BACKGROUND Madelung’s disease,also known as multiple symmetrical lipomatosis,is a rare,underrecognized disorder of fat metabolism that results in unusual accumulation of subcutaneous fat deposits around the neck,shou...BACKGROUND Madelung’s disease,also known as multiple symmetrical lipomatosis,is a rare,underrecognized disorder of fat metabolism that results in unusual accumulation of subcutaneous fat deposits around the neck,shoulders,upper arms,trunk,hips,and upper thighs.Our case demonstrates the importance of differential diagnosis and the value of a superb microvascular imaging technique for suspecting and confirming Madelung’s disease.Timely diagnosis and alcohol abstinence could prevent the progression of growing fatty masses and prevent surgery.CASE SUMMARY A 62-year-old male was admitted to the Rheumatology center complaining of symmetric subcutaneous tumors in the area of the parotid and submandibular salivary glands,small soft masses in the occiput and upper third of the forearm,rashes on calves.A high titer of rheumatoid factor and low concentrations of serum complements were detected.The high-end ultrasound and magnetic resonance imaging examinations of all affected areas of the soft tissues showed predominantly adipose tissue(lipomas)without suspicion of liposarcoma.The biopsy from the small salivary gland revealed no pathology.After evaluating the patient’s clinical presentation(symmetrical lipomatosis,cirrhosis,gynecomastia,anemia,hyperuricemia),Madelung’s disease,type I,along with the psoriatic rash and psoriatic arthritis and secondary liver cirrhosis were established.CONCLUSION Madelung’s disease consists of many co-occurring disorders imitating and overlapping with other conditions.Ultrasonography is the first choice for suspecting and confirming symmetrical lipomatosis.展开更多
Recently,the gut microbiota has been recognized as an obvious active player in addition to liver steatosis/steatohepatitis in the pathophysiological mechanisms of the development of hepatocellular carcinoma(HCC),even ...Recently,the gut microbiota has been recognized as an obvious active player in addition to liver steatosis/steatohepatitis in the pathophysiological mechanisms of the development of hepatocellular carcinoma(HCC),even in the absence of cirrhosis.Evidence from clinical and experimental studies shows the association of specific changes in the gut microbiome and the direct contribution to maintaining liver inflammation and/or cancerogenesis in nonalcoholic fatty liver disease-induced HCC.The composition of the gut microbiota differs significantly in obese and lean individuals,especially in the abundance of pro-inflammatory lipopolysaccharide-producing phyla,and,after establishing steatohepatitis,it undergoes minor changes during the progression of the disease toward advanced fibrosis.Experimental studies proved that the microbiota of obese subjects can induce steatohepatitis in normally fed mice.On the contrary,the transplantation of healthy microbiota to obese mice relieves steatosis.However,further studies are needed to confirm these findings and the mechanisms involved.In this review,we have evaluated well-documented clinical and experimental research on the role of the gut microbiota in the manifestation and promotion of HCC in nonalcoholic steatohepatitis(NASH).Furthermore,a literature review of microbiota alterations and consequences of dysbiosis for the promotion of NASH-induced HCC was performed,and the advantages and limitations of the microbiota as an early marker of the diagnosis of HCC were discussed.展开更多
BACKGROUND The inflammatory myofibroblastic tumor(IMT)is a rare,idiopathic,usually benign,mass-forming disease with myofibroblastic proliferation and a varying amount of inflammatory cells.Although it can affect vario...BACKGROUND The inflammatory myofibroblastic tumor(IMT)is a rare,idiopathic,usually benign,mass-forming disease with myofibroblastic proliferation and a varying amount of inflammatory cells.Although it can affect various organs,the biliary tract is a rare localization of primary IMT,clinically,endoscopically and radiologically imitating cholangiocarcinoma.The treatment options are based only on clinical practice experience.CASE SUMMARY A 70-year-old woman was referred to our center due to progressive fatigue,weight loss,abdominal pain,night sweats,and elevated liver enzymes.Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography(ERCP)revealed proximal common hepatic duct and hilar biliary strictures extending bilaterally to lobular bile ducts.Although initial clinical,endoscopic and radiological signs were typical for hilar cholangiocarcinoma,histological examination showed no signs of malignancy.In total,8 biopsies using different approaches were performed(several biopsies from dominant stricture during ERCP and direct cholangioscopy;ultrasound-guided liver biopsy;diagnostic laparoscopy with liver and lymph node biopsies).Histological examination revealed signs of IMT,and the final diagnosis of biliary IMT was stated.Although IMT is usually a benign disease,in our case,it was complicated.All pharmacological treatment measures were ineffective.The patient still needs permanent stenting,suffers from recurrent infections and mechanical jaundice.Despite that,the patient already survived 24 mo.CONCLUSION IMT presenting with hilar biliary strictures is a unique diagnostic and clinical challenge as it is indistinguishable from cholangiocarcinoma,and there are no evidence-based treatment options.Our goal is to increase the understanding of this rare disease and its possible course.展开更多
Approximately 20%of cirrhotic patients with ascites develop umbilical herniation.These patients usually suffer from multisystemic complications of cirrhosis,have a significantly higher risk of infection,and require ac...Approximately 20%of cirrhotic patients with ascites develop umbilical herniation.These patients usually suffer from multisystemic complications of cirrhosis,have a significantly higher risk of infection,and require accurate surveillance–especially in the context of the coronavirus disease 2019 pandemic.The rupture of an umbilical hernia,is an uncommon,life-threatening complication of largevolume ascites and end-stage liver disease resulting in spontaneous paracentesis,also known as Flood syndrome.Flood syndrome remains a challenging condition for clinicians,as recommendations for its management are lacking,and the available evidence for the best treatment approach remains controversial.In this paper,four key questions are addressed regarding the management and prevention of Flood syndrome:(1)Which is the best treatment approach–conservative treatment or urgent surgery?(2)How can we establish the individual risk for herniation and possible hernia rupture in cirrhotic patients?(3)How can we prevent umbilical hernia ruptures?And(4)How can we manage these patients in the conditions created by the coronavirus disease 2019 pandemic?展开更多
BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a multisystemic mass forming immune-mediated disease that affects almost every organ and is a diagnostic challenge for every clinician.There is a lack of adequat...BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a multisystemic mass forming immune-mediated disease that affects almost every organ and is a diagnostic challenge for every clinician.There is a lack of adequate epidemiological data worldwide,and evidence-based treatment recommendations are not yet established.We report the first case of IgG4-RD from Lithuania and the Baltic Sea region presented with thyroiditis,orbital myositis,orbitopathy,uveitis,scleritis,sialadenitis,autoimmune pancreatitis and prostatitis.CASE SUMMARY A 54-year-old Caucasian male was admitted to our tertiary Centre complaining of severe weight loss,diarrhoea,abdominal pain,salivary gland swelling,sicca symptoms and diplopia.On examination,bilateral palpable masses in the projection of major salivary glands,severe protrusion of the left eyeball and cachexia were noted.The patient was previously diagnosed with autoimmune thyroiditis and endocrine ophthalmopathy.The magnetic resonance imaging(MRI)of the head revealed enlarged extraocular muscles indicating orbital myositis.The biopsy from the salivary gland mass indicated sialadenitis.Abdominal MRI showed signs of autoimmune pancreatitis,and a serological test revealed the elevated serum IgG4 concentration.The patient was then diagnosed with IgG4-RD and successfully treated with prednisolone.There was a significant clinical,serological and radiological improvement after one month of treatment and no signs of relapse within twenty months.However,it took almost 18 years and the efforts of eight different medical specialists to establish the correct diagnosis.CONCLUSION A comprehensive approach to the patient is essential to improving the recognition of rare immune system conditions,such as IgG4-RD.展开更多
BACKGROUND Inflammatory bowel disease(IBD)is an autoimmune condition treated with immunosuppressive drugs.However,the need for immune system suppression becomes questionable when infection with the human immunodeficie...BACKGROUND Inflammatory bowel disease(IBD)is an autoimmune condition treated with immunosuppressive drugs.However,the need for immune system suppression becomes questionable when infection with the human immunodeficiency virus(HIV)occurs simultaneously and impacts the course of IBD.Our reported case represents the clinical course,prescribed treatment and its effect,as well as clinical challenges faced by physicians in a combination of such diseases.We also present a comprehensive literature review of similar cases.CASE SUMMARY A 49-year-old woman suffering from a newly diagnosed Crohn’s disease was hospitalized due to exacerbated symptoms(abdominal pain,fever,and weight loss).During her hospital stay,she tested positive for HIV.With conservative treatment,the patient improved and was discharged.In the outpatient clinic,her HIV infection was confirmed as stage C3,and antiretroviral treatment was initiated immediately.That notwithstanding,soon the patient was rehospitalized with pulmonary embolism and developed a series of complications because of the subsequent coexistence of IBD and HIV.After intensive and meticulous treatment,the patient’s condition has improved and she remains in remission.CONCLUSION The paucity of studies and data on the coexistence of HIV and IBD leaves clinicians doubting the optimal treatment options.展开更多
BACKGROUND Immune checkpoint inhibitors are one of the modern treatment methods for advanced malignancies.However,this group of medications is also associated with various immune-related adverse events,such as colitis...BACKGROUND Immune checkpoint inhibitors are one of the modern treatment methods for advanced malignancies.However,this group of medications is also associated with various immune-related adverse events,such as colitis or pneumonitis.Immune checkpoint inhibitor-induced gastritis is a less common adverse event.CASE SUMMARY We describe a 64-year-old woman presenting with diarrhea,nausea,and discomfort in the upper abdominal region.The patient had a history of metastatic lung cancer,which was treated with nivolumab.During the first endoscopy,an infiltrating gastric tumour was suspected.Later,based on endoscopic,histological and radiological findings,nivolumab-induced gastritis was diagnosed.The patient was successfully treated with three courses of omeprazole.CONCLUSION As a consequence of the increased use of immune checkpoint inhibitors,a growing number of reported immune-related adverse events could be expected.The diagnosis of immune checkpoint inhibitor-induced gastritis should be considered when assessing a patient treated with nivolumab with upper gastrointestinal distress.展开更多
文摘BACKGROUND Madelung’s disease,also known as multiple symmetrical lipomatosis,is a rare,underrecognized disorder of fat metabolism that results in unusual accumulation of subcutaneous fat deposits around the neck,shoulders,upper arms,trunk,hips,and upper thighs.Our case demonstrates the importance of differential diagnosis and the value of a superb microvascular imaging technique for suspecting and confirming Madelung’s disease.Timely diagnosis and alcohol abstinence could prevent the progression of growing fatty masses and prevent surgery.CASE SUMMARY A 62-year-old male was admitted to the Rheumatology center complaining of symmetric subcutaneous tumors in the area of the parotid and submandibular salivary glands,small soft masses in the occiput and upper third of the forearm,rashes on calves.A high titer of rheumatoid factor and low concentrations of serum complements were detected.The high-end ultrasound and magnetic resonance imaging examinations of all affected areas of the soft tissues showed predominantly adipose tissue(lipomas)without suspicion of liposarcoma.The biopsy from the small salivary gland revealed no pathology.After evaluating the patient’s clinical presentation(symmetrical lipomatosis,cirrhosis,gynecomastia,anemia,hyperuricemia),Madelung’s disease,type I,along with the psoriatic rash and psoriatic arthritis and secondary liver cirrhosis were established.CONCLUSION Madelung’s disease consists of many co-occurring disorders imitating and overlapping with other conditions.Ultrasonography is the first choice for suspecting and confirming symmetrical lipomatosis.
文摘Recently,the gut microbiota has been recognized as an obvious active player in addition to liver steatosis/steatohepatitis in the pathophysiological mechanisms of the development of hepatocellular carcinoma(HCC),even in the absence of cirrhosis.Evidence from clinical and experimental studies shows the association of specific changes in the gut microbiome and the direct contribution to maintaining liver inflammation and/or cancerogenesis in nonalcoholic fatty liver disease-induced HCC.The composition of the gut microbiota differs significantly in obese and lean individuals,especially in the abundance of pro-inflammatory lipopolysaccharide-producing phyla,and,after establishing steatohepatitis,it undergoes minor changes during the progression of the disease toward advanced fibrosis.Experimental studies proved that the microbiota of obese subjects can induce steatohepatitis in normally fed mice.On the contrary,the transplantation of healthy microbiota to obese mice relieves steatosis.However,further studies are needed to confirm these findings and the mechanisms involved.In this review,we have evaluated well-documented clinical and experimental research on the role of the gut microbiota in the manifestation and promotion of HCC in nonalcoholic steatohepatitis(NASH).Furthermore,a literature review of microbiota alterations and consequences of dysbiosis for the promotion of NASH-induced HCC was performed,and the advantages and limitations of the microbiota as an early marker of the diagnosis of HCC were discussed.
文摘BACKGROUND The inflammatory myofibroblastic tumor(IMT)is a rare,idiopathic,usually benign,mass-forming disease with myofibroblastic proliferation and a varying amount of inflammatory cells.Although it can affect various organs,the biliary tract is a rare localization of primary IMT,clinically,endoscopically and radiologically imitating cholangiocarcinoma.The treatment options are based only on clinical practice experience.CASE SUMMARY A 70-year-old woman was referred to our center due to progressive fatigue,weight loss,abdominal pain,night sweats,and elevated liver enzymes.Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography(ERCP)revealed proximal common hepatic duct and hilar biliary strictures extending bilaterally to lobular bile ducts.Although initial clinical,endoscopic and radiological signs were typical for hilar cholangiocarcinoma,histological examination showed no signs of malignancy.In total,8 biopsies using different approaches were performed(several biopsies from dominant stricture during ERCP and direct cholangioscopy;ultrasound-guided liver biopsy;diagnostic laparoscopy with liver and lymph node biopsies).Histological examination revealed signs of IMT,and the final diagnosis of biliary IMT was stated.Although IMT is usually a benign disease,in our case,it was complicated.All pharmacological treatment measures were ineffective.The patient still needs permanent stenting,suffers from recurrent infections and mechanical jaundice.Despite that,the patient already survived 24 mo.CONCLUSION IMT presenting with hilar biliary strictures is a unique diagnostic and clinical challenge as it is indistinguishable from cholangiocarcinoma,and there are no evidence-based treatment options.Our goal is to increase the understanding of this rare disease and its possible course.
文摘Approximately 20%of cirrhotic patients with ascites develop umbilical herniation.These patients usually suffer from multisystemic complications of cirrhosis,have a significantly higher risk of infection,and require accurate surveillance–especially in the context of the coronavirus disease 2019 pandemic.The rupture of an umbilical hernia,is an uncommon,life-threatening complication of largevolume ascites and end-stage liver disease resulting in spontaneous paracentesis,also known as Flood syndrome.Flood syndrome remains a challenging condition for clinicians,as recommendations for its management are lacking,and the available evidence for the best treatment approach remains controversial.In this paper,four key questions are addressed regarding the management and prevention of Flood syndrome:(1)Which is the best treatment approach–conservative treatment or urgent surgery?(2)How can we establish the individual risk for herniation and possible hernia rupture in cirrhotic patients?(3)How can we prevent umbilical hernia ruptures?And(4)How can we manage these patients in the conditions created by the coronavirus disease 2019 pandemic?
文摘BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a multisystemic mass forming immune-mediated disease that affects almost every organ and is a diagnostic challenge for every clinician.There is a lack of adequate epidemiological data worldwide,and evidence-based treatment recommendations are not yet established.We report the first case of IgG4-RD from Lithuania and the Baltic Sea region presented with thyroiditis,orbital myositis,orbitopathy,uveitis,scleritis,sialadenitis,autoimmune pancreatitis and prostatitis.CASE SUMMARY A 54-year-old Caucasian male was admitted to our tertiary Centre complaining of severe weight loss,diarrhoea,abdominal pain,salivary gland swelling,sicca symptoms and diplopia.On examination,bilateral palpable masses in the projection of major salivary glands,severe protrusion of the left eyeball and cachexia were noted.The patient was previously diagnosed with autoimmune thyroiditis and endocrine ophthalmopathy.The magnetic resonance imaging(MRI)of the head revealed enlarged extraocular muscles indicating orbital myositis.The biopsy from the salivary gland mass indicated sialadenitis.Abdominal MRI showed signs of autoimmune pancreatitis,and a serological test revealed the elevated serum IgG4 concentration.The patient was then diagnosed with IgG4-RD and successfully treated with prednisolone.There was a significant clinical,serological and radiological improvement after one month of treatment and no signs of relapse within twenty months.However,it took almost 18 years and the efforts of eight different medical specialists to establish the correct diagnosis.CONCLUSION A comprehensive approach to the patient is essential to improving the recognition of rare immune system conditions,such as IgG4-RD.
文摘BACKGROUND Inflammatory bowel disease(IBD)is an autoimmune condition treated with immunosuppressive drugs.However,the need for immune system suppression becomes questionable when infection with the human immunodeficiency virus(HIV)occurs simultaneously and impacts the course of IBD.Our reported case represents the clinical course,prescribed treatment and its effect,as well as clinical challenges faced by physicians in a combination of such diseases.We also present a comprehensive literature review of similar cases.CASE SUMMARY A 49-year-old woman suffering from a newly diagnosed Crohn’s disease was hospitalized due to exacerbated symptoms(abdominal pain,fever,and weight loss).During her hospital stay,she tested positive for HIV.With conservative treatment,the patient improved and was discharged.In the outpatient clinic,her HIV infection was confirmed as stage C3,and antiretroviral treatment was initiated immediately.That notwithstanding,soon the patient was rehospitalized with pulmonary embolism and developed a series of complications because of the subsequent coexistence of IBD and HIV.After intensive and meticulous treatment,the patient’s condition has improved and she remains in remission.CONCLUSION The paucity of studies and data on the coexistence of HIV and IBD leaves clinicians doubting the optimal treatment options.
文摘BACKGROUND Immune checkpoint inhibitors are one of the modern treatment methods for advanced malignancies.However,this group of medications is also associated with various immune-related adverse events,such as colitis or pneumonitis.Immune checkpoint inhibitor-induced gastritis is a less common adverse event.CASE SUMMARY We describe a 64-year-old woman presenting with diarrhea,nausea,and discomfort in the upper abdominal region.The patient had a history of metastatic lung cancer,which was treated with nivolumab.During the first endoscopy,an infiltrating gastric tumour was suspected.Later,based on endoscopic,histological and radiological findings,nivolumab-induced gastritis was diagnosed.The patient was successfully treated with three courses of omeprazole.CONCLUSION As a consequence of the increased use of immune checkpoint inhibitors,a growing number of reported immune-related adverse events could be expected.The diagnosis of immune checkpoint inhibitor-induced gastritis should be considered when assessing a patient treated with nivolumab with upper gastrointestinal distress.
