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Pediatric metabolic liver diseases:Evolving role of liver transplantation

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World Journal of Transplantation 2021年第6期11卷 161-179页

作者： Jagadeesh Menon Mukul vij Deepti Sachan Ashwin rammohan Naresh Shanmugam Ilankumaran Kaliamoorthy Mohamed Rela Institute of Liver Disease and Transplantation Dr Rela Institute and Medical CenterChennai 600044Tamil NaduIndia Department of Pathology Dr Rela Institute and Medical CenterChennai 600044Tamil NaduIndia Department of Transfusion Medicine Dr Rela Institute and Medical CenterChennai 600044Tamil NaduIndia

Metabolic liver diseases(MLD)are the second most common indication for liver transplantation(LT)in *** is based on the fact that the majority of enzymes involved in various metabolic pathways are present within the liver and LT can cure or at least control the disease *** is also performed in metabolic disorders for end-stage liver disease,its sequelae including hepatocellular *** is also performed for preventing metabolic crisis’,arresting progression of neurological dysfunction with a potential to reverse symptoms in some cases and for preventing damage to end organs like kidneys as in the case of primary hyperoxalosis and methyl malonic *** findings in explant liver with patients with metabolic disease include unremarkable liver to steatosis,cholestasis,inflammation,variable amount of fibrosis,and *** outcome of LT in metabolic disorders is excellent except for patients with mitochondrial disorders where significant extrahepatic involvement leads to poor outcomes and hence considered a contraindication for LT.A major advantage of LT is that in the post-operative period most patients can discontinue the special formula which they were having prior to the transplant and this increases their well-being and improves growth *** partial orthotopic LT has been described for patients with noncirrhotic MLD where a segmental graft is implanted in an orthotopic position after partial resection of the native *** retained native liver can be the potential target for future gene therapy when it becomes a clinical reality.

关键词： Liver transplantation Metabolic liver disease Tyrosinemia Wilson disease Glycogen storage diseases Urea cycle disorders Pathology Auxiliary liver transplant

A gist of gastrointestinal stromal tumors: A review

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World Journal of Gastrointestinal Oncology 2013年第6期5卷 102-112页

作者： Ashwin rammohan Jeswanth Sathyanesan Kamalakannan Rajendran Anbalagan Pitchaimuthu Senthil-Kumar Perumal UP Srinivasan Ravi Ramasamy Ravichandran Palaniappan Manoharan Govindan The Institute of Surgical Gastroenterology and Liver Transplantation Centre for GI Bleed Division of HPB Diseases Stanley Medical College Hospital

Gastrointestinal stromal tumors (GISTs) have been recognized as a biologically distinctive tumor type, different from smooth muscle and neural tumors of the gastrointestinal tract (GIT). They constitute the majority of gastrointestinal mesenchymal tumors of the GIT and are known to be refractory to conventional chemotherapy or radiation. They are defined and diagnosed by the expression of a proto-oncogene protein detected by immunohistochemistry which serves as a crucial diagnostic and therapeutic target. The identification of these mutations has resulted in a better understanding of their oncogenic mechanisms. The remarkable antitumor effects of the molecular inhibitor imatinib have necessitated accurate diagnosis of GIST and their distinction from other gastrointestinal mes-enchymal tumors. Both traditional and minimally invasive surgery are used to remove these tumors with minimal morbidity and excellent perioperative outcomes. The revolutionary use of specific, molecularlytargeted therapies, such as imatinib mesylate, reduces the frequency of disease recurrence when used as an adjuvant following complete resection. Neoadjuvant treatment with these agents appears to stabilize disease in the majority of patients and may reduce the extent of surgical resection required for subsequent complete tumor removal. The important interplay between the molecular genetics of GIST and responses to targeted therapeutics serves as a model for the study of targeted therapies in other solid tumors. This review summarizes our current knowledge and recent advances regarding the histogenesis, pathology, molecular biology, the basis for the novel targeted cancer therapy and current evidence based management of these unique tumors.

关键词： Gastrointestinal stromal tumors c-KIT Imatinib mesylate Surgery Review

Role of plasmapheresis in early allograft dysfunction following deceased donor liver transplantation

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World Journal of Hematology 2017年第1期6卷 24-27页

作者： Ashwin rammohan Deepti Sachan Satish Logidasan Jeswanth Sathyanesan Ravichandran Palaniappan Mohamed Rela Institute of Liver Disease and Transplantation Global Hospitals and Health City Institute of Surgical Gastroenterology and Liver Transplantation Govt Stanley Medical College Hospital Institute of Liver Studies King’s College Hospital

The role of plasmapheresis in liver failure and hepatic encephalopathy is undefined and its use as a strategy to salvage patients with severe allograft dysfunction after liver transplantation remains investigational. We present a case of early allograft dysfunction following deceased donor liver transplantation(DDLT) where plasmapheresis was effective as a bridge to recovery and possibly avoiding a retransplantation. A 16 years old boy, known to have decompensated Wilson's disease underwent DDLT at our Public Sector Hospital. He received a healthy liver from a brain-dead donor, whose liver was considered too large for the boy. The graft was reduced in situ to a left lobe graft. Surgery was uneventful and the recipient was well for the initial 96 h. On Doppler and further computed tomography scan, a partial portal vein thrombus was noted. He was reexplored and a Fogarty endothombecteomy was performed. Following the second surgery, he developed severe allograft dysfunction with a peak bilirubin of 40 mg/d L. He underwent imaging to rule out technical causes for the dysfunction, followed by a liver biopsy, which revealed acute cellular rejection. Multiple cycles of plasmapheresis were initiated. Over the next two weeks, the graft demonstrated a gradual recovery. He was discharged on the 30 th postoperative day, with a serum bilirubin of 5.5 mg/d L. He remains well on follow-up, with the liver function tests improving further. Our report demonstrates the beneficial effect of plasmapheresis, which appears to be an effective treatment option for early allograft dysfunction following liver transplantation and may obviate the need for retransplantation.

关键词： Liver transplantation Allograft dysfunction Plasmapheresis

Embolization of liver tumors:Past,present and future

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World Journal of Radiology 2012年第9期4卷 405-412页

作者： Ashwin rammohan Jeswanth Sathyanesan Sukumar Ramaswami Anand Lakshmanan Perumal Senthil-Kumar Ulagendra Perumal Srinivasan Ravi Ramasamy Palaniappan Ravichandran The Institute of Surgical Gastroenterology and Liver Transplantation Centre for GI BleedDivision of HPB DiseasesStanley Medical College Hospital Interventional RadiologistInstitute of Surgical Gastroenterology and Liver TransplantationCentre for GI BleedDivision of HPB DiseasesStanley Medical College Hospital

Curative therapies for hepatocellular carcinoma(HCC),such as resection and liver transplantation,can only be applied in selected patients with early *** advanced stages require local or systemic *** of HCC offers the only hope for *** in patients undergoing resection,recurrences are ***,a technique combining intraarterial chemotherapy with selective tumor ischemia,has been shown by randomized controlled trials to be efficacious in the palliative *** is now renewed interest in transarterial embolization/transarterial chemoembolization(TACE) with regards to its use as a palliative tool in a combined modality approach,as a neoadjuvant therapy,in bridging therapy before transplantation,for symptomatic indications,and even as an alternative to *** have also been rapid advances in the agents being embolized trans-arterially(genes,biological response modifiers,etc.).The current review provides an evidence-based overview of the past,present and future trends of TACE in patients with HCC.

关键词： Liver tumors Embolization Chemoembolization Review

Clinical-radiological-pathological correlation of cavernous sinus hemangioma: Incremental value of diffusion-weighted imaging

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World Journal of Radiology 2017年第8期9卷 330-338页

作者： Abhishek Mahajan vedula Rajni Kanth Rao Gudipati Anantaram Ashwin M Polnaya Sandeep Desai Paresh Desai rammohan vadapalli Manas Panigrahi Department of Radio- diagnosis Tata Memorial Centre Department of Radiology Krishna Institute of Medical Sciences Department of Radiodiagnosis Clumax Imaging Department of Radiology Apollo Victor Hospital Department of Radiology Vijaya Diagnostics Department of Neurosurgery Krishna Institute of Medical Sciences

AIMTo elucidate the clinical, magnetic resonance imaging (MRI), pathological features of these lesions and asses the incremental value of diffusion-weighted imaging (DWI) in diagnosing them. METHODSFifteen consecutive patients (11 females and 4 males; mean age 40.93 years; age range 13-63 years) with cavernous sinus hemangiomas (CSH) who underwent examination between November 2008 and May 2016 were included for the analysis. MRI, clinical and surgical findings of each patient was retrospectively reviewed. DWI were also analysed and mean-apparent diffusion coefficient (ADC) value was calculated. Eleven patients underwent surgical removal of the lesion and 2 patients had biopsy only. Diagnosis of CSH was confirmed histologically in 13 patients. RESULTSEleven patients (73%) presented with headaches and 10 (66%) had cranial nerve involvement. Extra cavernous sinus extension was noted in 14 (94%). Surgery was performed in 13 (87%) and post-operative radiation was given to 4 (28%) patients. Thirteen patients remained asymptomatic on follow up. Three conspicuous imaging features were highly suggestive of the diagnosis: Lack of diffusion restriction (100%), homogeneous hyperintensity on T2 weighted image sequences (93.3%) and intense post-contrast enhancement (100%). The mean ADC was 1.82 × 10-3 ± 0.2186 cm2/s. CONCLUSIONT1-weighted hypointensity with homogeneous hyperintensity on T2-weighted sequences, intense enhancement and absence of hemosiderin within the lesion on GRE sequence favour the diagnosis. Facilitated diffusion on DWI differentiates CSH from other solid cavernous sinus lesions and significantly improves the diagnostic accuracy, a critical factor for planning surgery.

关键词： Cavernous sinus hemangioma Cavernous sinus Magnetic resonance imaging Diffusion weighted imaging

Implications of the presence of an aberrant right hepatic artery in patients undergoing pancreaticoduodenectomy

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World Journal of Gastrointestinal Surgery 2014年第1期6卷 9-13页

作者： Ashwin rammohan Ravichandran Palaniappan Anbalagan Pitchaimuthu Kamalakannan Rajendran Senthil Kumar Perumal Kesavan Balaraman Ravi Ramasamy Jeswanth Sathyanesan Manoharan Govindan The Institute of Surgical Gastroenterology and Liver Transplantation Centre for GI Bleed Division of HPB diseases Stanley Medical College Hospital

AIM:To analyze the differences in outcomes and the clinical impact following pancreatoduodenectomy(PD)in patients with and without aberrant right hepatic artery(aRHA).METHODS:All patients undergoing PD between January 2008 and December 2012 were divided into two groups,one with aRHA and the other *** groups were compared to identify differences in the intraoperative variables,the oncological clearance and the postoperative morbidity,mortality and hospital ***:A total of 225 patients underwent PD,of which 43(19.1%)patients were found to have eitheraccessory or replaced right hepatic arteries(aRHA group).The aRHA was preserved in 79%of the *** was no significant difference in the intraoperative blood loss but operative time was prolonged,reflecting the complexity of the procedure[420±44(240-540)min vs 480±45(300-600)min,Pve complications(pancreatic leak,pancreatic fistula,delayed gastric emptying and mortality)and hospital *** clearance in the form of positive resection margins[13(7.1%)vs 3(6.9%)]and lymph node yield were also similar in the two ***:An aRHA is found in approximately one fifth of patients undergoing *** is technically possible in most patients and can increase the operative complexity but does not negatively affect the safety or oncological outcomes of the procedure.

关键词： Pancreatoduodenectomy Aberrant right hepatic artery Arterial anomalies Outcomes