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.

Intestinal lymphangiectasia:Understanding the bigger picture

Intestinal lymphangiectasia(IL)is characterized by the dilation of intestinal lymphatic vessels,which can rupture and cause loss of lymph into the intestine.Due to the high content of proteins,lipoproteins,and lymphocytes in the intestinal lymph,loss of lymph might result in hypoproteinemia,hypoalbuminemia,hypogammaglobulinemia,and lymphocytopenia.In addition,there may be a depletion of minerals,lipids,and fat-soluble vitamins.IL can be primary due to inherent malfunctioning of the lymphatic system,or secondly,a result of various factors that may hinder lymphatic drainage either directly or indirectly.This condition has emerged as a subject of significant clinical interest.Given that the intestinal lymphatic system plays an important role in the body’s fluid homeostasis,adaptive immunity,nutrient and drug absorption,intestinal transport,and systemic metabolism,its dysfunction may have wider implications.Although primary IL is rare,with varied clinical features,complications,treatment response,and outcomes,secondary IL is more common than previously believed.The definitive diagnosis of IL requires endoscopic demonstration of whitish villi(which frequently resemble snowflakes)and histological confirmation of dilated lacteals in the small intestinal mucosa.Treatment of IL is challenging and involves dietary modifications,managing underlying medical conditions,and using medications such as sirolimus and octreotide.Recognizing its prevalence and diverse etiology is crucial for targeted management of this challenging medical condition.This article provides a comprehensive exploration of the clinical implications associated with IL.In addition,it offers valuable insights into critical knowledge gaps in the existing diagnostic and management landscape.

A novel technique to identify and remove the conjunctival lymphangiectasia using indocyanine green gel

In this case report it describes a novel technique using indocyanine green gel for the surgical excision of conjunctival lymphangiectasia(CL).CL was found on the nasal side of left eye in a 50-year-old male.Surgical excision of the entire cystic lesion with an intact cyst wall was completed with the assistance of an indocyanine green(ICG)gel.No sutures were used throughout the entire procedure.Subsequent post operative follow-up was uneventful.Herein,we report a novel technique used to facilitate the identification and complete removal of an intraocular cystic lesion ensuring an intact cyst wall.

Preliminary study on the efficacy of lacrimal duct reconstruction with pedicled conjunctival flap in the treatment of severe lacrimal canalicular obstruction with conjunctivochalasis

AIM:To determine the efficacy and safety of pedicled conjunctival lacrimal duct reconstruction in the treatment of severe obstruction of superior and inferior lacrimal canaliculi with conjunctivochalasis.METHODS:This study was per formed as a retrospective analysis of patients who received conjunctival dacryocystorhinostomy with pedicled conjunctival flap reconstruction combined with tube intubation due to severe superior and inferior lacrimal canalicular obstruction with conjunctivochalasis from January 2019 to October 2019.The clinical data included the degree of preoperative epiphora and postoperative relief,preoperative examination of lacrimal duct computed tomography and ultrasound biomicroscopy,postoperative evaluation of lacrimal duct function by chloramphenicol taste and fluorescein dye disappearance test,etc.Syringing was carried out to determine the reconstruction and patency of the lacrimal duct.RESULTS:All 9 patients(9 eyes)had severe canalicular obstruction with conjunctivochalasis.The patients included 4 males and 5 females aged between 47–65y with an average age of 52.2±6.7y.At 3mo follow-up,the tube was removed and the patients were followed for a further 3mo.After tube removal,6 patients showed no epiphora.These patients also had positive chloramphenicol tastes and normal fluorescein dye disappearance test results.Two patientshad epiphora.Also,syringing showed partial patency of the reconstructed lacrimal duct.One patient had no improvement in epiphora with negative chloramphenicol taste and fluorescein dye disappearance test results and obstruction of the reconstructed lacrimal duct.The total effective rate of the operation was 8/9,with no serious complications.CONCLUSION:Pedicled conjunctival lacrimal duct reconstruction conjunctival dacryocystorhinostomy is safe and effective for superior and inferior canalicular obstruction with conjunctivochalasis.

ADAMTS3 and FLT4 gene mutations result in congenital lymphangiectasia in newborns:A case report

BACKGROUND Congenital lymphangiectasia is a rare disease characterized by dilated interstitial lymphatic vessels and cystic expansion of the lymphatic vessels.Congenital lymphangiectasia can affect various organ systems;however,it frequently occurs in the lungs accompanied with unexplained pleural effusion.Further,it might not be diagnosed during prenatal examination owing to the absence of pronounced abnormalities.However,after birth the newborn rapidly develops respiratory distress that quickly deteriorates.Genetic variations in proteins controlling the development of lymphatic vessels contribute to the pathophysiology of this disease.We report a rare case of heterozygous mutation of ADAMTS3 and FLT4 genes,which have not been reported previously.CASE SUMMARY We analysed the case of a neonate who had presented with only pleural effusion at a late gestational age and eventually died due to its inability to establish spontaneous breathing after birth.An autopsy revealed lymphangiectasia of the organ systems.Further,whole exome sequencing revealed heterozygous mutations of the lymphangiogenesis-controlling genes,ADAMTS3 and FLT4,and Sanger verification revealed similar lesions in the mother with no symptoms.CONCLUSION Considering the presented case,obstetricians should observe unexplained foetal pleural effusion,and perform pathology analysis and whole exome sequencing for a conclusive diagnosis and prompt treatment.

Effectiveness of conjunctival bleb scarring by knockdown of heat shock protein 47 in rat model

AIM:To evaluate the effectiveness of knock-down of heat shock protein 47(HSP47)on conjunctival bleb scarring in a rat model and its possible mechanism.METHODS:Male Sprague–Dawley rats were used for glaucoma filtration surgery(GFS)and were treated with either phosphate buffered solution,shControl,mitomycin C,or sh-HSP47 using a microsyringe immediately after GFS.The morphology of filtering blebs was observed postoperatively.The levels of HSP47 were analyzed at 2,5,8,and 11d after GFS via real‑time quantitative polymerase chain reaction(PCR)and Western blot.The silencing effect of HSP47,the expression of collagen I and III,and the potential signaling pathways of HSP47 during scarification were explored 11d post GFS.The protein levels of transforming growth factor-β1(TGF-β1),phospho-Smad2(pSmad2),phospho-Smad3(p-Smad3),and phospho-p38(p-p38)were also analyzed using Western blot.RESULTS:Sh-HSP47 treatment significantly prolonged the functional filtration bleb retention.The levels of HSP47 were increased significantly at 5,8,and 11d postoperatively compared to the control group(P<0.05,P<0.01,and P<0.001).The levels of HSP47 protein at day 11 postoperatively were significantly down-regulated after HSP47 silencing using sh-HSP47 adenovirus transfection(P<0.01).Expression levels of collagen I and III within the blebs were significantly reduced in the absence of HSP47(P<0.01).Moreover,the protein levels of TGF-β1,p-Smad2/3,and p-p38 were dramatically inhibited after treatment with sh-HSP47(P<0.01).CONCLUSION:The inhibitory effects of HSP47 knockdown on scarring after GFS have the potential to be an efficacious therapeutic option for the treatment of conjunctival bleb scarring.

Intestinal lymphangiectasia in adults

Intestinal lymphangiectasia in the adult may be characterized as a disorder with dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine and resultant hypoproteinemia, hypogamma-globulinemia, hypoalbuminemia and reduced number of circulating lymphocytes or lymphopenia. Most often, intestinal lymphangiectasia has been recorded in children, often in neonates, usually with other congenital abnormalities but initial def inition in adults including the elderly has become increasingly more common. Shared clinical features with the pediatric population such as bilateral lower limb edema, sometimes with lymphedema, pleural effusion and chylous ascites may occur but these reflect the severe end of the clinical spectrum. In some, diarrhea occurs with steatorrhea along with increased fecal loss of protein, reflected in increased fe- cal alpha-1-antitrypsin levels, while others may present with iron def iciency anemia, sometimes associated with occult small intestinal bleeding. Most lymphangiectasia in adults detected in recent years, however, appears to have few or no clinical features of malabsorption. Diagnosis remains dependent on endoscopic changes confirmed by small bowel biopsy showing histological evidence of intestinal lymphangiectasia. In some, video capsule endoscopy and enteroscopy have revealed moreextensive changes along the length of the small intes- tine. A critical diagnostic element in adults with lym- phangiectasia is the exclusion of entities (e.g. malignancies including lymphoma) that might lead to obstruction of the lymphatic system and "secondary" changes in the small bowel biopsy. In addition, occult infectious (e.g. Whipple's disease from Tropheryma whipplei) or in? am- matory disorders (e.g. Crohn's disease) may also present with profound changes in intestinal permeability and protein-losing enteropathy that also require exclusion. Conversely, rare B-cell type lymphomas have also been described even decades following initial diagnosis of intestinal lymphangiectasia. Treatment has been historically def ined to include a low fat diet with medium-chain triglyceride supplementation that leads to portal venous rather than lacteal uptake. A number of other pharma- cological measures have been reported or proposed but these are largely anecdotal. Finally, rare reports of localized surgical resection of involved areas of small intestine have been described but follow-up in these cases is often limited. 2011 Baishideng. All rights reserved.

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Primary intestinal lymphangiectasia(PIL)is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing enteropathy.Patients with PIL develop hypoalbuminemia,hypocalcemia,lymphopenia and hypogammaglobulinemia,and present with bilateral lower limb edema,fatigue,abdominal pain and diarrhea.Endoscopy reveals diffusely elongated,circumferential and polypoid mucosae covered with whitish enlarged villi,all of which indicate intestinal lymphangiectasia.Diagnosis is conf irmed by characteristic tissue pathology,which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged villi.The prevalence of PIL has increased since the introduction of capsule endoscopy.The etiology and prevalence of PIL remain unknown.Some studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to PIL.We report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome 4q25.The relationship between this deletion on chromosome 4 and PIL remains to be investigated.

Primary intestinal lymphangiectasia: Minireview

Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.

Ileal polypoid lymphangiectasia bleeding diagnosed and treated by double balloon enteroscopy

Intestinal lymphangiectasia is a rare disease characterized by focal or diffuse dilated enteric lymphatics with impaired lymph drainage.It causes protein-losing enteropathy and may lead to gastrointestinal bleeding.Commonly,lymphangiectasia presents as whitish spots or specks.To our knowledge,small bowel bleeding resulting from polypoid intestinal lymphangiectasia has not been reported.Here,we report a rare case of active bleeding from the small bowel caused by polypoid lymphangiectasia with a review of the relevant literature.An 80-year-old woman was hospitalized for melena.Esophagogastroduodenoscopy could not identify the source of bleeding.Subsequent colonoscopy showed fresh bloody material gushing from the small bowel.An abdominal-pelvic contrast-enhanced computed tomography scan did not reveal any abnormal findings.Video capsule endoscopy showed evidence of active and recent bleeding in the ileum.To localize the bleeding site,we performed double balloon enteroscopy by the anal approach.A small,bleeding,polypoid lesion was found in the distal ileum and was successfully removed using endoscopic snare electrocautery.

Primary intestinal lymphangiectasia in an adult patient:A case report and review of literature

BACKGROUND Primary intestinal lymphangiectasia(PIL),first described in 1961,is a rare disorder of unknown etiology resulting in protein-losing enteropathy.The disease is characterized by dilatation and leakage of intestinal lymph vessels leading to hypoalbuminemia,hypogammaglobulinemia,and lymphopenia.Since the severity and location of lymph vessels being affected can vary considerably,the range of associated symptoms is wide from mild lower-limb edema to generalized edema,abdominal and/or pleural effusion,and recurrent diarrhea,among others.Although usually developing in early childhood,we present the case of a 34-yearold woman with PIL.Moreover,we performed a literature review systematically assessing clinical presentation,and provide a practical approach to facilitate diagnosis and therapy of PIL in adults.CASE SUMMARY Our patient presented with unspecific symptoms of abdominal discomfort,fatigue,nausea,and recurrent edema of the lower limbs.Interestingly,a striking collinearity of clinical symptoms with female hormone status was evident.Additionally,polyglobulia,hypoalbuminemia,hypogammaglobulinemia,and transient lymphocytopenia were evident.Due to suspicion of a bone marrow disease,an extensive diagnostic investigation was carried out excluding secondary causes of polyglobulinemia and hypoalbuminemia.The diagnosis of primary intestinal lymphangiectasia was established after 22 wk by histological analysis of biopsy samples obtained via enteroscopy.Consecutively,the patient was put on a high-protein and low-fat diet with medium-chain triglycerides supplementation leading to significant improvement of clinical symptoms until 2 years of follow-up.CONCLUSION PIL can be the reason for cryptogenic hypoalbuminemia,hypogammaglobulinemia,and lymphopenia in adulthood.Due to difficulty in correct diagnosis,treatment initiation is often delayed despite being effective and welltolerated.This leads to a significant disease burden in affected patients.PIL is increasingly been recognized in adults since the majority of case reports were published within the last 10 years,pointing towards an underestimation of the true prevalence.The association with female hormone status warrants further investigation.

Efficacy of autologous conjunctival flap on repairing the late-onset filtering bleb leakage

AIM： To evaluate the effectiveness of autologous conjunctival flap surgery for repairing the late-onset filtering bleb leakage after trabeculectomy.METHODS： This study retrospectively reviewed 106 eyes from 106 patients with late-onset filtering bleb leakage after trabeculectomy who received autologous conjunctival flap surgery at the Zhongshan Ophthalmic Centre from 2005 to 2015. The basic information was recorded, and the interval time between trabeculectomy and autologous conjunctival flap surgery as well as related risk factors, intraocular pressure（IOP）, anterior chamber depth（ACD） and best corrected visual acuity（BCVA） were analysed. Moreover, 41 patients who completed the 1-year follow up were analysed to determine the IOP and BCVA changes and long-term success rates.RESULTS： The 50 male and 56 female subjects（average age 39.13±17.96 y） included 47（44.34%） and 33（31.13%） cases of primary open angle and primary angle-closure glaucoma. The mean interval between trabeculectomy and repair surgery was 60.60±56.92（3-264）mo. The mean mitomycin（MMC） concentration during trabeculectomy was 0.27±0.04（0.12-0.4） mg/m L in the fornix-based conjunctival flap group（68 patients） and 0.28±0.04（0.20-0.33） mg/m L in the limbal-based conjunctival flap group（11 patients）. After bleb leakage, the patients＇ vision remained stable while the IOP decreased from 10.25±4.76（3-20.86） to 9.44±4.33（2-21） mm Hg（P〈0.01）. In the 41 analysed patients, the IOP was controlled at 15.68±5.11（7-40） mm Hg in the 1^（st） year after autologous conjunctival flap surgery and recurrence was not observed, for a long-term success rate of 100%. CONCLUSION： Autologous conjunctival flap repairing surgery is an effective technique for sealing filtering bleb leakages and controlling IOP postoperatively.

Tuberous sclerosis complex presenting as primary intestinal lymphangiectasia: A case report

BACKGROUND Primary intestinal lymphangiectasia(PIL)is a rare congenital protein-losing enteropathy caused by dysplasia of the small intestinal lymphatics.The cause of the disease is unknown.Through a literature review,we found that PIL and tuberous sclerosis complex(TSC)have some common symptoms and molecular pathways.CASE SUMMARY Here,we present the case of a patient with a three-year history of primary intestinal lymphangiectasia.The patient most recently visited the hospital with abdominal distension and swelling of the left leg.His mother told us that she was diagnosed with TSC one year previously,which alerted us because the patient had multiple regions of pigmentation.To evaluate the condition of the child and make a definite diagnosis,multiple imaging examinations were performed,as was TSC gene analysis.The results met the diagnostic criteria for TSC.The patient was discharged after symptomatic treatment.Through a review of the literature,it can be seen that changes at the molecular gene level of TSC can lead to abnormal lymphatic vessels.CONCLUSION In summary,when patients with hypomelanotic macules or enamel hypoplasia are diagnosed with PIL,TSC gene screening may be important for further diagnosis.

Sulforaphane modulates TGFβ2-induced conjunctival fibroblasts activation and fibrosis by inhibiting PI3K/Akt signaling

AIM:To examine the effects of sulforaphane on fibrotic changes of transforming growth factor(TGFβ2)induced human conjunctival fibroblast(HCon Fs).METHODS:HCon Fs were cultured and divided into control,TGFβ2(1 ng/m L),sulforaphane and TGFβ2+sulforaphane groups.Cell viability and apoptosis were detected using the MTT and Apo Tox-Glo Triplex assay.Cell migration was detected using scratch and Transwell assay.Real-time quantitative PCR method was used to evaluate m RNA expression of TGFβ2,matrix metalloproteinase-2(MMP2),myosin light chain kinase(MYLK),integrinαV,integrinα5,fibronectin 1 andα-smooth muscle actin(α-SMA).The protein expression ofα-SMA,p-PI3 K,PI3 K,p-Akt,and Akt were detected by Western blot.RESULTS:The proliferation of HCon Fs was significantly(P<0.05)suppressed by sulforaphane compared to control cells with the increase of the concentration and treatment time.Cell proliferation after 48 h incubation was significantly reduced with 100μmol/L sulforaphane treatment by 17.53%(P<0.05).The Transwell assay showed sulforaphane decreased cell migration by 18.73%compared with TGFβ2-induced HCon F(P<0.05).TGFβ2-induced the increasing expression of fibronectin,type I collagen andα-SMA,and the phosphorylation of PI3 K and Akt were all significantly suppressed by sulforaphane pretreatment.CONCLUSION:Sulforaphane inhibits proliferation,migration,and synthesis of the extracellular matrix in HCon Fs,and inhibiting the PI3 K/Akt signaling pathway.Sulforaphane could be a potential therapeutic drug for prevention of scar formation in filtering bleb after trabeculectomy.

Ocular manifestations and SARS-CoV-2 detection in tears and conjunctival scrape from non-severe COVID-19 patients

AIM:To explore the ocular features of corona virus disease(COVID)-19 and severe acute respiratory syndrome coronavirus(SARS-CoV)-2 detection in tears and conjunctival scrapes in non-severe COVID-19 patients.METHODS:This is a multicenter observational clinical study with no intervention conducted from Jan 25th to March 1st,2020.Clinical data and samples of tears and conjunctival scraping were collected in consecutive laboratory-confirmed,non-severe COVID-19 patients from three hospitals.COVID-19 virus was analyzed by real-time reverse transcriptase polymerase chain reaction(RT-PCR)kits.RESULTS:Totally 255 laboratory-confirmed,non-severe COVID-19 patients were recruited for ocular manifestation investigation.Of them,54.9%were females,with a mean age of 49.4 y.None of the patients has evidence of uveitis;11 patients(4.3%)complained of mild asthenopia;2(0.8%)had mild conjunctival congestion and serous secretion.Twenty-five of them had performed tears and conjunctival scrape for COVID-19 virus detection,with 4 yield possible positive results in the nucleoprotein gene.One of them were asymptomatic with normal chest CT and positive pharyngeal swab result.CONCLUSION:Ocular manifestations are neither common nor specific in non-severe COVID-19 patients.Meanwhile,COVID-19 virus nucleotides can be detected in the tears and conjunctival scrape samples,warranting further research on the transmissibility by the ocular route.

Ocular leech infestation initially misdiagnosed as conjunctival pigmented nevus

Dear sir,Iam Dr.Wen-Wei Li,from the Department of Ophthalmology,Zhejiang Provincial People’s Hospital,Hangzhou,China.I write to present a case of ocular leech infestation initially misdiagnosed as conjunctival pigmented nevus.Although human interaction with leeches is not uncommon

Bulbar conjunctival vascular lesion combined with spontaneous retrobulbar hematoma:A case report

BACKGROUND Orbital hemorrhage can be classified as traumatic or spontaneous depending on its cause.Spontaneous orbital hemorrhage refers to an internal orbital hemorrhage without apparent cause.Therefore,we aimed to describe a case of an orbital hematoma after a severe cough the night before due to inhalation of cooking oil fumes.CASE SUMMARY A 46-year-old woman was referred to our hospital with a complaint of exophthalmos accompanied with blurred vision,pain,binocular diplopia,and dizziness lasting for 5 h noted on waking in the morning.She also experienced nausea and vomiting due to high pressure of orbit and dizziness.Based on the auxiliary examination and her medical history,the patient was finally diagnosed with bulbar conjunctival vascular lesion combined with spontaneous retrobulbar hematoma.The patient was administered tobramycin and dexamethasone eye ointment,and applied pressure dressing on the left eye to stop the bleeding.Simultaneously,we administered intravenous etamsylate,oral Yunnan Baiyao capsule,intravenous mannitol to reduce orbital pressure,and intravenous dexamethasone injection at 10 mg/dL combined with neurotrophic therapy to reduce tissue edema.Among them,the Yunnan Baiyao capsule is a traditional Chinese herbal medicine to remove stasis and stop bleeding;thus,it promotes blood circulation and relieves pain resulting in reduced edema of the lesion site.The symptoms did not improve significantly during the first 2 d of treatment.We speculate that high orbital pressure and binocular diplopia induced frequent nausea and vomiting in the patient,causing increased pressure on the superior vena cava and leading to repeated orbital bleeding.After the second day,the symptoms started gradually improving.CONCLUSION This case further emphasizes the importance of comprehensive,detailed medical history and careful ophthalmic examination of the patient.

Chronic ocular GVHD:limbal and conjunctival stem cell allografts from the same hematopoietic stem cell donor

Dear Editor,I am Dr.Elias F Jarade from the Beirut Eye and ENT Specialist Hospital, Beirut, Lebanon. I write to describe a novel surgical technique in the management of chronic ocular graft-versus-host disease（GVHD）.

Primary intestinal lymphangiectasia with generalized warts

Primary intestinal lymphangiectasia(PIL) is a rare protein-losing enteropathy with lymphatic leakage into the small intestine. Dilated lymphatics in the small intestinal wall and mesentery are observed in this disease. Laboratory tests of PIL patients revealed hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia and increased stool α-1 antitrypsin clearance. Cell-mediated immunodeficiency is also present in PIL patients because of loss of lymphocytes. As a result, the patients are vulnerable to chronic viral infection and lymphoma. However, cases of PIL with chronic viral infection, such as human papilloma virus-induced warts, are rarely reported. We report a rare case of PIL with generalized warts in a 36-year-old male patient. PIL was diagnosed by capsule endoscopy and colonoscopic biopsy with histological tissue confirmation. Generalized warts were observed on the head, chest, abdomen, back, anus, and upper and lower extremities, including the hands and feet of the patient.

Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic

The relationship between primary intestinal lymphangiectasia(PIL) and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder.A recent paper reported that a six-month lowfat diet improved liver fibrosis.We report the case of a 17-year-old girl affected by PIL whose hepatic fibrosis progressively worsened within one year,despite dietetic support.This and the previous case report describe extraordinary events,which do not allow clear-cut clinical aspects to be established.Nevertheless,both cases suggest that in patients with PIL,it is necessary to closely monitor liver morphology with in-depth investigations including not only ultrasonography,but also elastography.