Real-world data for marginal zone lymphoma patients in the French REALYSA cohort: The REALMA study.

Marginal Zone Lymphoma (MZL) comprises three subtypes: extranodal MZL (EMZL), splenic MZL (SMZL) and nodal MZL (NMZL). Since clinical trials have limited representativeness, there is a need for real-world data (RWD) evidence in MZL. Real-world data in Lymphoma and survival in Adults (REALYSA) is a prospective multicentric French cohort of newly diagnosed lymphoma patients. This study consists of the first abstraction of MZL patients prospectively included in REALYSA between 12/2018 and 01/2021 with at least 1 year of follow-up. It provides a landscape description of clinical characteristics, initial workup, quality of life and first-line therapy performed in routine practice. Among 207 included patients, 122 presented with EMZL, 51 with SMZL and 34 with NMZL. At baseline, median age was 67 years (range 28-96), and patients reported a favorable global health status (75/100 (IQR 58,83)) - which was higher in NMZL and lower in SMZL patients (p = 0.006). 18FDG-PET/CT was frequently performed at initial workup (EMZL 72%, SMZL 73%, NMZL 85%). Active surveillance was the initial management for 58 (28%) patients. The most prescribed therapies were rituximab-chlorambucil in the EMZL population (30%), rituximab monotherapy in the SMZL population (37%) and R-CHOP (24%)/bendamustine-rituximab (15%) in the NMZL population. At end of first line, overall response rate was 93% among treated patients with 75% of complete response. This French nationwide study provided for the first time prospective RWD on clinical characteristics, initial management and treatment response of MZL patients.

Prevalence of pancreatic exocrine insufficiency after pancreatic surgery measured by 13C mixed triglyceride breath test: A prospective cohort study.

BACKGROUND: Patients undergoing pancreatic surgery are at risk of pancreatic exocrine insufficiency (PEI) and needing pancreatic enzyme replacement therapy (PERT). METHODS: This study included 254 patients undergoing pancreatic surgery for oncologic indications. A13C mixed triglyceride breath test was performed immediately preoperative and postoperative. This test analyzes the pancreatic remnant lipase activity measuring 13CO2 in breath samples after a test meal with 1.3-distearyl-(13C-Carboxyl)octanol-glycerol. Cumulative percent dose recovery after 6 h of less than 23% confirms PEI. In addition, PEI was compared between pathology subgroups. RESULTS: In 197 patients undergoing pancreaticoduodenectomy, cPDR-6h decreased significantly from a median of 32.84% before to 15.80% after surgery (p < 0.0001). This decrease in exocrine function was significant in all pathology subgroups except in pancreatic neuroendocrine tumors. Exocrine function decreased most in pancreatic ductal adenocarcinoma (PDAC). In addition, the percentage of patients needing PERT because of PEI increased from 25.9% to 68.0% postoperative (p < 0.001). Overall, patients with an MPD diameter of more than 3 mm had a higher risk of developing postoperative PEI: 62.7% compared to 37.3% (p = 0.009), OR = 3.11. In contrast, the majority of the 57 patients undergoing a distal pancreatectomy did not experience any significant change in exocrine function. CONCLUSIONS: The vast majority of patients undergoing pancreaticoduodenectomy for oncologic indications experience a significant drop in exocrine function, are at high risk of developing pancreatic exocrine insufficiency and consequently need to be treated with pancreatic enzyme replacement therapy. Therefore, systematic screening for pancreatic exocrine insufficiency is needed after pancreaticoduodenectomy.

Prediction of exocrine and endocrine insufficiency after pancreaticoduodenectomy using volumetry.

Objectives: The aim of this study is to evaluate the use of pancreatic volumetric assessment to predict exocrine and endocrine insufficiency after pancreaticoduodenectomy.Methods: Thirty-seven patients who underwent pancreaticoduodenectomy were included in the study. Endocrine function was assessed in all patients without a history of diabetes using an oral glucose tolerance test. A 13C-labeled mixed triglyceride (MTG) breath test evaluated exocrine function before and after resection. Volumetric measurements were performed on CT or MRI.Results: The volumetric measurements could not predict pre- or postoperative diabetes. Moreover, the resected volume was significantly lower in patients who developed diabetes after resection. Comparing patients with a normal and disturbed postoperative MTG, postoperative volumes and parenchymal thickness were significantly different. The parenchymal thickness on postoperative imaging is withheld as a predictive factor (OR = .85 [95% CI .71-1.01], p = .049). The best cutoff value to predict exocrine insufficiency is a parenchymal thickness of less than 11.4 mm (AUC = .76, p = .025, sensitivity = 88.9%, specificity = 70.0%).Conclusions: Pancreatic remnant volumetry and parenchymal thickness measurement after pancreaticoduodenectomy are correlated with exocrine insufficiency, but with limited predictive value. None of the preoperative measurements are withheld to predict postoperative exocrine function. Pre- and postoperative volumetry appear to have no use in predicting postoperative diabetes.

Simultaneous kidney-parathyroid allotransplantation from a single donor after 20 years of tetany: a case report.

Persistent hypocalcemia after total parathyroidectomy and autotransplantation is rare and occasionally has been treated using allotransplantation of parathyroid tissue. We present the case of a 32-year-old woman with terminal renal failure who at age 5 years underwent a first renal transplantation from a brain-dead donor. The graft was lost as a result of acute rejection. Tertiary hypoparathyroidism developed, which was treated with total parathyroidectomy and implantation in the forearm of a standardized amount of parathyroid tissue. The graft failed, and hypoparathyroidism developed. Despite a second implantation of cryopreserved autologous tissue, severe hypocalcemia persisted with a tendency for tetany. Although the patient was highly dependent on high-dose vitamin D(3) (tacalcitol) and calcium supplements, regular paresthesias and tetany developed. At age 9 years, the patient underwent a second renal transplant from a living related donor (her mother). After 18 years, the graft was lost as a result of chronic cyclosporine toxicity and angiosclerosis. Four years later, the patient underwent combined kidney and parathyroid transplantation from a local brain-dead donor. Preservation of the parathyroid glands was in University of Wisconsin solution, with cold ischemia time of 14 hours. Directly after the renal transplantation, parathyroid transplantation was performed, with implantation in the forearm of the total amount of donor parathyroid tissue. Postoperatively, there was recovery of parathyroid function, and the patient was able to discontinue vitamin D and calcium supplements after more than 20 years.

Organ procurement after euthanasia: Belgian experience.

Euthanasia was legalized in Belgium in 2002 for adults under strict conditions. The patient must be in a medically futile condition and of constant and unbearable physical or mental suffering that cannot be alleviated, resulting from a serious and incurable disorder caused by illness or accident. Between 2005 and 2007, 4 patients (3 in Antwerp and 1 in Liège) expressed their will for organ donation after their request for euthanasia was granted. Patients were aged 43 to 50 years and had a debilitating neurologic disease, either after severe cerebrovascular accident or primary progressive multiple sclerosis. Ethical boards requested complete written scenario with informed consent of donor and relatives, clear separation between euthanasia and organ procurement procedure, and all procedures to be performed by senior staff members and nursing staff on a voluntary basis. The euthanasia procedure was performed by three independent physicians in the operating room. After clinical diagnosis of cardiac death, organ procurement was performed by femoral vessel cannulation or quick laparotomy. In 2 patients, the liver, both kidneys, and pancreatic islets (one case) were procured and transplanted; in the other 2 patients, there was additional lung procurement and transplantation. Transplant centers were informed of the nature of the case and the elements of organ procurement. There was primary function of all organs. The involved physicians and transplant teams had the well-discussed opinion that this strong request for organ donation after euthanasia could not be waived. A clear separation between the euthanasia request, the euthanasia procedure, and the organ procurement procedure is necessary.

Patients suffering from acute graft-versus-host disease after bone-marrow transplantation have functional CD4+CD25hiFoxp3+ regulatory T cells.

Acute Graft-Versus-Host Disease (aGVHD), mediated by CD4(+) and CD8(+) effector T cells, is a life-threatening complication in hematopoietic stem cell (HSC) transplantation. Naturally-occurring CD4(+)CD25(hi)(Foxp3(+)) regulatory T cells (T(reg)) have been shown to modulate tolerance to aGVHD in murine graft models. In this report, we investigated their role in the prevention of aGVHD in patients transplanted with bone-marrow-derived HSC. When CD4(+)CD25(hi)Foxp3(+) T cells were isolated from bone-marrow grafts, they showed no suppressive activity. The analysis of their function in patients suffering from aGVHD after transplantation revealed a gain of suppressive activity indicating their inability to control the aGVHD induction. Thus, our findings clearly demonstrate that CD4(+)CD25(+) and CD4(+)CD25(hi)Foxp3(+) T cells, when administered in steady-state physiological conditions, do not influence the outcome of aGVHD after bone-marrow transplantation.

Estimation of the future remnant liver function is a better tool to predict post-hepatectomy liver failure than platelet-based liver scores.

INTRODUCTION: Recently, there has been increasing interest in the preoperative prediction and prevention of post-hepatectomy liver failure (PHLF). This is a particular concern in colorectal liver metastases (CRLM), when surgery follows potentially hepatotoxic chemotherapy. Platelet-based liver scores (PBLS) such as APRI and FIB-4 are predictive of chemotherapy-associated liver injury (CALI) and PHLF. Estimation of the future liver remnant function (eFLRF) by combining 99mTc-Mebrofenin Hepatobiliary Scintigraphy (HBSBSA) with future liver remnant volume ratio (FLRV%), is predictive of PHLF and related mortality. We hypothesized that a HBSBSA based formula was a better predictor for PHLF than PBLS in chemotherapy-pretreated CRLM. METHODS: Between 2012 and 2016, 140 patients underwent liver resection for CRLM following systemic therapy. HBSBSA, FLRV%, eFLRF and PBLS were calculated and compared for their value in predicting PHLF. RESULTS: eFLRF and FLRV% had a better predictive value for PHLF than HBSBSA alone and APRI and FIB-4 (AUC = 0.800, 0.843 versus 0.652, 0.635 and 0.658 respectively). In a subgroup analysis (Oxaliplatin all, Oxaliplatin ≥ 6 cycles, Irinotecan all and Irinotecan ≥ 6 cycles), eFLRF was the only factor predictive for PHLF in all subgroups (all: p ≤ 0.05). Prediction of HBSBSA for chemotherapy associated steato-hepatitis (CASH) reached almost significance (p = 0.06). FIB-4 was predictive for sinusoidal obstruction syndrome (SOS) (p = 0.011). Only weak correlation was found between HBSBSA and PBLS. CONCLUSION: eFLRF is a better predictor of PHLF than PBLS or HBSBSA alone. PBLS seem to measure other aspects of liver function or damage than HBSBSA.

Single-Center Case Series of Donor-Related Malignancies: Rare Cases With Tremendous Impact.

BACKGROUND: Donor-related malignancy is a rare complication of organ transplantation. METHODS: In this case series, we discuss three cases of donor-related cancers in kidney transplant recipients who were registered in our center between 1979 and 2015. They account for an incidence of 0.29% of donor-related malignancies of a total of 1015 transplanted kidney grafts (deceased and living donors). The three cases that we describe presented in different ways and with different severity, although the response to the initiated treatment was comparable. RESULTS: All three patients not only survived their cancer episode but also had a complete oncological remission and underwent successful second kidney transplantation, accounting for a 100% survival rate in our small cohort. CONCLUSIONS: Despite the very low incidence of this complication, transplant clinicians must be aware of the occurrence of donor-related malignancies when selecting a donor and should be able to diagnose and treat a case of donor-related cancer.

Fibrous intimal thickening at implantation as a risk factor for the outcome of cadaveric renal allografts.

BACKGROUND: During the past decade, the donor age of cadaveric renal allografts steadily increased. Because cerebrovascular injury is the main cause of death in this donor population, an increased prevalence of atherosclerotic lesions in the retrieved grafts could be anticipated. In a prospective study, we investigated the predictive value of morphologic lesions at implantation for the functional and morphologic outcome of cadaveric renal allografts at 1 1/2 years. METHODS: In 50 consecutive adult recipients of a cadaveric renal allograft, under cyclosporine-based regimen, implantation biopsies and subsequent protocol biopsies at 18 months were performed, and morphometrically analyzed for the extent of glomerulosclerosis, interstitial fibrosis, and atherosclerosis. Risk factors were assessed at implantation and during the subsequent observation period of 18 months. Endpoints for this study were: the 24-hr creatinine clearance (normalized for body surface area) and the fractional interstitial volume at 1 1/2 years. RESULTS: In multivariate analysis, fibrous intimal thickening at implantation (FIT) was the main determinant of the functional and morphologic outcome at 1 1/2 years. FIT represented a relative risk of 4.55 for interstitial fibrosis (95% CI=1.855-11.138), and 1.89 for impaired renal function (95% CI=1.185-3.007) at 1 1/2 years. FIT adversely affected fractional interstitial volume at 1 1/2 years (34.3 vs. 27.7%, P=0.004), as well as renal function (54 vs. 68 ml/min/1.73 m2, P=0.028). CONCLUSIONS: Fibrous intimal thickening at implantation is a determinant risk factor for the functional and morphologic outcome of cadaveric renal allografts at 1 1/2 years.

Intragastric erosion of laparoscopic adjustable silicone gastric band.

Intragastric erosion of the adjustable silicone gastric band (ASGB) is a rare but severe complication of gastric banding, often leading to reoperation. We describe our experience with 4 cases referred to us. The best timing of removal and the choice of another bariatric procedure is still controversial. We advise to wait until migration of the band into the lumen is complete. With removal of the ASGB if another weight reduction procedure is advisable, conversion to a biliopancreatic diversion is possible.

Robot-assisted choledochotomy: feasibility.

Because clearing stones from the common bile duct is demanding with conventional laparoscopic techniques, the "da Vinci" robotic system can be of additional value in inserting a kehr drain and suturing the common bile duct. As far as we could ascertain, we report the first case of a laparoscopic choledochotomy with the assistance of this robotic system. Thanks to it, we were able to suture the common bile duct meticulously.

Rectovaginal fistulas.

The treatment of rectovaginal fistulas is controversial. The choice of the technique used for repair depends on many factors. Therefore the classification, etiology and treatment are discussed, in order to help decision making in the management of this troublesome disease.

Laparoscopic-assisted gastric button placement: a valuable alternative for a percutaneous endoscopic gastrostomy (PEG).

A percutaneous endoscopic gastrostomy remains the first choice when oral feeding is difficult. In some patients however an endoscopic placement of a gastrostomy tube is not possible. As an alternative, a laparoscopic-assisted insertion of a gastric button was performed to provide enteral feeding in seven patients. Enteral feeding could be resumed within one or two days after the procedure and no complications were encountered. This minimal invasive technique has certain advantages over a surgical gastrostomy by laparotomy. Therefore, a laparoscopically inserted gastric button should be considered a valuable alternative if percutaneous endoscopic gastrostomy is no longer possible.

Laparoscopic adrenalectomy with the aid of the AESOP 2000 robot.

The authors describe a left adrenalectomy for Cushing's disease with the aid of an AESOP (Automated Endoscopic System for Optimal Positioning) 2000 voice controlled robot. This device facilitated the procedure by producing a constant, stable picture allowing the operative team to concentrate principally on the dissection.

[Leiomyoma and leiomyosarcoma of the gastrointestinal system: experience with 5 patients and literature review]. / Leiomyomen en leiomyosarcomen van het gastro-intestinaal stelsel: ervaring met 5 patiënten en literatuuroverzicht.

Leiomyomas and leiomyosarcomas of the gastro-intestinal tract are quite uncommon tumours. Clinical findings are aspecific: abdominal pain and gastrointestinal bleeding. Diagnosis is made by X-ray with contrast, endoscopy and CT-scan. Although biopsies taken during endoscopy are considered to be useless, our experiences showed an accurate diagnosis with biopsies. Surgery is the only treatment. The aim is to reduce the mass of tumour as much as possible; second look operations with careful debulking enhances the survival in case of recidive. Resection of lymph-nodes is useless. Radiotherapy nor chemotherapy are proven to be useful. The grade of malignity determines the prognosis. The liver and the abdominal cavity are the predilected localisation for metastasis. Metastasis in lymph-nodes is uncommon. Five year survival is about 50%.

Giant ovarian cyst.

Case report of a patient with a giant ovarian cyst. On the basis of literature data, surgery and histology problems, as well as oncological therapy are discussed.

Congenital right-sided diaphragmatic hernia in an elderly patient. A case report.

Congenital diaphragmatic hernia is a rare condition in adulthood. It is even more exceptional when located on the right side. We describe a case of right-sided congenital diaphragmatic hernia in a 74-year old woman. The diagnosis was only made when the patient developed an acute intestinal obstruction after a laparotomy for a gynaecological benign tumour. The treatment of this condition is discussed. In our opinion, in elderly, the advantages of an elective operation in asymptomatic congenital diaphragmatic hernia have to outweigh the risks of the operation. However, if the patient shows symptoms of pulmonary dysfunction or motility disorders of the gut, or even when he has to undergo a laparotomy for a different reason, we advise to repair the hernia in order to prevent complications.

[Small intestinal ulceration and stenosis induced by non-steroidal anti-inflammatory agents]. / Dundarmulceratie en -stenose geïnduceerd door niet-steroïdale anti-inflammatoire farmaca.

UNLABELLED: We report a case who developed a small bowel ulceration in a long segment of distal duodenum and proximal jejunum, a few days after he had taken high doses of diclofenac. After a month, it was complicated by a stenosis of the same segment. Nonsteroidal antiinflammatory drugs have recently been recognised as a possible etiologic factor of small bowel ulcerations. Clinical findings are aspecific. Complications of small bowel ulcerations are: obstruction, perforation or bleeding. Those have to be treated by surgery. We proposed a physiopathologic model and correlated it with histological findings. CONCLUSION: careful prescription of those drugs has to be pursued and one should think about a small bowel ulceration in a patient with an abdominal syndrome, who takes non-steroidal antiinflammatory drugs.

The multicenter Belgian survey on liver transplantation for hepatocellular failure after bariatric surgery.

The prevalence of obesity has grown dramatically over the last decades, with nonalcoholic steatohepatitis increasingly observed. Therapeutic options for morbid obesity include bariatric surgery. Fatal liver failure (LF) has been recorded after jejunoileal bypass (JIB) but is controversial after biliopancreatic diversion (BPD, Scopinaro operation). We performed a survey on the frequency of liver transplantation (LT) after bariatric surgery in Belgium. An enquiry was sent to all Belgian liver transplant centers to investigate the occurrence of subacute and chronic LF after bariatric surgery. After weight-reduction surgery, 10 patients in 3 Belgian transplant centers were listed for LT due to severe hepatocellular failure. Nine of them had undergone a Scopinaro operation and 1 a jejunoileal bypass. The median time to develop LF was 5 years. The patient with JIB developed chronic LF after 25 years. Seven patients were transplanted; two died awaiting a graft and one is still on the waiting list. After LT, 1 patient developed rapid reappearance of LF at 10 months, requiring retransplantation. Two recipients died after LT because of multiorgan failure shortly after transplantation. In another case, a de novo cancer was fatal at 6 years' follow-up. The remaining recipients were doing well. According to this survey, the BPD operation carries a potential risk of LF. However, because there were only 10 cases, we remain unaware of the actual incidence of Scopinaro operation-induced LF. We advise strict follow-up of liver function and timely dismantling of BPD.