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| Name | Class |
|---|---|
| Royal Children's Hospital | OTHER |
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This is a multicentre prospective audit to determine the incidence of immunodeficiency in children with IPD.
Aims and/or research question of the project
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Children with IPD | Children with IPD will be referred for immunological evaluation. The protocol for immune work up will be the same but between centres there is variation in the age criteria for referral i.e. >2 years in some, >6 months in others. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Referral to Immunology | Other | Screening for primary immunodeficiency including splenic dysfunction |
|
| Measure | Description | Time Frame |
|---|---|---|
| incidence of primary immunodeficiency in children who present with invasive pneumococcal disease | This will be picked up upon prospective immunological testing, after identification of patients with invasive pneumococcal disease. A series of tests will be performed depending on the clinical opinion of the immunology team. Such tests may include lymphocyte subsets, complement function, serum immunoglobulin levels, memory B cells, vaccine antibody responses, etc. | 3 years |
| types of immunodeficiency associated with children who present with invasive pneumococcal disease | This will be determined based on the testing performed by the immunologists. | 3 years |
| Measure | Description | Time Frame |
|---|---|---|
| whether increased referrals of children who present with invasive pneumococcal disease for investigation of primary immunodeficiency facilitates early detection | This information will come from reviewing the case series of patients who are identified from this study. The incidence of those with primary immunodeficiency will be analysed against known background rates of diagnoses. | 3 years |
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Inclusion Criteria:
Exclusion Criteria:
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Children presenting with invasive pneumococcal disease at any of the participating sites listed.
Invasive pneumococcal disease is defined by isolation by culture or PCR of Streptococcus pneumoniae from sterile sites including blood, cerebrospinal fluid (CSF), joint fluid or pleural fluid. In those patients with bacteraemia, their presentation needs to be accompanied by signs of sepsis.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Sydney Children's Hospital | Sydney | New South Wales | 2031 | Australia | ||
| Adelaide Women's and Children's Hospital |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 24759830 | Background | Gaschignard J, Levy C, Chrabieh M, Boisson B, Bost-Bru C, Dauger S, Dubos F, Durand P, Gaudelus J, Gendrel D, Gras Le Guen C, Grimprel E, Guyon G, Jeudy C, Jeziorski E, Leclerc F, Leger PL, Lesage F, Lorrot M, Pellier I, Pinquier D, de Pontual L, Sachs P, Thomas C, Tissieres P, Valla FV, Desprez P, Fremeaux-Bacchi V, Varon E, Bossuyt X, Cohen R, Abel L, Casanova JL, Puel A, Picard C. Invasive pneumococcal disease in children can reveal a primary immunodeficiency. Clin Infect Dis. 2014 Jul 15;59(2):244-51. doi: 10.1093/cid/ciu274. Epub 2014 Apr 23. | |
| 14612669 |
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| Adelaide |
| South Australia |
| Australia |
| Monash Health | Clayton | Victoria | Australia |
| Royal Children's Hospital | Melbourne | Victoria | 3049 | Australia |
| Lady Cilento Children's Hospital | Brisbane | Australia |
| Capital and Coast District Health Board | Wellington | New Zealand |
| Background |
| Picard C, Puel A, Bustamante J, Ku CL, Casanova JL. Primary immunodeficiencies associated with pneumococcal disease. Curr Opin Allergy Clin Immunol. 2003 Dec;3(6):451-9. doi: 10.1097/00130832-200312000-00006. |
| 25738983 | Background | Alsina L, Basteiro MG, de Paz HD, Inigo M, de Sevilla MF, Trivino M, Juan M, Munoz-Almagro C. Recurrent invasive pneumococcal disease in children: underlying clinical conditions, and immunological and microbiological characteristics. PLoS One. 2015 Mar 4;10(3):e0118848. doi: 10.1371/journal.pone.0118848. eCollection 2015. |
| ID | Term |
|---|---|
| C564352 | IRAK4 Deficiency |
| C564468 | Invasive Pneumococcal Disease, Recurrent Isolated, 2 |
| D000081207 | Primary Immunodeficiency Diseases |
| ID | Term |
|---|---|
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007153 | Immunologic Deficiency Syndromes |
| D007154 | Immune System Diseases |
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