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Prevalence and outcomes of pancreatic cystic neoplasms in liver transplant recipients

World Journal of Gastroenterology 2017年第48期23卷 8526-8532页

作者： Ken Liu Vikram Joshi Louise van Camp Qi-Wei Yang Judith E Baars Simone I Strasser Geoffrey W McCaughan Avik Majumdar Payal Saxena Arthur J Kaffes Sydney Medical school The University of Sydney AW Morrow Gastroenterology and Liver Centre Royal Prince Alfred Hospital Department of Radiology Royal Prince Alfred Hospital

AIM To determine the prevalence,characteristics and clinical course of pancreatic cystic neoplasms(PCNs) in liver transplantation(LT) *** We retrospectively studied consecutive patients who underwent LT between January 1998 to April 2016. clinical and laboratory data were obtained from patient medical records. Imaging findings on computed tomography and magnetic resonance cholangiopancreatography were reviewed by two *** During the study period,872 patients underwent cadaveric LT. Pancreatic cysts were identified in 53/872(6.1%) and 31/53(58.5%) were PCNs [28 intraductal papillary mucinous neoplasm(IPMN),2 mucinous cystic neoplasm(MCN),1 serous cystadenoma]. Patients with PCNs exhibited less male predominance(55% vs 73%,P = 0.03) compared to patients without pancreatic cysts. Thirteen patients(42%) were diagnosed with PCN pre-LT while 18 patients(58%) developed PCN post-LT. The median size of PCNs was 13 mm [interquartile range(IQR) 10-20 mm]. All IPMNs were side-branch type. Most PCNs were found in the head and body of pancreas(37% each),followed by the tail(25%). Five patients underwent further evaluation with endoscopic ultrasound. Progress imaging was performed on 81% of patients. PCNs remained stable in size and number in all but 2 patients. During a median follow up of 39 mo(IQR 26-58 mo),the 2(6%) patients with MCN underwent pancreatectomy. No PCN patient developed pancreatic adenocarcinoma,while 5 died from illnesses unrelated to the PCN. Among patients without PCN,1/841(0.1%) developed pancreatic *** The prevalence of PCNs in LT recipients was similar to the general population(3.6%,31/872). Side-branch IPMNs do not appear to have accelerated malignant potential in post-LT patients,indicating the current surveillance guidelines are applicable to this group.

关键词： Pancreatic cystic neoplasm Intraductal papillary mucinous neoplasm Mucinous cystic neoplasm Liver transplantation Pancreatic adenocarcinoma Immunosuppression

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Congenital peritoneal encapsulation: A review and novel classification system

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World Journal of Gastroenterology 2019年第19期25卷 2294-2307页

作者： Aneesh Dave James Mc Mahon Assad Zahid Department of Colorectal Surgery Royal Prince Alfred Hospital Sydney Medical school Edward Ford Building the University of Sydney

Congenital peritoneal encapsulation(CPE) is a very rare, congenital condition characterised by the presence of an accessory peritoneal membrane which encases a variable extent of the small bowel. It is unclear how CPE develops,however it is currently understood to be a result of an aberrant adhesion in the peritoneal lining of the physiological hernia in foetal mid-gut development. The condition was first described in 1868, and subsequently there have been only 45 case reports of the phenomenon. No formal, systematised review of CPE has yet been performed, meaning the condition remains poorly understood,underdiagnosed and mismanaged. Diagnosis of CPE remains clinical with important adjuncts provided by imaging and diagnostic laparoscopy. Two thirds of patients present with abdominal pain, likely secondary to sub-acute bowel obstruction. A fixed, asymmetrical distension of the abdomen and differential consistency on abdominal palpation are more specific clinical features present in approximately 10% of cases. CPE is virtually undetectable on plain imaging, and is only detected on 40% of patients with computed tomography scan. Most patients will undergo diagnostic laparotomy to confirm the *** of CPE includes both medical management of the critically-unstable patient and surgical laparotomy, partial peritonectomy and *** following prompt surgical treatment is excellent, with a majority of patients being symptom free at follow up. This review summarises the current literature on the aetiology, diagnosis and treatment of this rare disease. We also introduce a novel classification system for encapsulating bowel diseases, which may distinguish CPE from the commoner, more morbid conditions of abdominal cocoon and encapsulating peritoneal sclerosis.

关键词： Congenital Encapsulation Peritoneum Cocoon Sclerosis

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Serum 25-hydroxyvitamin D deficiency and hepatic encephalopathy in chronic liver disease

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World Journal of Hepatology 2017年第10期9卷 510-518页

作者： Helen Vidot Alison Potter Robert Cheng Margaret Allman-Farinelli Nicholas Shackel Department Nutrition and Dietetics Royal Prince Alfred Hospital Centenary Institute University of Sydney royal prince alfred hospital school of Life and Environmental Sciences University of Sydney Faculty of Medicine University of Sydney South Western Sydney clinical school University of New South Wales

To investigate the relationship between 25-hydroxyvitamin D (25-OHD) deficiency and hepatic encephalopathy (HE) in patients with chronic liver disease (CLD). METHODSA retrospective analysis of the results of 392 adult patients with chronic liver disease who were assessed for liver transplantation between 2006 and 2010 was undertaken. HE, severity of CLD, nutritional status and 25-OHD were analysed in patients assessed for liver transplantation between 2006 and 2010. Patients who presented with acute, fulminant or subacute disease, with a primary diagnosis of liver cancer, were assessed for re-transplantation or who did not have a 25-OHD measurement were excluded from the analysis. RESULTSOne hundred and sixty-five patients were included in this analysis. The mean age of all patients was 53 ± 8 years. Moderate to severe 25-OHD deficiency was identified in 49 patients of whom 36 had grade 2-3 HE compared with 13 patients who were not encephalopathic (P ≤ 0.0001). Mild 25-OHD deficiency was not associated with HE. There was a significant correlation between the severity of 25-OHD deficiency and the severity of liver disease (r = 0.39, P ≤ 0.0001) and disease severity and the presence of HE (P ≤ 0.0001). Importantly, individuals with 25-OHD deficiency were more likely to have a diagnosis of overt HE (OHE) at a significantly lower model for end stage liver disease (MELD) score than individuals without OHE (P ≤ 0.0001). This significant difference was observed with MELD scores from 10 to 38. CONCLUSION25-OHD deficiency was observed in the majority of patients with CLD and for the first time was found to be significantly worse in patients with OHE.

关键词： Vitamin D Chronic liver disease Hepatic encephalopathy Model For End Stage Liver Disease Dementia Malnutrition Cognitive function

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Metabolic associated fatty liver disease:Addressing a new era in liver transplantation

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World Journal of Hepatology 2020年第12期12卷 1168-1181页

作者： Madeleine G Gill Avik Majumdar AW Morrow Gastroenterology and Liver Centre Royal Prince Alfred HospitalSydney 2050New South WalesAustralia central clinical school The University of SydneySydney 2050New South WalesAustralia

Metabolic associated fatty liver disease(MAFLD),previously termed nonalcoholic fatty liver disease,is the leading global cause of liver disease and is fast becoming the most common indication for liver *** recent change in nomenclature to MAFLD refocuses the conceptualisation of this disease entity to its metabolic underpinnings and may help to spur a paradigm shift in the approach to its management,including in the setting of liver *** with MAFLD present significant challenges in the pre-,peri-and posttransplant settings,largely due to the presence of medical comorbidities that include obesity,metabolic syndrome and cardiovascular risk *** the community prevalence of MAFLD increases concurrently with the obesity epidemic,donor liver steatosis is also a current and future *** review outlines current epidemiology,nomenclature,management issues and outcomes of liver transplantation in patients with MAFLD.

关键词： Fatty liver Metabolic associated fatty liver disease Non-alcoholic fatty liver disease Liver transplantation Cirrhosis Metabolic syndrome

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Colorectal surgeon consensus with diverticulitis clinical practice guidelines

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World Journal of Gastrointestinal Surgery 2017年第11期9卷 224-232页

作者： Javariah Siddiqui Assad Zahid Jonathan Hong Christopher John Young Discipline of Surgery University of Sydney Department of Colorectal Surgery Royal Prince Alfred Hospital

AIM To determine the application of clinical practice guidelines for the current management of diverticulitis and colorectal surgeon specialist consensus in Australia and New *** A survey was distributed to 205 colorectal surgeons in Australia and New Zealand, using 22 hypothetical clinical *** The response rate was 102(50%). For 19 guidelinebased scenarios, only 11(58%) reached consensus(defined as > 70% majority opinion) and agreed with guidelines; while 3(16%) reached consensus and did not agree with guidelines. The remaining 5(26%) scenarios showed community equipoise(defined as less than/equal to 70% majority opinion). These included diagnostic imaging where CT scan was contraindicated, management options in the failureof conservative therapy for complicated diverticulitis, surgical management of Hinchey grade 3, proximal extent of resection in sigmoid diverticulitis and use of oral mechanical bowel preparation and antibiotics for an elective colectomy. The consensus areas not agreeing with guidelines were management of simple diverticulitis, management following the failure of conservative therapy in uncomplicated diverticulitis and follow-up after an episode of complicated diverticulitis. Fifty-percent of rural/regional based surgeons would perform an urgent sigmoid colectomy in failed conservative therapy of diverticulitis compared to only 8% of surgeons city-based(Fisher's exact test P = 0.016). In right-sided complicated diverticulitis, a greater number of those in practice for more than ten years would perform an ileocecal resection and ileocolic anastomosis(79% vs 41%, P < 0.0001).CONCLUSION While there are areas of consensus in diverticulitis management, there are areas of community equipoise for future research, potentially in the form of RCTs.

关键词： Diverticulitis clinical practice guidelines Consensus

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Environmental insults:critical triggers for amyotrophic lateral sclerosis

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Translational Neurodegeneration 2017年第1期6卷 147-156页

作者： Bing Yu Roger Pamphlett Sydney Medical school(central) The University of SydneyCamperdownNSW 2006Australia Department of Medical Genomics Royal Prince Alfred Hospital and NSW Health PathologyCamperdownNSW 2050Australia Discipline of Pathology Brain and Mind CentreThe University of Sydney94 Mallett StCamperdownNSW 2050Australia Department of Neuropathology Royal Prince Alfred HospitalCamperdownNSW 2050Australia

Background:Amyotrophic lateral sclerosis(ALS)is a fatal neurodegenerative disease characterised by a rapid loss of lower and upper motor *** a complex disease,the ageing process and complicated gene-environment interactions are involved in the majority of *** body:Significant advances have been made in unravelling the genetic susceptibility to ALS with massively parallel sequencing technologies,while environmental insults remain a suspected but largely unexplored source of *** studies applying the strategy of Mendelian randomisation have strengthened the link between environmental insults and ALS,but none so far has proved *** propose a new ALS model which links the current knowledge of genetic factors,ageing and environmental *** model provides a mechanism as to how ALS is initiated,with environmental insults playing a critical ***:The available evidence has suggested that inherited defect(s)could cause mitochondrial dysfunction,which would establish the primary susceptibility to *** study of the underlying mechanism may shed light on ALS *** insults are a critical trigger for ALS,particularly in the aged individuals with other toxicant susceptible *** identification of ALS triggers could lead to preventive strategies for those individuals at risk.

关键词： Amyotrophic lateral sclerosis Environmental risk factors Mendelian randomisation Mitochondrial dysfunction Trigger Initiation Spread

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Associations between Bodyweight and clinical Outcome in Patients Post-Fontan Procedure: A Systematic Review

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Congenital Heart Disease 2022年第6期17卷 617-639页

作者： Emma Payne Thomas Wilson Marjan Haghighi Zoe McCallum Yves d’Udekem Julian Ayer The University of Sydney SydneyAustralia The University of Melbourne MelbourneAustralia The royal Children’s hospital MelbourneAustralia The royal prince alfred hospital SydneyAustralia The Children’s National hospital WashingtonUSA

Background: Patients born with a single ventricle circulation commonly experience growth failure in early life,which is associated with adverse outcomes in infancy. However, associations between bodyweight or weight trajectoryand clinical outcome post-Fontan procedure are yet to be determined. Methods: On the 1st of July 2021, asystematic review was performed in MEDLINE, EMBASE, the Cochrane Library, and Scopus of studies of patientswith clinical outcome data post-Fontan procedure and association with bodyweight. Quality of studies was evaluatedby Newcastle–Ottawa scale for cohort studies and Joanna Briggs Institute tool for cross-sectional ***: Of 527 studies that underwent title and abstract screening, 15 were selected for final review. An increasedrisk of adverse post-Fontan outcomes was found for low weight patients, consistent with findings in *** there is some evidence to suggest increased mortality in overweight adult patients, studies are conflictingas to whether overweight status is associated with increased heart failure. Increased BMI is associated with diminishedexercise capacity and deceased physiological functioning. Negative weight trajectory is associated withadverse outcomes in the peri-Fontan period, whereas a positive weight trajectory is associated with increased Fontanfailure in adulthood. Abnormal BMI (high or low) is associated with increased heart failure and poorer performancein quality-of-life scores. Conclusions: Bodyweight is a modifiable risk factor for poor clinical outcomein patients with a single ventricle circulation. Recognizing associations between bodyweight and Fontan pathophysiologymay help to define patient-centered exercise and diet interventions that minimize patient morbidityand mortality.

关键词： Fontan bodyweight congenital heart disease single ventricle

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Thyroid Nodules in Type 2 Diabetes Mellitus

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Current Medical Science 2019年第4期39卷 576-581页

作者： Hong-mei ZHANG Qi-wen FENG Yi-xin NIU Qing SU Xia WANG Department of Endocrinology Xinhua HospitalSchool of MedicineShanghai Jiaotong UniversityShanghai 200092China The George Institute for Global Health and royal prince alfred hospital the University of SydneySydneyNSW 2050Australia

The present analysis aims to investigate the prevalence of thyroid nodules in type 2 diabetes mellitus (T2DM) *** searched PubMed,EMBASE,and Web of Science from inception to the March 1,*** studies were selected to estimate the prevalence of thyroid nodules in T2DM subjects and to compare the prevalence of thyroid nodules in different glucose tolerance *** random effects model was used,and the outcome was presented as a pooled prevalence proportion with 95% confidence interval (95% CI) or a summary odds ratio (OR) with 95% *** the end,9 studies met the inclusion criteria and were included in the *** pooled prevalence of thyroid nodules was 60%(95% CI:0.52,0.68) for T2DM 2 diabetes patients,50%(95% CI:0.4& 0.51) for pre-diabetes,and 43%(95% CI:0.34,0.52) for normal glucose tolerance *** with patients without diabetes,diabetes subjects are more likely to develop thyroid nodules,adjusted OR for thyroid nodule was 1.78 (95% CI:1.25,2.55).Insulin resistance might be involved in thyroid nodule development.

关键词： thyroid nodule diabetes insulin resistance

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Indications and surgical options for small bowel, large bowel and perianal Crohn's disease

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World Journal of Gastroenterology 2016年第40期22卷 8892-8904页

作者： James WT Toh Peter Stewart Matthew JFX Rickard Rupert Leong Nelson Wang Christopher J Young Department of Colorectal Surgery Concord Repatriation General Hospital South Western Sydney clinical school University of New South Wales Liverpool Hospital school of Medicine Western Sydney University Liverpool Hospital Ingham Institute of Applied Research Liverpool Hospital Discipline of Surgery Sydney Medical School the University of Sydney Department of Gastroenterology Concord Repatriation General Hospital Department of Colorectal Surgery Royal Prince Alfred Hospital

Despite advancements in medical therapy of Crohn's disease(CD), majority of patients with CD will eventually require surgical intervention, with at least a third of patients requiring multiple surgeries. It is important to understand the role and timing of surgery, with the goals of therapy to reduce the need for surgery without increasing the odds of emergency surgery and its associated morbidity, as well as to limit surgical recurrence and avoid intestinal failure. The profile of CD patients requiring surgical intervention has changed over the decades with improvements in medical therapy with immunomodulators and biological agents. The most common indication for surgery is obstruction from stricturing disease, followed by abscesses and fistulae. The risk of gastrointestinal bleeding in CD is high but the likelihood of needing surgery for bleeding is low. Most major gastrointestinal bleeding episodes resolve spontaneously, albeit the risk of re-bleeding is high. The risk of colorectal cancer associated with CD is low. While current surgical guidelines recommend a total proctocolectomy for colorectal cancer associated with CD, subtotal colectomy or segmental colectomy with endoscopic surveillance may be a reasonable option. Approximately 20%-40% of CD patients will need perianal surgery during their lifetime. This review assesses the practice parameters and guidelines in the surgical management of CD, with a focus on the indications for surgery in CD(and when not to operate), and a critical evaluation of the timing and surgical options available to improve outcomes and reduce recurrence rates.

关键词： Surgery Crohn’s disease Major abdominal surgery Perianal Inflammatory bowel disease Colon cancer

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Sleep disordered breathing in interstitial lung disease: A review

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World Journal of clinical Cases 2014年第12期2卷 828-834页

作者： Lauren K Troy Tamera J Corte Department of Respiratory Medicine Royal Prince Alfred Hospital Camperdown Sydney NSW 2050 Australia Sydney Medical school University of Sydney Sydney NSW 2006 Australia

Patients with interstitial lung disease commonly exhibit abnormal sleep architecture and increased sleep fragmentation on polysomnography. Fatigue is a frequent complaint, and it is likely that poor sleep quality is a significant contributor. A number of studies have shown that sleep disordered breathing is prevalent in this population, particularly in the idiopathic pulmonary fibrosis subgroup. The factors that predispose these patients to obstructive sleep apnoea are not well understood, however it is believed that reduced caudal traction on the upper airway can enhance collapsibility. Ventilatory control system instability may also be an important factor, particularly in those with increased chemo-responsiveness, and in hypoxic conditions. Transient, repetitive nocturnal oxygen desaturation is frequently observed in interstitial lung disease, both with and without associated obstructive apnoeas. There is increasing evidence that sleep-desaturation is associated with increased mortality, and may be important in the pathogenesis of pulmonary hypertension in this population.

关键词： Sleep disordered breathing Interstitial lung disease Pulmonary fibrosis Nocturnal hypoxia Obstructive sleep apnoea

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