Background High anatomic location, fragility, and generous blood supply of the spleen makes laparoscopic splenectomy （LS） difficult to master, and few patients need splenectomy for benign disorders. The aim of this ...Background High anatomic location, fragility, and generous blood supply of the spleen makes laparoscopic splenectomy （LS） difficult to master, and few patients need splenectomy for benign disorders. The aim of this research was to assess operative outcomes and hematological results of a large series of patients treated with LS for chronic immune thrombocytopenic purpura （ITP） and to determine which clinical variables predict favorable hematological outcome.Methods LS was successfully performed for 154 patients with chronic ITP from September 1999 to April 2009 at the First Affiliated Hospital of Sun Yat-sen University. Operative outcomes were assessed retrospectively. Long-term follow-up data were obtained from outpatient medical records and phone interviews. Clinical and laboratory variables （including gender, age, disease duration before surgery, previous response to steroids, preoperative platelet count, and postoperative peak platelet count） were evaluated by univariate analysis to identify potential predictors of hematological outcome. Multivariate Logistic regression model was used to determine independent predictors of hematological outcome.Results One patient died from subphrenic abscess and postoperative sepsis. The overall major morbidity rate was 8.4%. None of the patients required a second surgery for complications. Of the 127 patients available for a mean follow-up of 43.6 months （range 9-114 months）, the overall initial response （i.e., at two months after LS） and long-term response to LS were achieved in 89.0% and 80.3%, respectively. Five patients （3.9%） developed pneumonia 3-35 months after LS. Univariate analysis showed a significant difference in mean age between responders （29.1 years） and nonresponders （38.8 years; P ＜0.05）. Patients who responded to steroid therapy had better hematological outcome than those who did not respond （P ＜0.05）. Compared to nonresponders, responders to LS had a significantly higher postoperative peak platelet count （404x109/L versus 213×109/L, P ＜0.001）. Multivariate Logistic regression analysis identified postoperative peak platelet count as the only independent predictor of favorable response to LS （P＜0.001）.Conclusiona LS is a safe and effective treatment for chronic ITP. Postoperative peak platelet count may serve as a major predictor of long-term response.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Background High anatomic location, fragility, and generous blood supply of the spleen makes laparoscopic splenectomy （LS） difficult to master, and few patients need splenectomy for benign disorders. The aim of this research was to assess operative outcomes and hematological results of a large series of patients treated with LS for chronic immune thrombocytopenic purpura （ITP） and to determine which clinical variables predict favorable hematological outcome.Methods LS was successfully performed for 154 patients with chronic ITP from September 1999 to April 2009 at the First Affiliated Hospital of Sun Yat-sen University. Operative outcomes were assessed retrospectively. Long-term follow-up data were obtained from outpatient medical records and phone interviews. Clinical and laboratory variables （including gender, age, disease duration before surgery, previous response to steroids, preoperative platelet count, and postoperative peak platelet count） were evaluated by univariate analysis to identify potential predictors of hematological outcome. Multivariate Logistic regression model was used to determine independent predictors of hematological outcome.Results One patient died from subphrenic abscess and postoperative sepsis. The overall major morbidity rate was 8.4%. None of the patients required a second surgery for complications. Of the 127 patients available for a mean follow-up of 43.6 months （range 9-114 months）, the overall initial response （i.e., at two months after LS） and long-term response to LS were achieved in 89.0% and 80.3%, respectively. Five patients （3.9%） developed pneumonia 3-35 months after LS. Univariate analysis showed a significant difference in mean age between responders （29.1 years） and nonresponders （38.8 years; P ＜0.05）. Patients who responded to steroid therapy had better hematological outcome than those who did not respond （P ＜0.05）. Compared to nonresponders, responders to LS had a significantly higher postoperative peak platelet count （404x109/L versus 213×109/L, P ＜0.001）. Multivariate Logistic regression analysis identified postoperative peak platelet count as the only independent predictor of favorable response to LS （P＜0.001）.Conclusiona LS is a safe and effective treatment for chronic ITP. Postoperative peak platelet count may serve as a major predictor of long-term response.
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.
