Characteristics of paroxysmal nocturnal hemoglobinuria patients in Brazil: A retrospective administrative claims database analysis of PNH patients in Brazilian public healthcare system

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art_GUALANDRO_Characteristics_of_paroxysmal_nocturnal_hemoglobinuria_patients_in_Brazil_2023.PDF (783.71 KB)
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0
Tipo de produção
article
Data de publicação
2023
DOI
Scopus
Web of Science
PubMed
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Título do Volume
Editora
PUBLIC LIBRARY SCIENCE
Autores
SALVINO, Marco Aurelio
JEHA, Thaina
Citação
PLOS ONE, v.18, n.7, 2023
Projetos de Pesquisa
Unidades Organizacionais
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IntroductionFew studies have reported the profile of patients with paroxysmal nocturnal hemoglobinuria (PNH) and their care in the Brazilian health system. ObjectiveTo describe clinical and epidemiological characteristics of patients with PNH in the Brazilian public health system including procedures performed, associated comorbidities and visits to health care professionals. MethodsIn a real-world observational, retrospective, population-based cohort study, anonymized secondary data provided by the Department of Informatics of the Brazilian Unified Health System (DATASUS) were analyzed. Patients were considered eligible if they had at least one procedure coded with the ICD-10 code D59.5 from January 1, 2008 to December 30, 2018. ResultsIn total, 675 individual PNH patients were identified (52.4% female; prevalence of 1:237,000 people). Around 15.8% of the patients included had myelodysplastic syndrome and about half of the sample had other aplastic anemias and/or other bone marrow failure syndromes. Portal vein thrombosis (I82 ICD code) was reported in 4.3% of patients. Regarding hospitalizations, 263 individual PNH patients had 416 inpatient admissions with the ICD code for PNH (D59.5) on admission. Twelve deaths occurred during the study period, of which two had the PNH ICD code related with the cause of death, while another three deaths were associated with acquired hemolytic anemia (D59.9), unspecified aplastic anemia (D61.9) and acute respiratory failure (J96.0), respectively. ConclusionDespite its limitations, this statistical analysis of data extracted from DATASUS reasonably describes PNH patients in Brazil and its variations across different regions of the country. Comorbidities frequently associated with PNH such as portal vein thrombosis were not as common in our study, but it is assumed that several thrombotic events at specific sites were coded under the broader I82 ICD code. The frequency of visits to different health professionals, including hematologists, increased after the diagnosis of PNH. Among hospitalized PNH patients, the mortality rate was 4.5%.
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https://observatorio.fm.usp.br/handle/OPI/55974
Referências
  1. Bittencourt Sonia Azevedo, 2006, Cad. Saúde Pública, V22, P19, DOI 10.1590/S0102-311X2006000100003
  2. Brasil. Ministerio da Saude, 2020, INP INF SYST SIH
  3. Brodsky RA, 2008, ANN INTERN MED, V148, P587, DOI 10.7326/0003-4819-148-8-200804150-00003
  4. Brodsky RA, 2014, BLOOD, V124, P2804, DOI 10.1182/blood-2014-02-522128
  5. Cunha M., 2011, REV BRAS HEMATOL HEM, V33, P35
  6. Curran KJ, 2012, PEDIATR BLOOD CANCER, V59, P525, DOI 10.1002/pbc.23410
  7. de Azambuja AP, 2021, BLOOD CELL MOL DIS, V92, DOI 10.1016/j.bcmd.2021.102605
  8. Hill A, 2007, BRIT J HAEMATOL, V137, P181, DOI 10.1111/j.1365-2141.2007.06554.x
  9. Hill A, 2012, BRIT J HAEMATOL, V158, P409, DOI 10.1111/j.1365-2141.2012.09166.x
  10. HILLMEN P, 1995, NEW ENGL J MED, V333, P1253, DOI 10.1056/NEJM199511093331904
  11. Instituto Brasileiro de Geografia e Estatistica, 2020, TAB 3175 POP RES COR
  12. Kelly Richard, 2009, Ther Clin Risk Manag, V5, P911
  13. Ministerio da Saude. Departamento de Informatica do Sistema Unico de Saude, 2020, OUTP INF SYST SIA
  14. Miotto BA, 2018, BMC HEALTH SERV RES, V18, DOI 10.1186/s12913-018-3076-z
  15. Naithani R, 2008, INDIAN J PEDIATR, V75, P575, DOI 10.1007/s12098-008-0111-9
  16. Parker CJ, 2016, HEMATOL-AM SOC HEMAT, P208, DOI 10.1182/asheducation-2016.1.208
  17. da Silva BGPP, 2022, ANN HEMATOL, V101, P999, DOI 10.1007/s00277-022-04797-9
  18. Risitano Antonio M, 2008, Biologics, V2, P205
  19. Sahin F, 2015, AM J BLOOD RES, V5, P1
  20. Sakurai M, 2019, INT J HEMATOL, V110, P411, DOI 10.1007/s12185-019-02699-7
  21. Schrezenmeier H, 2014, HAEMATOLOGICA, V99, P922, DOI 10.3324/haematol.2013.093161
  22. Socié G, 2016, INTERN MED J, V46, P1044, DOI 10.1111/imj.13160
  23. Socie G, 1996, LANCET, V348, P573, DOI 10.1016/S0140-6736(95)12360-1
  24. van den Heuvel-Eibrink MM, 2005, BRIT J HAEMATOL, V128, P571, DOI 10.1111/j.1365-2141.2004.05337.x
  25. Wang HC, 2017, KAOHSIUNG J MED SCI, V33, P405, DOI 10.1016/j.kjms.2017.05.011
  26. WARE RE, 1991, NEW ENGL J MED, V325, P991, DOI 10.1056/NEJM199110033251403
  27. Wells R., 2011, ICD 10 INT STAT CLAS, V10th
  28. Yamakawa PE, 2019, BLOOD, V134, DOI 10.1182/blood-2019-132023
  29. Yu F, 2016, INT J HEMATOL, V103, P649, DOI 10.1007/s12185-016-1995-1

Coleções
Artigos e Materiais de Revistas Científicas - FM/MCM
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/31