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1.

BACKGROUND

Pemphigusis a bullous, rare and chronic autoimmune disease. There are two major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and clinical outcome in patients diagnosed in the Brazilian Amazon states are still rare.

OBJECTIVES

To study the occurrence of the disease during the study period and analyze the epidemiological profile of patients, the most common subtype of pemphigus, and the clinical evolution of patients.

METHODS

Retrospective analysis of medical records of hospitalized patients with pemphigus foliaceus and pemphigus vulgaris in the period from 2003 to 2010 in Dermatology Service of Hospital Fundação Santa Casa de Misericórdia do Pará, Belém, Northern Brazil.

RESULTS

We found a total of 20 cases of pemphigus during the study period, 8 of which were of foliaceus pemphigus and 12 of vulgaris pemphigus. Pemphigus foliaceus had the predominance of male patients (75%), showed satisfactory clinical evolution, and was characterized by absence of pediatric cases. Pemphigus vulgaris affected more women (66.7%), showed mean hospital stay of 1 to 3 months (50%), and there were three cases of death (25%). The prescribed immunosuppressive drugs included prednisone with or without combination of azathioprine and/or dapsone. Sepsis was associated with 100% of the deaths.

CONCLUSIONS

The occurrence of the disease is rare, there are no familiar/endemic outbreaks in the sample. Evolution is usually favorable, but secondary infection is associated with worse prognosis. The choice of best drugs to treat pemphigus remains controversial.  相似文献   

2.

BACKGROUND

Pemphigus foliaceus and pemphigus vulgaris are endemic in the northeastern region of São Paulo State, Brazil. They are treated mainly with systemic corticosteroids, which may provoke osteoporosis; atherosclerosis, higher blood pressure, insulin resistance, glucose intolerance, hyperlipidemia and abdominal obesity. These side effects of corticoids also constitute criteria for the diagnosis of metabolic syndrome.

OBJECTIVE

The prevalence of metabolic syndrome and each component of metabolic syndrome in Pemphigus foliaceus and pemphigus vulgaris groups was compared with Brazilian casuistic samples.

METHODS

Data of 147 patients (pemphigus foliaceus 48.9% and pemphigus vulgaris 51.1%) were compiled from medical records regarding metabolic syndrome and its components, and included in the analysis.

RESULTS

There was no significant difference regarding the prevalence of metabolic syndrome in pemphigus groups compared with the Brazilian casuistic samples. The analysis of each component of metabolic syndrome showed a higher prevalence of: higher blood pressure in male subjects with pemphigus vulgaris, and in pemphigus foliaceus in both genders; diabetes mellitus in both genders for pemphigus vulgaris and pemphigus foliaceus; obesity in females for pemphigus vulgaris and pemphigus foliaceus, and hypertriglyceridemia in both genders for pemphigus vulgaris and pemphigus foliaceus groups that were statistically significant compared to the Brazilian reports. Furthermore, the study noted a higher incidence of cardiovascular events in both genders in pemphigus foliaceus and pemphigus vulgaris groups than in Brazilian casuistic samples.

CONCLUSION

The components of metabolic syndrome are more numerous in pemphigus when compared with Brazilian casuistic samples. Future studies are necessary to assure that metabolic syndrome may be associated with pemphigus per se, including a greater casuistic sample of patients who have not taken corticoids.  相似文献   

3.

BACKGROUND

Trichoscopy is becoming increasingly popular in diagnosing hair and scalp diseases. Scalp involvement in pemphigus is common. The scalp may be the first or only site of clinical manifestation of the disease.

OBJECTIVE

The aim of this study was to analyze whether trichoscopy may be useful in aiding differential diagnosis of scalp lesions in patients with pemphigus vulgaris and pemphigus foliaceus.

METHODS

Trichoscopy was performed in 19 patients with scalp lesions in the course of pemphigus (9 patients with pemphigus vulgaris and 10 with pemphigus foliaceus). In all patients, the diagnosis of scalp pemphigus was confirmed by histopathology. The working magnification was 20-fold and 70-fold.

RESULTS

The most frequently observed trichoscopy features of pemphigus lesions were: extravasations (18/19; 94.7%) and yellow hemorrhagic crusts (11/19; 57.9%). Yellow dots with whitish halo were observed in 6/19 (31.6%) patients with pemphigus. White polygonal structures were observed in pemphigus foliaceus (6/10; 60%), but not in pemphigus vulgaris. Vascular abnormalities were more frequent in pemphigus vulgaris, when compared to pemphigus foliaceus, and were associated with a severe course of disease. Linear serpentine vessels were the most frequent vascular abnormality in patients with pemphigus vulgaris and pemphigus foliaceus (77.8% and 30%, respectively).

CONCLUSION

Trichoscopy may serve as a useful supplementary method in the differential diagnosis of pemphigus, especially in cases of desquamative or exudative lesions limited to the scalp. Extravasations, yellow hemorrhagic crusts, yellow dots with whitish halo, white polygonal structures and linear serpentine vessels are trichoscopy features which may suggest the diagnosis of pemphigus.  相似文献   

4.

Background

Measuring the quality of life (QOL) is important in the evaluation of nonclinical aspects of diseases, for the discovery of functional and psychological limitations, and in choosing treatment in the initial phase of the disease. Pemphigus is a potentially fatal autoimmune bullous disease caused by autoantibodies against desmogleins (cadherin family proteins in desmosomes). Thus far, there has been no published study on QOL in Korean patients with pemphigus.

Objective

To study the impact of pemphigus on the QOL in a large number of Korean patients.

Methods

Sixty-six patients enrolled at the Gangnam Severance Hospital from March 2012 to March 2013 were assessed for QOL by using the Dermatology Life Quality Index (DLQI), and for anxiety and depression by using the General Health Questionnaire (GHQ). Spearman''s rank-order correlation, t-test, and ANOVA were used to identify the relations between the DLQI score and other clinical variables.

Results

Pemphigus vulgaris and pemphigus foliaceus significantly reduced the QOL of patients. The average DLQI score for all patients was 10.18. The mean DLQI score was 13.45 in patients in the active state and 5.15 in the patients in the remission state. The DLQI score highly correlated with disease severity, titer of anti-desmoglein 1 in enzyme-linked immunosorbent assay, and the corticosteroid dose. However, the QOL was not affected by sex, age, subtype of pemphigus, duration of disease, or comorbidities. Forty-two percent of the patients showed a positive result in the GHQ, reflecting probable minor psychiatric nonpsychotic conditions, and the GHQ score positively correlated to the DLQI score.

Conclusion

Pemphigus significantly impairs the QOL of patients. The QOL of Korean pemphigus patients significantly correlates with clinical severity. Therefore, considerable attention should be paid to the patients'' QOL and psychological states as well as clinical status.  相似文献   

5.

BACKGROUND

Previous studies have shown that some viral infections may be triggers for autoimmune diseases. The role of viral infections in the etiopathogenesis of pemphigus has also been investigated.

OBJECTIVES

To investigate the relationship between pemphigus and the hepatitis B and C virus infections.

METHODS

This retrospective study included 62 patients with pemphigus and 50 healthy controls of matching ages and genders. The control group included relatives who accompanied the orthopedic patients to the hospital but had no history of systemic and/or autoimmune diseases.

RESULTS

The group of patients with pemphigus was composed of 43 (69.3%) females and 19 (29.7%) males, and the mean age was 48.08 ± 15.38. The hepatitis C virus antibody was negative in all of the patients. Hepatitis B surface antigen (HBsAg), hepatitis B surface antibody (HBsAb), or both, were studied in 44 of the 62 patients. Two (4.3%) of these patients were positive for HBsAg and found to have HBV infection. Fourteen of them were HBsAb positive. Two (4%) of the 50 control subjects showed hepatitis C virus antibody positivity, while only 1 (2%) patient with pemphigus displayed HBsAg positivity. There was no statistically significant difference between the two groups for hepatitis B and C virus infections.

CONCLUSIONS

This study does not support an association between pemphigus and hepatitis B and/or C virus infections.  相似文献   

6.

BACKGROUND

Autoimmune bullous dermatoses are complex diseases triggered by autoantibodies action against epidermal antigens or the dermoepidermal junction. Blisters and vesicles that evolve with erosion areas characterize them. Although rare, they present high morbidity, affecting the quality of life of patients.

OBJECTIVES

To assess the magnitude of autoimmune bullous dermatoses on life quality of patients treated in a public university service in countryside of Brazil.

METHODS

This cross-sectional study was based on an inquiry with autoimmune bullous dermatoses patients assisted at outpatient university referral service. Elements related to quality of life were evaluated by the Dermatology Life Quality Index, as well as clinical and demographic data.

RESULTS

The study evaluated 43 patients with pemphigus foliaceus, 32 with pemphigus vulgaris, 6 with bullous pemphigoid and 3 with dermatitis herpetiformis. The average age was 48 ± 16 years and 34 (40%) were female. The median score (p25-p75) of the Dermatology Life Quality Index was 16 (9-19), classified as "severe impairment" of life quality, in which the greater impact was related to symptoms and feelings, daily and leisure activities.

CONCLUSIONS

Autoimmune bullous dermatoses inflict severe impairment of quality of life for patients followed by a public outpatient clinic in the countryside of Brazil.  相似文献   

7.
8.

Background:

In pemphigus, autoantibodies are directed against adhesion molecules, which make the junctions between keratinocytes, and thus determining their level can reflect the disease activity.

Aim:

The purpose of this study is to determine the clinical significance of the autoantibody levels in pemphigus management.

Materials and Methods:

The clinical features of 47 pemphigus vulgaris patients were assessed and patients′ sera were investigated by indirect immunofluorescence using monkey esophagus as a substrate for autoantibody levels.

Results:

We found a significant correlation between antibody titers and mucosal severity scores. Initial antibody titers of the patients with at least one mucosal lesion at the end of the first month of the therapy were found significantly higher than the patients who had no mucosal lesion. With the therapy, lesions resolved earlier than the antibody titers.

Conclusion:

In patients with pemphigus, especially in cases who were not treated before, sera antibody levels are a valuable tool in evaluating disease severity and choosing initial treatment. In patients who had been taking any systemic treatment, it is difficult to make a relationship between antibody levels and disease severity, because therapy improves disease earlier than the antibody titers. However, estimating antibody levels can be helpful for clinicians in disease management, in reducing or ceasing treatment dosage and anticipating recurrence.  相似文献   

9.

Background

Although intralesional methotrexate (MTX) is an effective, nonsurgical treatment of keratoacanthoma (KA), there have not been many reports of on the MTX treatment for KA in Korea.

Objective

The purpose of this study was to evaluate the clinical efficacy of the intralesional MTX for the treatment of KA in Korean patients.

Methods

We retrospectively studied seven patients with KA who received intralesional injection of MTX in our department. The efficacy was evaluated based on the physician assessment. Our review also included the cases of KA treated with intralesional MTX in Korean patients from the previous reports. We then analyzed the therapeutic regimens in the Korean patients by comparing them with the Caucasian patients.

Results

We identified 11 cases of Korean KA patients treated with an intralesional MTX, including seven from our institution and four from the Korean literature. Ten of the 11 patients (91%) showed a complete resolution with an intralesional MTX. No adverse events were observed during the treatment and the follow-up periods. No recurrence was found during the follow-up. In therapeutic analysis, the Korean patients required 2 to 7 injections (mean 4.6 injections) to achieve a tumor resolution with the mean time to clearing at 7.6 weeks.

Conclusion

Intralesional MTX can be an effective and safe non-operative treatment modality for most Koreans with KA.  相似文献   

10.

BACKGROUND

Immunofluorescence testing is an important tool for diagnosing blistering diseases.

OBJECTIVE

To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases.

METHODS

We retrospectively analyzed immunofluorescence results encompassing a 10-year period.

RESULTS

421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate.

CONCLUSION

Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect immunofluorescence when Salt-split skin technique is performed.  相似文献   

11.

Background

Acne vulgaris is the most common skin disease worldwide, with many available treatment modalities, including oral and topical medications and laser therapy. Recently, a novel device (Isolaz, Pleasanton, CA, USA) that combines vacuum pressure and a broadband light source (400 nm to 1,200 nm) was developed for the treatment of acne.

Objective

To determine the clinical efficacy and safety of photopneumatic therapy for the treatment of acne vulgaris of the face.

Methods

Twenty adults with mild to moderate facial acne vulgaris received 4 successive treatments on one side of the face with a combined photopneumatic device (intense pulsed light: fluence=5.8 J/cm2; negative pressure=iMP mode) at 2 week intervals. Acne lesions on the opposite side of the face were not treated. Lesion counts were performed at baseline, prior to each treatment session, and at 3 months after the final treatment session.

Results

Significant lesion improvements and reduced numbers of acne lesions were observed on the treated side of the faces. Most patients experienced global clinical improvement. No severe side effects occurred during the study, with only a few patients experiencing transient erythema, purpura and/or exacerbation of pre-existing acne.

Conclusion

Photopneumatic therapy is a safe and effective treatment for mild to moderate acne vulgaris.  相似文献   

12.

Background

Perioral dermatitis (POD) is a common inflammatory skin disease without standard therapy.

Objective

We sought to evaluate the clinical value of a soothing fluid for the treatment of POD.

Methods

We included 51 patients with POD in this 8-week clinical trial. The Toleriane Fluide Efficacy in Perioral Dermatitis (TOLPOD) study had an open-label design and involved twice-daily application of Toleriane Fluide, a soothing cosmetic fluid. Clinical assessment of POD was performed with a predefined questionnaire including the POD severity index (PODSI). Control visits were made after 4 and 8 weeks of treatment.

Results

The results were compared with those of a historical control group treated with a vehicle cream. Patients treated with the soothing fluid showed a continuous and significant improvement of the PODSI over time. The improvement of PODSI observed with the soothing fluid was better, but not significantly better, than that observed in the historical controls. In addition, the subjective complaints of patients such as disease burden, itching, distension of the skin, and appearance improved during treatment.

Conclusion

A soothing fluid could be a clinically useful treatment option for POD.  相似文献   

13.

Background

The regional lymph nodal status is the most powerful independent predictor of survival for patients with clinical N0 primary cutaneous malignant melanoma.

Objective

We wanted to evaluate the feasibility and morbidity of the sentinel lymph node biopsy (SLNB) staging using a multidisciplinary team approach, in cooperation with other surgical departments, at a university hospital setting.

Methods

Twenty two patients with cutaneous melanoma and who were treated at Kyungpook National University Hospital were included in this study. They all received SLNB, which was done by the Departments of Dermatology and General Surgery. We evaluated the feasibility and side effects of SLNB.

Results

Pathologically-positive sentinel nodes were found in 7 of the 22 cases (31.8%) and all 7 patients were consequently upstaged. The whole process involved in SLNB was well tolerated by nearly all the patients, with only mild and transient complications being observed.

Conclusion

We suggest that in a Korean setting, utilizing SLNB with a multi-disciplinary team approach is a technically feasible procedure that is able to detect occult nodal metastasis with low morbidity rates in patients with cutaneous malignant melanoma.  相似文献   

14.

Background

Female patients with atopic dermatitis (AD) often experience cutaneous deterioration associated with their pregnancy or menstrual cycle.

Objective

We wanted to determine the prevalence of symptom aggravation as related to pregnancy and the menstrual cycle in female patients with AD.

Methods

One hundred female patients with AD were included in the study and interviews were performed. The total IgE level and the Eczema Area and Severity Index score of the patients were retrospectively reviewed.

Results

Ninety seven patients replied the questionnaire, and among them, 23 patients had completed at least 1 pregnancy. Among the 23 women who experienced pregnancy, 14 (61%) had noticed deterioration of their clinical symptoms during pregnancy. Of the 97 females, 31 (32%) patients had noticed deterioration of their AD as related to their menstrual cycle. For the patients who were sub-grouped as the intrinsic type of AD, the prevalence of symptom aggravation as related to pregnancy was significantly higher as compared to that of the extrinsic type of AD patients (p=0.048).

Conclusion

Of the 97 patients, 45 (46%) females answered that they have experienced deterioration of AD during pregnancy or in relation to their menstrual cycle, and this suggests the relation of a hormonal influence on the clinical manifestations of AD.  相似文献   

15.

Background

Extramammary Paget disease (EMPD) is an uncommon malignant neoplasm affecting apocrine gland-bearing skin which usually occurs in the anogenital area of patients older than 50 years. Although Mohs micrographic surgery (MMS) is recommended for the treatment of EMPD, wide local excision has also been performed by many other surgeons including dermatosurgeons. However, the extent of an adequate resection margin is still under debate.

Objective

The efficacy of minimal surgical therapy consisting of a wide excision combined with preoperative multiple scouting biopsies and postoperative topical imiquimod was investigated for the treatment of EMPD in Korean patients.

Methods

Between 2006 and 2012, 10 patients with primary EMPD were treated with wide surgical excision, with a surgical margin of less than 2.5 cm. Multiple preoperative scouting biopsies and postoperative topical imiquimod were also performed to delineate the lesional boundaries and to reduce the recurrence rate.

Results

During the 6-year follow-up period, complications and recurrences were not observed.

Conclusion

Minimal surgical therapy may be an effective alternative when MMS is unavailable.  相似文献   

16.

BACKGROUND

aAlthough rare, bullous pemphigoid (BP) is the most common autoimmune blistering disease. Recent studies have shown that patients with bullous pemphigoid are more likely to have neurological and psychiatric diseases, particularly prior to the diagnosis of bullous pemphigoid.

OBJECTIVE

The aims were: (i) to evaluate the demographic and clinical features of bullous pemphigoid from a database of patients at a Portuguese university hospital and (ii) to compare the prevalence of comorbid conditions before the diagnosis of bullous pemphigoid with a control group.

METHODS

Seventy-seven patients with bullous pemphigoid were enrolled in the study. They were compared with 176 age- and gender-matched controls, which also had the same inpatient to outpatient ratio, but no history of bullous or cutaneous malignant disease. Univariate and multivariate analyses were used to calculate odds ratios for specific comorbid diseases.

RESULTS

At least one neurologic diagnosis was present in 55.8% of BP patients compared with 20.5% controls (p<0.001). Comparing cases to controls, stroke was seen in 35.1 vs. 6.8%, OR 8.10 (3.80-17.25); dementia in 37.7 vs. 11.9%, OR 5.25 (2.71-10.16); and Parkinson''s disease in 5.2 vs. 1.1%, OR 4.91 (0.88-27.44). Using multivariate analysis, all diseases except Parkinson''s retained their association with BP. Patients under systemic treatment were eight times more likely to have complications than those treated with topical steroids (p< 0.017).

CONCLUSIONS

The results of this study substantiate the association between BP and neurological diseases. In addition, they highlight the potential complications associated with the treatment of BP.  相似文献   

17.

Background

Venous malformation in the pediatric population can present with pain, bleeding, or a debilitating deformity that can be difficult to manage. Sclerotherapy, surgery, and more recently, long pulsed neodymium-doped yttrium aluminum garnet (Nd : YAG) laser have been used with variable success rates.

Objective

Our aims and objectives, were to identify the specific group of patients that would benefit from this treatment.

Methods

A prospective clinical trial was carried out with 59 consecutive patients. The treatment criteria included a large facial deformity and painful or bleeding lesions. One to three treatments were administered at 6~8-week intervals.

Results

A total of 59 patients were treated. The average follow-up was 24 months. All patients achieved good to excellent results in pain and bleeding control as well as in the reduction of the size of lesions in the lip and oral mucosa. The treatment however, is not effective in reducing the size of large, relatively high-flow lesions in the limbs. Complications from treatment include skin blistering (n=4), ulceration (n=4), and subsequent hypertrophic scarring (n=3). Three patients had partial recurrence after a 3-year follow-up.

Conclusion

Complex venous malformations cannot be cured but can be symptomatically controlled with the long-pulsed Nd : YAG laser treatment. The treatment satisfaction is high, and there is a small but definite risk of scarring from treatment.  相似文献   

18.

Background

Onychodystrophy refers to the various abnormalities in nail morphology due to changes in the attachment of the nail plate, changes in nail surface or color. The treatment principle of onychodystrophy largely relies on the discovery and verification of the cause. However, preventive treatment methods offer little help to the patient due to poor compliance, and the effect of corticosteroid is only temporary.

Objective

To evaluate the clinical efficacy of carotene-rich food intake in chronic idiopathic onychodystrophy.

Methods

Ten patients with chronic idiopathic onychodystrophy were recommended to drink one or two cups of carrot juice daily.

Results

Patients showed improvement of onychodystrophy after drinking carrot juice twice a day for at least 4 weeks. No specific adverse effects were noted.

Conclusion

Since there are no reliable treatment methods for chronic idiopathic onychodystrophy, we suggest a simple and compliant treatment method consisting of taking carotene-rich food, such as carrot juice, for patients with chronic idiopathic onychodystrophy.  相似文献   

19.

Background

Despite efforts to treat trachyonychia, there is no promising treatment modality.

Objective

This study evaluated the efficacy and safety of calcipotriol plus betamethasone dipropionate ointment on trachyonychia.

Methods

A total of 39 patients with 432 nails affected by trachyonychia were enrolled. All patients applied calcipotriol/betamethasone ointment once daily without occlusion for 6 months. Outcome measures were assessed by physician''s global assessment (degree of roughness: 0, clear; 1, mild; 2, moderate; 3, marked; 4, severe) at all time points.

Results

After 6 months of therapy, 98.6% (426/432) of nails showed significant clinical improvement; 4.2% were completely free from nail lesions. The mean physician global assessment score decreased significantly from 3.5 to 1.7 points (p< 0.05). No serious side effects were reported, except mild pruritus and erythema in 2 patients.

Conclusion

This is the first study to prospectively evaluate the efficacy and safety of calcipotriol/betamethasone ointment for the treatment of trachyonychia. The results indicate topical calcipotriol/betamethasone is an effective and safe treatment for symptom improvement of trachyonychia.  相似文献   

20.

Background

Recently, low fluence collimated Q-switched (QS) Nd:YAG laser has drawn attention for the treatment of melasma. However, it needs a lot of treatment sessions for the substantial results and repetitive laser exposures may end up with unwanted depigmentation.

Objective

We evaluated the clinical effects and safety of the combinational treatment, using intense pulsed light (IPL) and low fluence QS Nd:YAG laser.

Methods

Retrospective case series of 20 female patients, with mixed type melasma, were analyzed using medical records. They were treated with IPL one time, and 4 times of weekly successive low fluence Nd:YAG laser treatments. At each visit, digital photographs were taken under the same condition. Melanin index (MI) and erythema index (EI) were measured on the highest point on the cheekbones. Modified melasma area and severity index (MASI) scores were calculated by two investigators using digital photographs.

Results

The mean values of MI and EI decreased significantly after treatments. The modified MASI score has decreased by 59.35%, on average. Sixty percents of the participants did not require any more treatments, and no clinical aggravations were observed during the follow-up period (mean 5.9 months).

Conclusion

IPL and low fluence laser may elicit a clinical resolution in the mixed type melasma with long term benefits.  相似文献   

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