Newborn Screening for Cystic Fibrosis

The introduction and widespread implementation of newborn bloodspot screening (NBS) for cystic fibrosis (CF) has offered earlier diagnosis and better outcomes for children with CF in many countries of the world. It represents a paradigm shift in the diagnostic pathway for these families. In contrast...

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Format: Online
Language:English
Published: MDPI - Multidisciplinary Digital Publishing Institute 2021
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Online Access:ONIX_20210501_9783039369904_818
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collection Directory of Open Access Books
description The introduction and widespread implementation of newborn bloodspot screening (NBS) for cystic fibrosis (CF) has offered earlier diagnosis and better outcomes for children with CF in many countries of the world. It represents a paradigm shift in the diagnostic pathway for these families. In contrast to a clinical diagnosis, infants are now referred for diagnostic testing after a positive NBS result. The introduction of NBS has enabled the provision of early appropriate treatment to prevent the manifestations of the disease. In the near future, early diagnosis will facilitate the prompt use of new CFTR modulator therapies that correct the basic underlying molecular defect. NBS for CF has been a global success but continues to raise questions with many varied approaches and the development of new technologies, in particular the ability to undertake extensive gene examination. Which is the best protocol to achieve high sensitivity and specificity, and how to evaluate and manage infants with inconclusive diagnosis are all subjects of ongoing discussion. It is also open to question: what is the best approach to informing and counselling the parents about a positive or inconclusive NBS result? These questions are not easy to answer and require a balanced solution that reflects the local health care system and may appropriately result in different answers around the globe. The articles in this book try to answer these questions and give an overview of the current state of knowledge in NBS for CF.
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language eng
publishDate 2021
publishDateRange 2021
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publisher MDPI - Multidisciplinary Digital Publishing Institute
publisherStr MDPI - Multidisciplinary Digital Publishing Institute
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spelling doab-20.500.12854ir-690722024-03-30T23:22:03Z Newborn Screening for Cystic Fibrosis Barben, Jürg Southern, Kevin newborn screening immunoreactive trypsin(ogen) dried blood spot radioimmunoassay DNA cystic fibrosis incidence malnutrition cost health policy CF transmembrane conductance regulator-related metabolic syndrome CF screen positive inconclusive diagnosis DNA analysis next generation sequencing extended genetic analysis presumptive diagnosis sweat test parental information newborn bloodspot screening psychological impact biochemical screening pancreatitis associated protein immunoreactive trypsinogen cystic fibrosis screen positive inconclusive diagnosis (CFSPID) bioethics newborn screen target disorder missed case sensitivity CFSPID immunoreactive trypsin meconium ileus diagnosis therapy prognosis n/a thema EDItEUR::M Medicine and Nursing The introduction and widespread implementation of newborn bloodspot screening (NBS) for cystic fibrosis (CF) has offered earlier diagnosis and better outcomes for children with CF in many countries of the world. It represents a paradigm shift in the diagnostic pathway for these families. In contrast to a clinical diagnosis, infants are now referred for diagnostic testing after a positive NBS result. The introduction of NBS has enabled the provision of early appropriate treatment to prevent the manifestations of the disease. In the near future, early diagnosis will facilitate the prompt use of new CFTR modulator therapies that correct the basic underlying molecular defect. NBS for CF has been a global success but continues to raise questions with many varied approaches and the development of new technologies, in particular the ability to undertake extensive gene examination. Which is the best protocol to achieve high sensitivity and specificity, and how to evaluate and manage infants with inconclusive diagnosis are all subjects of ongoing discussion. It is also open to question: what is the best approach to informing and counselling the parents about a positive or inconclusive NBS result? These questions are not easy to answer and require a balanced solution that reflects the local health care system and may appropriately result in different answers around the globe. The articles in this book try to answer these questions and give an overview of the current state of knowledge in NBS for CF. 2021-05-01T15:40:30Z 2021-05-01T15:40:30Z 2020 book ONIX_20210501_9783039369904_818 9783039369904 9783039369911 https://directory.doabooks.org/handle/20.500.12854/69072 eng application/octet-stream Attribution 4.0 International https://mdpi.com/books/pdfview/book/2841 https://mdpi.com/books/pdfview/book/2841 MDPI - Multidisciplinary Digital Publishing Institute 10.3390/books978-3-03936-991-1 10.3390/books978-3-03936-991-1 46cabcaa-dd94-4bfe-87b4-55023c1b36d0 9783039369904 9783039369911 126 Basel, Switzerland open access
spellingShingle newborn screening
immunoreactive trypsin(ogen)
dried blood spot
radioimmunoassay
DNA
cystic fibrosis
incidence
malnutrition
cost
health policy
CF transmembrane conductance regulator-related metabolic syndrome
CF screen positive
inconclusive diagnosis
DNA analysis
next generation sequencing
extended genetic analysis
presumptive diagnosis
sweat test
parental information
newborn bloodspot screening
psychological impact
biochemical screening
pancreatitis associated protein
immunoreactive trypsinogen
cystic fibrosis screen positive
inconclusive diagnosis (CFSPID)
bioethics
newborn screen
target disorder
missed case
sensitivity
CFSPID
immunoreactive trypsin
meconium ileus
diagnosis
therapy
prognosis
n/a
thema EDItEUR::M Medicine and Nursing
Newborn Screening for Cystic Fibrosis
title Newborn Screening for Cystic Fibrosis
title_full Newborn Screening for Cystic Fibrosis
title_fullStr Newborn Screening for Cystic Fibrosis
title_full_unstemmed Newborn Screening for Cystic Fibrosis
title_short Newborn Screening for Cystic Fibrosis
title_sort newborn screening for cystic fibrosis
topic newborn screening
immunoreactive trypsin(ogen)
dried blood spot
radioimmunoassay
DNA
cystic fibrosis
incidence
malnutrition
cost
health policy
CF transmembrane conductance regulator-related metabolic syndrome
CF screen positive
inconclusive diagnosis
DNA analysis
next generation sequencing
extended genetic analysis
presumptive diagnosis
sweat test
parental information
newborn bloodspot screening
psychological impact
biochemical screening
pancreatitis associated protein
immunoreactive trypsinogen
cystic fibrosis screen positive
inconclusive diagnosis (CFSPID)
bioethics
newborn screen
target disorder
missed case
sensitivity
CFSPID
immunoreactive trypsin
meconium ileus
diagnosis
therapy
prognosis
n/a
thema EDItEUR::M Medicine and Nursing
topic_facet newborn screening
immunoreactive trypsin(ogen)
dried blood spot
radioimmunoassay
DNA
cystic fibrosis
incidence
malnutrition
cost
health policy
CF transmembrane conductance regulator-related metabolic syndrome
CF screen positive
inconclusive diagnosis
DNA analysis
next generation sequencing
extended genetic analysis
presumptive diagnosis
sweat test
parental information
newborn bloodspot screening
psychological impact
biochemical screening
pancreatitis associated protein
immunoreactive trypsinogen
cystic fibrosis screen positive
inconclusive diagnosis (CFSPID)
bioethics
newborn screen
target disorder
missed case
sensitivity
CFSPID
immunoreactive trypsin
meconium ileus
diagnosis
therapy
prognosis
n/a
thema EDItEUR::M Medicine and Nursing
url ONIX_20210501_9783039369904_818