ESID LISBOA 2018

PORTUGAL

ESID LISBOA 2018

 0

20 000

40 000

60 000

80 000

100 000

120 000

140 000

NEWBORNS

10.427.301 INHABITANTS

ESID LISBOA 2018

N=997

PIDs IN PORTUGAL

REGISTRY

• SINCE 1998

• NATIONAL REGISTRY- REPORID

• ESID REGISTRY

HH

H

HHH

140; 14%

534; 54%

32; 3%

61; 6%

148; 15%

SCID

CID

COMBINED SYNDROMIC

ANTIBODIES

IMMUNEDYSREGULATION

PHAGOCYTE

INNATE

AUTO-INFLAMMATORY

COMPLEMENT

MEDICAL STAFF

- FELLOWSHIPS IN WELL-RECOGNIZED PID CENTRES

- NEWCASTLE

- PARIS

- LONDON

- BARCELONA

- SÃO PAULO

- PARTICIPATION IN INTERNATIONAL MULTICENTRIC

PROJECTS

- EU CALL ON PUBLIC HEALTH (E. GAMBINERI)

- European Network for Immune Deficiencies (S. NEJENTSEV)

ESID LISBOA 2018

PIDs IN PORTUGAL

HH

H

HHH

ESID LISBOA 2018

PIDs IN PORTUGAL

Hemophagocytic lymphohistiocytosis in two patients with underlying IFN-γ receptor-deficiency

Bianca Tesi

1,2,*MD, Elena Sieni

3,*MD, Conceição Neves

4, Francesca Romano

5,Valentina

Cetica3PhD, Ana Isabel Cordeiro

4MD, Samuel Chiang

6MSc, Heinrich Schlums

6MSc, Luisa

Galli7MD, Stefano Avenali

8MD, Annalisa Tondo

3MD, Clementina Canessa

5MD, Jan-Inge

Henter1MD,PhD, Magnus Nordenskjöld

2MD,PhD, Amy P. Hsu

9, Steven M. Holland

9MD,

Joao F. Neves4,*

MD, Chiara Azzari5,*

MD,PhD, Yenan T. Bryceson6,*

PhD

1Childhood Cancer Research Unit, Department of Women's and Children's Health,

Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden; 2Clinical Genetics Unit, Department of Molecular Medicine and Surgery, and Center for

Molecular Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm,

Sweden; 3Department of Pediatric Hematology Oncology, Anna Meyer Children's University Hospital,

Florence, Italy; 4Primary Immunodeficiencies Unit, Hospital Dona Estefania, Pediatric University Hospital,

and CEDOC, Chronic Diseases Research Center, NOVA Medical School, Lisboa, Portugal; 5Department of Pediatric Immunology, University of Florence and Anna Meyer Children’s

University Hospital, Jeffrey Modell Center for Primary Immunodeficiencies, Florence, Italy; 6Centre for Infectious Medicine, Department of Medicine, Karolinska Institutet, Karolinska

University Hospital Huddinge, Stockholm, Sweden; 7Department of Pediatric Infectious Diseases, Anna Meyer Children’s University Hospital,

Florence, Italy; 8Department of Pediatric Intensive Care, Anna Meyer Children's University Hospital,

Florence, Italy; 9National Institute of Allergy and Infectious Diseases, National Institutes of Health,

Bethesda, MD, USA.

*Equal contribution of first authors and last authors, respectively.

Address correspondence to:

Yenan.Bryceson@ki.se

• IMUNOGLOBULIN REPLACEMENT THERAPY

• IVIG- 79 PATIENTS

• SCIG- 73 PATIENTS

• 36% children (N=56)

• HEMATOPOIETIC STEM CELL TRANSPLANTATION

• First HSCT in Portugal: 1994 (LAD1)

• 39 patients underwent HSCT for PID since 1994

• 10/39 deaths (26%)

• 1 haploidentical HSCT

ESID LISBOA 2018

PIDs IN PORTUGAL

HH

H

HH

H

ESID LISBOA 2018

PRESIDENT: JOSÉ GONÇALO MARQUES (PT)

Andrew Gennery

Helen Chapel

Graham Davies

Alain Fischer

Despina Moshous

Capucine Picard

Taco Kuijpers

Eleonora Gambineri

Klaus Warnatz

Bodo Grimbacher

Yenan Bryceson

Tayfun Güngör

Luigi Notarangelo

Steve Holland

Jean-Laurent Casanova

Magda Carneiro Sampaio

(UK)

(UK)

(UK)

(FR)

(FR)

(FR)

(NL)

(IT)

(GE)

(GE)

(SW)

(SZ)

(US)

(US)

(US)

(BR)

Susana Lopes da Silva

Isabel Esteves

Ana Espada de Sousa

João Farela Neves

Ana Cordeiro

Emília Faria

Laura Marques

Margarida Guedes

Júlia Vasconcelos

Esmeralda Neves

António Coutinho

João Castro e Melo

Manuel Vilanova

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

(PT)

ESID LISBOA 2018

LISBON CONGRESS CENTRE

10’ DRIVE FROM AIRPORT

5’ FROM THE CENTRE

2’ FROM BELEM, ONE OF THE MAJOR ATTRACTIONS

ESID LISBON 2018

LISBON CONGRESS CENTREYear Event Name Venue Participants

2013 RI - Annual Convention of Rotary International FIL 25.000

2012 ESICM - European Congress on Intensive Care Medicine CCL 4.000

2012 CIRSE - Annual Congress of the Cardiovascular and Interventional Radiological Society

of Europe

CCL 6.173

2011 EASD - Annual Meeting of the European Association for the Study of Diabetes FIL 18.000

2011 EADV - 20th International Congress of the European Academy of Dermatology and

Venereology

CCL 9.000

2011ISBT - XXIst Regional Congress of the International Society of Blood Transfusion

CCL 3.000

2010 FTTH Conference 2010 CCL 2.800

2010 IRF - 16th World Meeting of the International Road Federation CCL 1.103

2010 ESRS - 20th Congress of the European Sleep Research Society CCL 1.000-1.500

2009EFIC - 6th Congress of the European Federation of IASP Chapters "Pain in Europe

CCL 3.500

2009 CIRSE - Annual Congress of the Cardiovascular and Interventional Radiological Society

of Europe

CCL 5.360

2008EACTS - Annual Conference of the European Association for Cardio-Thoracic Surgery

CCL 3.000-5.000

2008 ESICM - European Congress on Intensive Care Medicine CCL 3.917

2008 EUPHA - European Public Health Conference CCL 1.168

2008 EOS - Congress of the European Orthodontic Society CCL 1.800

ESID LISBOA 2018

ESID LISBOA 2018

ESID LISBON 2018

ESID LISBOA 2018

CASCAISSINTRA

Increase of Public, Political and Health Care Workers arousal for PID

Reinforcement for the implementation of:

- SCID Newborn screening

- Establishment of a SINGLE HSCT Centre for PID

- Investment on both clinical and basic investigation

ESID LISBOA 2018

EXPECTATIONS

WELCOME TO PORTUGAL!

WELCOME TO PORTUGAL!