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.

Gastropatía hipertrófica perdedora de proteínas: enfermedad de Ménétrier: caso clínico / Hypertrophic protein-losing gastropathy: Ménétrier disease: a clinical case

Introduction: Ménétrier disease is a rare disorder characterized by gastric foveolar hyperplasia associated with secondary protein loss. In children, this condition is presented as an edematous syndrome without renal or hepatic impairment and differs from the adult form by the constant presence of edema and spontaneous remission. It has been related to infections in most published cases, especially to Cytomegalovirus (CMV) and Helicobacter pylori (H. pylori). Objective: To present a pediatric case of Ménétrier disease and endoscopic imaging obtained during the evolution of the patient. Case report: A five year old preschooler who presented a generalized edema, abdominal pain and malaise. After ruling out renal and hepatic pathologies, an upper endoscopy revealed a severe compromise of the gastric mucosa. Urease test for H. pylori and IgG test for CMV resulted positive. Albumin and H2 receptor antagonists were administered. The evolution was favorable and the patient was discharged after 14 days; 8 month follow-up endoscopy showed no abnormalities. Conclusion: The medical profile and endoscopy are enough evidence to suggest the diagnosis of hypertrophic protein-losing gastropathy. Further studies need to be developed that include a considerable number of patients to assess their association with CMV or H. pylori infections, as these viruses are very common in our population.

Introducción: La enfermedad de Ménétrier es una entidad clínica rara, de etiología desconocida, que se caracteriza por hiperplasia foveolar gástrica asociada a pérdida secundaria de proteínas. En niños, se presenta como un síndrome edematoso sin compromiso renal ni hepático y difiere de la forma adulta por la presencia constante de edema y la remisión espontánea En la mayoría de los casos publicados se la relaciona a infecciones, en especial a Cytomegalovirus (CMV) y Helicobacter pylori (Hp). Objetivo: Presentar un caso pediátrico de Enfermedad de Ménétrier y las imágenes endoscópicas que se obtuvieron durante su evolución. Caso clínico: Preescolar de 5 años que consultó por edema generalizado, dolor abdominal y compromiso del estado general. Habiéndose descartado patología renal y hepática se solicitó una endoscopía digestiva alta que reveló un severo compromiso de la mucosa gástrica. Test de ureasa para Hp e IgG para CMV positivos. Se administró albúmina y antagonistas de receptores H2. La evolución fue favorable con alta al día 14 y endoscopía normal a los 8 meses de seguimiento. Conclusión: El cuadro clínico y la endoscopía son suficientes para plantear el diagnóstico de "Gastropatía hipertrófica perdedora de proteínas". Es necesario desarrollar estudios con un número considerable de pacientes para evaluar su asociación con infección por CMV o Hp, considerando además que estas infecciones son muy frecuentes en nuestra población.

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.

Chronic Non-granulomatous Ulcerative Jejunoileitis Assessed by Wireless Capsule Endoscopy / 대한소화기학회지

Chronic non-granulomatous jejunoileitis is a rare disease characterized by malabsorption, abdominal pain, and diarrhea that causes shallow ulcers in the small bowel. The etiology of chronic non-granulomatous jejunolieitis remains unknown. A 69-year-old man complained of abdominal pain and lower extremity edema. A 99m-Tc albumin scan showed increased radioactivity at the left upper quadrant, suggesting protein-losing enteropathy. A small bowel follow-through did not disclose any lesions. Wireless capsule endoscopy revealed several small bowel ulcers and strictures. A jejunoileal segmentectomy with end-to-end anastomosis was performed, and the histologic examination revealed non-granulomatous ulcers with focal villous atrophy. Ruling out all other possible diagnoses, we diagnosed our patient with chronic non-granulomatous ulcerative jejunoileitis. Postoperatively, the patient's abdominal pain and lower extremity edema improved, and the serum albumin normalized. This is the first case of chronic non-granulomatous ulcerative jejunoileitis localized by wireless capsule endoscopy and treated successfully with segment resection.

Acrodermatitis Enteropathica-like Eruption Associated with Combined Nutritional Deficiency

We present here a case of acrodermatitis enteropathica-like eruption associated with essential free fatty acid and protein deficiencies as well as borderline zinc deficiency that occurred after Whipple's operation in a 31-yr-old woman. Her eruptions were improved not by zinc supplements alone, but her condition was improved by total parenteral nutrition including amino acids, albumin, lipid and zinc. Although we could not exactly decide which of the nutrients contributed the most to her manifestations, we inferred that all three elements in concert caused her dermatoses. This case shows that even though the patient's skin manifestations and laboratory results are suggestive of acrodermatitis enteropathica, the physicians should keep in mind the possibility that this disease can be associated with other nutritional deficiencies such as free fatty acid or protein deficiency.

Oral disodium cromoglycate and ketotifen for a patient with eosinophilic gastroenteritis, food allergy and protein-losing enteropathy.

We present a case report of a 10 years old boy with protein-losing enteropathy and eosinophilic gastroenteritis who had positive histamine release tests, increased allergen-specific IgE antibodies to some food items, and low levels of total serum protein and albumin. Upper gastrointestinal endoscopy revealed a number of polyps and diffuse gastritis. Biopsy specimens of the stomach and duodenum showed widespread eosinophilia and neutrophilia. Although a restricted diet was recommended, a diet which excluded foods with positive results to both histamine release test and allergen-specific IgE antibodies was poorly tolerated, and the patient rejected systemic administration of corticosteroids. Thus, we initiated an oral disodium cromoglycate (DSCG) and ketotifen therapy. After oral DSCG and ketotifen administration, the patient's condition improved gradually. Therefore, oral DSCG and ketotifen therapy might be considered as treatment option in patients with eosinophilic gastroenteritis and protein-losing enteropathy caused by food allergy.

Detecting protein losing enteropathy by Tc-99m dextran scintigraphy: a novel experience.

OBJECTIVE: To evaluate protien using enteropathy by Tc-99m dextran scintigraphy. METHODS: Methods for detecting protein loss from the intestine revolve around fecal nitrogen excretion, the clearance of alpha-1 antitrypsin in stools and by endoscopic biopsy. RESULT: The diagnosis of protein-losing enteropathy (PLE) can also be established by a scintigraphic method that is noninvasive, simple and requires no patient preparation or motivation. This diagnostic modality can also delineate the site of protein loss, thereby offering a targeted approach, and if need be, surgery. Radiolabelling of a non-protein, noncolloidal, nonparticulate and biofriendly molecule like dextran with Technetium-99m for imaging enteric protein loss was utilized in imaging eight children with PLE. CONCLUSION: The results were encouraging. The authors advocate the use of this diagnostic tool in identifying patients with PLE, particularly in the pediatric age group.

Enteropatia perdedora de proteína associada a retocolite ulcerativa inespecífica e infecçäo por salmonella / Protein-losing enteropathy associated to ulcerative colitis and salmonella intestinal infection

Os autores apresentam um provável caso de enteropatia perdedora de proteínas como complicaçäo de retocolite ulcerativa inespecífica e infecçäo intestinal por salmonella em uma mulher branca de 34 anos de idade, após a exclusäo de afecçäo renal ou hepática que pudesse explicar a hipoproteinemia. Comentam os principais aspectos clínicos, radiológicos, endoscópicos e histopatológicos e o tratamento.(au)

Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5 percent of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7 percent), protein-losing enteropathy (33.3 percent) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management

Enteropatía perdedora de proteínas como manifestación inicial de lupus eritematoso sistémico. Diagnostico y seguimiento con alfa-1 antitripsina

La enteropatía perdedora de proteínas es una de las manifestaciones gastrointestinales del lupus eritematoso sistémico (LES); se caracteriza por hipoalbuminemia sérica con o sin edema y algunos síntomas digestivos. Se informa el caso de una niña de 13 años quien presentó dolor abdominal, diarrea, edema generalizado e hipoalbuminemia. Se hizó el diagnóstico de enteropatía perdedora de proteínas con base en el marcado incremento de la depuración de alfa-1 antitripsina en las heces de 24 horas; la paciente tenía más de los cuatro criterios establecidos por el Colegio Americano de Reumatología para el diagnóstico de LES. Hasta la fecha se han descrito aproximadamente 22 casos de enteropatía perdedora de proteínas asociada al LES, pero únicamente tres en el lupus pediátrico. Sugerimos que se debe sospechar esta afección en todo individuo con LES que presente edema o hipoalbuminemia no nefrogénicos.

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

Clearance fecal de Ó1 antitripsina: valores normales en niños de la ciudad de Córdoba / Faecal clearence of the Ó1-antitrypsin: values normal in children of the Cordoba city

En las enteropatías perdedoras de proteínas (EPP) el incremento de la permeabilidad de las paredes del TGI causa exudación proteica superior a la normal que se manifiesta por elevada excreción proteica fecal. El estudio del turnover metabólico, mediante la inyección intravenosa de proteínas marcadas con radioisótopos del yodo, provee información sobre el metabolismo proteico, pero no es útil para medir pérdida proteica gastrointestinal pues el yodo libre atraviesa con facilidad la mucosa gástrica en ambos sentidos, lo que conduce a una valoración errónea de la excreción proteíca en la materia fecal (MF). La albúmina marcada con 51Cr permite valorar la pérdida proteíca gastrointestinal caracterizada por un patron de pérdida al bulto. Los mencionados son metodos de referencia, invasores, costosos y su empleo está reglamentado por la legislación especial pues es material radioactivo. El clearance fecal de Ó1 antitripsina es inocuo y accesible a laboratorios clínicos y posee eficacia clínica probada para valorar la excreción proteica gastrointestinal. Se ha implementado la metodología y establecido valores normales de referencia para la población infantil de la ciudad de Córdoba. La muestra estuvo conformoda por 30 niños clínicamente sanos de ambos sexos, cuyas edades estuvieron comprendidas entre los 14 y 120 meses; en el protocolo de estudio de identificación se registró la edad, sexo y análisis de laboratorio. La determinación de Ó1AT sérica y fecal se realizó por inmunodifusión radial simple (IDRS). En las enteropatías puede ocurrir malabsorción proteíca, causante de hipoalbuminemia, o pérdida proteica gastrointestinal (en enteropatías exudativas), la cual afecta las fracciones albúmina y globulinas. El clearance fecal de Ó1AT indica los mililitros de plasma depurados en 24 horas por el tracto gastrointestinal y provee orientación diagnóstica útil para el uso clínico de rutina. Los valores de referencia hallados en la población infantil de Córdoba son: -clearance fecal de Ó1AT= desde no determinable hasta 10.8ml/24h; -concentración de Ó1AT sérica = 118 a 396mg por ciento

"Clearance" fecal da alfa-1-antitripsina / Fecal clearance of alpha 1-antitrypsin

Há enteropatias perdedoras de proteínas (PLE) com e sem sintomas digestivos ou hipoproteinemia. O diagnóstico através da perda fecal de albumina é inviável, exceto por método radioativo. A alternativa da dosagem fecal da alfa-1-antitripsina (AAT) - uma proteína plasmática especialmente resistente à digestao proteolítica - caiu em descrédito: os resultados variavam, indiscriminadamente, com o grau de comprometimento da mucosa e com a concentraçäo plasmática de AAT. O clearance (relaçäo da massa média de AAT fecal por 24 horas com a concentraçäo plasmática da substância) elimina a inespecificidade e evita erros decorrentes do grau de hidrataçäo das fezes ou de seu conteúdo de sólidos.

Gastroenteropatias perdedoras de proteínas: comprovaçäo pelo teste de excreçäo fecal da albumina-51 Cr em 50 casos de diferentes etiologias / Protein-losing gastroentheropathis: evidence through fecal excretion test of 51 Cr-albumin in 50 cases of different etiologies

Entre setembro de 1976 e dezembro de 1986, determinou-se a excreçäo fecal de radioatividade após injeçäo intravenosa da albumina Cr, para detecçäo e medida da perda gastrointestinal de proteínas, em 128 testes realizados em um grupo de 116 pacientes. Destes, 20 eram controles, 38 tinham suspeita clínica da gastroenteropatia perdedora de proteínas e 58 apresentavam doenças presumivelmente associadas a perda protéica digestiva. Os resultados obtidos no grupo controle permitiram definir o limite superior de normalidade do exame em 2,6% da radioatividade administrada. Valores anormais foram encontrados em 23 dos 38 casos com evidências clínicas da gastroenteropatioa perdedora de proteínas, em 12 dos quais haviam alteraçöes primárias ou secundárias do sistema linfático. No subgrupo de pacientes com doenças associadas à perda protéica digestiva, valores anormais da excreçäo fecal da albumina Cr foram encontrados em 11 dos 19 pacientes com paracoccidioidomicose, em quatro dos 14 pacientes com hipertensäo portal esquistossomótica, em cinco dos 14 pacientes com insuficiência cardíaca congestiva crônica e em sete dos 11 pacientes com diferentes afecçöes primárias do tubo digestivo. Os resultados do teste da excreçäo fecal da albumina Cr apresentaram boa correlaçäo com os valores da medida do clearance gastrointestinal de proteínas plasmáticas e tiveram compatibilizaçäo satisfatória om outros dados de ordem clínica e laboratorial. Os 50 casos em que se comprovou perda excessiva de proteínas pelo trato gastrointestinal, em seu conjunto, ilustraram a grande diversidade das doenças que causam esta anormalidade, muitas delas sendo muito comuns em nosso meio, bem como evidenciaram a importância das afecçöes que acometem o sistema linfático como causa das gastroenteropatias perdedoras de proteínas

Gastroenteropatia perdedora de proteínas na infância: observaçoës clínicas, laboratoriais e terapêuticas em 13 pacientes / Protein losing gastroenterophaties in children: clinical, laboratory and therapeutic observations in 13 patients

Säo apresentados 13 crianças com gastroenteropatia perdedora de proteínas, comprovada pelo teste de excreçäo fecal de albumina 51Cr. As idades variaram de 3 meses a 12 anos com uma mediana de 6 anos e 11 meses, sendo que em 11 pacientes os sintomas tiveram início entre 3 a 7 anos. Desnutriçäo de I§ e § graus foi detectada em dez casos. A diarréia foi o achado clínico mais freqüente, econtrado em 11 pacientes, seguido de edema periférico, anorexia, palidez verificados em dez: hipotrofia muscular nove; distensäo abdominal em oito: ascite e emagrecimento em sete crianças e vômiotos referidos em apenas cinco casos. Em 12 pacientes, o níve de albumina sérica foi inferior a 2,5 g/100 ml. com mediana de 1,7g/100 ml. A fraçäo gamaglobulina estava abaixo de 1,0 g/100 ml em oito pacientes e a linfocitopenia (linfócitos < 1500/mm**3) foi verificada em cinco casos. As provas de absorçäo mostraram um comprometimento da absorçäo de gordura, uma vez dioidomicose (cinco pacientes), linfangiectasia intestinal primária (um), estrongiloidíase (um), pericardite constrictiva (um), cardite reumática com dupla lesäo mitral (um), doença de Ménétriéer (um), síndrome de má-absorçäo por intestino curto (um), doença celíaca (um) e intolerância às proteínas do leite de vaca (um). Os mecanismos responsáveis pela perda excessiva de proteínas para a luz do tubo digestivo, bem como os aspectos diagnósticos e terapêuticos dos pacientes apresentados, säo comentados. E feito um relato de quatro casos que exemplificam...

Depuración plasmática de alfa-1-antitripsina, método simple para el estudio de la pérdida proteica intestinal / Plasma clearance of alpha 1-antitrypsin, a simple method for the study of intestinal protein losing

Tratamos de corroborar el valor del clearence de alfa-antitripsina como una técnica eficaz para objetivar la pérdida de proteínas a través del tubo digestivo y su aplicación como método de diagnóstico en tal patología. Se estudiaron 22 individuos; 11 con patología intestinal capaz de producir pérdida proteica y 11 controles sin daño de la mucosa intestinal. Se determinó la concentración de alfa-1-antitripsina tanto en suero como en materia fecal en días consecutivos y pesando la materia fecal de 24 horas se pudo obtener el clearence intestinal de dicha proteína. En los pacientes con enfermedad perdedora de proteínas el valor del clearence fue siempre patológico mientras que en los controles se obtuvieron valores normales. Este método es útil para diagnosticar la pérdida proteica intestinal y tiene la ventaja de no utilizar material radioactivo para su determinación