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Subcutaneous immunoglobulin replacement therapy for primary immunodeficiency in children and young adults. Radana Zachová Department of Immunology University.

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Presentation on theme: "Subcutaneous immunoglobulin replacement therapy for primary immunodeficiency in children and young adults. Radana Zachová Department of Immunology University."— Presentation transcript:

1 Subcutaneous immunoglobulin replacement therapy for primary immunodeficiency in children and young adults. Radana Zachová Department of Immunology University hospital Motol, Charles University Prague, Czech Republic ESID Prague Spring Meeting 2009

2 Immunoglobulin replacement therapy in primary immunodeficiencies Life –long therapy for patients with PID Possible administration ways – intravenous, subcutaneous Historically - intramuscular Disorders: CVID X- linked agammaglobulinemia Specific antibody deficiencies ESID Prague Spring Meeting 2009

3 Subcutaneous immunoglobulin replacement therapy PatientDiagnosis Age (years) Subcutaneous therapy started Age at beginnig of therapy Results ZPCVID Home therapy MKCVID Home therapy JJCVID Home therapy SMCVID Home therapy MKXLA220092Home therapy FVCVID therapy in hospital SVXLP refused s.c.therapy

4 Small infusion pump for scig administration NOT refunded from insurance ! WE lend this pump from company

5 Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy. Case report: MK *2007 History: boy, 1. pregnancy, unrelative parents, born in term, 3100g, anorectal atresia, surgical treatment after delivery, 3 month - pyelonephritis 11 month - bilateral bronchopneumonia Immunoglobulin levels IgG, IgA, IgM – very low CD 19-1,1% CD 20-0,5% BTK mutation –pR28C missence mutation Family history: grandmother´s brother ( mother´s side) died at 18 years ( cerebral absces, recurrent bronchopneumonias)

6 Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy. MK *2007 Therapy: Immunolgobulin substitution therapy was indicated Weight : 10 kg after first application of IVIG m other refused intravenous application of immunoglobulins Intramuscular administration was started 1x in 14 days - painfull, uncomfortable - mother refused I.M. application too We started subcutaneous application of immunoglobulins

7 Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy. Thinks taken into consideration parents reffused IV and IM immunoglobulin substitution completelly subcutaneous therapy ? Disadvantages for SCIG low BMI – small subcutaneous tissue - big local swelling??? scar on abdominal wall after surgical treatment - only one side of abdominal wall can be used for replacement therapy 2 adult persons have to apply the therapy ( 1 has to fix the child) Actually: subcutaneous replacement therapy in home administration

8 Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy. MK *2007

9 Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy. MK *2007

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11 Our experience with SCIG therapy SCIG replacement therapy is not limited by age Patients pretreatet with IVIG may refuse SCIG SCIG is suitable for patients with previous adverse reactions to IVIG

12 Thank you for your attention! ESID Prague Spring Meeting 2009


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