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.

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.

Enteropatia perdedora de proteínas no lúpus eritematoso sistêmico: relato de caso / Protein-losing enteropathy in systemic lupus erythematosus: case report

A enteropatia perdedora de proteínas raramente pode ser observada em pacientes com lúpus eritematoso sistêmico. Essa situação clínica deve ser suspeitada quando houver hipoalbuminemia persistente, na presença de uma função hepática preservada, ingesta proteica adequada e ausência de proteinúria significativa. Descrevemos o caso de uma paciente de 48 anos com perda ponderal, derrames cavitários (ascite e derrame pleural) e edema de membros inferiores. O diagnóstico de lúpus foi firmado a partir da presença de linfopenia, proteinúria, FAN e autoanticorpos positivos (anti-Sm, anti-DNA e anti-Ro). Houve persistência de hipoalbuminemia mesmo com corticoterapia na dose de 1 mg/kg de peso, sendo diagnosticada enteropatia perdedora de proteínas por meio da cintilografia com albumina 99mTc. A melhora clínica e laboratorial da paciente veio após a associação da azatioprina com corticosteroide.

Protein-losing enteropathy is rarely seen in patients with systemic lupus erythematosus. This clinical condition should be suspected in the presence of persistent hypoalbuminemia despite normal liver function, adequate protein intake, and no significant proteinuria. We report the case of a 48-year-old female with weight loss, cavity effusions (ascites and pleural effusion), and lower extremity edema. The diagnosis of lupus was established based on the presence of lymphopenia, proteinuria, ANA, and positive autoantibodies (anti-Sm, anti-DNA, and anti-Ro). Because hypoalbuminemia persisted even with corticosteroid therapy at the dose of 1 mg/kg, protein-losing enteropathy was diagnosed by use of Tc-99m albumin scintigraphy. After adding azathioprine to the treatment, the symptoms subsided and serum albumin levels improved.

Intestinal lymphangiectasia in a patient with infantile systemic hyalinosis syndrome: a rare cause of protein-losing enteropathy

Infantile systemic hyalinosis [ISH] is a rare autosomal recessive disease. Typically, ISH patients present with progressive painful joint contractures, intractable diarrhea, hyperpigmented skin lesions, and perianal fleshy nodules. We report a case of a 19-month-old male child with atypical ISH presentation. His main clinical finding was protein-losing enteropathy due to intestinal lymphangectasia. This report is intended to enhance awareness about the gastrointestinal tract presentation of ISH

Implante de stents em conduto cirúrgico cavopulmonar: relato de dois casos / Stent implantation in surgical cavopulmonary conduit: report of two cases / Implante de stents en conducto quirúrgico cavopulmonar: dos casos clínicos

Apesar dos avanços na cirurgia de Fontan, obstruções nos condutos extracardíacos podem ocorrer e causar deterioração clínica. Relatamos dois casos em que foram realizados implante de stent para correção de estenose na cirurgia de Fontan. Ascite era o sinal clínico comum; um paciente tinha enteropatia perdedora de proteínas.Todos os procedimentos obtiveram sucesso angiográfico e clínico.

Despite advances in Fontan surgery, obstruction in extracardiac ducts may occur and cause clinical deterioration. We report two cases of stent implantation for correction of stenosis in the Fontan surgery. Ascites was a common clinical sign; a patient was protein losing enteropathy. All procedures were angiographically and clinically successful.

Pese a los avances en la cirugía de Fontan, obstrucciones en los conductos extracardíacos pueden producirse y ocasionar deterioro clínico. Relatamos dos casos en que se llevaron a cabo implante de stent para la corrección de estenosis en la cirugía de Fontan. Ascitis era o el signo clínico común; un paciente tenía enteropatía perdedora de proteínas. Todos los procedimientos obtuvieron éxito angiográfico y clínico.

Associação de bronquite plástica com enteropatia perdedora de proteínas após operação de Fontan / Association of plastic bronchitis to protein-losing enteropathy after fontan operation

Relatamos um caso incomum de associação de bronquite plástica (BP) com enteropatia perdedora de proteínas (EPP) em menina de 4 anos e 9 meses de idade. com dupla via de entrada de ventrículo único tipo esquerdo e concordância ventrículo-arterial submetida à cirurgia cavopulmonar total. com túnel lateral intracardíaco aos três anos. Tornaram-se chamativas a eliminação de molde brônquico de fibrina de 10 cm (BP) e a elevação de alfa-1-antitripsina de 52 mg/g de fezes. Em uso de sildenafila. programou-se. em caso de continuidade do processo. a ligadura do ducto torácico e transplante cardíaco.

We report an unusual case of association of plastic bronchitis (PB) to protein-losing enteropathy (PLE) in a girl of 4 years and 9 months of age with double inlet single left ventricle and ventriculoarterial concordance. submitted to total cavopulmonary surgery. with an intracardiac lateral tunnel at the age of three. The elimination of the 10 cm fibrin bronchial mold (PB) and the alpha-1-antitrypsin elevation of 52 mg/g in feces had both become outstanding. Using sildenafil. the thoracic duct ligature and the cardiac transplant were programmed in case of continuity of the process.

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.

Severe esophagitis in a child with Henoch-Schönlein purpura presenting as protein-losing enteropathy.

A 4-year-old boy was seen for vomiting, diarrhea and peripheral edema. He had no evidence of nephrosis or liver dysfunction. Upper gastrointestinal endoscopy was performed for investigating the etiology of protein-losing enteropathy. It showed severe esophagitis and multiple ulcers in the descending duodenum. The symptoms and endoscopic mucosal abnormalities subsided after three weeks of hospitalization without specific therapy. Ten days after being discharged he was seen again with characteristic rash of Henoch-Schönlein purpura and arthritis without gastrointestinal symptoms. Biopsy of the skin rash revealed leukocytoclastic vasculitis.

A Case of Severe Protein-losing Enteropathy as a Late Complication of Pelvic Irradiation

Department of Protein-losing enteropathy is the manifestation of a diverse set of disorders, and it is characterized by the excessive loss of plasma proteins into the affected portions of the gastrointestinal tract, and this results in hypoalbuminemia. We report here on a case of severe protein-losing enteropathy with the typical clinical features of hypoalbuminemia, dependent edema and increased alpha 1-antitrypsin (alpha1-AT) clearance, as measured by using 24hr stool testing. The associated disorder with the protein-losing enteropathy of our case was radiation enterocolitis and lymphatic obstruction that was due to radiation treatment and lymph node dissection in the remote past for the treatment of uterine cervical carcinoma. Our case suggests that chronic radiation enterocolitis can result in irreversible injury to the intestinal mucosa and a protein-losing enteropathy, which can bring about a very poor quality of life and even the loss of life.

A Systemic Lupus Erythematosus Patient with Protein Losing Enteropathy

Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE), and it is an uncommon cause of hypoalbuminemia without proteinuria. We describe a case of an 11 year-old girl who had diarrhea and periorbital edema as the initial symptoms of SLE. PLE was diagnosed by 24-hour stool alpha-1 antitrypsin clearance and 99mTc-human serum albumin scintigraphy.

Reactive arthritis, psoriasiform lesions and protein loosing enteropathy secondary to Strongyloidiasis.

Reactive arthritis is associated with demonstrable infection at a distant site without evidence of sepsis at the affected joint(s). We present a rare case reactive arthritis where no bacterial or chlamydial infections could be established, rather larvae of Strongyloides stercoralis could be demonstrated in the stool and duodenal biopsy. Reactive arthritis, psoriasiform lesions and malabsorption with hypoproteinaemia, responded to successful treatment with antihelminthic drugs. Early recognition and adequate treatment for gastrointestinal infections and infestations before complications is important.

Enteropatía neutropénica asociada a enfermedades autoinmunes. Una presentación más agresiva / Autoimmune disease-associated neutropenic enteropathy. A more aggressive presentation

Antecedentes: sólo existe un informe de caso de enteropatía neutropénica asociada a enfermedades autoinmunes. Método: se analizaron las autopsias de pacientes con diagnóstico de neoplasias y enfermedades autoinmunes, se revisaron las fotografías macroscópicas y preparaciones histológicas de tubo digestivo. En forma cegada se cuantificó el porcentaje de mucosa enteral con afección macroscópica. Los datos clínicos y de laboratorio se obtuvieron del expediente clínico y del resumen post mortem. Resultados: se identificaron 17 casos de enteropatía neutropénica en un periodo de 13 años (1,068 autopsias). En pacientes con enfermedades hematológicas ocurrió en 14 casos y tres asociados a enfermedades autoinmunes. Los síntomas agudos tuvieron una evolución de seis días caracterizados por dolor abdominal, diarrea, ascitis y fiebre en las enfermedades autoinmunes. La extensión del daño colónico fue de 58 por ciento y 13 por ciento en intestino delgado. Los casos asociados a enfermedades hematológicas tuvieron mayor tiempo de evolución con fiebre y dolor abdominal, lesiones colónicas en 21 por ciento de la superficie y lesiones en intestino delgado en 6 por ciento de la mucosa. No se observó infiltrado inflamatorio agudo alrededor de las zonas necróticas en ninguno de los grupos. Los medicamentos asociados con neutropenia fueron esteroides, azatioprina, metotrexate y agentes alquilantes. Conclusión: la enteropatía neutropénica asociada a enfermedades autoinmunes fue más grave que la asociada a enfermedades hematológicas atendiendo al porcentaje de mucosa enteral afectada y al curso clínico.

Linfangiectasia intestinal primaria / Primary intestinal lymphangiectasia

Informamos la observación de una niña de 18 meses con una enteropatía perdedora de proteínas con linfangiectasia intestinal, que presentó intenso edema generalizado, severa anemia, hipoalbuminemia e hipogamaglobulinemia, sin manifestaciones clínicas de malabsorción digestiva. La poca frecuencia de la entidad, la forma atípica de presentación y la excelente evolución lograda motivaron la presente comunicación

Protein-losing enteropathy in systemic lupus erythematosus

Enteropatia perdedora de proteina no lupus eritematoso sistemico. O caso de uma jovem de 23 anos com lupus eritematoso sistemico e enteropatia perdedora de proteina e descrito. A biopsia de delgado revelou linfangiectasia. O quadro regrediu com o uso de prednisona. A enteropatia perdedora de proteina deve ser suspeitada nos casos de lupus eritematoso sistemico com hipoalbuminemia e funcoes hepatica e renal preservadas. A revisao da literatura e apresentada salientando-se os aspectos fisiopatologicos envolvidos.