Quality in Primary Care Open Access

Introduction

Primary immunodeficiencies (PID) can be described as heterogeneous diseases with a shortcoming in the development and function of the immune system. Patients with this Primary immunodeficiency are generally more prone to develop recurrent infections and or autoimmune diseases. Primary immunodeficiency significantly influences patient quality of life, limiting their working ability, physical and social activities.

These conditions has been linked with a few negative effects on physical and social activities and is associated with increased anxiety and fatigue. Despite an increasing number of papers on health-related quality of life in Primary immune deficiency patients, little attention has been paid to the systematic evaluation of available data.

Causes of Primary Immunodeficiencies

Mutations in genes involved in the development and function of immune cells, organs, and molecules.Genetically determined, Usually disease of infancy and childhood. One in 2,000 children younger than eighteen years is assumed to possess a primary immunological disorder sickness. Antibody, combined B-cell and T-cell, phagocytic, and complement disorders square measure the foremost common sorts. kids with these diseases tend to possess microorganism or plant infections with uncommon organisms, or unusually severe and continual infections with common organisms. A case history of primary immunological disorder sickness is that the strongest predictor of someone having this kind of sickness. once associate degree immunological disorder sickness is suspected, initial laboratory screening ought to embrace a whole blood count with differential and measuring of body fluid immune gamma globulin and complement levels. The presence of blood disorder on complete blood count suggests a T-cell disorder, whereas a finding of leukopenia suggests a somatic cell disorder. Abnormal body fluid immune gamma globulin levels counsel a B-cell disorder. Abnormalities on assay of the classic or different complement pathways counsel a complement disorder. If laboratory results square measure abnormal, or if clinical suspicion continues despite traditional laboratory results, kids ought to be referred for more analysis. Human immunological disorder infection ought to even be thought-about, and testing ought to be performed, if appropriate; this infection typically clinically resembles a T-cell disorder.

Survival is often considered the most important outcome in study design; however, the impact of illness on health-related quality of life is receiving increasing recognition. Health related quality of life measurements are becoming important in analyzing not only the effect of the disease on patient well-being, but also satisfaction of patients with specific treatment regimens.

Because many patients with PID require therapy indefinitely, the form of administration and setting where the product is administered are important factors that can significantly affect Health related quality of life. Some Specific measures is to evaluate the outcome of an illness or its treatment include a quality of life, quantity of life and economic cost.

The following is a review of the medical literature that measured Health related quality of life in PID patients.

• Health related quality of life (HRQOL) indicators.

• Quality of Life in PID

• IVIG vs no therapy

• Routes of administration-SCIG vs IVIG