National Databases listed in WFH: Paula Bolton-Maggs
(see
attached file) file.ppt
Established in 1968 when department of health designated 36
hemophilia centers
First report 1969
Web based system
HCIS: haemophilia clinical information system at each local
center
DOH, HPA (health protection agency), Commissioners etc
DOH pays for most of the costs
Requires informed consent
Database under Data protection act
Have a leaflet that they prepared to distribute to patients
HCIS collects information about bleeding and treatment, while
the national database does not collect this information
Swiss Registry: Phillippe DeMoerloose (see
attached file) file.ppt
Limited registry
Currently no registry in Germany, Austria vWD < 10%
Under control of the Swiss Federal Agency of Data Protection
Run by two people
Requires informed consent - can be anonymous if required
More than a registry, as it has information about SSH, forum,
etc
North American Registry US: Amy D. Shapiro (see
attached file) file.ppt
Forthcoming registry will have flexible entry fields to cover
as many disorders as possible
Two levels of data desired:
1. Data not requiring individual consent,
2. More detailed data requiring individual consent
US has database platform for data collection at all hemophilia
treatment centers
Database presently being converted into a web based system
NHF has expressed interest in collaborating to develop and house
a resource center for RBD
Merging efforts of US RBD database and RBDD to conserve funds
that are raised
UK National database and patient consent issue:
Paula Bolton-Maggs (see
attached file) file.ppt
Established in 1968 when department of health designated 36
hemophilia centers
First report 1969
Web based system
HCIS: haemophilia clinical information system at each local
center
DOH, HPA (health protection agency), Commissioners etc
DOH pays for most of the costs
Requires informed consent
Database under Data protection act
Have a leaflet that they prepared to distribute to patients
HCIS collects information about bleeding and treatment, while
the national database does not collect this information
French: Jean Donadieu (see
attached file) file.ppt
Started in Oct 1994, limited to hemophilia, beginning of 2003
included vWD and RBD
Coordinating center, national agency for disease control (like
US CDC)
Have definitions of RBD
I < 0.1 g/l
II, V, VII, X, XI, XIII < 10%
137 patients with RBD in database
Collects information on demographics, genetics, therapy, main
events (hemorrhagia, inhibitors, viral infection, surgeries)
Proposal to include Glanzmann and thrombophilia
May coordinate with Belgium
Question: Where do the patients with combined FV & VIII
fit in?- their levels are higher than the definition required
for inclusion in this database (< 10%)
Egyptian National Registry and condition of
other EMBRO countries: Magdy El Ekiaby (see
attached file) file.ppt
Database on paper not an electronic version
National registry started in 1968
Governmental agency
Each patient referred to central agency and given an ID card
with unique patient number
5064 patients included, 3203 are hemophilia
No genotying data
Diagnosis dependent on phenotyping
Actual number of diagnosed cases that are still living is not
defined
Forming a registry committee to work on verification of data
EMBRO sates: northern Africa and east to Afghanistan included
South Africa
Iranian registry: Manijheh Lak and Mehran Karimi
(see
attached file) file.ppt
Frequency of 3-5%, increases 10-20 times where consanguineous
marriages are common
7000 patients with congenital bleeding disorders diagnosed
Have a national database
Ministry of health designed software to use throughout Iran,
which is more detailed than previously collected
RBDs in Iran - 970 patients, FVII most common (n=300), followed
by combined FV & VIII (n=150), FXIII (n=123) deficiency
Clinical presentation of RBDs in Iran, mentioned CNS bleeding
FVII severe
Treatment - most confined to cryoprecipitate and FFP, VII -
have rFVIIa, FII have PCCs
Molecular analysis performed in conjunction with Italy
Plan to establish computerized registry in all hemophilia centers
in Iran, improve lab diagnosis and molecular diagnosis
Indian Registry: Alok Srivastava (see
attached file) file.ppt
Started hemophilia registry in 1997 - 8-9,000 people are registered
but it does not include RBD for which there is no real registry
in India
Has his centers data
1.1 billion people in country
12-42% consanguinity in Southern India (only defined as first
and second degree)
10% of all bleeding disorders seen are rare, # of Glanzmann
is ranked third after hemophilia, vWD, FVII
30-60% of RBD have severe disease
Standardized format to collect symptoms
Joint bleeding more common in FVII def
FXIII & Fibrinogen - umbilical cord bleeding
Comparison of Iranian data to India - FV ~ same, Fibrinogen
different, FV & VIII similar to Israel/Iran/Bombay
FVII ~ same as Italy and Iran - Flora pointed out different
levels used in each registry or the report
FX ~ same
FXI not too many
FXIII lower incidence of joint bleeds compared to Iranian data
Security of RBDD: Marzia Menegatti (see
attached file) file.pdf
Uses anonymous data
RBDD located at -hospital-
Reviewed security of database
File sending from an external site
Primary data entry: https transmission - authentication of institution
(reserved area) - all data of the patient are in one or more
files, but in a single collected sending - file sending includes
an escape clause
Secondary: Direct inserting of data from external site - Authentication
of Institution via https specific client certification - a password
can be added to the specific client certification - client enters
the application RBDD with a specific authorization (display/insert)
only for its own data - inserted data are checked by an operator
before use
