1
40
2
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Text
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URL Address
<a href="http://doi.org/10.1016/j.ymgme.2020.09.006" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ymgme.2020.09.006</a>
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ISSN
1096-7206 1096-7192
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<a href="http://neomed.idm.oclc.org/login?url=http://doi.org/10.1016/j.ymgme.2020.09.006" target="_blank" rel="noreferrer noopener">NEOMED Full-text Holding (if available) - Proxy DOI: 10.1016/j.ymgme.2020.09.006</a>
<p>Users with a NEOMED Library login can search for full-text journal articles at the following url: <a href="https://libraryguides.neomed.edu/home">https://libraryguides.neomed.edu/home</a></p>
Update Year & Number
October 2020 List
NEOMED College
NEOMED College of Medicine
NEOMED Department
Department of Pediatrics
Department of General Surgery
NEOMED Student Publications
Affiliated Hospital
Akron Children's Hospital
Summa Health System Akron City Hospital
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Biochemical phenotype and its relationship to treatment in 16 individuals with PCCB c.1606A > G (p.Asn536Asp) variant propionic acidemia.
Publisher
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Molecular Genetics and Metabolism
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
2020-10-03
Subject
The topic of the resource
Treatment; Phenotype; Biomarker; Propionic acidemia
Creator
An entity primarily responsible for making the resource
Wenger O;Brown M;Smith B;Chowdhury D;Crosby AH;Baple EL;Yoder M;Laxen W;Tortorelli S;Strauss KA
Description
An account of the resource
Propionic acidemia (PA) is caused by inherited deficiency of mitochondrial propionyl-CoA carboxylase (PCC) and results in significant neurodevelopmental and cardiac morbidity. However, relationships among therapeutic intervention, biochemical markers, and disease progression are poorly understood. Sixteen individuals homozygous for PCCB c.1606A > G (p.Asn536Asp) variant PA participated in a two-week suspension of therapy. Standard metabolic markers (plasma amino acids, blood spot methylcitrate, plasma/urine acylcarnitines, urine organic acids) were obtained before and after stopping treatment. These same markers were obtained in sixteen unaffected siblings. Echocardiography and electrocardiography were obtained from all subjects. We characterized the baseline biochemical phenotype of untreated PCCB c.1606A > G homozygotes and impact of treatment on PCC deficiency biomarkers. Therapeutic regimens varied widely. Suspension of therapy did not significantly alter branched chain amino acid levels, their alpha-ketoacid derivatives, or urine ketones. Carnitine supplementation significantly increased urine propionylcarnitine and its ratio to total carnitine. Methylcitrate blood spot and urine levels did not correlate with other biochemical measures or cardiac outcomes. Treatment of PCCB c.1606A > G homozygotes with protein restriction, prescription formula, and/or various dietary supplements has a limited effect on core biomarkers of PCC deficiency. These patients require further longitudinal study with standardized approaches to better understand the relationship between biomarkers and disease burden.
Identifier
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<a href="http://doi.org/10.1016/j.ymgme.2020.09.006" target="_blank" rel="noreferrer noopener">10.1016/j.ymgme.2020.09.006</a>
Format
The file format, physical medium, or dimensions of the resource
journalArticle
2020
Baple EL
Biomarker
Brown M
Chowdhury D
Crosby AH
Department of General Surgery
Department of Pediatrics
journalArticle
Laxen W
Molecular Genetics and Metabolism
NEOMED College of Medicine
NEOMED Student Publications
October 2020 List
Phenotype
Propionic acidemia
Smith B
Strauss KA
Tortorelli S
Treatment
Wenger O
Yoder M
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.4293/JSLS.2020.00115" target="_blank" rel="noreferrer noopener">http://doi.org/10.4293/JSLS.2020.00115</a>
Issue
1
Volume
25
ISSN
1086-8089
Search for Full-text
Locate full-text within NEOMED Library's e-journal collections
<a href="http://neomed.idm.oclc.org/login?url=http://doi.org/10.4293/JSLS.2020.00115" target="_blank" rel="noreferrer noopener">NEOMED Full-text Holding (if available) - Proxy DOI: 10.4293/JSLS.2020.00115</a>
<p>Users with a NEOMED Library login can search for full-text journal articles at the following url: <a href="https://libraryguides.neomed.edu/home">https://libraryguides.neomed.edu/home</a></p>
Update Year & Number
July 2021 List
NEOMED College
NEOMED College of Medicine
NEOMED Department
Department of General Surgery
Affiliated Hospital
Summa Health System Akron City Hospital
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
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Outcomes of Laparoscopic Peritoneal Dialysis Catheter Placement Using an Optimal Placement Technique
Publisher
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JSLS-Journal Of The Society Of Laparoendoscopic Surgeons
Date
A point or period of time associated with an event in the lifecycle of the resource
2021
2021-01
Subject
The topic of the resource
Laparoscopy; Catheter placement; PD catheter; Peritoneal dialysis
Creator
An entity primarily responsible for making the resource
Smith B; Mirhaidari S; Shoemaker A; Douglas D; Dan AG
Description
An account of the resource
Background: Peritoneal dialysis (PD) is a widely employed renal replacement modality. A prospective study was conducted to determine the short-term and midterm outcomes and complication rates associated with a standardized optimal laparoscopic peritoneal dialysis catheter placement technique. Methods: All patients undergoing laparoscopic PD catheter placement by one surgeon using our standardized method over a 5-year period were entered into a prospective database. Patients were evaluated preoperatively and postoperatively through office visits. Development of complications was assessed using follow up telephone or mail surveys. Results: A total of 100 patients with a mean age of 56 years underwent laparoscopic PD catheter placement over the 5-year study period. In total, 103 laparoscopic PD catheter placement attempts were made in 100 patients. Placement was successful in 98 (95.1%) attempts and no placement required conversion to an open operation. Omentopexy was performed in 82 (83.7%) patients. There was no mortality reported within 30 days of the index operation. For patients who successfully underwent laparoscopic PD placement, early complications developed in 9 (9.2%) patients, of which 6 (6.1%) complications were directly related to the PD catheter. Midterm complications developed in 25 (25.5%) patients. Complication-related catheter repositioning was required for 12 (12.2%) catheters and catheter-related complication removal was required for 18 (18.4%) catheters. Conclusion: Laparoscopic placement of PD catheters can be successfully performed using a combination of described standardized laparoscopic maneuvers for optimal placement resulting in acceptable perioperative and short and midterm complication rates with negligible mortality rates.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.4293/JSLS.2020.00115" target="_blank" rel="noreferrer noopener">10.4293/JSLS.2020.00115</a>
Rights
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Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
Format
The file format, physical medium, or dimensions of the resource
journalArticle
2021
Catheter placement
Dan AG
Department of General Surgery
Douglas D
journalArticle
Jsls-Journal of the Society of Laparoendoscopic Surgeons
July 2021 List
Laparoscopy
Mirhaidari S
NEOMED College of Medicine
PD catheter
Peritoneal Dialysis
Shoemaker A
Smith B
Summa Health System Akron City Hospital