Clinical manifestations,diagnosis and long-term prognosis of adult autoimmune enteropathy:Experience from Peking Union Medical College Hospital

BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis and prognosis.METHODS We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic criteria.RESULTS Diarrhea in AIE patients was characterized by secretory diarrhea.The common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and ileum.Villous blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal biopsies.Moreover,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte antibodies.All patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)days.Immunosuppressants were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s lymphoma.The cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,respectively.CONCLUSION Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult AIE.The long-term prognosis is still unsatisfactory despite corticosteroid and immunosuppressant medications,which highlights the need for early diagnosis and novel medications.

Pathogenesis of chronic enteropathy associated with the SLCO2A1 gene:Hypotheses and conundrums

Chronic enteropathy associated with the SLCO2A1 gene(CEAS)is a complex gastroenterological condition characterized by multiple ulcers in the small intestine with chronic bleeding and protein loss.This review explores the potential mechanisms underlying the pathogenesis of CEAS,focusing on the role of SLCO2A1-encoded prostaglandin transporter OATP2A1 and its impact on prostaglandin E2(PGE2)levels.Studies have suggested that elevated PGE2 levels contribute to mucosal damage,inflammation,and disruption of the intestinal barrier.The effects of PGE2 on macrophage activation and Maxi-Cl channel functionality,as well as its interaction with nonsteroidal anti-inflammatory drugs play crucial roles in the progression of CEAS.Understanding the balance between its protective and pro-inflammatory effects and the complex interactions within the gastrointestinal tract can shed light on potential therapeutic targets for CEAS and guide the development of novel,targeted therapies.

Autoantibodies related to ataxia and other central nervous system manifestations of gluten enteropathy

Gluten ataxia and other central nervous system disorders could be linked to gluten enteropathy and related autoantibodies.In this narrative review,we focus on the various neuro-logical manifestations in patients with gluten sensitivity/celiac disease,immunological and autoimmune mechanisms of ataxia in connection to gluten sensitivity and the autoantibodies that could be used as a biomarker for diagnosing and following.We focused on the anti-gliadin antibodies,antibodies to different isoforms of tissue transglutaminase(TG)(anti-TG2,3,and 6 antibodies),anti-glycine receptor antibodies,anti-glutamine acid decarboxylase antibodies,anti-deamidated gliadin peptides antibodies,etc.Most studies found a higher prevalence of these antibodies in patients with gluten sensitivity and neurological dysfunction,presented as different neurological disorders.We also discuss the role of a gluten-free diet on the clinical improvement of patients and also on imaging of these disorders.

Eosinophilic enteritis requiring differentiation from chronic enteropathy associated with SLCO2A1 gene:A case report

BACKGROUND Eosinophilic gastrointestinal disease(EGID)is a disorder characterized by infiltration of eosinophils causing mucosal damage and dysfunction of the gastrointestinal tract.The endoscopic findings of eosinophilic enteritis(EoN),an EGID variant,are nonspecific and occasionally difficult to diagnose.In contrast,chronic enteropathy associated with SLCO2A1(CEAS)is a chronic persistent small intestinal disorder characterized by endoscopic findings such as multiple oblique and circular ulcers.CASE SUMMARY We report the case of a 10-year-old boy who had suffered abdominal pain and fatigue for the preceding 6 mo.He was referred to our institute for investigation of suspected gastrointestinal bleeding because of severe anemia with hypoproteinemia and positive fecal human hemoglobin.The upper and lower gastrointestinal endoscopic findings were normal;however,double-balloon small bowel endoscopy showed multiple oblique and circular ulcers with discrete margins and mild constriction of the intestinal lumen in the ileum.The findings were highly consistent with CEAS,but urine prostaglandin metabolites were within normal limits,and no previously reported mutations in the SLCO2A1 gene were identified.Histological evaluation demonstrated moderate to severe eosinophilic infiltration localized to the small intestine suggesting a diagnosis of EoN.Clinical remission was maintained with montelukast and a partial elemental diet,but emergent surgery for bowel obstruction due to small intestinal stenosis was performed two years after the initial treatment.CONCLUSION EoN should be considered in the differential diagnosis of CEAS-like small intestinal ulcerative lesions and normal urinary prostaglandin metabolite levels.

Portal hypertensive enteropathy

Portal hypertensive enteropathy(PHE) is a condition that describes the pathologic changes and mucosal abnormalities observed in the small intestine of patients with portal hypertension. This entity is being increasingly recognized and better understood over the past decade due to increased accessibility of the small intestine made possible by the introduction of video capsule endoscopy and deep enteroscopy. Though challenged by its diverse endoscopic appearance, multiple scoring systems have been proposed to classify the endoscopic presentationand grade its severity. Endoscopic findings can be broadly categorized into vascular and non-vascular lesions with many subtypes of both categories. Clinical manifestations of PHE can range from asymptomatic incidental findings to fatal gastrointestinal hemorrhage. Classic endoscopic findings in the setting of portal hypertension may lead to a prompt diagnosis. Occasionally histopathology and cross sectional imaging like computed tomography or magnetic resonance imaging may be helpful in establishing a diagnosis. Management of overt bleeding requires multidisciplinary approach involving hepatologists, endoscopists, surgeons, and interventional radiologists. Adequate resuscitation, reduction of portal pressure, and endoscopic therapeutic intervention remain the main principles of the initial treatment. This article reviews the existing evidence on PHE with emphasis on its classification, diagnosis, clinical manifestations, endoscopic appearance, pathological findings, and clinical management. A new schematic management of ectopic variceal bleed is also proposed.

Mucosal healing effect of mesalazine granules in naproxen-induced small bowel enteropathy

AIM:To investigate the effect of mesalazine granules on small intestinal injury induced by naproxen using capsule endoscopy (CE).METHODS:This was a single center,non-randomized,open-label,uncontrolled pilot study,using the PillCam SB CE system with RAPID 5 software.The Lewis Index Score (LIS) for small bowel injury was investigated to evaluate the severity of mucosal injury.Arthropathy patients with at least one month history of daily naproxen use of 1000 mg and proton pump inhibitor co-therapy were screened.Patients with a minimum LIS of 135 were eligible to enter the 4-wk treatment phase of the study.During this treatment period,3 × 1000 mg/d mesalazine granules were added to ongoing therapies of 1000 mg/d naproxen and 20 mg/d omeprazole.At the end of the 4-wk combined treatment period,a second small bowel CE was performed to re-evaluate the enteropathy according to the LIS results.The primary objective of this study was to assess the mucosal changes after 4 wk of mesalazine treatment.RESULTS:A total of 18 patients (16 females),ranging in age from 46 to 78 years (mean age 60.3 years) were screened,all had been taking 1000 mg/d naproxen for at least one month.Eight patients were excluded from the mesalazine therapeutic phase of the study for the following reasons:the screening CE showed normal small bowel mucosa or only insignificant damages (LIS < 135) in five patients,the screening esophagogastroduodenoscopy revealed gastric ulcer in one patient,capsule technical failure and incomplete CE due to poor small bowel cleanliness in two patients.Ten patients (9 female,mean age 56.2 years) whose initial LIS reached mild and moderate-to-severe enteropathy grades (between 135 and 790 and ≥ 790) entered the 4-wk therapeutic phase and a repeat CE was performed.When comparing the change in LIS from baseline to end of treatment in all patients,a marked decrease was seen (mean LIS:1236.4 ± 821.9 vs 925.2 ± 543.4,P=0.271).Moreover,a significant difference between pre-and post-treatment mean total LIS was detected in 7 patients who had moderate-tosevere enteropathy gradings at the inclusion CE (mean LIS:1615 ± 672vs 1064 ± 424,P=0.033).CONCLUSION:According to the small bowel CE evaluation mesalazine granules significantly attenuated mucosal injuries in patients with moderate-to-severe enteropathies induced by naproxen.

New mutation in EPCAM for congenital tufting enteropathy:A case report

BACKGROUND Congenital tufting enteropathy(CTE)is a rare cause of diarrhea in children.However,it can result in early-onset of chronic diarrhea and failure to thrive.Children with this disease have to depend on total parenteral nutrition(TPN),and eventually small intestine transplantation.The epithelial cell adhesion molecule(EPCAM)gene was identified to be associated with CTE.Here,we present a case of an infant with CTE due to a mutation not reported in the literature before.CASE SUMMARY A 1-year and 7-mo infant boy exhibited intractable watery diarrhea and mushy stool within 1 wk after birth,for which he had required medical treatment and hospitalization several times.His sister presented similar symptoms and died at the age of two.On admission,his body weight was 5700 g(-4.8 SDS)and measured 66 cm(-5.4 SDS)in height.Meanwhile,he cannot speak or climb.He exhibited mild anemia,hypocalcemia,hypomagnesemia,and an infection in the upper respiratory tract.Microvilli sparse and vacuolar degeneration of epithelial cells were reported by small intestine biopsy.Whole-exome sequencing showed a novel homozygous splice mutation(c.657+1[IVS6]G>A)in the EPCAM gene.He was treated with TPN and recombinant human growth hormone.After 2 mo,his body weight was up to 8500 g and he has been waiting for small bowel transplantation.CONCLUSION CTE is rare but fatal.Patients with CTE require rapid diagnosis and therapy to improve their survival.

Involvement of heat shock proteins in gluten-sensitive enteropathy

Gluten-sensitive enteropathy,also known as coeliac disease(CD),is an autoimmune disorder occurring in genetically susceptible individuals that damages the small intestine and interferes with the absorption of other nutrients.As it is triggered by dietary gluten and related prolamins present in wheat,rye and barley,the accepted treatment for CD is a strict gluten-free diet.However,a complete exclusion of gluten-containing cereals from the diet is often difficult,and new therapeutic strategies are urgently needed.A class of proteins that have already emerged as drug targets for other autoimmune diseases are the heat shock proteins(HSPs),which are highly conserved stress-induced chaperones that protect cells against harmful extracellular factors.HSPs are expressed in several tissues,including the gastrointestinal tract,and their levels are significantly increased under stress circumstances.HSPs exert immunomodulatory effects,and also play a crucial role in the maintenance of epithelial cell structure and function,as they are responsible for adequate protein folding,influence the degradation of proteins and cell repair processes after damage,and modulate cell signalling,cell proliferation and apoptosis.The present review discusses the involvement of HSPs in the pathophysiology of CD.Furthermore,HSPs may represent a useful therapeutic target for the treatment of CD due to the cytoprotective,immunomodulatory,and anti-apoptotic effects in the intestinal mucosal barrier.

Fibrinogen deficiency suppresses the development of early and delayed radiation enteropathy

AIM To determine the mechanistic role of fibrinogen, a key regulator of inflammation and fibrosis, in early and delayed radiation enteropathy.METHODS Fibrinogen wild-type(Fib+/+), fibrinogen heterozygous(Fib+/-), and fibrinogen knockout(Fib-/-) mice were exposed to localized intestinal irradiation and assessed for early and delayed structural changes in the intestinal tissue. A 5-cm segment of ileum of mice was exteriorized and exposed to 18.5 Gy of x-irradiation. Intestinal tissue injury was assessed by quantitative histology, morphometry, and immunohistochemistry at 2 wk and 26 wk after radiation. Plasma fibrinogen level was measured by enzyme-linked immunosorbent assay. RESULTS There was no difference between sham-irradiated Fib+/+ and Fib+/-mice in terms of fibrinogen concentration in plasma and intestinal tissue, intestinal histology, morphometry, intestinal smooth muscle cell proliferation, and neutrophil infiltration. Therefore, Fib+/-mice were used as littermate controls. Unlike sham-irradiated Fib+/+ and Fib+/-mice, no fibrinogen was detected in the plasma and intestinal tissue of sham-irradiated Fib-/-mice. Moreover, fibrinogen level was not elevated after irradiation in the intestinal tissue of Fib-/-mice, while significant increase in intestinal fibrinogen level was noticed in irradiated Fib+/+ and Fib+/-mice. Importantly, irradiated Fib-/-mice exhibited substantially less overall intestinal structural injury(RIS, P = 0.000002), intestinal wall thickness(P = 0.003), intestinal serosal thickness(P = 0.009), collagen deposition(P = 0.01), TGF-β immunoreactivity(P = 0.03), intestinal smooth muscle proliferation(P = 0.046), neutrophil infiltration(P = 0.01), and intestinal mucosal injury(P = 0.0003), compared to irradiated Fib+/+ and Fib+/-mice at both 2 wk and 26 wk. CONCLUSION These data demonstrate that fibrinogen deficiency directly attenuates development of early and delayed radiation enteropathy. Fibrinogen could be a novel target in treating intestinal damage.

Protein-losing enteropathy caused by a jejunal ulcer after an internal hernia in Petersen's space: A case report

BACKGROUND The incidence of internal hernias has recently increased in concordance with the popularization of laparoscopic surgery.Of particular concern are internal hernias occurring in Petersen's space,a space that is surgically created after treatment for gastric cancer and obesity.These hernias cause devastating sequelae,such as massive intestinal necrosis,fatal Roux limb necrosis,and superior mesenteric vein thrombus.In addition,protein-losing enteropathy(PLE)is a rare syndrome involving gastrointestinal protein loss,although its relationship with internal Petersen’s hernias remains unknown.CASE SUMMARY A 75-year-old man with a history of laparotomy for early gastric cancer developed Petersen's hernia 1 year and 5 mo after surgery.He was successfully treated by reducing the incarcerated small intestine and closure of Petersen’s defect without resection of the small intestine.Approximately 3 mo after his surgery for Petersen’s hernia,he developed bilateral leg edema and hypoalbuminemia.He was diagnosed with PLE with an alpha-1 antitrypsin clearance of 733 mL/24 h.Double-balloon enteroscopy revealed extensive jejunal ulceration as the etiology,and it facilitated minimum bowel resection.Pathological analysis showed extensive jejunal ulceration and collagen hyperplasia with nonspecific inflammation of all layers without lymphangiectasia,lymphoma,or vascular abnormalities.His postoperative course was unremarkable,and his bilateral leg edema and hypoalbuminemia improved after 1 mo.There was no relapse over the 5-year follow-up period.CONCLUSION PLE and extensive jejunal ulceration may occur after Petersen's hernia.Doubleballoon enteroscopy helps identify and resect these lesions.

Gut commensal bacteria, Paneth cells and their relations to radiation enteropathy

In steady state, the intestinal epithelium forms an important part of the gut barrier to defend against luminal bacterial attack. However, the intestinal epithelium is compromised by ionizing irradiation due to its inherent selfrenewing capacity. In this process, small intestinal bacterial overgrowth is a critical event that reciprocally alters the immune milieu. In other words, intestinal bacterial dysbiosis induces inflammation in response to intestinal injuries, thus influencing the repair process of irradiated lesions. In fact, it is accepted that commensal bacteria can generally enhance the host radiation sensitivity. To address the determination of radiation sensitivity, we hypothesize that Paneth cells press a critical "button" because these cells are central to intestinal health and disease by using their peptides, which are responsible for controlling stem cell development in the small intestine and luminal bacterial diversity. Herein,the most important question is whether Paneth cells alter their secretion profiles in the situation of ionizing irradiation. On this basis, the tolerance of Paneth cells to ionizing radiation and related mechanisms by which radiation affects Paneth cell survival and death will be discussed in this review. We hope that the relevant results will be helpful in developing new approaches against radiation enteropathy.

Autoimmune enteropathy and primary biliary cholangitis after proctocolectomy for ulcerative colitis:A case report and review of the literature

BACKGROUND Autoimmune enteropathy(AIE)and primary biliary cholangitis(PBC)are both immune-mediated diseases.AIE or PBC complicated with ulcerative colitis(UC)are rare.There are no cases of AIE and PBC diagnosed after proctocolectomy for UC reported before,and the pathogenesis of these comorbidities has not been revealed.CASE SUMMARY A middle-aged woman diagnosed with UC underwent subtotal colectomy and ileostomy due to the steroid-resistant refractory disease,and a restorative proctectomy with ileal pouch-anal anastomosis and proximal neoileostomy was postponed due to active residual rectal inflammation in January 2016.A few months after the neoileostomy,she began to suffer from recurrent episodes of watery diarrhea.She was diagnosed with postcolectomy enteritis and stoma closure acquired a good therapeutic effect.However,her symptoms of diarrhea relapsed in 2019,with different histological features of endoscopic biopsies compared with 2016,which showed apoptotic bodies,a lack of goblet and Paneth cells,and villous blunting.A diagnosis of AIE was established,and the patient’s stool volume decreased dramatically with the treatment of methylprednisolone 60 mg/d for 1 wk and tacrolimus 3 mg/d for 4 d.Meanwhile,her constantly evaluated cholestatic enzymes and high titers of antimitochondrial antibodies indicated the diagnosis of PBC,and treatment with ursodeoxycholic acid(16 mg/kg per day)achieved satisfactory results.CONCLUSION Some immune-mediated diseases may be promoted by operation due to microbial alterations in UC patients.Continuous follow-up is essential for UC patients with postoperative complications.

Experimental Study on the Correlation of Nitric Oxide with Portal Hypertensive Enteropathy

To explore the role of nitric oxide (NO) in the pathogenesis of portal hypertensive enteropathy (PHE), cirrhotic portal hypertension was induced in Wistar rats by subcutaneous administration of carbon tetrachloride. At the end of 12th week, NADPH-diaphorase staining was performed on ice frozen sections with the sample of fresh colonic tissues. NO synthase (NOS) activities and NOS mRNA expression of colonic tissues were investigated with chemoluminescence method and reverse transcription-polymerase chain reaction (RT-PCR), respectively. After NADPH-diaphorase histochemical staining, the computer image analysis and paired t test showed that NOS staining intensities of submucosal vascular endothelial cells and nerve fibers were all significantly higher in PHE group than those in normal group (P<0. 01 and P<0. 05, respectively), but the intensities of superficial epithelial cells were significantly lower than those of controls (P<0. 01). Chemoluminescence method demonstrated that general NOS activity of colonic mucosa was significantly higher in PHE group than that in control group (P<0. 01). Moreover, the degree of iNOS activity increase was greater than that of cNOS (104 % vs 35 %). RT-PCR revealed that NOS mRNA expressions were dramatically higher in PHE group than those in control group (P<0. 01). The results suggested that NO, with its property of vasodilatation, may be involved in the pathogenesis of vascular lesions of PHE in cirrhotic rats, and may also has something to do with mucosal lesions of colon in PHE.

Early-onset refractory diarrhea due to immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome associated with a novel mutation in the FOXP3 gene: A case report

BACKGROUND Immune dysregulation,polyendocrinopthy,enteropathy,X-linked(IPEX)syndrome is a rare X-linked recessive disease caused by mutations in the forkhead box protein 3(FOXP3)gene,which is a master transcriptional regulator for the development and function of CD4+CD25+regulatory T(Treg)cells.The dysfunction of these cells leads to multiple system autoimmune diseases.We present a case of IPEX due to a mutation not reported in the literature before.CASE SUMMARY We report a male patient with IPEX syndrome who presented with refractory diarrhea and malabsorption leading to failure to thrive,as well as with hypothyroidism and nephrotic syndrome.Laboratory investigation showed increased total IgE and Treg cells,decreased free triiodothyronine(FT3)and free thyroxine(FT4),and proteinuria.Multiple dietary and supportive treatments were introduced but did not improve the diarrhea during his hospital stay.Ultimately,whole exome sequencing revealed that the patient was hemizygous for the exon 5,c.542G>A(p.Ser181Asn)mutation of the FOXP3 gene,which has not been previously reported.The patient remains on prednisone and euthyrox while awaiting hematopoietic stem cell transplantation at the time of the compilation of this case report.CONCLUSION We report a novel FOXP3 gene mutation involved in IPEX.A high level of suspicion should be maintained in an early-onset refractory diarrhea patient.

The mechanism of Wumei Wan on treating diabetes enteropathy based on the network pharmacology

Objective:To explore the potential mechanism of Wumei Wan(WMW)in treating diabetes enteropathy(DE)basing on network pharmacology.Methods:The effective compound of WMW were collected by TCMSP,the potential target of WMW was obtained by means of PubChem and Swiss target prediction online tools,and the disease target of DE was obtained by Genecards,TTD and DisGeNET databases,Cytoscape 3.7.2 software was used to construct active ingredients of WMW-potential target-DE network,protein interaction network(PPI)was constructed by STRING database.In order to understanding the mechanism of WMW treating in DE,Omicshare platform was used for GO analysis and KEGG pathway enrichment analysis.The analysis results were verified through docking by Discovery Studio 2016.Results:Total of 128 active components and 139 targets of WMW were screened out from the ten drugs.A total of 714 disease targets were screened out from the disease databases.24 common targets were identified from both WMW and DE.AKT1,MMP9,SRC,PTGS2,PPARG,NOS2,etc.are potential major targets of WMW in the treatment of DE.61 entries(p<0.05)were enriched in GO biological process function related to fatty acid anabolism and ligand receptor binding,as unsaturated fatty acid metabolic process,icosanoid metabolic process,enzyme linked receptor protein signaling pathway,protein amino acid phosphorylation,cellular response to insulin stimulus.A total of 72 signaling pathways were obtained through KEGG pathway analysis(p<0.05).The signaling pathways closely related to DE are including relaxin signaling pathway,EGFR tyrosine kinase inhibitor resistance,CLRs signaling pathway,and VEGF signaling pathway.Conclusion:This study preliminarily revealed the material basis and mechanism of WMW in the treatment of DE from the synergistic aspects of intestinal immune balance,gastrointestinal wall structure reconstruction,intestinal microvascular disorder and neuronal activity.

Late post liver transplant protein losing enteropathy: Rare complication of incisional hernia

Development of oedema and hypoproteinaemia in a liver transplant recipient may be the first signs of graft dysfunction and should prompt a full assessment. We report the novel case of a patient who, years after liver transplantation developed a functional blind loop in an incisional hernia, which manifested as oedema and hypoproteinaemia secondary to protein losing enteropathy. After numerous investigations, the diagnosis was made by flurodeoxyglucose positron emmision tomography (FDG-PET) imaging. Surgical repair of the incisional hernia was followed several months later by resolution of the protein loss, and confirmed at a post operative FDG-PET scan at one year.

Lymphangiomatosis associated with protein losing enteropathy:A case report

BACKGROUND Lymphangiomatosis is a multisystem disorder that is rarely localized to the gastrointestinal tract.Lymphangiomatosis usually has no specific clinical presentation and is easily misdiagnosed.A case report and review of the literature on lymphangiomatosis associated with protein-losing enteropathy will help to improve the overall understanding of this disease.CASE SUMMARY We report a case of lymphangiomatosis of the bowel and other solid organs.A 78-year-old man presented with recurrent bowel bleeding and protein-losing enteropathy,as well as cystic lesions in the spleen,liver,and kidney.Imaging examinations revealed many cystic lesions on the spleen,liver,kidney,and thickened wall of the ascending colon,as well as pleural effusion and ascites.Colonoscopy revealed a strawberry mucosa,variable spontaneous bleeding,and surface erosion located in the terminal ileum.Several cystic masses with a translucent and smooth surface as well as diffuse white spots were located in the colon.A laterally spreading tumor(LST)was located in the ascending colon.Pathology indicated highly differentiated adenocarcinoma(LST)and lymphangiomatoid dilation,and D2-40 was positive.The final diagnosis was lymphangiomatosis.The patient underwent surgery for LST and then was administered thalidomide 75-150 mg/d.His condition,however,did not improve.He eventually died 6 mo after the initial diagnosis.CONCLUSION Lymphangiomatosis usually occurs diffusely and can involve many organs,such as the spleen,kidney,liver,lung,mesentery,and bowel.Recurrent bowel bleeding or protein-losing enteropathy is an important indicator that should alert clinicians about the possibility of this disease when it afflicts the bowel.Doctors should improve the medical understanding of lymphangiomatosis.

Experience of primary intestinal lymphangiectasia in adults: Twelve case series from a tertiary referral hospital

BACKGROUND While primary intestinal lymphangiectasia(PIL)is considered a rare condition,there have been several reported cases in adults.Nevertheless,the absence of clear guidance from diagnosis to treatment and prognosis poses challenges for both physicians and patients.AIM To enhance understanding by investigating clinical presentation,diagnosis,treatment,complications,and prognoses in adult PIL cases.METHODS We enrolled adult patients diagnosed with PIL between March 2016 and September 2021.The primary outcome involved examining the diagnosis and treatment process of these patients.The secondary outcomes included identifying complications(infections,thromboembolism)and assessing prognoses(frequency of hospitalization and mortality)during the follow-up period.RESULTS Among the 12 included patients,peripheral edema(100%)and diarrhea(75%)were the main presenting complaints.Laboratory tests showed that all the pati-ents exhibited symptoms of hypoalbuminemia and hypogammaglobulinemia.Radiologically,the predominant findings were edema of the small intestine(67%)and ascites(58%).The typical endoscopic finding with a snowflake appearance was observed in 75%of patients.Among the 12 patients,two responded positive-ly to octreotide and sirolimus,and eight who could undergo maintenance therapy discontinued subsequently.Complications due to PIL led to infection in half of the patients,thromboembolism in three patients,and one death.CONCLUSION PIL can be diagnosed in adults across various age groups,with different severity and treatment responses among patients,leading to diverse complications and prognoses.Consequently,tailored treatments will be necessary.We anticipate that our findings will contribute to the management of PIL,an etiology of protein-losing enteropathy.

Case of protein-losing enteropathy

A 77-year-old male patient was diagnosed with protein-losing enteropathy as he had diarrhea for 4 years accompanied with repeated and exacerbated diarrhea and edema of the both lower extremities for more than 1 and a half years.In this case,warming-needle moxibustion compiled with the abdominal rubbing that patient performed by himself were utilized.Zhongwan(中脘CV12),Guanyuan(关元CV4);bilateral Tianshu(天枢ST25),Zusanli(足三里ST36),Shangjuxu(上巨虚ST37).Xiajuxu(下巨虚ST39)and the point on three cun below ST39 were taken when warming-needle moxibustion was performed.The treatment was given once a day.After 3 weeks'treatment,the frequency of diarrhea was decreased,the edema disappeared,the seralbumin level was increased,and the symptoms of the patient disappered.Follow-up for half a year,the patients had good prognosis.

Protein losing enteropathy caused by eosinophilic gastroenteritis:A case report

Protein losing enteropathy(PLE), a very rare disease with hypoproteinemia and edema as its characteristics, is caused by various diseases resulting in protein depletion from the gut. The diagnosis is relatively difficult due to its complex pathogeneses.The present paper reported a case whose symptom started with acute diarrhea and hypoproteinemia.Gastrointestinal endoscopies showed digestive ulcersandcolonpolyp.Thetreatmentscontainedalbumin infusion, Chinese herbal decoction and other symptomatic therapies. The hypoproteinemia become even worse and edema occurred after 4 days' treatment.Alargerdoseofalbumininfusion(40-60g/d) and modified herbal decoctions were prescribed. A final diagnosis of eosinophilic gastroenteritis(EG) complicated with PLE was confirmed by histopathological examination of a repeated gastroscopy. After three weeks' treatment, the serum albumin level was raised and the edema subsided gradually. In conclusion, herbs may have an effect on PLE patients, but PLE resulting from EG is very complex and easy to misdiagnose, especially in atypical conditions. Further studies are required to find the exact mechanisms.