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Primary immunodeficiency (PID) is a heterogeneous group of rare disorders characterized by reduced or absent function of the innate and adaptive immune systems, resulting in increased susceptibility to infection and autoimmune disease. The estimated global prevalence of PID is 29.1-50.5 individuals per 100,000 people. Major advances in pediatric medicine have resulted in many patients with PID transitioning from having a potentially fatal disease to living with a chronic condition; however, this has led to a new set of challenges, as growing up with a chronic disease can place considerable psychological, social, emotional, and financial burden on the children themselves, as well as their relatives. Indeed, chronically ill children frequently report having lower health-related quality of life (HRQOL) compared to their healthy peers. In addition to reduced HRQOL, children with PID also have an increased risk of psychological problems and experience limitations in both physical and social activities. Recommended treatment for improving the overall well-being and quality of life of pediatric patients with PID?

Primary immunodeficiency (PID) is a heterogeneous group of rare disorders characterized by reduced or absent function of the innate and adaptive immune systems, resulting in increased susceptibility to infection and autoimmune disease. The estimated global prevalence of PID is 29.1-50.5 individuals per 100,000 people. Major advances in pediatric medicine have resulted in many patients with PID transitioning from having a potentially fatal disease to living with a chronic condition; however, this has led to a new set of challenges, as growing up with a chronic disease can place considerable psychological, social, emotional, and financial burden on the children themselves, as well as their relatives. Indeed, chronically ill children frequently report having lower health-related quality of life (HRQOL) compared to their healthy peers. In addition to reduced HRQOL, children with PID also have an increased risk of psychological problems and experience limitations in both physical and social activities. Recommended treatment for improving the overall well-being and quality of life of pediatric patients with PID?

Biochemistry
Biochemistry
9th Edition
ISBN:9781319114671
Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Chapter1: Biochemistry: An Evolving Science
Section: Chapter Questions
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Primary immunodeficiency (PID) is a heterogeneous group of rare disorders characterized by reduced or absent function of the innate and adaptive immune systems, resulting in increased susceptibility to infection and autoimmune disease. The estimated global prevalence of PID is 29.1-50.5 individuals per 100,000 people. Major advances in pediatric medicine have resulted in many patients with PID transitioning from having a potentially fatal disease to living with a chronic condition; however, this has led to a new set of challenges, as growing up with a chronic disease can place considerable psychological, social, emotional, and financial burden on the children themselves, as well as their relatives. Indeed, chronically ill children frequently report having lower health-related quality of life (HRQOL) compared to their healthy peers. In addition to reduced HRQOL, children with PID also have an increased risk of psychological problems and experience limitations in both physical and social activities. Recommended treatment for improving the overall well-being and quality of life of pediatric patients with PID? Hematopoietic stem cell transplantation may be the best approach for improving the overall well-being and quality of life of pediatric patients with PID. O Nearly 20% of pediatric patients with PID reported experiencing severe fatigue, and fatigue was reported among a wide range of PID subcategories. In addition, severe fatigue negatively affected the patient's quality of life and daily functioning, but was not associated with disease activity or comorbidity. Thus, targeting severe fatigue might be a promising strategy for improving the overall well-being and quality of life of pediatric patients with PID. Antibiotics may be the best approach for improving the overall well-being and quality of life of pediatric patients with PID. Immunoglobulin therapy may be the best approach for improving the overall well-being and quality of life of pediatric patients with PID.
Transcribed Image Text:Primary immunodeficiency (PID) is a heterogeneous group of rare disorders characterized by reduced or absent function of the innate and adaptive immune systems, resulting in increased susceptibility to infection and autoimmune disease. The estimated global prevalence of PID is 29.1-50.5 individuals per 100,000 people. Major advances in pediatric medicine have resulted in many patients with PID transitioning from having a potentially fatal disease to living with a chronic condition; however, this has led to a new set of challenges, as growing up with a chronic disease can place considerable psychological, social, emotional, and financial burden on the children themselves, as well as their relatives. Indeed, chronically ill children frequently report having lower health-related quality of life (HRQOL) compared to their healthy peers. In addition to reduced HRQOL, children with PID also have an increased risk of psychological problems and experience limitations in both physical and social activities. Recommended treatment for improving the overall well-being and quality of life of pediatric patients with PID? Hematopoietic stem cell transplantation may be the best approach for improving the overall well-being and quality of life of pediatric patients with PID. O Nearly 20% of pediatric patients with PID reported experiencing severe fatigue, and fatigue was reported among a wide range of PID subcategories. In addition, severe fatigue negatively affected the patient's quality of life and daily functioning, but was not associated with disease activity or comorbidity. Thus, targeting severe fatigue might be a promising strategy for improving the overall well-being and quality of life of pediatric patients with PID. Antibiotics may be the best approach for improving the overall well-being and quality of life of pediatric patients with PID. Immunoglobulin therapy may be the best approach for improving the overall well-being and quality of life of pediatric patients with PID.
Since 1993, more than 100 children with congenital athymia have been treated with cultured thymus tissue implantation (CTTI). Naïve T cells develop approximately 6 to 12 months after CTTI. What have immunologists discovered that should assist multidisciplinary teams in caring for children with CDGA both before and after CTTI. That CTT (cultured thymus tissue) must be protected from high doses of steroids which can damage CTT, and organs must be protected from adverse effects of immunosuppression. That humoral immune deficiencies were more common among patients with with congenital athymia. That there is still a significant deficit between those with DGS who have laboratory evidence of a humoral immune deficiency and those being treated for it. That there is a discordance in the reported prevalence of humoral immune deficiency in patients with DiGeorge syndrome (DGS) and its treatment.
Transcribed Image Text:Since 1993, more than 100 children with congenital athymia have been treated with cultured thymus tissue implantation (CTTI). Naïve T cells develop approximately 6 to 12 months after CTTI. What have immunologists discovered that should assist multidisciplinary teams in caring for children with CDGA both before and after CTTI. That CTT (cultured thymus tissue) must be protected from high doses of steroids which can damage CTT, and organs must be protected from adverse effects of immunosuppression. That humoral immune deficiencies were more common among patients with with congenital athymia. That there is still a significant deficit between those with DGS who have laboratory evidence of a humoral immune deficiency and those being treated for it. That there is a discordance in the reported prevalence of humoral immune deficiency in patients with DiGeorge syndrome (DGS) and its treatment.
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