Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0232487 (abdominal discomfort)
 1,724 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Conventional treatment of 15 children (aged 4 to 17 years) with cystic fibrosis and persistent failure to thrive was supplemented on an out-patient basis by a daily oral intake of 35 g of medium chain triglycerides (MCT) fat. Follow-up investigations were pursued for a longer period than described in the literature, namely up to 6 months after initiation of the MCT diet. While frequency of the bowel movements was reduced and abdominal discomfort disappeared no weight gain was observed. Clinically, steatorrhoea was clearly diminished. Pre-treatment serum cholesterol levels were lower (121 mg/dl, n=25) than in healthy subjects (182.2 mg/dl, n=56). No significant changes in either serum cholesterol or serum triglycerides were detected over the 6-month period. There were no clinical signs of an essential fatty acid deficiency in 4 patients followed up for 2 years so far. MCT fat should be included in the standard dietary regimen used in the management of any child with cystic fibrosis and failure to thrive. Its use is fully justified by the clinical improvement and alleviation of the steatorrhoea.
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PMID:[Long-term use of medium chain triglycerides in cystic fibrosis (author's transl)]. 99 39

In a double-blind cross-over randomized study adjunct therapy with ranitidine (2 x 150 mg) was compared with enprostil (2 x 35 micrograms) in eight adult cystic fibrosis (CF) patients receiving a fixed dose of enteric-coated microsphere capsules of pancreatin (Pancrease). The study consisted of two consecutive 14-day treatment periods. All patients kept a fixed daily fat intake during the last 5 days of each treatment period and performed 72-h faecal collections during the last 3 days of each treatment period. Gastrointestinal complaints were scored at the end of each treatment period. During treatment with ranitidine there was less faecal fat excretion (18.9% versus 25.1%; NS), less faecal weight (263 versus 303 g/day; NS), and a lower gastrointestinal complaints score (5.3 versus 3.1; P less than 0.05) compared with the treatment with enprostil. One patient dropped out during the treatment period with enprostil because of very severe diarrhoea and abdominal discomfort. We conclude from this study that adjunct therapy with ranitidine has significantly less side effects and may give a better reduction of faecal fat excretion and daily faecal weight in CF.
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PMID:Ranitidine compared with the dimethylprostaglandin E2 analogue enprostil as adjunct to pancreatic enzyme replacement in adult cystic fibrosis. 212 47

A 17-year-old white adolescent had a history of chronic diarrhea, delayed puberty, and growth failure. Investigations excluded cystic fibrosis, Shwachman syndrome, and endocrine causes of growth failure. Severe steatorrhea was diagnosed from fecal fat studies, and a jejunal suction biopsy showed total villus atrophy, consistent with a diagnosis of celiac disease. Following introduction of a gluten-free diet, his appetite and growth improved, but he continued to have abdominal discomfort and loose offensive bowel motions. One year later, severe steatorrhea was present. A repeat jejunal biopsy showed partial recovery of villus architecture. Serum immuno-reactive trypsinogen level was low, which was highly suggestive of exocrine pancreatic failure. Results of quantitative pancreatic stimulation test confirmed the presence of primary pancreatic insufficiency. After introduction of oral pancreatic enzyme supplements with meals, his gastrointestinal symptoms resolved and growth velocity accelerated. Previously, primary pancreatic insufficiency has only been described in elderly patients with long-standing untreated celiac disease. This case, however, emphasizes that pancreatic failure can occur with celiac disease at any age. Determination of a serum immunoreactive trypsinogen level should be considered a useful screening tool for pancreatic insufficiency in patients with celiac disease who have not responded to a gluten-free diet.
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PMID:Treatment failure in celiac disease due to coexistent exocrine pancreatic insufficiency. 368 5

Multilobular biliary cirrhosis and portal hypertension are frequent complications of cystic fibrosis liver disease, leading to esophageal varices and splenomegaly. Therapy is focused on variceal bleeding control; however, reduction of spleen volume is also important to restore gastric volume and resolve invalidating abdominal discomfort. We report long-term follow up (median duration, 5.5 years; range, 14 months-21.5 years) of 6 patients with cystic fibrosis (4 men, 2 women; median age, 14 years; range, 8-18 years) who underwent splenectomy with a splenorenal shunt operation. Three patients received elective surgery for massive splenomegaly with important abdominal discomfort, recurrent variceal bleeding, and hypersplenism. Three were urgently treated to control variceal bleeding after several sessions of sclerotherapy. All but 2 received antipneumococcal vaccination before surgery. Four patients had a weight gain of 10% within 3 months of surgery, and 3 developed spontaneous puberty. Lung function remained stable, and there was an overall reduction of respiratory tract infections. The youngest patient, however, died of overwhelming septicemia during treatment with steroids. Although total splenectomy has important risks, in well-selected cases, it can have benefits. Immuno- and chemoprophylaxis, combined with patient awareness of supplementary risk of infections is indispensable to minimize septic complications.
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PMID:Outcome of total splenectomy with portosystemic shunt for massive splenomegaly and variceal bleeding in cystic fibrosis. 1695 92

Exocrine pancreatic insufficiency (EPI) is a serious condition which occurs in several diseases including chronic pancreatitis (CP), cystic fibrosis, pancreatic cancer, and as a result of pancreatic surgery. The lack or absence of pancreatic enzymes leads to an inadequate absorption of fat, proteins, and carbohydrates, causing steatorrhoea and creathorrhea which results in abdominal discomfort, weight loss, and nutritional deficiencies. To avoid malnutrition related morbidity and mortality, it is pivotal to commence pancreatic enzyme replacement therapy (PERT) as soon as EPI is diagnosed. Factors as early acidic inactivation of ingested enzymes, under dosage, and patient incompliance may prevent normalisation of nutrient absorption, in particular of fat digestion. This review focuses on the current status of how to diagnose and treat EPI.
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PMID:Pancreatic enzyme replacement therapy in chronic pancreatitis. 2051 Aug 33

Children with cystic fibrosis commonly experience abdominal pain; however this remains poorly characterised. This prospective cross-sectional study with a longitudinal design, examined the prevalence, causes and effect of pain management via daily diaries, validated questionnaires for pain characteristics, anxiety status and quality of life. One hundred and thirty CF patients aged 8 to 18 years, regularly followed at our centre, were questioned on recurrent abdominal pain. Eight patients fulfilled the criteria; all wished to enter the study. Pain management included behavioural intervention with effective pain relief, and had a positive impact on anxiety and quality of life. This study is the first one to prospectively assess recurrent abdominal pain in CF. We documented a very low prevalence of 6%. We suggest that, ruling out abdominal discomfort, only a minority of CF children presented recurrent abdominal pain with a true negative impact on daily life. We emphasise the need for further studies including larger cohorts.
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PMID:Recurrent abdominal pain in children with cystic fibrosis: a pilot prospective longitudinal evaluation of characteristics and management. 2190 38

Small intestinal bacterial overgrowth (SIBO) is a disease of great clinical and socioeconomic importance caused by an excessive amount of bacteria in the upper alimentary tract. Physiological microbiota are replaced by pathogenic bacteria mainly from large intestine, which is called dysbacteriosis. SIBO disturbs digestion and absorption in the alimentary tract, which seems to cause inflammation. SIBO affects the morphology and function of the digestive system and causes systemic complications (e.g. osteoporosis, macrocytic anemia). Inflammation interferes with gene expression responsible for producing and secreting mucus, therefore, a correlation between SIBO and cystic fibrosis, irritable bowel syndrome and chronic abdominal pain are postulated. All conditions leading to bacterial growth such as congenital and anatomical abnormalities in the digestive tract, motility disorder or immunological deficits are risk factors of SIBO. A typical clinical manifestation of SIBO comprises meteorism, enterectasia, abdominal discomfort and diarrhea. Diagnostic procedures such as glucose, lactulose, methane, 13C mixed triglyceride breath tests are being used in diagnosing SIBO.
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PMID:Current views on the etiopathogenesis, clinical manifestation, diagnostics, treatment and correlation with other nosological entities of SIBO. 2565 82

Exocrine pancreatic insufficiency (EPI) is characterized by a deficiency of exocrine pancreatic enzymes, resulting in deficits in digestion of all macronutrients, with deficiencies in digestion of fats being the most clinically relevant. The leading cause of EPI is chronic pancreatitis. However, many other causes and conditions may be implicated, including cystic fibrosis, pancreatic duct obstruction, gastric and pancreatic surgery, diabetes mellitus and other conditions. Physical and biochemical causes of EPI include decreased production and secretion of lipase, increased lipase destruction, pancreatic duct obstruction, decreased lipase stimulation and degradation, as well as gastrointestinal motility disorders. EPI is largely diagnosed clinically, and is often identified by symptoms such as steatorrhea, weight loss, abdominal discomfort, and abdominal bloating. Lifestyle modifications (eg, smoking cessation, limiting or avoiding alcoholic drinks, and reducing dietary fat intake) and exogenous pancreatic enzyme supplements are commonly used to help restore normal digestion and absorption of dietary nutrients in patients with EPI.
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PMID:A primer on exocrine pancreatic insufficiency, fat malabsorption, and fatty acid abnormalities. 2872 74