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Background: Risk factors associated with negative outcomes in dogs with protein‐losing enteropathy (PLE) are well documented. However, mortality before hospital discharge and associated risk factors are not well described. Hypothesis/Objectives: Report the percentage of dogs with PLE that do not survive to hospital discharge and identify associated risk factors. Animals: One‐hundred and seven dogs presented to a referral hospital and diagnosed with PLE caused by inflammatory enteritis, intestinal lymphangiectasia or both. Methods: Retrospective cross‐sectional study assessing hospital records. Data on in‐hospital mortality and cause were assessed, and presenting signs, treatments prescribed, neutrophil count, lymphocyte count, serum albumin, globulin, and C‐reactive protein (CRP) concentrations, and histopathologic findings were compared between survivors and non‐survivors. Results: In‐hospital mortality was 21.5% with the most common causes including financial limitations, failure to improve and aspiration pneumonia. Factors associated with mortality during hospitalization included longer duration of hospitalization (P =.04), longer duration of clinical signs (P =.02) and an increase in serum CRP concentration after 1–3 days of in‐hospital treatment (P =.02). Higher mortality was identified in Pugs (odds ratio [OR], 4.93; 95% confidence interval [CI], 1.41–17.2; P =.01) and was a result of presumptive aspiration pneumonia in 5/6 of these dogs. Conclusions and Clinical Importance: Protein‐losing enteropathy in dogs has substantial mortality during hospitalization. Monitoring for improvement in CRP concentration after treatment during hospitalization may help predict survival to discharge. Pugs have increased in‐hospital mortality because of aspiration pneumonia; measures to prevent, recognize, and promptly treat this complication may improve outcomes in this breed. [ABSTRACT FROM AUTHOR]

A 28-year old Japanese man with Noonan syndrome (NS) presented to our emergency department with painful erythema of the trunk and lower extremities since the previous day. He had been diagnosed with protein-losing enteropathy (PLE) with intestinal lymphangiectasia at age 25 years, and undergone lymphaticovenular anastomosis (LVA) twice. Three episodes of cellulitis of both lower extremities had occurred in the past 2 years. Extensive cellulitis with sepsis was diagnosed and piperacillin/tazobactam was started, which was de-escalated to ceftriaxone. He was discharged after 13 days of antibiotic therapy. After discharge, low-dose trimethoprim-sulfamethoxazole (SMZ-TMP) was started as the primary prophylaxis, but three episodes of cellulitis occurred in the next year and were treated with other antibiotics. NS, an autosomal dominant disease known as a RASopathy, is caused by germline mutations in RAS-MAPK pathway genes. Lymphedema resulting from lymphatic abnormalities is a concomitant manifestation in 20 % of patients with NS, and can be a risk factor for cellulitis. Hypoalbuminemia and hypoglobulinemia associated with PLE facilitate infections such as cellulitis. As a treatment for lymphedema in the extremities, LVA has shown objective and subjective improvements in most patients, and some studies have also reported its efficacy for lymphedema in patients with NS. Targeted molecular therapy with mitogen-activated protein kinase enzyme (MEK) inhibitor is used in treatment of cancers with activation of the RAS/MAPK pathway. MEK inhibitors have recently been tried in patients with RASopathies and severe lymphatic disorders, and can lead to rapid resolution of symptoms. [ABSTRACT FROM AUTHOR]

Primary intestinal lymphangiectasia (PIL) is a rare disease that was discovered in 1961 by Waldmann et al., who first described the concept of intestinal lymphangiectasia. PIL is manifested by large and dilated intestinal lymphatic vessels that lead to the loss of lymphatic fluid to the intestinal lumen, including severe hypoalbuminemia and, consequently, generalized edema. The etiology of PIL is unknown; however, several genes are involved in the development of the lymphatic system. The case of a two-month-old patient with a prenatal diagnosis of ascites on third-trimester ultrasound was presented. The patient was born with rapidly progressive ascites and showed an endoscopic study with evidence of snowflake images and histology compatible with intestinal lymphangiectasia. The patient remained on octreotide infusion at baseline and subsequently with refractory ascites the dose was increased. Several evacuatory paracentesis were performed and he remained on enteral nutrition based on an extensively hydrolyzed formula with a 54% contribution of medium chain triglycerides (MCTs) and parenteral nutrition (PN). Subsequently, the patient was switched to a subcutaneous infusion of octreotide, which allowed hospital discharge and adequate outpatient follow-up with a favorable evolution. [ABSTRACT FROM AUTHOR]

Primary intestinal lymphangectasia (PIL), also known as Waldmann’s disease, is a rare disorder characterized by an exudative enteropathy resulting from morphologic abnormalities of the intestinal lymphatics. Moderate to severe oedema with pleural effusion, pericarditis, or chylous ascites is the main clinical manifestation but lymphoedema, abdominal pain, weight loss, moderate diarrhoea, vomiting, and fat-soluble vitamin deficiencies may also be present. Patients can also develop hypocalcaemia secondary to failure to absorb fat and fat-soluble vitamins. We report a nine-month-old male infant with a four-week history of diarrhoea, vomiting, failure to thrive, peripheral oedema and tetany. Hypoalbuminaemia, hypocalcaemia, low vitamin D levels and lymphopaenia were found on initial investigations. A raised stool alpha-1-antitrypsin supported a diagnosis of a protein losing enteropathy. At gastroscopy typical ‘cotton ball or frosted appearance’ was visible particularly in the second (D2) and third part (D3) of the duodenum. Biopsies from D3 revealed dilated lymphatics suggestive of primary intestinal lymphangectasia. The infant was managed with a high protein, high MCT, low fat diet with improvement in his symptoms and growth pattern. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Rare yet significant, this case sheds light on the uncommon presentation of Waldmann's disease in adults, showcasing the diagnostic challenges it poses. A multidisciplinary approach, integrating clinical, endoscopic, histological, and radiological evaluations, is crucial for accurate diagnosis and management. Further research is needed to deepen our understanding of this complex disorder. Waldmann's disease, or primary intestinal lymphangiectasia, is a rare disorder characterized by protein‐losing enteropathy due to dilation and leakage of intestinal lymphatic vessels. Although typically diagnosed in early childhood, we present a case of a 55‐year‐old male with a complex medical history. The patient's history included intestinal obstruction, multidrug‐resistant pulmonary tuberculosis, and primary antiphospholipid syndrome. He presented with a 2‐year history of chronic diarrhea, weight loss, and lower limb edema. Endoscopic and histological examination revealed scattered white spots in the duodenum and terminal ileum, indicative of intestinal lymphangiectasia. Nuclear medicine studies confirmed abnormal protein loss. The rarity of Waldmann's disease in adulthood and its association with other significant medical conditions pose diagnostic challenges. The distinct endoscopic and histological findings, coupled with scintigraphy results, contribute to a comprehensive understanding of this complex case. Differential diagnoses and management considerations are discussed. This case highlights the atypical presentation of Waldmann's disease in adulthood, emphasizing the importance of a multidisciplinary approach for accurate diagnosis and management. Further research is warranted to enhance our understanding of this uncommon disorder and its potential implications for patients with complex medical histories. [ABSTRACT FROM AUTHOR]

Introduction: Yellow nail syndrome (YNS), a very rare disorder of unknown etiology, is characterized by a triad associating yellow nails, respiratory manifestations, and lymphedema. YNS treatment remains non-codified. Method: This retrospective study was conducted from January 2008 to December 2022 in a single tertiary department exclusively dedicated to lymphatic diseases. All consecutive patients with YNS were included. Results: Thirteen men and 10 women were included. Three patients had yellow nails at birth or during childhood. For the other 20 patients, median (Q1–Q3) age at first sign was 50.8 (43–61) years, with first-YNS-sign-to-diagnosis interval of 17 (10–56) months. For 4 patients, YNS was associated with primary intestinal lymphangiectasia. The first YNS sign was chronic cough (45.5%), followed by yellow nails (27.3%), chronic sinusitis (18.2%), and lymphedema (9.1%). At first consultation for all patients, 69.6% had the complete triad, all had yellow nails and cough, 82.6% had chronic sinusitis, and 69.6% had lymphedema. Twelve patients' lymphedema involved only the lower limb(s), 2 the lower and upper limbs, and 2 the lower and upper limbs and face. Nineteen (82.6%) patients were prescribed fluconazole (100 mg/day [n = 8] or 300 mg/week [n = 11]) combined with vitamin E (1,000 mg/day) for a median of 13 months. Responses were complete for 4 (21.1%) patients, partial for 8 (42.1%), and therapeutic failures for 7 (36.8%). Conclusions: YNS is a rare disease that almost always starts with a chronic cough. Despite inconstant efficacy, fluconazole-vitamin E in combination can be prescribed to treat yellow nails. [ABSTRACT FROM AUTHOR]

Background: More than 50% of dogs with protein‐losing enteropathy (PLE) fail to respond to standard therapies. Octreotide, a somatostatin analogue, is used in cases of intestinal lymphangiectasia (IL) in humans with some success. Objectives: Describe the use of octreotide in dogs with PLE including reason for and details of prescription, adverse effects, and apparent response. Animals: Eighteen dogs with PLE, 13 with histopathology available. Ninety‐two percent (12/13) had IL diagnosed on biopsy. All 13 dogs had intestinal inflammatory infiltrates noted. Methods: Multicenter, retrospective, descriptive study. Cases were volunteered for inclusion by individual attending veterinarians who reported the use of octreotide in cases of PLE. Results: In 16/18 (89%) cases octreotide was prescribed to PLE dogs with a clinical suspicion or confirmed diagnosis of IL that were refractory to standard therapies. Median serum albumin at the time of octreotide prescription was 1.7 g/dL (range, 1.0‐3.1 g/dL). The median dose of octreotide prescribed was 20 μg/kg, SQ, daily with a range of 4‐39 μg/kg, SQ, daily. Adverse effects were noted in 3/18 (17%, 95% CI [4%, 41%]) of dogs; discontinuation of the drug was necessary in 1 dog. Improvement in clinical signs was noted in 6/12 (50%, 95% CI [21%, 79%]). Conclusions and Clinical Importance: Octreotide was most commonly prescribed to dogs with PLE and suspected or confirmed IL that had failed to respond to standard therapies. Though a benefit to PLE dogs cannot be confirmed, octreotide was well tolerated by the majority of dogs at the doses prescribed in this study. [ABSTRACT FROM AUTHOR]

Aim: to present a clinical and morphological observation of an extremely rare combination of medullary carcinoma of the jejunum and intestinal lymphangiectasia in a 33-year-old patient with clinical features of malabsorption syndrome over the 10 years.Key points. An autopsy revealed a tumor formation spreading from the wall of the jejunum to the mesentery, with metastases to the mesenteric lymph nodes. The medullary carcinoma with positive expression of СD117, DOG1, EMA, PanCK, PDL-1, vimentin, mosaic non-intense expression of CA19-9, calretinin, CD10, CDX2, CEA, MUC-5AC, SATB2, and negative reaction to ALK, CD3, CD8, CD20, CD30, CD31, CD34, CD45, CD56, chromogranin, CK7, CK20, desmin. The proliferative index was high: Ki-67 > 80 %. Moreover, during the histological examination of the intestinal wall, intestinal lymphangiectasia complicated by the malabsorption syndrome was revealed.Conclusion. The uniqueness of this clinical and morphological case is in the combination of medullary carcinoma of the jejunum metastasized to the mesenteric lymph nodes with the underlying intestinal lymphangiectasia accompanied by the development of malabsorption syndrome.

Abstract Background Intestinal lymphangiectasia (IL) is a rare protein-losing enteropathy caused by disorders of the intestinal lymphatics. There are only a few case reports and case series concerning the VCE (video capsule endoscopy) findings of IL. This work aimed to evaluate the VCE characteristics of small intestinal mucosal abnormalities in patients with IL, and to investigate the relationship between clinical and VCE characteristics. Methods Consecutive patients with IL who underwent VCE were enrolled in this retrospective study. The cases were classified into the white villi group and non-white villi group according to mucosal abnormalities detected by VCE. Clinical and endoscopic characteristics were investigated and analyzed. Results A total of 98 patients with IL with a median onset age of 26.3 ± 19.2 years were included. VCE revealed the following small intestinal lesions: (i) white villi type (57/98, 58.2%), i.e.: white-tipped or granular villi, white nodular villi or plaques; (ii) non-white villi type (41/98, 41.8%), i.e.: diffused low and round villi; (iii) complications (46/98, 46.9%), i.e.: bleeding, ulcers, protruding or vesicular-shaped lesions, stenosis and lymphatic leakage. A total of 58.2% (57) and 41.8% (41) of the cases were classified into the white villi and non-white villi groups respectively. The percentage of chylothorax in the white villi group was significantly lower than that in the non-white villi group (12/57 vs. 19/41, p = 0.008). In VCE, there were no significant differences in the involved segments and total detected rate of complications between the white villi and non-white villi groups (p > 0.05), while the detected rate of lymphatic leakage in the white villi group was significantly higher than that in the non-white villi group (31.6% vs. 12.2%, p = 0.026). Conclusions Our study evaluated the entire small intestinal mucosal abnormalities of IL by VCE, especially endoscopic complications. IL has specific VCE abnormalities in addition to classical endoscopic findings.

Introduction: Mulibrey nanism (MUL) is a rare disorder caused by TRIM37 gene variants characterized by growth failure, dysmorphic features, congestive heart failure (CHF), and an increased risk of Wilms' tumor. Although immune system impairment has been documented inMUL, the underlying mechanisms remain poorly understood. Methods: We present a case of MUL with progressive lymphopenia and review similar cases from the literature. Results: Our patient presented with prenatal onset growth restriction, characteristic dysmorphic features, and Wilms' tumor. She developed progressive lymphopenia starting at 10 years of age, leading to the initiation of intravenous immunoglobulin (IVIG) replacement therapy and infection prophylaxis. Genetic analysis detected a likely pathogenic variant on the maternal allele and copy number loss on the paternal allele in TRIM37. Subsequently a cardiac magnetic resonance imaging was conducted revealing signs of pericardial constriction raising concerns for intestinal lymphatic losses. The cessation of IVIG therapy did not coincide with any increase in the rate of infections. The patient exhibited a distinct immunological profile, characterized by hypogammaglobulinemia, impaired antibody responses, and skewed T-cell subsets with an altered CD4+/CD8+ ratio, consistent with previous reports. Normal thymocyte development assessed by artificial thymic organoid platform ruled out an early hematopoietic intrinsic defect of T-cell development. Discussion: The immunological profile of MUL patients reported so far shares similarities with that described in protein-losing enteropathy secondary to CHF in Fontan circulation and primary intestinal lymphangiectasia. These similarities include hypogammaglobulinemia, significant T-cell deficiency with decreased CD4+ and CD8+ counts, altered CD4+/CD8+ ratios, and significantly modified CD4+ and CD8 + T-cell phenotypes toward effector and terminal differentiated T cells, accompanied by a loss of naïve CD45RA+ T lymphocytes. In MUL, CHF is a cardinal feature, occurring in a significant proportion of patients and influencing prognosis. Signs of CHF or constrictive pericarditis have been evident in the case reported here and in all cases of MUL with documented immune dysfunction reported so far. These observations raise intriguing connections between these conditions. However, further investigation is warranted to in-depth define the immunological defect, providing valuable insights into the pathophysiology and treatment strategies for this condition. [ABSTRACT FROM AUTHOR]

Introduction: The lymphatic system has a pivotal role in immune homeostasis. To better understand this, we investigated the impact of Primary Lymphatic Anomalies (PLA) on lymphocyte numbers and phenotype. Methods: The study comprised (i) a retrospective cohort: 177 PLA subjects from the National Primary Lymphatic Anomaly Register with clinical and laboratory data, and (ii) a prospective cohort: 28 patients with PLA and 20 healthy controls. Patients were subdivided using established phenotypic diagnostic categories and grouped into simplex (localised tissue involvement only) and systemic (involvement of central lymphatics). Further grouping variables included genital involvement and the likelihood of co-existent intestinal lymphangiectasia. Haematology laboratory parameters were analysed in both cohorts. In the prospective cohort, prospective blood samples were analysed by flow cytometry for markers of proliferation, differentiation, activation, skin-homing, and for regulatory (CD4+Foxp3+) T cells (Treg). Results: In patients with PLA, lymphopaenia was frequent (22% of subjects), affected primarily the CD4+ T cell subset, and was more severe in subjects with systemic versus simplex patterns of disease (36% vs 9% for lymphopaenia; 70% vs 33% for CD4+ cells). B cells, NK cells and monocytes were better conserved (except in GATA2 deficiency characterised by monocytopaenia). Genital oedema and likelihood of concomitant intestinal lymphangiectasia independently predicted CD4+ T cell depletion. Analysing CD4+ and CD8+ T cells by differentiation markers revealed disproportionate depletion of naive cells, with a skewing towards a more differentiated effector profile. Systemic PLA conditions were associated with: increased expression of Ki67, indicative of recent cell division, in naive CD4+, but not CD8+ T cells; increased levels of activation in CD4+, but not CD8+ T cells; and an increased proportion of Treg. Skin-homing marker (CCR10, CLA and CCR4) expression was reduced in some patients with simplex phenotypes. Discussion: Patients with PLA who have dysfunctional lymphatics have a selective reduction in circulating lymphocytes which preferentially depletes naive CD4+ T cells. The presence of systemic disease, genital oedema, and intestinal lymphangiectasia independently predict CD4 lymphopaenia. The association of this depletion with immune activation and increased circulating Tregs suggests lymphatic-lymphocyte interactions and local inflammatory changes are pivotal in driving immunopathology. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Adequate evaluation of patients with Hennekam Syndrome (HS) is challenging for physicians, because of multi‐organ involvement and complex pathophysiology. We report the first case in an African American with lymphedema, who developed protein‐losing enteropathy (PLE) and was successfully diagnosed with HS from cause‐and‐effect complications by Waldmann's Disease (WD) and comorbid Celiac Disease (CD). As far as we know, this is the 51st case of HS worldwide and the first one in an African American. The examined patient met all diagnostic criteria for HS, suggesting a dysfunction in the development of the lymphatic system, with associated comorbidities including developmental delay, gastrointestinal pathologies, facial and hearing abnormalities, and cardiac defects. Primary intestinal lymphangiectasia (WD) is a consequence of HS, which ultimately results in PLE and worsening interstitial lymph buildup. Based on our findings, CD, a complication not yet reported in HS, may arise from WD. Other autoimmune diseases may be seen in HS: a previous report demonstrated positive anti‐thyroid stimulating hormone antibodies in HS patients. We propose that in HS, increased interstitial lymph (WD, if intestinal) with protein loss induces TNF‐α‐ and IL‐6‐mediated immune reactions in the affected visceral organs, causing autoimmune pathologies. The interstitial lymph fluid‐induced TNF‐α and IL‐6‐mediated immunopathogenic reactions lead to inflammation and subsequent destruction of the intestinal mucosa. The chronic inflammatory increase in TGF‐β causes gastric mucosa hypertrophy, which results in gastric fold thickening. Eventually, wider tight junctions develop, increasing gastric mucosa permeability, and leading to gastropathy. Considering the examined patient's history of gastroenteritis and the literature stating that CD is a non‐mucosal cause of gastropathy and PLE, it is suggested that sequelae of GI complications occur in a cause‐and‐effect chain in HS. HS results in WD, which causes CD, resulting in hypertrophic gastropathy and loss of parietal and chief cells, eventually leading to malabsorption and PLE (Figure 1). HS primarily affects various organs due to inflammatory‐mediated damage and accumulation of lymph fluid. Other findings for HS include keratoconjunctivitis sicca (dry eye disease), fibrous lymphedema exhibiting lymphorrhea, chylous ascites, anemia, and parathyroid abnormalities. Immune impairment in HS predisposes patients to autoimmune disorders, therefore autoimmunity (CD) and WD are concomitant comorbidities of HS. HS‐associated comorbidities are primarily due to inflammation and damage to immune cell transport or underlying health conditions affecting proper lymphatic function. However, it is suggested that HS mutations may disrupt the development of the lymphatic system leading to further complication. complications can be compound heterozygous, and there is a need for further research to identify nearby genes that can cause concomitant co‐morbidity. [ABSTRACT FROM AUTHOR]

A 30-year-old woman with past medical history of primary intestinal lymphangiectasia (PIL), hypothyroidism, and well-controlled asthma was evaluated in our Emergency Department with recurrent dyspnea. PLE of PIL can be confirmed by high 24-h stool 1-antitrypsin clearance and by the presence of intestinal lymphangiectasia on endoscopic biopsies, including the presence of lacteal juice and dilated lymphatic vessels. In this patient, we considered pulmonary lymphangiectasia, malignancy, large lymphatic duct injury during pregnancy, and post-partum cardiomyopathy. 4Chylothorax drainage of thick milky pleural fluid It was felt that her chylothorax likely developed secondary to either pre-existing pulmonary lymphangiectasia concurrent with her intestinal lymphangiectasia, or chylous ascites migrating through a new diaphragmatic peritoneal-pleural fistula that had formed during pregnancy. [Extracted from the article]

Primary intestinal lymphangiectasia (Waldmann's disease) is a rare exudative enteropathy without precisely assessed infectious risk. We report the case of a 49-year-old male patient with meningitis and cerebral vasculitis due to Cryptococcus neoformans complicating Waldmann's disease diagnosed 12 years ago. The treatment combined liposomal amphotericin B, 3 mg/kg daily plus flucytosine 25 mg/kg/6 h, both intravenously during 15 days, then fluconazole 800 mg daily during 8 weeks, and finally 200 mg daily indefinitely. Dexamethasone 0.4 mg/kg daily during the first week was gradually decreased over 2 months. The outcome was good, and the patient is still followed 3 years later without any recurrence. [ABSTRACT FROM AUTHOR]

Congenital chylous ascites (CCA) is a rare condition seen in the neonatal period. The pathogenesis is primarily related to congenital intestinal lymphangiectasis. Conservative treatment of chylous ascites involves paracentesis, total parenteral nutrition (TPN), medium-chain triglyceride (MCT)-based milk formula, use of somatostatin analogue and octreotide. Surgical treatment is considered when conservative treatment fails. We describe a laparoscopic treatment of CCA using the fibrin glue technique. A male infant, in whom foetal ascites was detected at 19 weeks of gestation, was born by caesarean section at 35 weeks of gestation weighing 3760 g. There was evidence of hydrops in the foetal scan. A diagnosis of chylous ascites was made by abdominal paracentesis. A magnetic resonance scan was suggestive of gross ascites, and no lymphatic malformation was identified. TPN and octreotide infusion was started and continued for 4 weeks, but the ascites persisted. The failure of conservative treatment led us to perform laparoscopic exploration. Intraoperatively, chylous ascites and multiple prominent lymphatic vessels around the root of the mesentery were noted. The fibrin glue was applied over the leaking mesenteric lymphatic vessels in the duodenopancreatic region. Oral feeding was started from post-operative day 7. After 2 weeks of the MCT formula, ascites progressed. Thus, laparoscopic exploration was necessary. We introduced an endoscopic applicator for fibrin glue and applied it into the place of leakage. The patient was doing well with no appearance of ascites reaccumulating and was discharged on the 45th post-operative day. Follow-up ultrasonography (1st, 3rd and 9th months after discharge) showed a small amount of ascitic fluid but with no clinical significance. Laparoscopic localisation and ligation of leakage sites could be difficult, especially in newborns and young infants due to the small size of lymphatic vessels. The use of fibrin glue to seal the lymphatic vessels is quite promising. [ABSTRACT FROM AUTHOR]

Background: The impact of undernutrition in dogs with protein‐losing enteropathy (PLE) caused by inflammatory enteritis, intestinal lymphangiectasia, or both and which variables are most predictive of outcome are unknown. Objectives: Develop an undernutrition screening score (USS) for use at the time of diagnosis of PLE in dogs, which is predictive of outcome. Animals: Fifty‐seven dogs with PLE prospectively recruited from 3 referral hospitals in the United Kingdom. Methods: An USS based on the presence and severity of 5 variables: appetite, weight loss, and body, muscle, and coat condition and scored out of 15, with higher scores reflecting worse undernutrition, was calculated at the time of diagnosis. Follow‐up information was obtained for at least 6 months. Results: Dogs that failed to achieve clinical remission within 6 months had higher USS at diagnosis compared with dogs that achieved remission (median, 7.5; range, 2‐14 and median, 5; range, 0‐14, respectively). The USS at diagnosis gave an area under the receiver operating characteristic curve (AUC) of 0.656 for predicting nonclinical remission within 6 months, whereas a score consisting of just epaxial muscle loss and coat condition resulted in a larger AUC of 0.728. Conclusions and Clinical Importance: Of the 5 variables assessed in the USS, a combination of epaxial muscle loss and coat condition was most predictive of not achieving clinical remission within 6 months in dogs with PLE. Additional studies will help determine the effect of changes in USS and the 5 associated variables after diagnosis on outcome variables in these dogs. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion, or chylous ascites. The goal of the treatment is to restrict the consumption of long‐chain fats and administer a formula that includes protein and medium‐chain triglycerides. Here, we report 11‐year‐old Syrian girl presented to the hospital with 2 years history of diarrhea and generalized edema. Furthermore, investigation showed ascites and pleural effusion. The disease was detected by several biopsies from duodenum that showed dilation of the lymph vessels within the lamina propria without any evidence for inflammation. She was managed by modifying her diet and albumin transfusion, which caused significant improvement in the child's condition. [ABSTRACT FROM AUTHOR]

Hennekam Lymphangiectasia–Lymphedema Syndrome 3 (HKLLS3) is a rare genetical disorder caused by mutations in a few genes including ADAMTS3. It is characterized by lymphatic dysplasia, intestinal lymphangiectasia, severe lymphedema and distinctive facial appearance. Up till now, no extensive studies have been conducted to elucidate the mechanism of the disease caused by various mutations. As a preliminary investigation of HKLLS3, we sorted out the most deleterious nonsynonymous single nucleotide polymorphisms (nsSNPs) that might affect the structure and function of ADAMTS3 protein by using a variety of in silico tools. A total of 919 nsSNPs in the ADAMTS3 gene were identified. 50 nsSNPs were predicted to be deleterious by multiple computational tools. 5 nsSNPs (G298R, C567Y, A370T, C567R and G374S) were found to be the most dangerous and can be associated with the disease as predicted by different bioinformatics tools. Modelling of the protein shows it can be divided into segments 1, 2 and 3, which are connected by short loops. Segment 3 mainly consists of loops without substantial secondary structures. With prediction tools and molecular dynamics simulation, some SNPs were found to significantly destabilize the protein structure and disrupt the secondary structures, especially in segment 2. The deleterious effects of mutations in segment 1 are possibly not from destabilization but from other factors such as the change in phosphorylation as suggested by post-translational modification (PTM) studies. This is the first-ever study of ADAMTS3 gene polymorphism, and the predicted nsSNPs in ADAMST3, some of which have not been reported yet in patients, will serve for diagnostic purposes and further therapeutic implications in Hennekam syndrome, contributing to better diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Objective To evaluate the clinical value of capsule endoscope in the diagnosis of unexplained abdominal pain. Methods We made a retrospective analysis of 191 patients with unexplained abdominal pain who sought medical help in our hospital and 25 normal controls. Capsule endoscopy was performed in both groups, small bowel lesions were detected, and clinical data were collected for further analysis. Results The total small bowel lesion detection rate was 52.87% (101/191) in abdominal pain (AP) patients and 20% (5/25) in the control group, respectively. The detection rate of significant findings (ulcers, erosions, polyps, diverticula, parasites, and neoplastic organisms) was only 16.23% (31/191) in AP patients. In the non-significant findings, no statistical difference in the detection rates for vascular malformation, capillary dilation, and lymphoid follicular hyperplasia were found between the two groups, while the detection rate of intestinal lymphangiectasia was significantly higher in the AP patients (23.56% vs. 4%, P＜0.05, OR=7.089). Conclusion Capsule endoscopy can be an optional choice for diagnosis of unexplained abdominal pain, while the relationship between positive findings and abdominal pain should be further investigated. [ABSTRACT FROM AUTHOR]

To investigate the correlation between capsule endoscopy (CE) classification of primary intestinal lymphangiectasia (PIL) and computed tomography (CT) lymphangiography (CTL). A total of 52 patients with diagnosed PIL were enrolled. All patients were examined using CTL and small intestinal CE before surgery. CE assessments included the morphology, scope, colour, and size of lesions. CTL assessments included intestinal wall, lymphatic vessel dilatation, lymph fluid reflux, and lymphatic fistula. Patients were divided into three groups according to type diagnosed by CE, and the CTL characteristics were analysed among the groups. CE showed 15 patients with type I, 27 with II, and 10 with type III. Intestinal wall thickening was observed in 15 type I, 21 type II, and seven type III. Pericardial effusion was observed in only three type I patients; the difference among types was statistically significant (p= 0.02). Abnormal contrast agent distribution in the intestinal wall and mesentery was observed in 15 type II patients, and the difference was significantly greater than that of types I and III (p= 0.02). Abnormal contrast agent distribution in the abdominal cavity was observed in 12 type II, and the difference was statistically significant (p= 0.03). The CE PIL classification reflects the extent and scope of intestinal mucosa lesions; CTL more systematically demonstrates abnormal lymphatic vessels or reflux, and its manifestations of PIL are related to the CE classification. The combination of CTL with CE is useful for accurately evaluating PIL, and provides guidance for preoperative assessment and treatment management of PIL patients. • Primary intestinal lymphangiectasia is a rare intestine lymphatic system disorder. • This disorder is often undiagnosed or misdiagnosed. • CTL could evaluate the abnormal dilatation and distribution of lymphatic vessels. • CE could reflect intestinal lymphatic dilatation. • CTL and CE have high value in diagnosis of this disease. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) has the potential to affect virtually every organ; however, gastrointestinal system manifestations are relatively rare compared to other autoimmune diseases such as systemic sclerosis and inflammatory bowel disease. A 29‐year‐old female patient attended to the emergency room with abdominal distention, acute onset abdominal pain and constipation. She had watery chronic diarrhea (4‐5 times/d) and weight loss (6 kg, 12%) for 4 months. While there was increased intestinal wall thickness, air‐liquid levels were shown on abdomen computed tomography scan. The patient underwent abdominal surgery due to diagnosis of ileus. Ileocecal resection was performed and pathologic evaluation revealed intestinal lymphangiectasia. Autoimmune serology was performed with the following resulats: anti‐nuclear antibody 1/3200 with homogenous pattern, anti‐DNA antibody and anti‐Sm/ribonucleoprotein antibodies were positive in addition to low complement levels (C3: 0.28 [0.9‐1.8 g/L], C4: 0.06 [0.1‐0.4 g/L]) indicating diagnosis of SLE. Development of intestinal involvement in SLE (lupus enteritis) is mainly grouped into 3 headings such as mesenteric vasculitis, pseudo‐obstruction, and protein‐losing enteropathy. Although the pathogenesis of intestinal lymphangiectasia remains unknown, it has been reported that immune complex‐mediated visceral vasculitis may result in bowel wall and mucosal edema. To our knowledge this is the first case report accompanying hyperinflammatory response in addition to intestinal lymphangiectasia in SLE. On the other hand, clinicians should be alert for other reasons for hyperinflammatory syndromes rather than COVID‐19, even during the pandemic. [ABSTRACT FROM AUTHOR]

Intestinal lymphangiectasia (IL) is a common complication in dogs. This study analyzed intestinal microbiota using 16S rRNA amplicon analysis as candidate factors that strongly influence the small intestinal lymphatic vessels in dogs with and without IL. Twelve dogs were included, of which six were diagnosed with lymphoplasmacytic enteritis, four with small-cell lymphoma, and two with large-cell lymphoma. Seven of these dogs had IL, whereas five did not. First, the microbial diversity analyzed by Faith pd index was significantly decreased in dogs with IL compared to dogs without IL. Then, the relative amounts of each bacterial taxa were compared between dogs with and without IL using Linear discriminant analysis effect size analysis. At the genus level, the Ruminococcus gnavus group significantly increased in dogs with IL compared to dogs without IL. A total of four genera, including Ruminococcus torques group and Faecalibacterium, which produce butyrate, significantly decreased in dogs with IL. This study showed decreased intestinal bacterial diversity and several alterations of intestinal microbiota, including a decrease in butyrate-producing bacteria in dogs with IL, compared to dogs without IL. [ABSTRACT FROM AUTHOR]

In conclusion, anti-IFC antibodies are capable of inducing an acute extravascular haemolytic transfusion reaction and IFC-compatible blood should be strongly considered for elective blood transfusion in patients with CHAPLE syndrome. CHAPLE syndrome is an extremely rare autosomal recessive condition hallmarked by a complete loss of the CD55 protein, hyperactivation of complement, angiopathic thrombosis and protein-losing enteropathy based on primary lymphangiectasia.[1] The mechanism behind this syndrome is believed to reside in the fact that CD55, also known as the decay accelerating factor, mitigates complement activation and thus modulates the immune response.[[1]] Importantly, the CD55 protein harbours the entire Cromer blood group system and patients with CHAPLE syndrome characteristically produce antibodies against these antigens when exposed to red blood cells (RBCs) from CD55 SP + sp individuals.[[3], [5]] The clinical characteristics of these antibodies have not been well documented and are thus largely unclear. It is possible that anti-IFC antibodies vary between patients in their potential of causing a haemolytic transfusion reaction. [Extracted from the article]

Abstract Background Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed. Case presentation A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL. Prominent hypomagnesemia was noticed. She was treated with a medium-chain triglyceride (MCT) diet and nutrient supplementation with satisfactory results. We also present a systematic review of hypomagnesemia in IL cases from the published literature. Conclusions Hypomagnesemia may be an overlooked complication of IL, thus monitoring serum magnesium concentrations in IL patients is crucial.

Simple Summary: Canine intestinal lymphangiectasia (IL) is a disorder characterized by dilation, obstruction, and/or dysfunction of the lymphatic vessels within the small intestine. Dogs with IL often suffer from diarrhea, weight loss, vomiting, and fluid accumulations secondary to protein loss from the intestine. This review compiles the current knowledge of the pathophysiology, diagnosis and management of disorders of the intestinal lymphatic vasculature in dogs and humans and aims to (1) improve understanding of these disorders and (2) identify areas where research is needed to improve outcomes for dogs with IL. Intestinal lymphangiectasia was first described in the dog over 50 years ago. Despite this, canine IL remains poorly understood and challenging to manage. Intestinal lymphangiectasia is characterized by variable intestinal lymphatic dilation, lymphatic obstruction, and/or lymphangitis, and is a common cause of protein-losing enteropathy in the dog. Breed predispositions are suggestive of a genetic cause, but IL can also occur as a secondary process. Similarly, both primary and secondary IL have been described in humans. Intestinal lymphangiectasia is definitively diagnosed via intestinal histopathology, but other diagnostic results can be suggestive of IL. Advanced imaging techniques are frequently utilized to aid in the diagnosis of IL in humans but have not been thoroughly investigated in the dog. Management strategies differ between humans and dogs. Dietary modification is the mainstay of therapy in humans with additional pharmacological therapies occasionally employed, and immunosuppressives are rarely used due to the lack of a recognized immune pathogenesis. In contrast, corticosteroid and immunosuppressive therapies are more commonly utilized in canine IL. This review aims toward a better understanding of canine IL with an emphasis on recent discoveries, comparative aspects, and necessary future investigations. [ABSTRACT FROM AUTHOR]

As protein-losing enteropathy (PLE) can lead to hypogammaglobulinemia and lymphopenia, and since common variable immunodeficiency (CVID) is associated with digestive complications, we wondered if (1) PLE could occur during CVID and (2) specific features could help determine whether a patient with antibody deficiency has CVID, PLE, or both. Eligible patients were thus classified in 3 groups: CVID + PLE (n = 8), CVID-only (= 19), and PLE-only (n = 13). PLE was diagnosed using fecal clearance of α1-antitrypsin or 111In-labeled albumin. Immunoglobulin (Ig) A, G, and M, naive/memory B and T cell subsets were compared between each group. CVID + PLE patients had multiple causes of PLE: duodenal villous atrophy (5/8), nodular follicular hyperplasia (4/8), inflammatory bowel disease-like (4/8), portal hypertension (4/8), giardiasis (3/8), and pernicious anemia (1/8). Compared to the CVID-only group, CVID + PLE patients had similar serum Ig levels, B cell subset counts, but lower naive T cell proportion and IgG replacement efficiency index. Compared to the CVID-only group, PLE-only patients did not develop infections but had higher serum levels of IgG (p = 0.03), IgA (p < 0.0001), and switched memory B cells (p = 0.001); and decreased naive T cells (CD4+: p = 0.005; CD8+: p < 0.0001). Compared to the PLE-only group, CVID + PLE patients had higher infection rates (p = 0.0003), and lower serum Ig (especially IgA: p < 0.001) and switched memory B cells levels. In conclusion, PLE can occur during CVID and requires higher IgG replacement therapy dosage. PLE can also mimic CVID and is associated with milder immunological abnormalities, notably mildly decreased to normal serum IgA and switched memory B cell levels. [ABSTRACT FROM AUTHOR]

We describe the first case of a previously asymptomatic infant who developed a severe vaccine-acquired rotavirus infection after receiving the first Rotarix dose, associating lymphopenia and hypoalbuminemia. The high similarity to the Rotarix vaccine strain and the failure to detect other common enteric pathogens reassure the likelihood of infection caused by the vaccine-derived strain. Keywords: attenuated vaccine; intestinal lymphangiectasis; rotavirus EN attenuated vaccine intestinal lymphangiectasis rotavirus 1 4 4 08/29/22 20220801 NES 220801 Key Message To date, severe cases of vaccine-related rotavirus infection have only been reported in infants with severe combined immunodeficiency after immunization. [Extracted from the article]

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The etiology of dilation of lymphatic vessels, termed as intestinal lymphangiectasia, remains unknown. In most cases, it occurs secondary to other pathologic conditions such as gastrointestinal neoplasms. However, only a few cases of canine intestinal lymphangiectasia concurrent with non-gastrointestinal neoplasms have been reported so far. Moreover, the correlation between intestinal lymphangiectasia and non-gastrointestinal neoplasms has not been discussed in any other literature. In this study, we report a rare case of intestinal lymphangiectasia concomitant with renal cell carcinoma in an 11 year old female mixed Maltese, suggesting that non-gastrointestinal neoplasms could be associated with the development of intestinal lymphangiectasia. On gross observation, the small intestine was irregularly swollen presenting an accordion like shape. Microscopic examination revealed prominent dilatation of the lymphatic vessels, especially, within the submucosa and muscularis layer. The lacteals within the villi were dilated and presented “club-shaped” tips. The carcinoma might trigger intestinal lymphangiectasia by compressing the main lymphatic vessels or the cisterna chyli, subsequently increasing the pressure of the lymphatic vessels in the gastrointestinal tract. Moreover, metastasis of the carcinoma to the gastrointestinal tract could induce intestinal lymphangiectasia. Thus, the occurrence of intestinal lymphangiectasia must be considered when an abdominal neoplasm is located around major lymphatic vessels.

Waldmann's disease, or primary intestinal lymphangiectasia, is an unusual cause of protein‐losing enteropathy primarily characterized by lymphopenia, hypoalbuminemia, and hypogammaglobulinemia. However, variable clinical presentations result dilemmas in diagnosis and effective management. We present a toddler diagnosed with Waldmann's disease managed with a high‐protein diet and medium‐chain triglyceride supplementation. [ABSTRACT FROM AUTHOR]

Background: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilation of lymphatic vessels and leakage of lymphatic fluids into the intestinal lumen, causing depletion of lymphocytes, protein, lipids, fat-soluble vitamins, and electrolytes. Hypomagnesemia can occur in IL patients but is seldom discussed.Case Presentation: A 30-year-old Tibetan woman who had chronic diarrhea, edema, tetany, and tingling was diagnosed with IL. Prominent hypomagnesemia was noticed. She was treated with a medium-chain triglyceride (MCT) diet and nutrient supplementation with satisfactory results. We also present a systematic review of hypomagnesemia in IL cases from the published literature.Conclusions: Hypomagnesemia may be an overlooked complication of IL, thus monitoring serum magnesium concentrations in IL patients is crucial. [ABSTRACT FROM AUTHOR]

Intestinal lymphangiectasia (IL) is often observed in dogs with chronic small intestinal diseases. Hypoplasia of the lymphatic vessel due to decreased lymphangiogenesis, which has been suggested in human idiopathic IL, may contribute to the pathogenesis of canine IL. This study aimed to evaluate the diameter and number of lymphatic vessels in fullthickness small intestinal specimens of dogs with IL. Immunohistochemical labeling of lymphatic endothelial cell markers was performed on retrospectively retrieved full-thickness small intestinal specimens. Sixteen dogs with histologically confirmed IL were included, of which 10 had lymphoplasmacytic enteritis (LPE), and six had granulomatous lymphangitis (GL). Nine dogs that died from non-gastrointestinal disorders and with little or no abnormalities in the small intestine were used as controls. Lymphatic vessel diameters in dogs with IL were significantly increased in all layers of the small intestine, including the villus lacteal, lamina propria, submucosa, muscularis, and mesentery, compared with controls (all P<0.01). There was no significant difference in the lymphatic vessel diameters between dogs with LPE and GL (all P>0.05). There was no significant difference in the number of lymphatic vessels between dogs with IL and the controls in all layers of the small intestine (all P>0.05). This study demonstrated that IL was observed in all layers of the small intestine, including the submucosa, muscularis, and mesentery, independent of the underlying disease. Factors other than reduced lymphatic vessels would contribute to the pathogenesis of IL in dogs. [ABSTRACT FROM AUTHOR]

Chylous ascites (CA), albeit rare, should be considered early as a differential diagnosis in fetal and neonatal ascites. Chylous ascites (CA) arises due to leakage of lipid-rich lymph into the peritoneal cavity and is characterised by milky, triglyceride (TG)-rich peritoneal fluid. Diagnostic abdominal paracentesis with peritoneal fluid analysis is the mainstay of diagnosing CA in neonatal ascites. [Extracted from the article]

Lymphangiomas are benign lymphatic system abnormalities that can appear anywhere on the skin and mucous membranes. Lymphangiomas are caused by congenital or acquired lymphatic system disorders. In the congenital form, although the cause is unknown it is said that it is formed by the incorrect attachment of lymphatic channels to the main lymphatic drainage duct before the age of 5 years. lymphangiectasia as a subgroup of lymphangioma occurs seldom in the small bowel, especially in adults. If that happens, protein-losing enteropathy will be the most common presenting sign. In the present study, we introduce a case of a 40-year-old man without a history of any congenital or acquired diseases who was admitted to the emergency room due to long-lasting obscure overt gastrointestinal (GI) bleeding. Normal upper and lower GI endoscopies were suggestive of GI bleeding originating from the small intestine. Despite receiving iron supplements, he continued to have melena and remained anemic. Further evaluation of the small intestine by deep enteroscopy revealed multiple white spots histologically consistent with dilated lymphatics. Intestinal lymphangiectasia was eventually introduced to be the final diagnosis of the patient. [ABSTRACT FROM AUTHOR]

Abstract Waldmann's disease, or primary intestinal lymphangiectasia, is an unusual cause of protein‐losing enteropathy primarily characterized by lymphopenia, hypoalbuminemia, and hypogammaglobulinemia. However, variable clinical presentations result dilemmas in diagnosis and effective management. We present a toddler diagnosed with Waldmann's disease managed with a high‐protein diet and medium‐chain triglyceride supplementation.

Protein-losing enteropathy (PLE) is a clinical disorder in which an excessive amount of serum protein is lost into the gastrointestinal tract, resulting in hypoproteinemia and malnutrition. PLE is associated with a wide range of gastrointestinal disorders and the rare complication of constrictive pericarditis. We report a case in which pericardiectomy achieved marked improvement of extremely severe hypoalbuminemia caused by PLE associated with tuberculosis-related constrictive pericarditis. The formation of diarrhea and edema was aggravated by PLE, resulting in hypoalbuminemia. Cardiac computed tomography showed a calcified pericardium. Echocardiography showed decreased cardiac function underlying PLE. Functional imaging with technetium-99m serum albumin identified the region of protein leakage as the intestine. After pericardiectomy, the diarrhea ceased completely. Serum albumin concentrations were increased (3.3–3.7 g/dL), which indicated resolution of the PLE.

This article, published in the journal Endoscopy, presents a case study of a 51-year-old female with bleeding intestinal lymphangiectasia in the jejunum. The patient did not have any prior chronic medical conditions, highlighting the rare association between lymphangiectasia and gastrointestinal bleeding. The article discusses the detailed clinical history, diagnostic workup, and histopathological findings, including endoscopy and laparoscopic resection. The patient's anemia persisted despite initial investigations, but video capsule endoscopy revealed a mucosal lesion with blood oozing in the proximal jejunum. Enteroscopy confirmed focal lymphangiectasia, and laparoscopic resection was performed successfully, leading to symptom resolution. The authors emphasize the importance of timely recognition and management of this rare condition for improved patient outcomes. [Extracted from the article]

Key Messages Dilated lacteals coupled with signs and symptoms of a protein-losing enteropathy should raise concern for intestinal lymphangiectasia that can arise as a primary disorder or secondary to impaired lymphatic drainage from other causes (e.g., cardiac pathology, portal hypertension, and fibrotic processes). While rare, gastrointestinal bleeding is a known manifestation of intestinal lymphangiectasia with both occult and overt bleeding reported. CT or MRI imaging of intestinal lymphangiectasia demonstrates nonspecific small-bowel wall thickening and submucosal edema, or edema and congestion of the small-bowel mesentery. Surgical and endoscopic approaches have been described, with resection of a bleeding single polypoid lymphangiectasia with endoscopic snare electrocautery [[10]], as well as a segmental proximal small-bowel resection of localized bleeding intestinal lymphangiectasia [[14]]. [Extracted from the article]

Intestinal lymphangiectasia (IL) is a common complication in dogs. Since nitric oxide (NO) is known to relax the lymphatic vessel, we evaluated inducible NO synthase (iNOS) expression using immunohistochemistry in 13 dogs with lymphoplasmacytic enteritis (LPE) with or without IL. The duodenal iNOS expressing cells were significantly increased in dogs with ILnegative or IL-positive LPE dogs (P=0.025, P=0.007) compared with control dogs. However, there was no significant difference in iNOS expression between IL-positive and IL-negative tissues. Based on these results, there is no clear evidence for the NO overproduction in the pathogenesis of IL in dogs with LPE. Factors other than NO could, thus, contribute to IL in dogs with LPE. [ABSTRACT FROM AUTHOR]

Background: Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Increasing evidence has demonstrated an association between PIL and lymphoma.Case Presentation: A 54-year-old man with a 20-year history of abdominal distension and bilateral lower limb edema was admitted. Laboratory investigations revealed lymphopenia, hypoalbuminemia, decreased triglyceride and cholesterol level. Colonoscopy showed multiple smooth pseudo polyps in the ileocecal valve and terminal ileum and histological examination showed conspicuous dilation of the lymphatic channels in the mucosa and submucosa. A diagnosis of PIL was made. Three years later colonoscopy of the patient showed an intraluminal proliferative mass in the ascending colon and biopsy examination confirmed a malignant non-Hodgkin lymphoma. Then the patient was been underwent chemotherapy, and his clinical condition is satisfactory.Conclusion: Our report supports the hypothesis that PIL is associated with lymphoma development. [ABSTRACT FROM AUTHOR]

Pancreas-sparing duodenectomy (PSD) is a known surgical technique used in patients with duodenal pathologies in the adult age group. We present a 3-year-old female patient with intestinal lymphangiectasia who underwent PSD. This is the first case in which this surgical technique was used in childhood. We believe that PSD can be used in the pediatric age group for benign pathologies. Introducing a stent to the common bile duct and the main pancreatic duct is not a requirement, especially if the ampulla is preserved as a “button” duodenal patch.

Intestinal lymphangiectasia was first described in the dog over 50 years ago. Despite this, canine IL remains poorly understood and challenging to manage. Intestinal lymphangiectasia is characterized by variable intestinal lymphatic dilation, lymphatic obstruction, and/or lymphangitis, and is a common cause of protein-losing enteropathy in the dog. Breed predispositions are suggestive of a genetic cause, but IL can also occur as a secondary process. Similarly, both primary and secondary IL have been described in humans. Intestinal lymphangiectasia is definitively diagnosed via intestinal histopathology, but other diagnostic results can be suggestive of IL. Advanced imaging techniques are frequently utilized to aid in the diagnosis of IL in humans but have not been thoroughly investigated in the dog. Management strategies differ between humans and dogs. Dietary modification is the mainstay of therapy in humans with additional pharmacological therapies occasionally employed, and immunosuppressives are rarely used due to the lack of a recognized immune pathogenesis. In contrast, corticosteroid and immunosuppressive therapies are more commonly utilized in canine IL. This review aims toward a better understanding of canine IL with an emphasis on recent discoveries, comparative aspects, and necessary future investigations.

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals that result in leakage of excessive serum proteins and lymphocytes into the gastrointestinal (GI) tract culminating in protein-losing enteropathy. The GI loss of protein and possible antithrombin III (AT-III) loss creates a prothrombotic environment. The surgical management of congenital heart disease (CHD) in presence of PIL can present with altered heparin response and can impose problems in instituting cardiopulmonary bypass (CPB). We report a case of surgical closure of ventricular septal defect with PIL with altered heparin response. Such an association of PIL with altered heparin response in CHD has not been reported in literature.

Yellow Nail Syndrome (YNS) is a rare clinical syndrome charecterised by the classical triad of thickened and dystrophic yellow nails, pleural effusion and primary lymphoedema. It usually occurs in older adults of over 50 years but there is no sex predilection. This was a case report of a 21-year-old male, who presented with dyspnea on exertion, dry cough and abdominal distension for past 15 days and bilateral lower limb swelling for past two years. On general examination, patient had pallor with bilateral non pitting oedema of lower limbs and had yellow thick and dystrophic nails. There was clinical evidence of pleural effusion and ascites. Pleural fluid analysis showed lymphocytic and exudative picture with low Lactate Dehydrogenase (LDH) and Adenosine Deaminase (ADA) levels and was negative for malignancy. Duodenum Second part (D2) biopsy showed Primary Intestinal Lymphangiectasia (PIL). Hence, a final diagnosis of yellow nail syndrome was confirmed. Symptomatic management was done following which patient had relief of symptoms and in subsequent follow-ups patient was doing better. A detailed clinical history with investigations helps in attaining the diagnosis. Although there is no definitive treatment but diagnosis helps in avoiding unnecessary treatment and symptom based management helps in improving the quality of life of the patient. [ABSTRACT FROM AUTHOR]

Background and Objectives: Primary Intestinal Lymphangiectasia (PIL) is a rare congenital and digestive disease, which could present through a broad spectrum of clinical manifestations, diagnostic and treatment management. The aim of this study was to introduce the diagnosis and nutrition treatment of children with PIL through the twelve years of experience. Methods and Study Design: The patients diagnosed with PIL admitted to the Department of Gastroenterology and Nutrition in Xinhua Hospital from June 2006 to September 2017 were included in the study. Results: Ten patients were found to have PIL, and 5 of them were male. The mean age was 66 months at the time of diagnosis and 11 months at onset. The main clinical manifestations were diarrhea, edemas and abdominal distention. Marked dilatation of the intestinal lymphatic vessels was the characteristic of the endoscopic. All the patients presented with hypoproteinemia and hypoimmunoglobulinia. Six of them were treated with parenteral nutrition, and 9 of them were treated with a low-long-chain triglycerides (LCT), high-protein diet supplemented with medium-chain triglycerides (MCT). The clinical symptoms of the patients have improved after the MCT diet therapy. Conclusions: PIL should be considered first when there are clinical manifestations of chronic diarrhea, edema and abdominal distention, and biochemical results indicated the hypoproteinemia and hypoimmunoglobulinia, and the general treatment is invalid. Gastroscopy and E-colonoscopy with biopsies are the preferred method of diagnosis. Diet intervention (MCT diet) is the cornerstone and longtime medical treatment, which can improve the nutritional status and promote the survival quality of patients with PIL. [ABSTRACT FROM AUTHOR]