Lab Matters Summer 2017 | Page 27

newborn screening Each of these disorders presents a unique challenge for clinical providers and the newborn screening community in that the severity and age of onset differ in each individual, making it difficult to diagnose these conditions. and equipment, accessing education and resources, and clinical intervention. To help overcome these challenges, programs received information and potential solutions from policy, laboratory, follow-up and clinical leaders. Despite these dilemmas, early initiation of newborn screening can delay adverse outcomes, especially in babies born with the most severe forms of these disorders. Babies born with classic infantile-onset Pompe, MPS I H (Hurler syndrome) or childhood cerebral X-ALD often die within the first year of life or early childhood. Early detection through newborn screening allows for life-saving medical intervention before symptom onset of these disorders. At the meeting, state programs exchanged their experiences implementing new disorders and shared strategies and resources. Implementation challenges included gaining legislative or statutory approval, hiring laboratory and follow-up staff, obtaining funding for personnel NewSTEPs will continue to support state newborn screening programs to implement screening for Pompe, MPS I and X-ALD through webinars, trainings and educational workshops, policy work, national meetings, readiness assessments and RFPs. n Muscle biopsy showing large vacuoles in a case of pompes disease (acid maltase deficiency, HE stain, frozen section). DIGITAL EXTRA: For more information regarding NewSTEPs services, please visit the website. Newborn Screening Your Starting Point for Pediatric Health NBS04 | Newborn Screening by Tandem Mass Spectrometry ! W NE 2nd Edition NBS04 Newborn Screening by Tandem Mass Spectrometry ! This guideline serves as a reference for the multiple activities W NE related to operating a tandem mass spectrometry laboratory as part of public and private newborn screening programs. A guideline for global application developed through the Clinical and Laboratory Standards Institute consensus process. 1st Edition NBS07 Newborn Blood Spot Screening for Pompe Disease by Lysosomal Acid α-Glucosidase Activity Assays Keep your laboratory up-to-date on new test methods for the detection of metabolic disorders in newborns using tandem mass spectrometry. NBS07 | Newborn Blood Spot Screening for Pompe Disease by Lysosomal Acid α-Glucosidase Activity Assays Learn about current methods for detection of Pompe Disease (PD) using dried blood spot specimens to measure acid α-glucosidase enzyme activity. This report discusses the detection of Pompe disease (PD) by population-based newborn screening using dried blood spot specimens to measure acid α-glucosidase enzyme activity. Classic infantile-onset PD is a lethal disorder that is not evident at birth, and therapy effectiveness is improved by presymptomatic detection. Browse these and more of our Newborn Screening products at clsi.org/nbs. A CLSI report for global application. PublicHealthLabs @APHL APHL.org Summer 2017 LAB MATTERS 25