Overview

This report summarizes a Brazilian survey of specialized centers caring for patients with primary ciliary dyskinesia (PCD). The analysis is descriptive and center-level. It includes confirmed PCD patients, clinically suspected PCD patients, diagnostic method availability, and geographic distribution of centers.

The dataset includes 24 responding centers across 13 Brazilian states.

24
Centers
13
States
229
Confirmed PCD patients
396
Clinically suspected patients
625
Total confirmed + suspected

Patient Volume by Center

The figure below shows confirmed and clinically suspected PCD patients in each center. Bars are ordered by total patient volume.

Center City State Age group served Confirmed PCD Clinical suspicion Total
UNICAMP (Pediatria) Campinas SP Children and adolescents 25 60 85
InCor-USP São Paulo SP Adults 50 30 80
Hospital de Messejana - Dr Carlis Alberto Studart Gomes Fortaleza CE Adults 0 62 62
Instituto da Criança e do Adolescente HCFMUSP São Paulo SP Children and adolescents 25 25 50
Hospital São Lucas-PUCRS Porto Alegre RS All ages 7 40 47
Hospital do Pulmão Blumenau SC All ages 0 40 40
Instituto Nacional da Saúde da Mulher, Criança e Adolescente Fernandes Figueira - IFF-FIOCRUZ Rio de Janeiro RJ Children and adolescents 17 18 35
Universidade do Estado do Rio de Janeiro Rio de Janeiro RJ Adults 8 17 25
Hospital de Clínicas-UFRGS (Adultos) Porto Alegre RS Adults 4 20 24
Hospital Infantil Albert Sabin Fortaleza CE Children and adolescents 2 18 20
Hospital Infantil João Paulo II Belo Horizonte MG Children and adolescents 8 10 18
Hospital Universitario Onofre Lopes-UFRN Natal RN All ages 10 7 17
Hospital de Clínicas-UFG Goiânia GO All ages 9 7 16
Hospital Estadual Infantil Nossa Senhora da Glória Vitória ES Children and adolescents 5 11 16
Hospital Universitário Professor Edgard Santos-UFBA Salvador BA Children and adolescents 12 2 14
Hospital de Clínicas-UFPR Curitiba PR Children and adolescents 7 7 14
IMIP - Instituto de Medicina Integral Professor Fernando Figueira Recife PE All ages 10 0 10
Hospital da Criança de Brasilia José Alencar Brasília DF Children and adolescents 9 0 9
Hospital de Clínicas-UFRGS (Pediatria) Porto Alegre RS Children and adolescents 3 6 9
UNICAMP (Adultos) Campinas SP Adults 8 0 8
Hospital Universitário - UFJF Juiz de Fora MG All ages 5 3 8
Hospital Santa Casa de Misericórdia de Vitória Vitória ES Adults 1 7 8
Hospital das Clinicas-UFMG Belo Horizonte MG Children and adolescents 2 5 7
Hospital da Criança Santo Antonio Porto Alegre RS Children and adolescents 2 1 3

Geographic Distribution

Circle area is proportional to the total number of confirmed plus clinically suspected PCD patients in each center. Centers in the same city were slightly offset to keep overlapping circles visible.

Diagnostic Method Availability

The next figure summarizes the availability status of each diagnostic method as the percentage of centers reporting each category.

The center-by-method matrix shows the reported operational status for every center and diagnostic modality.

Method Status Centers Percent
Electron microscopy On-site 2 8.3%
Electron microscopy Partnered service 5 20.8%
Electron microscopy Being implemented 1 4.2%
Electron microscopy Not available 16 66.7%
Genetics On-site 5 20.8%
Genetics Partnered service 11 45.8%
Genetics Being implemented 2 8.3%
Genetics Not available 6 25.0%
Immunofluorescence On-site 1 4.2%
Immunofluorescence Partnered service 7 29.2%
Immunofluorescence Not available 16 66.7%
Nasal NO On-site 3 12.5%
Nasal NO Partnered service 4 16.7%
Nasal NO Being implemented 2 8.3%
Nasal NO Not available 15 62.5%
Sweat test On-site 15 62.5%
Sweat test Partnered service 7 29.2%
Sweat test Being implemented 1 4.2%
Sweat test Not available 1 4.2%
Videomicroscopy On-site 1 4.2%
Videomicroscopy Partnered service 3 12.5%
Videomicroscopy Not available 20 83.3%

Planned Diagnostic Implementation

The table below summarizes diagnostic methods that centers reported planning to implement in the next two years.

Method Centers Percent
Genetics 11 45.8%
Nasal NO 10 41.7%
Immunofluorescence 4 16.7%
Electron microscopy 3 12.5%
Videomicroscopy 1 4.2%

Nasal Brushing for Immunofluorescence

Interest in nasal brushing sample referral Centers Percent
Yes 24 100.0%

Responsible Physicians and Centers

The list below includes the physician responsible for each center, the hospital or institution, city, and state. The first author is listed first; the remaining centers are ordered by total patient volume.

Order Responsible physician(s) Hospital or institution City State
1 Marcus Jones PUCRS Porto Alegre RS
2 José Dirceu Ribeiro UNICAMP (Pediatria) Campinas SP
3 Rodrigo Athanazio e Samia Rached InCor-USP São Paulo SP
4 Mara Rúbia Fernandes de Figueiredo Hospital de Messejana - Dr Carlis Alberto Studart Gomes Fortaleza CE
5 Luiz Vicente Ribeiro F da Silva-Filho Instituto da Criança e do Adolescente HCFMUSP São Paulo SP
6 Marina Andrade Lima Hospital do Pulmão Blumenau SC
7 Patrícia Fernandes Barreto Machado Costa Instituto Nacional da Saúde da Mulher, Criança e Adolescente Fernandes Figueira - IFF-FIOCRUZ Rio de Janeiro RJ
8 Raphael Freitas Jaber de Oliveira Universidade do Estado do Rio de Janeiro Rio de Janeiro RJ
9 Paulo de Tarso Roth Dalcin Hospital de Clínicas-UFRGS (Adultos) Porto Alegre RS
10 Ângela Elizabeth Holanda Araújo Freitas Hospital Infantil Albert Sabin Fortaleza CE
11 Chalene Guimarães soares Mezêncio Hospital Infantil João Paulo II Belo Horizonte MG
12 Vera Maria Dantas Hospital Universitario Onofre Lopes-UFRN Natal RN
13 Lusmaia Damaceno Camargo Costa Hospital de Clínicas-UFG Goiânia GO
14 Nyella Miossi Pinto Hospital Estadual Infantil Nossa Senhora da Glória Vitória ES
15 Edna Lúcia Souza Hospital Universitário Professor Edgard Santos-UFBA Salvador BA
16 Kharine Yukari Fujii e Débora Chong Hospital de Clínicas-UFPR Curitiba PR
17 Patrícia Gomes de Matos Bezerra IMIP - Instituto de Medicina Integral Professor Fernando Figueira Recife PE
18 Luciana Monte Hospital da Criança de Brasilia José Alencar Brasília DF
19 Valentina C. B. Gava Chakr Hospital de Clínicas-UFRGS (Pediatria) Porto Alegre RS
20 Eulalia Sakano UNICAMP (Adultos) Campinas SP
21 Marta Cristina Duarte; Célio de Barros Barbosa Hospital Universitário - UFJF Juiz de Fora MG
22 Faradiba Sarquis Serpa Hospital Santa Casa de Misericórdia de Vitória Vitória ES
23 Claudia Ribeiro de Andrade Hospital das Clinicas-UFMG Belo Horizonte MG
24 Gilberto Bueno Fischer Hospital da Criança Santo Antonio Porto Alegre RS