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41.
OBJECTIVE: Although incentive spirometry (IS) is frequently used to prevent postoperative pulmonary complications, its efficacy in patients with COPD has not been documented. The aim of this study was to evaluate the effects of IS on pulmonary function tests, arterial blood gases, dyspnoea and health-related quality of life in patients hospitalized for COPD. METHODOLOGY: A total of 27 consecutive patients (mean age, 68.4 +/- 7.9 years; 26 males) admitted for COPD exacerbations were recruited for the study. In total, 15 (IS treatment group) used IS for 2 months, together with medical treatment. The remaining 12 (medical treatment group) were given only medical treatment. Pulmonary function and blood gases were measured. Assessment of dyspnoea by visual analogue scale (VAS) and quality of life using the St. George's Respiratory Questionnaire (SGRQ) were performed at admission and after 2 months of treatment. RESULTS: The activity, impact and total scores for the SGRQ improved (all P < or = 0.0001), PaCO2 values decreased (P = 0.02), PaO2 and PAO2 values increased (P = 0.02 and P = 0.01, respectively) in the IS treatment group. However, there were no significant differences between the measurements made pretreatment and after 2 months of medical therapy in the medical treatment group, with regards to pulmonary function, blood gases, SGRQ scores and VAS. CONCLUSION: The use of IS appears to improve arterial blood gases and health-related quality of life in patients with COPD exacerbations, although it does not alter pulmonary function parameters.  相似文献   
42.
OBJECTIVES: 1) To compare the sensitivity of serum amyloid A protein (A-SAA) and other acute phase proteins (APPs) in determining subclinical inflammation in patients with familial Mediterranean fever (FMF) during an attack-free period; 2) to define those clinical, laboratory features that may modify the A-SAA level; and 3) to evaluate the effect of an increase in the colchicine dose on the A-SAA level. METHODS: A-SAA, CRP, ESR, fibrinogen and ferritin levels were measured in 183 patients [88 F, 95 M; median age 11.0 years (1.0-20.0)] with FMF during an attack-free period. Mutational analysis was available in 157 patients. The colchicine dose was increased in 26 randomly chosen patients with no attacks within the last year; laboratory studies were repeated at the end of the second month. RESULTS: During an attack-free period, the median A-SAA level was 74 (6-1,500) mg/L; other APPs were within normal ranges in 49-93% of the patients. Age, gender, age at onset, age at diagnosis, the duration of treatment and the frequency of attacks had no significant effect on the A-SAA level. Homozygous and compound heterozygous patients had higher A-SAA levels than heterozygous patients [129 mg/L (8-1,500) versus 29 mg/L (6-216); p < 0.005]. There was a dramatic decrease in the A-SAA level [from 244 mg/L (16-1,400) to 35.5 mg/L (8-1,120); p < 0.001] and an increase in the hemoglobin (1.89 +/- 0.10 mmol/L to 1.98 +/- 0.19 mmol/L; p < 0.005) after the increase in colchicine dose in 26 patients. CONCLUSION: Subclinical inflammation continues during an attack-free period in FMF patients. A-SAA was the best marker of subclinical inflammation. Patients who are homozygous or compound heterozygotes of MEFV mutations had higher A-SAA levels. An increase in the colchicine dose resulted in a dramatic decrease in A-SAA and an increase in hemoglobin level. These findings favor the use of A-SAA in drug monitoring.  相似文献   
43.
Summary The immunoinflammatory pathogenesis of juvenile chronic arthritis (JCA) involves the activation of many pathways including various cytokines. We have evaluated the levels of interleukin (IL)-1, IL-6 and IL-8 in 29 JCA patients. The age range was 1–16 with a mean of 10.1. A disease activity score was developed on the basis of: 1.constitutional symptoms and/or morning stiffness, 2.presence of joint swelling, 3.warmth, 4.limited range of motion, and 5.joint pain. This score correlated very significantly with laboratory disease activity markers such as erythrocyte sedimentation rate (ESR) and CRP (both p=0.006) and also correlated with IL-1 and IL-6 levels. The levels of IL-1 decreased in four of the five patients with improved disease activity. IL-6 but not IL-1 correlated significantly with the number of inflamed joints (p=0.013); IL-6 also strongly correlated with rheumatoid factor supporting this cytokine's role in B cell induction (p=0). Haemoglobin values correlated negatively with the activity index, ESR, CRP, IL-1 and IL-6. IL-8 did not correlate with disease activity markers. In the systemic patients all cytokines tended to be higher. Our data suggest that interleukins 1 and 6 are effective in the pathogenesis of JCA. Whether cytokines may be used for monitoring therapy may be clarified with further studies.  相似文献   
44.

Purpose

IPEX (Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked) is a rare X-linked recessive life-threatening disorder characterized by autoimmunity and early death. Pulmonary complication related with IPEX has not been elucidated exactly. Here, we report 4 IPEX patients, 3 of which died from severe pulmonary disease.

Methods

Clinical data and laboratory findings including autoantibodies, immunoglobulin levels as well as number of T, B and NK cells were evaluated. FOXP3 expression and T reg activity were analyzed. The FOXP3 gene was sequenced and RNA analysis was performed.

Results

Patient I (PI) presented with nephrotic syndrome at 3 years of age and then developed autoimmune hepatitis without eczema, enteropathy or high IgE and died at 9 years of age due to acute respiratory distress syndrome (ARDS). Two cousins of PI had the same hypomorphic splice site mutation leading to a deletion of 27 amino acids, but normal FOXP3 protein expression and normal suppressive capacity of T reg in a proliferation inhibition assay. However, they exhibited typical symptoms such as eczema, diabetes and enteropathy with eosinophilia at early age (PII, PIII) and were transplanted in infancy. One of them had severe respiratory distress right after birth (PIII). Patient IV from another family presented with chronic diarrhea without autoimmune manifestations and died due to ARDS.

Conclusion

Lung disease related to IPEX syndrome has not been reported before and this entity could be a critical factor in disease outcome.  相似文献   
45.

Purpose

To increase the knowledge of central nervous system (CNS) imaging features in deficiency of adenosine deaminase 2 (DADA2) by examining magnetic resonance imaging (MRI) studies of a relatively large number of patients.

Methods

We retrospectively examined neuroimages of 12 patients (7 male, 5 female) diagnosed with DADA2. The mean age of the patients at the time of initial brain MRI was 16.7 ± 10.2 years. Seven patients (58.3%) fulfilled the classification criteria of polyarteritis nodosa. Brain MRI studies were assessed with respect to findings of ischemia, intracranial hemorrhages, focal parenchymal signal abnormalities, cerebral/cerebellar volume loss, and abnormal contrast enhancement. Angiographic studies of 7 patients were evaluated for the signs of vasculitis.

Results

The most frequent finding was acute and/or chronic lacunar ischemic lesions in the brainstem and/or deep gray matter (n = 9, 75%). Six patients (50%) revealed MRI findings compatible with recurrent ischemic attacks. Small nodular contrast enhancement (n = 2, 16.6%), acute putaminal hemorrhage (n = 1, 8.3%) and findings compatible with posterior reversible encephalopathy syndrome (n = 1, 8.3%) were also detected. Slight-to-moderate diffuse cerebral and/or cerebellar volume loss (n = 7, 58.3%), decreased T1 signal of the bone marrow (n = 6, 50%) and optic atrophy (n = 1, 8.3%) were the other findings on brain MRI. The only abnormal angiographic finding was reduced caliber of the right distal posterior cerebral artery in MRA of a patient (14.6%).

Conclusion

DADA2 should be included in the differential diagnosis of young patients presenting with ischemic and/or hemorrhagic lesions located in the brainstem and deep gray matter, especially if they have a family history or additional systemic abnormalities.  相似文献   
46.
INTRODUCTION Gastrointestinal hormones are secreted by endocrine cells which are distributed throughout the mucosa of the gastrointestinal tract. They play important roles in the overall regulation of digestive processes such as nutrient absorption, gut m…  相似文献   
47.
Human Parvovirus B19 has previously been implicated in the pathogenesis of testicular germ cell tumors, but this could not have been confirmed. This study was designed to investigate the testicular persistence of Parvovirus B19 and possible associations with germ cell tumors. Paraffin-embedded or fresh tissues from 36 germ cell tumors, 20 germ cell aplasias, 26 normal testicular tissues, 20 liver tissues, and 20 spleen tissues were evaluated by two different molecular assays: a nested PCR for Parvovirus B19 capsid genes and a commercial quantitative real-time PCR. Positive results were further confirmed by another commercial real-time PCR assay. Viral DNA was detected in 3 of 36 (8.3%) germ cell tumors, but not in other groups. Viral loads observed in all positive samples were less than 20 IU/reaction, suggesting very low levels of viral replication or latency. These results either directly or indirectly imply the involvement of Parvovirus B19 with testicular germ cell tumors. Viral persistence in normal testis, germ cell aplasia tissues, or hepatic/splenic tissues was not observed in this study.  相似文献   
48.
Single-port laparoscopic surgery has the advantage of a hidden scar and reduced abdominal wall trauma. Although single-port laparoscopic surgery is widely performed for other organs, its application is very limited for liver resection. Here, we report our experience with nine patients who underwent single-port laparoscopic liver resection. Nine patients underwent single-port laparoscopic liver resection for the indications of hydatid cyst, hepatocellular carcinoma, and colorectal cancer liver metastasis. Nine patients were successfully treated with single-port laparoscopic surgery. The operative time was between 60 and 240 min. The only operative complication was bleeding up to 650 mL in a patient with cirrhosis. No postoperative complications occurred. All patients were discharged earlier than usual. Single-port laparoscopic liver surgery is a challenging surgery. Surgeon with the experience of laparoscopic liver surgery should perform the single-port laparoscopic liver surgery. It is technically feasible with a good outcome in well-selected patients. Initial cases must be benign lesions to avoid jeopardizing oncological safety.  相似文献   
49.
Purpose: To determine factors affecting the survival of colorectal cancer patients developing liver metastasis.

Material and methods: 850 colorectal cancer patients who had been operated on between 1995 and 2005 were retrospectively reviewed and patients who developed liver metastases were selected. Patients’ demographics and data regarding the characteristics and management of primary tumors and liver metastases were assessed. Survival data were analyzed using Cox proportional hazard method to identify factors affecting prognosis of such patients. Results: 154 (18.1%) patients developed liver metastasis; however, only 60 patients (39 males and 21 females; median age: 61 (31–77) years) had complete follow-up data, of whom 28 had undergone metastasis resection. The median survival time of patients who underwent resection and who did not was 42 (22–118) vs. 13 (2–52) months (p < 0.001). Curative resection of the primary tumor (p = 0.32; Exp(B) = 2.93), metastases that were less than 5 in number and localized to one of the right or left lobes (p = 0.004; Exp(B) = 3.38), and surgical resection of the metastases (p = 0.001; Exp(B) = 3.76) were independent risk factors.

Conclusions: Patients with colorectal liver metastases that are less than 5 and confined into one lobe, could benefit from metastasis surgery most if the primary tumor is resected.  相似文献   
50.
In many countries currently, health-care professionals experience burnout in their professional life. This study explored the relationship between burnout, and role conflict and role ambiguity in nurses and physicians at a university hospital in Turkey. The data were collected by questionnaires that included sociodemographic variables, Maslach's Burnout Inventory (MBI), and Rizzo's Role Conflict and Role Ambiguity Scales. Two hundred and fifty one health-care professionals (170 physicians and 81 nurses) responded to the survey. There was a strong positive correlation between the MBI and Rizzo's Role Conflict and Role Ambiguity Scales. The nurses showed significantly higher levels of role conflict, role ambiguity, and burnout compared to the physicians. A multiple regression analysis showed that role conflict and role ambiguity might help to explain the higher level of burnout experienced by the nurses compared to the physicians. Future research is needed to develop preventive measures for the burnout of health-care professionals.  相似文献   
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