Matches in SemOpenAlex for { <https://semopenalex.org/work/W4313361565> ?p ?o ?g. }
Showing items 1 to 85 of
85
with 100 items per page.
- W4313361565 endingPage "85" @default.
- W4313361565 startingPage "80" @default.
- W4313361565 abstract "Purpose: To determine the prevalence, clinical manifestations, and outcomes of renal involvements in pediatric Alagille syndrome (ALGS).Methods: A total of 21 patients diagnosed with ALGS at age under 18 years who visited Samsung Medical Center from March 1999 to March 2022 were enrolled. ALGS was diagnosed either by clinical manifestations, targeted JAG1 sequencing, and/or liver biopsy. Medical records including sex, age, renal manifestations, urinalysis, serum creatinine, JAG1 sequencing, and ultrasonography were retrospectively reviewed.Results: The male to female ratio was 9:12. The mean age of patients at confirmative diagnosis of ALGS was 18.4 months. Sanger sequencing was performed for 17 patients. Sixteen of 21 patients (76.1%) showed JAG1 mutations. Renal involvement was found in 10 patients (47.6%). The most common type of anomaly was renal dysplasia (40%). One patient having renal dysplasia was pathologically confirmed with glomerular lipid deposition. Two patients (20%) manifested nephrocalcinosis/nephrolithiasis. Among eight renal-involved patients who survived, four (50%) progressed to chronic kidney disease stage 3. Two of these chronic kidney disease patients were diagnosed with hepatorenal syndrome. The other four patients had renal functions preserved, including two without any interventions and two who underwent urological interventions.Conclusions: The current study revealed a high prevalence of renal involvement in Korean pediatric ALGS with diverse phenotypes." @default.
- W4313361565 created "2023-01-06" @default.
- W4313361565 creator A5030819901 @default.
- W4313361565 creator A5049056419 @default.
- W4313361565 creator A5081670771 @default.
- W4313361565 date "2022-12-13" @default.
- W4313361565 modified "2023-09-25" @default.
- W4313361565 title "Diverse renal manifestations of Alagille syndrome in Korean children" @default.
- W4313361565 cites W1960255425 @default.
- W4313361565 cites W1974185766 @default.
- W4313361565 cites W1975631744 @default.
- W4313361565 cites W1986155425 @default.
- W4313361565 cites W2001443677 @default.
- W4313361565 cites W2010576506 @default.
- W4313361565 cites W2023005398 @default.
- W4313361565 cites W2029969016 @default.
- W4313361565 cites W2034593633 @default.
- W4313361565 cites W2040947899 @default.
- W4313361565 cites W2042382218 @default.
- W4313361565 cites W2042534735 @default.
- W4313361565 cites W2049948540 @default.
- W4313361565 cites W2076630231 @default.
- W4313361565 cites W2099255831 @default.
- W4313361565 cites W2102584442 @default.
- W4313361565 cites W2137936382 @default.
- W4313361565 cites W2145111609 @default.
- W4313361565 cites W2159875568 @default.
- W4313361565 cites W2322564967 @default.
- W4313361565 cites W2552242970 @default.
- W4313361565 cites W2948063688 @default.
- W4313361565 cites W2959887800 @default.
- W4313361565 cites W2963461095 @default.
- W4313361565 cites W3091136215 @default.
- W4313361565 cites W3177199147 @default.
- W4313361565 doi "https://doi.org/10.3339/ckd.22.036" @default.
- W4313361565 hasPublicationYear "2022" @default.
- W4313361565 type Work @default.
- W4313361565 citedByCount "0" @default.
- W4313361565 crossrefType "journal-article" @default.
- W4313361565 hasAuthorship W4313361565A5030819901 @default.
- W4313361565 hasAuthorship W4313361565A5049056419 @default.
- W4313361565 hasAuthorship W4313361565A5081670771 @default.
- W4313361565 hasBestOaLocation W43133615651 @default.
- W4313361565 hasConcept C126322002 @default.
- W4313361565 hasConcept C159641895 @default.
- W4313361565 hasConcept C2778593092 @default.
- W4313361565 hasConcept C2778653478 @default.
- W4313361565 hasConcept C2780091579 @default.
- W4313361565 hasConcept C2780306776 @default.
- W4313361565 hasConcept C2780971011 @default.
- W4313361565 hasConcept C2781145721 @default.
- W4313361565 hasConcept C2910137039 @default.
- W4313361565 hasConcept C71924100 @default.
- W4313361565 hasConcept C90924648 @default.
- W4313361565 hasConceptScore W4313361565C126322002 @default.
- W4313361565 hasConceptScore W4313361565C159641895 @default.
- W4313361565 hasConceptScore W4313361565C2778593092 @default.
- W4313361565 hasConceptScore W4313361565C2778653478 @default.
- W4313361565 hasConceptScore W4313361565C2780091579 @default.
- W4313361565 hasConceptScore W4313361565C2780306776 @default.
- W4313361565 hasConceptScore W4313361565C2780971011 @default.
- W4313361565 hasConceptScore W4313361565C2781145721 @default.
- W4313361565 hasConceptScore W4313361565C2910137039 @default.
- W4313361565 hasConceptScore W4313361565C71924100 @default.
- W4313361565 hasConceptScore W4313361565C90924648 @default.
- W4313361565 hasIssue "2" @default.
- W4313361565 hasLocation W43133615651 @default.
- W4313361565 hasOpenAccess W4313361565 @default.
- W4313361565 hasPrimaryLocation W43133615651 @default.
- W4313361565 hasRelatedWork W1857105667 @default.
- W4313361565 hasRelatedWork W2042710484 @default.
- W4313361565 hasRelatedWork W2058466993 @default.
- W4313361565 hasRelatedWork W2101525493 @default.
- W4313361565 hasRelatedWork W2113389249 @default.
- W4313361565 hasRelatedWork W2560482142 @default.
- W4313361565 hasRelatedWork W2607916424 @default.
- W4313361565 hasRelatedWork W2614568041 @default.
- W4313361565 hasRelatedWork W2968996197 @default.
- W4313361565 hasRelatedWork W4214885596 @default.
- W4313361565 hasVolume "26" @default.
- W4313361565 isParatext "false" @default.
- W4313361565 isRetracted "false" @default.
- W4313361565 workType "article" @default.