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1/22. 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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2/22. 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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ranking = 0.14285714285714
keywords = nutritional status, status
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3/22. Delayed dilated cardiomyopathy for major burn injuries.

    PURPOSE: The early impact on the heart of severe burns has all been well documented previously. Here, we report on the late effects of burns upon the heart, and dilated cardiomyopathy, such aspects having been little reported previously in the literature. methods: Over the past 8 years (June 1991 to December 1998), 55 scalded or flame-burnt patients for whom the total burnt body surface area (TBSA) exceeded 50% were treated. Seventeen patients survived and were followed up. The mean age was 44 years. The mean follow-up period was 47.3 months; four patients displayed a previous history of heart disease. We used several parameters to evaluate and follow cardiac status to see the late effect of burns upon the heart. RESULTS: Of 17 patients, 3 patients (17.7%) were found to exhibit dilated cardiomyopathies after a mean time of 6 months post-injury. A dramatic recovery from symptoms and roentgenographic findings were observed for all patients following symptomatic treatment. CONCLUSION: Here we report three cases of delayed dilated cardiomyopathy, a condition that has been little reported in the past. There may be many causating factors. Several recommendations are described.
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ranking = 8.3728421958839E-6
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4/22. Body dysmorphic disorder: diagnosis and treatment.

    Our society constantly sees images of athletes and models in movies and magazines and on television. These people often are presented as representing the ideals of the human body. These images can have a significant effect on people who already are predisposed to being preoccupied with their appearance, those who suffer from body dysmorphic disorder (BDD). People who have this disorder are preoccupied with certain aspects of their bodies and often seek to remedy their perceived flaws with cosmetic treatment. A general dentist may be the first health care worker who has an opportunity to intervene and assist these people in getting treatment. Patients may have unrealistic expectations for certain procedures, so assessing their psychological status is an important part of treatment planning and diagnosis. Patient expectations should be determined and the ability to provide care that meets those expectations should be discussed.
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ranking = 8.3728421958839E-6
keywords = status
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5/22. 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 = 0.14285714285714
keywords = nutritional status, status
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6/22. Management of methylmalonic acidaemia by combined liver-kidney transplantation.

    Methylmalonic acidaemia (MMA) is a rare autosomal recessive inborn error of metabolism that typically presents in infancy with recurrent episodes of metabolic acidosis, developmental delay and failure to thrive. The disease course is complicated by the development of chronic tubulointerstitial nephritis progressing to end-stage renal disease in adolescence. We describe two adolescents with cobalamin-nonresponsive MMA (mut0) who developed polyuria, chronic tubulointerstitial nephritis, dystonia but normal synthetic liver function. Both patients received combined liver-kidney transplantation (CLKT), preceded by a single pretransplant haemodialysis for clearance of methylmalonic acid. Post CLKT there was 95-97% reduction in serum and urine methylmalonic acid, leading to significant liberalization of dietary protein intake and a consequent increase in body mass index, muscle strength and energy. In addition, renal function normalized and clinical neurological status stabilized. We propose that CLKT be considered as a therapeutic option early in the course of cobalamin-nonresponsive MMA. Progressive tubulointerstitial nephritis with disabling polyuria is a confounder in patient management even in the absence of end-stage renal disease. Successful CLKT restores methylmalonyl-coa mutase enzyme levels in the liver and kidney, improves clearance of methylmalonic acid with resultant dietary protein liberalization, and offers excellent graft and patient outcomes with improvement in quality of life.
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ranking = 8.3728421958839E-6
keywords = status
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7/22. Growth characteristics in patients with Bartter's syndrome.

    Studies on the growth and development of patients with Bartter's syndrome indicate that severe growth retardation occurs during infancy and early childhood. Delayed adolescent growth spurt has occurred in all patients studied thus far who had manifested the syndrome during infancy. Normal stature is eventually attained. Mental development ranges from normal to brain damage and dysfunction; however, the majority of patients show some degree of mental retardation. The coexistence of Leigh's encephalopathy with Bartter's syndrome in one patient and the finding of severe motor and congnitive retardation with communicating hydrocephalus in another indicate that the prognosis of mental development in some cases of Bartter's syndrome is guarded. Particular attention should be given to maintaining normal nutritional status in all patients, particularly during infancy and early childhood.
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ranking = 0.14285714285714
keywords = nutritional status, status
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8/22. 'Apathetic' thyrotoxicosis presenting with hypercalcaemia and spurious normalization of serum thyroid hormone levels.

    A patient with thyrotoxicosis presented with weight loss and hypercalcaemia, leading to an erroneous diagnosis of occult malignant disease. Intercurrent illness and drug treatment of hypercalcaemia in this patient caused a depression of circulating thyroid hormone levels, leading to a delay in diagnosis. Radionuclide studies of thyroid function, in contrast, consistently suggested a thyrotoxic state. It is suggested that in this situation, radionuclide studies may give a more accurate assessment of thyroid status than biochemical tests, which may be difficult to interpret in the presence of non-thyroidal illness.
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ranking = 8.3728421958839E-6
keywords = status
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9/22. Total parenteral nutrition in pregnancy: conception to delivery.

    The delivery of safe and nutritionally successful total parenteral nutrition (TPN), including intravenous lipid emulsion, to a pregnant woman with Crohn's disease and short bowel syndrome from conception to delivery is reported. Maternal nutritional status improved during the pregnancy. fetal development was normal, and a healthy, full-term infant was delivered. The placenta was normal. Specific intravenous macronutrient and micronutrient requirements during pregnancy are not well defined. Our experience has resulted in the generation of additional recommendations to optimize the intravenous nutritional therapy of this high-risk population.
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ranking = 0.14285714285714
keywords = nutritional status, status
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10/22. Familial parkinsonism, apathy, weight loss, and central hypoventilation: successful long-term management.

    We report a third family with six members with features of the syndrome of familial parkinsonism, depression, weight loss, and central hypoventilation. Patients with this syndrome typically die suddenly, presumably from central respiratory failure. Following multiple respiratory arrests, one affected member was successfully managed with aggressive pulmonary care, tracheostomy, and intermittent home mechanical ventilation, which, combined with carbidopa/levodopa, allowed for a functional lifestyle with improvement in apathy, mobility, and nutritional status.
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ranking = 0.14285714285714
keywords = nutritional status, status
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