Metabolic control during the neonatal period in phenylketonuria: associations with childhood IQ
Background In phenylketonuria, treatment and subsequent lowering of phenylalanine levels usually occur within the first month of life. This study investigated whether different indicators of metabolic control during the neonatal period were associated with IQ during late childhood/early adolescence....
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| Veröffentlicht in: | Pediatric research 2022-03, Vol.91 (4), p.874-878 |
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| creator | Liemburg, Geertje B. Huijbregts, Stephan C. J. Rutsch, Frank Feldmann, Reinhold Jahja, Rianne Weglage, Josef Och, Ulrike Burgerhof, Johannes G. M. van Spronsen, Francjan J. |
| description | Background
In phenylketonuria, treatment and subsequent lowering of phenylalanine levels usually occur within the first month of life. This study investigated whether different indicators of metabolic control during the neonatal period were associated with IQ during late childhood/early adolescence.
Methods
Overall phenylalanine concentration during the first month of life (total “area under the curve”), proportion of phenylalanine concentrations above upper target level (360 μmol/L) and proportion below lower target level (120 μmol/L) during this period, diagnostic phenylalanine levels, number of days until phenylalanine levels were 360 μmol/L during the first month of life negatively correlated with IQ in late childhood/early adolescence. Separately, phenylalanine concentrations during different periods within the first month of life (0–10 days, 11–20 days, 21–30 days) were negatively correlated with later IQ as well, but correlation strengths did not differ significantly. No further significant associations were found.
Conclusions
In phenylketonuria, achievement of target-range phenylalanine levels during the neonatal period is important for cognition later in life, also when compared to other indicators of metabolic control.
Impact
In phenylketonuria, it remains unclear during which age periods or developmental stages metabolic control is most important for later cognitive outcomes.
Phenylalanine levels during the neonatal period were clearly and negatively related to later IQ, whereas no significant associations were observed for other indices of metabolic control. This emphasizes the relative importance of this period for cognitive development in phenylketonuria.
No further distinctions were observed in strength of associations with later IQ between different indicators of metabolic control during the neonatal period. Thus, achievement of good metabolic control within 1 month after birth appears “safe” with respect to later cognitive outcomes. |
| doi_str_mv | 10.1038/s41390-021-01728-8 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2571053077</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2571053077</sourcerecordid><originalsourceid>FETCH-LOGICAL-c375t-1f12407f517c69d2dc63e8a86f84baf864e522ba391ceb2da446ab95e44043e83</originalsourceid><addsrcrecordid>eNp9kE1LHTEUhkOx1Kv2D7iQgBs3U_M5SborF7_AUgp1HTKZTCc6N5kmGcR_b65XW3DR1YHDc95zzgPAMUZfMKLyPDNMFWoQwQ3CgshGfgArzGltMSb2wAohihuqlNwHBznfI4QZl-wT2KeMKUG5WgH93RXTxclbaGMoKU6wX5IPv2EZHQwuBlPMBGeXfOyhD3AeXXiaHlyJoXLmKzQ5R-tN8TFk-OjLCO3op36Mlb_5eQQ-DmbK7vNrPQR3lxe_1tfN7Y-rm_W328ZSwUuDB0wYEgPHwraqJ71tqZNGtoNknRlkyxwnpDNUYes60hvGWtMp7hhDrJL0EJztcucU_ywuF73x2bppMvWHJWvCBUZVjRAVPX2H3sclhXqdJi2XSjLZ4kqRHWVTzDm5Qc_Jb0x60hjprX6906-rfv2iX2-vOHmNXrqN6_-OvPmuAN0Bed5Kdunf7v_EPgPbwpCF</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2658984861</pqid></control><display><type>article</type><title>Metabolic control during the neonatal period in phenylketonuria: associations with childhood IQ</title><source>MEDLINE</source><source>SpringerLink Journals</source><source>Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals</source><source>Alma/SFX Local Collection</source><creator>Liemburg, Geertje B. ; Huijbregts, Stephan C. J. ; Rutsch, Frank ; Feldmann, Reinhold ; Jahja, Rianne ; Weglage, Josef ; Och, Ulrike ; Burgerhof, Johannes G. M. ; van Spronsen, Francjan J.</creator><creatorcontrib>Liemburg, Geertje B. ; Huijbregts, Stephan C. J. ; Rutsch, Frank ; Feldmann, Reinhold ; Jahja, Rianne ; Weglage, Josef ; Och, Ulrike ; Burgerhof, Johannes G. M. ; van Spronsen, Francjan J.</creatorcontrib><description>Background
In phenylketonuria, treatment and subsequent lowering of phenylalanine levels usually occur within the first month of life. This study investigated whether different indicators of metabolic control during the neonatal period were associated with IQ during late childhood/early adolescence.
Methods
Overall phenylalanine concentration during the first month of life (total “area under the curve”), proportion of phenylalanine concentrations above upper target level (360 μmol/L) and proportion below lower target level (120 μmol/L) during this period, diagnostic phenylalanine levels, number of days until phenylalanine levels were <360 μmol/L, and lifetime and concurrent phenylalanine levels were correlated with IQ scores of 64 PKU patients (mean age 10.8 years, SD 2.9).
Results
Overall phenylalanine concentration and proportion of phenylalanine concentrations >360 μmol/L during the first month of life negatively correlated with IQ in late childhood/early adolescence. Separately, phenylalanine concentrations during different periods within the first month of life (0–10 days, 11–20 days, 21–30 days) were negatively correlated with later IQ as well, but correlation strengths did not differ significantly. No further significant associations were found.
Conclusions
In phenylketonuria, achievement of target-range phenylalanine levels during the neonatal period is important for cognition later in life, also when compared to other indicators of metabolic control.
Impact
In phenylketonuria, it remains unclear during which age periods or developmental stages metabolic control is most important for later cognitive outcomes.
Phenylalanine levels during the neonatal period were clearly and negatively related to later IQ, whereas no significant associations were observed for other indices of metabolic control. This emphasizes the relative importance of this period for cognitive development in phenylketonuria.
No further distinctions were observed in strength of associations with later IQ between different indicators of metabolic control during the neonatal period. Thus, achievement of good metabolic control within 1 month after birth appears “safe” with respect to later cognitive outcomes.</description><identifier>ISSN: 0031-3998</identifier><identifier>EISSN: 1530-0447</identifier><identifier>DOI: 10.1038/s41390-021-01728-8</identifier><identifier>PMID: 34497359</identifier><language>eng</language><publisher>New York: Nature Publishing Group US</publisher><subject>Adolescent ; Attention ; Child ; Child development ; Childhood ; Clinical Research Article ; Cognition ; Humans ; Infant, Newborn ; Medicine ; Medicine & Public Health ; Metabolism ; Pediatric Surgery ; Pediatrics ; Phenylalanine ; Phenylketonuria ; Phenylketonurias - psychology</subject><ispartof>Pediatric research, 2022-03, Vol.91 (4), p.874-878</ispartof><rights>The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc 2021</rights><rights>2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.</rights><rights>The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc 2021.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c375t-1f12407f517c69d2dc63e8a86f84baf864e522ba391ceb2da446ab95e44043e83</citedby><cites>FETCH-LOGICAL-c375t-1f12407f517c69d2dc63e8a86f84baf864e522ba391ceb2da446ab95e44043e83</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1038/s41390-021-01728-8$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1038/s41390-021-01728-8$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/34497359$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Liemburg, Geertje B.</creatorcontrib><creatorcontrib>Huijbregts, Stephan C. J.</creatorcontrib><creatorcontrib>Rutsch, Frank</creatorcontrib><creatorcontrib>Feldmann, Reinhold</creatorcontrib><creatorcontrib>Jahja, Rianne</creatorcontrib><creatorcontrib>Weglage, Josef</creatorcontrib><creatorcontrib>Och, Ulrike</creatorcontrib><creatorcontrib>Burgerhof, Johannes G. M.</creatorcontrib><creatorcontrib>van Spronsen, Francjan J.</creatorcontrib><title>Metabolic control during the neonatal period in phenylketonuria: associations with childhood IQ</title><title>Pediatric research</title><addtitle>Pediatr Res</addtitle><addtitle>Pediatr Res</addtitle><description>Background
In phenylketonuria, treatment and subsequent lowering of phenylalanine levels usually occur within the first month of life. This study investigated whether different indicators of metabolic control during the neonatal period were associated with IQ during late childhood/early adolescence.
Methods
Overall phenylalanine concentration during the first month of life (total “area under the curve”), proportion of phenylalanine concentrations above upper target level (360 μmol/L) and proportion below lower target level (120 μmol/L) during this period, diagnostic phenylalanine levels, number of days until phenylalanine levels were <360 μmol/L, and lifetime and concurrent phenylalanine levels were correlated with IQ scores of 64 PKU patients (mean age 10.8 years, SD 2.9).
Results
Overall phenylalanine concentration and proportion of phenylalanine concentrations >360 μmol/L during the first month of life negatively correlated with IQ in late childhood/early adolescence. Separately, phenylalanine concentrations during different periods within the first month of life (0–10 days, 11–20 days, 21–30 days) were negatively correlated with later IQ as well, but correlation strengths did not differ significantly. No further significant associations were found.
Conclusions
In phenylketonuria, achievement of target-range phenylalanine levels during the neonatal period is important for cognition later in life, also when compared to other indicators of metabolic control.
Impact
In phenylketonuria, it remains unclear during which age periods or developmental stages metabolic control is most important for later cognitive outcomes.
Phenylalanine levels during the neonatal period were clearly and negatively related to later IQ, whereas no significant associations were observed for other indices of metabolic control. This emphasizes the relative importance of this period for cognitive development in phenylketonuria.
No further distinctions were observed in strength of associations with later IQ between different indicators of metabolic control during the neonatal period. Thus, achievement of good metabolic control within 1 month after birth appears “safe” with respect to later cognitive outcomes.</description><subject>Adolescent</subject><subject>Attention</subject><subject>Child</subject><subject>Child development</subject><subject>Childhood</subject><subject>Clinical Research Article</subject><subject>Cognition</subject><subject>Humans</subject><subject>Infant, Newborn</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Metabolism</subject><subject>Pediatric Surgery</subject><subject>Pediatrics</subject><subject>Phenylalanine</subject><subject>Phenylketonuria</subject><subject>Phenylketonurias - psychology</subject><issn>0031-3998</issn><issn>1530-0447</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp9kE1LHTEUhkOx1Kv2D7iQgBs3U_M5SborF7_AUgp1HTKZTCc6N5kmGcR_b65XW3DR1YHDc95zzgPAMUZfMKLyPDNMFWoQwQ3CgshGfgArzGltMSb2wAohihuqlNwHBznfI4QZl-wT2KeMKUG5WgH93RXTxclbaGMoKU6wX5IPv2EZHQwuBlPMBGeXfOyhD3AeXXiaHlyJoXLmKzQ5R-tN8TFk-OjLCO3op36Mlb_5eQQ-DmbK7vNrPQR3lxe_1tfN7Y-rm_W328ZSwUuDB0wYEgPHwraqJ71tqZNGtoNknRlkyxwnpDNUYes60hvGWtMp7hhDrJL0EJztcucU_ywuF73x2bppMvWHJWvCBUZVjRAVPX2H3sclhXqdJi2XSjLZ4kqRHWVTzDm5Qc_Jb0x60hjprX6906-rfv2iX2-vOHmNXrqN6_-OvPmuAN0Bed5Kdunf7v_EPgPbwpCF</recordid><startdate>20220301</startdate><enddate>20220301</enddate><creator>Liemburg, Geertje B.</creator><creator>Huijbregts, Stephan C. J.</creator><creator>Rutsch, Frank</creator><creator>Feldmann, Reinhold</creator><creator>Jahja, Rianne</creator><creator>Weglage, Josef</creator><creator>Och, Ulrike</creator><creator>Burgerhof, Johannes G. M.</creator><creator>van Spronsen, Francjan J.</creator><general>Nature Publishing Group US</general><general>Nature Publishing Group</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8C1</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20220301</creationdate><title>Metabolic control during the neonatal period in phenylketonuria: associations with childhood IQ</title><author>Liemburg, Geertje B. ; Huijbregts, Stephan C. J. ; Rutsch, Frank ; Feldmann, Reinhold ; Jahja, Rianne ; Weglage, Josef ; Och, Ulrike ; Burgerhof, Johannes G. M. ; van Spronsen, Francjan J.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c375t-1f12407f517c69d2dc63e8a86f84baf864e522ba391ceb2da446ab95e44043e83</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Adolescent</topic><topic>Attention</topic><topic>Child</topic><topic>Child development</topic><topic>Childhood</topic><topic>Clinical Research Article</topic><topic>Cognition</topic><topic>Humans</topic><topic>Infant, Newborn</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Metabolism</topic><topic>Pediatric Surgery</topic><topic>Pediatrics</topic><topic>Phenylalanine</topic><topic>Phenylketonuria</topic><topic>Phenylketonurias - psychology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Liemburg, Geertje B.</creatorcontrib><creatorcontrib>Huijbregts, Stephan C. J.</creatorcontrib><creatorcontrib>Rutsch, Frank</creatorcontrib><creatorcontrib>Feldmann, Reinhold</creatorcontrib><creatorcontrib>Jahja, Rianne</creatorcontrib><creatorcontrib>Weglage, Josef</creatorcontrib><creatorcontrib>Och, Ulrike</creatorcontrib><creatorcontrib>Burgerhof, Johannes G. M.</creatorcontrib><creatorcontrib>van Spronsen, Francjan J.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Public Health Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Liemburg, Geertje B.</au><au>Huijbregts, Stephan C. J.</au><au>Rutsch, Frank</au><au>Feldmann, Reinhold</au><au>Jahja, Rianne</au><au>Weglage, Josef</au><au>Och, Ulrike</au><au>Burgerhof, Johannes G. M.</au><au>van Spronsen, Francjan J.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Metabolic control during the neonatal period in phenylketonuria: associations with childhood IQ</atitle><jtitle>Pediatric research</jtitle><stitle>Pediatr Res</stitle><addtitle>Pediatr Res</addtitle><date>2022-03-01</date><risdate>2022</risdate><volume>91</volume><issue>4</issue><spage>874</spage><epage>878</epage><pages>874-878</pages><issn>0031-3998</issn><eissn>1530-0447</eissn><abstract>Background
In phenylketonuria, treatment and subsequent lowering of phenylalanine levels usually occur within the first month of life. This study investigated whether different indicators of metabolic control during the neonatal period were associated with IQ during late childhood/early adolescence.
Methods
Overall phenylalanine concentration during the first month of life (total “area under the curve”), proportion of phenylalanine concentrations above upper target level (360 μmol/L) and proportion below lower target level (120 μmol/L) during this period, diagnostic phenylalanine levels, number of days until phenylalanine levels were <360 μmol/L, and lifetime and concurrent phenylalanine levels were correlated with IQ scores of 64 PKU patients (mean age 10.8 years, SD 2.9).
Results
Overall phenylalanine concentration and proportion of phenylalanine concentrations >360 μmol/L during the first month of life negatively correlated with IQ in late childhood/early adolescence. Separately, phenylalanine concentrations during different periods within the first month of life (0–10 days, 11–20 days, 21–30 days) were negatively correlated with later IQ as well, but correlation strengths did not differ significantly. No further significant associations were found.
Conclusions
In phenylketonuria, achievement of target-range phenylalanine levels during the neonatal period is important for cognition later in life, also when compared to other indicators of metabolic control.
Impact
In phenylketonuria, it remains unclear during which age periods or developmental stages metabolic control is most important for later cognitive outcomes.
Phenylalanine levels during the neonatal period were clearly and negatively related to later IQ, whereas no significant associations were observed for other indices of metabolic control. This emphasizes the relative importance of this period for cognitive development in phenylketonuria.
No further distinctions were observed in strength of associations with later IQ between different indicators of metabolic control during the neonatal period. Thus, achievement of good metabolic control within 1 month after birth appears “safe” with respect to later cognitive outcomes.</abstract><cop>New York</cop><pub>Nature Publishing Group US</pub><pmid>34497359</pmid><doi>10.1038/s41390-021-01728-8</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; SpringerLink Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection |
| subjects | Adolescent Attention Child Child development Childhood Clinical Research Article Cognition Humans Infant, Newborn Medicine Medicine & Public Health Metabolism Pediatric Surgery Pediatrics Phenylalanine Phenylketonuria Phenylketonurias - psychology |
| title | Metabolic control during the neonatal period in phenylketonuria: associations with childhood IQ |
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