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
Background Birth asphyxia may result in multiple organ dysfunction such as lung injury. Inhalation of nebulized nitric oxide precursor can selectively reduce pulmonary hypertension. However, it is unknown whether such...Background Birth asphyxia may result in multiple organ dysfunction such as lung injury. Inhalation of nebulized nitric oxide precursor can selectively reduce pulmonary hypertension. However, it is unknown whether such precursors can alleviate lung injury induced by hypoxia. We evaluated the effect of inhalation of nebulized nitroglycerine and sodium nitroprusside on acute hypoxic lung injury in newborn piglets. Methods Acute hypoxic lung injury was induced by inspiring 10% 02 for 1 hour. Twenty-four anaesthetized and mechanically ventilated piglets （5-7 days old） were randomly divided into four groups： （1） group S, not hypoxic; （2） group C, nebulized saline after hypoxia; （3） group NTG, nebulized nitroglycerine after hypoxia; （4） group SNP, nebulized sodium nitroprusside after hypoxia, Respiratory dynamic compliance and resistance of respiratory system were recorded at baseline, 0.5 hour and 1 hour of hypoxia; then 0.5 hour, 1 hour, 3 hours and 5 hours following hypoxia. After nebulization, arterial blood was collected for measuring methaemoglobin and nitrate/nitrite levels. Right lung tissue, wet-dry ratio and myeloperoxidase level were determined. White blood cell count （WBC）, total surfactant phospholipids （TPL） and disaturated phosphatidyl choline （DSPC） of the bronchoalveolar lavage fluid （BALF） were calculated. Left lungs were used for examining pathological changes. Results No significant difference was observed in respiratory dynamic compliance, resistance of respiratory system, wet-dry ratio, levels of methaemoglobin and nitrate/nitrite after nebulization, TPL or DSPC/TPL among four groups. WBC in BALF in groups NTG and SNP significantly decreased as compared with group C： similarly for myeloperoxidase level in lung tissue. Lung histological findings showed infiltration of neutrophils in groups NTG and SNP decreased significantly as compared with group C. Conclusion Inhalation of nebulized nitroglycerine or sodium nitroprusside can alleviate the infiltration of neutrophils, while it affects neither the metabolism of phospholipids nor water content in the lungs.展开更多
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.
文摘Background Birth asphyxia may result in multiple organ dysfunction such as lung injury. Inhalation of nebulized nitric oxide precursor can selectively reduce pulmonary hypertension. However, it is unknown whether such precursors can alleviate lung injury induced by hypoxia. We evaluated the effect of inhalation of nebulized nitroglycerine and sodium nitroprusside on acute hypoxic lung injury in newborn piglets. Methods Acute hypoxic lung injury was induced by inspiring 10% 02 for 1 hour. Twenty-four anaesthetized and mechanically ventilated piglets （5-7 days old） were randomly divided into four groups： （1） group S, not hypoxic; （2） group C, nebulized saline after hypoxia; （3） group NTG, nebulized nitroglycerine after hypoxia; （4） group SNP, nebulized sodium nitroprusside after hypoxia, Respiratory dynamic compliance and resistance of respiratory system were recorded at baseline, 0.5 hour and 1 hour of hypoxia; then 0.5 hour, 1 hour, 3 hours and 5 hours following hypoxia. After nebulization, arterial blood was collected for measuring methaemoglobin and nitrate/nitrite levels. Right lung tissue, wet-dry ratio and myeloperoxidase level were determined. White blood cell count （WBC）, total surfactant phospholipids （TPL） and disaturated phosphatidyl choline （DSPC） of the bronchoalveolar lavage fluid （BALF） were calculated. Left lungs were used for examining pathological changes. Results No significant difference was observed in respiratory dynamic compliance, resistance of respiratory system, wet-dry ratio, levels of methaemoglobin and nitrate/nitrite after nebulization, TPL or DSPC/TPL among four groups. WBC in BALF in groups NTG and SNP significantly decreased as compared with group C： similarly for myeloperoxidase level in lung tissue. Lung histological findings showed infiltration of neutrophils in groups NTG and SNP decreased significantly as compared with group C. Conclusion Inhalation of nebulized nitroglycerine or sodium nitroprusside can alleviate the infiltration of neutrophils, while it affects neither the metabolism of phospholipids nor water content in the lungs.
