1/2. Effect of enzyme-replacement therapy on gastrointestinal symptoms in fabry disease.fabry disease is an X-linked recessive lysosomal storage disorder caused by deficiency of lysosomal alpha-galactosidase A. The disease affects not only kidney, myocardium, central nervous system and the skin but also, in many patients, the gastrointestinal tract. The recent advent of enzyme-replacement therapy has been reported to show beneficial effects on cardiomyopathy, renal function and autonomous nervous function. We report on a 34-year-old patient with fabry disease in whom gastrointestinal symptoms were major complaints. Enzyme replacements led to remarkable improvement of diarrhoea and constipation. abdominal pain, the feeling of fullness and meteorism improved, and metoclopramide, which previously had been used regularly, could be discontinued. There were also marked improvements of appetite, body weight, body mass index, physical activity and overall wellbeing. This observation should prompt further investigations into the pathophysiology of gastrointestinal manifestations in fabry disease and the mechanisms of enzyme-replacement effects on gut function.- - - - - - - - - - ranking = 1keywords = index (Clic here for more details about this article) |
2/2. methamphetamine use following bariatric surgery in an adolescent.bariatric surgery is increasingly popular as a therapeutic strategy for morbidly obese adolescents. Adolescence represents a sensitive period of psychosocial development, and children with considerable weight loss may experience greater peer acceptance, accompanied by both positive and negative influences. Substance abuse exists as one of these negative influences. We present the case of an adolescent bariatric surgical patient who abused methamphetamines in the postoperative period, with consequent nutritional instability. A concerted effort must be made in the preoperative assessment of adolescent bariatric patients to delineate a history of illicit drug use, including abuse of diet pills and stimulants. Excessive postoperative weight loss or micronutrient supplementation non-compliance should raise a suspicion of stimulant use and appropriate screening tests should be performed. The consequent appetite suppression may manifest with signs of malnutrition such as bradycardia, hypotension, and weakness. Inpatient nutritional rehabilitation and psychiatric assessment should be considered.- - - - - - - - - - ranking = 10.626831370296keywords = assessment (Clic here for more details about this article) |