Immunoglobulin replacement therapy contribution in agammaglobulinemia: 10 case reports]

Intravenous immunoglobulin (Ig IV) has been used for many years in the treatment of primary antibody deficiencies. We performed a retrospective study of the clinical features and outcome of agammaglobulinemia children who received prolonged Ig IV infusions. PATIENTS AND METHODS: Ten children, 9 male et 1 female, with agammaglobulinemia diagnosis were studied for the clinical manifestations before and during the Ig IV replacement therapy. Serum Ig levels were quantified by nephelometry. Circulating B ant T cells were counted by immunofluorescence labeling by monoclonal antibodies. T-cell functions were assessed by using mitogen and antigen -induced T-cell proliferation assays in vitro. Patients clinical status was evaluated respectively, before initiation and at every moment (when patients had an infection) of the replacement therapy. RESULTS: Ig IV therapy was performed for 866 cumulated months, median 108 months. The median Ig IV doses administered to the 10 patients was 500 mg/kg/month. Residual serum IgG mean level was 3,9 g/L. All patients had 99 bacterial infections/year before Ig IV, mainly respiratory tract infections (48,5%), and 4 patients had bronchiectasis before Ig replacement therapy. The number of infection/year fall to 25 during IgIV replacement, and the infection/patient/year rate decreases significantly. One patient developed an Echovirus 27 meningoencephalitis during this treatment. CONCLUSION: Ig IV therapy with residual IgG mean level of 3,9 g/l reduced significantly the rate of bacterial infections. The use of specific antibiotherapy and respiratory kinesitherapy led to a lower rate of respiratory tract infections, and the stabilisation of the bronchiectasis. However this intravenous replacement therapy does not protect against viral meningoencephalitis.     

手指离断再植术后护理

目的 探讨断指再植护理措施。方法 采用指体的摆放，病房适宜温度20～25℃，烤灯局部保温，术后观察血运指标，小儿特殊护理及康复指导。结果 成活优良率91．6％，住院天数平均16天，功能恢复满意率75％。结论 护理措施恰当，可显降低并发症的发生，提高治愈率。     

Risk factors of type 2 diabetes among Korean adults: The 2001 Korean national health and nutrition examination survey

This study aimed to identify risk factors for type 2 diabetes (T2D) in Korea, a rapidly changing country. Data of 5,132 adults aged 20-85 were used from the 2001 Korean Health and Nutrition Examination Survey. Multiple logistic regression was carried out to identify risk factors for T2D. Three models were specified: (i) socioeconomic and demographic factors (model 1: age, gender, education, poverty income ratio, employment), (ii) behavioral risk factors and covariates (model 2: obesity, physical activity, smoking, alcohol drinking, dietary quality, family history of T2D, co-morbidity) and (iii) socioeconomic, demographic, and behavioral factors (model 3). The prevalence of T2D was 7.4%. Less education (OR 1.41, 95% CI 1.08-1.84), age (OR 2.19, 95% CI 1.56-3.08 in 40-59 yrs, OR 4.05, 95% CI 2.76-5.95 in 60 yrs + comparing to 20-39 yrs) and abdominal obesity (OR 2.24, 95% CI 1.79-2.82) were risk factors for T2D even after controlling for other factors simultaneously. There was a significant association of T2D with ever smoking (OR 1.34, 95% CI 1.06-1.67). The relationship of age with T2D was modified by gender in model 1 and the relationship of smoking with T2D was modified by obesity in model 2. Less educated, older, obese or ever smokers were more likely to have T2D. Gender mediated the relationship of age, and obesity mediated the relationship of smoking, with T2D. Intervention programs for T2D in Korea should take the interactions among risk factors into account.     

Chronic painful physical conditions, disturbed sleep and psychiatric morbidity: results from an elderly survey.

BACKGROUND: The main purpose of this study is to investigate the association of disturbed sleep, chronic physical pain and psychiatric morbidity in people aged 60 years and over. METHODS: A population-based random sample of 7040 household residents aged 60 years and over, was examined in a face-to-face interview. Painful medical conditions were assessed through questions evaluating medical treatment, hospitalizations, and consultations for medical problems. Disturbed sleep was assessed through questions concerning the presence of sleeping problems in the past 4 weeks. RESULTS: The overall prevalence of disturbed sleep is 33.7% (95% CI: 32.5-34.8) and the 6-month prevalence of any chronic pain was 76.2% (95% CI: 74.2-78.2%). A substantial burden of disturbed sleep is associated with the presence of physical pain morbidity, 42.5% for back pain to 49.7% for headaches. The prevalence of pain among persons with disturbed sleep ranges from 25.8% for gastrointestinal pain to 54.6% for joint pain. The presence of comorbid pain and disturbed sleep has an important disability impact as assessed by socioecomomic/professional data, health care utilization, self-rated health and physical activity. In logistic regression models, headaches, psychiatric morbidity, rural origin, Caucasians, self-rated health and number of chronic pain conditions were significantly associated with disturbed sleep after controlling for demographic variables and comorbidities. Age has a negative effect on sleep complaints. CONCLUSIONS: Disturbed sleep is highly comorbid with other pain conditions particularly headaches and psychiatric morbidity in later life. The combination of pain and disturbed sleep leads to important role disability and increased utilization of medical services.     

Long-term improvement of metabolic control without increased risk of hypoglycaemia by intensive insulin regimens in type 1 diabetes patients treated in a regular clinical setting.

AIM: To evaluate if intensive insulin regimen with multiple daily injections (MDI) is successful for treating type 1 diabetes patients over a long period of time in a regular clinical setting. METHOD: This is a prospective, observational seven-year study. Fifty-nine (35 male) type 1 diabetic patients with bad metabolic control (HbA1c> or =9%), aged 31.9 years, range 18-47 were included in the present study. All of them had had at least 5 years of diabetes duration after diagnosis and showed negative responses of serum C-peptide to a standard breakfast. The main control variables are: Metabolic control measured by serum HbA1c values (values < 6.2 % was the treatment objective) and the frequency of hypoglycaemic episodes (episodes/patient-month). RESULTS: Significant decreases in mean+/-SD HbA1c values in this group of patients were observed from the first year of follow-up, with the mean values being: 7.5+/-1.5%, 7.2+/-1.8%, 7.6+/-1.6%, 7.1+/-1.7%, 7+/-1.4, 6.6+/-1.6% and 6.8+/-1.4% for the first, second, third, fourth, fifth, sixth and seventh years of follow-up respectively. Sixteen %, 27.5%, 15.7%, 33.3%, 28.6%, 42% and 33% of the patients reached the treatment objective (HbA1c values<6.2%) for each year of follow-up. Throughout the study period the rate of severe hypoglycaemia (episodes/patient-year) was 0.32+/-0.2 which was not significantly different compared with the value of 0.28+/-0.1 observed the year before the study began. Similarly frequencies of mild/moderate hypoglycaemia episodes (episodes/patient-month) varies between 16.5+/-4 and 21.7+/-5, which are not significantly different from the value of 17.7+/-6 observed the year before the study began. CONCLUSION: Long-term improvement in metabolic control was observed in this group of type 1 diabetes patients with previous bad control, during treatment in a regular clinical setting. A considerable percentage of type 1 diabetic patients with MDI reached the treatment objective in every year of follow-up. Furthermore improvement in metabolic control is not associated with significantly increased frequency of hypoglycaemia episodes.     

Cystic fibrosis mortality trends in France.

BACKGROUND: In 1992 France set up a national cystic fibrosis observatory (Observatoire national de la mucoviscidose, ONM) to monitor the state of health of patients on an annual basis. Using the ONM data, this study estimates the main indicators for life expectancy and assesses the total number of cystic fibrosis patients. METHODS: The data for the years 1994 to 2003 are divided into 3-year periods. Life tables are drawn up for these periods, from which mean and median lengths of life are determined. Using the most recent life table, the number of births in 2003 and the incidence of the disease, the total population of patients can be estimated, assuming a stationary population. RESULTS: In 2001-2003, life expectancy at birth of patients registered with the ONM was 39.1 years and median length of life was 36.4 years. These results, substantially better than those of 1994-1996, are linked to improved conditions of patient inclusion in the ONM database, to improvements in their healthcare, but also to the limitations of the life tables. Based on the 2003 data, the total theoretical number of patients is 6490, and coverage by the ONM database is thus 63.2%. CONCLUSIONS: These provisional results demonstrate the need to convert the ONM observatory into a registry providing exhaustive coverage of all patients.