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<script type="text/javascript" src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/jr.boots.min.js"> </script><title>Sapropterin - Drugs and Lactation Database (LactMed®) - NCBI Bookshelf</title>
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<meta name="citation_inbook_title" content="Drugs and Lactation Database (LactMed®) [Internet]">
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<meta name="citation_title" content="Sapropterin">
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<meta name="citation_publisher" content="National Institute of Child Health and Human Development">
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<meta name="citation_date" content="2024/05/15">
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<meta name="citation_keywords" content="Sapropterin">
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<meta name="citation_keywords" content="Kuvan">
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<meta name="citation_keywords" content="Biopterin">
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<meta name="citation_keywords" content="6R-BH4">
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<meta name="citation_keywords" content="BH4">
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<meta name="citation_keywords" content="Dapropterin">
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<meta name="citation_keywords" content="R-THBP">
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<meta name="citation_keywords" content="Sapropterina">
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<meta name="citation_keywords" content="Sapropterinum">
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<meta name="citation_keywords" content="(6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-3H-pteridin-4-one">
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<meta name="description" content="Sapropterin is a synthetic form of the naturally occurring enzyme cofactor tetrahydrobiopterin (BH4) and is used in the treatment of phenylketonuria. BH4 is found in normal human milk and is a cofactor in multiple reactions including serving as a catalyst to phenylalanine hydroxylase. In two postmarketing pregnancy registries of women taking sapropterin, a total of 16 women were identified as breastfeeding for a mean of 3.5 months. No lactation-related safety concerns were reported in infants of mothers nursing during maternal treatment with sapropterin. United States and European guidelines state that sapropterin dihydrochloride supplementation is not contraindicated as an adjunct to dietary therapy in breastfeeding women who are responsive to BH4.[1,2]">
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<meta name="og:description" content="Sapropterin is a synthetic form of the naturally occurring enzyme cofactor tetrahydrobiopterin (BH4) and is used in the treatment of phenylketonuria. BH4 is found in normal human milk and is a cofactor in multiple reactions including serving as a catalyst to phenylalanine hydroxylase. In two postmarketing pregnancy registries of women taking sapropterin, a total of 16 women were identified as breastfeeding for a mean of 3.5 months. No lactation-related safety concerns were reported in infants of mothers nursing during maternal treatment with sapropterin. United States and European guidelines state that sapropterin dihydrochloride supplementation is not contraindicated as an adjunct to dietary therapy in breastfeeding women who are responsive to BH4.[1,2]">
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class="wsprkl btn" title="Jump to next match">▶</a></nav></nav></div><div id="jr-epub-interstitial" class="hidden"></div><div id="jr-content"><article data-type="main"><div class="main-content lit-style" itemscope="itemscope" itemtype="http://schema.org/CreativeWork"><div class="meta-content fm-sec"><div class="fm-sec"><h1 id="_NBK556883_"><span class="title" itemprop="name">Sapropterin</span></h1><p class="fm-aai"><a href="#_NBK556883_pubdet_">Publication Details</a></p><p><em>Estimated reading time: 2 minutes</em></p></div></div><div class="jig-ncbiinpagenav body-content whole_rhythm" data-jigconfig="allHeadingLevels: ['h2'],smoothScroll: false" itemprop="text"><p>CASRN: 62989-33-7</p><a href="https://pubchem.ncbi.nlm.nih.gov/substance/390671247" title="View this structure in PubChem" class="img_link" ref="pagearea=body&targetsite=entrez&targetcat=link&targettype=pubchem"><img src="https://pubchem.ncbi.nlm.nih.gov/image/imgsrv.fcgi?t=l&sid=390671247" alt="image 390671247 in the ncbi pubchem database" /></a><div id="sapropterin.Drug_Levels_and_Effects"><h2 id="_sapropterin_Drug_Levels_and_Effects_">Drug Levels and Effects</h2><div id="sapropterin.Summary_of_Use_during_Lactat"><h3>Summary of Use during Lactation</h3><p>Sapropterin is a synthetic form of the naturally occurring enzyme cofactor tetrahydrobiopterin (BH4) and is used in the treatment of phenylketonuria. BH4 is found in normal human milk and is a cofactor in multiple reactions including serving as a catalyst to phenylalanine hydroxylase. In two postmarketing pregnancy registries of women taking sapropterin, a total of 16 women were identified as breastfeeding for a mean of 3.5 months. No lactation-related safety concerns were reported in infants of mothers nursing during maternal treatment with sapropterin. United States and European guidelines state that sapropterin dihydrochloride supplementation is not contraindicated as an adjunct to dietary therapy in breastfeeding women who are responsive to BH4.[<a class="bibr" href="#sapropterin.REF.1" rid="sapropterin.REF.1">1</a>,<a class="bibr" href="#sapropterin.REF.2" rid="sapropterin.REF.2">2</a>]</p></div><div id="sapropterin.Drug_Levels"><h3>Drug Levels</h3><p><i>Maternal Levels.</i> Breastmilk was obtained from 10 women, colostrum from 9 and mature milk from 6. Colostrum levels of BH4 averaged about 100 nmol/L (241 mcg/L) and mature milk levels averaged about 500 nmol/L (1200 mcg/L). The difference between colostrum and mature milk was statistically significant.[<a class="bibr" href="#sapropterin.REF.3" rid="sapropterin.REF.3">3</a>]</p><p>Three mothers who were taking sapropterin for phenylketonuria had breastmilk samples taken at times that were not reported. Their milk BH4 levels were greater than reported in normal human milk, but specific values were not reported.[<a class="bibr" href="#sapropterin.REF.4" rid="sapropterin.REF.4">4</a>]</p><p><i>Infant Levels.</i> Relevant published information was not found as of the revision date.</p></div><div id="sapropterin.Effects_in_Breastfed_Infants"><h3>Effects in Breastfed Infants</h3><p>A Japanese woman with phenylketonuria took sapropterin dihydrochloride 500 mg (10 mg/kg) daily during pregnancy and postpartum. She breastfed her infant (extent not stated) until 25 months of age. The infant had normal developmental milestones and normal growth at 31 months of age.[<a class="bibr" href="#sapropterin.REF.5" rid="sapropterin.REF.5">5</a>]</p></div><div id="sapropterin.Effects_on_Lactation_and_Bre"><h3>Effects on Lactation and Breastmilk</h3><p>Relevant published information was not found as of the revision date.</p></div><div id="sapropterin.References"><h3>References</h3><dl class="temp-labeled-list"><dl class="bkr_refwrap"><dt>1.</dt><dd><div class="bk_ref" id="sapropterin.REF.1">Management of women with phenylalanine hydroxylase deficiency (phenylketonuria): ACOG Committee Opinion, Number 802.
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Obstet Gynecol
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2020;135:e167-e170.
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[<a href="https://pubmed.ncbi.nlm.nih.gov/32217978" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 32217978</span></a>]</div></dd></dl><dl class="bkr_refwrap"><dt>2.</dt><dd><div class="bk_ref" id="sapropterin.REF.2">van Wegberg
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AMJ, MacDonald
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A, Ahring
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K, et al.
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The complete European guidelines on phenylketonuria: Diagnosis and treatment.
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Orphanet J Rare Dis
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2017;12:162.
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[<a href="/pmc/articles/PMC5639803/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC5639803</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/29025426" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 29025426</span></a>]</div></dd></dl><dl class="bkr_refwrap"><dt>3.</dt><dd><div class="bk_ref" id="sapropterin.REF.3">Weinmann
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A, Post
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M, Pan
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J, et al.
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Tetrahydrobiopterin is present in high quantity in human milk and has a vasorelaxing effect on newborn rat mesenteric arteries.
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Pediatr Res
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2011;69:325-9.
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[<a href="https://pubmed.ncbi.nlm.nih.gov/21178821" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 21178821</span></a>]</div></dd></dl><dl class="bkr_refwrap"><dt>4.</dt><dd><div class="bk_ref" id="sapropterin.REF.4">Hillman
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R, Peck
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D, Grange
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DK, et al.
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Breast milk and plasma pterin levels and offspring outcomes of 25 pregnancies in women exposed to sapropterin dihydrochloride prior to or during pregnancy: An interim report of the PKU moms sub-registry.
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J Inherit Metab Dis
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2013;36:S120. doi:10.1007/s10545-013-9633-z [<a href="http://dx.crossref.org/10.1007/s10545-013-9633-z" ref="pagearea=cite-ref&targetsite=external&targetcat=link&targettype=uri">CrossRef</a>]</div></dd></dl><dl class="bkr_refwrap"><dt>5.</dt><dd><div class="bk_ref" id="sapropterin.REF.5">Nyuzuki
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H, Yamazaki
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T, Saito
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M, et al.
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First Japanese case of maternal phenylketonuria treated with sapropterin dihydrochloride and the normal growth and development of the child.
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Mol Genet Metab Rep
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2019;21:100526.
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[<a href="/pmc/articles/PMC6839020/" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pmc">PMC free article<span class="bk_prnt">: PMC6839020</span></a>] [<a href="https://pubmed.ncbi.nlm.nih.gov/31720228" ref="pagearea=cite-ref&targetsite=entrez&targetcat=link&targettype=pubmed">PubMed<span class="bk_prnt">: 31720228</span></a>]</div></dd></dl></dl></div></div><div id="sapropterin.Substance_Identification"><h2 id="_sapropterin_Substance_Identification_">Substance Identification</h2><div id="sapropterin.Substance_Name"><h3>Substance Name</h3><p>Sapropterin</p></div><div id="sapropterin.CAS_Registry_Number"><h3>CAS Registry Number</h3><p>62989-33-7</p></div><div id="sapropterin.Drug_Class"><h3>Drug Class</h3><p>Breast Feeding</p><p>Lactation</p><p>Milk, Human</p><p>Coenzymes</p></div></div><div><dl class="temp-labeled-list small"><dl class="bkr_refwrap"><dt></dt><dd><div><p class="no_top_margin"><p><b>Disclaimer: </b>Information presented in this database is not meant as a substitute for professional judgment. You should consult your healthcare provider for breastfeeding advice related to your particular situation. The U.S. government does not warrant or assume any liability or responsibility for the accuracy or completeness of the information on this Site.</p></p></div></dd></dl></dl></div><div id="bk_toc_contnr"></div></div></div><div class="fm-sec"><h2 id="_NBK556883_pubdet_">Publication Details</h2><h3>Publication History</h3><p class="small">Last Revision: <span itemprop="dateModified">May 15, 2024</span>.</p><h3>Copyright</h3><div><div class="half_rhythm"><a href="/books/about/copyright/">Copyright Notice</a><p class="small"><b>Attribution Statement:</b> LactMed is a registered trademark of the U.S. Department of Health and Human Services.</p></div></div><h3>Publisher</h3><p><a href="https://www.nlm.nih.gov/" ref="pagearea=page-banner&targetsite=external&targetcat=link&targettype=publisher">National Institute of Child Health and Human Development</a>, Bethesda (MD)</p><h3>NLM Citation</h3><p>Drugs and Lactation Database (LactMed®) [Internet]. Bethesda (MD): National Institute of Child Health and Human Development; 2006-. Sapropterin. [Updated 2024 May 15].<span class="bk_cite_avail"></span></p></div><div class="small-screen-prev"><a href="/books/n/lactmed/LM1130/?report=reader"><svg xmlns="http://www.w3.org/2000/svg" viewBox="0 0 100 100" preserveAspectRatio="none"><path d="M75,30 c-80,60 -80,0 0,60 c-30,-60 -30,0 0,-60"></path><text x="20" y="28" textLength="60" style="font-size:25px">Prev</text></svg></a></div><div class="small-screen-next"><a href="/books/n/lactmed/LM642/?report=reader"><svg xmlns="http://www.w3.org/2000/svg" viewBox="0 0 100 100" preserveAspectRatio="none"><path d="M25,30c80,60 80,0 0,60 c30,-60 30,0 0,-60"></path><text x="20" y="28" textLength="60" style="font-size:25px">Next</text></svg></a></div></article></div><div id="jr-scripts"><script src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/libs.min.js"> </script><script src="/corehtml/pmc/jatsreader/ptpmc_3.22/js/jr.min.js"> </script></div></div>
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