Fontan Conversion for Patients with Protein-Losing Enteropathy after a Björk Procedure with Patent Right Atrium to Innominate Vein Bypass / 日本心臓血管外科学会雑誌

A 37-year-old male patient who had previously undergone left original Blalock-Taussig shunt, original Glenn shunt, left pulmonary artery patch plasty, and a Björk procedure was referred to our hospital due to protein-losing enteropathy. Because he suffered from severe low-cardiac output syndrome immediately after the Björk procedure, mechanical circulatory support and construction of the bypass between the right atrial appendage and the innominate vein using an artificial graft were required. We performed a Fontan-revision operation : total cavopulmonary connection with extra-cardiac conduit, right atrial ablation, pacemaker lead implantation, construction of fenestration between the conduit and the atrium, and reconstruction of the left pulmonary artery in front of the ascending aorta successfully. His postoperative course was uneventful and protein-losing enteropathy had not recurred 3 years after the operation.

An unusual cause of edema in a child with congenital heart disease post Fontan procedure

Protein Losing Enteropathy Post Fontan procedure. Protein Losing Enteropathy (PLE) is an uncommon cause of edema in children with congenital heart disease. Protein-Losing Enteropathy may be defined as excessive loss of proteins across the intestinal mucosa and is due to either a primary gastrointestinal abnormality or secondary to cardiac disease. Protein-losing enteropathy (PLE) is a rare complication of the Fontan palliation for functional single-ventricle. Although PLE occurs in about 3.5% of patients post-Fontan, it confers marked morbidity and high mortality within 5 years of diagnosis. The pathogenesis of Fontan-related PLE is not completely understood, and it is unclear why it develops in some patients post-Fontan and not others. We describe a child with Double Inlet Right Ventricle who had undergone Fontan procedure, and presented to us with generalised oedema. The child had hypoproteinaemia, the common causes for which were ruled out and was diagnosed as Protein Losing Enteropathy (PLE) related to his surgical intervention. Though, not frequently encountered it should be kept in mind as one of the causes of anasarca.

Magnetic Resonance Imaging Assessment of Blood Flow Distribution in Fenestrated and Completed Fontan Circulation with Special Emphasis on Abdominal Blood Flow

OBJECTIVE: To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI). MATERIALS AND METHODS: We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.3–17.0 years) with Fontan circulation in whom flow volumes across the thoracic and abdominal arteries and veins were measured by using MRI. The patients were divided into three groups: fenestrated Fontan circulation group with MRI performed under general anesthesia (GA) (Group 1, 15 patients; average age, 5.9 years), completed Fontan circulation group with MRI performed under GA (Group 2, 6 patients; average age, 8.7 years), and completed Fontan circulation group with MRI performed without GA (Group 3, 18 patients; average age, 12.5 years). The patient data were compared with the reference ranges in healthy controls. RESULTS: In comparison with the controls, Group 1 showed normal cardiac output (3.92 ± 0.40 vs. 3.72 ± 0.69 L/min/m2, p = 0.30), while Group 3 showed decreased cardiac output (3.24 ± 0.71 vs. 3.96 ± 0.64 L/min/m2, p = 0.003). Groups 1 and 3 showed reduced abdominal flow (1.21 ± 0.28 vs. 2.37 ± 0.45 L/min/m2, p < 0.001 and 1.89 ± 0.39 vs. 2.64 ± 0.38 L/min/m2, p < 0.001, respectively), which was mainly due to the diversion of the cardiac output to the aortopulmonary collaterals in Group 1 and the reduced cardiac output in Group 3. Superior mesenteric and portal venous flows were more severely reduced in Group 3 than in Group 1 (ratios between the flow volumes of the patients and healthy controls was 0.26 and 0.37 in Group 3 and 0.63 and 0.53 in Group 1, respectively). Hepatic arterial flow was decreased in Group 1 (0.11 ± 0.22 vs. 0.34 ± 0.38 L/min/m2, p = 0.04) and markedly increased in Group 3 (0.38 ± 0.22 vs. −0.08 ± 0.29 L/min/m2, p < 0.0001). Group 2 showed a mixture of the patterns seen in Groups 1 and 3. CONCLUSION: Fontan circulation is associated with reduced abdominal flow, which can be attributed to reduced cardiac output and portal venous return in completed Fontan circulation, and diversion of the cardiac output to the aortopulmonary collaterals in fenestrated Fontan circulation.

Protein-losing enteropathy in children / 中国实用儿科杂志

Protein-losing enteropathy,or protein-losing gastroenteropathy,refers to a group of diseases in which serum proteins are excessively lost from the gastrointestinal tract due to various causes. The pathogenesis mainly includes mucosal erosion and exudation,increased permeability and intestinal lymphatic obstruction. Clinical manifestations vary greatly with underlying diseases,and the common manifestations are diarrhea,edema and malnutrition. The main characteristics of laboratory examination are the decrease of serum albumin and globulin. Increase in 24-hour alpha-1 antitryptase clearance rate or lymphonuclide intestinal protein tracing image can be used as an evidence of intestinal protein loss. Treatment includes dietary therapy and treatment for underlying diseases.

A Case of Protein Losing Enteropathy as Only Clinical manifestation of Systemic Lupus Erythematosus / 고신대학교의과대학학술지

Protein losing enteropathy (PLE) due to systemic lupus erythematosus (SLE) is relatively uncommon. PLE may be appeared sequentially after the diagnosis of SLE or concurrently with SLE. In most of concurrent cases, PLE was diagnosed one of various symptoms of SLE. Cases of PLE as the initial and only clinical presentation of SLE have been rarely reported. We described a 30-year old woman with general edema and abdominal distension was diagnosed PLE after stool alpha 1 antitrypsin clearance test. Her symptoms were getting worse even though the treatment with intravenous albumin. She was finally diagnosed PLE associated with SLE by additional laboratory findings (positive antinuclear antibody and anti-dsDNA IgG and low C3, C4 and CH50). She was treated with high dose of steroids and her symptoms were improved.

Tripla infecção com HTLV-1, leishmaniose visceral e estrongiloidíase complicada por pancreatite aguda / Triple infection with HTLV-1, visceral leishmaniasis and strongyloidiasis complicated by acute pancreatitis

lntrodução: a estrongiloidíase tem grande importância médica devido à capacidade de o Strongyloides stercoralis completar seu ciclo de vida no homem e gerar a síndrome de hiperinfecção principalmente em imunocomprometidos. Devido à dificuldade em estruturar a resposta Th2, os pacientes infectados com o Vírus Linfotrópico de Células T Humanas Tipo 1 (HTLV-1) têm maior tendência a apresentar infecção maciça. A leishmaniose visceral, doença relevante em países em desenvolvimento, causa alterações imunológicas semelhantes, porém há poucos relatos de suscetibilidade específica ao Strongyloides stercoralis nos infectados por Leishmania sp. O presente trabalho tem objetivo de relatar um caso de coinfecção HTLV e calazar, que apresentou-se como pancreatite aguda e enteropatia perdedora de proteínas secundárias à estrongiloidíase maciça. Relato de caso: trata-se de um paciente de 34 anos com história de leishmaniose prévia que deu entrada no nosso Serviço com pancreatite aguda idiopática leve, além de história de diarreia crônica há um ano com anasarca e hipoalbuminemia associadas. Apresentou endoscopia digestiva alta com atrofia duodenal importante, tendo sido identificados Strongyloides stercoralis em biópsia, além de sorologia para HTLV positiva. Apresentou translocação bacteriana com sepse grave de foco abdominal, após início do tratamento com ivermectina, tendo posteriormente evoluído com melhora clínica importante e remissão dos sintomas. Fez investigação com punção de medula óssea, em que foram identificadas as formas amastigotas da leishmania. Discussão e conclusão: a presença de HLTV é um fator de risco para a síndrome de hiperinfecção por Strongyloides stercoralis, tendo predisposto o paciente às manifestações graves e raras descritas. A identificação de leishmania na medula óssea, entretanto, é um fator de risco ainda pouco conhecido para estrongiloidíase disseminada, porém com plausibilidade biológica por afetar o sistema imunológico do hospedeiro.(AU)

Introduction: strongyloidiasis has great medical importance because of the ability of the Strongyloides stercoralis to complete its life cycle in man and cause hyperinfection syndrome especially in immunocompromised hosts. Because of the difficulty in triggering The response, Human T-cell lymphotropic virus type 1 (HTLV-1) infected patients has susceptibility for massive infection. Visceral leishmaniasis, a relevant disease in developing countries, causes similar immunological changes, but there are few reports of specific susceptibility to Strongyloides stercoralis on infected by Leishmania sp. This study aimed to report a case of HTLV and kala azar coinfection, presenting as acute pancreatitis and protein losing enteropathy secondary to massive strongyloidiasis. Case report: a 34-year-old patient previously treated for leishmaniasis has presented at our service with idiopathic acute pancreatitis and chronic diarrhea for one year with anasarca and hypoalbuminemia. Upper endoscopy revealed duodenal atrophy in which biopsy identified Strongyloides stercoralis, and HLTV serology was positive. He presented with bacterial translocation and severe sepsis after first dose of ivermectin, but has clinical improvement and remission of symptoms afterwards. Bone marrow aspiration identified amastigote forms of Leishmania. Discussion and Conclusion: the presence of HLTV is a risk factor for Strongyloides stercoralis hyperinfection, and predisposed this patient to the serious and rare events described. The identification of Leishmania in bone marrow, however, is an poorly known risk factor for disseminated strongyloidiasis, but with biological plausibility because it affects the immune system of the host.(AU)

Prognostic factors of protein-losing enteropathy in a Mexican cohort of patients with congenital heart disease, surgically corrected with Fontan procedure / Factores pronósticos de enteropatía perdedora de proteínas en una cohorte mexicana de pacientes con cardiopatía congénita, corregidas quirúrgicamente con cirugía de Fontan

Background: Protein-losing enteropathy (PLE) is a known postoperative complication affecting about 10% of patients surgically managed with Fontan procedure. The mortality rate associated with this complication increases to 50%. Objective: To determine the risk factors associated to the development of PLE in patients surgically managed with Fontan procedure. Methods: This was a case-cohort study, and the universe of the trial comprised all patients treated with univentricular surgery. We included male and female patients with congenital heart disease that conditioned a single ventricle syndrome. Those patients with previous intestinal disease causing protein loss, were excluded, cow's milk protein allergy, intestinal resection (previous or after heart surgery), use of cyclic parenteral nutrition or Fontan's dismantlement. Follow-up began immediately after hospital discharge from Fontan procedure. Outcome variable was the development of PLE; independent variables were some before and after surgery hemodynamic and echocardiographic variables, infections and treatment. Statistical analysis: We used measures of statistical dispersion and central tendency. Risk was calculated for each variable estimating the hazard ratio (HR), adjusted for confounding factors; and Kaplan-Meier estimator was used for survival analysis. Results: Eleven (26%) out from patients 42 developed PLE. The median of time between Fontan procedure and the development of this complication was five years. The prognostic variables were: systolic pressure of pulmonary artery between 12-15 mmHg, > 3 years between Glenn and Fontan procedures, aggravated chronic malnutrition, direct bilirubin values > 1.5 mg/dL, pulmonary resistances (APR) between 3-3.5 Wood units, previous hepatomegaly and pleural effusion > 6 day-period. The probability of dying from PLE was 63% in a 10-year period. Conclusions: The prognostic factors associated with PLE are previous hepatic damage and borderlines values of venous pressure.

Antecedentes: La enteropatía perdedora de proteínas (EPP) es una conocida complicación que afecta alrededor del 10% de los sujetos operados con el procedimiento de Fontan. La mortalidad asociada a esta complicación se eleva al 50%. Objetivo: Determinar los factores de riesgo asociados al desarrollo de EPP en pacientes postoperados de procedimiento de Fontan. Métodos: Este es un estudio de caso-cohorte y el universo comprendió a todos los pacientes corregidos con cirugía univentricular. Se incluyeron pacientes de ambos géneros, con cardiopatías que condicionaran síndrome de ventrículo único. Se excluyeron aquellos con enfermedad previa intestinal causante de pérdida de proteínas, alergia a las proteínas de la leche de vaca, resección intestinal (previa o después de la cirugía cardiaca), aquellos con nutrición parenteral cíclica o desmantelamiento del Fontan. El inicio de seguimiento comenzó inmediatamente después del egreso de la cirugía de Fontan. La variable de desenlace fue el desarrollo de enteropatía perdedora de proteínas. Las variables independientes estudiadas fueron algunas variables hemodinámicas y ecocardiográficas pre- y postquirúrgicas, infecciones y tratamiento. Análisis estadístico: Se usaron medidas de dispersión y tendencia central. Se calculó el riesgo por cada variable, estimando el cociente de riesgo (Hazard Ratio, HR en inglés), ajustándose a variables de confusión. Se utilizó el estimador de Kaplan-Meier para el análisis de supervivencia. Resultados: Once de 42 pacientes (26%) desarrollaron EPP. La mediana de tiempo entre la cirugía de Fontan y el desarrollo de esta complicación fue de cinco años. Las variables pronósticas fueron presión sistólica de la arteria pulmonar entre 12-15 mmHg, el tiempo > 3 años entre las intervenciones de Glenn y Fontan, la desnutrición crónica agudizada, una cifra de bilirrubina directa > 1.5 mg/dL, URP entre 3 y 3.5 Unidades Wood, hepatomegalia previa y derrame > 6 días. La probabilidad de mortalidad al desarrollar EPP a 10 años fue de 63%. Conclusiones: Los factores pronósticos asociados a EPP son el daño hepático previo y las variables limítrofes de presión venosa.

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.

Linfangiectasia intestinal: Reporte de un caso / A case report of Intestinal Lymphangiectasia

A propósito del caso de un paciente de 7 meses de vida remitido desde Yopal a la ciudad de Bogotá, se revisa el tema de la linfagiectasia intestinal. Esta es una rara enfermedad que involucra los vasos linfáticos intestinales, y origina hipoproteinemia, edemas, ascitis y enteropatía perdedora de proteínas.

This is the case report of a 7 month old child from Yopal with intestinal lymphangiectasia who was sent to Bogota. We also review the issue of intestinal lymphangiectasia, a rare disease involving intestinal lymphatic vessels which caused hypoproteinemia, edema, ascites and protein-losing enteropathy.

Linfangiectasia intestinal asociada a hemihipertrofia: Reporte de caso / Intestinal Lymphangiectasia associated with Hemihypertrophy: A case report

La linfangiectasia intestinal primaria es una enfermedad caracterizada por dilatación de los vasos linfáticos del intestino, manifestándose como una enteropatía perdedora de proteínas. Presentamos un paciente con diarrea crónica, prolapso rectal recurrente y hemihipertrofia de miembro superior izquierdo, asociado a linfopenia e hipoalbuminemia. Por endoscopia digestiva superior y biopsia se diagnostica linfangiectasia intestinal primaria y se inicia tratamiento nutricional exitosamente.

Primary intestinal lymphangiectasia is a disease characterized by dilated intestinal lymph vessels which manifests as a protein losing enteropathy. We present a patient with chronic diarrhea, recurrent rectal prolapse and left upper limb hemihypertrophy associated with lymphopenia and hypoalbuminemia. Primary intestinal lymphangiectasia was diagnosed with upper endoscopy and biopsy. Nutritional treatment was successfully begun.

Evaluation of paediatric patients with protein losing enteropathy a single centre experience / Evaluación de los pacientes pediátricos con enteropatía perdedora de proteínas experiencia sola en un centro

OBJECTIVE: The aim of the study is to evaluate paediatric patients with protein losing enteropathy (PLE). METHODS: Fourteen cases diagnosed as PLE were evaluated in terms of aetiologies, diagnostic methods, laboratory findings, treatment procedures and longterm prognosis. RESULTS: Four of the cases had coeliac disease, three intestinal lymphangiectasia, three giardia infection, one H pylori infection and three cytomegalovirus (CMV) infection. Histopathological examinations of duodenum specimens revealed total villous atrophy in four cases, lymphatic dilatation in three cases, severe nodular appearance in four cases and no pathology in four cases. All of the cases except patients with intestinal lymphangiectasia were controlled by the appropriate treatment given for the underlying disease. The cases with CMV infection were treated with only supportive treatment and gancyclovir therapy was not needed. CONCLUSION: When proteinuria is not detected in wellappearing children admitted with oedema, PLE must be considered.

OBJETIVO: El objetivo del estudio es evaluar a pacientes con enteropatía perdedora de proteínas (EPP). MÉTODOS: Catorce casos diagnosticados con EPP fueron evaluados en términos de etiologías, métodos de diagnóstico, resultados de laboratorio, procedimientos de tratamiento, y prognósis a largo plazo. RESULTADOS: Cuatro de los casos tenían enfermedad celíaca, tres padecían de linfangiectasia intestinal, tres sufrían de infección por giardias, uno tenía infección por H pylori, y tres presentaba infección por citomegalovirus (CMV). Los exámenes histopatológicos de especímenes duodenales revelaron atrofia de las vellosidades intestinales en cuatro de los casos, dilatación linfática en tres casos, apariencia nodular severa en cuatro casos, y ausencia de patología en cuatro casos. Todos los casos - excepto los pacientes con linfangiectasia intestinal - fueron controlados mediante el tratamiento adecuado para la enfermedad subyacente. Los casos con infección por CMV fueron tratados con tratamiento de apoyo, y no se necesitó terapia con ganciclovir. CONCLUSIÓN: Cuando no se detecta proteinuria en niños con buena apariencia ingresados con edema, hay que considerar principalmente la posibilidad de EPP.

A Case of Protein Supplement Effect in Protein-Losing Enteropathy

The objective of this article is to report improvement of nutritional status by protein supplements in the patient with protein-losing enteropathy. The patient was a female whose age was 25 and underwent medical treatment of Crohn's disease, an inflammatory bowl disease, after diagnosis of cryptogenic multifocal ulcerous enteritis. The weight was 33.3 kg (68% of IBW) in the severe underweight and suffered from ascites and subcutaneous edema with hypoalbuminemia (1.3 g/dL) at the time of hospitalization. The patient consumed food restrictively due to abdominal discomfort. Despite various attempts of oral feeding, the levels of calorie and protein intake fell into 40-50% of the required amount, which was 800-900 kcal/d (24-27 kcal/kg/d) for calorie and 34 g/d (1 g/kg/d) for protein. It was planned to supplement the patient with caloric supplementation (40-50 kcal/kg) and protein supplementation (2.5 g/kg) to increase body weight and improve hypoproteinemia. It was also planned to increase the level of protein intake slowly to target 55 g/d in about 2 weeks starting from 10 g/d and monitored kidney load with high protein supplementation. The weight loss was 1.0 kg when the patient was discharged from the hospital (hospitalization periods of 4 weeks), however, serum albumin was improved from 1.3 g/dL to 2.5 g/dL and there was no abdominal discomfort. She kept supplement of protein at 55 g/d for 5 months after the discharge from the hospital and kept it at 35 g/d for about 2 months and then 25 g/d. The body weight increased gradually from 32.3 kg (65% of IBW) to 44.0 kg (89% of IBW) by 36% for the period of F/u and serum albumin was kept above 2.8 g/dL without intravenous injection of albumin. The performance status was improved from 4 points of 'very tired' to 2 points of 'a little tired' out of 5-point scale measurement and the use of diuretic stopped from the time of 4th month after the discharge from the hospital owing to improvement in edema and ascites. During this period, the results of blood test such as BUN, Cr, and electrolytes were within the normal range. In conclusion, hypoproteinemia and weight loss were improved by increasing protein intake through utilization of protein supplements in protein-losing enteropathy.

An Indigenous Case of Intestinal Capillariasis with Protein-Losing Enteropathy in Korea

We encountered an indigenous case of intestinal capillariasis with protein-losing enteropathy in the Republic of Korea. A 37-year-old man, residing in Sacheon-si, Gyeongsangnam-do, admitted to the Gyeongsang National University Hospital (GNUH) due to long-lasting diarrhea, abdominal pain, anasarca, and weight loss. He recalled that he frequently ate raw fish, especially the common blackish goby (Acanthogobius flavimanus) and has never been abroad. Under the suspicion of protein-losing enteropathy, he received various kinds of medical examinations, and was diagnosed as intestinal capillariasis based on characteristic sectional findings of nematode worms in the biopsied small intestine. Adults, juvenile worms, and eggs were also detected in the diarrheic stools collected before and after medication. The clinical symptoms became much better after treatment with albendazole 400 mg daily for 3 days, and all findings were in normal range in laboratory examinations performed after 1 month. The present study is the 6th Korean case of intestinal capillariasis and the 3rd indigenous one in the Republic of Korea.

Primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy . We report a series of 4 children from Chennai, India presenting with anasarca, recurrent diarrhea, hypoproteinemia and confirmatory features of PIL on endoscopy and histopathology.

A Case of Protein-losing Enteropathy Associated with Small Bowel Villous Atrophy / 대한소화기학회지

Protein losing enteropathy is described as a diverse group of disorders associated with excessive loss of serum proteins into the gastrointestinal (GI) tract. The etiology of protein losing enteropathy is various. Increased mucosal permeability to protein as a result of cell damage, mucosal erosion, or lymphatic obstruction may develop protein losing enteropathy. Celiac disease is a common cause of protein losing enteropathy associated with small bowel villous atrophy in Europe. We experienced a case of protein losing enteropathy associated with small bowel villous atrophy of unknown origin. A 36-year-old woman was admitted due to chronic watery diarrhea and weight loss. Laboratory findings showed total protein 4.7 g/dL, albumin 2.7 g/dL, cholesterol 100 mg/dL, WBC 6,000/mm(3) (lymphocyte 13.6%) with the absence of proteinuria. On esophagogastroduodenoscopic examination, duodenal ulcer scar was noted on the bulb and colonoscopic finding was nonspecific. On small bowel enteroscopy, jejunal and ileal villi was scantly noticed. Small bowel biopsy showed marked villous atrophy. Her symptoms did not improve after supportive care. Gluten free diet was tried because celiac disease could not be ruled out completely. Diarrhea ceased and body weight regained after gluten free diet.

A Case of Annular Polycyclic Type of Subacute Cutaneous Lupus Erythematosus with Protein Losing Enteropathy / 대한피부과학회지

We present a case of annular polycylic type of subacute lupus erythematosus which fulfilled the criteria of systemic lupus erythematosus and was associated with protein losing enteropathy. A 37-year-old male had erythematous annular rashes with several painful, scattered ulcers and a generalized edematous appearance. Abnormal laboratory findings were hypoalbuminemia, low complement, positive Anti-Ro, La, and ANA in a speckled pattern. Tc-99m human serum albumin scintigraphy revealed extravasation within the small bowel. The histopathologic findings showed vacuolar degeneration, upper dermal edema and cleft with perivascular lymphocytic infiltration. Direct IF revealed granular deposition of IgG along the dermo-epidermal junction. The patient was treated with intravenous steroids and oral hydroxychloroquine.

A Case of Protein Losing Enteropathy Caused by Primary Intestinal Lymphangiectasia / 대한소화기내시경학회지

Primary intestinal lymphangiectasia is a rare congenital cause of protein losing enteropathy that is characterized by chronic diarrhea, generalized edema, ascites, hypoproteinemia, hypoalbuminemia, and lymphopenia. We encountered an 18-year-old woman who suffered from longstanding diarrhea and progressive leg edema. The laboratory findings showed the typical features of this disorder. The presence of enteric protein loss was documented with the 24 hour fecal clearance of alpha(1)-antitrypsin and (99m)Tc human serum albumin scintigraphy. A duodenoscopy and biopsy showed scattered white spots and markedly dilated lymphatics in the tips of the villi, respectively. The patient's clinical symptoms improved after placing her on a high protein and low fat diet with medium chain triglyceride supplements.

A case of constrictive pericarditis presenting with protein-losing enteropathy / 소아과

Constrictive pericarditis represents a rare cause of protein-losing enteropathy in children. Reported is an 11-year-old girl with protein-losing enteropathy (PLE) as the principal manifestations of constrictive pericarditis. After total pericardiectomy, symptoms and signs of PLE disappeared. Doppler echocardiography including tissue Doppler imaging is a useful noninvasive initial diagnostic tool for differential diagnosis of diastolic heart failure.

A Case of Protein-losing Enteropathy Treated with High Dose Intravenous Glucocorticoid Therapy in Systemic Lupus Erythematosus / 영남의대학술지

Generalized edema and hypoalbuminemia are relatively common presenting manifestations in many clinical situations. The differential diagnosis of hypoalbuminemia include: Kwashiorkor, synthetic dysfunction of the liver, and excessive protein loss as in nephrotic syndrome. In systemic lupus erythematosus (SLE), hypoalbuminemia and generalized edema are most commonly due to protein loss associated with lupus nephritis; gastrointestinal involvement is uncommon, and therefore protein loss through the gastrointestinal tract is quite rare. We report a case of a protein losing enteropathy (PLE) associated with SLE. The patient was referred to our hospital for generalized edema, arthralgia and facial rash. After clinical evaluation, the patient met the criteria for the SLE diagnosis; hypoalbuminemia with general edema was consistent with a protein losing enteropathy. After two weeks of therapy with parenteral high dose glucocorticoid, the patients was improved in laboratory findings as well as clinical symptoms.

A Case of Protein-losing Enteropathy with an Abnormal Cortisol Response to ACTH Stimulation / 대한내분비학회지

We hereby report a case of a 62-year-old male patient who was misdiagnosed with adrenal insufficiency during the course of protein-losing enteropathy caused by superior mesenteric arterial thrombosis. The patient was suspected to have adrenal insufficiency due to hyponatremia and severe weakness. The cortisol responses to the initial challenge of 250microgram ACTH were inadequate (maximum serum cortisol level after ACTH challenge was 10.9microgram/dL), while the serum albumin concentration was 1.9g/dL. Subsequently, intravenous steroid therapy was given to the patient. However, after bowel resection, the serum albumin level increased to 3.4g/dL and the cortisol response to the follow-up rapid ACTH stimulation was completely normal. Accordingly, we discontinued steroid replacement and discharged the patient without any problem. In conclusion, measuring total serum cortisol in a patient with hypo-pro-teinemia may lead to misdiagnosis of adrenal insufficiency. In such cases, caution should be exercised in interpreting the results in terms of total serum cortisol level or measurement of serum free cortisol levels should be considered.