Literature DB >> 27207231

Neonatal screening for severe combined immunodeficiency in Brazil.

Marilia Pyles Patto Kanegae1, Lucila Akune Barreiros1, Juliana Themudo Lessa Mazzucchelli2, Sonia Marchezi Hadachi3, Laura Maria de Figueiredo Ferreira Guilhoto3, Ana Lúcia Acquesta4, Isabel Rugue Genov5, Silvia Maia Holanda6, Regina Sumiko Watanabe Di Gesu7, Ana Lucia Goulart2, Amélia Miyashiro Nunes Dos Santos2, Newton Bellesi8, Beatriz Tavares Costa-Carvalho2, Antonio Condino-Neto9.   

Abstract

OBJECTIVE: To apply, in Brazil, the T-cell receptor excision circles (TRECs) quantification technique using real-time polymerase chain reaction in newborn screening for severe combined immunodeficiency and assess the feasibility of implementing it on a large scale in Brazil.
METHODS: 8715 newborn blood samples were collected on filter paper and, after DNA elution, TRECs were quantified by real-time polymerase chain reaction. The cutoff value to determine whether a sample was abnormal was determined by ROC curve analysis, using SSPS.
RESULTS: The concentration of TRECs in 8,682 samples ranged from 2 to 2,181TRECs/μL of blood, with mean and median of 324 and 259TRECs/μL, respectively. Forty-nine (0.56%) samples were below the cutoff (30TRECs/μL) and were reanalyzed. Four (0.05%) samples had abnormal results (between 16 and 29TRECs/μL). Samples from patients previously identified as having severe combined immunodeficiency or DiGeorge syndrome were used to validate the assay and all of them showed TRECs below the cutoff. Preterm infants had lower levels of TRECs than full-term neonates. The ROC curve showed a cutoff of 26TRECs/μL, with 100% sensitivity for detecting severe combined immunodeficiency. Using this value, retest and referral rates were 0.43% (37 samples) and 0.03% (3 samples), respectively.
CONCLUSION: The technique is reliable and can be applied on a large scale after the training of technical teams throughout Brazil.
Copyright © 2016. Published by Elsevier Editora Ltda.

Entities:  

Keywords:  Combined immunodeficiency; Imunodeficiência combinada; Imunodeficiência primária; Linfócitos T; Neonatal screening; Primary immunodeficiency; SCID; T lymphocytes; TRECs; Triagem neonatal

Mesh:

Substances:

Year:  2016        PMID: 27207231     DOI: 10.1016/j.jped.2015.10.006

Source DB:  PubMed          Journal:  J Pediatr (Rio J)        ISSN: 0021-7557            Impact factor:   2.197


  8 in total

1.  Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries.

Authors:  Juliana Folloni Fernandes; Samantha Nichele; Liane E Daudt; Rita B Tavares; Adriana Seber; Fábio R Kerbauy; Adriana Koliski; Gisele Loth; Ana K Vieira; Luiz G Darrigo-Junior; Vanderson Rocha; Alessandra A Gomes; Vergílio Colturato; Luiz F Mantovani; Andreza F Ribeiro; Lisandro L Ribeiro; Cilmara Kuwahara; Ana L M Rodrigues; Victor G Zecchin; Beatriz T Costa-Carvalho; Magda Carneiro-Sampaio; Antonio Condino-Neto; Anders Fasth; Andrew Gennery; Ricardo Pasquini; Nelson Hamerschlak; Carmem Bonfim
Journal:  J Clin Immunol       Date:  2018-11-24       Impact factor: 8.317

2.  Recommendations for uniform definitions used in newborn screening for severe combined immunodeficiency.

Authors:  Maartje Blom; Rolf H Zetterström; Asbjørg Stray-Pedersen; Kimberly Gilmour; Andrew R Gennery; Jennifer M Puck; Mirjam van der Burg
Journal:  J Allergy Clin Immunol       Date:  2021-09-16       Impact factor: 14.290

3.  Utilization of genomic sequencing for population screening of immunodeficiencies in the newborn.

Authors:  Ashleigh R Pavey; Dale L Bodian; Thierry Vilboux; Alina Khromykh; Natalie S Hauser; Kathi Huddleston; Elisabeth Klein; Aaron Black; Megan S Kane; Ramaswamy K Iyer; John E Niederhuber; Benjamin D Solomon
Journal:  Genet Med       Date:  2017-06-15       Impact factor: 8.822

4.  A Novel Homozygous JAK3 Mutation Leading to T-B+NK- SCID in Two Brazilian Patients.

Authors:  Lucila A Barreiros; Gesmar R S Segundo; Anete S Grumach; Pérsio Roxo-Júnior; Troy R Torgerson; Hans D Ochs; Antonio Condino-Neto
Journal:  Front Pediatr       Date:  2018-08-20       Impact factor: 3.418

5.  IN TIME: THE VALUE AND GLOBAL IMPLICATIONSOF NEWBORN SCREENING FORSEVERE COMBINED IMMUNODEFICIENCY.

Authors:  Cristina Meehan; Carmem Bonfim; Joseph F Dasso; Beatriz Tavares Costa-Carvalho; Antonio Condino-Neto; Jolan Walter
Journal:  Rev Paul Pediatr       Date:  2018 Oct-Dec

Review 6.  A Practical Approach to Newborn Screening for Severe Combined Immunodeficiency Using the T Cell Receptor Excision Circle Assay.

Authors:  Monica S Thakar; Mary K Hintermeyer; Miranda G Gries; John M Routes; James W Verbsky
Journal:  Front Immunol       Date:  2017-11-08       Impact factor: 7.561

7.  Primary immunodeficiencies: a diagnostic challenge?

Authors:  Dirceu Solé
Journal:  J Pediatr (Rio J)       Date:  2020-12-19       Impact factor: 2.990

8.  NEWBORN SCREENING FOR SEVERE COMBINED IMMUNODEFICIENCIES USING TRECS AND KRECS: SECOND PILOT STUDY IN BRAZIL.

Authors:  Marilia Pyles P Kanegae; Lucila Akune Barreiros; Jusley Lira Sousa; Marco Antônio S Brito; Edgar Borges de Oliveira; Lara Pereira Soares; Juliana Themudo L Mazzucchelli; Débora Quiorato Fernandes; Sonia Marchezi Hadachi; Silvia Maia Holanda; Flavia Alice T M Guimarães; Maura Aparecida P V V Boacnin; Marley Aparecida L Pereira; Joaquina Maria C Bueno; Anete Sevciovic Grumach; Regina Sumiko W Di Gesu; Amélia Miyashiro N Dos Santos; Newton Bellesi; Beatriz T Costa-Carvalho; Antonio Condino-Neto
Journal:  Rev Paul Pediatr       Date:  2017 Jan-Mar
  8 in total

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