Brazilian Cystic Fibrosis Registry 2011

Brazilian Cystic Fibrosis Registry 2011

Brazilian
Cystic Fibrosis
Patient Registry
2011 Annual Report
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
BRAZILIAN CYSTIC FIBROSIS PATIENT REGISTRY (REBRAFC)
2011 ANNUAL REPORT
Patient Registry
The Web platform incorporates several security mechanisms for data input and information storage, so that
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available for research or multicenter studies without the agreement of each participant center.
This report contains data obtained from several Brazilian Centers of Cystic Fibrosis (CF) care. It is the third annual edition produced, and a trend for growth can be observed yet, with a total of 2,182 patients enrolled in the
database. The evolution of registries and follow-up data is depicted in the Figure below:
%FTQJUFUIFTJHOJţDBOUJODSFBTFJOUIFQBSUJDJQBUJPOPGTFWFSBMOBUJPOBM$'DFOUFSTNBOZPGUIFNIBWFOPUZFU
entered data or did it incompletely, for various reasons, such as difficulties in the approval by the local ethJDTDPNNJUUFFTPSTJNQMZCZMBDLPGJOJUJBUJWFPGUIFQSPGFTTJPOBMT5IF$FOUFSTXIPTJHOFEUIFDPOţEFOUJBMJUZ
In order to improve the treatment of CF in Brazil, detailed information regarding patients’ characteristics and
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current treatment in different Centers and country regions is essential.
The Brazilian Cystic Fibrosis Patient Registry was implemented and is fully maintained by the Brazilian Cystic
Fibrosis Study Group (GBEFC), with the following objectives:
About Cystic Fibrosis and the GBEFC:
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new therapeutic strategies;
some specialized centers with health professionals dedicated to patient care for many years. Treatment is quite
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5IF#SB[JMJBO$ZTUJD'JCSPTJT4UVEZ(SPVQ(#&'$
JTBOPOQSPţUPSHBOJ[BUJPODPNQSJTFEPGIFBMUIDBSFQSPfessionals working in the area, created on November 2003. Among the activities of the GBEFC, we can cite
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SFTFBSDITUBŢUSBJOJOHBOEBJEJOUIFEFWFMPQNFOUPG$'USFBUNFOUDFOUFSTJOUIFDPVOUSZPSHBOJ[JOHTDJFOUJţD
Ŕ*EFOUJGZTQFDJBMHSPVQTGPSNVMUJDFOUFSTUVEJFT
NFFUJOHTGPVSFEJUJPOTPGUIF#SB[JMJBO$'$POHSFTT
XPSLJOHXJUIUIF.JOJTUSZPG)FBMUIUPEFţOFBOBUJPOBM
Ŕ*EFOUJGZUIFDIBSBDUFSJTUJDTPGBTTJTUBODFJOFBDI#SB[JMJBOTUBUF$FOUFSGPSQMBOOJOHBTTJTUBODFBDUJPOT
protocol of CF care and implementation of Newborn Screening in all Brazilian states.
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5IF(#&'$NBJOUBJOTBO*OUFSOFUTJUFXXXHCFGDPSHCS
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publications and resources, and also displays the Patient Registry Reports in Portuguese and English versions
for free download.
The staff of the Laboratório de Sistemas Integráveis (LSI) from Escola Politécnica de Engenharia of University of São Paulo is responsible for
the development and maintenance of the Web
platform where the REBRAFC runs. All participat-
2009
2500
2010
2182
2000
1798
1500
ing centers were asked to obtain approval of the
local Ethics Committees and to obtain consent
1440
1000
1562
1252
993
GSPNQBSFOUTBOEPSHVBSEJBOTBOEEJSFDUMZGSPN
older patients, to allow insertion of their data in
2011
500
UIF EBUBCBTF " MFBŤFU FYQMBJOJOH UIF 3FHJTUSZ
SVMFTBOECFOFţUTXBTEJTUSJCVUFEUPQBUJFOUTBOE
QBSFOUTDBSFHJWFST
2
0
Registries
Follow-ups
3
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
EXECUTIVE COMMITTE OF THE BRAZILIAN CYSTIC FIBROSIS REGISTRY:
Patient Registry
It’s interesting to notice that near 30% of the patients
already have three years of follow-up data inserted
Dr. Luiz Vicente Ribeiro Ferreira da Silva Filho
(Table 1).
Ŕ &YFDVUJWFDPPSEJOBUPSPGUIF3&#3"'$
In the description of demographic and diagnostic data,
Ŕ "TTJTUBOUQSPGFTTPSBUUIF1FEJBUSJD1VMNPOPMPHZ6OJU*OTUJUVUPEB$SJBO£B)$'.641
all patients were included. Only data from the year 2011
Table 1
Patients’ distribution by time of
follow-up.
n
%
No follow-up
183
8.4
1 year
654
30.0
2 years
698
32.0
Dr. Francisco José Caldeira Reis
3 years
645
29.6
Ŕ 1SPGFTTPSPG1FEJBUSJDTBU'FEFSBM6OJWFSTJUZPG.JOBT(FSBJT
4 years
2
0.1
2,182
100
Ŕ 3FTFBSDIFSBUUIF3FTFBSDIBOE-FBSOJOH*OTUJUVUFPG)PTQJUBM*TSBFMJUB"MCFSU&JOTUFJOBOEBU*OTUJUVUPEF.FEJDJOB
Tropical of University of São Paulo
(1,562 patients) were included in the Follow-up data.
Time of follow-up
Ŕ Vice-President of the Brazilian Cystic Fibrosis Study Group (GBEFC)
Ŕ President of the Brazilian Cystic Fibrosis Study Group (GBEFC)
Total
Ŕ 1FEJBUSJD1VMNPOPMPHJTUUSBJOFEBU6OJWFSTJUZPG.BOJUPCBŊ$IJMEFOōT)PTQJUBMPG8JOOJQFHŊ.BOJUPCBŊ$BOBEB
Ŕ "EWJTPSPGUIF)PTQJUBM*OGBOUJM+PŸP1BVMP**3FEF')&.*(#FMP)PSJ[POUF.JOBT(FSBJT
Dra. Neiva Damaceno
Ŕ "TTJTUBOU1SPGFTTPSPGUIF1FEJBUSJD1VMNPOPMPHZ(SPVQPG'BDVMEBEFEF$J¦ODJBT.¥EJDBTEB4BOUB$BTBEF4ŸP
Paulo
Ŕ &Y1SFTJEFOUPGUIF#SB[JMJBO$ZTUJD'JCSPTJT4UVEZ(SPVQ(#&'$
Adilson Yuuji Hira
Ŕ Laboratory of Integrated Systems, Escola Politécnica of University of São Paulo.
4
5
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Table 2
Figure 1
Distribution of patients by Brazilian State of origin (birth place).
Distribution of patients by Brazilian State of origin (birth place).
Brazilian State of origin
n (%)
Brazilian State of origin
n (%)
São Paulo
Rio Grande do Sul
Bahia
Minas Gerais
Santa Catarina
Paraná
Espirito Santo
Rio de Janeiro
Ceará
Pará
Outros
Alagoas
Rio Grande do Norte
744
316
306
São Paulo
.BUP(SPTTP
12 (0.5%)
Rio Grande do Sul
316 (14.5%)
.BUP(SPTTPEP4VM
Bahia
306 (14.0%)
Pernambuco
6 (0.3%)
.JOBT(FSBJT
Amazonas
5 (0.2%)
Santa Catarina
138 (6.3%)
Sergipe
4 (0.2%)
Paraná
Distrito Federal
3 (0.1%)
Espirito Santo
Piauí
3 (0.2%)
3JPEF+BOFJSP
Rondônia
2 (0.1%)
Ceará
61 (2.8%)
Acre
1 (0.04%)
3FHBSEJOH#SB[JMJBO4UBUFPGPSJHJOBTJHOJţDBOUJODSFBTFXBTOPUJDFEJO&TQJSJUP4BOUPDBTFJOBOE
Pará
54 (2.5%)
.BSBOIŸP
1 (0.04%)
DBTFTJO
BOEJO1BSBOUPDBTFT
/POFXDBTFTXFSFSFHJTUFSFEJOUIF4UBUFTPG.BUP(SPTTP
Alagoas
26 (1.2%)
Paraíba
1 (0.04%)
Rio Grande do Norte
22 (1.0%)
Roraima
1 (0.04%)
Not informed
19 (0.9%)
207
138
97
75
71
61
54
46
26
22
0
100
200
300
400
500
600
700
800
Number of patients
.BSBOIŸPBOEBMTPJOUIF4UBUFTPGUIF/PSUI#SB[JMJBO3FHJPO
800
700
744
650
600
500
400
278
300
316
269
306
Espirito Santo
Santa Catarina
Minas Gerais
Bahia
Rio Grande do Sul
2010
6
97
54 54
53 61
1
São Paulo
0
63
2011
25 26
22 22
41 46
Outros
75 66 71
100
Rio Grande do Norte
138
Ceará
121
Pará
142
Alagoas
207
200
Paraná
2,182 (100%)
Rio de Janeiro
Total
7
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Table 3
Table 4
Distribution of patients by Brazilian Region of origin (birth place).
Distribution of patients by Brazilian State where they are treated.
Brazilian Region of origin
Southeast
n (%)
Brazilian Region of origin
n (%)
Brazilian State of treatment
n (%)
Brazilian State of treatment
n (%)
North
63 (2.9%)
São Paulo
Pará
55 (2.5%)
South
551 (25.3%)
Center West
22 (1.0%)
Rio Grande do Sul
Ceará
62 (2.8%)
Northeast
Not informed
19 (0.9%)
Bahia
314 (14.4%)
Alagoas
26 (1.2%)
.JOBT(FSBJT
195 (8.9%)
Rio Grande do Norte
23 (1.1%)
Santa Catarina
123 (5.6%)
Pernambuco
2 (0.1%)
Paraná
Distrito Federal
3JPEF+BOFJSP
65 (3.0%)
Total
2,182 (100%)
Figure 2
1 (0.04%)
Distribution of patients by Brazilian Region of origin (birth place).
Total of patients
2,182 (100%)
Not informed
1%
Center West
1%
Southeast
50%
North
3%
Northeast
20%
South
25%
8
9
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 3
Table 5
Distribution of patients by Brazilian State where they are treated.
Distribution of patients by Brazilian State where they are treated
using only follow-up data from years 2009, 2010, and 2011.
800
790
Follow-up year
700
2009
2010
2011
n (%)
n (%)
n (%)
São Paulo
392 (39.5%)
612 (42.5%)
Rio Grande do Sul
240 (24.2%)
268 (18.6%)
285 (18.2%)
Bahia
216 (21.8%)
210 (14.6%)
202 (12.9%)
28 (2.8%)
122 (8.5%)
Santa Catarina
5 (0.5%)
88 (6.1%)
106 (6.8%)
Paraná
40 (4%)
41 (2.6%)
-
18 (1.2%)
55 (5.5%)
-
-
1 (0.1%)
3 (0.2%)
49 (3.1%)
-
22 (1,5%)
23 (1.5%)
16 (1.6%)
22 (1.5%)
23 (1.5%)
Pernambuco
-
1 (0.1%)
-
Espírito Santo
-
-
Distrito Federal
-
-
1 (0.1%)
993 (100%)
1,440 (100%)
1,562 (100%)
600
500
400
343
314
300
.JOBT(FSBJT
195
62
Brazilian State of treatment
23
2
1
Distrito Federal
26
Pernanbuco
55
Rio Grande do Norte
Rio de Janeiro
Santa Catarina
Minas Gerais
Bahia
Rio Grande do Sul
São Paulo
0
65
Alagoas
100
103
Ceará
123
Pará
200
Paraná
Number of patients
Brazilian State of treatment
3JPEF+BOFJSP
Pará
Ceará
Alagoas
Rio Grande do Norte
Total of patients
10
11
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 4
Figure 5
Figure 6
Distribution of patients by Brazilian State where they are treated.
Distribution of patients by
gender.
Distribution of patients by
ethnic group.
Asian
0%
700
612
600
Black
6%
570
Follow-up year
392
285
268
240
216
210
300
200
Mestizo
22%
Male
54%
202
167
23
18 16 22
0
22 23
1 3
0 1 0
0 0
1 0 1
Table 7
Figure 7
Description of current age data
(best spirometry or last clinical
visit of the year)
Distribution of patients
according to current age (years).
Age (years)
.FBOTUBOEBSE
deviation)
Brazilian State of treatment
.FEJBOQQ
13.18 (10.90)
Ŋ
Table 6
.JOJNVN.BYJNVN
Gender and ethnic group of Brazilian patients.
Gender
n (%)
Ethnic Group
.BMF
1,168 (53.5%)
Caucasian
Female
1,014 (46.5%)
.FTUJ[P
Total of patients
2,182 (100%)
Black
138 (6.3%)
Asian
5 (0.2%)
Total of patients
12
n (%)
Ŋ
Total of patients
1,950
No information
232
Frequency
0
Distrito Federal
Santa Catarina
0 0
49
Espírito Santo
39
Pernanbuco
41
77
55
Ceará
5
Minas Gerais
Bahia
Rio Grande do Sul
53
Alagoas
40
28
São Paulo
106
Rio Grande do Norte
88
100
Rio de Janeiro
122
0
Female
46%
White
72%
Pará
400
2009 (993 patients)
2010 (1440 patients)
2011 (1562 patients)
Paraná
Number of patients
500
Age ( years )
2,182 (100%)
13
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Table 8
Figure 8
Distribution of patients by age group.
Distribution of patients by age group.
n (%)
Age group (adult-pediatric)
30,0%
n (%)
Up to 5 years
388 (19.9%)
.FOPTEFBOPT
> 5 a 10
522 (26.8%)
18 anos ou mais
415 (21.3%)
>10 a 15
431 (22.1%)
Total of patients
1,950 (100%)
>15 a 20
Patients without information
>20 a 25
120 (6.2%)
>25 a 30
>30 a 35
43 (2.2%)
>35 a 40
>40 a 45
>45 a 50
15 (0.8%)
>50 anos
29 (1.5%)
20,0%
232
Percent
Age Group
10,0%
0,0%
Total of patients
1,950 (100%)
Patients without
information
232
<= 5
5-10
10-15 15-20
20-25 25-30 30-35 35-40 40-45 45-50 >50
anos
Age range un years
Figure 9
Distribution of patients by age group category (pediatric / adult).
18 years and
older 21%
Younger than
18 years old
79%
14
15
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
DATA AT DIAGNOSIS
Table 9
Figure 10
Table 10
Age of patients at diagnosis.
Distribution of patients by age
at diagnosis.
Conditions for diagnosis.
Conditions for diagnosis
Age (years)
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
Patients without
information*
Ŋ
Ŋ
2,176
6
Frequency
.FBOTUBOEBSE
deviation)
Respiratory symptoms
Age at diagnosis (years)
16
n (%)
%FţDJUPGHSPXUINBMOVUSJUJPO
4UFBUPSSIFBPS.BMBCTPSQUJPO
850 (39.0%)
Neonatal screening (IRT)
481 (22.0%)
Clinical or surgical meconium ileus
Familial history
Sinus disease
148 (6.8%)
.FUBCPMJDEJTUVSCBODF
120 (5.5%)
&EFNBBOFNJB
Unknown condition
41 (1.9%)
Rectal prolapse
20 (0.9%)
Prolonged jaundice
Infertility
9 (0.4%)
Others*
120 (5.5%)
Total of patients
2,182 (100%)
17
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Table 11
Table 13
Sweat chloride testing results.
Diagnosis by neonatal screening - Dosage of immunoreactive
trypsinogen (IRT).
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
Chloride
Mass
Conductivity
(mEq/l)
(mg)
(mmol/l)
104.2 (18.10)
89.50 (69.00-105)
132.50 (100-181)
105.0 (96-115)
5.22-204.00
33-160
1,833
1,402
215
Dosage of immunoreactive
trypsinogen (IRT). (ng/ml)
1st dosage
2nd dosage
Mean of 2 dosages
.FBOTUBOEBSEEFWJBUJPO
210.5 (128.0)
14-902
449
337
454
1st dosage
2nd dosage
Mean of 2 dosages
n (%)
n (%)
n (%)
OHNM
15 (4.5%)
16 (3.5%)
šOHNM
322 (95.5%)
438 (96.5%)
OHNM
58 (12.9%)
šOHNM
392 (86.3%)
449
337
454
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
Cut-off limits for IRT values
Table 12
Other tests reported for diagnosis.
n (%)
.FBTVSFPGOBTBMQPUFOUJBMEJŢFSFODF
92 (4.2%)
Rectal biopsy
Total of patients
18
Total of patients
2,182 (100%)
19
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
GENETICS DATA
Table 14
Age in years at the diagnosis: with or without neonatal screening.
Genetics data contained in this report should be cautiously interpreted, since the approach for CF genetic
testing in Brazil is highly heterogeneous. Some Centers only perform detection of DeltaF508 mutation, while
others search panels of 2, 4 or up to 30 or more mutations. There are also Centers reporting genetic polymor-
Neonatal screening
Age (years)
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
phisms in the CFTR gene, that were not included in this report because they may not be used as diagnostic
No
Yes
Total
0.41 (1.09)
3.50 (0.63-9.39)
0.14 (0.09-0.30)
Ŋ
0-10.18
Ŋ
1,697
480
2,177
4
1
5
.JOJNVN.BYJNVN
criteria without the presence of other mutations.
Table 15
Status of patients regarding genetic testing for CF.
Genetic testing
Total of patients
Patients without information
Age at diagnosis ( years )
Box-plot graph showing distribution of age at the diagnosis in
patients diagnosed with or without neonatal screening test.
n (%)
1,212 (55.5%)
%'%'
260 (26.8%)
Yes
%'0UIFST
124 (12.8%)
%'/POJEFOUJţFE
285 (29.4%)
Quantity of mutations identified
per patient
2,182 (100%)
211 (21.8%)
One
338 (34.8%)
Two or more
421 (43.4%)
Total of patients with genetic
testing reported
970 (100%)
0UIFST/POJEFOUJţFE
53 (5.5%)
Non identified / Non identified
211 (21.8%)
Total of pacientes with genetic
testing reported
970 (100%)
n (%)
%'%'
260 (26.8%)
%'0UIFSTPSOPOJEFOUJţFE
409 (42.2%)
0UIFST0UIFSTPSOPO
JEFOUJţFE
0UIFST0UIFST
n (%)
None
Genotype - description
No
Genotype - description
No
Total of patients
Figure 11
n (%)
90 (9.3%)
/POJEFOUJţFE
211 (21.8%)
Total of pacientes with genetic
testing reported
970 (100%)
Yes
Neonatal screening
20
21
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Figure 12
Table 16
Distribution of patients by genotype (n=970)
Frequency of identified mutations (970 patients, 1,940 alleles).
Not
Identified
22%
DF508/DF508
27%
Others/Otherss
09%
DF508/Others
42%
Figure 13
Frequency of identified mutations (970 patients, 1,940 alleles).
34.50%
DF508
4.28%
G542X
R1162X
0.77%
R334W
0.72%
G85E
0.67%
3120+1G>A
0.62%
W1282X
0.62%
N1303K
0.57%
G551D
0.26%
1078deIT
0.21%
3849+10kbC>T
0.21%
Heterozigose M470V
0.21%
R553X
0.21%
Homozigose M470V
0.15%
0%
22
Patient Registry
5%
10%
15%
20%
25%
30%
35%
40%
Mutations
DF508
G542X
R1162X
R334W
G85E
3120+1G>A
W1282X
N1303K
G551D
R553X
)FUFSP[JHPVT.7
3849+10kbC>T
EFM5
)PNP[JHPVT.7
S549R
(5
2183AA>G
1812-1G>A
("
Y1092X
W543X
W1089X
32
31
R10665
29
S4X
P205S
.,
I331N
*
D3956
%)
$(5
c1929delC
$("
55
3659delc
UU
("
1898+1G>A
1656AA
Blank cells
Total of alleles (970 patients)
n
669
83
15
14
13
12
12
11
5
4
4
4
4
3
2
2
2
2
2
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
2
1
1
943
1,940
%
34.5%
4.28%
0.62%
0.62%
0.26%
0.21%
0.21%
0.21%
0.21%
0.15%
0.10%
0.10%
0.10%
0.10%
0.10%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.05%
0.10%
0.05%
0.05%
48.61%
100%
23
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
ANTHROPOMETRIC DATA
Figure 14
Anthropometric data were obtained at the day of pulmonary function testing or in the last visit of the year
Box-plot graph showing distribution of NCHS percentile for weight
by age group of the population aging up to 20 years old.
(when pulmonary function testing data was not available).
Percentile distribution of anthropometric data and Z-score of weight and height was calculated by using CDC
HSPXUIDIBSUTBTSFGFSFODFBWBJMBCMFJOIUUQXXXDEDHPWHSPXUIDIBSUT
100
Table 17
Anthropometric data
WEIGHT (kg)
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
HEIGHT (cm)
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
Measure
NCHS Percentile
Z Score
34.19 (30.38)
-0.69 (1.29)
30.00 (18.80-48.00)
-0.61 (-1.54- 0.18)
4.30-98.4
0-100
1,506
1,224
1,224
Measure
NCHS Percentile
Z Score
133.05 (29.89)
35.43 (29.68)
-0.56 (1.24)
136.00 (111.00-158.00)
29.00 (8.00-56.00)
-0.54 (-1.38-0.16)
54.5-190.00
0-100
-3.90-3.98
1,499
1,199
1,199
Measure
NCHS Percentile
0-100
1,499
1,000
Weight percentile
80
60
40
20
0
<= 5
BMI (kg/m2)
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
24
> 5 - 10
> 10 - 15
> 15 - 20
Age range in years
25
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 15
Figure 16
Box-plot graph showing distribution of Z-score values for weight by
age group of the population aged up to 20 years old.
Box-plot graph showing distribution of NCHS percentile for
height by age group of the population aging up to 20 years old.
100
4
80
Height percentile
Z score - Weight
2
0
60
40
-2
20
0
-4
<=5
> 5 - 10
> 10 - 15
Age range in years
26
> 15 - 20
<= 5
> 5 - 10
> 10 - 15
> 15 - 20
Age range in years
27
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 17
Figure 18
Box-plot graph showing distribution of Z-score values for height
by age group of the population aged up to 20 years old.
Box-plot graph showing distribution of NCHS percentile for
weight by age group of infants and preschool age population.
100
4
3
80
Weight percentile
Z score - Height
2
1
0
-1
60
40
-2
20
-3
0
-4
<= 5
> 5 - 10
> 10 - 15
Age range in years
28
> 15 - 20
<= 1
>1-2
>2-3
>3-4
>4-5
Age range in years
29
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 19
Figura 20
Box-plot graph showing distribution of Z-score values for weight by
age group of infants and preschool age population.
Box-plot graph showing distribution of NCHS percentile for height
by age group of infants and preschool age population.
4
100
80
Height Percentile
Z score - Weight
2
0
60
40
-2
20
0
-4
<= 1
>1-2
>2-3
Age range in years
30
>3-4
>4-5
<= 1
>1-2
>2-3
>3-4
>4-5
Age range in years
31
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 21
Figure 22
Box-plot graph showing distribution of Z-score values for height by
age group of infants and preschool age population.
Box-plot graph showing distribution of Z-score values for BMI (Body
Mass Index) by age group of the adult population.
40
4
35
30
BMI (kg/m2)
Z score - Height
2
0
25
20
-2
15
-4
10
<= 1
>1-2
>2-3
Age range in years
32
>3-4
>4-5
20 - 25
> 25 - 30
> 30 - 35
> 35
Age range in years
33
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
PULMONARY FUNCTION DATA
Figure 23
5IFWBMVFTPG'7$'&7BOE'7$'&7SFDPSEFECZUIFQBSUJDJQBOUTXFSFUIPTFPCUBJOFEJOUIFCFTUMVOHGVOD-
Box-plot graph showing distribution of percent of predicted values of
forced vital capacity by age group.
tion test of the reported year. The predicted values of pulmonary function were obtained from the publication
of Stanojevic S et al: Spirometry Centile Charts for Young Caucasian Children: The Asthma UK Collaborative
*OJUJBUJWF"NFSJDBO+PVSOBMPG3FTQJSBUPSZBOE$SJUJDBM$BSF.FEJDJOF
150,00
Table 18
Pulmonary function data.
No
Yes
Total of patients
n (%)
801 (51.3%)
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
1,562 (100%)
.JOJNVN.BYJNVN
Total of patients
FVC (liters)
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
125,00
FEV1 (liters)
1.81 (0.84)
1.63 (1.20-2.23)
761
Z score - FEV1
2.36 (1.05)
2.15 (1.61-2.95)
0.28-5.83
761
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
-2.10 (2.15)
752
FVC % Predicted
Spirometry performed
100,00
75,00
50,00
FEV1/FVC
Z score - FVC
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
-1.51 (2.01)
-6.92; 4.46
752
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
25,00
0.33-1.00
761
>5 - 10
> 10 - 15
> 15 - 20
> 20 - 25
> 25 - 30
> 30 - 35
> 35
Age range in years
34
35
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 24
Figure 25
Box-plot graph showing distribution of percent of predicted values
of forced expiratory volume in the 1st second by age group.
Box-plot graph showing distribution of Z-score values of forced
vital capacity by age group and gender.
200,00
Gender
Male
Female
4,00
150,00
Z Escore - FVC
FEV1 % Predicted
2,00
100,00
50,00
,00
-2,00
-4,00
-6,00
,00
>5 - 10
> 10 - 15
> 15 - 20
> 20 - 25
> 25 - 30
Age range in years
> 30 - 35
> 35
-8,00
>5 - 10
> 10 - 15
> 15 - 20
> 20 - 25
> 25 - 30
> 30 - 35
> 35
Age range in years
36
37
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 26
MICROBIOLOGY DATA
Box-plot graph showing distribution of Z-score values of forced
expiratory volume in the 1st second by age group and gender.
.JDSPCJPMPHZEBUBEFTDSJCFQPTJUJWFSFTVMUTGPSUIFSFTQJSBUPSZQBUIPHFOBUMFBTUPODFJOUIFHJWFOGPMMPXVQ
ZFBSTJODFUIFSFJTOPUBTUBOEBSEJ[BUJPOPGNJDSPCJPMPHZQSPDFTTJOHPGSFTQJSBUPSZUSBDUTBNQMFTPGDZTUJDţbrosis patients in our country, these data have to be cautiously interpreted.
Gender
Male
Female
5,00
Table 19
Description of microorganisms identified.
Microorganisms identified
n
%
Methicillin-sensitive Staphylococcus aureus
901
Pseudomonas aeruginosa
45.3%
Non-mucoid Pseudomonas aeruginosa
483
30.9%
.VDPJE1TFVEPNPOBTBFSVHJOPTB
338
21.6%
#VSLIPMEFSJBDFQBDJBDPNQMFY
163
10.4%
.FUIJDJMMJOSFTJTUBOU4UBQIZMPDPDDVTBVSFVT
122
)BFNPQIJMVTJOŤVFO[BF
134
8.6%
Stenotrophomonas maltophilia
89
Klebsiella pneumoniae
68
4.4%
Serratia sp.
45
2.9%
Achromobacter sp.
33
2.1%
Candida sp.
56
3.6%
Aspergillus fumigatus
29
1.9%
Escherichia coli
26
Other Pseudomonas
1.1%
/POUVCFSDVMPVT.ZDPCBDUFSJB
4
0.3%
.ZDPCBDUFSJVNUVCFSDVMPTJT
4
02%
1,562
100%
Z Escore - FEV1
2,50
,00
-2,50
-5,00
-7,50
>5 - 10
> 10 - 15
> 15 - 20
> 20 - 25
> 25 - 30
Age range in years
> 30 - 35
> 35
Total of patients
38
39
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 27
Table 20
Microorganisms identified
Micrroganisms identified - distribution by age group
Methicilin sensitive S. aureus
Non-mucoid Pseudomonas aeruginosa
Mucoid Pseudomonas aeruginosa
Burkholderia cepacia complex
10.40%
Haemophilus influenzae
8.60%
Methicilin resistant S. aureus
7.80%
Stenotrophomonas maltophilia
5.70%
Klebsiella pneumoniae
4.40%
Candida sp
3.60%
Serratia sp.
2.90%
Achromobacter sp.
2.10%
Aspergillus fumigatus
1.90%
Escherichia coli
1.70%
other Pseudomonas
1.10%
Non-tuberculous Mycobacterium 0.30%
Mycobacterium tuberculosis 0.20%
0%
10%
20%
Microrganismos identificados
57.70%
30.90%
21.60%
MethicillinBurkholderia
resistant
cepacia
Staphylococcus
complex
aureus
Age group
Methicillinsensitive
Staphylococcus
aureus
Pseudomonas
aeruginosa
Up to 5 years
61.0%
39.3%
8.5%
> 5 to 10
65.3%
38.3%
>10 to 15
59.9%
>15 to 20
Haemophilus
influenzae
Stenotrophomonas
maltophilia
nº
12.6%
5.3%
318
8.5%
9.9%
426
48.2%
12.9%
9.0%
54.5%
54.0%
10.6%
8.1%
6.1%
4.5%
198
>20 to 25
49.5%
56.8%
9.5%
3.2%
95
>25 to 30
14.8%
9.3%
54
>30 to 35
33.3%
54.5%
6.1%
15.2%
0%
12.1%
33
>35 years
36.4%
56.4%
5.5%
3.6%
3.6%
1.8%
55
Figure 28
30%
40%
50%
60%
Prevalence of identified pathogens by age group.
70
60
50
40
30
20
10
0
Up to 5 years
old
>5 to 10
>10 to 15
>15 to 20
Methicillin sensitive S. aureus
Burkholderia cepacia complex
Haemophilus influenzae
40
>20 to 25
>25 to 30
>30 to 35
>35 years
Pseudomonas aeruginosa
Methicillin resistant S. aureus
Stenotrophomonas maltophilia
41
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
CLINICAL TREATMENT DATA
Figure 29
Distribution of the number of consultations per patient in 2011.
Patient Registry
Table 21
Table 22
Deaths
Shwachman-Kulczycki score.
Death
Total score
n (%)
No
1,550 (99.2%)
Yes
12 (0.8%)
.FEJBOQQ
.JOJNVN.BYJNVN
Causes of death*
400
300
.FBOTUBOEBSEEFWJBUJPO
3FTQJSBUPSZGBJMVSFŊ
DISPOJDBDVUF
10
4FQUJDTIPDLTFQTJT
3
Acute pulmonar
FYBDFSCBUJPO
n (%)
15-100
CLASSIFICATION
Severe (≤ 40)
64 (4.9%)
.PEFSBUFB
116 (8.8%)
1
.FEJBOB
242 (18.4%)
Acynetobacter sepsis
1
(PPEB
424 (32.3%)
Dehidration
1
&YDFMMFOU
Total of patients
1,562 (100%)
Total of patients
1,313 (100%)
Age at death (years)
Count
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
200
.JOJNVN.BYJNVN
14.00 (6.64)
Figure 30
Distribution of patients by Shwachman-Kulczycki score.
100
Good
32%
0
1
2
3
4
5
6
7
8
9
10
Number of visits
11
12
13
14
15
Excellent
36%
Moderate
9%
Severe
5%
42
Median
18%
43
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Table 23
Figure 32
Shwachman-Kulczycki score: Total score by age group.
Box-plot graph showing distribution of Shwachman-Kulczycki score
by age group.
Age group
Up to 5 years
> 5 to 10
>10 to 15
>15 to 20
>20 years
Total
Severe (≤ 40)
1 (0.4%)
15 (9.0%)
32 (15.4%)
.PEFSBUFB
6 (2.1%)
18 (5.1%)
34 (11.9%)
19 (11.4%)
38 (18.3%)
115 (8.9%)
.FEJBOB
30 (10.6%)
54 (15.4%)
44 (26.3%)
50 (24.0%)
240 (18.5%)
(PPEB
89 (31.3%)
114 (32.5%)
109 (38.1%)
49 (29.3%)
419 (32.3%)
&YDFMMFOU
158 (55.6%)
159 (45.3%)
40 (24.0%)
30 (14.4%)
461 (35.6%)
Total of patients
284 (100%)
351 (100%)
286 (100%)
167 (100%)
208 (100%)
1,296* (100%)
Figure 31
Bar graph showing distribution of Shwachman-Kulczycki score by
age group.
Total Score
60%
Severe
Moderate
Median
Good
Excellent
50%
100
80
Shwachman-Kulczycki Score
Total score
60
40
20
40%
0
30%
<= 5
20%
> 5 - 10
> 10 - 15
> 15 - 20
> 20
Age range in years
10%
0%
Up to 5
years old
(n=284)
>5 to 10
(n=351)
>10 to 15
(n=286)
>15 to 20
(n=167)
>20 years old
(n=208)
Total
(n=1296)
Age group
44
45
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Figure 33
Table 24
Table 25
Graph showing 95% confidence interval values of ShwachmanKulczycki score by age group.
Complications in the given
year-2011
Transplantation
Transplantation
Complications in the given year (2011)
95% CI Shwachman-Kulczycki Score
90
85
80
75
n (%)
Asthma
231 (14.8%)
(BTUSPFTPQIBHFBMSFŤVY
Evidences of hepatic disease
Nasal polyposis
)FNPQUZTJT
52 (3.3%)
Diabetes
53 (3.4%)
0TUFPQFOJB0TUFPQPSPTJT
Chronic atelectasis
36 (2.3%)
Allergic broncopulmonary aspergillosis
6 (0.4%)
Distal intestinal obstruction
70
65
60
<= 5
> 5 - 10
> 10 - 15
Age range in years
> 15 - 20
> 20
Pulmonary transplantation
Corpse
Live donor
Liver transplantation
Total of patients
Oxygen therapy
4 (0.3%)
1,562 (100%)
n (%)
No
1,509 (96.6%)
Yes
53 (3.4%)
Continuous
28 (1.8%)
Nocturnal
25 (1.6%)
Pulmonary hypertension
10 (0.6%)
Cirrosis with portal hypertension
9 (0.6%)
1OFVNPUIPSBY
6 (0.4%)
Table 27
Pancreatitis
6 (0.4%)
Insulin usage
)FNBUFNFTJT
4 (0.3%)
Insulin usage
Total of patients
1,562 (100%)
n (%)
No
1,493 (95.6%)
Yes
69 (4.4%)
Total of patients
46
10
Oxygen therapy
10 (0.6%)
1,562 (100%)
10 (0.6%)
Table 26
Colelythiasis
Total of patients
n (%)
1,562 (100%)
47
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Table 28
Table 29
Inhaled medications
Oral medications
Bronchodilators
n (%)
Mucolitics
Short acting Beta 2 agonist
499 (31.9%)
Alfa dornase
Long acting Beta 2 agonist
281 (18.5%)
N Acetylcystein
Anticholinergic
48 (3.1%)
Saline solutions
Antibiotics
n (%)
Inhaled Tobramycin solution 300mg 528 (33.8%)
0.9% saline solution
)ZQFSUPOJDTBMJOF
n (%)
383 (24.5%)
Colomycin
)ZQFSUPOJDTBMJOF
63 (4.0%)
Gentamycin
32 (2.0%)
)ZQFSUPOJDTBMJOF
291 (18.6%)
Others
20 (1.3%)
Total of patients
Injectable Tobramicyn solution
Vancomycin
8 (0.5%)
Amikacin
8 (0.5%)
n (%)
n (%)
n (%)
Pancreatic enzymes
Azithromycin
540 (34.6%)
-FTTUIBO6LHEJB
6STPEFPYZDIPMJDBDJE
349 (22.3%)
6LHEJB
644 (41.2%)
Proton pump inhibitors
320 (20.5%)
.PSFUIBO6LHEJB
231 (14.8%)
)CMPDLFST
129 (8.3%)
Corticosteroid
88 (5.6%)
Ibuprofen (for lung disease)
Ibuprofen or other NSAI* (for
arthropathy)
5 (0.3%)
44 (2.8%)
Unknown
Dietary supplements
Oral
Gastrostomy
29 (1.9%)
Gastric tubes
6 (0.4%)
Unknown
Total of patients
1,562 (100%)
Figure 34
Oral medications
Pancreatic Enzymes
82.7%
Dietary Supplements
63.20%
Azithromycin
34.60%
Ursodeoxycholic Acid
22.30%
Proton Pump Inhibitors
20.50%
H2 Blockers
8.30%
Corticosteroid
5.60%
Ibuprofen (Lung Disease)
0.70%
Ibuprofen or other NSAI (for)
0.30%
0%
48
1,562 (100%)
10%
20% 30% 40% 50% 60% 70% 80% 90% 100%
49
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Table 30
Table 32
P. aeruginosa eradication treatment
Intravenous antibiotics: days of treatment by age group.
P. aeruginosa eradication treatment
Age group
n (%)
Up to 5
years
> 5 to 10
>10 to 15
>15 to 20
>20 years
Total
.FBOTUBOEBSEEFWJBUJPO
20.9 (22.2)
21.3 (16.8)
24.1 (29.6)
.FEJBOQQ
14 (14-21)
14 (14-21)
14 (14-23)
14.5 (14-22.5)
15 (14-30)
14 (14-21)
3-152
3-154
11-122
9-160
71
64
89
50
58
338
Yes
316 (20.2%)
Days
No
664 (42.5%)
Unknown
Total of patients
.JOJNVN.BYJNVN
1,562 (100%)
Total of patients
Table 33
Intravenous antibiotics – drugs utilized.
Table 31
Intravenous treatments - admissions
Drugs utilized
Treatment
No admission
)PNFDBSF
)PTQJUBMBENJTTJPO
)PTQJUBMBOEIPNFDBSFBENJTTJPO
Total of patients
n (%)
56 (3.6%)
298 (19.1%)
14 (0.9%)
Days
.FBOTUBOEBSEEFWJBUJPO
.FEJBOQQ
.JOJNVN.BYJNVN
Total of patients
Ciclos
.FEJBOQQ
50
14 (14-21)
339
1,562 (100%)
Implanted cathether
.FBOTUBOEBSEEFWJBUJPO
24.05 (29.60)
1.51 (1.00)
1 (1-2)
.JOJNVN.BYJNVN
1-8
Total of patients
332
n (%)
No
1,533 (98.1%)
Yes
29 (1.9%)
Total of patients
1,562 (100%)
n
(%)
Ceftazidime
228
14.6%
Amikacin
211
13.5%
0YBDJMMJO
119
$JQSPŤPYBDJO
88
5.6%
Tobramycin
62
4.0%
*NJQFOFNPS.FSPQFOFN
60
3.8%
Vancomycin
55
3.5%
5SJNFUIPQSJNTVMGBNFUIPYB[PMF
54
3.5%
Cefepime
35
2.2%
1JQFSBDJMJOF5B[PCBDUBN
28
1.8%
Gentamicin
20
1.3%
Others
1.1%
5JDBSDJMMJO1JQFSBDJMMJO
13
0.8%
Linezolid
0.4%
$FGVSPYJNF
5
0.3%
Colomycin
5
0.3%
Aztreonam
4
0.3%
Chloramphenicol
2
0.1%
Total of patients
1,562
100%
51
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Acknowledgements:
Figure 35
Intravenous antibiotics – drugs utilized.
This work would not be possible without the support of some Pharmaceutical Companies listed below, who
14.6%
Ceftazidime
access to data nor of availability of marketing actions.
13.5%
Amikacin
7.6%
Oxacilin
Ŕ Roche Brasil
5.6%
Ciprofloxacin
Ŕ Novartis Brasil
4.0%
Tobramycin
8F UIBOL BMM IFBMUI QSPGFTTJPOBMT JOWPMWFE JO UIF DBSF PG DZTUJD ţCSPTJT QBUJFOUT CZ UIFJS DPPQFSBUJPO JO UIJT
3.8%
Imipenema or Meropenem
Vancomicin
Sulpha_Trimetoprim
3.5%
JOJUJBUJWFXIJDIXFCFMJFWFXJMMSFTVMUJOTJHOJţDBOUBEWBODFTJOUIFDBSFPGDZTUJDţCSPTJTQBUJFOUTJOPVSDPVOUSZ
3.5%
8FBMTPUIBOL1SPG.BSDFMP,O²SJDI;VŢPGVMMQSPGFTTPSPG&TDPMB1PMJU¥DOJDBGSPN6OJWFSTJUZPG4ŸP1BVMPCZ
2.2%
Cefepime
sponsored the initiative with enthusiasm and ethically, even without any perspective neither of a privileged
the support and incentive to the team of Laboratório de Sistemas Integráveis (LSI-Poli-USP).
1.8%
Piperacililn_Tazobactam
1.3%
Gentamicin
1.1%
Others
0.8%
Ticarcillin_Piperacillin
Linezolid
0.4%
Colomycin
0.3%
Cefuroxime
0.3%
Aztreonam
0.3%
Chloranphenicol 0.1%
0%
2%
4%
5%
7%
9%
11%
12%
14%
16%
Table 34
Data of adult population.
Gender
Male
Female
Total
26 (18.1%)
-
26 (8.9%)
-
8 (5.4%)
Common law marriage
26 (18.1%)
46 (31.1%)
Employment
48 (32.4%)
105 (36.0%)
144
148
292
"[PPTQFSNJBPS)ZQPTQFSNJB
Pregnancy
Total of patients older than 18 years with follow-up data
52
53
Brazilian
Brazilian
Cystic Fibrosis
Cystic Fibrosis
2011 Annual Report
2011 Annual Report
Patient Registry
Patient Registry
Centers that celebrated a confidentiality agreement with the GBEFC to
participate in the REBRAFC (updated in October 2012)
Name
City
Estate
Responsible
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Katharina Vidal de Negreiros
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Salvador
BA
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Salvador
BA
Edna Lúcia Santos de Souza
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Fortaleza
CE
Cláudia de Castro e Silva
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Brasília
DF
Luciana de Freitas Velloso
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Vitória
ES
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Vitória
ES
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Goiânia GO
GO
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Goiânia
GO
Lusmaia Damaceno
Camargo Costa
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Belo
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Belo
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Alberto Andrade Vergara
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City
Estate
Responsible
Instituto Fernandes Figueira
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Daniela de Souza Paiva
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Natal
RN
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Porto Alegre
RS
Gilberto Bueno Fischer
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Porto Alegre
RS
Fernando Antônio de Abreu
e Silva
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Porto Alegre
RS
Paulo de Tarso Roth Dalcin
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Porto Alegre
RS
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Florianópolis
SC
Norberto Ludwig Neto
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Florianópolis
SC
Concetta Espósito
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Blumenau
SC
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SC
Tiago Neves Veras e Rafaela
de Campos Benvenutti da
Costa
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Paulo
São Paulo
SP
Neiva Damaceno
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São Paulo
SP
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São Paulo
SP
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Ribeirão Preto
SP
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Torres
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Campinas
SP
Antônio Fernando Ribeiro
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Botucatu
SP
Giesela Fleischer Ferrari
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Rio Preto
SP
Kátia Izabel de Oliveira e
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Campinas
SP
Ilma Aparecida Paschoal e
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)PTQJUBMEBT$M©OJDBTEB641BEVMUPT
São Paulo
SP
Rafael Stelmach
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do Norte
Uberlândia
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Campo
Grande
.4
Valéria Cristina de Ruchkys
e Lílian Cristina Ferreira
Andries
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Cuiabá
.5
Arlan de Azevedo Ferreira
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Belém
PA
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PB
Constantino Giovanni Braga
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Campina
Grande
PB
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Recife
PE
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Teresina
PI
Luiz Edson dos Santos Costa
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Curitiba
PR
Paulo Kussek
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Curitiba
PR
Carlos Antônio Riedi
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Cascavel
PR
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São Paulo
SP
Luiz Vicente R. F. da Silva
Filho
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Curitiba
PR
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Consultório Fabíola Adde
São Paulo
SP
Fabíola Villac Adde
Universidade Federal de Campina Grande
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Figueira
54
Name
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do Rio Preto
55
GRUPO
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FIBROSE CÍSTICA
www.gbefc.org.br