Association of 1-deoxy-sphingolipids with steatosis but not steatohepatitis nor fibrosis in non-alcoholic fatty liver disease.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is the most important cause of chronic liver disease in the western world. Steatosis can be accompanied by inflammation and cell damage (non-alcoholic steatohepatitis, NASH), and even liver fibrosis. Sphingolipids are a heterogeneous class of lipids and essential components of the plasma membrane and plasma lipoproteins. The atypical class of deoxy-sphingolipids has been implicated in the metabolic syndrome and type 2 diabetes. AIM: To determine if circulating (deoxy)sphingolipids are associated with NAFLD and its different entities, steatosis, inflammatory changes (inflammation and ballooning) and fibrosis. METHODS: Sphingolipids were analysed by LC-MS after hydrolysing the N-acyl and O-linked headgroups in plasma of obese adults who underwent a liver biopsy in suspicion of NAFLD. RESULTS: Two-hundred and eighty-eight patients were included. There was no association between typical sphingolipids and NAFLD and its different entities. There was a significant association between the presence of steatosis and the concentrations of deoxy-sphinganine [exp(B) 11.163 with CI (3.432, 36.306) and p < 0.001] and deoxy-sphingosine [exp(B) 8.486 with CI (3.437, 20.949) and p < 0.001]. There was no association between these deoxy-sphingolipids and activity of the steatohepatitis, nor was there any association with fibrosis. Differences in deoxy-sphingolipids also correlated independently with the presence of the metabolic syndrome, but not diabetes. CONCLUSION: Deoxy-sphingolipids are elevated in patients with steatosis compared to those without fatty liver, but not different between the different NAFLD subtypes, suggesting that deoxy-sphingolipid bases might be involved in steatogenesis, but not in the further progression of NAFLD to NASH nor in fibrogenesis.

Vague abdominal pain after Roux-en-Y gastric bypass: not always an internal herniation: case report and literature review.

Background: Roux-en-Y gastric bypass is a frequently carried out bariatric procedure, proven to be effective in the management of obesity and its accompanying health issues. Following its popularity, admission to the emergency department for abdominal pain is often seen with known early and late onset causes. We present a case of a young woman with vague abdominal pain years after her gastric bypass, who eventually underwent a resection of a 'candy cane' like biliopancreatic blind loop.Methods: A healthy 23-year-old woman has been suffering of vague abdominal complaints after a gastric bypass procedure 4 years earlier. Postprandial pain, diarrhoea and abdominal distension were present at a daily to weekly basis. Several investigations and management options were administered by surgeons, gastroenterologists as well as endocrinologists. On a performed explorative laparoscopy, a large blind loop at the entero-enteric anastomosis was seen and resected.Results: At current follow-up of 15 months the resection of the candy cane like blind end of the biliopancreatic loop resulted in a complete withdrawal of our patient's symptoms. A tentative diagnosis of bacterial overgrowth in the blind loop was made.Conclusions: Abdominal pain after gastric bypass is a frequent cause of admission to the emergency department. Besides the more serious complications, internal hernia is often withheld as possible diagnosis in the differential diagnosis of late onset, postprandial epigastric pain. This case report highlights another possibility. At initial surgery, a candy cane shaped blind loop should be avoided both at the gastro-jejunal as well as the entero-enteric anastomosis.

Perineal hernia mesh repair: a fixation with glue, sutures and tacks. How to do it.

Introduction: Many different approaches for the repair of a perineal hernia (PH) after abdomino-perineal rectum amputation (APR) have been described throughout the years. Literature shows us that the use of a mesh had the best results with relatively low rates of recurrence compared to primary suture. Yet there is still no consensus on the best technique for fixation of the mesh in the perineal cavity. Methods: We want to introduce an addition to the surgical repair technique, using laparoscopic glue to ensure a solid fixation of the mesh. This is to prevent small bowel from sliding into the perineal area due to the high abdominal pressure when standing and to avoid the difficulties and risks of laparoscopical suturing in this area. Results: This use of glue for fixation of the mesh has been successful in the three cases described, with no recurrence after one-year follow-up. Conclusion: A recurrent PH can be prevented by a solid fixation of the mesh using the technique of a glued mesh repair. This technique shows to be easy, fast and without recurrence.

The Belgian Association for Study of the Liver Guidance Document on the Management of Adult and Paediatric Non-Alcoholic Fatty Liver Disease.

Non-Alcoholic Fatty Liver Disease (NAFLD) is highly prevalent and associated with considerable liver-related and non-liverrelated morbidity and mortality. There is, however, a lot of uncertainty on how to handle NAFLD in clinical practice. The current guidance document, compiled under the aegis of the Belgian Association for the Study of the Liver by a panel of experts in NAFLD, from a broad range of different specialties, covers many questions encountered in daily clinical practice regarding diagnosis, screening, therapy and follow-up in adult and paediatric patients. Guidance statements in this document are based on the available evidence whenever possible. In case of absence of evidence or inconsistency of the data, guidance statements were formulated based on consensus of the expert panel. This guidance document is intended as a help for clinicians (general practitioners and all involved specialties) to implement the most recent evidence and insights in the field of NAFLD within a Belgian perspective.

Intussusception in pregnancy after gastric bypass: a case report.

Laparoscopic Roux-en-Y gastric bypass (RYGB) remains the gold standard procedure in obesity surgery and is mostly performed in young women of reproductive age. Since the worldwide prevalence of obesity is increasing and fertility improves after surgery, more complications in the pregnant population will emerge. The differential diagnosis of acute abdominal pain in patients with a history of gastric bypass is rather broad and includes mainly anastomotic ulcers, leaks, and small bowel obstructions. Early diagnosis and treatment of these complications is of utmost importance and should be performed on a multidisciplinary basis. Whether surgery should be performed by laparoscopy or laparotomy remains subject of discussion. We report a case of a 29-year-old pregnant woman at 33 + 5 weeks gestational age, presenting with an intussusception after RYGB. A successful surgical reduction was performed by laparotomy.

The zebrafish mutant lessen: an experimental model for congenital enteric neuropathies.

BACKGROUND: Congenital enteric neuropathies of the distal intestine (CEN) are characterized by the partial or complete absence of enteric neurons. Over the last decade, zebrafish has emerged as a leading model organism in experimental research. Our aim was to demonstrate that the mutant zebrafish, lessen, expressing CEN characteristics, is an equally valuable animal model alongside mammalian models for CEN, by studying its enteric phenotype. METHODS: The effect of the lessen mutation on the development of the enteric nervous system (ENS), interstitial cells of Cajal (ICC), and intestinal motility in each intestinal region of mutant and wild-type (wt) zebrafish embryos at 3-6 dpf, was analyzed by immunofluorescent detection of neurochemical markers and motility assays. KEY RESULTS: Development of intestinal motility in the mutant was delayed and the majority of the observed contractions were disturbed. A significant disturbance in ENS development resulted in a distal intestine that was almost free of neuronal elements, in reduced neuronal density in the proximal and mid-intestine, and in a defect in the expression of neurochemical markers. Furthermore, markedly disturbed development of ICC gave rise to a less dense network of ICC. CONCLUSIONS & INFERENCES: The observed alterations in intestinal motility, intrinsic innervation and ICC network of the mutant in comparison with the wt zebrafish, are similar to those seen in the oligo- and aganglionic regions of the intestine of CEN patients. It is concluded that the zebrafish mutant lessen is an appropriate animal model to investigate CEN.

Benefits of Prophylactic Proton Pump Inhibitors after Roux-en-Y Gastric Bypass Surgery: A Retrospective Study.

BACKGROUND: Worldwide, proton pump inhibitors (PPIs) are frequently used to prevent marginal ulcers after Roux-en-Y gastric bypass (RYGB) surgery. The aim of this retrospective study was to assess the prophylactic value of PPIs after a RYGB. METHODS: We retrospectively collected data on 200 patients with morbid obesity, who underwent RYGB surgery between August 2012 and August 2013. The patients were further divided into two groups according to whether or not they had been given prophylactic PPIs after the RYGB. Marginal ulcers were diagnosed based on reported symptoms and confirmed by endoscopy. RESULTS: The average follow-up time was 6.7 months. Postoperative gastroscopy was performed in 23 patients. A marginal ulcer was diagnosed in 13 patients. Only four of these patients were taking prophylactic PPIs, of which three patients had already started taking them prior to surgery. Although marginal ulcers occurred less frequently in patients undergoing prophylactic treatment with PPIs (p-value 0.200), we were unable to demonstrate any statistically significant reduction in the number of ulcers. In addition, major risk factors such as nicotine abuse, NSAIDS and H. pylori did not appear to influence the incidence. CONCLUSIONS: Although our results suggest that PPIs reduce the incidence of marginal ulcers, we were unable to confirm this statistically. Our study was limited by the small study population and short follow-up period. In light of the lack of available scientific evidence and the growing problem of obesity, we believe that robust, large-scale prospective studies are necessary.

The Development of an Electronic Portfolio for Postgraduate Surgical Training in Flanders.

BACKGROUND: Contemporary surgical postgraduate training is characterized by clear outcomes for the profession and an assessment program that shows that trainees master these outcomes. The tool used to collect assessment and feedback instruments is the portfolio, nowadays used in many countries worldwide. METHODS: The four Flemish surgical coordinators, together with experts from different universities, devised an electronic portfolio. This portfolio holds both the logbook, as imposed by the evaluation committee and assessment instruments used for the Master in Specialized Medicine. RESULTS: The e-portfolio is now used by a number of surgical trainees and has been approved by the evaluation committee. In 2015, all Flemish surgical trainees will be using one and the same e-portfolio. CONCLUSIONS: Although the e-portfolio for surgical training has now been devised and accepted by all major parties involved, a lot of work has to be done to implement the instrument. As resident duty hours show no improvement on education in surgery (but rather a perception of worsened education) surgery training is fazing huge challenges.

Interrater and intrarater reliability of the Semmes Weinstein aesthesiometer to assess touch pressure threshold in burn scars.

Burn scars are frequently accompanied with sensory deficits often remaining present months or even years after injury. Clinimetric properties of assessment tools remain understudied within burn literature. Tactile sense of touch can be examined with the touch pressure threshold (TPT) method using the Semmes Weinstein monofilament test (SWMT). There is in recent research no consensus on the exact measurement procedure when using the SWMT. The aim of this paper was to determine the interrater and intrarater reliability of TPT within burn scars and healthy controls using the 'ascending descending' measurement procedure. We used the newly developed guidelines for reporting reliability and agreement studies (GRRAS) as a basis to report this reliability study. In total 36 individuals were tested; a healthy control group and a scar group. The interrater reliability was excellent in the scar group (ICC=0.908/SEM=0.21) and fair to good in the control group (ICC=0.731/SEM=0.12). In the scar group intrarater ICC value was excellent (ICC=0.822/SEM=0.33). Within the control group also an excellent intrarater reliability (ICC=0.807/SEM=0.27) was found. In conclusion this study shows that the SWMT with the 'ascending descending' measurement procedure is a feasible and reliable objective measure to evaluate TPT in (older) upper extremities burn scars as well as in healthy skin.

The Development of an Electronic Portfolio for Postgraduate Surgical Training in Flanders.

BACKGROUND: Contemporary surgical postgraduate training is characterized by clear outcomes for the profession and an assessment program that shows that trainees master these outcomes. The tool used to collect assessment and feedback instruments is the portfolio, nowadays used in many countries worldwide. METHODS: The four Flemish surgical coordinators, together with experts from different universities, devised an electronic portfolio. This portfolio holds both the logbook, as imposed by the evaluation committee and assessment instruments used for the Master in Specialized Medicine. RESULTS: The e-portfolio is now used by a number of surgical trainees and has been approved by the evaluation committee. In 1015, all Flemish surgical trainees will be using one and the same e-portfolio. CONCLUSION: Although the e-portfolio for surgical training has now been devised and accepted by all major parties involved, a lot of work has to be done to implement the instrument. As resident duty hours show no improvement on education in surgery (but rather a perception of worsened education) surgery training is fazing huge challenges.

Classification of quality of life subscales within the ICF framework in burn research: identifying overlaps and gaps.

OBJECTIVE: Quality of life (QOL) is one of the leading outcomes in burn care research. This study classifies subscales of common QOL measures within the International Classification of Functioning disability and health (ICF) framework to determine to which extent the measures are complementary or overlapping and to investigate whether the instruments are able to describe the full spectrum of patients' functioning. METHODS: A literature search was performed to determine the most frequently used questionnaires in burn research. The subscales of the three mostly used questionnaires were classified within the ICF framework. RESULTS: Two generic measures, the Short Form-36 items (SF-36) and the European Quality of Life 5 Dimensions (EQ-5D), and a disease specific measure, the Burn Specific Health Scale-Brief (BSHS-B), were analyzed. The BSHS-B covered most domains and was the only scale that included personal factors. The SF-36 included only one domain in the activity limitations and similar to the EQ-5D no contextual factors were included. Environmental factors were not addressed in the questionnaires, even though these may have an impact on the quality of life in patients with burns. CONCLUSION: To capture the full spectrum of dysfunctioning a combination of the BSHS-B with a generic questionnaire seems obligatory. However still some domains of functioning remain uncovered.

A gene variant of PNPLA3, but not of APOC3, is associated with histological parameters of NAFLD in an obese population.

OBJECTIVE: Mechanisms explaining the relationship in non-alcoholic fatty liver disease (NAFLD), obesity, and insulin resistance are poorly understood. A genetic basis has been suggested. We studied the association between the genes patatin-like phospholipase domain-containing protein 3 (PNPLA3) and apolipoprotein C3 (APOC3) and metabolic and histological parameters of NAFLD in obese patients. DESIGN AND METHODS: Overweight and obese patients underwent a metabolic and liver assessment. If NAFLD was suspected, liver biopsy was proposed. APOC3 variant rs2854117 and PNPLA3 variant rs738409 were genotyped. RESULTS: Four hundred seventy patients were included (61.1% had liver biopsy). The percentage of patients with non-alcoholic steatohepatitis (NASH) was significantly different according to the PNPLA3 variant. After adjustment for age and body mass index, the PNPLA3 variant was associated with alanine aminotransferase (P < 0.001) and aspartate aminotransferase (P < 0.001). The PNPLA3 variant was associated with more severe features of steatohepatitis: steatosis (P < 0.001), lobular inflammation (P < 0.001), and ballooning (P = 0.002), but not with liver fibrosis, anthropometry, or insulin resistance. No significant difference in liver histology, anthropometric, or metabolic parameters was found between carriers and non-carriers of the APOC3 variant. CONCLUSIONS: PNPLA3 polymorphism rs738409 was associated with NASH and the severity of necroinflammatory changes independently of metabolic factors. No association between APOC3 gene variant rs2854117 and histological or metabolic parameters of NAFLD was found.

Multiple abdominal cysts in a patient with Gorlin-Goltz syndrome: a case report.

A rare case of symptomatic mesenteric cysts in a patient with Gorlin-Goltz syndrome, associated with various neoplasms, is presented. The patient, known with Gorlin-Goltz syndrome, consulted with increasingly severe abdominal pain and large abdominal cysts. At surgery, the cysts were excised and the postoperative course was uneventful. In conclusion, this case reminds clinicians to always maintain a wide differential diagnosis when dealing with patients known with Gorlin-Goltz syndrome.

Ectopic pancreatic tissue at the umbilicus in childhood: a case report and review of the literature.

Ectopic pancreatic tissue at the umbilicus is very rare. To our best knowledge, only fourteen cases of ectopic pancreatic tissue at the umbilicus are reported. In this paper we present the case of a two-year-old boy with an abrasion at the umbilicus. He had a poorly healing scar that started bleeding after recurrent injuries. Abdominal ultrasound revealed an unclear cystic structure with no communication to intra-abdominal structures. Surgical resection was performed without complications. Histology diagnosed an ectopic pancreatic tissue with reactive epidermal changes. We present a review of the literature and the clinical manifestations and treatment of the previously reported fourteen cases.

Giant adrenal myelolipoma: a case report.

Adrenal myelolipomas are rare, benign, hormonally inactive tumours composed of mature adipose tissue and haematopoietic elements. Currently, most diagnosed tumours are discovered incidentally because of modern imaging. Myelolipomas are usually asymptomatic, but symptoms such as abdominal pain, haematuria and abdominal mass are described as the result of tumour bulk, haemorrhage or tumour necrosis. Myelolipomas are usually small, although there are descriptions of giant myelolipomas in the literature. We report the case of a giant adrenal myelolipoma in a 79-year-old female who presented with epigastric pain and discomfort. The resected tumour weighed 1777 g and measured 20.5 x 18 x 9.0 cm.

Visceral adiposity and insulin resistance are independent predictors of the presence of non-cirrhotic NAFLD-related portal hypertension.

INTRODUCTION: We previously demonstrated in an animal model that steatosis, in the absence of fibrosis, induces a significant rise in portal pressure, indicating substantial changes in liver hemodynamics. As assessment of portal pressure is an invasive procedure, non-invasive parameters are needed to identify patients at risk. AIMS: To study the portal pressure in nonalcoholic fatty liver disease patients and to identify factors that are possibly related to steatosis-induced changes in liver hemodynamics. MATERIALS AND METHODS: Patients presenting with a problem of overweight or obesity, and in whom non-invasive investigations showed signs of liver involvement, were proposed for transjugular hepatic vein catheterization and liver biopsy. The biopsy was scored according to the Nonalcoholic Steatohepatitis Clinical Research Network Scoring System. RESULTS: A total of 50 consecutive patients were studied. Their mean age was 47.9 ± 1.8 years; 31 (62%) were female. Hepatic venous pressure gradient was normal in 36 (72%) and elevated in 14 (28%) patients. The degree of steatosis was the only histological parameter that differed significantly between the two groups (P=0.016), and was a predictor of the presence of portal hypertension (PHT) in regression analysis (P=0.010). Comparing normal versus portal hypertensive patients, waist circumference (117 ± 2 versus 128 ± 4 cm, P=0.005), waist-hip ratio (0.96 ± 0.06 versus 1.04 ± 0.03, P=0.003), visceral fat (229 ± 15 versus 292 ± 35 cm(2), P=0.022), fasting insulin (15.4 ± 1.7 versus 21.8 ± 2.4 µU ml(-1), P=0.032), fasting c-peptide (1.22 ± 0.06 versus 1.49 ± 0.09 nmol l(-1), P=0.035) and homeostasis model assessment-insulin resistance (HOMA IR) (3.28 ± 0.29 versus 4.81 ± 0.57, P=0.019) were significantly higher. Age, gender, liver enzymes, ferritin and high-sensitive C-reactive protein were not significantly different. In regression analysis, waist circumference (P=0.008) and HOMA IR (P=0.043) were independent predictors of PHT. CONCLUSIONS: Estimates of both visceral adiposity and IR are predictors for the presence of PHT, related to the degree of steatosis, and may help in identifying patients who are at risk of developing steatosis-related complications.

Encapsulating peritoneal sclerosis in a peritoneal dialysis patient with prune-belly syndrome: a case report.

This case describes a prune-belly syndrome patient who had a kidney transplantation and was diagnosed with Encapsulating Peritoneal Sclerosis (EPS), a rare but potentially fatal condition, mostly associated with Peritoneal Dialysis (PD). The definition of EPS is based on the clinical findings linked to bowel obstruction and on the demonstration of peritoneal thickening. Surgical treatment is the only established basic treatment for the condition. Prune-belly syndrome is characterized by the triad of deficient abdominal musculature, urinary tract abnormality and cryptorchidism. Because it is often associated with end-stage renal disease, PD is essential in the treatment of patients with prune-belly syndrome. The aetiology of EPS follows a 'two-hit theory': the first 'hit' is peritoneal deterioration, caused by long-time exposure to PD. This causes peritoneal disruption which predisposes the patient to a second hit. In our patient, PD discontinuation and renal transplantation are possible 'second hits' that triggered the development of EPS. This case of prune-belly syndrome has all the necessary elements for the development of EPS, and we felt we should report it as the peroperative diagnosis was unexpected.

Roux-en-Y gastric bypass procedure performed with the da Vinci robot system: is it worth it?

BACKGROUND: The Roux-en-Y gastric bypass procedure (RYGBP) is in many countries the gold standard for obtaining long-lasting weight reduction and improvement of obesity-related comorbidities. However, performing this operation by standard laparoscopic techniques requires important surgical skills because of the anastomoses involved. The da Vinci surgical robot system with its enhanced degrees of freedom in motion and three-dimensional vision is designed to overcome the difficulties encountered in traditional laparoscopic surgery with suturing and delicate tissue handling. METHODS: For this study, 45 patients (9 men) with a mean body mass index (BMI) of 44.2 (range, 35.1-55.4) underwent RYGBP with the aid of the da Vinci robot system. They were compared with 45 consecutive patients with a mean BMI of 43.9 (range, 35.1-56.2) who underwent a laparoscopic RYGBP by the same surgeon during the same period. RESULTS: Overall, the total operating time was shorter for the laparoscopic cases (127 vs 212 min; p < 0.05). However, the last 10 robotic cases were performed in the same time span as the laparoscopic cases (136 vs 127 min). The total robotic setup time remained constant at about 30 min. There were no differences in postoperative complications between the two groups in terms of anastomotic leakage or stenosis. In the robotic group, more conversions to open surgery were noted. Early in the study, four patients (9%) had to undergo conversion to standard laparoscopic techniques due to inadequate setup of the robotic arms. Five patients (11%), however, had to undergo conversion to open surgery because of intestinal laceration during manipulation of the intestines with the robotic instruments. The costs were higher for robotic surgery than for standard laparoscopic RYGBP, mainly because of the extra equipment used, such as ultrasonic devices. CONCLUSION: The RYGBP procedure can be performed safely with the da Vinci robot after a learning curve of about 35 cases. At this writing, however, it is not clear whether the da Vinci system offers a real advantage over standard laparoscopic techniques.