Parasitoses intestinais no nordeste entre 2012 e 2021: uma revisão integrativa de literatura / Intestinal parasitosis in the northeast between 2012 and 2021: an integrative literature review / Parasitosis intestinales en el noreste entre 2012 y 2021: una revisión bibliográfica integradora

As parasitoses intestinais ainda constituem um problema de saúde pública no Brasil, especialmente nas regiões onde o saneamento básico e condições socioeconômicas são frágeis, como na região Nordeste. São um conjunto de doenças causadas por parasitas helmínticos ou protozoários, que acometem o intestino humano, causando sintomas, como diarreia, dor abdominal, astenia, náuseas, vômitos, entre outros. Seu diagnóstico é feito pelo parasitológico de fezes e o tratamento é baseado no uso de anti-protozoários ou anti-helmínticos. Objetivo: analisar os principais aspectos das parasitoses intestinais presentes no Nordeste, identificando aquelas mais prevalentes e as populações de maiores riscos. Metodologia: o estudo foi do tipo descritivo, utilizando-se uma revisão integrativa de literatura do período de 2012 a 2021, nas bases de dados eletrônicas Scielo, BVS e Google Acadêmico. Para o processo de busca e análise dos artigos, foi utilizado o diagrama flow. Todos os dados da pesquisa foram analisados no Microsoft Word e, posteriormente os resultados foram transformados em quadros e tabelas para melhor organização e interpretação das informações colhidas. Resultados: observou-se, a partir da análise dos 10 artigos, uma variação de 26,00% a 92,85% na taxa de infecções por parasitas intestinais na região Nordeste, em diversas populações, desde pré-escolares, escolares e adultos. Os fatores de risco que estiveram associados ao desenvolvimento, foram: falta de higiene pessoal e dos alimentos, ausência ou precariedade dos serviços de saneamento básico, baixa escolaridade e renda dos pais, qualidade da água de beber e práticas de andar descalço por parte das crianças. Considerações finais: Os resultados apontam para a necessidade de medidas preventivas e tratamento das enteroparasitoses, como mudanças de hábitos de higiene e melhorias nas condições de vida, como o acesso à água tratada e saneamento básico adequado. Além disso, é importante que surjam mais estudos epidemiológicos para subsidiar medidas de controle e prevenção adequadas e eficazes.

Intestinal parasites are still a public health problem in Brazil, especially in regions where basic sanitation and socioeconomic conditions are fragile, as in the Northeast region. They are a set of diseases caused by helminthic or protozoan parasites, which affect the human intestine, causing symptoms such as diarrhea, abdominal pain, asthenia, nausea, vomiting, among others. Its diagnosis is made by fecal parasitology and treatment is based on the use of anti-protozoal or anthelmintic agents. Objective: to analyze the main aspects of intestinal parasites present in the Northeast, identifying the most prevalent ones and the populations at greatest risk. Methodology: the study was descriptive, using an integrative literature review from 2012 to 2021, in the electronic databases Scielo, BVS and Google Scholar. For the process of searching and analyzing the articles, the flow diagram was used. All survey data were analyzed in Microsoft Excel and, later, the results were transformed into graphs and tables for better interpretation of the collected information. RESULTS: from the analysis of the 10 articles, a variation from 26.00% to 92.85% was observed in the rate of infections by intestinal parasites in the Northeast region, in different populations, from preschoolers, schoolchildren and adults. The risk factors that were associated with development were: lack of personal hygiene and food, absence or precariousness of basic sanitation services, low parental education and income, quality of drinking water and practices of walking barefoot by children. Final Considerations: The results point to the need for preventive measures and treatment of intestinal parasites, such as changes in hygiene habits and improvements in living conditions, such as access to treated water and adequate basic sanitation. In addition, it is important that more epidemiological studies appear to support appropriate and effective control and prevention measures.

Las parasitosis intestinales siguen siendo un problema de salud pública en Brasil, especialmente en regiones donde el saneamiento básico y las condiciones socioeconómicas son frágiles, como en la región Nordeste. Son un conjunto de enfermedades causadas por parásitos helmínticos o protozoarios, que afectan el intestino humano, causando síntomas como diarrea, dolor abdominal, astenia, náuseas, vómitos, entre otros. Su diagnóstico se realiza mediante parasitología fecal y el tratamiento se basa en el uso de agentes antiprotozoarios o antihelmínticos. Objetivo: analizar los principales aspectos de los parásitos intestinales presentes en el Nordeste, identificando los más prevalentes y las poblaciones de mayor riesgo. Metodología: el estudio fue descriptivo, utilizando una revisión bibliográfica integradora de 2012 a 2021, en las bases de datos electrónicas Scielo, BVS y Google Scholar. Para el proceso de búsqueda y análisis de los artículos, se utilizó el diagrama de flujo. Todos los datos de la encuesta fueron analizados en Microsoft Excel y, posteriormente, los resultados fueron transformados en gráficos y tablas para una mejor interpretación de la información recogida. Resultados: a partir del análisis de los 10 artículos, se observó una variación de 26,00% a 92,85% en la tasa de infecciones por parásitos intestinales en la región Nordeste, en diferentes poblaciones, desde preescolares, escolares y adultos. Los factores de riesgo que se asociaron al desarrollo fueron: falta de higiene personal y alimentaria, ausencia o precariedad de servicios de saneamiento básico, baja escolaridad e ingresos de los padres, calidad del agua de consumo y prácticas de caminar descalzo por parte de los niños. Consideraciones Finales: Los resultados apuntan a la necesidad de medidas preventivas y de tratamiento de los parásitos intestinales, como cambios en los hábitos de higiene y mejoras en las condiciones de vida, como el acceso a agua tratada y a un saneamiento básico adecuado. Además, es importante que aparezcan más estudios epidemiológicos que respalden medidas de control y prevención adecuadas y eficaces.

Enteropatías congénitas y la implicación diagnóstica en la displasia intestinal o enteropatía en penacho

RESUMEN Introducción: la displasia epitelial intestinal o enteropatía en penacho, es una rara y grave enfermedad debido a mutaciones genéticas, categorizada como enteropatía congénita por defecto del transporte de los enterocitos y su polaridad. Objetivo: Examinar los conocimientos más recientes para la orientación diagnóstica de las enteropatías congénitas en el contexto clínico de las diarreas crónicas intratables del período posnatal y primeros meses de vida. Métodos: Análisis de publicaciones en español e inglés en PubMed, Scimago, ScIELO hasta abril 2021 relacionadas con esta temática. Se usaron los términos diarreas intratables, enteropatías congénitas, displasia epitelial intestinal, enteropatía en penacho y enfermedades para diagnóstico diferencial. Análisis e integración de la información: Se describieron criterios sobre diarreas intratables, enteropatías congénitas; su clasificación. Se revisaron rasgos de la displasia epitelial intestinal y sus manifestaciones de formas clínicas, histopatológicas y mutaciones genéticas, epidemiología, diagnóstico de certeza y diferencial por afecciones debido a defectos congénitos relacionados con el tránsito epitelial y polaridad; tratamiento, pronóstico y actualización sobre trascendencia de las enfermedades raras. Conclusiones: Se revisan los conocimientos más recientes relacionados con enfermedades raras por enteropatías congénitas y en particular sobre la displasia epitelial intestinal o enteropatía en penacho. Se describen sus manifestaciones clínicas, histopatológicas y genéticas. La epidemiología, el tratamiento y sus retos. Se enfatizó en criterios sobre la trascendencia diagnóstica de enfermedades raras relacionadas con enteropatías congénitas.

ABSTRACT Introduction: Intestinal epithelial dysplasia or plume enteropathy is a rare and serious disease due to genetic mutations, categorized as congenital enteropathy due to the defect of enterocyte transport and their polarity. Objective: Examine the most recent knowledge on the diagnostic orientation of congenital enteropathies in the clinical context of intractable chronic diarrhea of the postnatal period and first months of life. Methods: Analysis of publications in Spanish and English in PubMed, Scimago, ScIELO until April 2021 related to this topic. The terms intractable diarrhea, congenital enteropathies, intestinal epithelial dysplasia, plume enteropathy and diseases were used for differential diagnosis. Analysis and integration of information: Criteria on intractable diarrhea, and congenital enteropathies were described; and their classification. Features of intestinal epithelial dysplasia and its manifestations of clinical, histopathological forms and genetic mutations, epidemiology, diagnosis of certainty and differential for conditions due to congenital defects related to epithelial transit and polarity were reviewed; treatment, prognosis and update on the importance of rare diseases. Conclusions: The most recent knowledge related to rare diseases due to congenital enteropathies and in particular about intestinal epithelial dysplasia or plume enteropathy is reviewed. Its clinical, histopathological and genetic manifestations are described, epidemiology, treatment and its challenges. Emphasis was placed on criteria on the diagnostic significance of rare diseases related to congenital enteropathies.

Echocardiographic and Ultrasonographic Parameters Associated with Protein-losing Enteropathy in Patients with Fontan Physiology: a Systematic Review with Meta-analysis

Abstract Background: Fontan circulation can be associated with significant morbidity, especially Protein-Losing Enteropathy (PLE). Echocardiographic parameters can provide valuable diagnostic information about a patient's risk of developing PLE after Fontan surgery. Objectives: To describe echocardiographic/ultrasonographic parameters associated with PLE in patients after Fontan surgery through a systematic review with meta-analysis. Methods: A literature search was performed in electronic databases to identify relevant studies about echocardiographic parameters and PLE prediction in children after Fontan surgery. The search terms used were: "echocardiography", "ultrasonography", "Fontan," and "protein-losing enteropathy". A p < 0.05 was considered statistically significant. Results: A total of 653 abstracts were obtained from electronic databases and bibliographic references. From these, six articles met criteria to be included in the qualitative analysis and three in the quantitative (meta-analysis). The resistance index in the superior mesenteric artery was described in three studies, and the quantitative analysis showed statistical significance (p < 0.001). Other echocardiographic and ultrasonographic parameters were also described, albeit in single studies not allowing a meta-analysis. Conclusion: This systematic review with meta-analysis identified echocardiographic and ultrasonographic parameters related to PLE in patients with Fontan physiology. Vascular ultrasonography seems to play a prominent role in this aspect, but additional studies are needed to increase the degree of evidence.

Tríada de Herbst: una presentación inusual de una patología común. Reporte de caso / Herbst Triad: A rare clinical manifestation of a common pathology

Resumen La tríada de Herbst es una manifestación inusual de la enfermedad por reflujo gastroesofágico y de otras patologías esofágicas. Se caracteriza por la presencia de anemia, acropaquias (hipocratismo digital) y enteropatía perdedora de proteínas. Al ser una condición anecdótica, la información disponible deriva de los reportes de caso. La fisiopatología aún no es clara. Se reporta el caso de una escolar, en quien se revierten los síntomas una vez se realiza el manejo quirúrgico.

Abstract The Herbst triad is a rare manifestation of gastroesophageal reflux disease and other esophageal pathologies. It is characterized by the presence of anemia, digital clubbing, and protein-losing enteropathy. Since evidence on this condition is anecdotal, the available information is mostly derived from case reports and its physiopathology remains unclear. The following is the case of a schoolchild, whose symptoms were reversed once she underwent surgery.

Enteropatia perdedora de proteína relacionada a criptococose: um diagnóstico diferencial de imunodeficiência secundária / Protein-losing enteropathy related to cryptococcosis: a differential diagnosis of secondary immunodeficiency

A criptococose é uma doença oportunista que ocorre com maior frequência em pacientes imunossuprimidos, ocasionando piora clínica e imunológica importante. Porém, é raro quando a doença ocorre em pacientes imunocompetentes. Relatamos aqui um caso de paciente previamente hígido que evoluiu com enteropatia perdedora de proteína, hipogamaglobulinemia secundária causada por criptococose disseminada.

Cryptococcosis is an opportunistic disease that occurs more frequently in immunosuppressed patients, causing important clinical and immunological deterioration. However, the disease rarely occurs in immunocompetent patients. We report a case of a previously healthy patient who progressed with protein-losing enteropathy, secondary hypogammaglobulinemia caused by disseminated cryptococcosis.

Linfangiectasia intestinal primaria: primer reporte de caso en Perú / Primary intestinal lymphangiectasia: first case report in Peru

La linfangiectasia intestinal primaria es una entidad clínica poco común de etiología desconocida. La edad típica de presentación de esta enfermedad es durante los 3 primeros años de vida, pero también se han reportado casos en adultos. Posee sintomatología variable, pero la manifestación clínica principal es el edema, puede presentarse también diarrea y pérdida de peso. La pérdida de fluido linfático en el tracto gastointestinal conlleva también a hipoproteinemia y linfopenia. El diagnóstico se establece en base a la clínica, a los estudios de laboratorio, al estudio endoscópico y se confirma con la evaluación histológica de la biopsia realizada. El manejo se da mediante una dieta rica en proteínas, baja en grasas y triglicéridos de cadena media. A continuación, se presenta el caso de un paciente varón de 1 año de edad que presenta edema generalizado, con predominio de miembros inferiores, y diarrea. Los exámenes de laboratorio muestran la presencia de hipoproteinemia marcada. Posteriormente, se realiza una endoscopía digestiva alta y una biopsia duodenal. El estudio histológico confirma el diagnóstico de linfangiectasia intestinal primaria. El paciente recibe el tratamiento establecido para esta enfermedad y, finalmente es dado de alta.

Primary intestinal lymphangiectasia is a rare clinical condition of unknown etiology. The common age of presentation is during the first 3 years of life, but cases in adults have also been reported. It has a variable symptomatology, but the main clinical manifestation is edema, also diarrhea and weight loss can occur. The loss of lymph fluid into the gastrointestinal tract also leads to hypoproteinemia and lymphopenia. Diagnosis is based on clinical manifestations, laboratory and endoscopic findings, and is confirmed on histopathological examination of biopsy. The main treatment is a protein rich, low in fat and medium chain triglyceride diet. We present the case of a 1-year-old male patient who presents with generalized edema, predominantly in lower limbs, and diarrhea. Laboratory findings show the presence of marked hypoproteinemia. Then an endoscopy and a duodenal biopsy are performed, and the histopathological study confirms the diagnosis of primary intestinal lymphangiectasia. The patient is treated and after a satisfactory evolution, is discharged.

Congenital diarrhea and enteropathies in children / 中国实用儿科杂志

Congenital diarrhea and enteropathies(CODEs)are typically monogenic disorders. Patients always present with persistent and severe chronic diarrhea. The diagnosis of CODEs is oftern delayed and there is a high mortality. Application of endoscopy,histologic evaluation and next generation sequencing might facilitate the diagnosis,and precision medicine to patients with CODEs.

Resolution of Protein-Losing Enteropathy after Congenital Heart Disease Repair by Selective Lymphatic Embolization / 대한소아소화기영양학회지

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

Ménétrier’s Disease as a Gastrointestinal Manifestation of Active Cytomegalovirus Infection in a 22-Month-Old Boy: A Case Report with a Review of the Literature of Korean Pediatric Cases

Ménétrier’s disease (MD), which is characterized by hypertrophic gastric folds and foveolar cell hyperplasia, is the most common gastrointestinal (GI) cause of protein-losing enteropathy (PLE). The clinical course of MD in childhood differs from that in adults and has often been reported to be associated with cytomegalovirus (CMV) infection. We present a case of a previously healthy 22-month-old boy presenting with PLE, who was initially suspected to have an eosinophilic GI disorder. However, he was eventually confirmed, by detection of CMV DNA using polymerase chain reaction (PCR) with gastric tissue, to have MD associated with an active CMV infection. We suggest that endoscopic and pathological evaluation is necessary for the differential diagnosis of MD. In addition, CMV DNA detection using PCR analysis of biopsy tissue is recommended to confirm the etiologic agent of MD regardless of the patient’s age or immune status.

Síndrome de desregulação imune, poliendocrinopatia e enteropatia ligada ao X (IPEX): a importância da história familiar para o diagnóstico precoce / Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: the importance of family history for early diagnosis

A síndrome de desregulação imune, poliendocrinopatia e enteropatia ligada ao X (IPEX) é uma síndrome de imunodeficiência primária rara, de herança recessiva, que afeta lactentes do sexo masculino. A doença cursa com enteropatia perdedora de proteínas, dermatite eczematosa e poliendocrinopatias, podendo ser fatal naqueles sem tratamento apropriado. O objetivo deste relato é descrever um caso de IPEX, enfatizando a importância da história familiar para o diagnóstico precoce. O caso descreve um lactente com tipo grave da síndrome, com apresentação clínica precoce e história familiar característica, com episódios de morte prematura em doze homens pertencentes à linhagem materna. O diagnóstico por mapeamento genético demostrando mutação no gene FOXP3 foi obtido após o óbito do paciente, decorrente de choque séptico. O transplante de células-tronco hematopoiéticas é o melhor tratamento disponível, e na sua ausência, a síndrome IPEX pode ser fatal nos primeiros dois anos de vida. Assim, assegurar um diagnóstico precoce é fundamental.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare recessive primary immunodeficiency syndrome that affects male infants. The disease course is characterized by protein-losing enteropathy, eczematous dermatitis, and polyendocrinopathies, and may be fatal if not appropriately treated. The aim of this report was to describe a case of IPEX, emphasizing the importance of family history for early diagnosis. The case describes an infant with a severe manifestation of the syndrome, with early clinical presentation and characteristic family history, with episodes of premature death affecting 12 men belonging to the mother's lineage. Diagnosis was established by genetic mapping after the patient's death due to septic shock; a mutation in the FOXP3 gene was found. Hematopoietic stem cell transplantation is the best treatment available; in its absence, the IPEX syndrome can be fatal in the first 2 years of life. Therefore, ensuring early diagnosis is critical.

Multicausal etiology of the enteric syndrome in rabbits from Mexico / Multicausalidad del síndrome entérico en conejos de México

Enteropathies in rabbits are difficult to diagnose; their etiology involves pathogens that act synergistically, causing damage to the intestine. The aim of the present study was isolate enteric pathogens from rabbits in Mexico. Using parasitological, bacteriological and molecular analyses, we screened 58 samples of the intestinal content of rabbits having a clinical history of enteric disease from the southeastern part of the State of Mexico. Out of the 58 samples analyzed, a total of 86 identifications were made, Eimeria spp. were found in 77.5%, followed by Aeromonas spp. in 15.5% and Escherichia coli in 8.6%, which were identified as enteropathogenic E. coli (EPEC), and the presence of the following agents was also confirmed: Salmonella spp., Klebsiella spp., Streptococcus spp., Staphylococcus aureus, Enterococcus spp., Mannheimia spp. and Rotavirus. The concurrent presence of Eimeria spp. with Aeromonas was frequent (15.5%); there was statistical significance for the presence of an association between the clinical profiles and Eimeria spp. (p = 0.000), Mannheimia spp. (p = 0.001), Salmonella spp., Klebsiella spp., Streptococcus spp. and Enterococcus spp. (p = 0.006).

Las enteropatías en conejos son difíciles de diagnosticar, debido a que en su etiología participan patógenos que actúan en sinergia y causan daño al intestino. El objetivo de este estudio fue el aislamiento de patógenos de cuadros entéricos en conejos de México. Mediante métodos parasitológicos, bacteriológicos y moleculares, se analizaron 58 muestras de contenido intestinal de conejos con el antecedente de cuadro clínico entérico procedentes de granjas cunícolas de la zona suroriente del Estado de México. A partir de las 58 muestras se realizaron un total de 86 identificaciones, los patógenos más frecuentes fueron Eimeria spp. (77,6%), seguidas de Aeromonas spp. (15,5%) y de Escherichia coli (8,6%), identificadas como E. coli enterpatogénicas (EPEC). También se determinó la presencia de otros agentes: Salmonella spp., Klebsiella spp., Streptococcus spp., Staphylococcus aureus, Enterococcus spp., Mannheimia spp. y Rotavirus. La presencia concurrente de Eimeria spp. y Aeromonas spp. (15,5%) fue la observación más frecuente. Se encontró asociación estadísticamente significativa entre la presentación del cuadro clínico en conejos y la presencia de Eimeria spp. (p = 0,000), Mannheimia spp. (p = 0,001), Salmonella spp., Klebsiella spp., Streptococcus spp. y Enterococcus spp. (p = 0,006).

Protein-Losing Enteropathy as a Complication of the Ketogenic Diet

The ketogenic diet is an effective treatment for the patients with intractable epilepsy, however, the diet therapy can sometimes be discontinued by complications. Protein–losing enteropathy is a rarely reported serious complication of the ketogenic diet. We present a 16-month-old Down syndrome baby with protein-losing enteropathy during the ketogenic diet as a treatment for West syndrome. He suffered from diarrhea, general edema and hypoalbuminemia which were not controlled by conservative care for over 1 month. Esophagogastroduodenoscopy and stool alpha-1 antitrypsin indicated protein-losing enteropathy. Related symptoms were relieved after cessation of the ketogenic diet. Unexplained hypoalbuminemia combined with edema and diarrhea during ketogenic suggests the possibility of protein-losing enteropathy, and proper evaluation is recommended in order to expeditiously detect it and to act accordingly.

Disseminated Cytomegalovirus Infection and Protein Losing Enteropathy as Presenting Feature of Pediatric Patient with Crohn's Disease / 대한소아소화기영양학회지

We report a pediatric patient admitted with abdominal pain, diffuse lower extremity edema and watery diarrhea for two months. Laboratory findings including complete blood count, serum albumin, lipid and immunoglobulin levels were compatible with protein losing enteropathy. Colonoscopic examination revealed diffuse ulcers with smooth raised edge (like "punched out holes") in the colon and terminal ileum. Histopathological examination showed active colitis, ulcerations and inclusion bodies. Immunostaining for cytomegalovirus was positive. Despite supportive management, antiviral therapy, the clinical condition of the patient worsened and developed disseminated cytomegalovirus infection and the patient died. Protein losing enteropathy and disseminated cytomegalovirus infection a presenting of feature in steroid-naive patient with inflammatory bowel disease is very rare. Hypogammaglobulinemia associated with protein losing enteropathy in Crohn's disease may predispose the cytomegalovirus infection in previously healthy children.

Factors related to outcomes in lupus-related protein-losing enteropathy

BACKGROUND/AIMS: Protein-losing enteropathy (PLE), characterized by severe hypoalbuminemia and peripheral edema, is a rare manifestation of systemic lupus erythematosus. This present study aimed to identify the distinctive features of lupus-related PLE and evaluate the factors related to the treatment response. METHODS: From March 1998 to March 2014, the clinical data of 14 patients with lupus PLE and seven patients with idiopathic PLE from a tertiary center were reviewed. PLE was defined as a demonstration of protein leakage from the gastrointestinal tract by either technetium 99m-labelled human albumin scanning or fecal alpha1-antitrypsin clearance. A positive steroid response was defined as a return of serum albumin to > or = 3.0 g/dL within 4 weeks after initial steroid monotherapy, and remission as maintenance of serum albumin > or = 3.0 g/dL for at least 3 months. A high serum total cholesterol level was defined as a level of > or = 240 mg/dL. RESULTS: The mean age of the lupus-related PLE patients was 37.0 years, and the mean follow-up duration was 55.8 months. Significantly higher erythrocyte sedimentation rate and serum total cholesterol levels were found for lupus PLE than for idiopathic PLE. Among the 14 patients with lupus PLE, eight experienced a positive steroid response, and the serum total cholesterol level was significantly higher in the positive steroid response group. A positive steroid response was associated with an initial high serum total cholesterol level and achievement of remission within 6 months. CONCLUSIONS: In lupus-related PLE, a high serum total cholesterol level could be a predictive factor for the initial steroid response, indicating a good response to steroid therapy alone.

An analysis of seven cases of intestinal lymphangiectasia complicated by portal vein hypertension / 中华消化杂志

Objective To investigate the incidence,diagnosis,treatment and prognosis of abnormal lymphatic vessels in intestinal lymphangiectasia (IL) patients complicated by portal vein hypertension.Methods General information,clinical manifestations,laboratory tests,endoscopy and pathological examination,the results of direct and nuclide lymphangiography,treatment and prognosis of seven IL patients complicated by portal vein hypertension were retrospective analyzed.Results Among seven IL patients complicated by portal vein hypertension,there were five cases with abnormal lymphatic vessels and two cases were merely secondary to portal vein hypertension.The main symptoms were edema,abdominal distension and diarrhea.The results of laboratory tests mainly were lymphocytopenia (0.3× 109 to 1.7 × 109/L),hypoalbuminemia (16.0 to 27.8g/L) and hypoglobulinemia (13.2 to 17.7 g/L).Under endoscope,mucosal edema,chronic inflammatory reaction,intestinal villi spot and patched white nodular changes and polyp-like changes appeared.For treatment,the low-fat with medium-chain triglyceride diet and symptomatic support was the basic treatment.The condition could be relieved by surgical remission of abnormal lymphatic vessels or partial small-bowel resection.If the etiological factors could not be removed,the therapeutic effect was poor in the patients merely secondary to portal vein hypertension.Conclusion The suspicious IL patients complicated by portal vein hypertension need further examination to explore whether abnormal lymphatic vessels existed,and to provide evidence for surgical intervention.

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.

A case of cytomegalovirus-negative Menetrier's disease with eosinophilia in a child / 소아과

Menetrier's disease is a rare form of acquired gastropathy characterized by giant rugal folds in the stomach and protein-losing gastropathy. Children with Menetrier's disease tend to follow a benign self-limited course with symptoms typically completely resolving within 2 to 10 weeks in contrast to the chronic course in adults. A 9-year-old girl presented with a history of gradually worsening abdominal distension, increasing body weight, and abdominal pain for 2 weeks. Physical examination on admission indicated periorbital swelling, pitting edema in both the legs, and abdominal distension with mild diffuse tenderness and shifting dullness. Laboratory tests on admission showed hypoalbuminemia, hypoproteinemia, and peripheral eosinophilia. The test result for anticytomegalovirus immunoglobulin M was negative. Increased fecal alpha 1 anti-trypsin excretion was observed. Radiological findings showed massive ascites and pleural effusion in both the lungs. On gastroscopy, large gastric folds, erythema, erosion, and exudation were noted in the body and fundus of the stomach. Microscopic findings showed infiltration of eosinophils and neutrophils in the gastric mucosa. Her symptoms improved with conservative treatment from day 7 of hospitalization and resolved completely.

A Case of Acute Lymphoblastic Leukemia Presenting with Protein-Losing Enteropathy / 대한소화기학회지

Protein-losing enteropathy (PLE) is a syndrome characterized by excessive gastrointestinal protein loss, resulting in hypoproteinemia and edema. A variety of benign and malignant conditions can be associated with PLE and acute leukemia is a very rare cause of PLE. We report a case of PLE associated with acute lymphoblastic leukemia. A 27-year-old man was admitted due to watery diarrhea, epigastric pain and bilateral leg edema. Laboratory findings showed hypoproteinemia and polycythemia. The diagnosis of PLE and acute lymphoblastic leukemia were confirmed on the measurement of fecal alpha1-antitrypsin clearance and bone marrow examination. After systemic chemotherapy and autologous stem cell transplantation, his clinical symptoms and abnormal laboratory findings were gradually improved.

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.

A clinical analysis of 61 cases of protein-losing enteropathy / 中华内科杂志

Objective To increase the understanding in protein-losing enteropathy (PLE).Methods Sixty-one PLE patients were enrolled in the study and the clinical characteristics, complicated disease, diagnosis and treatment were analyzed. Results The age of the patients was 16-77 (40±15)years, and the gender ratio was 35:26 (female: male). The main clinical manifestations were bilateral lower limb edema in 51 cases, ascites in 41 cases, bilateral pleural effusion in 23 cases, pericardial effusion in 13cases, abdominal pain in 16 cases and diarrhea in 33 cases. The prominent abnormality in laboratory examinations was hypoalbuminemia. The underlying diseases include systemic lupus erythematosus (SLE) in 28 cases, intestinal lymphangiectasia in 12 cases, hepatic cirrhosis in 5 cases, heart diseases in 5 cases,Crohn's disease in 3 cases, membranous nephropathy in 2 cases, Budd-Chiari syndrome in 1 case. Four cases happened after abdominal operation and 1 case after radiation therapy of gastric cardia cancer. Thirtyseven cases were diagnosed by 99Tcm-labelled human serum albumin scintigraphy and 24 cases were diagnosed clinically. Treatment was focused on underlying diseases. The clinical manifestations in 21 cases of SLE improved after SLE was controlled. In 2 cases of intestinal lymphangiectasia and one with Crohn's disease, the clinical manifestations improved after surgery. The other patients had no improvement.Conclusions PLE was not uncommon in clinical practice. Its predominant characteristics were severe hypoalbuminemia, edema and dropsy of serous cavity. PLE can complicate other diseases such as SLE,intestinal lymphangiectasia. Treatment should be focused on primary disease.