Matches in SemOpenAlex for { <https://semopenalex.org/work/W4250860709> ?p ?o ?g. }
Showing items 1 to 62 of
62
with 100 items per page.
- W4250860709 endingPage "347" @default.
- W4250860709 startingPage "335" @default.
- W4250860709 abstract "Hermansky–Pudlak syndrome (HPS) consists of oculocutaneous albinism, a platelet storage pool deficiency and, in patients with HPS1 gene mutations, a progressive, fatal pulmonary fibrosis. We investigated the safety and efficacy of an antifibrotic agent, pirfenidone (800 mg, t.i.d.), in treating 21 adult Puerto Rican HPS patients, including 20 homozygous for the same HPS1 mutation. Patients were examined every 4 months for up to 44 months in a randomized, placebo-controlled trial, with rate of change in pulmonary function values as outcome parameters. Using the complete data set of 130 patient admissions, a repeated measures model showed that 11 pirfenidone-treated patients lost FVC at a rate 5% of predicted (∼400 mL) per year slower than 10 placebo-treated patients (p=0.001). A random coefficients model showed no significant difference. However, using data restricted to patients with an initial FVC >50% of predicted, both models showed the pirfenidone group losing FVC (p<0.022), FEV1 (p<0.0007), TLC (p<0.001), and DLCO (p<0.122) at a rate ∼8%/year slower than the placebo group. Clinical and laboratory side effects were similar in the two groups. Pirfenidone appears to slow the progression of pulmonary fibrosis in HPS patients who have significant residual lung function." @default.
- W4250860709 created "2022-05-12" @default.
- W4250860709 date "2002-12-01" @default.
- W4250860709 modified "2023-10-17" @default.
- W4250860709 title "Cumulative Subject Index" @default.
- W4250860709 doi "https://doi.org/10.1016/s1096-7192(02)00212-3" @default.
- W4250860709 hasPublicationYear "2002" @default.
- W4250860709 type Work @default.
- W4250860709 citedByCount "0" @default.
- W4250860709 crossrefType "journal-article" @default.
- W4250860709 hasConcept C126322002 @default.
- W4250860709 hasConcept C141071460 @default.
- W4250860709 hasConcept C142724271 @default.
- W4250860709 hasConcept C16020263 @default.
- W4250860709 hasConcept C165637977 @default.
- W4250860709 hasConcept C204787440 @default.
- W4250860709 hasConcept C27081682 @default.
- W4250860709 hasConcept C2777714996 @default.
- W4250860709 hasConcept C2778341716 @default.
- W4250860709 hasConcept C2779778433 @default.
- W4250860709 hasConcept C2779832356 @default.
- W4250860709 hasConcept C2781244666 @default.
- W4250860709 hasConcept C3018587741 @default.
- W4250860709 hasConcept C71924100 @default.
- W4250860709 hasConcept C75603125 @default.
- W4250860709 hasConcept C90924648 @default.
- W4250860709 hasConceptScore W4250860709C126322002 @default.
- W4250860709 hasConceptScore W4250860709C141071460 @default.
- W4250860709 hasConceptScore W4250860709C142724271 @default.
- W4250860709 hasConceptScore W4250860709C16020263 @default.
- W4250860709 hasConceptScore W4250860709C165637977 @default.
- W4250860709 hasConceptScore W4250860709C204787440 @default.
- W4250860709 hasConceptScore W4250860709C27081682 @default.
- W4250860709 hasConceptScore W4250860709C2777714996 @default.
- W4250860709 hasConceptScore W4250860709C2778341716 @default.
- W4250860709 hasConceptScore W4250860709C2779778433 @default.
- W4250860709 hasConceptScore W4250860709C2779832356 @default.
- W4250860709 hasConceptScore W4250860709C2781244666 @default.
- W4250860709 hasConceptScore W4250860709C3018587741 @default.
- W4250860709 hasConceptScore W4250860709C71924100 @default.
- W4250860709 hasConceptScore W4250860709C75603125 @default.
- W4250860709 hasConceptScore W4250860709C90924648 @default.
- W4250860709 hasIssue "4" @default.
- W4250860709 hasLocation W42508607091 @default.
- W4250860709 hasOpenAccess W4250860709 @default.
- W4250860709 hasPrimaryLocation W42508607091 @default.
- W4250860709 hasRelatedWork W2022275011 @default.
- W4250860709 hasRelatedWork W2085097145 @default.
- W4250860709 hasRelatedWork W2340104373 @default.
- W4250860709 hasRelatedWork W2555155415 @default.
- W4250860709 hasRelatedWork W2556745572 @default.
- W4250860709 hasRelatedWork W2564804152 @default.
- W4250860709 hasRelatedWork W2604375962 @default.
- W4250860709 hasRelatedWork W2972156133 @default.
- W4250860709 hasRelatedWork W3092981400 @default.
- W4250860709 hasRelatedWork W3161875924 @default.
- W4250860709 hasVolume "77" @default.
- W4250860709 isParatext "false" @default.
- W4250860709 isRetracted "false" @default.
- W4250860709 workType "article" @default.