TY  - CHAP
M1  - Book, Section
TI  - Sickle Cell Disease in Brazil
A1  - Costa, Author: Fernando Ferreira
A2  - Gladwin, Mark T.
A2  - Kato, Gregory J.
A2  - Novelli, Enrico M.
Y1  - 2021
N1  - 
T2  - Sickle Cell Disease
AB  - Brazil  is  a  continental  country  with  a  very  heterogeneous  population  in  regard  to  ethnic  origin.  Although  most  of  the  Native  American  (ie,  Indian)  population  is  extinct  today,  their  genomic  inheritance  has  contributed  to  the  ethnic  mix  of  the  current  Brazilian  population.  The  country  was  colonized  by  the  Portuguese,  but  from  the  16th  to  19th  centuries,  it  received  about  4  million  slaves  from  Africa.  From  the  19th  century  to  the  middle  of  20th  century,  a  very  important  contingent  of  immigrants  from  several  countries,  including  Italy  (1.5  million),  Spain  (0.6  million),  Japan,  Germany,  and  Lebanon,  arrived  and  integrated  into  the  Brazilian  genetic  pool.  Several  studies  have  shown  that  these  different  ethnic  groups  are  continuously  in  a  process  of  admixture,  which  indicates  that  Brazil  has  an  extremely  unique  population  regarding  genetic  diseases  and,  specifically,  regarding  hemoglobinopathies.  In  addition,  it  is  important  to  emphasize  that  the  population  is  heterogeneously  distributed  within  its  different  geographical  regions.  Hence,  patients  with  sickle  cell  disease  (SCD)  in  Brazil  constitute  a  very  singular  admixture-rich  ethnic  population.  Studies  that  have  compared  the  genetic  makeup  of  SCD  patient  cohorts  from  Brazil  and  the  United  States  have  revealed  high  levels  of  divergence  among  cases,  with  a  higher  European  genomic  background  in  Brazil.  Moreover,  in  some  region  of  the  country,  patients  with  HbS/β  thalassemia  compose  almost  30%  of  all  SCD  patients.  Almost  all  patients  with  SCD  in  the  country  are  treated  within  the  Public  Healthcare  System  (Sistema  Único  de  Saúde  [SUS]),  which  provides  universal  care  and  covers  all  health-related  costs.  To  provide  care  to  patients  with  SCD,  the  system  is  organized  by  government-funded  special  hematology  centers  affiliated  with  state  or  federal  medical  institutions,  and  these  are  named  hemocentros  (hemocenters).  In  each  of  the  26  Brazilian  states,  at  least  1  of  these  centers  is  responsible  for  the  diagnosis  and  treatment  of  SCD  patients.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - hemonc.mhmedical.com/content.aspx?aid=1179345866
ER  -