Seizure caused by Hypocalcemia as a Rare Manifestation in an Infant with Eosinophilic Gastroenteritis.

Eosinophilic gastroenteritis (EGE) can occur throughout the gastrointestinal tract, from the stomach to the colon. Typical known symptoms are abdominal pain, nausea, vomiting, and diarrhea. In addition, lesions in the intestinal mucosa may cause weight loss, protein-losing enteropathy (PLE), and other problems. A 6-month-old girl with no previous medical history was brought to our hospital after an afebrile 1-min clonic seizure. Blood tests showed low concentrations of serum calcium and albumin. After the correction of hypocalcemia with gluconic acid, there was no recurrence of seizure. Technetium-99m scintigraphy showed slight leakage of protein from the intestinal tract, which led us to conclude that the hypocalcemia and hypoalbuminemia were caused by PLE. Gastrointestinal endoscopy and biopsy performed to detect the cause of PLE revealed the presence of EGE. After starting administration of an amino acid-based formula, gastrointestinal symptoms of diarrhea or vomiting did not reappear. The serum albumin concentration normalized, and her weight gain improved. We report the first case of EGE in an infant who was diagnosed based on seizure. This case shows that infants with EGE may present with seizure resulting from hypocalcemia caused by PLE.

Protein-Losing Enteropathy and Plastic Bronchitis Following the Total Cavopulmonary Connections.

BACKGROUND: We aimed to evaluate incidence, outcomes, and predictors of protein-losing enteropathy (PLE) and plastic bronchitis (PB) in a cohort of total cavopulmonary connection (TCPC). METHODS: We included 620 consecutive patients undergoing TCPC between 1994 and 2021. Prevalence and predictors for onset of PLE/PB were evaluated. Death and heart transplantation after onset of PLE/PB were examined. RESULTS: A total of 41 patients presented with PLE/PB (31 with PLE, 15 with PB, and 5 developed both PLE and PB). Their median age at TCPC was 2.2 (interquartile ranges [IQRs], 1.7-3.7) years, and time period to onset for PLE was 2.6 (IQR: 1.0-6.6) years and for PB was 1.1 (IQR: 0.3-4.1) years after TCPC. Independent factors for developing PLE/PB were dominant right ventricle (RV, hazard ratio [HR], 2.243; 95% confidence interval [CI], 1.129-4.458, P = .021) and prolonged pleural effusion after TCPC (HR, 2.101; 95% CI, 1.090-4.049, P = .027). In PLE/PB population, freedom from death or transplantation after PLE/PB diagnosis at 5 and 10 years were 88.7% and 76.4%, respectively. Eleven surgical interventions were performed in 10 patients, comprising atrioventricular valve repairs (n = 4), Fontan pathway revisions (n = 2), pacemaker implantation (n = 2), secondary fenestration (n = 1), diaphragm plication (n = 1), and ventricular assist device implantation (n = 1). In nine patients, a recovery from PLE with the resolution of PLE symptoms and normal protein levels was achieved. Eight patients died and the remaining continued to have challenging protein loss. CONCLUSIONS: Protein-losing enteropathy and PB remain severe complications in the cohort of TCPC. Patients with dominant RV, and prolonged pleural effusions, were at risk for PLE/PB.

Lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs' syndrome and successfully treated with hydroxychloroquine.

We herein report the first case of lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs' syndrome. Lupus-related protein-losing enteropathy and pseudo-pseudo Meigs' syndrome are extremely rare complications in patients with systemic lupus erythematosus, Both have a similar clinical course characterized by producing marked ascites, and respond to steroids in typical cases. However, in our case, steroid monotherapy was inadequate and the addition of hydroxychloroquine was effective for their treatment. Furthermore, no reports have previously confirmed elevated CA 125 levels with lupus-related protein-losing enteropathy or increased 99mTc-HSA activity with pseudo-pseudo Meigs' syndrome. In addition, we are the first to report an evaluation of the histopathology of lupus-related protein-losing enteropathy. Previously reported cases have been described as being caused by either pseudo-Meigs's syndrome or lupus-related protein-losing enteropathy as the cause of the rare pathology that causes marked pleural effusion and ascites in patients with systemic lupus erythematosus, but it has not been evaluated whether the other is co-occurring. Our case highlights that there is a potential case of overlapping lupus-related protein-losing enteropathy and pseudo-Pseudo-Meigs's syndrome. Furthermore, it is possible that patients with marked ascites with elevated CA 125 levels were mistakenly diagnosed with Meigs's syndrome or pseudo-Meigs's syndrome associated with malignant or benign ovarian tumors and underwent surgery. Clinicians should not forget SLE with pseudo-Pseudo-Meigs's syndrome as one of the differential diagnoses for marked ascites with elevated CA 125 levels.

The Lymphatic System in the Fontan Patient-Pathophysiology, Imaging, and Interventions: What the Anesthesiologist Should Know.

The Fontan surgery was developed as a palliative intervention for congenital heart disease (CHD) patients with single-ventricle physiology who are not candidates for a biventricular repair. Improvements in the surgery and medical management of these patients have increased survival, yet this population remains at risk for complications and end-organ dysfunction due to Fontan failure. Lymphatic vessels maintain a fluid balance within the extracellular space, participate in fat reabsorption from the small intestine, and play an important role in the body's immune response. Altered Starling forces at the capillary level, capillary leak, and lymphatic obstruction contribute to lymphatic dysfunction in patients with Fontan physiology. These lymphatic complications include edema, pleural effusions, plastic bronchitis (PB), and protein-losing enteropathy (PLE). Over the past decade, there have been innovations in lymphatic imaging. These new imaging techniques include noncontrast magnetic resonance (MR) lymphangiography, intranodal lymphangiography (IL), dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), and liver lymphangiography. These imaging techniques help in delineating anatomy and guiding the appropriate therapeutic approach. Lymphatic interventions then may be performed to decompress the lymphatic system or to identify and occlude abnormal lymphatic vessels and drainage pathways. The anesthesiologist should have an understanding of the effects of lymphatic disorders on the Fontan circulation and apply appropriate management techniques for the associated interventions. The Fontan surgery was developed as a palliative intervention for CHD patients with single-ventricle physiology who are not candidates for a biventricular repair. The surgery creates a series systemic and pulmonary circulation with the energy necessary to provide gradient-driven pulmonary blood flow generated by the ventricle.1 In the past decades, improvements in the surgery and medical management of these patients have increased survival, with 30-year survival rates close to 85%.2 Despite these improvements, this population remains at risk for complications and end-organ dysfunction due to Fontan failure, which is characterized by elevated systemic venous pressures and low cardiac output. These complications include arrhythmias, cardiac dysfunction, ascites, liver fibrosis/cirrhosis, renal dysfunction, pulmonary failure, and lymphatic complications such as edema, pleural effusions, PB, and PLE. Complications ultimately contribute to increased risk for hospitalization, death, and need for heart transplantation.3,4 For this reason, there has been increasing interest in the role of abnormal lymphatic circulation in the genesis of Fontan failure. The authors characterize the lymphatic pathophysiology associated with Fontan physiology and review the imaging and interventional strategies used to treat these patients.

Protein-losing Enteropathy Complicated with Primary Intestinal Follicular Lymphoma.

Protein-losing enteropathy (PLE) is a rare syndrome characterized by hypoproteinemia due to gastrointestinal (GI) protein loss. Primary intestinal follicular lymphoma (PIFL), a specific variant of follicular lymphoma with essential only GI involvement, has not been reported as an etiology of PLE. We herein report a case of PLE complicated with PIFL that was successfully treated with rituximab, resulting in rapid improvement of PLE and a complete response of PIFL. Macroscopic findings of ulcerative lesions with diffuse involvement, which were precisely described by capsule and double-balloon enteroscopy at the diagnosis, also improved following the treatment. This case provides a clue suggesting factors that promote PLE in PIFL.

Sjögren syndrome associated with protein-losing enteropathy: case-based review.

The association between Sjögren's syndrome (SS) and protein-losing enteropathy (PLE) was scarcly reported. To analyze the clinical, therapeutic, and outcome characteristics of patients with SS and PLE and also to delineate the potential mechanisms and pathways connecting the gut to SS targeted organ's pathology. Systematic screening was conducted using PubMed/MEDLINE, LILACS, SciELO, Web of Science, and Cochrane, dating 1980 to 2020. SS and PLE were the key words. Eighteen patients with SS and PLE were summarized. The patient's ages ranged between 20 and 88 years, and only 4 were males. Primary SS was observed in most cases. Anti-Ro was detected in 100% of the cases while anti-La was reported in 64% of them. The clinical manifestations were protein loss, edema of the lower limbs, pleural effusion, ascites, facial edema, anasarca, diarrhea, and weight loss. Among these clinical manifestations, edema of the lower limbs was the most severe. Albumin concentration was 0.9-3.4 g/dL which increased to 2.8-4.3 g/dL after treatment. Small bowel biopsy was performed in all of the cases. Concerning the therapy, all the patients received systemic glucocorticoids. All of them improved. The period of onset of improvement ranged from 3 weeks to 36 months (an average of 3 months). The early diagnosis and appropriate therapy of PLE in patients with anti-Ro positive SS and who present edema, anasarca, or hypoalbuminemia is vital for a beneficial outcome. An excellent clinical improvement in all the cases was observed when treated early enough by cortico-therapy, thus preventing patient's deterioration, complications, and reducing morbidity and potential mortality.

Glycomacropeptide Ameliorates Indomethacin-Induced Enteropathy in Rats by Modifying Intestinal Inflammation and Oxidative Stress.

Nonsteroidal anti-inflammatory drug (NSAID)-induced enteropathy is considered a serious and increasing clinical problem without available treatment. Glycomacropeptide (GMP) is a 64-amino acid peptide derived from milk κ-casein with numerous biological activities. The aim of this study was to investigate the protective effect of GMP on NSAID enteropathy in rats. Enteropathy was induced by seven days oral indomethacin administration. Rats were orally GMP treated from seven days previous and during the establishment of the enteropathy model. Changes in metabolism, hematological and biochemical blood alterations, intestinal inflammation and oxidative damage were analyzed. Integrity barrier markers, macroscopic intestinal damage and survival rate were also evaluated. GMP treatment prevented anorexia and weight loss in animals. Furthermore, prophylaxis with GMP ameliorated the decline in hemoglobin, hematocrit, albumin and total protein levels. The treatment had no therapeutic efficacy on the decrease of occludin and mucin (MUC)-2 expression in intestinal tissue. However, GMP markedly decreased neutrophil infiltration, and CXCL1, interleukin-1ß and inducible nitric oxide synthase expression. Nitric oxide production and lipid hydroperoxide level in the small intestine were also diminished. These beneficial effects were mirrored by preventing ulcer development and increasing animal survival. These results suggest that GMP may protect against NSAID enteropathy through anti-inflammatory and antioxidant properties.

A Systemic Ventricular Assist Device With a Fenestration in a Failing Fontan Patient.

Mechanical circulatory support in failing Fontan patients with Fontan circuit failure remains challenging. Herein, we describe a failing fenestrated Fontan patient who underwent systemic ventricular assist device support leaving the fenestration open. Perioperative course and hemodynamics during mechanical support are described in detail.

Unusual Late-onset Enteropathy in a Patient With Lipopolysaccharide-responsive Beige-like Anchor Protein Deficiency.

In recent years, monogenic causes of immune dysregulation syndromes, with variable phenotypes, have been documented. Mutations in the lipopolysaccharide-responsive beige-like anchor (LRBA) protein are associated with common variable immunodeficiency, autoimmunity, chronic enteropathy, and immune dysregulation disorders. The LRBA protein prevents degradation of cytotoxic T-lymphocyte antigen 4 (CTLA4) protein, thus inhibiting immune responses. Both LRBA and CTLA4 deficiencies usually present with immune dysregulation, mostly characterized by autoimmunity and lymphoproliferation. In this report, we describe a patient with an atypical clinical onset of LRBA deficiency and the patient's response to abatacept, a fusion protein-drug that mimics the action of CTLA4.

Gastrointestinal haemorrhage due to lymphangiectasia caused by protein-losing enteropathy in the Fontan circulation.

We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management.

Lupus protein-losing enteropathy patient with protein C and protein S deficiency-induced thrombosis: A case report with review of the literature.

A case report of SLE with PLE in an Asian female; presented with edema, pleural effusion, ascites and profound hypoalbuminemia. She also had severe protein C and protein S depletion from GI loss which caused extensive thrombosis. Her disease was refractory to the treatment with high dose steroid, azathioprine, mycophenolate mofetil and cyclophosphamide. Bowel resection was performed without improvement. Fortunately, the patient responded to another course of pulse methyl prednisolone and a second line medication after surgery.

Thromboembolism in Dogs with Protein-Losing Enteropathy with Non-Neoplastic Chronic Small Intestinal Disease.

Dogs with protein-losing enteropathy (PLE) are suggested to be at increased risk of developing thromboembolic events. However, with some exceptions, there are very few reports of thromboembolism in such dogs. This multicentre retrospective observational study describes a case series of thromboembolism (TE) in eight dogs with PLE secondary to non-neoplastic, chronic small intestinal disease. Seven dogs had poorly controlled PLE when the thromboembolic event occurred. Pulmonary thromboembolism (PTE) occurred in six dogs, while one dog developed splenic vein thrombosis and another had concurrent splenic vein and aortic TE. Six dogs died, all with PTE. Antithrombin activity was decreased in one of two dogs in which it was measured. Serum cobalamin and folate concentrations were measured in three dogs and cobalamin was subnormal in all three. Serum magnesium, measured in two dogs, was low in both. Dogs with uncontrolled chronic small intestinal disease and PLE are at risk for developing serious life-threatening TE, mostly PTE.

Protein-loosing enteropathy in sclerosing mesenteritis.

Sclerosing mesenteritis (SM) is a rare, idiopathic disorder of unknown aetiology that involves the adipose tissue of the mesentery, being characterized by chronic and non-specific fibrous inflammation. Patients usually present with non-specific clinical manifestations, such as abdominal pain and diarrhoea. The diagnosis of SM is difficult and it can be definitely established only by means of surgical or imaging-guided biopsy. Different therapeutic strategies have been used in case series with different rate of success. The disease is generally self-limiting, and the long-term prognosis is good, even if some cases of severe SM are reported in literature. Here, we report a fatal case of sclerosing mesenteritis associated to protein-losing enteropathy.

Enteropatía perdedora de proteínas en pacientes con corazón univentricular / Protein-losing enteropathy found in patients with univentricular heart

INTRODU: la enteropatía perdedora de proteínas puede aparecer en la evolución de los pacientes con corazón univentricular que sobreviven a la derivación cavopulmonar total. Una vez que se diagnostica, la mortalidad es alta. OBJETIVO: identificar los posibles factores de riesgo de esta complicación. MÉTODOS: se realizó un estudio de cohorte prospectivo de la evolución en 74 pacientes con derivación cavopulmonar total, intervenidos en el Cardiocentro Pediátrico "William Soler", desde enero de 1992 hasta enero de 2011. RESULTADOS: el tiempo promedio de evolución fue de 8 años. Sufrió enteropatía perdedora de proteínas 8,1 % de los pacientes. Se presentó con mayor frecuencia en los operados con la técnica intratrial, en los operados con más de 6 años de edad, y en quienes sufrieron derrames pleurales persistentes en el posoperatorio inmediato. Se encontró relación significativa entre la enteropatía y la disfunción ventricular posoperatoria, con RR= 11,45 (IC: 95 %: 2,37 a 55,16). El análisis multivariado identificó a la disfunción ventricular como factor de riesgo. CONCLUSIÓN: la detección de disfunción ventricular en la evolución del paciente con derivación cavopulmonar debe orientar el tratamiento, en aras de evitar la aparición de enteropatía perdedora de proteínas.

INTRODUCTION: protein-losing enteropathy may occur in the progression of patients with univentricular heart, who survived total cavopulmonary shunt. Once diagnosed, the mortality rate of the condition is high. ONJECTIVE: to identify the possible risk factor of this complication. METHODS: a prospective cohort study of the progression of 74 patients with total cavopulmonary shunt was conducted from January 1992 through January 2011. They had been operated on at "William Soler" pediatric cardiac center. RESULTS: the average time of progression was 8 years. In this group, 8.1 % of patients suffered protein-losing enteropathy that was more frequently seen in patients operated on by the intraatrial technique, aged over 6 years and in those suffering persistent pleural effusion in the immediate postoperative period. Significant statistical relation was found between enteropathy and postoperative ventricular dysfunction with RR= 11.45 (CI: 95 %: 2.37 to 55.16). The multivariate analysis showed that the ventricular dysfunction was a risk factor. CONCLUSIONS: Detection of the ventricular dysfunction in the progression of a patient with cavopulmonary shunt should guide the treatment to avoid occurrence of protein-losing enteropathy.

[Non-cirrhotic ascites: pathophysiology, diagnosis and etiology]. / L'ascite non liée à la cirrhose : physiopathologie, diagnostic et étiologies.

Ascites, in 20% of cases, is not linked to liver cirrhosis. The pathophysiology is most often different. The understanding of these pathophysiological mechanisms can lead to etiologic diagnosis. The diagnostic approach is mainly based on the biological study of ascites, especially protein concentration and albumin gradient between serum and ascites. In Western countries, tumors and heart diseases are the predominant causes, while developing countries are mainly concerned by infectious diseases, among which tuberculosis is the leading cause. Other uncommon causes must be recognized, as ascites may be the presenting feature of the disease. Their knowledge will facilitate the therapeutic approach.

Cryptogenia multifocal ulcerous stenosing enteritis: an entity on its own as a cause of abdominal pain, iron deficiency anemia and protein-losing enteropathy.

We studied a patient with edema secondary to protein losing enteropathy, and recurrent bouts of bloating and abdominal pain secondary to intestinal subocclusion episodes. After the clinical study, the patient was diagnosed of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE), that is a rare disease, probably caused by mutations in the gene PLA2G4A, and characterized by multiple short stenosis of the small bowel with superficial ulcers, which do not exceed the submucosa layer. Inflammatory bowel disease (Chron's disease), intestinal tuberculosis and intestinal ulcers secondary to non-steroidal anti-inflammatory drugs are the main differential diagnosis. To sum up, physicians should included CMUSE in the differential diagnosis of recurrent abdominal pain, iron deficiency anaemia, occult intestinal bleeding, edema and protein losing enteropathy.