Publications by authors named "Fabry H"

JCO Whether adjuvant hyperthermic intraperitoneal chemotherapy (HIPEC) might prevent peritoneal metastases after curative surgery for high-risk colon cancer is an ongoing debate. This study aimed to determine 5-year oncologic outcomes of the randomized multicenter COLOPEC trial, which included patients with clinical or pathologic T4N0-2M0 or perforated colon cancer and randomly assigned (1:1) to either adjuvant systemic chemotherapy and HIPEC (n = 100) or adjuvant systemic chemotherapy alone (n = 102). HIPEC was performed using a one-time administration of oxaliplatin (460 mg/m, 30 minutes, 42°C, concurrent fluorouracil/leucovorin intravenously), either simultaneously (9%) or within 5-8 weeks (91%) after primary tumor resection.

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Background: Involved lateral lymph nodes (LLNs) have been associated with increased local recurrence (LR) and ipsi-lateral LR (LLR) rates. However, consensus regarding the indication and type of surgical treatment for suspicious LLNs is lacking. This study evaluated the surgical treatment of LLNs in an untrained setting at a national level.

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Article Synopsis
  • Abdominoperineal resection (APR) for rectal cancer traditionally uses primary perineal wound closure, but new methods like the gluteal turnover flap may enhance healing by providing well-vascularized tissue without donor site scars.
  • This multicenter randomized controlled trial will involve 160 patients undergoing APR, comparing the gluteal flap technique to standard closure, with patients and assessors blinded to the technique used.
  • The primary goal is to determine the rate of uncomplicated perineal wound healing at 30 days, with secondary measures including complications, quality of life, and urogenital function, aiming to increase healing rates significantly from 65% to 85%.
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Background: In the recent years two innovative approaches have become available for minimally invasive en bloc resections of large non-pedunculated rectal lesions (polyps and early cancers). One is Transanal Minimally Invasive Surgery (TAMIS), the other is Endoscopic Submucosal Dissection (ESD). Both techniques are standard of care, but a direct randomised comparison is lacking.

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Background: Transanal total mesorectal excision (TaTME) has been proposed as an approach in patients with mid and low rectal cancer. The TaTME procedure has been introduced in the Netherlands in a structured training pathway, including proctoring. This study evaluated the local recurrence rate during the implementation phase of TaTME.

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Background: Nearly a quarter of patients with locally advanced (T4 stage) or perforated colon cancer are at risk of developing peritoneal metastases, often without curative treatment options. We aimed to determine the efficacy of adjuvant hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with locally advanced colon cancer.

Methods: This multicentre, open-label trial was done in nine hospitals that specialised in HIPEC in the Netherlands.

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Background And Objectives: It is increasingly accepted that quality of colon cancer surgery might be secured by combining volume standards with audit implementation. However, debate remains about other structural factors also influencing this quality, such as hospital teaching status. This study evaluates short-term outcomes after colon cancer surgery of patients treated in general, teaching or academic hospitals.

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Introduction: Next to locoregional control, good cosmetic outcome is one of the main goals of breast conserving treatment (BCT) for breast cancer surgery. Factors affecting cosmetic outcome are well known. The sentinel node (SN) procedure avoids lymphedema in the breast, which might influence cosmetic outcome.

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The aim of this study was to investigate closely the nature of premalignant lesions that occur in prophylactically removed breast tissue from patients at hereditary high risk of breast cancer. Breast tissues obtained from 41 patients who underwent prophylactic mastectomy (pM) because of a hereditary high risk of breast cancer and control tissues from 82 age-matched healthy controls who underwent breast reduction surgery were screened for premalignant lesions. Premalignant and malignant lesions were more frequent (p = 0.

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Purpose: To evaluate the rate of axillary recurrences in sentinel node (SN) negative breast cancer patients without further axillary lymph node dissection (ALND).

Patients And Methods: Between October 1994 and November 1999, all SN negative breast cancer patients who did not underwent complete ALND were enrolled in this prospective study. SN biopsy was performed by using the triple technique which combines preoperative lymphoscintigraphy, intraoperative use of blue dye, and a handheld gamma probe to visualize and localize the SN.

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Background And Objectives: Preoperative lymphoscintigraphy contributes highly to the accuracy of the sentinel node procedure. Besides routing towards the axilla, in a number of patients additional parasternal focal accumulation may be observed. So far the clinical consequences of this parasternal uptake remains unclarified, i.

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Since its introduction in the early 1990s, the sentinel node (SN) concept in breast cancer has been validated by many studies. Because SN biopsy in breast cancer enables the identification of node-negative axillae, the potential morbidity of an axillary lymph node dissection (ALND) can be avoided. The SN procedure is still surrounded by many variables and uncertainties, such as the clinical relevance of micrometastases.

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This paper gives a brief historical overview of Fabry disease. First described in 1898 and called angiokeratoma corporis diffusum, a succession of observations and pioneering clinical research over the past 100 years has led to our present understanding of the disease. The major milestones during this time are described, as is the work that resulted in the successful introduction of enzyme replacement therapy.

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Background: The wire-guided excision of nonpalpable breast cancer often results in tumor resections with inadequate margins. This prospective, randomized trial was undertaken to investigate whether intraoperative ultrasound (US) guidance enables a better margin clearance than the wire-guided technique in the breast-conserving treatment of nonpalpable breast cancers.

Methods: Patients with a preoperative histological diagnosis of nonpalpable breast cancer that could be visualized both with US and mammography were included.

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Background: Laparoscopic adjustable gastric banding is a safe and effective treatment method for morbid obesity. Injection port dislocation, tube perforation and access port infection are generally classified among the minor complications, although they can require a reoperation at the port-site or even at the level of the band which may have to be removed. We designed a technique to fix the port, that can avoid unnecessary complications.

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Background: Biliopancreatic diversion (BPD) according to Scopinaro's method is a safe and effective technique for treatment of morbid obesity. In this operation a side-to-side enteroenterostomy is created 60 cm proximal to the ileocecal valve. Malabsorption of fat and starch is one of the main goals of the BPD.

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Cryosurgery is considered an alternative method in the treatment of basal cell carcinomas of the face and usually leads to excellent functional and cosmetic results. This technique can be modified depending on the number of freeze-thaw cycles. According to our experiences (220 cases), a single freeze-thaw cycle is indicated for those basal cell carcinomas which do not go beyond the middle dermis; these cases were characterized by fast wound healing and a very satisfying cosmetic outcome.

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The case of a 14-year-old boy who had oral ulcers with histologic proof of granulomatous disease nine months before the diagnosis of intestinal Crohn's disease is presented. Additional extraintestinal manifestations of this case were cheilitis, anal fissures, and "metastatic" disease to the umbilicus. The diagnosis was established after the onset of abdominal symptoms.

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Hallux valgus leads to lateral deviation of the big toe's longitudinal axis. In consequence, the forces working on the tip of the toe during unrolling of the foot are shifted in medial direction. As a result, subungual clavi, unguis incarnatus, unguis convolutus, or laterally turning onychogryposis like a cork-screw develop.

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A multicentre, between-patient, comparative trial was carried out by nine dermatologists in the Federal Republic of Germany to compare the efficacy and tolerability of 0.05 halometasone ointment with those of an ointment, containing 0.25% fluocortolone + 0.

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