Adenocarcinoma of the Ethmoid sinuses

New therapeutic modalities for Ethmoidal Adenocarcinomas are presented. Thirty three patients harbouring such a tumour have been treated during the last four years. Twenty three were included in the following protocol:--the first step consisted in inductive chemotherapy based on a four-day course of continuous cisplatine (CDDP) and 5-fluoro-uracyl (5-FU infusion)--the second step was the tumour removal, which was performed through a combined transfacial and subfrontal approach. A contralateral ethmoidectomy was always performed. The integrity of the sphenoidal sinus was systematically checked. The cranial base was reconstructed with madreporic coral grafts; then a large extra-dural pediculated galea flap was placed onto the anterior base to line the sub-frontal dura. The authors discuss the results of this series of rare tumours.     

Long-Term Follow-up Results of Renal Transplantation in Pediatric Patients With Focal Segmental Glomerulosclerosis: A Single-Center Experience

Introduction and AimFocal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease in children. We analyzed the long-term outcome of pediatric patients with FSGS undergoing renal transplantation. The objective of the study is to report the experience of a single center and determine the incidence of recurrence, rejection, graft loss, and related risk factors.Materials and MethodThis retrospective cohort study was performed between 1991 and 2018. Thirty patients with a pathologic diagnosis of primary FSGS were included in the study. The patients were diagnosed with FSGS according to histologic features in biopsies.ResultsTwenty-one of the donors were deceased (70%) and 9 were alive (30%). FSGS recurred in only 2 patients. Graft loss occurred in 6 patients (20%). The causes of graft loss were chronic rejection in 4 patients and acute rejection in 2. Our graft survival rate was 100% at 1 year, 91% at 5 years, 80% at 10 years, 70% at 15 years, and 42% at 20 years. Five- and 10-year graft survival rates were 83% and 83% in living donors and 94% and 79% in deceased donors, respectively. According to Kaplan-Meier analysis, there was no statistically significant difference in terms of graft survival between living and deceased donors.ConclusionThis study, with its contribution to literature in terms of long follow-up of FSGS patients from childhood to adulthood, is important. However, further studies are required.     

Combined Liver-Kidney Transplantation in Children: Single-Center Experiences and Long-Term Results

Combined liver-kidney transplantation (CLKT) is a rare procedure in pediatric patients in which liver and kidney from 1 donor are transplanted to a recipient during a single operation. The aim of our study was to analyze indications and results of CLKT in children.

The Freestyle Stentless Bioprosthesis in more than 1000 Patients: A Single-Center Experience over 10 Years

Abstract   Background and Aim: Early and mid-term clinical outcomes after aortic valve replacement (AVR) with stentless bioprostheses in a large cohort of patients are presented. Methods: Between April 1996 and November 2005, 1014 patients underwent AVR with the stentless Medtronic Freestyle bioprosthesis, with 168 using the full-root technique. The mean age was 73 ± 3 (range: 20 to 90) years. Follow-up included 2953 patient-years and was 95% complete for adverse events. Results: Operative mortality was 3.4% (N = 34). Overall survival was 46 ± 9% at nine years and similar to age- and gender-matched German general population. Freedom from prosthetic valve endocarditis, major bleeding, neurological events, and reoperation after nine years was 97 ± 6%, 92 ± 7%, 70 ± 16%, and 92 ± 9%, respectively. Freedom from structural valve deterioration was 97 ± 5% at 9 years. During the learning phase, mean transprosthetic gradients of 23.5 ± 3.0 mmHg and 24.8 ± 3.1 mmHg were observed for valve sizes 21 and 23 mm, respectively, 10 days after subcoronary implantation in 1997, which could be lowered to 16 ± 2.1 mmHg and 14.9 ± 0.9 mmHg in 2005, respectively, with increasing experience of the surgeons. During the follow-up period, mean gradients dropped on average by 15 mmHg in patients presenting higher gradients at discharge. Conclusions: The Freestyle stentless bioprosthesis showed encouraging midterm durability with low rates of valve-related morbidity, and can be safely implanted without increased operative risk even during the learning phase. Special training of the surgeons is recommended to achieve optimal hemodynamic performance.     

Closed Mitral Valvotomy: Actuarial Analysis of Results in 654 Patients over 12 Years and Analysis of Preoperative Predictors of Long-Term Survival

The records of 654 patients with mitral stenosis who underwent closed mitral valvotomy over a 12-year period were submitted to actuarial analysis. This revealed a low (2.97%) operative mortality. At 12 years, the overall cumulative proportion surviving was 78%; 47% of patients survived without reoperation. The usual clinical indicators of suitability for closed valvotomy were successful in predicting improved survival. The surgeon's assessment of the suitability of the valve correlated well with outcome. Valvotomy during pregnancy was associated with a good long-term outlook. The presence of pulmonary hypertension and atrial fibrillation did not alter survival significantly. Sex and age were not associated with adverse prognosis. We conclude that closed mitral valvotomy still has a place in the management of mobile mitral stenosis, particularly in areas where there is a high incidence of rheumatic heart disease and a large number of young patients have mobile mitral stenosis.     

Results of Lung Transplantations Among Cystic Fibrosis Patients: A Single-Center Study

BackgroundLung transplantation remains the ultimate treatment for patients who have exhausted all other therapeutic options in the course of end-stage lung disease due to cystic fibrosis (CF). The aim of the study was to assess the results of lung transplantations performed via mini-thoracotomy in a single center.MethodsThis retrospective study assesses the survival and need for reoperation among 56 primary lung transplant recipients due to CF in a single center between 2018 and 2021. Intraoperative death was also assessed, yet it was established as an exclusion criterion for the post-transplant survival analysis.ResultsOnly one patient died intraoperatively (1.79%). Reoperation at an early postoperative stage was required among 2 patients (3.58%), due to vascular complication for one and pulmonary leakage for the other. Mortality at 30 days was 0%. In-hospital mortality was low (3.58%). Survival at 1, 2, and 3 years was respectively 87%, 85%, and 75%. Mean forced expiratory volume in 1 second as a percentage of predicted value at discharge was approximately 60% and did not decrease after 12 and 24 months. Mean BMI at 12-month follow-up was 20.11 (range, 13-28.7) with 71.4% of patients being qualified as presenting within the normal range of 18.5 to 24.9.Conclusions. Double lung transplantation is a safe and feasible surgical option. Despite being more technically difficult and challenging than clamshell approach for surgeons, it is more beneficial for patients.     

Long-Term Survival After Multidisciplinary Management of Resected Pancreatic Adenocarcinoma

Introduction  Actual 5-year survival rates of 10–18% have been reported for patients with resected pancreatic adenocarcinoma (PC), but the use of multimodality therapy was uncommon in these series. We evaluated long-term survival and patterns of recurrence in patients treated for PC with contemporary staging and multimodality therapy. Methods  We analyzed 329 consecutive patients with PC evaluated between 1990 and 2002 who underwent resection. Each received a multidisciplinary evaluation and a standard operative approach. Pre- or postoperative chemotherapy and/or chemoradiation were routine. Surgical specimens of 5-year survivors were rereviewed. A multivariate model of factors associated with long-term survival was constructed. Presented at the 2008 Annual Meeting of the Society of Surgical Oncology in Chicago, IL.     

Long-Term Outcome After Surgery for a Localized Retroperitoneal Soft Tissue Sarcoma in Elderly Patients: Results from a Retrospective,Single-Center Study

Background

To evaluate short- and long-term results after curative surgery for a retroperitoneal sarcoma (RPS) in elderly patients.

Methods

We retrospectively analyzed data of all patients operated in our single, tertiary care center for a nonmetastatic RPS and identified patients aged 70 years and older.

Results

Among 296 patients with an RPS treated between 1994 and 2015, 60 (20%) were aged 70 years and older (median age 74 years; range 70–85). The median tumor size was 24 cm (range 6–46). Forty-six patients (77%) had mass-related symptoms at the time of diagnosis. The most frequent histological subtypes were de-differentiated liposarcoma (53%, n?=?32) and well-differentiated liposarcoma (35%, n?=?21). Twenty-two patients (37%) had perioperative radiotherapy and/or chemotherapy. Fifty-eight patients (97%) had macroscopically complete resection. The postoperative mortality was 8% and severe morbidity (Dindo/Clavien ≥ 3) was 32%. A reoperation was required for ten patients (17%). After a median follow-up of 20 months (range 1–121), the 5-year overall survival (OS) rate was 90% (95% confidence interval [CI] 79–100%), and median OS was not reached. The cancer-specific death rate was 88%. No prognostic factor for disease-specific survival was detected. The 5-year disease-free survival (DFS) rate was 52% (95% CI 33–84%) and 5-year locoregional recurrence-free survival rate was 52% (95% CI 33–84%). Median DFS was 94 months (95% CI 35–NA). Reoperation after inappropriate surgery and postoperative morbidity were independent predictive factors of locoregional relapse. No predictive factors of distant metastasis were found.

Conclusions

Curative surgery is feasible in selected elderly patients but with higher mortality and morbidity rates than in younger patients. It enables a prolonged survival. Future studies should focus on selection process to minimize postoperative mortality and morbidity.

     

Long-Term Results of Laparoscopic Roux-en-Y Gastric Bypass: Evaluation After 9 Years

This retrospective study aimed to evaluate the long-term results of the laparoscopic Roux-en-Y gastric bypass (LRYGB) procedure performed at our department of bariatric surgery. The 126 consecutive patients treated by LRYGB between January 1, 2001 and December 31, 2002 were analyzed in August 2011. Seventy-seven patients (61.1 %), including 18 who had had previous bariatric surgery, were available for evaluation after 9.4?±?0.6 years (range, 8.7-10.9 years). Eight patients (10.4 %) suffered from type 2 diabetes mellitus (DMII) at the time of surgery. Initial body mass index (BMI) was 40.3?±?7.5 kg/m(2) (range, 24.5-66.1 kg/m(2)). There was no postoperative mortality, but two patients died of causes unrelated to the surgery. Some 9 % of the patients suffered from internal herniation, despite the closure of potential hernia sites. With time, the patients had the tendency to experience weight regain: percentage of excess BMI lost was 56.2?±?29.3 % (range, -78.8 to 117.9 %), down from a maximum of 88.0?±?29.6 % (range, -19.7 to 197.1 %), that had been obtained after a median of 2.0 years (range, 1-8 years). LRYGB was effective for diabetes control in 85.7 % of the affected patients, but, surprisingly, 27.9 % developed new-onset diabetes. The weight regain in this latter patient group was statistically not different from the nondiabetic group. Conversely, four patients required hospitalization for hypoglycemic syndrome. Two patients underwent reversal of their bypass for problems linked to glucose metabolism (one hypoglycemia, one DMII). Patient quality of life was fair. The patient satisfaction remained good in 76 % of the cases.     

Temporary Peritoneal Dialysis in Newborns and Children: A Single-Center Experience over Five Years

Aim: To evaluate the indications, complications, and outcomes of temporary peritoneal dialysis (TPD) in children with acute renal failure (ARF). Patients and methods: All patients undergoing TPD between February 2006 and January 2011 in a children’s hospital were included in the study. Patient characteristics, indications, complications, and duration of TPD (DPD), requirement of re-operation, length of stay, presence of sepsis, and outcome were recorded. Results: There were 21 newborns (14 prematures), 9 infants, and 9 children. The main nephrotoxic agents were gentamicin (n = 7), netilmisin (n = 5), vancomycin (n = 3), and ibuprophen (n = 3). Patients with multiorgan failure (n = 9) had significantly higher blood urea nitrogen (BUN) and creatinine levels than those without multiorgan failure (n = 30) [BUN: 94 ± 27.3 vs. 34.3 ± 4.9) and creatinine: 4.1 ± 0.8 vs. 1.9 ± 0.2)]. The mean DPD was longer in mature patients than in prematures (newborn: 3.7; children: 7.1). Nine complications were observed (23%) (leakage in three and poor drainage in six patients). Twenty-five patients (64.1%) responded to TPD treatment and were discharged, and 14 patients (10 newborns and 7 of them were premature) died (35.9%). Mortality rate was higher in prematures (n = 7) and patients with a history of nephrotoxic agent (n = 10). Conclusion: TPD is effective especially in neonates with ARF and it is a reliable alternative to the hemodialysis or other continuous renal replacement therapies but it is not free of complications. It has limited effects, particularly in patients with multiorgan failure.     

Temporary Peritoneal Dialysis in Newborns and Children: A Single-Center Experience over Five Years

Aim: To evaluate the indications, complications, and outcomes of temporary peritoneal dialysis (TPD) in children with acute renal failure (ARF). Patients and methods: All patients undergoing TPD between February 2006 and January 2011 in a children's hospital were included in the study. Patient characteristics, indications, complications, and duration of TPD (DPD), requirement of re-operation, length of stay, presence of sepsis, and outcome were recorded. Results: There were 21 newborns (14 prematures), 9 infants, and 9 children. The main nephrotoxic agents were gentamicin (n = 7), netilmisin (n = 5), vancomycin (n = 3), and ibuprophen (n = 3). Patients with multiorgan failure (n = 9) had significantly higher blood urea nitrogen (BUN) and creatinine levels than those without multiorgan failure (n = 30) [BUN: 94 ± 27.3 vs. 34.3 ± 4.9) and creatinine: 4.1 ± 0.8 vs. 1.9 ± 0.2)]. The mean DPD was longer in mature patients than in prematures (newborn: 3.7; children: 7.1). Nine complications were observed (23%) (leakage in three and poor drainage in six patients). Twenty-five patients (64.1%) responded to TPD treatment and were discharged, and 14 patients (10 newborns and 7 of them were premature) died (35.9%). Mortality rate was higher in prematures (n = 7) and patients with a history of nephrotoxic agent (n = 10). Conclusion: TPD is effective especially in neonates with ARF and it is a reliable alternative to the hemodialysis or other continuous renal replacement therapies but it is not free of complications. It has limited effects, particularly in patients with multiorgan failure.     

Characteristics of Long-Term Immunosuppressive Therapy in Chinese Pediatric Renal Transplant Patients: A Single-Center Experience

We performed this study to investigate the trend and characteristics of various immunosuppressive regimens as well as their efficacy and safety for long-term survival of Chinese pediatric renal allograft recipients.

Methods

Thirty-four patients who underwent kidney transplantation between January 1985 and July 2002 had ≥5 years follow up. We retrospectively reviewed the baseline characteristics, patient and kidney survival rates, renal function, immunosuppressive regimens, drug levels, and adverse effects of immunosuppressive medications.

Results

The 1-, 3-, and 5-year recipient versus graft survival rates were 100% and 97.1%; 91.2% and 88.2%; 85.3% and 82.4%, respectively. The proportions of patients treated with cyclosporine- or tacrolimus-based immunosuppressive regimen at these times were 48.5%/51.5%; 60.0%/40.0%; and 53.6/46.4%. There were no significant differences in the dosages and drug levels after 1 year (P > .05). The proportions of azathioprine versus mycophenolate mofetil adjunctive therapy were 21.3/78.8%; 23.3%/70%; and 32.1%/60.7%, respectively. Forty percent of the surviving recipients developed complications, including hypertension, hyperlipidemia, gingival hyperplasia, hirsutism, liver dysfunction, herpes zoster, diabetes mellitus or cataracts.

Conclusions

Cyclosporine or tacrolimus, plus mycophenolate mofetil or azathioprine, and prednisone triple therapies showed promising long-term results with similar efficacy and safety in pediatric renal recipients. Periodic drug level monitoring is required to facilitate individualization of immunosuppressive regimens. Drug doses and levels differed markedly from non-Chinese patients because of the ethnic discrepancy.     

Operative Stabilization of Chest Wall Trauma: Single-Center Report of Initial Management and Long-Term Outcome

Background

Conservative treatment of even severe thoracic trauma including flail chest was traditionally the standard of care. Recently, we reported possible benefits of surgical chest wall stabilization in accordance with other groups. The aim of this study was to critically review our indications and results of internal fixation of rib fractures in the long-term course.

Methods

We retrospectively analyzed the data of a consecutive series of patients with internal rib fracture fixation at our institution from 8/2009 until 12/2014, and we retrospectively studied the late outcome through clinical examination or personal interview.

Results

From 1398 patients, 235 sustained a severe thoracic trauma (AIS ≥3). In 23 of these patients, 88 internal rib fixations were performed using the MatrixRIB^® system. The median age of these operated patients was 56 years [interquartile range (IQR) 49–63] with a median ISS of 21 [IQR 16–29]. From 18 local resident patients, follow-up was obtained after an average time period of 27.6 (12–68) months. Most of these patients were free of pain and had no limitations in their daily routine. Out of all implants, 5 splint tips perforated the ribs in the postoperative course, but all patients remained clinically asymptomatic. Plate osteosynthesis showed no loss of reduction in the postoperative course. No cases of hardware prominence, wound infection or non-union occurred.

Conclusions

In our carefully selected thoracic trauma patients, locked plate rib fixation seemed to be safe and beneficial not only in the early posttraumatic course, but also after months and years, patients remain asymptomatic and complete recovery as a rule.Trial registration number KEK BASEC Nr. 2016-01679.

     

Long-Term Outcome of Renal Transplantation in Patients With Familial Mediterranean Fever Amyloidosis: A Single-Center Experience

IntroductionFamilial Mediterranean fever (FMF) is an autosomal-recessive disorder, affecting multiple organs. The AA type of amyloidosis is most common and serious complication cause nephropathy and end-stage renal disease (ESRD). Renal transplantation (RTX) remains treatment of choice for ESRD. We aimed to investigate long-term results of RTX in patients with FMF amyloidosis.Patients and MethodsWe compared the outcomes of 18 patients (12 men and 6 women) with FMF amyloidosis among 601 (2.9%) transplants with 200 control patients. Demographic data and gene analysis were evaluated.ResultsIn our study the 1-year graft and patient survivals were 94.44% and 100%, respectively. At 5 years after RTX, they were 94.73% and 88.88%, respectively, in the FMF group without difference from controls. Mean creatinine level at 1 and 5 years were 1.43 ± 0.54 and 1.73 ± 0.89, respectively. The results of MEFV mutation analyses were: M694V/M694V homozygote in 1 patient, M694V/EQ148 in 3, M694V/V726A in 2, 680M-I/E148Q in 3, M694V/M680I in 5, R202Q/M680I in 2, and M694V/R202Q in 2. Recurrence was noticed in 1 patient with M694V/M680I. One patient died because of graft loss and cardiac complications with M694V/M680I gene analysis. Colchicine was reduced in 4 patients owing to side effects.ConclusionLong-term outcomes of transplantation in patients with amyloidosis secondary to FMF is similar to that in the general transplant population and maintenance colchicine, even after decreasing its dose, effectively prevents recurrence of amyloidosis in the allograft.     

Preliminary Screening Results of Fabry Disease in Kidney Transplantation Patients: A Single-Center Study

Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by the deficiency of alfa-galactosidase A (AGALA) and leads to progressive impairment of renal function in almost all male patients and in a significant proportion of female patients. FD is underdiagnosed or even misdiagnosed in patients undergoing kidney transplantation. We initiated a selective screening study for FD among kidney transplant patients in our center. In this study, 1095 male and female patients were included. Dried blood samples on Guthrie papers were used to analyze galactosidase A enzyme for male patients. Genetic analyses were performed in all female and male patients with low enzyme activity. In total, 648 female and 447 male patients with functioning grafts were evaluated. Among 1095 patients, 5 male patients had AGALA activity below threshold and 3 female patients had galactosidase alpha gene DNA variations. One male patient had a disease-causing mutation. The other 4 patients had polymorphisms causing low enzyme activity. All the 3 female patients had mutations that were associated with FD according to Human Gene Mutation Database (ID: CM025441). In contrast, these mutations were reported as unknown clinical significance in Clinvar (rs149391489). The patients with clinical findings suggesting FD were planned to be analyzed for Lyso Gb3. In our selective screening study, 8 variations were found among 1095 kidney transplantation patients, which needs further investigation to determine causes of FD. Clinical findings, physical examination, and family history are also necessary to evaluate the genetic changes as a mutation in this selected population.