Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017168 (gastroesophageal reflux disease)
 11,783 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In conclusion, plain-film radiography and the UGI series are useful, easily obtained tests for screening the pediatric UGI tract and differentiating between medical and surgical disease. Ultrasound continues to develop as an initial test for evaluation of the vomiting infant because of its reliability in diagnosing HPS. Endoscopy remains the superior method for diagnosis of mucosal diseases given the limitations of single-contrast barium studies and the newcomer status of ultrasound. Although scintigraphy is the most sensitive and physiologic imaging method for evaluation of gastroesophageal reflux, the UGI series and endoscopy currently play dominant roles in the evaluation of the child with "significant" GERD. CT, MR imaging, and endoscopy are secondary procedures because of their preparation, sedation, monitoring requirements, and cost. Each modality provides the radiologist a different perspective on pediatric UGI disease and therefore improves our ability to understand and diagnose abnormalities in the pediatric UGI tract.
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PMID:Pediatric upper gastrointestinal tract. 797 99

Hermansky-Pudlak syndrome (HPS) is a rare disorder of oculocutaneous albinism, platelet dysfunction, and in some subtypes, fatal pulmonary fibrosis. There is no effective treatment for the pulmonary fibrosis except lung transplantation, but an initial trial using pirfenidone, an anti-fibrotic agent, showed promising results. The current, randomized, placebo-controlled, prospective, double-blind trial investigated the safety and efficacy of pirfenidone for mild to moderate HPS-1 and 4 pulmonary fibrosis. Subjects were evaluated every 4 months at the National Institutes of Health Clinical Center, and the primary outcome parameter was change in forced vital capacity using repeated measures analysis with random coefficients. Thirty-five subjects with HPS-1 pulmonary fibrosis were enrolled during a 4-year interval; 23 subjects received pirfenidone and 12 received placebo. Four subjects withdrew from the trial, 3 subjects died, and 10 serious adverse events were reported. Both groups experienced similar side effects, especially gastroesophageal reflux. Interim analysis of the primary outcome parameter, performed 12 months after 30 patients were enrolled, showed no statistical difference between the placebo and pirfenidone groups, and the study was stopped due to futility. There were no significant safety concerns. Other clinical trials are indicated to identify single or multiple drug regimens that may be effective in treatment for progressive HPS-1 pulmonary fibrosis.
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PMID:Pirfenidone for the treatment of Hermansky-Pudlak syndrome pulmonary fibrosis. 2142 Aug 88