. Consequently, clinical management of growth failure in rett syndrome is not evidence based.
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keywords = nutrition
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4/43. Normal statural growth in 2 infants on chronic peritoneal dialysis: anecdotal or whole management-related.

    AIMS: Growth retardation is usual in children on chronic peritoneal dialysis (CPD). Despite attention to many contributing factors (nutrition, dialysis dose, hemoglobin level, adynamic bone disease, hyperparathyroidism or rickets, growth hormone resistance, etc.), normal growth is rarely obtained in infants on CPD. MATERIALS AND methods: We had the chance to observe normal growth over a 1 year period in 2 consecutively treated infants on CPD. Louise (renal hypodysplasia) required CPD at the age of 1 month: creatinine 430 micromol/l; oliguric, creatinine clearance lower than 5 ml/min/1.73 m2. Nutrition was achieved orally with human milk during the first 6 months of life. Tidal peritoneal dialysis allowed a high dialysis dose Kt/V urea 3.8/week and Kcreatinine 105 l/week/1.73 m2. Hemoglobin was maintained over 13 g/dl and low levels of vitamin d analogue were prescribed to avoid adynamic bone disease. At the age of 1 year her height was 75 cm. i.e. in the normal range for age. Madeline (renal hypodysplasia) commenced on CPD at the age of 6 weeks and managed similarly. Her height at 1 year of age was 74 cm. RESULTS: In our 20 years of experience with children on dialysis, these 2 cases of normal statural growth for age at 1 year warrant discussion. As well as nutritional support, the new and recent therapeutic options in our team were: firstly, to avoid high doses of activated vitamin d to control PTH, as high doses are able to induce both a risk of adynamic bone disease and a direct bone cartilage toxicity: secondly, to maintain normal hemoglobin level; and thirdly, to deliver a high dialysis dose (urea, creatinine clearance) based on an individually adapted prescription. CONCLUSION: We feel this management approach is necessary to achieve optimal statural growth in children on chronic peritoneal dialysis. But this management concept only based on clinical anecdotal observations needs further evaluation before its use in clinical guidelines.
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keywords = nutrition
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5/43. megestrol acetate to correct the nutritional status in an adolescent with growth hormone deficiency: Increase of appetite and body weight but only by increase of body water and fat mass followed by profound cortisol and testosterone depletion.

    megestrol acetate (MA) is a synthetic, orally active derivative of the naturally occurring hormone progesterone. MA is increasingly used to correct loss of appetite and improve the nutritional status. We used MA in an adolescent with growth hormone (GH) deficiency due to former irradiation therapy in order to evaluate if MA can improve the nutritional status. In fact, MA increased appetite and weight dose-dependent. The energy expenditure measured by indirect calorimetry changed from hypo- to normometabolism. However, weight gain was first primarily due to an increase in body water and then in fat mass. The gain of fat mass was much more prominent than the gain of fat free mass. As important side-effect, MA lead to rapid and profound cortisol and testosterone depletion after only 10 days with a long-lasting effect on testosterone depletion. Therefore, MA as a single therapy cannot be recommended to improve the nutritional status. If MA is given, cortisol and testosterone levels have to be monitored and supplemented as needed.
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6/43. abetalipoproteinemia-like lipid profile and acanthocytosis in a young woman with anorexia nervosa.

    We report the case of a 17-year-old woman with anorexia nervosa (AN) who developed an abetalipoproteinemia-like lipid profile and acanthocytosis. These abnormalities resolved slowly as her nutritional status improved. We considered 3 possible causes of an abetalipoproteinemia-like lipid profile in AN: (1) depletion of hepatic substrate for apolipoprotein B synthesis, (2) lack of exogenous fatty acids with exhaustion of endogenous stores of triglycerides in adipose tissue, and (3) preservation of the lipoprotein lipase (LPL) mass. This unusual case provides important clues that enhance our understanding of lipid metabolism under exogenous and endogenous fat deprivation and highlights the pivotal role of LPL as a gatekeeper of the energy source.
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keywords = nutrition
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7/43. Studies of tryptophan and albumin metabolism in a patient with carcinoid syndrome, pellagra, and hypoproteinemia.

    Detailed studies of protein metabolism were undertaken in a patient with pellagra and hypoproteinemia associated with the carcinoid syndrome both before and after treatment. The synthesis of albumin improved from 82 mg per kg per day to 135 mg per kg per day with little change in the daily excretion of 5-hydroxyindole acetic acid. After treatment with nicotinamide the patient made good progress with a complete resolution of the signs of pellagra and protein malnutrition. These results support the hypothesis that a reduced availability of the essential amino acid L-tryptophan may limit the synthesis of albumin and nicotinic acid in patients with the carcinoid syndrome who become anoretic.
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keywords = nutrition
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8/43. The use of intradialytic parenteral nutrition to treat malnutrition: a case study.

    Protein energy malnutrition in dialysis patients has been well-described in the literature. Most malnourished patients with end stage renal disease (ESRD) suffer from a mixed marasmus-kwashiorkor type of malnutrition with loss of both somatic and visceral protein mass. malnutrition is associated with increased risk of morbidity and mortality. Up to 50% of patients on dialysis have protein energy malnutrition (Mortelmans & Vanholder, 1999). malnutrition may be under-recognized and under-reported in dialysis patients. malnutrition may result from inadequate food intake secondary to the uremic condition, nausea, vomiting, loss of appetite, altered taste and other physiologic conditions that impede food intake or metabolism. The usual indices of nutritional assessment--body weight, body mass index (BMI), anthropometrics, etc., may be inaccurate in patients with ESRD, as the results are often skewed by fluid retention. Therefore, we often rely on weight loss, bloodwork, a pre-dialysis low serum potassium, phosphorus and urea, as early signs of a decreased food intake. When patients are malnourished, measures such as oral supplements and/or tube feedings may be used to augment protein and calorie intake. However, when these interventions are inadequate to reverse the malnutrition condition, intradialytic parenteral nutrition (IDPN) should be implemented. Although there is no definite supportive data to show that the use of IDPN improves morbidity and mortality of dialysis patients, there are data to support that IDPN has positive effects on numerous nutritional parameters (Acchiardo, 2000; Capelli et al., 1994; Foulks, 1999; Hiroshige et al., 1998; Ikizler et al., 1995; Korzets et al., 1999; Mortelmans & Vanholder, 1999; Saunders et al., 1999; Smolle et al., 1995). In this article, we will discuss the causes of malnutrition in dialysis patients, the use of IDPN on one of our patients, and the potential complications associated with IDPN.
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ranking = 19
keywords = nutrition
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9/43. Essential fatty acid deficiency in infants receiving parenteral nutrition.

    The clinical and biochemical features of essential fatty acid deficiency are described in an infant with gastroschisis who required long-term (6 mo) parenteral nutrition. The deficiency responded to therapy with Intralipid, topical sunflower oil, and breast milk. In a prospective study of three infants with gastroschisis, biochemical essential fatty acid deficiency developed in each during the first week of lipid-free parenteral nutrition; clinical signs of the deficiency were absent. The biochemical features were progressive in the one patient followed for 19 days, and were associated with a decrease in weight gain. Both the deficiency and weight gain were corrected by Intralipid. Biochemical essential fatty acid deficiency did not develop in three other gastroschisis infants who were given prophylactic Intralipid (two patients) or topical sunflower oil (one patient). We conclude that all infants on parenteral nutrition should receive a source of linoleic acid to prevent essential fatty acid deficiency.
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ranking = 7
keywords = nutrition
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10/43. Conjugated bile acid replacement therapy in short bowel syndrome patients with a residual colon.

    AIM: To test the efficacy of cholylsarcosine (synthetic conjugated bile acid) and ox bile extracts (mixture of natural conjugated bile acids) on fat absorption, diarrhea, and nutritional state in four short bowel syndrome (SBS) patients with a residual colon not requiring parenteral alimentation. methods: The effect of cholylsarcosine (2 g/meal) on steatorrhea and diarrhea was examined in short-term balance studies with a constant fat intake in all four patients. The effect of continuous cholylsarcosine ingestion on nutritional state was assessed by changes in body weight in three patients. In two patients, the effects of cholylsarcosine were compared with those of ox bile extracts. Because of the low incidence rate of SBS this is not a controlled study. RESULTS: In balance studies, cholylsarcosine increased fat absorption from 65.5 to 94.5 g/day (a 44 % increment), an energy gain of 261 kcal/d. Fecal weight increased by 26 %. In two patients natural conjugated bile acids also reduced steatorrhea, but greatly increased diarrhea. As outpatients consuming an unrestricted diet and ingesting cholylsarcosine, three patients gained weight at an average rate of 0.9 kg/week without worsening of diarrheal symptoms. CONCLUSIONS: Cholylsarcosine is efficacious and safe for enhancing fat absorption and nutritional status in short bowel syndrome patients with residual colon. Natural conjugated bile acids improve steatorrhea to a smaller extent and greatly worsen diarrhea.
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ranking = 3
keywords = nutrition
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