Egyptian National Registry on RBD Condition of Other EMRO States

21/05/06 ISTH, RBD, Vancouver

Egyptian National Registry on RBD

Condition of Other EMRO States

Magdy El Ekiaby, MDShabrawishi HTC

21/05/06 ISTH, RBD, Vancouver

21/05/06 ISTH, RBD, Vancouver

Edwin Smith Papyrus

21/05/06 ISTH, RBD, Vancouver

Modern Egypt

21/05/06 ISTH, RBD, Vancouver

National Registry of Hereditary Bleeding Disorders (HBD)

• First specialized unit for diagnosis of HBD in CHL, 1968 (referral lab)

• Establishment of ESH, 1971 with mutual registry of HBD since then

• University hospital hematology labs confirm their diagnosis in CHL which allows for central registration

• All diagnosed patients are referred to ESH for registration and will have an ID

21/05/06 ISTH, RBD, Vancouver

21/05/06 ISTH, RBD, Vancouver

ESH

CHL University HTC

9 university hosp 9 out of 14 centers

21/05/06 ISTH, RBD, Vancouver

Registry of HBD, 2005 (5064 patients)

3203756

375

730

A

B

vWD

others

Courtesy of Dr. Nadia Moharram, CHL, 2005

21/05/06 ISTH, RBD, Vancouver

National Registry of RBD, 2005 (403 patients)

93

0

123483

59

77 0FI

FII

FV

FVII

FV & VIII

FX

FXI&XII

FXIII

10 cases of FXIII deficiency diagnosed in the centers & not included in National Registry

Courtesy of Dr. Nadia Moharram, CHL, 2005

21/05/06 ISTH, RBD, Vancouver

Characteristics of National Registry

• Diagnosis is dependent on phenotyping

• No genotyping• Cumulative registry since 1971,

actual number of diagnosed cases, who are still living is not defined

• The registry committee is working on its verification

21/05/06 ISTH, RBD, Vancouver

EMRO States Registry

21/05/06 ISTH, RBD, Vancouver

Communication

• E mails using the address book of Dr. Assad Haffar for EMRO and Africa

21/05/06 ISTH, RBD, Vancouver

National Registry of Rare Hereditary Bleeding Disorders

• Country:-• Institution:-• Name of contact person:-• Telephone:- E Mail address:-• Questions:-

– Is there a national registry for HBD?– Does the registry include RHBD?– Number of Diagnosed cases of:-

• FI, FII, FV, FVII, FV& VIII, FX, FXI&XII, FXIII

21/05/06 ISTH, RBD, Vancouver

Response

• Response from:-– Morocco:- no registry– Algeria:- no registry– KSA:- no registry– Kenya:- no registry– Botswana:- one case FX– Lebanon:- to be verified– Qatar :- center registry– Pakistan:- chapter registry– Jordan:- hospital based registry– South Africa:- national registry– Lesotho:- no registry

21/05/06 ISTH, RBD, Vancouver

QatarPakistan, Lahore

Pakistan, Islamabad/

Rawalpindi

Jordan (Al

Bashir H.

South Africa

FI04209

FII00200

FV0116141

FVII276417

FV&VIII00004

FX056410

FXI&XII510329

FXIII34064

21/05/06 ISTH, RBD, Vancouver

DiagnosisEgyptEMRO& Africa

FI9315

FII02

FV12359

FVII4836

FV&VIII34

FX5926

FXI&XII7738

FXIII10*17

*from centers & not in registry

21/05/06 ISTH, RBD, Vancouver

Acknowledgement

• M El Khorassani, Morocco• L Lamhene & S El Fegoun, Algeria• H B Jibril, Botswana• G Zaher, KSA• M Walter, Kenya• T Zafar, Rawalpindi, Pakistan• S Sohail, Lahore, Pakistan• A Khoriaty, Lebanon• M Abulaban, Qatar• M Alfallah, Jordan• Anne-Louise Cruikshank, South Africa• Grace Phiri, Lesotho

21/05/06 ISTH, RBD, Vancouver

Delighted to have you all in Cairo 2010,

&

Thank You