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Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates

Hemoglobinopathies are highly prevalent diseases and impose a public health burden. Early diagnosis and treatment can ameliorate the course of these diseases and improve survival. Despite purported high incidence of hemoglobinopathies in Lebanon, there are no nationwide screening programs. In this s...

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Autores principales: Khoriaty, Evelyne, Halaby, Rim, Berro, Mohamad, Sweid, Ahmad, Abbas, Hussein A., Inati, Adlette
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4152148/
https://www.ncbi.nlm.nih.gov/pubmed/25180595
http://dx.doi.org/10.1371/journal.pone.0105109
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author Khoriaty, Evelyne
Halaby, Rim
Berro, Mohamad
Sweid, Ahmad
Abbas, Hussein A.
Inati, Adlette
author_facet Khoriaty, Evelyne
Halaby, Rim
Berro, Mohamad
Sweid, Ahmad
Abbas, Hussein A.
Inati, Adlette
author_sort Khoriaty, Evelyne
collection PubMed
description Hemoglobinopathies are highly prevalent diseases and impose a public health burden. Early diagnosis and treatment can ameliorate the course of these diseases and improve survival. Despite purported high incidence of hemoglobinopathies in Lebanon, there are no nationwide screening programs. In this study, newborn screening utilizing high pressure liquid chromatography was executed in all public hospitals across Lebanon between 2010 and 2013. All newborns with an abnormal hemoglobin (Hb) were offered genetic counseling and all those with disease were enrolled in comprehensive hemoglobinopathy clinics. Among newborns, 2.1% were found to have an abnormal Hb variant with sickle Hb being the most common while 0.1% were found to have sickle cell disease (SCD). The majority of those with SCD had non-Lebanese origins. The most common causes of hospitalizations in infants with SCD were acute splenic sequestration and pain crises. No bacteremia or other life threatening infections were noted. At a median follow up 14 months (follow up range 7 to 34 months), all children with disease are alive and compliant with treatment. Systematic screening for SCD and other Hb variants was shown to be feasible, cost effective, and of accurate predictive value. This program was also clinically effective because it led to the identification of babies with disease and to providing them with free early multidisciplinary care. Conclusively, a newborn screening program should be implemented across Lebanon to detect hemoglobinopathies and initiate early therapeutic and preventive strategies and genetic counseling.
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spelling pubmed-41521482014-09-05 Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates Khoriaty, Evelyne Halaby, Rim Berro, Mohamad Sweid, Ahmad Abbas, Hussein A. Inati, Adlette PLoS One Research Article Hemoglobinopathies are highly prevalent diseases and impose a public health burden. Early diagnosis and treatment can ameliorate the course of these diseases and improve survival. Despite purported high incidence of hemoglobinopathies in Lebanon, there are no nationwide screening programs. In this study, newborn screening utilizing high pressure liquid chromatography was executed in all public hospitals across Lebanon between 2010 and 2013. All newborns with an abnormal hemoglobin (Hb) were offered genetic counseling and all those with disease were enrolled in comprehensive hemoglobinopathy clinics. Among newborns, 2.1% were found to have an abnormal Hb variant with sickle Hb being the most common while 0.1% were found to have sickle cell disease (SCD). The majority of those with SCD had non-Lebanese origins. The most common causes of hospitalizations in infants with SCD were acute splenic sequestration and pain crises. No bacteremia or other life threatening infections were noted. At a median follow up 14 months (follow up range 7 to 34 months), all children with disease are alive and compliant with treatment. Systematic screening for SCD and other Hb variants was shown to be feasible, cost effective, and of accurate predictive value. This program was also clinically effective because it led to the identification of babies with disease and to providing them with free early multidisciplinary care. Conclusively, a newborn screening program should be implemented across Lebanon to detect hemoglobinopathies and initiate early therapeutic and preventive strategies and genetic counseling. Public Library of Science 2014-09-02 /pmc/articles/PMC4152148/ /pubmed/25180595 http://dx.doi.org/10.1371/journal.pone.0105109 Text en © 2014 Khoriaty et al http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Research Article
Khoriaty, Evelyne
Halaby, Rim
Berro, Mohamad
Sweid, Ahmad
Abbas, Hussein A.
Inati, Adlette
Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates
title Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates
title_full Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates
title_fullStr Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates
title_full_unstemmed Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates
title_short Incidence of Sickle Cell Disease and Other Hemoglobin Variants in 10,095 Lebanese Neonates
title_sort incidence of sickle cell disease and other hemoglobin variants in 10,095 lebanese neonates
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4152148/
https://www.ncbi.nlm.nih.gov/pubmed/25180595
http://dx.doi.org/10.1371/journal.pone.0105109
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