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1/14. A case of chronic pancreatic insufficiency due to valproic acid in a child.

    A 14-year-old child treated with valproic acid over several years for a seizure disorder developed abdominal pain with radiological evidence of acute pancreatitis. The association with valproic acid was not recognized, and the child continued to take the drug. The patient eventually developed steatorrhea and weight loss that improved with pancreatic enzyme replacement. Radiological evaluation showed an atrophic pancreas. Without evidence of other etiological factors, valproic acid by itself appeared to be the cause of chronic pancreatitis with exocrine pancreatic insufficiency in this patient.
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ranking = 1
keywords = enzyme replacement, enzyme, replacement
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2/14. Secondary pancreatic involvement by diffuse large B-cell lymphoma presenting as acute pancreatitis: treatment and outcome.

    We report the case of a 33-year-old man who presented with a large B-cell non Hodgkin's lymphoma presenting as acute pancreatitis. Abdominal CT showed diffuse swelling of the pancreas, with two distinct masses in the corpus and the tail. Thoracic CT showed a markedly enlarged mediastinum, with a voluminous mass in the middle mediastinum. Direct biopsy of this mass revealed a large B-cell lymphoma. Chemotherapy followed by peripheral blood cell autotransplantation led to complete disappearance of the pancreatic and mediastinal masses. Fatty diarrhea occurred after chemotherapy, probably owing to gland destruction by lymphomatous infiltration. Twenty-six months later, the patient is disease-free but continues to require pancreatic enzyme supplements.
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ranking = 0.00080688613060637
keywords = enzyme
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3/14. Reversible fulminant lactic acidosis and liver failure in an infant with hepatic cytochrome-c oxidase deficiency.

    Cytochrome-c oxidase (COX) is the most common respiratory chain complex involved in liver failure, either as a single enzyme deficiency or as part of multiple enzyme deficiencies. We describe an infant who presented with fulminant lactic acidosis in the neonatal period. The lactic acidosis resolved spontaneously but liver and pancreatic insufficiency ensued. Isolated cytochrome-c oxidase deficiency was found in liver but not in muscle and fibroblasts. mtDNA rearrangements or depletion were ruled out. By the age of one year, liver and pancreatic functions have normalized completely and neurodevelopment is normal.
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ranking = 0.0016137722612127
keywords = enzyme
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4/14. Chronic pancreatitis and maldigestion.

    patients with chronic pancreatitis may suffer from maldigestion and malnutrition. Longstanding inflammation and fibrosis in the gland can destroy exocrine tissue, leading to inadequate delivery of digestive enzymes to the duodenum in the prandial and postprandial period and subsequent maldigestion. Maldigestion is augmented by inadequate bicarbonate delivery to the duodenum, with secondary inactivation of enzymes and bile acids by gastric acid. abdominal pain, sitophobia, nausea, vomiting, postprandial satiety, and on-going alcohol abuse may contribute to poor oral intake. Gastric dysmotility and mechanical gastric outlet obstruction from fibrosis in the pancreatic head may contribute to malnutrition and clinical decline. patients with chronic pancreatitis may at times experience profound steatorrhea and weight loss. In this article, we examine the natural history of exocrine insufficiency in chronic pancreatitis, outline the important nutritional issues in these patients, review the methods of diagnosis of maldigestion, and discuss the approach to therapy.
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ranking = 0.0016137722612127
keywords = enzyme
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5/14. Concurrent pancreatic and growth hormone insufficiency in Johanson-Blizzard syndrome.

    exocrine pancreatic insufficiency is one of the recognised features of the Johanson-Blizzard syndrome; other features include hypothyroidism, sensorineural deafness, aplastic alae nasi, developmental delay, and growth retardation. Twenty-one cases of the syndrome have been described to date. The child reported here was diagnosed in the neonatal period, but despite early pancreatic and thyroid replacement therapy his growth remained poor. Anterior pituitary function studies demonstrated growth hormone deficiency, subsequent administration of growth hormone has resulted in an improvement in his growth velocity. This feature has not been well documented and should be considered as a factor contributing to the growth retardation which is a constant feature of this syndrome.
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ranking = 0.00025173851846854
keywords = replacement
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6/14. Pancreatic function testing in meconium disease in CF: two case reports.

    We report two infants with cystic fibrosis (CF), presenting with meconium ileus and meconium plug, who had no clinical or biochemical evidence of pancreatic insufficiency during infancy. They underwent pancreatic secretory function testing at 11 and 9 months of age, respectively. Both patients had sufficient lipase and colipase secretion to maintain normal digestion of fat, confirming that meconium disease in CF does not necessarily imply pancreatic insufficiency and the need for enzyme supplementation in infancy. Nonetheless, we documented markedly reduced enzyme secretion in both patients, implying a potential role for the pancreas in the pathogenesis of meconium disease, even when clinical pancreatic insufficiency is absent. In addition, our patient with meconium ileus had a severely limited fluid secretory capacity (10.3% of mean normal values). In contrast, the patient with the milder presentation of meconium plug had a far greater ability to secrete fluid (75% of mean normal), but had poorer pancreatic proteolytic activity. We suggest that impaired fluid secretion may be a very significant factor in the pathogenesis of meconium ileus, and we speculate that an inability to maintain sufficient intraluminal fluid relative to the degree of pancreatic proteolytic deficiency may more adequately explain the risk of occurrence and the severity of intestinal obstruction in CF than either factor alone.
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ranking = 0.0016137722612127
keywords = enzyme
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7/14. treatment failure in celiac disease due to coexistent exocrine pancreatic insufficiency.

    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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ranking = 0.00080688613060637
keywords = enzyme
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8/14. Congenital cutis laxa associated with exocrine pancreatic insufficiency.

    We report a patient who has congenital cutis laxa and exocrine pancreatic insufficiency. collagen analysis has provided evidence for defective elastin cross linking, possibly related to a mild copper deficiency. connective tissue anomalies, in the presence of poor growth and/or symptoms of malabsorption should alert the physician to investigate pancreatic function. Adequate replacement of pancreatic enzymes will result in normal growth and development.
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ranking = 0.0010586246490749
keywords = enzyme, replacement
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9/14. Low sweat electrolytes in a patient with cystic fibrosis.

    A patient with the clinical syndrome of cystic fibrosis characterized by chronic pulmonary disease, infection with mucoid pseudomonas aeruginosa, sinusitis, nasal polyposis, abnormal pancreatic bicarbonate response to secretin stimulation, but normal levels of trypsin and chymotrypsin in the duodenal drainage, and a sibling with autopsy-documented cystic fibrosis, is described. sweat chloride ranged from 20 to 44 meq/liter and sweat sodium from 36 to 55 meq/liter. Immunoglobulin deficiency, alpha 1-antitrypsin deficiency, tuberculosis and abnormalities of ciliary ultrastructure were excluded. review of sweat electrolytes in 213 patients with cystic fibrosis revealed that patients with normal pancreatic enzyme release have significantly lower sweat sodium and chloride concentrations (p < 0.0005) than do patients with pancreatic insufficiency. Chronic pulmonary disease, pancreatic insufficiency and elevated levels of sweat electrolytes comprise the classic diagnostic triad for cystic fibrosis. The expression of these features may be variable, but the sweat test remains the cardinal laboratory confirmation of the diagnosis. Over 98 percent of patients with cystic fibrosis have sweat chloride values greater than 60 meq/liter, 1 to 2 percent between 50 and 60 meq/liter, and only about one in 1,000, like our patient, less than 50 meq/liter. patients with cystic fibrosis with borderline sweat chloride values frequently have chronic pulmonary disease but intact pancreatic enzyme release. In such patients, family history, ancillary clinical features and systemic exclusion of other syndromes assume special diagnostic importance.
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ranking = 0.0016137722612127
keywords = enzyme
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10/14. pancreatic extracts are necessary for the absorption of elemental and polymeric enteral diets in severe pancreatic insufficiency.

    Tube feeding nutrition, either elemental or polymeric, is increasingly used in patients with digestive problems. Pancreatic insufficiency is a widely accepted indication for the use of an elemental formula, which requires less residual digestive capacity. To confirm this assumption, we have compared the absorption of elemental and polymeric diets and the effect of exogenous pancreatic enzymes in a patient on long-term total enteral feeding after total pancreatectomy. Malabsorption of both formulas was observed without enzyme supplementation. A marked improvement of fat and nitrogen absorption was obtained when pancreatic enzymes were added to both enteral diets. It is concluded that pancreatic enzymes should always be added to liquid diets in pancreatic insufficiency. No clear advantage is to be anticipated by the use of elemental as compared with polymeric diets.
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ranking = 0.0032275445224255
keywords = enzyme
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