Browsing by Subject "SINGLE-CENTER"

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  • Hölttä, Tuula; Gordin, Daniel; Rahkonen, Otto; Turanlahti, Maila; Holmström, Miia; Tainio, Juuso; Rönnholm, Kai; Jalanko, Hannu (2020)
    Over the past 30 years, there has been an improvement in both patient and graft survival after pediatric renal transplantation (RTX). Despite this success, these patients still carry an elevated risk for untimely death, partly through premature aging of the vasculature. The aim of this study was thus to investigate the long-term outcome of individuals with RTX in childhood, as well as to explore the cardiovascular health of these adults more than a decade later. We studied 131 individuals who had undergone a RTX between the years 1979 and 2005. Furthermore, left ventricular hypertrophy (LVH), coronary artery calcifications (CAC), and related metabolic factors were investigated in a cross-sectional study including 52 individuals as part of the initial cohort. The mortality rate (n = 131) was 12.2%. The median estimated graft survival was 17.5 years (95% CI 13.6-21.3), being significantly better in children transplanted below the age of 5 years (18.6 vs. 14.3 years, P <0.01) compared with older ones. CAC were found in 9.8% and LVH in 13% of the patients. Those with cardiac calcifications had longer dialysis vintage and higher values of parathyroid hormone (PTH) during dialysis. Left ventricular mass correlated positively with systolic blood pressure, PTH, and phosphate measured at the time of the study.
  • Ruuska, Satu Maria; Laaperi, Mitja Tapani; Hukkinen, Maria; Jalanko, Hannu; Kolho, Kaija-Leena; Pakarinen, Mikko P. (2019)
    We addressed growth of biliary atresia (BA) patients living with native livers between ages 0-6 and effects of post-surgical corticosteroid treatment on growth. Growth charts of 28 BA patients born in Finland between 1987 and 2017 were retrospectively evaluated. Dosage and length of corticosteroid treatment and hydrocortisone substitution were reviewed. At birth, BA patients were shorter (median height -0.6 (interquartile range (IQR) -1.3 to -0.1) SDS, n=28, P
  • Molander, Pauliina; Farkkila, Martti; Kemppainen, Helena; Blomster, Timo; Jussila, Airi; Mustonen, Harri; Sipponen, Taina (2017)
    Background: Little data exist on the long-term prognosis of patients with inflammatory bowel disease (IBD) after stopping TNF alpha-blocking therapy in deep remission. Existing data indicate that approximately 50% of patients on combination therapy who discontinued TNF alpha-blockers are still in remission 24 months later. The aims of this follow-up analysis were to evaluate the long-term remission rate after cessation of TNF alpha-blocking therapy, the predicting factors of a relapse and the response to restarting TNF alpha-blockers. Methods: The first follow-up data of 51 IBD patients (17 Crohn's disease [CD], 30 ulcerative colitis [UC] and four inflammatory bowel disease type unclassified [IBDU]) in deep remission at the time of cessation of TNF alpha-blocking therapy have been published earlier. The long-term data was collected retrospectively after the first follow-up year to evaluate the remission rate and risk factors for the relapse after a median of 36 months. Results: After the first relapse-free year, 14 out of the remaining 34 IBD patients relapsed (41%; 5/12 [42%] CD and 9/22 [41%] UC/IBDU). Univariate analysis indicated no associations with any predictive factors. Re-treatment was effective in 90% (26/29) of patients. Conclusion: Of IBD patients in deep remission at the time of cessation of TNF alpha-blocking therapy, up to 60% experience a clinical or endoscopic relapse after a median follow-up time of 36 months (95% CI 31-41 months). No individual risk factors predicting relapse could be identified. However, the initial response to a restart of TNF alpha-blockers seems to be effective and well tolerated.
  • Luoto, Topi T.; Koivusalo, Antti; Pakarinen, Mikko P. (2020)
    Objectives: Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. Methods: All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded. Results: Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at a median age of 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2, and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient-reported perception of health on a scale of 1 to 10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices. Conclusions: Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient-reported health of long-term survivors was encouraging.
  • Koivusalo, Antti I.; Sistonen, Saara J.; Lindahl, Harry G.; Rintala, Risto J.; Pakarinen, Mikko P. (2017)
    Purpose: Because of an extended gap between esophageal pouches a variety of methods are employed to treat oesophageal atresia (OA) without (type A) or with (type B) proximal tracheooesophageal fistula. This retrospective observational study describes their single centre long-term outcomes from 1947 to 2014. Methods: Of 693 patients treated for OA 68 (9.7%) had type A (n = 58, 8.3%) or B (n = 10, 1.4%). Hospital records were reviewed. Main outcome measures were survival and oral intake. Results: Nine (13%) patients had early and 10 (15%) delayed primary anastomosis, 30 (44%) underwent reconstruction including colonic interposition (n = 13), reversed gastric tube (n = 11) and jejunum interposition (n = 6), whereas19 (28%) had died without a definite repair. Median follow up was 35 (interquartile range, 7.4-40) years. Thirty-one (63%) of 49 patients with definitive repair survived long term. Survival was 22% for early and 80% for delayed primary anastomosis, 57% for colon interposition, 82% for gastric tube and 84% for jejunum interposition. Gastrooesophageal reflux was most common after gastric tube (80%), dysphagia after colon interposition (50%), and 3 (60%) of 5 survivors with jejunum interposition had permanent feeding ostomy because of neurological disorder. Endoscopic follow-up disclosed no oesophageal cancer or dysplasia. Repair in the most recent patients from 1985 to 2014 (n = 14) included delayed primary anastomosis (n = 7), jejunum interposition (n = 6) and gastric tube (n = 1) with 93% long-term survival. Conclusion: Morbidity among long-term survivors of type A or B OA is high. With modern management survival is, however, excellent and patients without neurological disorder achieve full oral intake either after primary anastomosis or reconstruction. (C) 2017 Elsevier Inc. All rights reserved.
  • Nordic Pediat Surg Study Consortiu (2018)
    Background/purpose: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. Methods: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016. Results: Of the 154 patients, 148 underwent portoenterostomy mostly by assigned surgical teams at median age of 64 (interquartile range 37-79) days, and 95 patients (64%) normalized their serum bilirubin concentration while living with native liver. Postoperative adjuvant medical therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >3 patients and diagnostic protocol without routine liver biopsy predicted early performance of portoenterostomy. The cumulative 5-year native liver and overall survival estimate was 53% (95% CI 45-62) and 88% (95% CI 83-94), respectively. Portoenterostomy age 3 patients were predictive for long-term native liver survival, while normalization of serum bilirubin after portoenterostomy was the major predictor of both native liver and overall 5-year survival. Conclusions: The outcomes of biliary atresia in the Nordic countries compared well with previous European studies. Further improvement should be pursued by active measures to reduce patient age at portoenterostomy. Retrospective prognosis study: Level II. (C) 2017 Elsevier Inc. All rights reserved.
  • Weismueller, Tobias J.; Trivedi, Palak J.; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Färkkilä, Martti; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouilleres, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; de Valle, Maria Benito; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Int PSC Study Grp (2017)
    BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS: We performed a retrospective outcome analysis of patients diagnosed with PSC from 1980 through 2010 at 37 centers in Europe, North America, and Australia. For each patient, we collected data on sex, clinician-reported age at and date of PSC and IBD diagnoses, phenotypes of IBD and PSC, and date and indication of IBD-related surgeries. The primary and secondary endpoints were liver transplantation or death (LTD) and hepatopancreatobiliary malignancy, respectively. Cox proportional hazards models were applied to determine the effects of individual covariates on rates of clinical events, with time-to-event analysis ascertained through Kaplan-Meier estimates. RESULTS: Of the 7121 patients in the cohort, 2616 met the primary endpoint (median time to event of 14.5 years) and 721 developed hepatopancreatobiliary malignancy. The most common malignancy was cholangiocarcinoma (n = 594); patients of advanced age at diagnosis had an increased incidence compared with younger patients (incidence rate: 1.2 per 100 patient-years for patients younger than 20 years old, 6.0 per 100 patient-years for patients 21-30 years old, 9.0 per 100 patient-years for patients 31-40 years old, 14.0 per 100 patient-years for patients 4150 years old, 15.2 per 100 patient-years for patients 51-60 years old, and 21.0 per 100 patient-years for patients older than 60 years). Of all patients with PSC studied, 65.5% were men, 89.8% had classical or large-duct disease, and 70.0% developed IBD at some point. Assessing the development of IBD as a time-dependent covariate, Crohn's disease and no IBD (both vs ulcerative colitis) were associated with a lower risk of LTD (unadjusted hazard ratio [HR], 0.62; P
  • Ratasvuori, Maire; Sillanpaa, Niko; Wedin, Rikard; Trovik, Clement; Hansen, Bjarne H.; Laitinen, Minna (2016)
    Background and purpose - Surgery for metastases of renal cell carcinoma has increased in the last decade. It carries a risk of massive blood loss, as tumors are hypervascular and the surgery is often extensive. Preoperative embolization is believed to facilitate surgery. We evaluated the effect of preoperative embolization and resection margin on intraoperative blood loss, operation time, and survival in non-spinal skeletal metastases of renal cell carcinoma. Patients and methods - This retrospective study involved 144 patients, 56 of which were treated preoperatively with embolization. The primary outcome was intraoperative blood loss. We also identified factors affecting operating time and survival. Results - We did not find statistically significant effects on intraoperative blood loss of preoperative embolization of skeletal non-spinal metastases. Pelvic localization and large tumor size increased intraoperative blood loss. Marginal resection compared to intralesional resection, nephrectomy, level of hemoglobin, and solitary metastases were associated with better survival. Interpretation - Tumor size, but not embolization, was an independent factor for intraoperative blood loss. Marginal resection rather than intralesional resection should be the gold standard treatment for skeletal metastases in non-spinal renal cell carcinoma, especially in the case of a solitary lesion, as this improved the overall survival.
  • Rahmanian, Abdolkarim; Ghaffarpasand, Fariborz; Alibai, Ehsanali; Choque-Velasquez, Joham; Jahromi, Behnam Rezai; Hernesniemi, Juha (2018)
    OBJECTIVES: To report the outcome of patients with very small intracranial aneurysm (VSIA) undergoing surgical clipping using a double-clip technique. METHODS: This cross-sectional study was conducted in Namazi Hospital, the main referral neurovascular center in Southern Iran during a 6-year period from September 2010 to March 2016. All patients with VSIAs ( RESULTS: Operations were performed on 32 VSIAs in 26 patients with a mean +/- SD age of 55.7 +/- 10.1 years. Middle cerebral artery was the most common location for VSIA (50.0%). There was no neck remnant, and the complete occlusion rate was 100%. The rate of intraoperative aneurysm rupture was 30.8%, and none of the patients experienced rebleeding. The 6-month mortality rate was 0% in ruptured VSIAs and 6.25% in unruptured VSIAs. Most of the patients had favorable outcomes (88.5%), and the overall mortality rate was 11.5%. The rate of permanent neurologic deficit was 10.0% in ruptured and 12.5% in unruptured VSIAs. Multivariate logistic regression analysis revealed no association between baseline and clinical characteristics and outcome in this series. CONCLUSION: VSIAs are difficult to treat because of their small sizes; therefore, with a double-clip technique, one can reduce complications related to the treatment of small aneurysms.
  • Bonaros, Nikolaos; Petzina, Rainer; Cocchieri, Riccardo; Jagielak, Dariusz; Aiello, Marco; Lapeze, Joel; Laine, Mika; Chocron, Sidney; Muir, Douglas; Eichinger, Walter; Thielmann, Matthias; Labrousse, Louis; Bapat, Vinayak; Rein, Kjell Arne; Verhoye, Jean-Philippe; Gerosa, Gino; Baumbach, Hardy; Kofler, Markus; Bramlage, Peter; Deutsch, Cornelia; Thoenes, Martin; Frank, Derk; Romano, Mauro (2017)
    OBJECTIVES: Transaortic transcatheter aortic valve implantation (TAo-TAVI) is a recently developed alternative to transapical (TA) or transfemoral (TF) TAVI. We aimed to analyse the effectiveness and safety of TAo-TAVI as a first line approach and to compare it to patients receiving TAo-TAVI as a last resort, which is current practice. METHODS: ROUTE is a prospective, multicentre registry to assess the clinical outcomes of TAo-TAVI. Patients without contraindications for TA-and TF-TAVI (TAo-first) were compared to patients with contraindications for both of these access routes (TAo-last). Outcome analysis was based on VARC II defined clinical end-points. RESULTS: Three hundred and one patients were included, of which 224 patients met TAo-first and 77 TAo-last criteria. The valve was delivered and catheter retrieved successfully in all patients. In the TAo-first group, rates of conversion to open surgery and requirement for a second valve were low and not different compared to TAo-last patients (1% vs. 3%, P = 0.46 and 1% vs. 3%, P = 0.46, respectively). This was also true for the rate of paravalvular regurgitation (>= moderate: 4% vs. 3%). All-cause mortality at 30-days was 6% vs. 5% (P = 0.76), rates of stroke 2% vs. 0% (P = 0.24), pacemaker implantation (11% vs. 4%, P = 0.093), and life-threatening bleeding 4% vs. 3% (P = 0.70). Valve safety (both 85%, P = 0.98) and clinical efficacy (80% vs. 82%; P = 0.73) did not differ between groups. CONCLUSIONS: Although comparative data to TA and TF procedures were not available in the present analysis, findings suggest that TAo may be considered not only as a last resort strategy when classical access routes are deemed unfeasible, but also as a potential first-line option, with only low rates of paravalvular regurgitation and permanent pacemaker implantation.
  • Hukkinen, Maria; Kerola, Anna; Lohi, Jouko; Heikkilä, Päivi; Merras-Salmio, Laura; Jahnukainen, Timo; Koivusalo, Antti; Jalanko, Hannu; Pakarinen, Mikko P. (2018)
    BACKGROUND: Different treatment policies can influence biliary atresia outcomes, but the pathophysiology of expanding fibrosis occurring even after successful portoenterostomy remains unclear. STUDY DESIGN: Clearance of jaundice (COJ) (bilirubin <20 mu mol/L), native liver survival, and overall survival rates of biliary atresia patients were analyzed before and after national centralization of management, as well as in relation to native liver histopathology of protocol biopsies. RESULTS: Of the 59 patients, 35 were managed after centralization and received standardized postoperative adjuvant therapy, including corticosteroids. After centralization, age at portoenterostomy decreased from 73 days to 54 days (p = 0.014) and COJ rate increased from 42% to 80% (p = 0.005), 5-year native liver survival increased from 38% to 70% (p = 0.014), and 5-year overall survival increased from 68% to 94% (p = 0.007). High-grade portal inflammation at portoenterostomy predicted COJ (odds ratio 3.66; p = 0.011) and slower fibrosis progression (beta = -0.74; p = 0.005). Native liver survival was extended in patients with high-grade portal inflammation (p + 0.002) and in patients whose bilirubin normalized within 3 months (p <0.001). Portal inflammation and cholestasis reduced only after COJ (p<0.001), and persisting ductal reaction, reflected by cytokeratin 7-positive proliferating bile ductules and periportal hepatocytes, correlated with follow-up fibrosis (r = 0.454 to 0.763; p <0.001 to 0.003). Cytokeratin 7 immunopositivity of periportal hepatocytes increased after COJ (p = 0.015) and was the only predictor of follow-up liver fibrosis (beta = 0.36; p = 0.002) in multiple regression. CONCLUSIONS: Biliary atresia outcomes improved significantly after centralization and standardized management. Resolution of cholestasis and reduction of high-grade portal inflammation postoperatively predict slower fibrosis progression and improved native liver survival, and persisting ductal reaction parallels progressive native liver fibrosis despite COJ. (C) 2017 by the American College of Surgeons. Published by Elsevier Inc. All rights reserved.