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Biomedical subjects

S T Amann

Publications and source records attributed to S T Amann.

10 recordsLinked to original sources
PubMed1997

Chronic diarrhea associated with thymoma and hypogammaglobulinemia (Good's syndrome).

We report the case of a patient with severe diarrhea and malabsorption who was subsequently found to have hypogammaglobulinemia and thymoma (Good's syndrome). The mechanism by which hypogammaglobulinemia and/or thymoma causes diarrhea is unclear. It may be related to malabsorption caused by a mucosal lesion resembling villous atrophy, which may resolve with restoration of immunologic status. Diarrhea in some patients may respond to commercial gamma globulin injections, fresh frozen plasma, or cholestyramine therapy. The etiologic relationship between thymoma and acquired hypogammaglobulinemia remains unclear. Thymectomy is generally ineffective in improving immunologic deficiencies and coexisting conditions in patients with acquired hypogammaglobulinemia. In our patient's case, severe diarrhea resolved after resection of the thymoma.

Agammaglobulinemia↗
PubMed1997

Acid steatocrit: a simple, rapid gravimetric method to determine steatorrhea.

OBJECTIVES: The detection and evaluation of steatorrhea in a rapid, quantitative fashion are clinically needed in patients with suspected steatorrhea. Our aim was to evaluate the acid steatocrit method, on random spot stools in adults with and without steatorrhea, relative to the qualitative (microscopic) and quantitative assessments for fecal fat. METHODS: Stool samples were collected 72 h after a diet of 100 g of fat per day and randomly from 15 healthy controls, 14 patients with chronic pancreatitis, and seven patients with small bowel disease. All stools had quantitative, qualitative, and acid steatocrit analyses performed for fecal fat. RESULTS: The sensitivity and specificity for the detection of steatorrhea by the spot stool qualitative fecal fat were 78 and 70%, respectively. The spot stool acid steatocrit correlated linearly with the 72-h stool quantitative fecal fat (g/24 h), r = 0.761 and p < 0.001. The acid steatocrit on random spot stools, compared with the 72-h stool quantitative fecal fat, revealed a sensitivity of 100%, a specificity of 95%, and a positive predictive value of 90% for the detection of steatorrhea. It also estimated the quantitative fecal fat. CONCLUSIONS: The acid steatocrit can be performed accurately on random spot stools and can be used to detect the presence of steatorrhea and estimate the quantitative fecal fat. This assay can be done with readily available equipment for rapid evaluation. Use of a spot stool sample simplifies the acid steatocrit, further improving on the practicality of this test. This study also confirms the clinical usefulness of this simplified method to detect steatorrhea.

Adult↗
PubMed1996

Hereditary pancreatitis is caused by a mutation in the cationic trypsinogen gene.

Hereditary pancreatitis (HP) is a rare, early-onset genetic disorder characterized by epigastric pain and often more serious complications. We now report that an Arg-His substitution at residue 117 of the cationic trypsinogen gene is associated with the HP phenotype. This mutation was observed in all HP affected individuals and obligate carriers from five kindreds, but not in individuals who married into the families nor in 140 unrelated individuals. X-ray crystal structure analysis, molecular modelling, and protein digest data indicate that the Arg 117 residue is a trypsin-sensitive site. Cleavage at this site is probably part of a fail-safe mechanism by which trypsin, which is activated within the pancreas, may be inactivated; loss of this cleavage site would permit autodigestion resulting in pancreatitis.

Arginine↗
PubMed1996

Stevens-Johnson syndrome resulting from whole-brain radiation and phenytoin.

Phenytoin, one of the most widely prescribed anticonvulsants, and steroids are routinely utilized for seizure prophylaxis in patients with various intracranial tumors. We report a case of severe Stevens-Johnson syndrome (SJS), documented by biopsy, which occurred in a patient, with metastatic squamous cell carcinoma receiving phenytoin, whole-brain radiation therapy (WBRT), and a tapering steroid dose. The pathogenesis and implications are then briefly discussed.

Anticonvulsants↗
PubMed1996

Fecal pancreatic elastase 1 is inaccurate in the diagnosis of chronic pancreatitis.

Many tests are available to assess pancreatic function. The ideal test would be simple and have adequate sensitivity in mild to moderate chronic pancreatitis (MCP) and severe CP (SCP). Fecal pancreatic elastase 1 (FPE1) assay (ScheBo Tech) has been proposed as a reliable test to evaluate pancreatic exocrine function, with sensitivities of up to 100% in diagnosing CP. Cutoff values (microgram/g stool) of < 100 have been suggested as SCP, 100-200 as MCP, and > 200 as normal. The test's ability to detect MCP distinguished by the absence of steatorrhea, and its specificity among various etiologies of malabsorption, has not been fully evaluated. The aim of this study was to evaluate this assay in subjects including patients with SCP with steatorrhea, patients with MCP with no steatorrhea, healthy controls, and diseased controls with nonpancreatic malabsorption. Thirty-six subjects [15 healthy controls, 7 malabsorption controls, and 14 subjects with CP (7 MCP, 7 SCP)] had FPE1 assays. One hundred fifty-four assays for FPE1 were run for analysis. The intraassay and interassay intraclass correlation coefficients were 0.93 and 0.90, respectively. All SCP had values of < 100 micrograms/g but more than half of the MCP subjects had FPE1 levels within the normal range. The subjects with nonpancreatic malabsorption had FPE1 values ranging from 55 to > 500 micrograms/g of stool. Although the assay detected SCP with steatorrhea, it did not consistently separate the MCP patients from normals. The majority of those with nonpancreatic malabsorption had false-positive values. These results may differ from previously described data because of the purposeful inclusion of MCP subjects, documented by the lack of steatorrhea, and the inclusion of disease controls with nonpancreatic malabsorption. Although PE1 concentrates in the stool and is not significantly degraded, subtle changes in this enzyme, as in MCP, do not seem to be detectable by this assay. This group continues to be the most difficult group to diagnose clinically.

Adult↗
PubMed1994

Severe diarrhea and Cushing's syndrome from an atypical bronchial carcinoid.

Bronchial carcinoids are uncommon pulmonary tumors, considered neuroendocrine in origin and all types may produce various hormones. We describe a young woman with a 2-year history of radiographically stable atypical bronchial carcinoid, ectopic ACTH production, and a markedly elevated calcitonin level. The Cushing's syndrome and diarrheal illness were due to the ectopic hormones.

ACTH Syndrome, Ectopic↗