ObjectiveTo investigate the pathogenesis, clinical manifestations, diagnosis and treatment of common variable immune deficiency (CVID).MethodsOne case of CVID with cellular immunodeficiency leading to bronchiectasis and liver cirrhosis was analyzed retrospectively. Relevant literatures were also searched through WanFang Database, China National Knowledge Infrastructure and PubMed, Ovid, Embase, Cochrane using the key words " common variable immunodeficiency”, " common variable hypogammaglobulinemia” in Chinese and English.ResultsA 52-year-old female patient, complained of cough, expectoration for 20 years, edema for 7 years and aggravated for 3 months with a history of recurrent respiratory infections was hospitalized in the West China Hospital of Sichuan University. The chest computed tomography revealed bronchiectasis, liver cirrhosis and portal hypertension. Laboratory tests showed remarkable hypogammaglobulinemia. The CD4+ T-cell count was below the normal range. Probable diagnosis of CVID was made based on clinical characteristics and laboratory tests. Immunoglobulin infusion with a dose of 20 g was given and the symptoms were relieved. About 288 case reports including 8 000 patients were searched. Most of them were reported individually. Conclusions CVID has a low morbidity and is rare in China. It is mostly caused by genetic factors. When there are recurrent infections in common areas of body, infections in rare areas or infections of conditioned pathogen, clinicians should be vigilant and give intervention as soon as possible. Family and genetic researches could be done when permitted.
ObjectiveTo explore the feasibility and clinical application value of low attenuation areas (LAA) scoring system in patients with acute exacerbation of chronic obstructive pulmonary disease (AECOPD).MethodsA total of 380 patients with AECOPD were included. Clinical data including general information, laboratory examinations and treatments during hospitalization were collected. According to the high-resolution computed CT (HRCT) imaging performance, the patients were divided into bronchitis phenotype and emphysema phenotype. The clinical data between these two groups were compared to analyze the differences between different phenotypes and the feasibility of LAA scoring system.ResultsIn patients of bronchitis phenotype, the levels of body mass index, C-reactive protein, interleukin-6, procalcitonin, neutrophil-to-lymphocyte ratio, and eosinophil counts on admission were higher than those of emphysema phenotype (P<0.05). Patients with emphysema phenotype had a higher proportion of male, a higher smoking index, higher cystatin C levels and lower bilirubin levels on admission (P<0.05), the rates of using mechanical ventilation and systemic glucocorticoids were higher as also (P<0.05). LAA scores had a positive correlation with the use of mechanical ventilation and systemic glucocorticoids and cystatin C levels, and a negative correlation with interleukin-6 levels (P<0.05).ConclusionsFor patients with AECOPD, using LAA scoring system to classify different phenotype through HRCT has relevant accuracy and clinical practicability. The LAA scoring system might help to evaluate the patient's condition and prognosis to a certain extent.