Abdominal cocoon syndrome-a rare culprit behind small bowel ischemia and obstruction:Three case reports

BACKGROUND Abdominal cocoon syndrome(ACS)represents a category within sclerosing encapsulating peritonitis,characterized by the encapsulation of internal organs with a fibrous,cocoon-like membrane of unknown origin,resulting in bowel obstruction and ischemia.Diagnosing this condition before surgery poses a cha-llenge,often requiring confirmation during laparotomy.In this context,we depict three instances of ACS:One linked to intestinal obstruction,the second exclu-sively manifesting as intestinal ischemia without any obstruction,and the final case involving a discrepancy between the radiologist and the surgeon.CASE SUMMARY Three male patients,aged 53,58,and 61 originating from Northern Thailand,arrived at our medical facility complaining of abdominal pain without any prior surgeries.Their vital signs remained stable during the assessment.The diagnosis of abdominal cocoon was confirmed through abdominal computed tomography(CT)before surgery.In the first case,the CT scan revealed capsules around the small bowel loops,showing no enhancement,along with mesenteric congestion affecting both small and large bowel loops,without a clear obstruction.The second case showed intestinal obstruction due to an encapsulated capsule on the CT scan.In the final case,a patient presented with recurring abdominal pain.Initially,the radiologist suspected enteritis as the cause after the CT scan.However,a detailed review led the surgeon to suspect encapsulating peritoneal sclerosis(ACS)and subsequently perform surgery.The surgical procedure involved complete removal of the encapsulating structure,resection of a portion of the small bowel,and end-to-end anastomosis.No complications occurred during surgery,and the patients had a smooth recovery after surgery,eventually discharged in good health.The histopathological examination of the fibrous membrane(cocoon)across all cases consistently revealed the presence of fibro-collagenous tissue,without any indications of malignancy.CONCLUSION Individuals diagnosed with abdominal cocoons commonly manifest vague symptoms of abdominal discomfort.An elevated degree of clinical suspicion,combined with the application of appropriate radiological evaluations,markedly improves the probability of identifying the abdominal cocoon before surgical intervention.In cases of complete bowel obstruction or ischemia,the established norm is the comprehensive removal of the peritoneal sac as part of standard care.Resection with intestinal anastomosis is advised solely when ischemia and gangrene have been confirmed.

Causal role of immune cells in obstructive sleep apnea hypopnea syndrome:Mendelian randomization study

BACKGROUND Despite being one of the most prevalent sleep disorders,obstructive sleep apnea hypoventilation syndrome(OSAHS)has limited information on its immunologic foundation.The immunological underpinnings of certain major psychiatric diseases have been uncovered in recent years thanks to the extensive use of genome-wide association studies(GWAS)and genotyping techniques using highdensity genetic markers(e.g.,SNP or CNVs).But this tactic hasn't yet been applied to OSAHS.Using a Mendelian randomization analysis,we analyzed the causal link between immune cells and the illness in order to comprehend the immunological bases of OSAHS.AIM To investigate the immune cells'association with OSAHS via genetic methods,guiding future clinical research.METHODS A comprehensive two-sample mendelian randomization study was conducted to investigate the causal relationship between immune cell characteristics and OSAHS.Summary statistics for each immune cell feature were obtained from the GWAS catalog.Information on 731 immune cell properties,such as morphologic parameters,median fluorescence intensity,absolute cellular,and relative cellular,was compiled using publicly available genetic databases.The results'robustness,heterogeneity,and horizontal pleiotropy were confirmed using extensive sensitivity examination.RESULTS Following false discovery rate(FDR)correction,no statistically significant effect of OSAHS on immunophenotypes was observed.However,two lymphocyte subsets were found to have a significant association with the risk of OSAHS:Basophil%CD33dim HLA DR-CD66b-(OR=1.03,95%CI=1.01-1.03,P<0.001);CD38 on IgD+CD24-B cell(OR=1.04,95%CI=1.02-1.04,P=0.019).CONCLUSION This study shows a strong link between immune cells and OSAHS through a gene approach,thus offering direction for potential future medical research.

Clinical efficacy and mechanism study of mid-frequency anti-snoring device in treating moderate obstructive sleep apnea-hypopnea syndrome

BACKGROUND Obstructive sleep apnea-hypopnea syndrome(OSAHS)is primarily caused by airway obstruction due to narrowing and blockage in the nasal and nasopha-ryngeal,oropharyngeal,soft palate,and tongue base areas.The mid-frequency anti-snoring device is a new technology based on sublingual nerve stimulation.Its principle is to improve the degree of oropharyngeal airway stenosis in OSAHS patients under mid-frequency wave stimulation.Nevertheless,there is a lack of clinical application and imaging evidence.METHODS We selected 50 patients diagnosed with moderate OSAHS in our hospital between July 2022 and August 2023.They underwent a 4-wk treatment regimen involving the mid-frequency anti-snoring device during nighttime sleep.Following the treatment,we monitored and assessed the sleep apnea quality of life index and Epworth Sleepiness Scale scores.Additionally,we performed computed tomo-graphy scans of the oropharynx in the awake state,during snoring,and while using the mid-frequency anti-snoring device.Cross-sectional area measurements in different states were taken at the narrowest airway point in the soft palate posterior and retrolingual areas.RESULTS Compared to pretreatment measurements,patients exhibited a significant reduction in the apnea-hypopnea index,the percentage of time with oxygen saturation below 90%,snoring frequency,and the duration of the most prolonged apnea event.The lowest oxygen saturation showed a notable increase,and both sleep apnea quality of life index and Epworth Sleepiness Scale scores improved.Oropharyngeal computed tomography scans revealed that in OSAHS patients cross-sectional areas of the oropharyngeal airway in the soft palate posterior area and retrolingual area decreased during snoring compared to the awake state.Conversely,during mid-frequency anti-snoring device treatment,these areas increased compared to snoring.CONCLUSION The mid-frequency anti-snoring device demonstrates the potential to enhance various sleep parameters in patients with moderate OSAHS,thereby improving their quality of life and reducing daytime sleepiness.These therapeutic effects are attributed to the device’s ability to ameliorate the narrowing of the oropharynx in OSAHS patients.

Idiopathic hypereosinophilic syndrome with hepatic sinusoidal obstruction syndrome:A case report and literature review

BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoidal obstruction syndrome(HSOS)is characterized by damage to the endothelial cells of the hepatic sinusoids of the hepatic venules,with occlusion of the hepatic venules,and hepatocyte necrosis.We report a case of IHES with HSOS of uncertain etiology.CASE SUMMARY A 70-year-old male patient was admitted to our hospital with pruritus and a rash on the extremities for>5 mo.He had previously undergone antiallergic treatment and herbal therapy in the local hospital,but the symptoms recurred.Relevant examinations were completed after admission.Bone marrow aspiration biopsy showed a significantly higher percentage of eosinophils(23%)with approximately normal morphology.Ultrasound-guided hepatic aspiration biopsy indicated HSOS.Contrast-enhanced computed tomography(CT)of the upper abdomen showed hepatic venule congestion with hydrothorax and ascites.The patient was initially diagnosed with IHES and hepatic venule occlusion.Prednisone,low molecular weight heparin and ursodeoxycholic acid were given for treatment,followed by discontinuation of low molecular weight heparin due to ecchymosis.Routine blood tests,biochemical tests,and imaging such as enhanced CT of the upper abdomen and pelvis were reviewed regularly.CONCLUSION Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.

Postoperative abdominal herpes zoster complicated by intestinal obstruction:A case report

BACKGROUND Intestinal obstruction is a common occurrence in clinical practice.However,the occurrence of herpes zoster complicated by intestinal obstruction after abdominal surgery is exceedingly rare.In the diagnostic and treatment process,clinicians consider it crucial to identify the primary causes of its occurrence to ensure effective treatment and avoiding misdiagnosis.CASE SUMMARY Herein,we present the case of a 40-year-old female patient with intestinal obstruction who underwent laparoscopic appendectomy and developed herpes zoster after surgery.Combining the patient's clinical manifestations and relevant laboratory tests,it was suggested that the varicella zoster virus reactivated during the latent period after abdominal surgery,causing herpes zoster.Subsequently,the herpes virus invaded the visceral nerve fibers,causing gastrointestinal dysfunction and loss of intestinal peristalsis,which eventually led to intestinal obstruction.The patient was successfully treated through conservative treatment and antiviral therapy and subsequently discharged from the hospital.CONCLUSION Pseudo-intestinal obstruction secondary to herpes zoster infection is difficult to distinguish from mechanical intestinal obstruction owing to various causes.In cases of inexplicable intestinal obstructions,considering the possibility of a viral infection is essential to minimize misdiagnosis and missed diagnoses。

Clinical profile and outcomes of hepatocellular carcinoma in primary Budd-Chiari syndrome

BACKGROUND There is scant literature on hepatocellular carcinoma(HCC)in patients with Budd-Chiari syndrome(BCS).AIM To assess the magnitude,clinical characteristics,feasibility,and outcomes of treatment in BCS-HCC.METHODS A total of 904 BCS patients from New Delhi,India and 1140 from Mumbai,India were included.The prevalence and incidence of HCC were determined,and among patients with BCS-HCC,the viability and outcomes of interventional therapy were evaluated.RESULTS In the New Delhi cohort of 35 BCS-HCC patients,18 had HCC at index presentation(prevalence 1.99%),and 17 developed HCC over a follow-up of 4601 person-years,[incidence 0.36(0.22-0.57)per 100 person-years].BCS-HCC patients were older when compared to patients with BCS alone(P=0.001)and had a higher proportion of inferior vena cava block,cirrhosis,and long-segment vascular obstruction.The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up(13029 ng/mL vs 500 ng/mL,P=0.01).Of the 35 BCS-HCC,26(74.3%)underwent radiological interventions for BCS,and 22(62.8%)patients underwent treatment for HCC[transarterial chemoembolization in 18(81.8%),oral tyrosine kinase inhibitor in 3(13.6%),and transarterial radioembolization in 1(4.5%)].The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo(P=0.0001).In contrast to the New Delhi cohort,the Mumbai cohort of BCS-HCC patients were predominantly males,presented with a more advanced HCC[Barcelona Clinic Liver Cancer C and D],and 2 patients underwent liver transplantation.CONCLUSION HCC is not uncommon in patients with BCS.Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.

Pyrrolizidine alkaloids-induced hepatic sinusoidal obstruction syndrome: Pathogenesis, clinical manifestations, diagnosis,treatment, and outcomes

Hepatic sinusoidal obstruction syndrome (HSOS) can be caused by the intake of pyrrolizidine alkaloids (PAs). To date, PAs-induced HSOS has not been extensively studied. In view of the difference in etiology of HSOS between the West and China, clinical profiles, imaging findings, treatment, and outcomes of HSOS associated with hematopoietic stem cell transplantation or oxaliplatin might be hardly extrapolated to PAs-induced HSOS. Reactive metabolites derived from PAs form pyrrole-protein adducts that result in toxic destruction of hepatic sinusoidal endothelial cells. PAs-induced HSOS typically manifests as painful hepatomegaly, ascites, and jaundice. Laboratory tests revealed abnormal liver function tests were observed in most of the patients with PAs-induced HSOS. In addition, contrast computed tomography and magnetic resonance imaging scan show that patients with PAs-induced HSOS have distinct imaging features, which reveal that radiological imaging provides an effective noninvasive method for the diagnosis of PAs-induced HSOS. Liver biopsy and histological examination showed that PAs-induced HSOS displayed distinct features in acute and chronic stages. Therapeutic strategies for PAs-induced HSOS include rigorous fluid management, anticoagulant therapy, glucocorticoids, transjugular intrahepatic portosystemic shunt, liver transplantation, etc. The aim of this review is to describe the pathogenesis, clinical profiles, diagnostic criteria, treatment, and outcomes of PAs-induced HSOS.

Long-term follow-up of distal intestinal obstruction syndrome in cystic fibrosis

AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome(DIOS) in Israeli cystic fibrosis(CF) patients.METHODS: This is a multi-center,comparative,retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012.Patients diagnosed with DIOS were defined as the study group.The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan.For the control group,CF patients with no DIOS were matched to the patients in the study group for age,sex,and cystic fibrosis transmembrane conductance regulator(CFTR) mutations.For both groups,the collected data included age,sex,CFTR genotype,weight,height,and body mass index.Clinical data included respiratory function tests in the last five years prior to the study,respiratory function test immediately before and after the DIOS event,number of hospitalizations,sputum culture results,and CFrelated conditions diagnosed according to the CF clinical practice guidelines.In the study group,data on the DIOS treatment and tendency for DIOS recurrence were also analyzed.RESULTS: The medical charts for a total of 350 CF patients were reviewed.Of the 350 CF patients,26(7.4%) were diagnosed with DIOS.The control group included 31 CF patients with no DIOS diagnosis.The mean follow-up period was 21.6 ± 8.2 years.The total of DIOS episodes in the follow-up period was 60.The distribution of DIOS episodes was as follows: 6/26(23.1%) study patients had one episode of DIOS intheir lifetime,7/26(26.9%) had two episodes,7/26(26.9%) had three episodes,and 6/26(23.1%) had four or more episodes.Compared to the control group,DIOS patients had a significantly higher incidence of meconium ileus in the past(65.4% vs 0%,respectively,P < 0.02),more Aspergillus spp.colonization(34.6% vs 3.2%,respectively,P < 0.02),and a higher number of hospitalizations due to respiratory exacerbations(8.6 vs 6.2 mean total hospitalizations per follow-up period,respectively,P < 0.02).No other significant differences were found between the control and study groups.The conservative treatment of DIOS,which mainly includes hydration and stool softeners,was successful in 82% of the episodes.The survival rate was similar for both groups.CONCLUSION: CF patients with DIOS suffer from recurrent hospitalizations and airway pathogen acquisition.Although recurrence of DIOS is common,conservative treatment is successful in most patients.

Rare etiology of mechanical intestinal obstruction: Abdominal cocoon syndrome

Abdominal cocoon syndrome is a rare cause of intestinal obstruction with unknown etiology. Diagnosis of this syndrome, which can be summarized as the small intestine being surrounded by a fibrous capsule not containing the mesothelium, is difficult in the preoperative period. A 47-year-old male patient was referred to the emergency department with complaints of abdominal pain, nausea, and vomiting for two days. The abdominal computed tomography examination detected dilated small intestinal loops containing air-fluid levels clustered in the left upper quadrant of the abdomen and surrounded by a thick, saclike, contrast-enhanced membrane. During exploratory surgery, a capsular structure was identified in the upper left quadrant with a regular surface that was solid-fibrous in nature. Ab-dominal cocoon syndrome is a rarely seen condition, for which the preoperative diagnosis is difficult. The combination of physical examination and radiological signs, and the knowledge of "recurrent characteristics of the complaints" that can be learned by a careful history, may be helpful in diagnosis.

Idiopathic abdominal cocoon syndrome with unilateral abdominal cryptorchidism and greater omentum hypoplasia in a young case of small bowel obstruction

Abdominal cocoon syndrome(ACS) is a rare cause of intestinal obstruction due to total or partial encapsulation of the small intestine by a fibrocollagenous membrane. Idiopathic ACS with abdominal cryptorchidism and greater omentum hypoplasia is even rarer clinically. We successfully treated a 26-year-old male case of small bowel obstruction with acute peritonitis. He was finally diagnosed with idiopathic ACS with unilateral abdominal cryptorchidism and greater omentum hypoplasia during exploratory laparotomy. He then underwent enterolysis, cryptorchidectomy, and appendectomy. He recovered gradually from the operations and early postoperative inflammatory ileus. There has been no recurrence of intestinal obstruction since the operation, and he is still in follow-up. We analyzed his clinical data and retrospectively reviewed the literature, and our findings may be helpful for the clinical diagnosis and treatment on ACS.

Evaluation of Airway Obstruction at Soft Palate Level in Male Patients with Obstructive Sleep Apnea/Hypopnea Syndrome:Dynamic 3-Dimensional CT Imaging of Upper Airway

This study examined the dynamic characteristics of upper airway collapse at soft palate level in patients with obstructive sleep apnea/hypopnea syndrome（OSAHS） by using dynamic 3-Dimensional（3-D） CT imaging.A total of 41 male patients who presented with 2 of the following symptoms,i.e.,daytime sleepiness and fatigue,frequent snoring,and apnea with witness,were diagnosed as having OSAHS.They underwent full-night polysomnography and then dynamic 3-D CT imaging of the upper airway during quiet breathing and in Muller＇s maneuver.The soft palate length（SPL）,the minimal cross-sectional area of the retropalatal region（mXSA-RP）,and the vertical distance from the hard palate to the upper posterior part of the hyoid（hhL） were compared between the two breathing states.These parameters,together with hard palate length（HPL）,were also compared between mild/moderate and severe OSAHS groups.Association of these parameters with the severity of OSAHS [as reflected by apnea hypopnea index（AHI） and the lowest saturation of blood oxygen（LSaO2）] was examined.The results showed that 31 patients had severe OSAHS,and 10 mild/moderate OSAHS.All the patients had airway obstruction at soft palate level.mXSA-RP was significantly decreased and SPL remarkably increased during Muller＇s maneuver as compared with the quiet breathing state.There were no significant differences in these airway parameters（except the position of the hyoid bone） between severe and mild/moderate OSAHS groups.And no significant correlation between these airway parameters and the severity of OSAHS was found.The position of hyoid was lower in the severe OSAHS group than in the mild/moderate OSAHS group.The patients in group with body mass index（BMI）≥26 had higher collapse ratio of mXSA-RP,greater neck circumference and smaller mXSA-RP in the Muller＇s maneuver than those in group with BMI26（P0.05 for all）.It was concluded that dynamic 3-D CT imaging could dynamically show the upper airway changes at soft palate level in OSAHS patients.All the OSAHS patients had airway obstruction of various degrees at soft palate level.But no correlation was observed between the airway change at soft palate level and the severity of OSAHS.The patients in group with BMI≥26 were more likely to develop airway obstruction at soft palate level than those with BMI26.

Transjugular intrahepatic portosystemic shunt for pyrrolizidine alkaloid-related hepatic sinusoidal obstruction syndrome

BACKGROUND Treatments for hepatic sinusoidal obstruction syndrome(HSOS)are limited.AIM To evaluate transjugular intrahepatic portosystemic shunting(TIPS)as a treatment for pyrrolidine alkaloid-related HSOS(PA-HSOS).METHODS This retrospective analysis included patients with PA-HSOS admitted to the First Affiliated Hospital of the University of Science and Technology of China(June 2015 to January 2019).Baseline clinical characteristics and follow-up data were extracted from the medical records.All patients included in this study experienced failure of initial therapy.Patients were divided into the TIPS and conservative treatment groups according to the therapy they received.Liver function,maximal ascites depth,imaging characteristics,pathology findings,and survival were compared between groups.RESULTS The TIPS group included 37 patients(28 males),and the conservative treatment group included 17 patients(11 males).Baseline characteristics were similar between groups.There were two deaths in the TIPS group and seven deaths in the conservative treatment group during follow-up(3-48 mo).The 3-,6-,12-and 24-mo survival rates were 94.6%,94.6%,94.6%and 94.6%,respectively,in the TIPS group and 70.6%,57.8%,57.8%and 57.8%,respectively,in the conservative treatment group.Kaplan-Meier analysis revealed significantly longer survival for the TIPS group than for the conservative treatment group(P=0.001).Compared with the pre-treatment value,maximal ascites depth was significantly lower at 1 wk,2 wk,1 mo,and 3 mo for the TIPS group(all P<0.05)but not in the conservative treatment group.Contrast-enhanced computed tomography demonstrated the disappearance of patchy liver enhancement after TIPS.Pathology showed that liver congestion and hepatocyte swelling improved with time after TIPS placement.CONCLUSION TIPS may achieve better outcomes than conventional symptomatic treatment in patients with PA-HSOS.

Laparoscopic treatment of an upper gastrointestinal obstruction due to Bouveret's syndrome

Bouveret's syndrome is an extremely rare type of gallstone-induced ileus with atypical clinical manifestations,such as abdominal distension and pain,nausea and vomiting,fever or even gastrointestinal bleeding,which may easily be misdiagnosed. In the present case,a 55-year-old male was admitted to the hospital with upper gastrointestinal obstructive symptoms but without pain,fever,jaundice or melena. At first,gastrolithiasis and peptic ulcer combined with pyloric obstruction were suspected after gastroscopy revealed a large,hard stone in the duodenal bulb. A revised diagnosis of Bouveret's syndrome was made following abdominal computed tomography. Subsequently,the patient exhibited a good postoperative recovery after laparoscopic duodenotomy for gallstone removal and subtotal cholecystectomy. The condition of the patient remained stable after being followed up for 6 mo. The successful application of laparoscopic therapy to treat Bouveret's syndrome has seldom been reported. Laparoscopic enterolithotomy is safe and effective,with good patient tolerability,rapid postoperative recovery and few wound-related complications. The laparoscopic treatment of Bouveret's syndrome is worth exploring.

Liver stiffness and perfusion changes for hepatic sinusoidal obstruction syndrome in rabbit model

BACKGROUND Hepatic sinusoidal obstruction syndrome(SOS)is caused by damage to hepatic sinusoidal endothelial cells that results in fibrous obliteration of intrahepatic venules and necrosis of hepatocytes.Currently the diagnosis is primarily based on nonspecific clinical features and invasive liver biopsy.Therefore,noninvasive imaging methods are required for the early diagnosis and severity assessment of hepatic SOS.AIM To determine the effectiveness of supersonic shear wave imaging(SSI)and dual energy computed tomography(DECT)for diagnosing hepatic SOS using a rabbit model.METHODS Among nine New Zealand white rabbits(3-4 kg,male),three in control group ingested normal saline for 20 d and six in the SOS group ingested 6-thioguanine(5 mg/kg/d)for 20 d.Liver stiffness was measured using SSI on days 0,3,10,and 20.On the same days,liver perfusion was evaluated from virtual monochromatic images of 55 keV and iodine map using DECT.Morphologic changes in the liver were assessed using CT.Final pathology scores were compared between the two groups.Liver stiffness and perfusion parameters were compared according to the groups,days,and pathology scores.RESULTS Final pathology scores were significantly higher in the SOS than the control group(median 22 vs 2,P=0.024).No gross morphologic changes were seen in livers.Liver stiffness,Hounsfield Unit values,and iodine concentrations were higher in the SOS compared to the control group on days 10 and 20(all,P≤0.007).Compared to day 0,liver stiffness and perfusion parameters were higher on day 20 in the SOS group(all,P≤0.001).Correlation coefficients for liver stiffness(r=0.635),Hounsfield Unit values(r=0.587),and iodine concentration(r=0.611)with final pathology scores were positive without significance(all,P>0.05).CONCLUSION Liver stiffness and perfusion parameters were significantly increased in the livers of a rabbit SOS model.SSI and DECT might aid in early diagnosis of hepatic SOS.

Sinusoidal obstruction syndrome: A systematic review of etiologies,clinical symptoms, and magnetic resonance imaging features

BACKGROUND Sinusoidal obstruction syndrome (SOS) is a kind of rare liver disease which is characterized by damage to small hepatic vessels, affecting particularly the sinusoidal endothelium. Due to the special etiology and high mortality, early diagnosis of SOS is significant for clinical survival and prognosis. AIM To generalize the common etiologies and clinical symptoms of SOS and summarize the characteristic magnetic resonance imaging (MRI) features so as to provide more valuable information for early diagnosis of SOS. METHOD We searched PubMed, Web of science, Wanfang Data, China Knowledge Resource Integrated, VIP, and Cochrane Library databases without a limiting period and the types of articles. The search process mainly revolved around the etiologies, common clinical symptoms, and MRI imaging features of SOS. Ultimately, 29 full articles were included in this review and 222 articles were excluded. RESULTS Eleven case reports included 13 patients. The etiologies of these patients including chemotherapy (5/13), medicinal herbs containing pyrrolidine alkaloids (PAs, e.g. Tusanqi)(4/13), hematopoietic stem cell transplantation (HSCT)(2/13), drug toxicity (6-thioguanine)(1/13), and “poppers”, a recreational drug used during anal intercourse (1/13). Eighteen case series including 497 patients, and SOS in 465 (93.6%) patients was caused by PAs. Ascites, abdominal pain and swelling, jaundice were the most common clinical symptoms. Alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (GGT), total bilirubin (TBil), direct bilirubin (DBil), and prothrombin time (PT) had varying degrees of elevation.Heterogeneous signals on T1 weighted imaging/T2 weighted imaging (T1WI/T2WI), heterogeneous enhancement of liver parenchyma, ascites, hepatomegaly, narrowing and blurring of intrahepatic inferior vena cava and three main hepatic veins, edema around the portal vein, and gallbladder wall edema were the most common MRI imaging features of SOS. CONCLUSION In the West, SOS was mostly secondary to HSCT. Some SOS developed in the process of chemotherapy for hepatic metastatic tumor. A few SOS were caused by toxicity of certain drugs. In the East, Tusanqi was a major cause of SOS. Ascites, abdominal pain and swelling, jaundice were the common clinical symptoms. Elevations of ALT, AST, GGT, ALP, TBil, and DBil could be used as predictors of liver function damage. Numerous characteristic MRI imaging features could provide more valuable information for early diagnosis of SOS.

Primary hepatic angiosarcoma manifesting as hepatic sinusoidal obstruction syndrome:A case report

BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a high mortality.To our knowledge,PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome.Herein,we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome,diagnosed using transjugular liver biopsy,that resulted in the death of the patient.CASE SUMMARY A 71-year-old man was admitted with the primary complaint of abdominal distension,decreased appetite,fatigue in the previous month,and loss of 10 kg of weight in the past 2 years.Both the liver and spleen were enlarged,and the liver had a medium-hard texture on percussion.Laboratory examinations were performed,and abdominal plain computed tomography(CT)and contrastenhanced CT showed hepatomegaly and splenomegaly,as well as diffuse lowdensity shadows distributed in the liver and spleen.Contrast-enhanced CT revealed diffuse,hypodense,nodular or flake shadows in the liver and heterogeneous enhancement in the spleen.A transjugular liver biopsy was performed.Based on the pathology results,the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA.The patient’s status further deteriorated and he developed serious hepatic failure.The patient was discharged,and died 3 d later.CONCLUSION PHA is rare and has a poor prognosis;however,transjugular liver biopsy can be safely performed to aid in diagnosis.

Primary Sjgren's Syndrome Accompanied by Intestinal Obstruction: a Case Report and Literature Review

SJ(O)GREN'S syndrome (SS) is a chronic inflammatory autoimmune disease characterized by the infiltration of lymphocytes and plasma cells in exocrine glands,especially salivary and lacrimal gland interstitium.The clinical manifestations of SS are complex.When the digestive system is involved,the patient always presents with liver damage,atrophic gastritis,and chronic diarrhea.Intestinal obstruction is rare and the specific pathogenesis remains unclear.1 Currently,there is no clear treatment guidelines for SS with intestinal obstruction.The general measures include gastrointestinal decompression,spasmolysis,and other symptomatic treatments and the use of glucocorticoid to control the progressing of disease.Here we describe a case of primary SS with intestinal obstruction who was treated with conservative treatment.

Reversible sinusoidal obstruction syndrome associated with tacrolimus following liver transplantation

Sinusoidal obstruction syndrome(SOS), previously known as hepatic veno-occlusive disease, is a rare disorder in solid organ transplant patients, and is an uncommon complication after liver transplantation. Severe SOS with hepatic failure causes considerable mortality. Tacrolimus has been reported to be an offending agent, which potentially plays a role in the pathophysiological process of SOS. SOS due to tacrolimus has been reported in lung and pancreatic transplantations, but has never been described in a liver transplant recipient. Herein, we present a case of SOS after liver transplantation, which was possibly related to tacrolimus. A 27-year-old man developed typical symptoms of SOS with painful hepatomegaly, ascites and jaundice after liver transplantation, which regressed following withdrawal of tacrolimus. By excluding other possible predisposing factors, we concluded that tacrolimus was the most likely cause of SOS.

Intermittent gastric outlet obstruction due to a gallstone migrated through a cholecysto-gastric fistula:A new variant of “Bouveret’s syndrome”

Bouveret's syndrome, defined as gastric outlet obstruction due to a large gallstone, is still one of the most dramatic biliary gallstone complications. Although new radiological and endoscopic techniques have made pre-surgical diagnosis possible in most cases and the death rate has dropped dramatically, "one-stage surgery" (biliary surgery carried out at the same time as the removal of the gut obstruction) should be still considered as the gold standard for the treatment of gallstone ileus.In this case, partial gastric outlet obstruction resulted in an atypical and insidious clinical presentation that allowed us to perform the conventional one-stage laparatomic procedure that completely solved the problem, thus avoiding any further complications.

Hepatic Sinusoidal Obstruction Syndrome without Preceding Medical Events

Sinusoidal obstruction syndrome (SOS) is one of the severe complications of radiation, anticancer chemotherapy and immunosuppressive agents for transplantation. Autopsy of a case of rapidly progressive, uncontrollable severe ascites, without apparent signs of preceding drug toxicity, revealed a tensely enlarged liver and spleen, and 3000 ml of ascites attributed to secondary portal hypertension. Histopathological analysis disclosed sinusoidal endothelial damage and fibrous expansion from central veins. All the foregoing indicated hepatic SOS that needs to be included in the differential diagnosis of progressive ascites in patients without an apparent history of malignancy or transplantation.