The Impact of Early Indication of Living Donor Liver Transplantation on the Outcomes of Patients With Propionic Acidemia: A Single‐Center Experience Article Swipe
Toshimasa Nakao
,
Seisuke Sakamoto
,
Seiichi Shimizu
,
Akinari Fukuda
,
Hajime Uchida
,
Yusuke Yanagi
,
Ryuji Komine
,
Tasuku Kodama
,
Aoi Ninomiya
,
Masaki Yamada
,
Hiroshi Ono
,
Shunsuke Nosaka
,
Reiko Horikawa
,
Mureo Kasahara
·
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.1111/petr.14886
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.1111/petr.14886
Background Liver transplantation has been indicated for propionic acidemia (PA) patients with frequent metabolic decompensation and performed as an enzyme replacement therapy. We retrospectively evaluated the outcomes of patients with PA and analyze the appropriate timing of living donor liver transplantation (LDLT). Methods We reviewed 12 children with PA who underwent LDLT, who were divided into early (period from the first episode of metabolic decompensation to LDLT < 1 year; n = 6) and late (> 1 year; n = 6) indication groups depending on the timing of LDLT. Results The patient and graft survival rates were 100% in 12 children with PA, and the median observation period was 61 months (5–193 months). None of the patients experienced any episodes of metabolic decompensation after LDLT. The timing of LDLT did not influence the incidence of surgical complications. Two patients in the late indication group had episodes of cardiac arrest and long QT syndrome before LDLT, and one patient showed prolongation of QT interval after LDLT. Two of the six patients in the late indication group had findings of metabolic stroke of the brain on MRI before LDLT. Although LDLT improved the findings of metabolic stroke, a decrease in development quotient score was shown in the post‐LDLT course. Conclusions LDLT may be an effective therapeutic option for improving metabolic control. Early LDLT might be help prevent cardiomyopathy and neurological impairment.
Related Topics
Concepts
Medicine
Propionic acidemia
Liver transplantation
Single Center
Transplantation
Living donor liver transplantation
Pediatrics
Intensive care medicine
Surgery
Internal medicine
Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1111/petr.14886
- https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/petr.14886
- OA Status
- bronze
- Cited By
- 1
- References
- 31
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4404147428
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W4404147428Canonical identifier for this work in OpenAlex
- DOI
-
https://doi.org/10.1111/petr.14886Digital Object Identifier
- Title
-
The Impact of Early Indication of Living Donor Liver Transplantation on the Outcomes of Patients With Propionic Acidemia: A Single‐Center ExperienceWork title
- Type
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articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2024Year of publication
- Publication date
-
2024-11-07Full publication date if available
- Authors
-
Toshimasa Nakao, Seisuke Sakamoto, Seiichi Shimizu, Akinari Fukuda, Hajime Uchida, Yusuke Yanagi, Ryuji Komine, Tasuku Kodama, Aoi Ninomiya, Masaki Yamada, Hiroshi Ono, Shunsuke Nosaka, Reiko Horikawa, Mureo KasaharaList of authors in order
- Landing page
-
https://doi.org/10.1111/petr.14886Publisher landing page
- PDF URL
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https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/petr.14886Direct link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
bronzeOpen access status per OpenAlex
- OA URL
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https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/petr.14886Direct OA link when available
- Concepts
-
Medicine, Propionic acidemia, Liver transplantation, Single Center, Transplantation, Living donor liver transplantation, Pediatrics, Intensive care medicine, Surgery, Internal medicineTop concepts (fields/topics) attached by OpenAlex
- Cited by
-
1Total citation count in OpenAlex
- Citations by year (recent)
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2025: 1Per-year citation counts (last 5 years)
- References (count)
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31Number of works referenced by this work
- Related works (count)
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10Other works algorithmically related by OpenAlex
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| abstract_inverted_index.graft | 94 |
| abstract_inverted_index.group | 144, 175 |
| abstract_inverted_index.liver | 40 |
| abstract_inverted_index.might | 222 |
| abstract_inverted_index.rates | 96 |
| abstract_inverted_index.score | 201 |
| abstract_inverted_index.shown | 203 |
| abstract_inverted_index.year; | 70, 78 |
| abstract_inverted_index.arrest | 149 |
| abstract_inverted_index.before | 154, 186 |
| abstract_inverted_index.enzyme | 20 |
| abstract_inverted_index.groups | 83 |
| abstract_inverted_index.living | 38 |
| abstract_inverted_index.median | 106 |
| abstract_inverted_index.months | 111 |
| abstract_inverted_index.option | 215 |
| abstract_inverted_index.period | 108 |
| abstract_inverted_index.showed | 159 |
| abstract_inverted_index.stroke | 180 |
| abstract_inverted_index.timing | 36, 87, 127 |
| abstract_inverted_index.(LDLT). | 42 |
| abstract_inverted_index.(period | 58 |
| abstract_inverted_index.Methods | 43 |
| abstract_inverted_index.Results | 90 |
| abstract_inverted_index.analyze | 33 |
| abstract_inverted_index.cardiac | 148 |
| abstract_inverted_index.course. | 207 |
| abstract_inverted_index.divided | 55 |
| abstract_inverted_index.episode | 62 |
| abstract_inverted_index.patient | 92, 158 |
| abstract_inverted_index.prevent | 225 |
| abstract_inverted_index.stroke, | 195 |
| abstract_inverted_index.(5–193 | 112 |
| abstract_inverted_index.ABSTRACT | 0 |
| abstract_inverted_index.Although | 188 |
| abstract_inverted_index.acidemia | 9 |
| abstract_inverted_index.children | 47, 101 |
| abstract_inverted_index.control. | 219 |
| abstract_inverted_index.decrease | 197 |
| abstract_inverted_index.episodes | 120, 146 |
| abstract_inverted_index.findings | 177, 192 |
| abstract_inverted_index.frequent | 13 |
| abstract_inverted_index.improved | 190 |
| abstract_inverted_index.interval | 163 |
| abstract_inverted_index.months). | 113 |
| abstract_inverted_index.outcomes | 27 |
| abstract_inverted_index.patients | 11, 29, 117, 139, 170 |
| abstract_inverted_index.quotient | 200 |
| abstract_inverted_index.reviewed | 45 |
| abstract_inverted_index.surgical | 136 |
| abstract_inverted_index.survival | 95 |
| abstract_inverted_index.syndrome | 153 |
| abstract_inverted_index.therapy. | 22 |
| abstract_inverted_index.depending | 84 |
| abstract_inverted_index.effective | 213 |
| abstract_inverted_index.evaluated | 25 |
| abstract_inverted_index.improving | 217 |
| abstract_inverted_index.incidence | 134 |
| abstract_inverted_index.indicated | 6 |
| abstract_inverted_index.influence | 132 |
| abstract_inverted_index.metabolic | 14, 64, 122, 179, 194, 218 |
| abstract_inverted_index.performed | 17 |
| abstract_inverted_index.propionic | 8 |
| abstract_inverted_index.underwent | 51 |
| abstract_inverted_index.Background | 1 |
| abstract_inverted_index.indication | 82, 143, 174 |
| abstract_inverted_index.Conclusions | 208 |
| abstract_inverted_index.appropriate | 35 |
| abstract_inverted_index.development | 199 |
| abstract_inverted_index.experienced | 118 |
| abstract_inverted_index.impairment. | 229 |
| abstract_inverted_index.observation | 107 |
| abstract_inverted_index.post‐LDLT | 206 |
| abstract_inverted_index.replacement | 21 |
| abstract_inverted_index.therapeutic | 214 |
| abstract_inverted_index.neurological | 228 |
| abstract_inverted_index.prolongation | 160 |
| abstract_inverted_index.cardiomyopathy | 226 |
| abstract_inverted_index.complications. | 137 |
| abstract_inverted_index.decompensation | 15, 65, 123 |
| abstract_inverted_index.retrospectively | 24 |
| abstract_inverted_index.transplantation | 3, 41 |
| cited_by_percentile_year.max | 95 |
| cited_by_percentile_year.min | 91 |
| corresponding_author_ids | https://openalex.org/A5078625189 |
| countries_distinct_count | 1 |
| institutions_distinct_count | 14 |
| corresponding_institution_ids | https://openalex.org/I4210132193 |
| citation_normalized_percentile.value | 0.70577015 |
| citation_normalized_percentile.is_in_top_1_percent | False |
| citation_normalized_percentile.is_in_top_10_percent | False |