复旦大学：《儿科学》课程教学资源（PPT课件讲稿，英文版）Immunodeficiency diseases

Immunodeficiency diseases Wang Xiaochuan Department of Clinical Immunology. Childrens Hospital of Fudan University Wang Xiaochuan Children's Hospital

Wang Xiaochuan Children’s Hospital Immunodeficiency diseases Wang, Xiaochuan Department of Clinical Immunology, Children’s Hospital of Fudan University

Immunologic disorders Pathogens nections Immunity immunity Tinan PID Autoimmune disease Allergy Tumor Immunity Environment Material Wang Xiaochuan Children's Hospital

Wang Xiaochuan Children’s Hospital Pathogens Environment Material Immunity Infectious Immunity Allergy Tumor Immunity Autoimmune Disease PID

Immunocompromised Host Individual who has one or more defects in the body's natural defense mechanisms which are sufficiently severe to predispose the host to life threatening infection Primary -PID, damage to anatomical barriers Secondary -infection, tumor, drug, others Physiology- Infants, aged Wang Xiaochuan Children's Hospital

Wang Xiaochuan Children’s Hospital Immunocompromised Host • Individual who has one or more defects in the body’s natural defense mechanisms which are sufficiently severe to predispose the host to life￾threatening infection • Primary - PID, damage to anatomical barriers • Secondary - infection, tumor, drug, others • Physiology- Infants, aged

Characteristics of Immunoglobulines Igg lgm iga led lgE Serum con.(mg/d)120012020030.01 Percentage 70-805-1010-15<1<0.01 Half life( days) 21 72.82.3 Distribution (9o) 45 80 457550 Quantity(mg/kg)1150492301.50.04 Synthesis(mg/kg/D) 35 7 25 0.4 0.02 Transport through placenta Wang Xiaochuan Children's Hospital

Wang Xiaochuan Children’s Hospital Characteristics of Immunoglobulines IgG IgM IgA IgD IgE Serum Con. (mg/dl) 1200 120 200 3 0.01 Percentage 70-80 5-10 10-15 <1 <0.01 Half life(days) 21 5 7 2.8 2.3 Distribution (%) 45 80 45 75 50 Quantity (mg/kg) 1150 49 230 1.5 0.04 Synthesis(mg/kg/D) 35 7 25 0.4 0.02 Transport through placenta + - - - -

Development of Immunoglobulin Igg level of Infant 100% Igg from mother igg of infant birth Wang Xiaochuan Children's Hospital

Wang Xiaochuan Children’s Hospital Development of Immunoglobulin IgG from motherIgG of infant birth 6M 100% IgG level of Infant

Age dependent changes of serum Igs levels(g/L) Ages Igg IgA leM Neonate6.46-17740.004-0.0170.05-0.27 Im- 2.75-7.500.05-0.60 0.10-0.70 4m 3.70-8.300.140.50 0.33-125 7m 3.50-8.900.06-0.54 0.36-1.20 5.52-11.460.06-0.74 0.60-2.12 4.95-12.740.33-0.89 0.65-2.01 7 6.09-12.850.52-2.16 0.67-2.46 12 6.98-14260.92-2.50 0.56-2.18 15-18y7.54-16.020.89-3.24 0.72-228 Wang Xiaochuan Children's Hospital

Wang Xiaochuan Children’s Hospital Age dependent changes of serum Igs levels(g/L) Ages IgG IgA IgM Neonate 6.46-17.74 0.004-0.017 0.05-0.27 1m- 2.75-7.50 0.05-0.60 0.10-0.70 4m- 3.70-8.30 0.14-0.50 0.33-1.25 7m- 3.50-8.90 0.06-0.54 0.36-1.20 1y- 5.52-11.46 0.06-0.74 0.60-2.12 3y- 4.95-12.74 0.33-0.89 0.65-2.01 7y- 6.09-12.85 0.52-2.16 0.67-2.46 12y- 6.98-14.26 0.92-2.50 0.56-2.18 15-18y 7.54-16.02 0.89-3.24 0.72-2.28

Schematic diagram of the exposure of microorganism during early life pathogens probiotics fetus Full tern 6M Day care Wang Xiaochuan Children's Hospital

Wang Xiaochuan Children’s Hospital Schematic diagram of the exposure of microorganism during early life fetus Full tern 6M Day care pathogens probiotics

Period of susceptible children premature 6M Day care Full term Wang Xiaochuan Childrens Hospital

Wang Xiaochuan Children’s Hospital Period of susceptible children premature Full term 6M Day care

Management Exclusion of pid Clinical features Frequency of infection Severit Complications Follow up Wang Xiaochuan Childrens Hospital

Wang Xiaochuan Children’s Hospital Management • Exclusion of PID • Clinical features – Frequency of infection – Severity – Complications • Follow up

Management General medical care(prevention, nutrition exercise Complication Treatment Medication Antibiotics Immune regulator Immunizations Wang Xiaochuan Childrens Hospital

Wang Xiaochuan Children’s Hospital Management • General medical care (prevention、nutrition、 exercise) • Complication Treatment • Medication – Antibiotics – Immune regulator – Immunizations