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1.
2.

Background

Membranoproliferative glomerulonephritis (MPGN) is an uncommon form of glomerulonephritis and it can be particularly difficult to predict outcomes and manage women with this disorder during pregnancy.

Materials and Methods

The management of 3 successful pregnancies in women with MPGN from 1 center and previously described cases from the world literature are reviewed. This includes a number of large studies of pregnancy in women with underlying glomerular disease as well as small case series and individual reports. Courses of these pregnancies, maternal and fetal outcomes, and management, when described, were included in this review.

Results

Some successful outcomes used antiplatelet therapy and plasmapheresis, but high-dose intravenous, followed by oral, corticosteroid therapy was used most frequently in patients with successful outcomes.

Conclusions

The data provided is meant as a guide for clinicians who provide care for women with MPGN who are considering pregnancy or women who present with this disorder while pregnant.  相似文献   

3.

Background

Pulmonary fibrosis (PF) is a destructive pulmonary disease and the molecular mechanisms underlying PF are unclear. This study investigated differentially expressed proteins associated with the occurrence and development of PF in rat lung tissue with bleomycin-induced PF.

Methods

Sixteen Sprague-Dawley rats were randomly divided into 2 groups: the PF model group (n = 8) and the control group (n = 8). After successfully establishing the rat PF model induced by bleomycin, the differentially expressed proteins in the 2 groups were identified through isobaric tag for relative and absolute quantitation coupled with liquid chromatography-mass spectrometry and bioinformatics analysis.

Results

A total of 146 differentially expressed proteins were identified; 88 of which displayed increased abundance and 58 were downregulated in the PF rat model group. Most functional proteins were associated with extracellular matrix, inflammation, damage response, vitamin A synthesis and metabolism. Critical proteins related to PF development and progression was identified, such as type V collagen-3, arachidonic acid 12-lipoxygenase, arachidonic acid 15-lipoxygenase and cytochrome P4501A1. Kyoto Encyclopedia of Genes and Genomes pathway analysis showed that these differentially expressed proteins were enriched in extracellular matrix receptor interaction pathway, renin-angiotensin system and metabolic pathway of retinol.

Conclusions

The proteins expressed in bleomycin-induced PF rat model provide important data for further functional analysis of proteins involved in PF.  相似文献   

4.

Introduction

Parathyromatosis is a rare cause of recurrent hyperparathyroidism. It results from hyperfunctioning parathyroid tissue scattered throughout the thyroid bed region.

Case report

A 51-year-old man with a history of parathyroidectomy, presented 18 years later with recurrent primary hyperparathyroidism. Surgical exploration identified a single parathyroid gland. The act was completed by a central compartment dissection and ipsilateral lobectomy. The patient was free of recurrence after a one-year follow-up.

Conclusion

Parathyromatosis a rare cause of recurrent hyperparathyroidism. Its management is challenging. Extensive surgery is required with clearance of the central neck compartment and homolateral lobectomy. Medical therapy could be used to decrease parathormone level in recurrent parathyromatosis.  相似文献   

5.

Objective

Hepatic fibrosis is characterized by the activation of hepatic stellate cells (HSCs). Focal adhesion kinase (FAK)-phosphatidylinositol 3-kinase (PI3K) signals participate in the activation of HSCs. We evaluated the effect of FAK-related nonkinase (FRNK) on the adhesion and migration of HSCs.

Materials and Methods

Hepatic fibrosis was induced in Sprague-Dawley rats by means of bile duct ligation. Livers were harvested at 1, 2, 3 and 4 weeks after the ligation; livers of sham-operated animals were harvested at 4 weeks after ligation. Histopathologic features were evaluated in liver sections stained with hematoxylin-eosin and Sirius Red stain. HSCs were transfected with FRNK plasmid. The adhesion of HSCs was examined with a toluidine blue colorimetric assay. The migration of HSCs was evaluated by the use of an improved Boyden double-chamber method. Protein and messenger RNA levels in the liver and the HSCs were determined by Western blot analysis and real-time polymerase chain reaction, respectively.

Results

Hematoxylin-eosin staining of the liver documented the presence of fibrosis in the rats. Actin and PI3K expression was increased in parallel with the development of hepatic fibrosis. At the same time, activator protein-1 (AP-1) (c-fos, c-jun) mRNA in the livers was increased. Overexpression of FRNK inhibited the adhesion and migration of HSCs time-dependently. Simultaneously, FRNK inhibited PI3K mRNA and protein expression and c-jun mRNA expression.

Conclusions

FRNK inhibited the adhesion and migration of HSCs by decreasing the expressions of the FAK-PI3K-AP-1 signal pathway.  相似文献   

6.

Background

Although nontuberculous mycobacteria (NTM) lung diseases can occur in association with lung cancer, no study has evaluated the effect of lung cancer treatment on NTM lung diseases. Therefore, the present study aimed to retrospectively examine the effect of lung cancer treatment on NTM lung diseases.

Methods

Patients diagnosed with NTM lung diseases in combination with cytologically or histologically proven lung cancer between January 1, 2010 and October 31, 2014 were enroled. The clinical history of eligible patients was retrospectively reviewed.

Results

Seven hundred twenty-eight patients were diagnosed with NTM lung diseases. Among these patients, 29 (3.9%) also had lung cancer. Of the 29 patients with NTM and lung cancer, 62% had Mycobacterium avium complex as the pathogenic organism. The most common lung cancer histology was adenocarcinoma (62.1%). Anti-cancer cytotoxic chemotherapy was administered to seven patients, and the two patients who did not receive NTM treatment showed worsening of their NTM lung disease.

Conclusion

Whether NTM lung disease should be treated during anti-cancer chemotherapy has not been not clarified by this study. Induction of anti-NTM therapy should be made after careful consideration, because the duration of anti-NTM treatment is long and anti-mycobacterial drugs have extensive effects on anti-cancer drugs. However, we think that anti-NTM therapy should be introduced after consideration of the worsening of symptoms and radiological findings associated with NTM lung disease.  相似文献   

7.

Background

Primary headaches include migraines, tension-type headaches and other primary headache syndromes. Migraines and tension-type headaches are associated with patient discomfort and other diseases. This study aimed to investigate the association between primary headaches and the risk of developing dementia, and to clarify the association between different types of headaches and dementia.

Materials and Methods

We conducted a nationwide matched cohort population-based study. A total of 3,620 patients with newly diagnosed primary headaches, including migraines and tension-type headaches, between January 1 and December 31, 2000 were selected from the National Health Insurance Research Database of Taiwan, along with 10,860 controls matched for sex and age. After adjusting for confounding factors, Fine and Gray?s competing risk analysis was used to compare the risk of developing dementia during 10 years of follow-up.

Results

Of the study subjects, 170 (4.70 %) developed dementia compared with 433 (3.99%) of the controls. Fine and Gray?s competing risk analysis revealed that the study subjects were more likely to develop dementia (hazard ratio = 2.057; 95% CI: 1.718-2.462; P < 0.001). After adjusting for sex, age, monthly income, urbanization level, geographic region of residence and comorbidities, the hazard ratio for dementia was 2.048 (95% CI: 1.705-2.461, P < 0.001). Migraines and tension-type headaches were associated with nonvascular dementia but not vascular dementia.

Conclusions

The patients with headaches had a 105% increased risk of dementia. Further studies are needed to elucidate the underlying mechanisms.  相似文献   

8.
9.

Background

Organizing pneumonia (OP) is a histopathological response pattern to lung inflammation. It is clinically classified into cryptogenic OP and secondary OP, which is associated with various clinical conditions. Rapid resolution with corticosteroids and frequent relapses are common in OP. However, few studies have investigated the factors associated with OP relapse.

Methods

The medical records of 75 patients with biopsy-proven OP, diagnosed between January 2010 and August 2015, who underwent corticosteroid therapy were retrospectively reviewed. Initially, the patients were all treated successfully; however, 31 patients experienced relapse thereafter (R group), whereas the others did not (NR group; 44 patients). The clinical, radiological, and pathological characteristics and administered corticosteroid doses were compared between the two groups.

Results

The neutrophil percentage in the bronchoalveolar lavage (BAL) fluid and the level of fibrin deposition in lung biopsy specimens were higher in the R group than in the NR group (P=0.01 and P=0.002, respectively). The multivariate analysis demonstrated that both factors were statistically significant predictors of OP relapse.

Conclusions

A high neutrophil percentage in the BAL and the level of fibrin deposition in lung biopsy specimens are considered predictive factors of OP relapse during the tapering or after the cessation of steroid therapy. Patients without these findings may be treated with low-dose corticosteroids.  相似文献   

10.

Background

Hepatitis C virus (HCV) infection is a common susceptibility factor for porphyria cutanea tarda (PCT). Experience on HCV treatment in patients with PCT is limited. Recently, HCV treatment has improved with direct-acting antivirals (DAA). We review our experience on HCV treatment in patients with PCT with older and newer regimens.

Materials and Methods

A retrospective chart review was conducted. HCV treatment was attempted 22 times in 13 patients with PCT (5 attempts in 1, 2 in 5 and 1 in the other 7 patients).

Results

Before starting HCV treatment, PCT was in complete remission in 16, partial remission in 2, unknown status in 2 and active in 2 instances. PCT relapsed during therapy 6 times (all interferon-based regimens and 2 including telaprevir), 4 requiring treatment interruption. Treatment was interrupted for reasons other than PCT relapse in 2 patients treated with interferon-based regimens. To prevent PCT recurrence, hydroxychloroquine was continued during HCV therapy 6 times (3 interferon regimens, 2 ribavirin regimens without interferon and 1 DAA alone). Twelve patients achieved sustained viral response, 3 with interferon regimens and 9 with DAA. Two patients with active PCT were treated with DAA, with reduction of plasma porphyrins in 1 and normalization in the other at the end of HCV therapy.

Conclusions

HCV treatment regimens including interferon or ribavirin may precipitate PCT relapse. Hydroxychloroquine may be useful to prevent such relapses. In this limited experience, DAA were not associated with PCT relapse. Studies are needed to examine DAA as a primary PCT treatment in HCV-infected patients.  相似文献   

11.

Background

Dyslipidemia is a risk factor for nonalcoholic fatty liver disease (NAFLD). The aim of our study was to determine the associations of serum lipid indexes with NAFLD in adult males.

Materials and Methods

In this cross-sectional study, 830 patients with NAFLD and 2,357 healthy individuals were assessed. Serum total cholesterol (TC), triglyceride (TG), high-density lipoprotein cholesterol and low-density lipoprotein cholesterol (LDL-C) levels were compared between patients with NAFLD and controls. The associations of dyslipidemia indexes with NAFLD occurrence were assessed by univariate analysis, and multivariate analysis was performed to identify independent dyslipidemia factors predictive of NAFLD.

Results

Of the 3,187 study subjects, NAFLD occurred in 830 (26.04%), there were 504 (60.72%) patients with mild disease and 326 (39.28%) patients with moderate-to-severe disease. Although the frequency of normal TC, TG, LDL-C and high-density lipoprotein cholesterol levels in patients with NAFLD was similar to the controls, the frequencies of patients with NAFLD with marginally high and high TC, TG and LDL-C levels were significantly different when compared with controls. Interestingly, the association of the number of abnormal serum lipid indexes and NAFLD was highly significant with 2 abnormalities (odds ratio = 1.977; 95% CI: 1.436-2.722; P < 0.001) and ≥3 abnormalities (odds ratio = 3.505; 95% CI: 2.466-4.982; P < 0.001).

Conclusions

A significant positive association was found between dyslipidemia characteristics and NAFLD in adult males.  相似文献   

12.

Background

Increased oxidative stress (OS) and lipid peroxidation may be involved in the pathogenesis of preeclampsia (PE). We conducted a case-control study to evaluate the levels of plasma lipids and trace elements as well as activity of superoxide dismutase (SOD) in PE.

Materials and Methods

The study consisted of 100 patients who had been diagnosed with PE and 100 normotensive pregnant women who underwent medical checkups that served as the control group. Lipid profile, zinc (Zn) and copper (Cu) levels and SOD activities were measured in the plasma of all subjects.

Results

Our results showed that the plasma levels of triglycerides and SOD activity were significantly elevated and the levels of Zn and Cu were significantly reduced in patients with PE compared with healthy controls. Increased levels of SOD may indicate antioxidant protective mechanisms against OS in PE-complicated pregnancies. This finding may suggest an involvement of OS in the pathophysiology of PE.

Conclusion

This study demonstrated a significant negative correlation between SOD activity and levels of trace elements. Furthermore, we suggest that higher triglyceride levels and SOD activity combined with lower Zn and Cu levels may be associated with an increased risk of PE.  相似文献   

13.

Background

Aortic dissection (AD) is a rare complication of Takayasu arteritis (TA). The clinical presentation and long-term management outcomes of AD in patients with TA have not been well described.

Materials and Methods

We conducted a retrospective study of patients with TA along with AD admitted to Fuwai Hospital between January 1985 and March 2016. Clinical data and follow-up data were collected and analyzed.

Results

Of the 1,154 patients with TA, we identified 10 patients (0.87%) with AD, which was likely to be associated with vasculitis. All patients were females with a median age at TA onset of 26.5 years (range: 18.3-33.3 years), had type III TA and had a history of hypertension, which was much more common than that in previously reported cases. Stanford type B or DeBakey category III was the dominant anatomic classification of AD. Four patients developed AD after the diagnosis of TA, and 6 developed AD near the time of TA diagnosis. Nine patients underwent conservative treatment, whereas 1 patient underwent endovascular repair due to extensive dissection. At a median 70.5-month follow-up (range: 31.5-138.5), we found that 7 patients had no AD progression, 1 patient had progressed without symptoms, 1 patient was lost and 1 patient died.

Conclusions

Patients with TA along with long-standing and poorly controlled hypertension are liable to develop AD. Those with extensive AD in TA should be carefully treated and intensively followed up.  相似文献   

14.
15.

Background

Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population.

Method

Demographics, comorbidities, symptoms, family histories, physical examination and laboratory findings were reviewed in 298 retrospective and 115 prospective patients with MCAS. Blood samples from prospective subjects were examined by flow cytometry for clonal mast cell disease and tested for cytokines potentially driving the monocytosis frequent in MCAS.

Results

Demographically, white females dominated. Median ages at symptom onset and diagnosis were 9 and 49 years, respectively (range: 0-88 and 16-92, respectively) and median time from symptom onset to diagnosis was 30 years (range: 1-85). Median numbers of comorbidities, symptoms, and family medical issues were 11, 20, and 4, respectively (range: 1-66, 2-84, and 0-33, respectively). Gastroesophageal reflux, fatigue and dermatographism were the most common comorbidity, symptom and examination finding. Abnormalities in routine laboratories were common and diverse but typically modest. The most useful diagnostic markers were heparin, prostaglandin D2, histamine and chromogranin A. Flow cytometric and cytokine assessments were unhelpful.

Conclusions

Our study highlights MCAS?s morbidity burden and challenging heterogeneity. Recognition is important given good survival and treatment prospects.  相似文献   

16.

Background

We sought to establish the characteristics of symptomatic nontyphoidal Salmonella (NTS) urinary tract infection (UTI) without concomitant gastroenteritis (GE) as a separate clinical entity.

Materials and Methods

We conducted a retrospective cohort single-center study and reviewed all cases of NTS bacteriuria between 1995 and 2016. Patients were assigned to a group according to their clinical presentation, namely, symptomatic NTS UTI without GE, GE with NTS bacteriuria or isolated asymptomatic NTS bacteriuria. We compared the characteristics of patients in the NTS UTI group to those of the latter 2 groups.

Results

NTS bacteriuria was found in 77 patients, of which 61 had records available for review. Twenty-one patients (including 17 adults) presented with NTS UTI, 30 patients presented with features of GE with NTS bacteriuria and 10 patients had asymptomatic NTS bacteriuria. NTS UTI was not significantly associated with older age, male sex, diabetes, immunosuppressive states or urologic abnormalities. There was a significant difference in the proportion of patients with an underlying urologic malignancy in the NTS UTI group (4 of 17 patients [23.5%]) as compared to those in the other groups (0 of 24 patients), P = 0.023.

Conclusions

A unique group of patients with symptomatic NTS UTI without GE was identified. A significant association with urologic malignancies was demonstrated in patients with NTS UTI compared to those with GE and NTS bacteriuria or asymptomatic NTS bacteriuria.  相似文献   

17.

Background

Percutaneous coronary intervention (PCI) has been the main therapy in acute coronary syndromes, and early antithrombotic agents as well as 1-year dual antiplatelet therapy are required for adjuvant therapy. However, the development of post-PCI gastrointestinal (GI) bleeding may increase all-cause mortality. We compared the characteristics and outcomes of patients with GI bleeding within 1 year after PCI to those who did not develop bleeding.

Methods

A retrospective cohort study was conducted using data from 384 PCI procedures performed between January 2011 and December 2013 at our hospital. End points were identified after 30 days, 90 days and 1 year postprocedure for evidence of GI bleeding or new onset anemia. Variables were compared between patients with and without GI bleeding using t test and Fisher exact test. Kaplan-Meier curve was constructed for estimating bleeding-free survival probability.

Results

In a more than 1-year follow-up period, there were 39 cases (10.2%, 95% CI: 0.073-0.136) of documented GI bleeds. Females were found to have a significantly higher frequency of GI bleeding than males (16.8% vs. 8.0%, P = 0.018), and Hispanics more than non-Hispanics (11.7% vs. 1.7%, P = 0.017). All patients with GI bleeding survived at 1 year.

Conclusion

In our study of a predominantly Hispanic population, a high incidence of GI bleeding after PCI occurred. However, there was no association between the incidence of GI bleeding and all-cause mortality, whether PCI was performed in the setting of acute coronary syndrome or as an elective procedure. There is a need to conduct a larger prospective study to validate the findings of our study.  相似文献   

18.

Background

Fanconi anemia (FA) is a condition characterized by genetic instability and short stature, which is due to growth hormone (GH) deficiency in most cases. However, no apparent relationships have been identified between FA complementation group genes and GH. In this study, we thereby considered an association between FA and Laron syndrome (LS) (insulin-like growth factor 1 [IGF-1] deficiency).

Methods

A 21-year-old female Mexican patient with a genetic diagnosis of FA was referred to our research department for an evaluation of her short stature. Upon admission to our facility, her phenotype led to a suspicion of LS; accordingly, serum levels of IGF-1 and IGF binding protein 3 were analyzed and a GH stimulation test was performed. In addition, we used a next-generation sequencing approach for a molecular evaluation of FA disease-causing mutations and genes involved in the GH-IGF signaling pathway.

Results

Tests revealed low levels of IGF-1 and IGF binding protein 3 that remained within normal ranges, as well as a lack of response to GH stimulation. Sequencing confirmed a defect in the GH receptor signaling pathway.

Conclusions

To the best of our knowledge, this study is the first to suggest an association between FA and LS. We propose that IGF-1 administration might improve some FA complications and functions based upon IGF-1 beneficial actions observed in animal, cell and indirect clinical models: erythropoiesis modulation, immune function improvement and metabolic regulation.  相似文献   

19.

Background

Field walk tests such as the incremental shuttle walk test (ISWT) are simple tests for assessing the degree of disability in individuals with chronic obstructive pulmonary disease (COPD). In the present study, the correlations between exercise performance in the ISWT, lung function, and health status were examined in patients with COPD of varying severities.

Methods

A retrospective examination of 277 COPD patients was performed using the ISWT and lung function tests along with assessment of health status using St. George?s Respiratory Questionnaire (SGRQ). In addition, we assessed the correlations between the walking distance, lung function parameters, and SGRQ scores.

Results

ISWT distances were poorly correlated with lung function parameters and SGRQ scores in mild COPD patients. In contrast, ISWT distances were significantly correlated with pulmonary function parameters, such as vital capacity (%predicted) and forced expiratory volume in one second, and SGRQ scores in moderate and severe COPD patients.

Conclusions

The ISWT is more independent of health status and pulmonary function in patients with mild COPD compared to moderate or severe cases. Therefore, the exercise capacity of patients with mild COPD should be estimated by the ISWT.  相似文献   

20.

Background

Useful biomarkers that can serve as prognostic predictors are of great value in clinical practice because of the complex individual response to sepsis. Pentraxin 3 (PTX3), as a multifunctional pattern-recognition molecule, has been reported to be closely associated with the severity of infectious diseases in intensive care units (ICU). The aim of this study was to investigate whether PTX3 could serve as a potential prognostic biomarker in patients with septic shock.

Materials and Methods

This single-center prospective observational study was conducted during May 2012-May 2015 in the ICU of Taizhou People?s Hospital. We compared the clinical data and laboratory tests in surviving and deceased patients with septic shock within 28 days from admission. Potential independent prognostic factors for septic shock were analyzed by using univariate and multiple Cox proportional hazards regression analyses.

Results

A total of 112 patients admitted to the ICU with septic shock were enrolled in our study with an overall 28-day mortality of 25.9% (29 of 112 patients). PTX3 level was the only independent risk factor for the 28-day mortality by univariate and multivariate Cox analysis (hazard ratio = 3.87; 95% CI: 1.66-8.81, P = 0.004). The deceased patients had significant higher levels of PTX3 at the 4 different points (baseline, day 1, day 2 and day 3) versus the survivors (P < 0.001). Results from Kaplan-Meier curves and log-rank test revealed that high PTX3 level (above the median value) was statistically associated with a lower 28-day survival rate (P = 0.014).

Conclusions

The baseline PTX3 level was an independent predictor for 28-day mortality in patients with septic shock.  相似文献   

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