Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders

Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primar...
Ausführliche Beschreibung

Gespeichert in:

Autor*in:	James E. Heubi [verfasserIn] Kenneth D.R. Setchell [verfasserIn] Kevin E. Bove [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2018

Schlagwörter:	Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder

Übergeordnetes Werk:	In: Case Reports in Gastroenterology - Karger Publishers, 2009, 12(2018), 2, Seite 360-372
Übergeordnetes Werk:	volume:12 ; year:2018 ; number:2 ; pages:360-372

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1159/000490095

Katalog-ID:	DOAJ055595847

Internformat


LEADER	01000caa a22002652 4500
001	DOAJ055595847
003	DE-627
005	20230503023452.0
007	cr uuu---uuuuu
008	230227s2018 xx \|\|\|\|\|o 00\| \|\|eng c
024	7		\|a 10.1159/000490095 \|2 doi
035			\|a (DE-627)DOAJ055595847
035			\|a (DE-599)DOAJa2927b923ef44448885a1d530884080f
040			\|a DE-627 \|b ger \|c DE-627 \|e rakwb
041			\|a eng
050		0	\|a RC799-869
100	0		\|a James E. Heubi \|e verfasserin \|4 aut
245	1	0	\|a Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders
264		1	\|c 2018
336			\|a Text \|b txt \|2 rdacontent
337			\|a Computermedien \|b c \|2 rdamedia
338			\|a Online-Ressource \|b cr \|2 rdacarrier
520			\|a Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs.
650		4	\|a Bile acid synthesis
650		4	\|a Cholic acid
650		4	\|a Zellweger spectrum disorder
650		4	\|a Liver function
650		4	\|a Long-term treatment
650		4	\|a Peroxisomal biogenesis disorder
653		0	\|a Diseases of the digestive system. Gastroenterology
700	0		\|a Kenneth D.R. Setchell \|e verfasserin \|4 aut
700	0		\|a Kevin E. Bove \|e verfasserin \|4 aut
773	0	8	\|i In \|t Case Reports in Gastroenterology \|d Karger Publishers, 2009 \|g 12(2018), 2, Seite 360-372 \|w (DE-627)57309635X \|w (DE-600)2440540-1 \|x 16620631 \|7 nnns
773	1	8	\|g volume:12 \|g year:2018 \|g number:2 \|g pages:360-372
856	4	0	\|u https://doi.org/10.1159/000490095 \|z kostenfrei
856	4	0	\|u https://doaj.org/article/a2927b923ef44448885a1d530884080f \|z kostenfrei
856	4	0	\|u https://www.karger.com/Article/FullText/490095 \|z kostenfrei
856	4	2	\|u https://doaj.org/toc/1662-0631 \|y Journal toc \|z kostenfrei
912			\|a GBV_USEFLAG_A
912			\|a SYSFLAG_A
912			\|a GBV_DOAJ
912			\|a SSG-OLC-PHA
912			\|a GBV_ILN_20
912			\|a GBV_ILN_22
912			\|a GBV_ILN_23
912			\|a GBV_ILN_24
912			\|a GBV_ILN_31
912			\|a GBV_ILN_39
912			\|a GBV_ILN_40
912			\|a GBV_ILN_60
912			\|a GBV_ILN_62
912			\|a GBV_ILN_63
912			\|a GBV_ILN_65
912			\|a GBV_ILN_69
912			\|a GBV_ILN_73
912			\|a GBV_ILN_74
912			\|a GBV_ILN_95
912			\|a GBV_ILN_105
912			\|a GBV_ILN_110
912			\|a GBV_ILN_151
912			\|a GBV_ILN_161
912			\|a GBV_ILN_170
912			\|a GBV_ILN_206
912			\|a GBV_ILN_213
912			\|a GBV_ILN_230
912			\|a GBV_ILN_285
912			\|a GBV_ILN_293
912			\|a GBV_ILN_602
912			\|a GBV_ILN_2005
912			\|a GBV_ILN_2009
912			\|a GBV_ILN_2014
912			\|a GBV_ILN_2018
912			\|a GBV_ILN_2055
912			\|a GBV_ILN_2111
912			\|a GBV_ILN_4012
912			\|a GBV_ILN_4037
912			\|a GBV_ILN_4112
912			\|a GBV_ILN_4125
912			\|a GBV_ILN_4126
912			\|a GBV_ILN_4249
912			\|a GBV_ILN_4305
912			\|a GBV_ILN_4306
912			\|a GBV_ILN_4307
912			\|a GBV_ILN_4313
912			\|a GBV_ILN_4322
912			\|a GBV_ILN_4323
912			\|a GBV_ILN_4324
912			\|a GBV_ILN_4325
912			\|a GBV_ILN_4338
912			\|a GBV_ILN_4367
912			\|a GBV_ILN_4700
951			\|a AR
952			\|d 12 \|j 2018 \|e 2 \|h 360-372

Indexfelder

author_variant	j h jh k s ks k b kb
matchkey_str	article:16620631:2018----::ogemhlccdhrpizlwgrp
hierarchy_sort_str	2018
callnumber-subject-code	RC
publishDate	2018
allfields	10.1159/000490095 doi (DE-627)DOAJ055595847 (DE-599)DOAJa2927b923ef44448885a1d530884080f DE-627 ger DE-627 rakwb eng RC799-869 James E. Heubi verfasserin aut Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs. Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder Diseases of the digestive system. Gastroenterology Kenneth D.R. Setchell verfasserin aut Kevin E. Bove verfasserin aut In Case Reports in Gastroenterology Karger Publishers, 2009 12(2018), 2, Seite 360-372 (DE-627)57309635X (DE-600)2440540-1 16620631 nnns volume:12 year:2018 number:2 pages:360-372 https://doi.org/10.1159/000490095 kostenfrei https://doaj.org/article/a2927b923ef44448885a1d530884080f kostenfrei https://www.karger.com/Article/FullText/490095 kostenfrei https://doaj.org/toc/1662-0631 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2018 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2018 2 360-372
spelling	10.1159/000490095 doi (DE-627)DOAJ055595847 (DE-599)DOAJa2927b923ef44448885a1d530884080f DE-627 ger DE-627 rakwb eng RC799-869 James E. Heubi verfasserin aut Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs. Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder Diseases of the digestive system. Gastroenterology Kenneth D.R. Setchell verfasserin aut Kevin E. Bove verfasserin aut In Case Reports in Gastroenterology Karger Publishers, 2009 12(2018), 2, Seite 360-372 (DE-627)57309635X (DE-600)2440540-1 16620631 nnns volume:12 year:2018 number:2 pages:360-372 https://doi.org/10.1159/000490095 kostenfrei https://doaj.org/article/a2927b923ef44448885a1d530884080f kostenfrei https://www.karger.com/Article/FullText/490095 kostenfrei https://doaj.org/toc/1662-0631 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2018 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2018 2 360-372
allfields_unstemmed	10.1159/000490095 doi (DE-627)DOAJ055595847 (DE-599)DOAJa2927b923ef44448885a1d530884080f DE-627 ger DE-627 rakwb eng RC799-869 James E. Heubi verfasserin aut Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs. Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder Diseases of the digestive system. Gastroenterology Kenneth D.R. Setchell verfasserin aut Kevin E. Bove verfasserin aut In Case Reports in Gastroenterology Karger Publishers, 2009 12(2018), 2, Seite 360-372 (DE-627)57309635X (DE-600)2440540-1 16620631 nnns volume:12 year:2018 number:2 pages:360-372 https://doi.org/10.1159/000490095 kostenfrei https://doaj.org/article/a2927b923ef44448885a1d530884080f kostenfrei https://www.karger.com/Article/FullText/490095 kostenfrei https://doaj.org/toc/1662-0631 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2018 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2018 2 360-372
allfieldsGer	10.1159/000490095 doi (DE-627)DOAJ055595847 (DE-599)DOAJa2927b923ef44448885a1d530884080f DE-627 ger DE-627 rakwb eng RC799-869 James E. Heubi verfasserin aut Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs. Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder Diseases of the digestive system. Gastroenterology Kenneth D.R. Setchell verfasserin aut Kevin E. Bove verfasserin aut In Case Reports in Gastroenterology Karger Publishers, 2009 12(2018), 2, Seite 360-372 (DE-627)57309635X (DE-600)2440540-1 16620631 nnns volume:12 year:2018 number:2 pages:360-372 https://doi.org/10.1159/000490095 kostenfrei https://doaj.org/article/a2927b923ef44448885a1d530884080f kostenfrei https://www.karger.com/Article/FullText/490095 kostenfrei https://doaj.org/toc/1662-0631 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2018 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2018 2 360-372
allfieldsSound	10.1159/000490095 doi (DE-627)DOAJ055595847 (DE-599)DOAJa2927b923ef44448885a1d530884080f DE-627 ger DE-627 rakwb eng RC799-869 James E. Heubi verfasserin aut Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders 2018 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs. Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder Diseases of the digestive system. Gastroenterology Kenneth D.R. Setchell verfasserin aut Kevin E. Bove verfasserin aut In Case Reports in Gastroenterology Karger Publishers, 2009 12(2018), 2, Seite 360-372 (DE-627)57309635X (DE-600)2440540-1 16620631 nnns volume:12 year:2018 number:2 pages:360-372 https://doi.org/10.1159/000490095 kostenfrei https://doaj.org/article/a2927b923ef44448885a1d530884080f kostenfrei https://www.karger.com/Article/FullText/490095 kostenfrei https://doaj.org/toc/1662-0631 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2018 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 12 2018 2 360-372
language	English
source	In Case Reports in Gastroenterology 12(2018), 2, Seite 360-372 volume:12 year:2018 number:2 pages:360-372
sourceStr	In Case Reports in Gastroenterology 12(2018), 2, Seite 360-372 volume:12 year:2018 number:2 pages:360-372
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder Diseases of the digestive system. Gastroenterology
isfreeaccess_bool	true
container_title	Case Reports in Gastroenterology
authorswithroles_txt_mv	James E. Heubi @@aut@@ Kenneth D.R. Setchell @@aut@@ Kevin E. Bove @@aut@@
publishDateDaySort_date	2018-01-01T00:00:00Z
hierarchy_top_id	57309635X
id	DOAJ055595847
language_de	englisch
fullrecord	<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">DOAJ055595847</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230503023452.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">230227s2018 xx \|\|\|\|\|o 00\| \|\|eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1159/000490095</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)DOAJ055595847</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-599)DOAJa2927b923ef44448885a1d530884080f</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="050" ind1=" " ind2="0"><subfield code="a">RC799-869</subfield></datafield><datafield tag="100" ind1="0" ind2=" "><subfield code="a">James E. Heubi</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2018</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Bile acid synthesis</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Cholic acid</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Zellweger spectrum disorder</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Liver function</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Long-term treatment</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Peroxisomal biogenesis disorder</subfield></datafield><datafield tag="653" ind1=" " ind2="0"><subfield code="a">Diseases of the digestive system. Gastroenterology</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Kenneth D.R. Setchell</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Kevin E. Bove</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">In</subfield><subfield code="t">Case Reports in Gastroenterology</subfield><subfield code="d">Karger Publishers, 2009</subfield><subfield code="g">12(2018), 2, Seite 360-372</subfield><subfield code="w">(DE-627)57309635X</subfield><subfield code="w">(DE-600)2440540-1</subfield><subfield code="x">16620631</subfield><subfield code="7">nnns</subfield></datafield><datafield tag="773" ind1="1" ind2="8"><subfield code="g">volume:12</subfield><subfield code="g">year:2018</subfield><subfield code="g">number:2</subfield><subfield code="g">pages:360-372</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://doi.org/10.1159/000490095</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://doaj.org/article/a2927b923ef44448885a1d530884080f</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://www.karger.com/Article/FullText/490095</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="856" ind1="4" ind2="2"><subfield code="u">https://doaj.org/toc/1662-0631</subfield><subfield code="y">Journal toc</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_USEFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SYSFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_DOAJ</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SSG-OLC-PHA</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_20</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_22</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_23</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_24</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_31</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_39</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_40</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_60</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_62</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_63</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_65</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_69</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_73</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_74</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_95</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_105</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_110</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_151</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_161</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_170</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_206</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_213</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_230</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_285</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_293</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_602</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2005</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2009</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2014</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2018</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2055</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2111</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4012</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4037</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4112</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4125</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4126</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4249</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4305</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4306</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4307</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4313</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4322</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4323</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4324</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4325</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4338</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4367</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4700</subfield></datafield><datafield tag="951" ind1=" " ind2=" "><subfield code="a">AR</subfield></datafield><datafield tag="952" ind1=" " ind2=" "><subfield code="d">12</subfield><subfield code="j">2018</subfield><subfield code="e">2</subfield><subfield code="h">360-372</subfield></datafield></record></collection>
callnumber-first	R - Medicine
author	James E. Heubi
spellingShingle	James E. Heubi misc RC799-869 misc Bile acid synthesis misc Cholic acid misc Zellweger spectrum disorder misc Liver function misc Long-term treatment misc Peroxisomal biogenesis disorder misc Diseases of the digestive system. Gastroenterology Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders
authorStr	James E. Heubi
ppnlink_with_tag_str_mv	@@773@@(DE-627)57309635X
format	electronic Article
delete_txt_mv	keep
author_role	aut aut aut
collection	DOAJ
remote_str	true
callnumber-label	RC799-869
illustrated	Not Illustrated
issn	16620631
topic_title	RC799-869 Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders Bile acid synthesis Cholic acid Zellweger spectrum disorder Liver function Long-term treatment Peroxisomal biogenesis disorder
topic	misc RC799-869 misc Bile acid synthesis misc Cholic acid misc Zellweger spectrum disorder misc Liver function misc Long-term treatment misc Peroxisomal biogenesis disorder misc Diseases of the digestive system. Gastroenterology
topic_unstemmed	misc RC799-869 misc Bile acid synthesis misc Cholic acid misc Zellweger spectrum disorder misc Liver function misc Long-term treatment misc Peroxisomal biogenesis disorder misc Diseases of the digestive system. Gastroenterology
topic_browse	misc RC799-869 misc Bile acid synthesis misc Cholic acid misc Zellweger spectrum disorder misc Liver function misc Long-term treatment misc Peroxisomal biogenesis disorder misc Diseases of the digestive system. Gastroenterology
format_facet	Elektronische Aufsätze Aufsätze Elektronische Ressource
format_main_str_mv	Text Zeitschrift/Artikel
carriertype_str_mv	cr
hierarchy_parent_title	Case Reports in Gastroenterology
hierarchy_parent_id	57309635X
hierarchy_top_title	Case Reports in Gastroenterology
isfreeaccess_txt	true
familylinks_str_mv	(DE-627)57309635X (DE-600)2440540-1
title	Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders
ctrlnum	(DE-627)DOAJ055595847 (DE-599)DOAJa2927b923ef44448885a1d530884080f
title_full	Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders
author_sort	James E. Heubi
journal	Case Reports in Gastroenterology
journalStr	Case Reports in Gastroenterology
callnumber-first-code	R
lang_code	eng
isOA_bool	true
recordtype	marc
publishDateSort	2018
contenttype_str_mv	txt
container_start_page	360
author_browse	James E. Heubi Kenneth D.R. Setchell Kevin E. Bove
container_volume	12
class	RC799-869
format_se	Elektronische Aufsätze
author-letter	James E. Heubi
doi_str_mv	10.1159/000490095
author2-role	verfasserin
title_sort	long-term cholic acid therapy in zellweger spectrum disorders
callnumber	RC799-869
title_auth	Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders
abstract	Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs.
abstractGer	Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs.
abstract_unstemmed	Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs.
collection_details	GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2018 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700
container_issue	2
title_short	Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders
url	https://doi.org/10.1159/000490095 https://doaj.org/article/a2927b923ef44448885a1d530884080f https://www.karger.com/Article/FullText/490095 https://doaj.org/toc/1662-0631
remote_bool	true
author2	Kenneth D.R. Setchell Kevin E. Bove
author2Str	Kenneth D.R. Setchell Kevin E. Bove
ppnlink	57309635X
callnumber-subject	RC - Internal Medicine
mediatype_str_mv	c
isOA_txt	true
hochschulschrift_bool	false
doi_str	10.1159/000490095
callnumber-a	RC799-869
up_date	2024-07-03T15:55:39.660Z
_version_	1803573935119269888
fullrecord_marcxml	<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">DOAJ055595847</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230503023452.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">230227s2018 xx \|\|\|\|\|o 00\| \|\|eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1159/000490095</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)DOAJ055595847</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-599)DOAJa2927b923ef44448885a1d530884080f</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="050" ind1=" " ind2="0"><subfield code="a">RC799-869</subfield></datafield><datafield tag="100" ind1="0" ind2=" "><subfield code="a">James E. Heubi</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2018</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β-oxidation of these intermediates to form primary C24-bile acids. Oral treatment with primary bile acid, cholic acid (CA), inhibits formation of hepatotoxic C27-bile acids by restoring normal physiologic feedback inhibition on bile acid synthesis. We present the long-term CA treatment and liver-related outcomes for 3 pediatric patients with ZSDs who have received CA treatment for ≥15 years. Ongoing CA treatment was associated with stabilized liver function, as shown by serum biochemistries and liver histopathology, and no treatment-related adverse effects were observed. All 3 patients have attended regular school with classroom accommodations and attained a good quality of life. Our patient outcomes suggest that early and ongoing CA therapy may sustain liver function in patients with ZSDs.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Bile acid synthesis</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Cholic acid</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Zellweger spectrum disorder</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Liver function</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Long-term treatment</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Peroxisomal biogenesis disorder</subfield></datafield><datafield tag="653" ind1=" " ind2="0"><subfield code="a">Diseases of the digestive system. Gastroenterology</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Kenneth D.R. Setchell</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Kevin E. Bove</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">In</subfield><subfield code="t">Case Reports in Gastroenterology</subfield><subfield code="d">Karger Publishers, 2009</subfield><subfield code="g">12(2018), 2, Seite 360-372</subfield><subfield code="w">(DE-627)57309635X</subfield><subfield code="w">(DE-600)2440540-1</subfield><subfield code="x">16620631</subfield><subfield code="7">nnns</subfield></datafield><datafield tag="773" ind1="1" ind2="8"><subfield code="g">volume:12</subfield><subfield code="g">year:2018</subfield><subfield code="g">number:2</subfield><subfield code="g">pages:360-372</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://doi.org/10.1159/000490095</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://doaj.org/article/a2927b923ef44448885a1d530884080f</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">https://www.karger.com/Article/FullText/490095</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="856" ind1="4" ind2="2"><subfield code="u">https://doaj.org/toc/1662-0631</subfield><subfield code="y">Journal toc</subfield><subfield code="z">kostenfrei</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_USEFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SYSFLAG_A</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_DOAJ</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">SSG-OLC-PHA</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_20</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_22</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_23</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_24</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_31</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_39</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_40</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_60</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_62</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_63</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_65</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_69</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_73</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_74</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_95</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_105</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_110</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_151</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_161</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_170</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_206</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_213</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_230</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_285</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_293</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_602</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2005</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2009</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2014</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2018</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2055</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_2111</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4012</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4037</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4112</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4125</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4126</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4249</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4305</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4306</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4307</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4313</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4322</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4323</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4324</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4325</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4338</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4367</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_ILN_4700</subfield></datafield><datafield tag="951" ind1=" " ind2=" "><subfield code="a">AR</subfield></datafield><datafield tag="952" ind1=" " ind2=" "><subfield code="d">12</subfield><subfield code="j">2018</subfield><subfield code="e">2</subfield><subfield code="h">360-372</subfield></datafield></record></collection>
score	7.400943

Nicht das Richtige dabei?

Schreiben Sie uns!

Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders

Nicht das Richtige dabei?

Zugang & Verfügbarkeit

Vorhandene Bände

Nicht das Richtige dabei?