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Metabolic dysfunction-associated steatotic liver disease:A silent pandemic

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World Journal of Hepatology 2024年第4期16卷 511-516页

作者： Arghya Samanta moinak sen sarma Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

The worldwide epidemiology of non-alcoholic fatty liver disease(NAFLD)is showing an upward trend,parallel to the rising trend of metabolic syndrome,owing to lifestyle *** pathogenesis of NAFLD has not been fully understood ***,NAFLD has emerged as a public health concern in the field of hepatology and metabolisms *** changes in the nomenclature from NAFLD to metabolic dysfunction-associated steatotic liver disease have brought a positive outlook changes in the understanding of the disease process and doctor-patient *** changes are the main treatment ***,clinical trial using drugs that target‘insulin resistance’which is the driving force behind NAFLD,have shown promising *** translational research is needed to better understand the underlying pathophysiological mechanism of NAFLD which may open newer avenues of therapeutic *** role of gut dysbiosis in etiopathogenesis and use of fecal microbiota modification in the treatment should be studied *** of this silent epidemic by spreading awareness and early intervention should be our priority.

关键词： Metabolic dysfunction Fatty liver Obesity Insulin resistance

Hepatic pseudotumor:A diagnostic challenge

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World Journal of Hepatology 2024年第5期16卷 667-670页

作者： Arghya Samanta moinak sen sarma Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

Hepatic pseudotumors are rare lesions of unknown origin,characterized by the proliferation of fibrous connective tissue and inflammatory cell *** mimic malignant lesions clinically,and radiologically,given their non-specific clinical and imaging *** pathophysiology of hepatic pseudotumor is incompletely understood and there are no standardized criteria for *** have been reported to develop in various organs in the body with the lung and liver being the most common *** pseudotumors develop in patients with underlying triggers of liver inflammation and injury,including infections,autoimmune liver diseases,bile duct injury,or *** pse-udotumors respond well to conservative treatment with antibiotics,and steroids and some may regress spontaneously,thus avoiding unnecessary *** condition is rewarding to *** is important to recognize pseudotumor as a distinct clinical entity and include it in the differential of liver masses with atypical imaging features.

关键词： Hepatic pseudotumor Infection Stroglyloides Hepatic resection

Portal Cavernoma Cholangiopathy in Children and the Management Dilemmas

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Journal of Clinical and Translational Hepatology 2020年第1期8卷 61-68页

作者： moinak sen sarma Aathira Ravindranath Department of Pediatric Gastroenterology Sanjay Gandhi Post Graduate Institute of Medical SciencesLucknowUttar PradeshIndia

Portal cavernoma cholangiopathy(PCC)is one of the most harrowing complications of extrahepatic portal venous obstruc-tion,as it determines the long-term hepatobiliary *** symptomatic PCC is rare in children,asymptomatic PCC is as common as that in ***,there are major gaps in the literature with regard to the best imaging strategy and management modality in ***,natural history of PCC and effect of portosystemic shunt surgeries in children are *** PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic ther-apy or bilioenteric anastomosis,both of which are challenging in the presence of extensive *** are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to *** this review,we aimed to collate all existing literature on PCC in childhood and also compare with adult *** highlight the difficul-ties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.

关键词： Extra-hepatic portal vein obstruction Portal cavernoma cholangiopathy Portosystemic shunt

Mitochondrial hepatopathy: Respiratory chain disorders- ‘breathing in and out of the liver’

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World Journal of Hepatology 2021年第11期13卷 1707-1726页

作者： Amrit Gopan moinak sen sarma Department of Gastroenterology Seth G.S Medical College and K.E.M HospitalMumbai 400012India Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

Mitochondria,the powerhouse of a cell,are closely linked to the pathophysiology of various common as well as not so uncommon disorders of the liver and *** supports a prokaryotic descent,and,unsurprisingly,the organelle is worthy of being labeled an organism in *** highly metabolically active organs require a continuous feed of energy,any dysfunction in the structure and function of mitochondria can have variable impact,with the worse end of the spectrum producing catastrophic consequences with a multisystem *** categorized a hepatopathy,mitochondrial respiratory chain defects are not limited to the liver in time and *** liver involvement is also variable in clinical presentation as well as in age of onset,from acute liver failure,cholestasis,or chronic liver *** organs like eye,muscle,central and peripheral nervous system,gastrointestinal tract,hematological,endocrine,and renal systems are also variably *** hinges on recognition of subtle clinical clues,screening metabolic investigations,evaluation of the extrahepatic involvement,and role of genetics and tissue *** is aimed at both circumventing the acute metabolic crisis and long-term management including nutritional *** review lists and discusses the burden of mitochondrial respiratory chain defects,including various settings when to suspect,their evolution with time,including certain specific disorders,their tiered evaluation with diagnostic algorithms,management dilemmas,role of liver transplantation,and the future research tools.

关键词： Mitochondrial hepatopathy Respiratory chain defects Maternal inheritance Neonatal liver failure DNA depletion syndrome Pearson syndrome

Pediatric non-cirrhotic portal hypertension:Endoscopic outcome and perspectives from developing nations

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World Journal of Hepatology 2021年第10期13卷 1269-1288页

作者： moinak sen sarma Jayendra Seetharaman Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia

Non-cirrhotic portal hypertension(NCPH)forms an important subset of portal hypertension in *** bleed and splenomegaly are their predominant *** features show cytopenias(hypersplenism)and preserved hepatic synthetic *** sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all *** variceal eradication,there is an increased risk of other complications like secondary gastric varices,cholangiopathy,colopathy,growth failure,especially in extra-hepatic portal vein obstruction(EHPVO).Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL).Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in *** portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues *** surgeries prevent rebleed,improve growth and ***-cirrhotic portal fibrosis(NCPF)is a less common cause of portal hypertension in children in developing *** in the second decade,massive splenomegaly and patent portal vein are discriminating features of *** surgery is required in severe cases when endotherapy is insufficient for the *** hepatic fibrosis(CHF)presents with firm palpable liver and *** plate malformation forms the histological hallmark of *** is commonly associated with Caroli’s disease,renal cysts,and syndromes associated with neurological *** CHF has a favourable prognosis requiring *** transplanta-tion is required when there is decompensation or recurrent cholangitis,especially in Caroli’s *** liver-kidney transplantation is indicated when both liver and renal issues are present.

关键词： Extrahepatic portal vein obstruction Non-cirrhotic portal fibrosis Portosystemic shunt surgery Congenital hepatic fibrosis

Mitochondrial hepatopathy: Anticipated difficulties in management of fatty acid oxidation defects and urea cycle defects

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World Journal of Hepatology 2022年第1期14卷 180-194页

作者： Aathira Ravindranath moinak sen sarma Division of Pediatric Gastroenterology Institute of Gastrointestinal SciencesApollo BGS HospitalsMysore 570023KarnatakaIndia Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia

Fatty acid oxidation defects(FAOD)and urea cycle defects(UCD)are among the most common metabolic liver *** of these disorders is dotted with challenges as the strategies differ based on the type and severity of the *** those with FAOD the cornerstone of management is avoiding hypoglycemia which in turn prevents the triggering of fatty acid *** this review,we discuss the role of carnitine supplementation,dietary interventions,newer therapies like triheptanoin,long-term treatment and approach to positive newborn *** UCD the general goal is to avoid excessive protein intake and indigenous protein ***,one size does not fit all and striking the right balance between avoiding hyperammonemia and preventing deficiencies of essential nutrients is a formidable *** issues during the acute presentation including differential diagnosis of hyperammonemia,dietary dilemmas,the role of liver transplantation,management of the asymptomatic individual and monitoring are described in detail.A multi-disciplinary team consisting of hepatologists,metabolic specialists and dieticians is required for optimum management and improvement in quality of life for these patients.

关键词： Mitochondrial hepatopathy Metabolic liver disease Liver transplantation Hyperammonemia Hypoglycemia Carnitine

Chelation therapy in liver diseases of childhood: Current status and response

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World Journal of Hepatology 2021年第11期13卷 1552-1567页

作者： Jayendra Seetharaman moinak sen sarma Department of Pediatric Gastroenterology Sanjay Gandhi Post-graduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia

Chelation is the mainstay of therapy in certain pediatric liver *** and iron related disorders require ***’s disease(WD),one of the common causes of cirrhosis in children is treated primarily with copper chelating agents like D-penicillamine and trientine.D-Penicillamine though widely used due its high efficacy in hepatic WD is fraught with frequent adverse effects resulting ***,an alternative drug has comparable efficacy in hepatic WD but has lower frequency of adverse *** role of ammonium tetra-thiomolybdate is presently experimental in hepatic *** childhood cirrhosis is related to excessive copper ingestion,rarely seen in present *** is effective in the early part of this disease with reversal of clinical *** chelators are commonly used in secondary hemochromatosis of liver in hemolytic *** are strict chelation protocols during bone marrow *** role of iron chelation in neonatal hemochromatosis is presently not in vogue due to its poor efficacy and availability of other modalities of *** hemochromatosis is rare in children and the use of iron chelators in this condition is limited.

关键词： Wilson’s disease D-Penicillamine Trientine Indian childhood cirrhosis Deferoxamine Deferasirox Hemochromatosis

Pediatric acute viral hepatitis with atypical variants:Clinical dilemmas and natural history

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World Journal of Hepatology 2022年第5期14卷 944-955页

作者： moinak sen sarma Aathira Ravindranath Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia Division of Pediatric Gastroenterology Apollo BGS HospitalsMysuru 570023KarnatakaIndia

Classical acute viral hepatitis(AVH)has an uncomplicated *** liver failure has a grave *** manifestations of AVH are a group of disorders that causes significant morbidity and dilemmas in *** include prolonged cholestasis,relapsing hepatitis,ascitic form of AVH,late-onset hepatic failure(LOHF),intravascular hemolysis,and provoking an autoimmune trigger leading to autoimmune *** entities cause significant liver dysfunction or worsening and are often difficult to differentiate from chronic liver disease(CLD).Ascitic form of AVH,LOHF,decompensated CLD and acute-onchronic liver failure have significant overlapping features that need to be carefully dissected *** many cases,only on long-term follow-up,these clinical entities can be separately *** hemolysis is usually caused by associated glucose-6-phosphate dehydrogenase *** CLD such as Wilson disease and autoimmune hepatitis can also present with hemolysis in the initial presentation,which can mimic AVH with *** deviations from typical manifestations aid in avoiding unnecessary investigations,allowing focused therapy and alleviating anxiety.

关键词： Viral Hepatitis Atypical Cholestasis Relapsing Hemolysis Ascites

Bile acid synthetic defects: Simplified approach in a nutshell

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Hepatobiliary & Pancreatic Diseases International 2020年第1期19卷 80-84页

作者： Aathira Ravindranath moinak sen sarma Surender Kumar Yachha Department of Pediatric Gastroenterology Sanjay Gandhi Post Graduate Institute of Medical Sciences

Bile acid synthetic defects(BASD)constitute a rare group of disorders with manifestations in children as well as *** the individual BASD mandates a lucid understanding of the bile acid synthetic ***,the synthesis pathway is complex,consisting of numerous enzymes and intermediate products that are volatile in the physician’s *** paucity of literature,a simplified approach to the BASD is presented in this paper.

关键词： synthetic simplified approach