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1.

Background

Congenital chylous ascites poses a significant challenge in neonatal care, and often results in prolonged, complex hospital stays and increased mortality. Few effective options exist in refractory cases.

Methods

Patients aged 0 to 12?months with refractory chylous ascites underwent retroperitoneal exploration after medical treatment and minimally invasive therapies were unsuccessful. The retroperitoneum was completely exposed via left and right medial visceral rotation and opening the lesser sac. Visible leaks were ligated, and alternating layers of fibrin glue and Vicryl mesh were used to cover the entire retroperitoneum.

Results

All 4 patients had resolution of their chylous ascites. None required reoperation or reintervention for chyle leaks. All achieved goal enteral feeds at a median of 29?days postoperatively and were discharged from hospital at a median of 42?days postoperatively.

Conclusions

Management of chylous ascites is extremely challenging in refractory cases. Complete retroperitoneal exposure with fibrin glue and Vicryl mesh application offers a definitive, reliable therapy for achieving cessation of lymphatic leakage and ultimate recovery for patients who fail all nonoperative approaches.

Study type

Therapeutic.

Level of evidence

IV  相似文献   

2.

Background

Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive malignant renal tumor. We describe our experience with neoadjuvant transcatheter arterial chemoembolization (TACE) and systematic chemotherapy for the treatment of advanced CCSK in children.

Methods

Between January 2010 and December 2016, seven patients (3 boys and 4 girls; median 2.2?years) with advanced CCSK received preoperative TACE of renal artery and systemic chemotherapy. The chemoembolic emulsion for TACE consisted of cisplatin, pirarubicin, vindesine, and iodized oil. Preoperative systemic chemotherapy with vindesine, ifosfamide, and etoposide was administered three weeks after TACE. Nephrectomy was performed three weeks after systemic chemotherapy. After surgery, patients received radiotherapy and postoperative chemotherapy.

Results

No cardiotoxicity, renal insufficiency, or hepatic dysfunction was found in any patients. Grade II–III marrow suppression developed in four patients. One patient with tumor progress during neoadjuvant therapy failed to successfully undergo surgery and died. Six patients underwent nephrectomy after neoadjuvant therapy. Median follow-up period was 49.5?months (range, 11–83?months). Five patients have recurrence-free survival. One patient is still in postoperative chemotherapy after nephrectomy, radiotherapy and thoracoscopic resection of lung metastases.

Conclusions

Neoadjuvant TACE and systemic chemotherapy appeared to be feasible in the treatment of advanced CCSK in this pilot study.

The type of study

A case series with no comparison group.

Level of evidence

Level IV.  相似文献   

3.
4.

Background/Purpose

Many survivors of childhood cancer will experience premature gonadal insufficiency or infertility as a consequence of their medical treatments. Ovarian tissue cryopreservation (OTC) remains an experimental means of fertility preservation with few reports focused on the surgical technique and postoperative outcomes for OTC in children.

Methods

This is a single institution, retrospective review of OTC cases from January 2011 to December 2017. Children were eligible for OTC if they had a greater than 80% risk of premature ovarian insufficiency or infertility owing to their anticipated gonadotoxic medical treatment.

Results

OTC was performed in 64 patients. Median age was 12?years old (range: 5?months–23?years). Nearly half (48%) of the patients were premenarchal. Laparoscopic unilateral oophorectomy was performed in 84% of patients. There were no surgical complications. In 76% of patients, OTC was performed in conjunction with an ancillary procedure. The majority (96%) of patients were discharged within 24 hours. Median time from operation to medical therapy was six days, with no unanticipated treatments delays attributable to OTC.

Conclusions

Laparoscopic unilateral oophorectomy for OTC can be performed safely, in combination with other ancillary procedures, as an outpatient procedure without delaying medical therapy for children facing a fertility-threatening diagnosis or treatment.

Level of Evidence

IV.  相似文献   

5.

Purpose

Lingual thyroglossal duct cysts (L-TGDCs) are rare and sometimes lethal owing to their association with asphyxia. We aimed to analyze our single institutional experience with L-TGDCs.

Methods

Twelve L-TGDC cases treated at our institution between January 2010 and December 2017 were investigated.

Results

The male/female ratio was 6/6. The age at the diagnosis was 2?±?1.4?months (7?days to 6?months), and 3 patients were diagnosed in the neonatal period. The patients presented with stridor (n?=?12; 100%), growth retardation (n?=?5; 42%), apnea (n?=?3; 25%), and vomiting (n?=?1; 8.3%). Lateral X-rays were obtained in 8 cases (66.7%); a lingual mass was suspected in 7 (87.5%). Transoral marsupialization of the cyst was performed under direct vision in all cases. All cases were nasally and orally intubated using a laryngoscope, bronchoscope, or airway scope. The mean operative time was 18?±?2.9?min. The mean cyst size was 10.5?±?1.8?mm. No recurrence was observed during the follow-up period (37.5?±?18?months).

Conclusion

L-TGDC requires a precise diagnosis and rapid intervention because of the risk of asphyxia resulting in sudden death. Transoral marsupialization under direct vision is an effective and secure approach. L-TGDC should be considered when patients younger than six months of age present with respiratory distress.

Type of study

Retrospective Study.

Level of evidence

Level IV.  相似文献   

6.

Purpose

To raise awareness of colocolonic intussusception as a gastrointestinal complication of CF mimicking distal intestinal obstruction syndrome (DIOS) and discuss risk of recurrence.

Case summary

A 33-year-old Caucasian male with cystic fibrosis presented with an acute abdomen diagnosed via imaging as colocolonic intussusception. He was managed with fluid replacement therapy and polyethylene glycol. He was re-admitted due to recurrence likely secondary to recurrent constipation and development of a fecalith. Surgery was contraindicated due to absence of tissue ischemia or necrosis.

Discussion

Several possible etiological factors have been described, especially some that tend to occur within the context of CF disease, such as DIOS and PERT, and symptoms of colocolonic intussusception are similar to those of other causes of an acute abdomen but distinguishable by advanced imaging modalities. Due to risk of recurrence, an etiology of intussusception should be sought.

Conclusion

Colo-colonic intussusception is a rare cause of an acute abdomen in the adult Cystic Fibrosis (CF) patient and may be associated with underlying constipation or presence of a fecalith.  相似文献   

7.

Objective

The purpose of this study is to evaluate the effect of Intravesical Botulinum toxin injection on the symptoms and urodynamic parameters in pediatric patients with idiopathic overactive bladder (iOAB) refractory to medical treatment.

Materials and methods

The study was designed as an open-label uncontrolled therapeutic clinical trial. The eligible patients who underwent Intravesical botulinum toxin injection were evaluated before treatment. The evaluation included a 7-day paper bladder diary to assess OAB symptoms (frequency, urgency urinary incontinence (UUI) and nocturnal enuresis (NE)), filling the Arabic International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form (ICIQ-UI short form), and conducting urodynamic study. The Urodynamic parameters obtained were the maximum filling detrusor pressure, cystometric bladder capacity, and compliance. After 12?weeks of the intravesical injection, the patients were revaluated and the results were compared using paired samples t-test.

Results

The study enrolled 75 patients. And of those, statistical analysis was done on 46 patients who did follow the study protocols. The mean age was 8.9?years and male to female ratio was 1:4. There was a statistically significant improvement in overactive bladder symptoms and urodynamic parameters in the patient injected with botulinum toxin with minimal side effects.

Conclusion

The evidence in this study would support the safety and efficacy of Intravesical botulinum toxin injection in children with refractory idiopathic OAB with significant improvement of symptoms, quality of life, as well as urodynamic parameters.

Type of Study

Open-label uncontrolled therapeutic clinical trial.

Level of Evidence

III  相似文献   

8.

Aim

Tracheomalacia is flaccidity of the tracheal wall leading to tracheal collapse, particularly on expiration. When severe it can warrant surgical intervention. Aortopexy is one surgical option and has been described using a variety of approaches. We report outcomes of aortopexy performed via a suprasternal incision in a single centre by a single surgeon.

Methods

All patients undergoing aortopexy between February 2016 and May 2018 were prospectively included. Patients were managed by a multi-disciplinary team (MDT) and underwent standardized work-up, including dynamic flexible bronchoscopy (DFB) and contrast enhanced CT. Aortopexy was performed via a suprasternal skin crease incision. Surgery was guided by intra-operative flexible bronchoscopy. Data regarding pre-operative symptoms, degree of tracheomalacia, and outcomes were recorded.

Results

Twenty-two patients were included, 18 boys, median age 5?months (range 1?month–5?years). Two aortopexy sutures were used in all cases, and three patients required an additional tracheopexy suture. Median operating time was 1?h 24?min (range 47?min–2?h 35?min). Median pre-operative tracheal collapse on DFB was 85% (range 80–95%), improving to 35% (35–80%) intra-operatively. Median length of stay was 4?days (range 1–118).

Conclusions

Aortopexy via a suprasternal incision is a useful treatment modality for tracheomalacia with short operating times and rapid recovery. Outcomes vary with the best results in the TOF and premature groups. Careful patient selection and MDT work-up are essential to optimize outcomes.

Level of Evidence

Level 3 (Case Series).  相似文献   

9.

Aim of the Study

The goals of urinary reconstruction in urogenital sinus and cloacal repair include: (1) positioning of the bladder neck above the urogenital diaphragm to maximize future urinary continence, and (2) creating a visible urethra that can be catheterized if needed. A recent algorithm in cloacal reconstruction proposed a urethral length of 1.5?cm as the key determinant in deciding whether to perform a total urogenital mobilization or a urogenital separation, the hypothesis being that a 1.5?cm length urethra is needed for the patient to remain dry. We wondered if the normal female urethral length correlated with this empiric technical determinant.

Methods

We reviewed voiding cystourethrograms of healthy female patients between ages 6 and 36?months and measured the patient's urethral length.

Results

Ninety-one children were included. The mean urethral length for patients age 6–12?months was 2.50?cm, age 12–24?months was 2.31?cm, and age 24–36?months was 2.59?cm. There was no difference between the urethral length in the three groups (p?=?0.38). Of 91 patients, 87 (96%) had a urethral length > 1.5?cm.

Conclusion

A urethra of at least 1.5?cm was present in the majority of normal control patients. We believe therefore that for urogenital sinus and cloacal repair, surgeons can extrapolate that patients need a 1.5?cm urethra at the end of the reconstruction. Additional follow-up is needed to determine if this urethral length as an independent factor maintains dryness in the long term after cloacal repair.

Type of Study

Case Series.

Level of Evidence

III.  相似文献   

10.

Importance

Telemedicine is an emerging strategy for healthcare delivery that has the potential to expand access, optimize efficiency, minimize cost, and enhance patient satisfaction.

Objective

To review the current spectrum, potential strategies, and implementation process of telemedicine in pediatric surgery.

Design

Review and opinion design.

Setting

n/a.

Participants

n/a.

Main outcomes and measures

n/a.

Results

n/a.

Conclusions and relevance

Telemedicine is an emerging approach with the potential to facilitate efficient, cost-effective delivery of pediatric surgical services.

Brief Abstract

Telemedicine is an emerging strategy for healthcare delivery that has the potential to expand access, optimize efficiency, minimize cost, and enhance patient satisfaction. The objectives of this review are to explore common terms in telemedicine, provide an overview of current legislative and billing guidelines, review the current state of telemedicine in surgery and pediatric surgery, and provide basic themes for successful implementation of a pediatric surgical telemedicine program.

Type of Study

Review.

Level of Evidence

Level V.  相似文献   

11.

Background

Appendicitis presents on a spectrum ranging from inflammation to gangrene to perforation. Studies suggest that gangrenous appendicitis has lower postoperative infection rates relative to perforated cases. We hypothesized that gangrenous appendicitis could be successfully treated as simple appendicitis, reducing length of stay (LOS) and antibiotic usage without increasing postoperative infections.

Methods

In February 2016, we strictly defined complex appendicitis as a hole in the appendix, extraluminal fecalith, diffuse pus or a well-formed abscess. We switched gangrenous appendicitis to a simple pathway and reviewed all patients undergoing laparoscopic appendectomy for 12?months before (Group 1) and 12?months after (Group 2) the protocol change. Data collected included demographics, appendicitis classification, LOS, presence of a postoperative infection, and 30-day readmissions.

Results

Patients in Group 1 and Group 2 were similar, but more cases of simple appendicitis occurred in Group 2. Average LOS for gangrenous appendicitis patients decreased from 2.5 to 1.4?days (p?<?0.001) and antibiotic doses decreased from 5.2 to 1.3 (p?<?0.001). Only one gangrenous appendicitis patient required readmission, and one patient in each group developed a superficial infection; there were no postoperative abscesses.

Conclusions

Gangrenous appendicitis can be safely treated as simple appendicitis without increasing postoperative infections or readmissions.

Type of study

Prognosis study.

Level of evidence

Level II.  相似文献   

12.

Background

To assess the outcome of patients with biliary atresia (BA) who underwent a surgical shunt (SS) for severe portal hypertension (PH) following a Kasai procedure.

Methods

We collected and analyzed the data and outcomes of patients with BA who underwent SS for severe PH following a Kasai procedure between 1974 and 2014, focusing on complications related to the procedure, overall survival (OS), and transplant-free survival (TFS).

Results

SS was performed at a median age of 5.5?years [2–13.5] in 38 patients. Conjugated bilirubin level (cBL) was ≤ 20 μmol/l in 24 patients at time of SS. Median follow-up was 15?years [1–32]. OS at 5 and 10?years was 91% and 87% respectively. TFS at 5 and 10?years was 84% and 70% respectively. Long-term complications included hepatic encephalopathy in 9 patients, and hepatopulmonary syndrome in 3. At last follow-up, 10/14 patients without LT and 18/ 24 who had a delayed LT at a median delay of 11?years [1.5–22] were alive.

Conclusion

Surgical shunt for severe portal hypertension in biliary atresia may delay the need for liver transplantation. However complications are indications for transplantation.

Level of evidence

Type of study: Therapeutic.Level of evidence III.  相似文献   

13.

Background/purpose

Computed tomography (CT) derived Haller Index (HI) remains the standard for quantifying severity in patient with pectus excavatum (PE). Optical scanning described in literature reports optimistic results and new indices that correlate with HI. This study assessed the feasibility of a handheld White Light Scanner (WLS) to obtain 3D measurements and indices of PE deformity.

Methods

From April 2015–April 2017, WLS scanning was conducted by orthotists during clinical visits. Included were children with PE up to 18?years. Analysis assessed correlation of a WLS-derived severity index, Hebal-Malas Index (HMI), with physician measured PE Depth (PED), and CT-derived HI.

Results

Of 195 participants, 185(94%) patients with PE were scanned and 127(69%) had complete WLS data. For 88 patients undergoing monitoring, HMI correlated with PED (r?=?0.42, p?=?0.004). For 39 patients with pre-operative CT, HMI demonstrated strong correlation with HI (r?=?0.87, p < 0.0001).

Conclusions

WLS demonstrated high feasibility of scanning PE. WLS-derived HMI best correlates with HI for patients with severe pectus deformity. Our current data is suggestive that WLS is best applied for severe deformities and yet to be established for milder deformities. Future yearly WLS will provide data on deformity progression and surgical therapy.

Level of Evidence

IV.

Type of Study

Diagnostic Study.  相似文献   

14.

Objective

Disruption of the enterohepatic circulation of bile acids (BAs) is part of the gastrointestinal phenotype of cystic fibrosis (CF). Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, improves pulmonary function in CF patients with class III gating mutations. We studied the effect of ivacaftor on the enterohepatic circulation by assessing markers of BA homeostasis and their changes in CF patients.

Methods

In CF patients with an S1251N mutation (N?=?16; age 9–35?years S125N study/NTR4873) or a G551D mutation (N?=?101; age 10–24?years; GOAL study/ NCT01521338) we analyzed plasma fibroblast growth factor 19 (FGF19) and 7α-hydroxy-4-cholesten-3-one (C4) levels, surrogate markers for intestinal BA absorption and hepatic synthesis, respectively, before and after treatment with ivacaftor.

Results

At baseline, median FGF19 was lower (52% and 53%, P?<?.001) and median C4 higher (350% and 364%, P?<?.001), respectively, for the S1251?N and G551D mutation patient groups compared to healthy controls. Treatment with ivacaftor significantly increased FGF19 and reduced C4 levels towards normalization in both cohorts but this did not correlate with CFTR function in other organs, as measured by sweat chloride levels or pulmonary function.

Conclusions

We demonstrate that patients with CFTR gating mutations display interruption of the enterohepatic circulation of BAs reflected by lower FGF19 and elevated C4 levels. Treatment with ivacaftor partially restored this disruption of BA homeostasis. The improvement did not correlate with established outcome measures of CF, suggesting involvement of modulating factors of CFTR correction in different organs.  相似文献   

15.

Background

Firearm injuries are now the third leading cause of death in children. Understanding the circumstances surrounding pediatric firearm injuries will allow for targeted injury prevention efforts. We hypothesized that younger children are more likely to be victims of unintentional firearm injury.

Methods

A multicenter, retrospective review of patients < 18?years old who sustained firearm injuries in Los Angeles County from 2006 to 2015 was performed. Unintentional injuries were defined as accidental firearm discharge without violent intent. Intentional injuries were defined as firearm discharge with intent to injure (including suicide).

Results

After review of 304 pediatric firearm injuries, 206 had sufficient narrative to determine intent with 10% of injuries classified as unintentional. Unintentional injuries were more common in younger children, more frequently caused by a firearm from within the home, and more likely to involve friend/family (all p?<?0.05). Intentional injuries were associated with more injuries and accounted for all deaths in our study cohort.

Conclusions

In pediatric firearm injury, younger children are more susceptible to unintentional injuries, but intentional injuries are more common overall. Future interventions need to target both intentional violence in older children and unintentional firearm injury in young children if the frequency is to be reduced.

Type of study

Epidemiologic study.

Level of evidence

Level III.  相似文献   

16.

Background

Penetrating stab wounds in children are relatively rare and no clear recommendations for the optimal evaluation have been devised. An acceptable traditional approach to the patient with an abdominal stab wound who does not require urgent surgery is selective nonoperative management and serial exams. The use of routine computed tomography remains an actively utilized investigation for these patients at many institutions.

Purpose

We hypothesize that the approach to pediatric stab wound victims should be distinctly different than that of adult counterparts in order to minimize radiation exposure.

Methods

A retrospective cohort study involving abdominal stab wounds among pediatric trauma patients (age < 14) compared with adults between the years 1997 and 2016 was conducted utilizing the Israeli National Trauma Registry.

Results

A total of 92 children and 4444 adults were identified from the registry for inclusion. Among the children 20 (21.7%) patients had intraabdominal injury compared to 1730 (38.9%) among adult counterparts. Four children were hemodynamically unstable, two of them were referred directly to operating room and two others were treated without surgery. Among the remaining 88 children there was no observed mortality.

Conclusions

The majority of pediatric stab wounds trauma victims have minor abdominal injuries. We do not recommend the routine utilization of abdominal CT scan in the evaluation of abdominal stab wounds. Observation with serial exams and minimization of radiation exposure from CT are warranted in this unique population.

Type of study

Retrospective comparative study.

Level of evidence

3.  相似文献   

17.

Background/objectives

An incidental appendectomy is performed by some surgeons during operative treatment for intussusception to eliminate future appendicitis as a diagnostic consideration. However, an appendectomy can increase the risk of infection and other noninfectious complications making an incidental appendectomy controversial. We examined outcomes for surgical intervention for intussusception with appendectomy (SWA) compared to surgical reduction alone (SRA).

Methods

The Pediatric Health Information System database, 8/2008–9/2015, was retrospectively analyzed for patients under the age of five who required an operative intervention for intussusception without bowel resection. Demographic data and postoperative outcomes were analyzed. Available data included need for postoperative enema, subsequent small bowel obstruction, recurrent intussusception, length of stay (LOS), and adjusted total cost (ATC).

Results

Fifty-seven percent (748/1312) of patients had appendectomy performed during surgical reduction, 564 (43%) did not. ATC ($10,594 vs. $8939, p?<?0.001) and LOS (3.0 vs. 2.48, p?<?0.001) are higher in the SWA group. Rates of readmission are similar, but post-operative small bowel obstruction may be higher in the SWA group (1.3% vs. 0.35%, p?=?0.06).

Conclusion

There is a higher mean LOS and ATC in the SWA group. This study suggests that appendectomy during surgery for uncomplicated intussusception should be reconsidered and requires further investigation.

Type of Study

retrospective comparative study.

Level of Evidence

III.  相似文献   

18.

Background

Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement.

Methods

We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography.

Results

Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well.

Conclusions

Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options.

Level of evidence

IV.  相似文献   

19.

Purpose

To investigate the factors affecting primary bladder closure in cloacal exstrophy (CE). A successful primary closure is important for optimizing reconstructive outcomes, and it is a critical first-step in the reconstruction of CE. The authors' hypothesize that a smaller diastasis and use of an osteotomy are independent predictors of a successful closure.

Methods

A prospectively maintained database of 1332 exstrophy-epispadias complex (EEC) patients was reviewed for CE patients closed between 1975 and 2015. Univariate and multivariable analyses were performed to identify significant factors associated with CE primary bladder closure.

Results

Of 143?CE patients identified, 99 patients met inclusion criteria. Median follow-up time was 8.82 [IQR 5.43–14.26] years. In the multivariable model, the odds of having a successful closure are about 4 times greater for the staged cloacal approach compared to the 1-stage approach (OR, 3.7; 95% CI 1.2–11.5; p-value?=?0.023). Also, having an osteotomy increases the chance of a successful closure by almost six-fold (OR, 5.8; 95% CI 1.7–19.6; p-value?=?0.004).

Conclusions

Using the staged approach with a pelvic osteotomy is paramount to a successful primary closure in CE. The authors strongly recommend using the staged approach and osteotomy as these factors independently increase the chance for a successful primary bladder closure.

Study Type

Therapeutic study.

Level of Evidence

Level III, Retrospective comparative study.  相似文献   

20.

Background

Pulmonary exacerbations (PEx) in cystic fibrosis (CF) patients decrease lung function, increase symptoms and reduce health-related quality of life (HRQoL). We evaluated associations between 8 symptom-based questions from the Cystic Fibrosis Respiratory Symptom Diary - Chronic Respiratory Infection Symptom Score (CFRSD-CRISS) and the 5-level EuroQOL-5 Dimensions (EQ-5D-5?L) summary score and hypothesized the CFRSD-CRISS would be well-correlated with quality-of-life measures among CF patients with PEx.

Methods

CF patients who had CFRSD-CRISS and EQ-5D-5L measurements on the day of the initial PEx, 7?days later, and at the end of intravenous antibiotic treatment were included. We examined age-stratified (<18 versus ≥18?years old) characteristics, including the percent predicted of forced expiratory volume in 1?s (ppFEV1), CFRSD-CRISS measurements, and domains of the EQ-5D. We also calculated age-stratified Pearson correlation coefficients between the EQ-5D-5L and CFRSD-CRISS items at each of the 3 time points.

Results

A total of 169 patients were analyzed. Patients reported having problems performing usual activities and with pain/discomfort on the first day of the PEx and these measures improved by the end of treatment. PpFEV1 improved in both age categories by the end of PEx treatment but was not associated with the change in summary EQ-5D-5?L over the time of PEx treatment (r-squared?=?0.029). Correlations were weak (generally <0.4) between the elements of the EQ-5D-5?L versus the CFRSD-CRISS.

Conclusions

Value assessment of treatments for CF PEx will require the collection of preference-weighted measures rather than only the symptom-based questions of the CFRSD-CRISS.  相似文献   

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