Humans ; Immunologic Deficiency Syndromes/therapy*

China ; Humans ; Immunologic Deficiency Syndromes ; diagnosis ; therapy

Child ; China ; Humans ; Immunologic Deficiency Syndromes ; therapy ; Pediatrics

Primary immunodeficiency diseases (PID) is a congenital disease caused by single gene germline mutation related to the immune system. PID patients have immune dysregulation, and are susceptible to infectious diseases, autoimmune diseases, autoimmune diseases, allergic diseases, and malignant tumors. The first symptom of some PID patients is atopic disease, therefore they go to the department of allergy, department of pediatrics and other relevant departments. How to identify and diagnose PID in allergic patients, to reduce diagnosis delay and prevent disease aggravation are the abilities that allergists, pediatricians, and doctors in other relevant departments need to master. This article summarizes the warning signs of PID in allergic patients and the mechanism of allergy combined with PID, and then summarizes the common types of PID in allergic patients, the evaluation, treatment and prevention in patients with PID and allergy.
Autoimmune Diseases ; Child ; Humans ; Hypersensitivity/therapy* ; Immunologic Deficiency Syndromes/therapy* ; Primary Immunodeficiency Diseases/therapy*

OBJECTIVETo summarize clinical features of primary immunodeficiency diseases (PID) in children.

METHODSThe clinical data of 35 children with PID from September 2005 to December 2008 were studied retrospectively, including illness history, birth history, family history, clinical manifestations, laboratory findings, diagnosis, treatment and outcome.

RESULTSOf the 35 cases of PID, 6 cases were confirmed with combined T- and B-cell immunodeficiency, 4 cases with X-linked agammaglobulinaemia, 22 cases with selective IgG subclass deficiency, 1 case with common variable immunodeficiency and 2 cases with chronic granulomatous disease. All cases had fever and recurrent infections. Respiratory and digestive tract infections were the most common clinical manifestation. Some of the PID cases lagged behind the normal children of the same age in growth and development. Human gamma-globulin transfusion and anti-infection therapy were administered. Two patients discontinued the therapy, one was transferred to the other hospital and the other 32 patients were discharged following improvement in clinical symptoms.

CONCLUSIONSPID should be considered in children who suffer from recurrent infections and autoimmune diseases or do not respond to long-term use of antibiotics. Immunologic tests should be done as early as possible for the children.

Child, Preschool ; Female ; Humans ; Immunologic Deficiency Syndromes ; complications ; diagnosis ; therapy ; Infant ; Infant, Newborn ; Male ; Retrospective Studies

Chronic Disease ; Humans ; Immunologic Deficiency Syndromes ; complications ; immunology ; therapy ; Medicine, Chinese Traditional ; methods ; Thrombocytopenia ; complications ; immunology ; therapy

Adult ; Carrier State ; drug therapy ; Female ; Hepatitis B, Chronic ; complications ; drug therapy ; immunology ; Humans ; Immunologic Deficiency Syndromes ; complications ; Lamivudine ; therapeutic use ; Male ; Middle Aged ; Secondary Prevention

Child ; China ; epidemiology ; Diagnosis, Differential ; Early Diagnosis ; Hematopoietic Stem Cell Transplantation ; methods ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; pharmacology ; Immunologic Deficiency Syndromes ; diagnosis ; epidemiology ; prevention & control ; therapy ; Immunologic Factors ; administration & dosage ; pharmacology ; Infant, Newborn ; Neonatal Screening

Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.
Fatal Outcome ; Granuloma, Respiratory Tract/diagnosis/*etiology/therapy ; Humans ; Immunologic Deficiency Syndromes/*complications/immunology/pathology ; Lung Diseases/diagnosis/*etiology/therapy ; Male ; Middle Aged ; Thymoma/*complications/immunology/pathology ; Thymus Neoplasms/*complications/immunology/pathology

Anemia, Aplastic ; therapy ; Bone Marrow Transplantation ; statistics & numerical data ; China ; Cooperative Behavior ; Family ; Hematopoietic Stem Cell Transplantation ; statistics & numerical data ; Hemoglobinopathies ; therapy ; Hong Kong ; Humans ; Immunologic Deficiency Syndromes ; therapy ; Leukemia ; therapy ; Lymphoma ; therapy ; Malaysia ; Myelodysplastic Syndromes ; therapy ; Peripheral Blood Stem Cell Transplantation ; statistics & numerical data ; Philippines ; Singapore ; Thailand ; Tissue Donors ; statistics & numerical data ; Transplantation, Autologous ; statistics & numerical data ; Transplantation, Homologous ; statistics & numerical data