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101.
Objective: To evaluate the early results of a new method to repair malfunctioning bicuspid aortic valves by creating a tricuspid valve with a crown-like (i.e. anatomic) annulus. Material and methods: Twelve patients (ages from 10 to 27 years) with chronic regurgitation (and flow-dependent stenosis) of a bicuspid aortic valve underwent repair with the principle of creating a tricuspid valve and a crown-like annulus. The fused leaflets were trimmed and reinserted underneath the existing aortic annulus to create one new native cusp. The third leaflet was fashioned out of a xenopericard patch and was inserted underneath the existing annulus as well to restore the crown-like anatomy of a normal aortic annulus. A tricuspid aortic valve with a morphologically normal annulus was thus created, which resulted in improved coaptation of the leaflets. The repair was immediately assessed by transesophageal echocardiography (TEE) with the heart loaded at 50%. In two patients, a second run helped fine-tune the repair. Median cross-clamping time was 82 min. Follow-up ranged from 3 to 46 months (median 13 months). Results: No significant complication occurred. The function of the aortic valve was excellent with trivial or mild regurgitation in 11 patients and moderate regurgitation in 1 patient. There was no stenosis across the valve. The repair remained stable over time. Remodelling of the left ventricle occurred as expected. Conclusions: Aortic valve repair is feasible in some dysfunctioning bicuspid aortic valves. Tricuspidisation of the valve can result in excellent systolic and diastolic functions. The creation of a crown-like annulus results in improved coaptation of the cusps and could lead to more reliable outcome. Although long-term results are needed, this anatomic correction seems to be a good alternative to valvular replacement in certain sub-groups of patients.  相似文献   
102.
After kidney transplantation, C4d is an incomplete marker of acute antibody‐mediated rejection (AMR) and C1q‐binding donor‐specific antibodies (DSA) have been associated with allograft survival. However, the impact on allograft survival of C1q+ DSA after clinical AMR has not been studied yet. We analysed retrospectively in clinical AMR C4d staining and C1q‐binding impact on allograft survival. We compared clinical, histological and serological features of C4d− and C4d+ AMR, C1q+ and C1q− DSA AMR and analysed C4d and C1q‐binding impact on allograft survival. Among 500 for‐cause kidney allograft biopsies, 48 fulfilled AMR criteria. C4d+ AMR [= 18 (37.5%)] have significantly higher number class I DSA (P = 0.02), higher microvascular score (P = 0.02) and more transplant glomerulopathy (P = 0.04). C1q+ AMR [N = 20 (44%)] presented with significantly more class I and class II DSA (P = 0.005 and 0.04) and C4d+ staining (P = 0.01). Graft losses were significantly higher in the C4d+ group (P = 0.04) but similar in C1q groups. C4d+ but not C1q+ binding was an independent risk factor for graft loss [HR = 2.65; (1.11–6.34); P = 0.028]. In our cohort of clinical AMR, C4d+ staining but not C1q+ binding is an independent risk factor for graft loss. Allograft loss and patient survival were similar in C1q+ and C1q− AMR.  相似文献   
103.

Objective

Isolated visceral artery dissections are rare entities with no current consensus guidelines for treatment and follow-up. This study aims to evaluate the presentation, management, outcomes, and follow-up practices for patients with isolated visceral artery dissections and to compare those with and without symptoms.

Methods

In this retrospective analysis, we identified all patients with isolated celiac artery and/or isolated superior mesenteric artery dissections at a single institution between September 2006 and December 2014. Patients with concomitant aortic dissections were excluded. Cases were stratified by symptom status. Presentation, anatomic findings, treatment, outcomes, and follow-up imaging were then compared between symptomatic and asymptomatic patients.

Results

We identified 25 patients including 15 with symptoms and 10 without. There were no differences in patient comorbidities; however, symptomatic patients more frequently presented with thrombus (n = 10; 67% vs n = 1; 10%; P = .01) and inflammation (n = 8; 53% vs n = 1; 10%; P = .04), and trended toward increased stenosis (n = 12; 80% vs n = 4; 40%; P = .09) compared with asymptomatic patients. All asymptomatic patients were treated with observation alone with vessel diameter enlargement noted in 33% (n = 2) of patients on follow-up imaging. Among symptomatic patients, standard treatment included a short course of anticoagulation (mean, 4.5 months) with lifelong antiplatelet therapy. Three patients underwent operative intervention for persistent or worsening symptoms, two during the index admission and one 10 months after presentation for chronic abdominal pain. Approximately 70% (n = 17) of patients in each group had follow-up imaging (computed tomography angiography: n = 14; 56%; magnetic resonance angiography: n = 4; 16%; ultrasound: n = 13; 52%). Among patients treated nonoperatively, no patients complained of symptoms at follow-up, and 50% of those with inflammation on initial imaging had resolution. Twenty-five percent (n = 4) of patients had an increase in vessel size; however, all vessels remained less than 2 cm in maximal diameter. There were no ruptures or related deaths in either group.

Conclusions

Among patients with visceral artery dissection, no ruptures occurred but diameter enlargement was documented. This disease progression suggests that routine surveillance may be appropriate; however, transitioning early to ultrasound imaging should be considered to decrease radiation, contrast, and associated costs.  相似文献   
104.

Background

Kasai portoenterostomy (KP) remains the initial surgical therapy for biliary atresia (BA). Liver transplantation (LTx) is offered after a failed KP or if KP is not feasible. The timing of LTx in these children is not well established. We attempted to define factors that may help choose the optimal timing for LTx in children with BA managed by a multidisciplinary team including a pediatric surgeon, hepatologist, and liver transplant surgeon.

Methods

Records of children who underwent LTx for BA at our institution between January 1998 and December 2006 were reviewed. Clinical data such as pre-LTx pediatric end-stage liver disease (PELD) score, location of KP, and outcome were evaluated.

Results

Seventy one children underwent 77 liver transplants for BA at an average age of 25 months (range, 3-216 months). Sixty-one had a previous KP, 30 at our institution. Ten had LTx without KP. The overall patient survival was 94.4% and overall graft survival was 87% at median follow-up of 58 months (range, 6-111 months). Four patients died, 1 because of vascular thrombosis despite repeat LTx, 1 because of fungal infection after LTx, and 2 because of causes unrelated to LTx. Six children required retransplantation. Living donor liver transplantation was performed in 32 of these children with 91% patient and graft survival. Fifty-three children had a PELD score of 10 or higher with patient and graft survivals of 92% and 86%, respectively. Eighteen children had a PELD score of less than 10 with patient and graft survivals of 100%. For the 30 children who underwent KP at our institution, the median age at LTx was 9 months (range, 3-168 months), and patient and graft survival were both 93%.

Conclusions

Outcome of LTx for BA is excellent. Children with higher PELD scores (≥10) at LTx may have worse outcome. Children with a PELD score of less than 10 survived with their original grafts. In children with BA, the PELD score should be monitored and may help stratify patients for eventual LTx. When a child with BA is deemed a candidate for LTx, the PELD score should be determined. A PELD score that approaches 10 should trigger discussion of LTx and living donor liver transplantation with the family.  相似文献   
105.
106.
107.
108.

Background

The safety of laparoscopic major liver resections is still uncertain. The aim of this study was to compare our results for laparoscopic right hepatectomy (LRH) with those for open right hepatectomy (ORH).

Methods

Patients undergoing LRH were compared with retrospectively selected patients from our ORH database. The 2 groups were well matched for sex, age, American Society of Anesthesiologists score, body mass index, liver disease, and tumor size. Surgical and postsurgical outcomes were compared.

Results

Seventy-two patients were analyzed: 22 in the LRH group and 50 in the ORH group. Operating time was similar. Blood loss was significantly less in laparoscopic resections (P = .038). Specific morbidity rates were not different, general morbidity was lower after laparoscopy (P = .04), and the severity of postsurgical complications was not different. Mean hospital stay was significantly shorter after laparoscopy (P = .009).

Comments

Laparoscopy improved surgical and postsurgical outcomes for ORH in selected patients. This is the first comparative study to demonstrate an advantage of laparoscopy for a major liver resection. Prospective randomized studies with a greater number of cases are needed to confirm the role of laparoscopy in major liver resections.  相似文献   
109.
Human epidermal growth factor receptor 3 (HER3) is a member of the human epidermal growth factor receptor (HER) family. The main characteristic of HER3 is that it does not possess tyrosine kinase activity, unlike other HERs. The role of HER3 in tumorigenesis has now been recognized, particularly in head and neck squamous cell carcinomas (HNSCCs). Despite conflicting studies, HER3 was found to be overexpressed in HNSCC samples, and correlates with disease progression and poor survival, especially when it is coexpressed with other HERs. HER3 is a significant factor in HNSCC treatment resistance. Indeed, HER3 is a major mechanism described for cetuximab resistance because of modification of epidermal growth factor receptor (EGFR) internalization and by phosphotidylinositol‐3‐kinase (PI3K)/AKT signaling pathway activation. HER3 also affects resistance to tyrosine kinase inhibitors (TKIs) and thereby promotes treatment escape and radiotherapy resistance by activation of the survival signaling pathway. To counteract this, pharmacologic inhibitors of HER3 are currently in development and could significantly improve HNSCC treatment. © 2016 Wiley Periodicals, Inc. Head Neck 38 : E2412–E2418, 2016  相似文献   
110.
The course of autosomal dominant polycystic kidney disease (ADPKD) varies among individuals, with some reaching ESRD before 40 years of age and others never requiring RRT. In this study, we developed a prognostic model to predict renal outcomes in patients with ADPKD on the basis of genetic and clinical data. We conducted a cross-sectional study of 1341 patients from the Genkyst cohort and evaluated the influence of clinical and genetic factors on renal survival. Multivariate survival analysis identified four variables that were significantly associated with age at ESRD onset, and a scoring system from 0 to 9 was developed as follows: being male: 1 point; hypertension before 35 years of age: 2 points; first urologic event before 35 years of age: 2 points; PKD2 mutation: 0 points; nontruncating PKD1 mutation: 2 points; and truncating PKD1 mutation: 4 points. Three risk categories were subsequently defined as low risk (0–3 points), intermediate risk (4–6 points), and high risk (7–9 points) of progression to ESRD, with corresponding median ages for ESRD onset of 70.6, 56.9, and 49 years, respectively. Whereas a score ≤3 eliminates evolution to ESRD before 60 years of age with a negative predictive value of 81.4%, a score >6 forecasts ESRD onset before 60 years of age with a positive predictive value of 90.9%. This new prognostic score accurately predicts renal outcomes in patients with ADPKD and may enable the personalization of therapeutic management of ADPKD.  相似文献   
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