Positive impacts of universal newborn screening on the outcome of children with sickle cell disease in the province of Quebec: A retrospective cohort study.

A universal newborn screening program for sickle cell disease (uNS-SCD) was implemented in the province of Québec (Qc) in November 2013, close in time to the recommendation of early initiation of hydroxyurea (HU) therapy for children. This retrospective cohort study evaluated the impact of such a program on children first seen between January 2000 and December 2019. Cohorts pre-SCD-uNS in Qc (pre-QcNS) (n = 253) and post-QcNS (n = 157) for patients seen prior to or after Nov 2013 were compared. Kaplan-Meier curves, Poisson regression, and logistic regressions were used for statistical analysis, using Software R version 4.2.1. Median age at first visit decreased significantly from 14.4 [interquartile range: 2.4-72.0] to 1.2 months [1.2-57.6] (p < 0.001). The percentage of children born in Qc undiagnosed at birth and referred after a first SCD-related complication dropped from 42.6% to 0.0% (p < 0.0001). The median age of HU introduction for patients with SS/Sß°-thalassemia decreased from 56.4 [31.2-96.0] to 9.0 months post-QcNS [8.0-12.1] (p < 0.001). Event-free survival improved significantly for any type of hospitalization as well as for vaso-occlusive crisis (VOC) (140-257 days (p < 0.001) and 1320 vs. 573 days (p < 0.002), respectively), resulting in a reduction from 2 [interquartile range: 1.0-3.0] to 1.0 hospitalizations/patient-year [0.6-1.4] (p < 0.001). Children with SS/Sß°-thalassemia referred post-QcNS also had fewer emergency department visits for VOC (RR: 0.69, 95% confidence interval: 0.54-0.88). The Universal NS program allows early detection and referral of children with SCD to comprehensive care centers. Earlier access ensures that children benefit from essential preventive interventions, reducing disease burden. This cohort study highlights that uNS-SCD is an essential public health measure.

[Iodine concentration in cooking salt consumed in Lubumbashi and the iodine status of vulnerable people: case study of pregnant women living in underprivileged areas]. / La teneur en iode du sel de cuisine consommé à Lubumbashi et le statut iode des personnes vulnérables: cas de femmes enceintes de milieux défavorisés.

INTRODUCTION: Consumption of low iodine salt can cause different types of disorders associated with iodine deficiency. This study aims to determine iodine content in table salt consumed in Lubumbashi and iodine status of pregnant women who are the main target of iodine deficiency. METHODS: aAdescriptive cross-sectional study was devoted to an iodometric iodine analysis of 739 salt samples collected from the households and the markets of Lubumbashi in 2014. Previously, urinary iodine concentrations were determined in 225 pregnant women received for consultation from 15 March 2009 to 25 April 2011 by mineralization technique using ammonium persulphate. RESULTS: Our survey found that 47.5% of the cooking salt samples were adequately iodized (from 15 to 40 ppm), 36,9% of the samples had low iodine levels, 7,4% of the samples had too much iodine and 8,1% of the samples were not iodized. Iodine concentration in the analyzed cooking salt reached an overall average of 54,9%, being clearly below the WHO Standards (90%). By measuring urinary iodine concentration of pregnant women, iodine deficiency (urinary iodine <150 µg/l) was observed in 52%. CONCLUSION: The low availability of iodine from consumed salt in Lubumbashi could be responsible for a large proportion of the observed iodine deficiency in pregnant women, exposing them to the major risks for disorders associated with iodine deficiency.