This paper described the therapeutic efficacy of immunosuppressive (IS) treatment agents (ATG and CSA) and androgens in the treatment of childhood aplastic anemia (AA). The results showed that the overall curative rat...This paper described the therapeutic efficacy of immunosuppressive (IS) treatment agents (ATG and CSA) and androgens in the treatment of childhood aplastic anemia (AA). The results showed that the overall curative rate was 52.4% in the ATG therapy group (21 cases) and 58.3% in the CSA therapy group (12 cases) respectively. The effective rate of all patients (SAA and CAA) was 58.1% in the IS group (18/31) and 40.4% in the androgens group (42/104), P】0.05. But, in the childhood patients with SAA, the clinical effective rate was 68.4% in the IS group and 7.9% in the androgens group, P【0.01. The laboratory tests revealed that the majority of the AA patients displalyed abnormal immunological states: inversed CD4/ CD8 ratio and increased IL-2 activity. These abnormal immunological states could be normalized in several patients when clinical response was abtained following IS therapy with ATG and CSA.展开更多
Background Hypogammaglobulinemia is common in infant humoral immunodeficiencies and has complicated causes and outcomes. We aimed to determine the clinical manifestations, immunological changes and outcomes of Shangha...Background Hypogammaglobulinemia is common in infant humoral immunodeficiencies and has complicated causes and outcomes. We aimed to determine the clinical manifestations, immunological changes and outcomes of Shanghai infants with hypogammaglobulinemia. Methods Patients under 2 years old, having one or more warning signs of primary immunodeficiency disorders, serum immunoglobulin levels below the lower limit of reference range per age, and with normal numbers for lymphocyte subsets were analyzed and followed up for 2 to 3 years. Results A total of 91 children （male-to-female ratio： 2.25： 1） participated in the study. Initial clinical presentation was recurrent upper respiratory tract infection （46%）, invasive infection （3%）, atopic disease （32%）. IgA reduction （77%） was prevalent; 34% patients had more than one isotype reduced. During follow-up, 51 of 62 patients （82.25%） had immunoglobulins normalized at the age between 12-48 months; these were diagnosed as transient hypogammaglobulinemia of infancy （THI）. Long-term follow-up may reveal a diagnosis for the remaining 11 infants with persistent lower Jmmunoglobulin levels, who did not have antibody titers measured. Earlier onset was correlated with higher rates of normalization. More patients were diagnosed with isolated hypogammaglobulinemia in 2006 compared with the previous 4 years （2002-2005）. Conclusions The awareness of immunodeficiency among pediatricians has been greatly improved. Recurrent otitis media was not a major infection in our patients. THI is a relatively common condition associated with infant hypogammaglobulinemia. In the absence of specific antibody titers, the diagnosis of THI can be confirmed retrospectively with Ig levels normalized in follow-up visits. Therefore, long-term follow-up and frequent re-evaluation of these patients are necessary to distinguish them from true primary immunodeficiency.展开更多
文摘This paper described the therapeutic efficacy of immunosuppressive (IS) treatment agents (ATG and CSA) and androgens in the treatment of childhood aplastic anemia (AA). The results showed that the overall curative rate was 52.4% in the ATG therapy group (21 cases) and 58.3% in the CSA therapy group (12 cases) respectively. The effective rate of all patients (SAA and CAA) was 58.1% in the IS group (18/31) and 40.4% in the androgens group (42/104), P】0.05. But, in the childhood patients with SAA, the clinical effective rate was 68.4% in the IS group and 7.9% in the androgens group, P【0.01. The laboratory tests revealed that the majority of the AA patients displalyed abnormal immunological states: inversed CD4/ CD8 ratio and increased IL-2 activity. These abnormal immunological states could be normalized in several patients when clinical response was abtained following IS therapy with ATG and CSA.
文摘Background Hypogammaglobulinemia is common in infant humoral immunodeficiencies and has complicated causes and outcomes. We aimed to determine the clinical manifestations, immunological changes and outcomes of Shanghai infants with hypogammaglobulinemia. Methods Patients under 2 years old, having one or more warning signs of primary immunodeficiency disorders, serum immunoglobulin levels below the lower limit of reference range per age, and with normal numbers for lymphocyte subsets were analyzed and followed up for 2 to 3 years. Results A total of 91 children （male-to-female ratio： 2.25： 1） participated in the study. Initial clinical presentation was recurrent upper respiratory tract infection （46%）, invasive infection （3%）, atopic disease （32%）. IgA reduction （77%） was prevalent; 34% patients had more than one isotype reduced. During follow-up, 51 of 62 patients （82.25%） had immunoglobulins normalized at the age between 12-48 months; these were diagnosed as transient hypogammaglobulinemia of infancy （THI）. Long-term follow-up may reveal a diagnosis for the remaining 11 infants with persistent lower Jmmunoglobulin levels, who did not have antibody titers measured. Earlier onset was correlated with higher rates of normalization. More patients were diagnosed with isolated hypogammaglobulinemia in 2006 compared with the previous 4 years （2002-2005）. Conclusions The awareness of immunodeficiency among pediatricians has been greatly improved. Recurrent otitis media was not a major infection in our patients. THI is a relatively common condition associated with infant hypogammaglobulinemia. In the absence of specific antibody titers, the diagnosis of THI can be confirmed retrospectively with Ig levels normalized in follow-up visits. Therefore, long-term follow-up and frequent re-evaluation of these patients are necessary to distinguish them from true primary immunodeficiency.
