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Showing 1-20 of 5,409 results
  1. Mothers’ lived experience of caring for children with inborn errors of amino acid metabolism

    Background

    Inborn errors of amino acid metabolism are chronic conditions that have many sequels. Mothers of these children are facing different...

    Sara Shirdelzade, Monir Ramezani, ... Abbas Heydari in BMC Pediatrics
    Article Open access 07 June 2023
  2. Inborn Errors of Metabolism Associated With Autism Among Children: A Multicenter Study from Iran

    Objective

    This study aimed to find the common inborn errors of metabolism in Iranian patients with autism spectrum disorder.

    Methods

    In this...

    Hossein Moravej, Soroor Inaloo, ... Zahra Hadipour in Indian Pediatrics
    Article 02 January 2023
  3. Observational study of birth outcomes in children with inborn errors of metabolism

    Background

    We examined the birth outcomes of children with inborn errors of metabolism detected at birth or later in life.

    Methods

    We carried out a...

    Nathalie Auger, Marianne Bilodeau-Bertrand, ... Laura Arbour in Pediatric Research
    Article 20 January 2022
  4. Chinese genetic variation database of inborn errors of metabolism: a systematic review of published variants in 13 genes

    Background

    Population-specific variation database of inborn errors of metabolism (IEMs) is essential for precise genetic diagnosis and disease...

    Yongchao Guo, Jianhui Jiang, Zhongyao Xu in Orphanet Journal of Rare Diseases
    Article Open access 12 June 2023
  5. Biochemical testing for inborn errors of metabolism: experience from a large tertiary neonatal centre

    Inborn errors of metabolism are an individually rare but collectively significant cause of mortality and morbidity in the neonatal period. They are...

    Esme Dunne, Daniel O’Reilly, ... Afif El Khuffash in European Journal of Pediatrics
    Article Open access 10 August 2022
  6. Neuroimaging findings of inborn errors of metabolism: urea cycle disorders, aminoacidopathies, and organic acidopathies

    Although there are many types of inborn errors of metabolism (IEMs) affecting the central nervous system, also referred to as neurometabolic...

    Mikako Enokizono, Noriko Aida, ... Harushi Mori in Japanese Journal of Radiology
    Article Open access 02 February 2023
  7. Hematopoietic Stem Cell Transplantation for Children With Inborn Errors of Metabolism: Single Center Experience Over Two Decades

    Objective

    We present outcome data on hematopoietic stem cell transplantation (HSCT) in children with inborn errors of metabolism (IEM).

    ...
    Venkateswaran Vellaichamy Swaminathan, Satishkumar Meena, ... Revathi Raj in Indian Pediatrics
    Article 27 June 2022
  8. Instability of acylcarnitine and amino acids in dried blood spots preserved at various temperatures: the impact on retrospective analysis of inborn errors of metabolism biomarkers

    Stored dried blood spots (DBSs) give significant specimens for retrospective detection of inborn errors of metabolisms (IEM) including for validation...

    Zaed A. Asiri, Zahir H. Alshehry, ... Abdullah S. Alshehri in Comparative Clinical Pathology
    Article 29 April 2024
  9. A newborn Screening Programme for Inborn errors of metabolism in Galicia: 22 years of evaluation and follow-up

    Background

    There is a notable lack of harmonisation in newborn screening (NBS) programmes worldwide. The Galician programme for early detection of...

    María L. Couce, María-Dolores Bóveda, ... Paula Sánchez in Orphanet Journal of Rare Diseases
    Article Open access 17 May 2024
  10. Out-of-pocket health expenditures in patients living with ınborn errors of metabolism

    Aim

    The implementation of newborn screening programs for inborn errors of metabolism has advanced the diagnosis and management of affected infants and...

    Mehmet Gündüz, Yasemin Yüksel Güdek, Çiğdem Seher Kasapkara in Orphanet Journal of Rare Diseases
    Article Open access 06 July 2023
  11. Inborn errors of metabolism: Lessons from iPSC models

    The possibility of reprogramming human somatic cells to pluripotency has opened unprecedented opportunities for creating genuinely human experimental...

    Rubén Escribá, Raquel Ferrer-Lorente, Ángel Raya in Reviews in Endocrine and Metabolic Disorders
    Article Open access 09 July 2021
  12. Acute hemodialysis therapy in neonates with inborn errors of metabolism

    Background

    Inborn errors of metabolism (IEM), including organic acidemias and urea cycle defects, are characterized by systemic accumulation of toxic...

    Israel Eisenstein, Shirley Pollack, ... Daniella Magen in Pediatric Nephrology
    Article 03 March 2022
  13. Clinical and Genetic Spectrum of 50 Children with Inborn Errors of Metabolism from Central India

    This is a single-center, retrospective analysis of children confirmed to have an inborn error of metabolism in the pediatric department of a teaching...

    Gouri Rao Passi, Akash Wakchaure, Shree Prakash Jaiswal in Indian Journal of Pediatrics
    Article 25 November 2021
  14. Key patient-reported outcomes in children and adolescents with intoxication-type inborn errors of metabolism: an international Delphi-based consensus

    Background

    Acute intoxication-type inborn errors of metabolism (IT-IEM) such as urea cycle disorders and non-acute IT-IEM such as phenylketonuria...

    Florin Bösch, Nina A. Zeltner, ... Markus A. Landolt in Orphanet Journal of Rare Diseases
    Article Open access 29 January 2022
  15. Inborn Errors of Metabolism—Approach to Diagnosis and Management in Neonates

    Inborn errors of metabolism (IEM), otherwise known as inherited metabolic disorders (IMD), are individually rare, but collectively common. IEM pose a...

    Umamaheswari Balakrishnan in Indian Journal of Pediatrics
    Article 07 June 2021
  16. Psychiatric Symptoms Associated with Inborn Errors of Metabolism

    Inborn errors of metabolism (IEM) are individually rare but collectively common disorders, occurring in 1:800 to 1:1000 births. There are more than...

    Susan Beckwitt Turkel, Derek Wong, Linda Randolph in SN Comprehensive Clinical Medicine
    Article 13 August 2020
  17. Pancreatic involvement in patients with inborn errors of metabolism

    Background

    Repeated inflammation of the pancreas can cause pancreatitis or diabetes. It is well recognized that the organic acidemias may be...

    Woo ** Hwang, Han Hyuk Lim, ... Sook-Za Kim in Orphanet Journal of Rare Diseases
    Article Open access 20 January 2021
  18. Trends of congenital hypothyroidism and inborn errors of metabolism in Pakistan

    Background

    Metabolic disorders are heterogeneous group of genetic disorders that are responsible for significant neonatal and infant morbidity and...

    Sumreena Mansoor in Orphanet Journal of Rare Diseases
    Article Open access 14 November 2020
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