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.

A case of Clostridium difficile infection complicated by acute respiratory distress syndrome treated with fecal microbiota transplantation

Acute respiratory distress syndrome is a life-threatening disorder caused mainly by pneumonia.Clostridium difficile infection(CDI)is a common nosocomial diarrheal disease.Disruption of normal intestinal flora by antibiotics is the main risk factor for CDI.The use of broadspectrum antibiotics for serious medical conditions can make it difficult to treat CDI complicated by acute respiratory distress syndrome.Fecal microbiota transplantation is a highly effective treatment in patients with refractory CDI.Here we report on a patient with refractory CDI and acute respiratory distress syndrome caused by pneumonia who was treated with fecal microbiota transplantation.

CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease: multi-institutional case series

AIM:To report CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease(IgG4-MALT lymphoma),a rare but clinically important complication of ocular adnexal IgG4-related disease.METHODS:We retrospectively reviewed all cases of histologically confirmed ocular adnexal IgG4-related disease at three ter tiary and one secondary referral centers,between February 2003 and December 2016.Seven cases of histopathologically diagnosed IgG4-MALT lymphoma were identified.CT and MR images were analyzed by consensus of two experienced head and neck radiologists.RESULTS:Lacrimal glands were the main site of involvement in all seven patients.The lesions typically showed well-demarcated margins,iso-to hyperattenuation on precontrast CT,T2 hypo-to isointensity,T1 isointensity,and homogenous internal architecture with homogenous enhancement pattern.Lesions were mostly hyperdense and isointense to normal extraocular muscles on postcontrast CT and MR images,respectively.CONCLUSION:Unlike in typical ocular adnexal IgG4-related disease,T2 isointensity and hyperattenuation on precontrast CT images were noted in some IgG4-MALT lymphoma cases.Although the findings may be nonspecific,the possibility of accompanying MALT lymphoma may need to be considered,when ocular adnexal lesions in patients clinically suspected of having IgG4-related disease are refractory to glucocorticoids and show T2 isointensity and hyperattenuation on precontrast CT for the optimal management of the patients.However,this is a case series of a very rare complication of ocular adnexal IgG4-related disease,and thus caution is warranted to generalize the conclusion.

Low-dose amitriptyline combined with proton pump inhibitor for functional chest pain

AIM:To investigate the efficacy of amitriptyline with proton pump inhibitor(PPI)for the treatment of functional chest pain(FCP).METHODS:This was a randomized,open-label trial investigating the addition of low dose amitriptyline(10 mg at bedtime)to a conventional dose of rabeprazole(20 mg/d)(group A,n = 20)vs a double-dose of rabeprazole(20 mg twice daily)(group B,n = 20)for patients with FCP whose symptoms were refractory to PPI.The primary efficacy endpoints were assessed by global symptom score assessment and the total number of individuals with > 50% improvement in their symptom score.RESULTS:The between-group difference in global symptom scores was statistically significant during the last week of treatment(overall mean difference;3.75 ± 0.31 vs 4.35 ± 0.29,the between-group difference;P < 0.001).Furthermore,70.6% of patients in group A had their symptoms improve by > 50%,whereas only 26.3% of patients in group B had a similar treatment response(70.6% vs 26.3%,P = 0.008).Specifically,patients in group A had a significantly greater improvement in the domains of body pain and general health perception than did patients in group B(52.37 ± 17.00 vs 41.32 ± 12.34,P = 0.031 and 47.95 ± 18.58 vs 31.84 ± 16.84,P = 0.01,respectively).CONCLUSION:Adding amitriptyline to a PPI was more effective than a double-dose of PPI in patients with FCP refractory to a conventional dose of PPI.

Median arcuate ligamentum syndrome:Four case reports

BACKGROUND Median arcuate ligamentum syndrome(MALS)is a disease entity with unclear pathogenesis.If it is not considered in advance,the clinical diagnosis of the disease is very difficult because patients complain of digestive discomfort including pain.However,this characteristic is not specific to MALS.There have been no studies to assist in making a quick diagnosis.The aim of this case series was to recognize that MALS must be considered as a differential factor in the cause of abdominal pain.CASE SUMMARY We described cases in which four patients complained of abdominal pain over a long period but in whom a diagnosis of MALS could not be made.If the gastroenterologist does not take into account abdominal pain in advance,the patient is considered an asymptomatic gallstone patient and has their gallbladder removed despite imaging evaluation.The patient may also be considered a psychiatric patient and may be administered psychiatric drugs over a long period.In all four cases in this report,the patients experienced abdominal pain.In three cases,the diagnosis was possible by the clinician’s judgment considering both clinical symptoms and imaging techniques shortly after the onset of symptoms.However,in one case that lasted over 20 years,a clear diagnosis was not possible.Even after complaining of colicky pain and performing a cholecystectomy,the diagnosis was made only after the symptoms persisted.In all four cases,the symptoms were relieved by neuromodulators.CONCLUSION MALS is a rare disease and it is easy to miss because it is not malignant,but patients can suffer from pain over a long period.For the accurate diagnosis of a patient complaining of abdominal pain,the diagnosis must be differentiated.In addition,as there are asymptomatic patients,patients who need treatment should be carefully selected,and improvement with medical treatment can be expected.Large-scale studies are also needed.

Efficient photovoltaic effect in graphene/h-BN/silicon heterostructure self-powered photodetector

Graphene(Gr)/Si-based optoelectronic devices have attracted a lot of academic attention due to the simpler fabrication processes,low costs,and higher performance of their two-dimensional(2D)/three-dimensional(3D)hybrid interfaces in Schottky junction that promotes electron-hole separation.However,due to the built-in potential of Gr/Si as a photodetector,the Iph/Idark ratio is often hindered near zero-bias at relatively low illumination intensity.This is a major drawback in self-powered photodetectors.In this study,we have demonstrated a self-powered van der Waals heterostructure photodetector in the visible range using a Gr/hexagonal boron nitride(h-BN)/Si structure and clarified that the thin h-BN insertion can engineer asymmetric carrier transport and avoid interlayer coupling at the interface.The dark current was able to be suppressed by inserting an h-BN insulator layer,while maintaining the photocurrent with minimal decrease at near zero-bias.As a result,the normalized photocurrent-to-dark ratio(NPDR)is improved more than 104 times.Also,both Iph/Idark ratio and detectivity,increase by more than 104 times at−0.03 V drain voltage.The proposed Gr/h-BN/Si heterostructure is able to contribute to the introduction of next-generation photodetectors and photovoltaic devices based on graphene or silicon.

Effect of esmolol and lidocaine on agitation in awake phase of anesthesia among children:a double-blind,randomized clinical study

Background:Sevoflurane is widely used to anesthetize children because of its rapid action with minimal irritation of the airways.However,there is a high risk of agitation after emergence from anesthesia.Strabismus surgery,in particular,can trigger agitation because patients have their eyes covered in the postoperative period.The aim of this study was to determine whether or not esmolol and lidocaine could decrease emergence agitation in children.Methods:Eighty-four patients aged 3 to 9 years undergoing strabismus surgery were randomly assigned to a control group(saline only),a group that received intravenous lidocaine 1.5 mg/kg,and a group that received intravenous esmolol 0.5 mg/kg and lidocaine 1.5 mg/kg.Agitation was measured using the objective pain score,Cole 5-point score,and Richmond Agitation Sedation Scale score at the end of surgery,on arrival in the recovery room,and 10 and 30 min after arrival.Results:The group that received the combination of esmolol and lidocaine showed lower OPS and RASS scores than the other two groups when patients awoke from anesthesia(OPS=0(0-4),RASS=-4[(-5)-1])and were transferred to the recovery room(OPS=0(0-8),RASS=-1[(-5)-3])(P<0.05).There was no significant difference in the severity of agitation among the three groups at other time points(P>0.05).Conclusions:When pediatric strabismus surgery is accompanied by sevoflurane anesthesia,an intravenous injection of esmolol and lidocaine could alleviate agitation until arrival in the recovery room.

Effects of aqueous extract from red Liriope platyphylla on phthalic-anhydride-induced atopic dermatitis in Interleukin-4/Luciferase/Consensus non-coding sequence-1 transgenic mice evaluated in terms of luciferase signal and general phenotype biomarkers

OBJECTIVE: To quantitatively evaluate the therapeutic effects of Red Liriope platyphylla(RLP) on atopic dermatitis(AD), alterations in the luciferase(Luc) signal and general phenotype biomarkers were compared in phthalic anhydride(PA) treated Interleukin-4(IL-4)/Luc/Consensus non-coding sequence-1(CNS-1) transgenic(Tg) mice following treatment with aqueous extract of RLP(AEt RLP) for4 weeks.METHODS: Alterations in AD phenotypes were measured in IL-4/Luc/CNS-1 Tg mice following treatment with AEt RLP using inflammation parameter analysis, bioluminescence imaging analysis, histological analysis, reverse transcription-polymerase chain reaction, enzyme linked immunosorbent assay and Western blot analysis.RESULTS: RLP contained high concentrations of total phenolic compounds, total flavonoid compounds and 5-HNE related to AD therapy. The Luc signal was only detected in the abdominal region and the submandibular lymph node(SL), mesenteric lymph node(ML), thymus and pancreas of the PA treated group. This signal was significantly decreased by 28%-73% throughout the body and in the four organs in PA + AEt RLP treated group. Furthermore, the lymph node weight, immunoglobulin E concentration and dermal thickness were decreased by 37%-67% in the PA + AEt RLP treated group.CONCLUSION: Our findings suggest that the therapeutic effect of AEt RLP on PA induced AD could be successfully quantified by comparison of Luc signals and AD phenotype markers in IL-4/Luc/CNS-1Tg mice, and that the Luc signal was as sensitive as the general AD phenotypes, enabling detection of effects without euthanasia.