Coagulopatía asociada a síndrome de malabsorción por enfermedad celíaca / Coagulopathy associated with celiac disease malabsorption syndrome

This article describes a 19-y-old patient with abdominal pain and signs of malnutrition. She had been treated previously with an antibiotic for chronic diarrhea. Laboratory analyses showed the presence mild hypoalbuminemia, and considerably prolonged prothrombin time. Tests revealed that hemostasis improved after the patient received fresh frozen plasma and vitamin k. A coagulation profile showed a decrease in clotting factors V, VII, IX, and fibrinogen. Positive serology (immunoglobulin A antitissue transglutaminase and immunoglobulin A antiendomysial antibodies) and small bowel mucosal histopathology confirmed the presence of celiac disease (CD). The girl recovered completely after she was put on a gluten-free diet. Vitamin K­deficiency is a rare complication that occurs in celiac disease manifestations. In addition to antibiotic therapy, treatment with other drugs that influence vitamin K resorption and metabolism may increase the risk of bleeding in patients with CD with hypoprothrombinemia.

Enfermedad por inclusión microvellositaria como causa de diarrea congénita severa: caso clínico / Microvillous inclusion disease as a cause of severe congenital diarrhea: case report

Las diarreas congénitas son patologías graves de baja frecuencia y alta mortalidad. Se manifiestan durante los primeros días o meses de vida con severa diarrea, generando insuficiencia intestinal y dependencia de nutrición parenteral. Se debe sospechar ante un recién nacido o lactante con pérdidas masivas hidroelectrolíticas, y se diagnostican utilizando parámetros clínicos, endoscópicos, histológicos y eventualmente genéticos. El tratamiento es de soporte, con reposición hidroelectrolítica intensa y nutricional. OBJETIVO: Presentar un caso de diarrea congénita, identificada como Enfermedad por Inclusión Microvellositaria, de presentación neonatal. CASO CLÍNICO: Paciente varón edad actual 3 años, hijo de padres consanguíneos, quien debutó a los 10 días de vida con diarrea secretora severa, requiriendo ingreso a unidad de paciente crítico y nutrición parenteral permanente. Inicialmente además con síndrome de Fanconi, que luego se recupera. Se confirmó la sospecha de Enfermedad de Inclusión Microvellositaria utilizando microscopia óptica, electrónica e inmunohistoquímica. Se obtuvo una favorable evolución utilizando nutrición parenteral total (NPT) a domicilio. CONCLUSIONES: Se presenta el primer caso conocido en Chile de un paciente con diarrea congénita por inclusión microvellositaria manejado y su evolución.

Congenital diarrheas correspond to a severe and low frequency digestive disease, with a high mortality. They start a few days or months after birth, leading to intestinal insufficiency and dependence on parenteral nutrition. It must be highly suspected in newborns or infants with diarrhea and severe electrolyte disorders. The diagnosis is based on clinical, endoscopic, histologic and eventually genetic findings. Treatment is supportive with intensive correction of electrolyte imbalances as well as parenteral nutrition. OBJECTIVE: To present a case report of congenital diarrhea identified as microvillous inclusion disease presenting in the neonatal period. CASE REPORT: Male patient currently 3 years of age, son of consanguineous parents. At 10 days of age presents a severe secretory diarrhea, requiring treatment in a critical care unit and parenteral nutrition. Initially he also presented with Fanconi syndrome, which improved afterwards. The suspicion of congenital microvillous inclusion was confirmed later by optic and electronic microscopy, and inmunohistochemistry. A succesful evolution was later achieved maintaining home parenteral nutrition after discharge. CONCLUSION: We present the first known case in Chile of congenital diarrhea due to microvillous inclusión disease and his evolution.

Detection of enteric viruses in pancreas and spleen of broilers with runting-stunting syndrome (RSS) / Detecção de vírus entéricos em pâncreas e baço de frangos com a síndrome de nanismo e raquitismo (RSS)

Enteric disease is a multifactorial problem in chickens, which causes gastrointestinal alterations, elevated feed conversions and impairment. In the last years, several enteric viruses were implicated in enteric disease; case reports have shown their presence alone or in concomitant infections during outbreaks and have suggested that they might be determining factors in the aetiology of enteric disease. This study shows high detection rates of enteric viruses in the pancreas and spleen in samples from an outbreak of enteritis and malabsorption in 16 chicken flocks (n=80 broilers). Avian nephritis virus (ANV) was the most ubiquitous virus, present in 75% of the flocks followed by avian rotavirus group A (ART-A) with 68.75%, and by chicken astrovirus (CAstV) and chicken parvovirus (ChPV) in 43.75% of samples. Viruses were present in the pancreas of positive flocks at extremely high rates: 100% for ART-A, 91.7% for ANV, 100% for CAstV and 57.14% for ChPV. By contrast, only 16.7% and 57.14% of intestine samples were positive for ANV and CAstV, respectively. Avian reovirus (AReo) and avian adenovirus group 1 (FAdV-1) were not detected. These results suggest that high viral detection rates in pancreas samples may be a result of viremia during enteric disease, with subsequent damage of the exocrine pancreas, leading to runting-stunting syndrome (RSS).(AU)

A doença entérica é um problema multifatorial em galinhas que causa alterações gastrointestinais, conversão alimentar elevada e deficiência de crescimento. Nos últimos anos, os vírus entéricos foram associados à doença entérica; casos reportados mostraram a infecção de um único vírus e também infecções concomitantes durante os surtos sugerindo a presença de múltiplos fatores etiológicos nas doenças entéricas. Este estudo mostra uma alta taxa de detecção dos vírus entéricos em amostras de pâncreas e baço de um surto de enterite e má-absorção em 16 lotes de frangos (n=80 frangos). O vírus de nefrite aviária (ANV) foi o vírus mais detectado, estando presente em 75% dos lotes seguido pelo rotavírus aviário grupo A (ART-A) em 68,75% dos casos, e pelo astrovirus (CAstV) e parvovírus aviários (ChPV), ambos em 43,75% das amostras. Os vírus estavam presentes no pâncreas dos lotes positivos em percentuais elevados: 100% para ART-A e CAstV; 91,7% para ANV, e em 57,14% para ChPV. Em contraste, somente 16,7% e 57,14%, em amostras de intestino, foram positivos para ANV e CAstV, respectivamente. Reovírus aviário (AReo) e o adenovírus do grupo 1 (FAdV-1) não foram detectados. Estes resultados sugerem que os elevados percentuais de vírus detectados em amostras de pâncreas podem estar associados à viremia durante a doença entérica, com subsequente lesão no pâncreas exócrino das aves levando ao desenvolvimento da síndrome de nanismo e raquitismo.(AU)

Fructose malabsrrption in children with functional digestive disordrss / Ma absorcao a frutose em criancas com transtornos digestivos funcionais

Context Fructose is a monosaccharide frequently present in natural and artificial juice fruits. When the concentration of fructose in certain food is present in excess of glucose concentration some individuals may develop fructose malabsorption. Objectives To report the frequency of fructose malabsorption utilizing the hydrogen breath test in children with gastrointestinal and/or nutritional disorders. Methods Between July 2011 and July 2012, 43 patients with gastrointestinal and/or nutritional disorders, from both sexes, were consecutively studied, utilizing the hydrogen breath test with loads of the following carbohydrates: lactose, glucose, fructose and lactulose. Fructose was offered in a 10% aqueous solution in the dose of 1 g/kg body weight. Samples were collected fasting and at every 15 minutes after the intake of the aqueous solution for a 2 hour period. Malabsorption was considered when there was an increase of >20 ppm of hydrogen over the fasting level, and intolerance was diagnosed if gastrointestinal symptoms would appear. Results The age of the patients varied from 3 months to 16 years, 24 were boys. The following diagnosis were established: irritable bowel syndrome with diarrhea in 16, functional abdominal pain in 8, short stature in 10, lactose intolerance in 3, celiac disease in 1, food allergy in 1 and giardiasis in 1 patient. Fructose malabsorption was characterized in 13 (30.2%) patients, and intolerance in 1 (2.3%) patient. The most frequent fructose malabsorption was characterized in 7 (16.3%) patients with irritable bowel syndrome and in 4 (9.3%) patients with functional abdominal pain. Conclusions Patients with irritable bowel syndrome and functional abdominal pain were the main cause of fructose malabsorption. .

Contexto Frutose é um monossacarídeo frequentemente presente em sucos de frutas naturais e artificiais. Quando a concentração de frutose em determinados alimentos está presente em excesso de glicose, alguns indivíduos podem apresentar má absorção à frutose. Objetivo Descrever a freqüência de má absorção à frutose utilizando o teste do hidrogênio no ar expirado em crianças com transtornos digestivos e/ou nutricionais. Métodos Durante o período compreendido entre julho de 2011 e julho de 2012 foram investigados de forma consecutiva 43 pacientes, de ambos os sexos, com suspeita de má absorção, por meio do teste hidrogênio no ar expirado com sobrecarga dos seguintes carboidratos: lactose, glicose, frutose e lactulose. A frutose foi administrada em solução aquosa 10% à dose de 1 g/kg de peso. Foram obtidas amostras em jejum e, após a ingestão da solução, a cada 15 minutos durante 2 horas. Foi considerada má absorção quando houve incremento >20 ppm de hidrogênio no ar expirado em relação ao jejum, e intolerância caso surgissem sintomas após a sobrecarga. Resultados A idade dos pacientes variou de 3 meses a 16 anos, 24 meninos. Foram estabelecidos os seguintes diagnósticos: síndrome do intestino irritável com diarréia 16, dor abdominal funcional 8, baixa estatura 10, intolerância à lactose 3, doença celíaca 1, alergia alimentar 1 e giardíase 1. Má-absorção à frutose foi caracterizada em 13 (30,2%) pacientes; observou-se intolerância em 1 (2,3%) deles. Síndrome do intestino irritável com diarréia em 7 (16,3%) e dor abdominal funcional em 4 (9,3%) pacientes foram os transtornos com maior prevalência ...

Respuesta a la vacunación contra hepatitis B en pacientes pediátricos con enfermedad celíaca / Hepatitis B vaccination in children with celiac disease, inmunological response

Determinar la respuesta inmunológica a la vacunación contra virus de hepatitis B en pacientes pediátricos con enfermedad celíaca. Se revisaron 25 historias clínicas de celíacos diagnosticados entre 2001-2010 por estudios serológico, histopatológicos, genético y esquema de vacunación hepatitis B completo, de los cuales se excluyeron 11 pacientes. Se solicitó anticuerpo contra antígeno de superficie virus hepatitis B. El grupo de celíacos no respondedores fue evaluado posterior a revacunación bajo apego a dieta sin gluten. Se estudiaron 14 pacientes (35,7% hembras, 64,3% varones). 8 pacientes se vacunaron al nacer y 6 después de los 8 años de edad. De los cuales 28,6% tuvieron baja respuesta a la vacunación y 71,4% sin respuesta. La respuesta fue positiva en 3/8 (37,5%) de los pacientes vacunados al nacer y en 1/6 de los mayores de 8 años (16,7%). 4 no respondedores fueron revacunados cumpliendo dieta libre de gluten, evidenciándose respuesta inmunológica positiva en el 75%. Existe una disminución en la respuesta a la vacunación contra hepatitis B en pacientes celíacos, asociado a HLA DQ2 y que la dieta libre de gluten puede mejorar la respuesta inmunológica

To determine the immune response to the vaccination against hepatitis B virus in pediatric patients with celiac disease. Reviewed 25 medical histories of celiac diagnosed between 2001-2010 by serological, histopathological, genetic studies and full hepatitis B vaccination scheme, of which 11 patients were excluded. Also ask antibody against hepatitis B virus surface antigen The group of non-responder celiac was evaluated after revaccination under attachment to diet without gluten. 14 Patients were studied (35.7% females, 64.3% boys). 8 patients vaccinated at birth and 6 after eight years of age. Of which 28.6% had low response to vaccination and 71.4% unanswered. The answer was positive in 3/8 (37.5%) of patients vaccinated at birth and 1/6 of the 8 older (16.7%). not answering. 4 were fulfilling gluten-free diet, showing response immunological positive in 75%. There is a decrease in the response to vaccination against hepatitis B in celiac patients, conditional primarily by HLA DQ2 and that the gluten-free diet can improve the immune response

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.

Estudio comparativo de dos equipos para el test de H2 en aire espirado / Evaluation of breath H2 measured by means of two different analyzers

El test de hidrógeno (H2) en aire espirado es ampliamente utilizado en el estudio de malabsorción de hidratos de carbono, sobrecrecimiento bacteriano intestinal (SBI) y tiempo de tránsito orocecal (TTOC). Objetivo: Comparar los resultados obtenidos por dos equiposde detección de H2, uno de ellos de introducción reciente en nuestro medio. Material y Métodos: 50 pacientes, edad promedio 38,5 +/- 5,2 años (rango 7-77 años), 40 mujeres, se les realizó el test de H2 en aire espirado en paralelo con ambos equipos bajo métodos estandarizados. En 25 de ellos se investigó la presencia de malabsorción de lactosa, y en los otros 25, la presencia de SBI con lactulosa. Se evaluaron los valores de H2 obtenidos con ambos equipos. Resultados: Las lecturas de H2 con ambos equipos no mostraron diferencias significativas tanto para lactosa (p > 0,1), como para lactulosa (p > 0,5). Tampoco hubo diferencias en el TTOC de los pacientes (90 +/- 33,75 min v/s 90.8 +/- 32,42 min) (p > 1). Se obtuvo un índice de concordancia Kappa de 0,92 entre ambos equipos con el test con lactosa y con lactulosa. Conclusión: El equipo portátil es altamente confiable, entregando resultados con una excelente concordancia con respectoal equipo de referencia.

The hydrogen (H2) breath test (BT) is widely used to investigate carbohydrates malabsorption, small intestinal bacterial overgrowth (SIBO) and orocaecal transit time (OTT). Aim: To compare the results of two hydrogen breath devices, one of them, of recent introduction in our country. Methods: Fifty patients were included, mean age 38.5 +/- 5.2years (range 7-77 yrs), 40 women, H2 BT was performed in parallel with both analyzers under standardized methods. Lactose malabsorption was investigated in 25 patients with lactose, and the presence of SIBO in the resting 25 patients, with lactulose, hydrogen values obtained with both devices were evaluated. Results: No differences were observed between the H2 concentrations for lactose BT (p > 0.1) as well as lactulose BT (p > 0.5)and also between the OTT measured by the two devices (90 +/- 33.75 min. v/s 90.8 +/- 32.42 min) (p > 1). We detected a Kappa concordance index of 0.92 for both equipments. Conclusion: The portable device is highly reliable to detect the presence of breath hydrogen, giving results with an excellent concordance to the reference device.

Selective amyloidosis of the small intestine presenting as malabsorption syndrome.

Amyloidosis is a unique metabolic storage disease that affects the gastrointestinal tract but rarely causes severe symptoms. We describe the case of a 51-year-old woman presenting with chronic small bowel diarrhoea and malabsorption. Barium contrast studies and enteroscopy revealed a coarse mucosal pattern and thickening of the jejunal and proximal ileal folds. Histological examination revealed massive deposits of amyloid in thejejunal wall. No predisposing factor was found and there were no other sites of deposition. This is a rare case report of primary selective small intestinal amyloidosis manifesting as the malabsorption syndrome.

Como avaliar paciente com má-absorção intestinal? / How evaluate patient with intestinal malabsorption?

Tendo como base uma revisão de literatura, esse artigo visa relatar manifestações clínicas e complicações de má-absorção, bem como descrever testes que podem ser usados para avaliar a função digestiva e absortiva e fornecer uma hipótese diagnóstica apropriada para cada paciente.

Diagnóstico de los síndromes de malabsorción / Malabsorption syndrome diagnosis

Los síndromes de malabsorsión son una de las principales causas de diarrea crónica de tipo entérico y con compromiso del estado general. Los tres principales síndromes de malabsorción son la enfermedad celíaca; la insuficiencia pancreática exocrina y los síndromes de asa ciega con proliferación de la flora intestinal y colonización del intestino delgado. La sospecha diagnóstica inicial de malabsorción es clínica y luego se comprueba mediante la demostración de esteatorrea y con el test de absorsión de D-xylosa. El diagnóstico se confirma finalmente mediante el estudio histológico de la biopsia de intestino delgado, que revela la existencia de atrofia vellositaria y microvellositaria. Ultimamente han adquirido importancia los exámenes inmunológicos (anticuerpos antiendomisio), los que son altamente sensibles y específicos. El tratamiento es propio de cada variedad fisiopatológica de malabsorción y consiste en: - Régimen sin gluten (enfermedad celíaca)- Antibióticos (síndrome de asa ciega)- Pancreatina o extractos pancreáticos (insuficiencia pancreática exocrina).

Chronic diarrhea and malabsorption caused by Leishmania donovani.

Chronic diarrhea and malabsorption are uncommon in immunocompetent patients with visceral leishmaniasis. We report two immunocompetent patients with visceral leishmaniasis where the predominant presentation was chronic diarrhea. One of them had clinically overt malabsorption and duodenal mucosa was loaded with Leishmania donovani bodies. The other patient had diffuse colonic aphthous and discrete ulcerations and Leishmania donovani bodies were seen in the crush smears of the colonic mucosa. With amphotericin B, there was reversal of malabsorption and healing of colonic ulcers.

Pseudomalabsorcão de levotiroxina: relato de um caso / Pseudomalabsorption of levothyroxine: a case report

Uma mulher de 49 anos, em tratamento de hipotireoidismo com levotiroxina, após diagnóstico de tireoidite de Hashimoto e tireoidectomia total para bócio multinodular atóxico, foi avaliada devido a hipotireoidismo persistente apesar do uso de altas doses de levotiroxina (600æg/dia). Clinicamente, a paciente apresentava sinais e sintomas de hipotireoidismo, e os exames laboratoriais mostravam tiroxina livre de 0,20ng/dL; hormônio tireoestimulante de 351æUI/mL; triiodotironina total de 27ng/dL. Foi confirmada a não aderência ao tratamento e considerados os diagnósticos de sindrome de Munchausen e transtorno factício, levando ao quadro de pseudomalabsorcão de levotiroxina.

Síndrome de malabsorción intestinal provocado por Strongyloides stercoralis / Intestinal malabsorption syndrome cause by Strongyloides stercoralis

Son varios los cuadros clínicos producidos por el Strongyloides stercoralis, desde infestación subclínica hasta una hiperinfestación que puede ser mortal. El aparato digestivo es el asiento más importante de la infestación, el duodeno y el yeyuno son los más gravemente afectados y la magnitud de trastorno va desde leve edema de la mucosa hasta la infrecuente enteritis hemorrágica mortal. Se presenta un paciente de 30 años de edad que ingresa a Terapia Intensiva con diagnóstico clínico, electromiográfico y por líquido de síndrome de Guillain Barré, que presentara desde su ingreso diarreas profusas; observándose en un exámen parasitológico de materia fecal, abundantes larvas de Strongyloides stercoralis. Recibió tratamiento específico para el mismo, aunque incompleto. Varios estudios confirmaron la presencia de Síndrome de Malabsorción, que obligó el uso prolongado de alimentación parenteral total. En su evolución presentó complicaciones infecciosas, desnutrición calórica protéica severa y un cuadro peritoneal agudo que obligó a una laparotomía exploradora de urgencia, hallándose extensa necrosis ilial de aproximadamente 1,2 metros extensión. La biopsia demostró enteritis aguda hemorágica parasitaria, con presencia de abundantes larvas y vermes adultos de Strongyloides stercolaris...

Endoscopía por cápsula / Endoscopy by capsule

Los avances tecnológicos también involucran al área endoscópica, mediante el desarrollo de una cápsula con capacidad de registrar imágenes de gran resolución en el intestino delgado. Las principales indicaciones de este procedimiento incluyen hemorragia digestiva oculta y casos de diarrea crónica con malabsorción, anemia y enfermedad inflamatoria intestinal (Enfermedad de Crohn), entre otras. Los estudios actuales están comparando el rendimiento de la endoscopía por cápsula contra enteroscopía, para la detección de angiodisplasia, tumores, pólipos y detalles de la superficie mucosa. Esta revisión describe aspectos técnicos y clínicos relacionados a su potencial aplicación en la práctica médica

Absorção intestinal de D-xilose em crianças infectadas pelo vírus da imunodeficiência humana / Intestinal absorption of D-xylose in children infected with the human immunodeficiency virus

AIM: To evaluate the intestinal absorption in HIV-infected children children 14 months to 14 years and to investigate its relationship to diarrhea, nutritional status, immune dysfunction, classical enteric parasites and Cryptosporidium. METHODS: Intestinal absorption was investigated by measuring serum D-xylose. Fecal samples were investigated for classical pathogens and Cryptosporidium. The sample size was calculated considering a 30% prevalence of altered D-xylose absorption in HIV-infected children with a 5% accuracy. Statistical procedures used were: descriptive measurements, multiple correspondence analysis and logistic regression. RESULTS: D-xylose absorption was altered in only 8 out of 104 (7.7%) and Cryptosporidium was positive in 33 out of 104 (31.73%) HIV-infected children. The multiple correspondence analysis suggested an association between an altered D-xylose test and Cryptosporidium. D-xylose malabsorption was not associated with diarrhea, nutritional status, immune disfunction and classic enteric parasites. CONCLUSIONS: Intestinal malabsorption evaluated through the D-xylose test was an uncommon finding in HIV-infected children. Intestinal dysfunction when present seems to be related to Cryptosporidium, but not to diarrhea, nutritional status, immune disfunction and classic enteric parasites

Intolerancia a lactosa en el niño. Antecedentes / Lactose intolerance in children. A historical review

Se hace una revisión de los primeros trabajos sobre intolerancia a la lactosa en niños con gastroenteritis realizados en México. En 1963 en el Hospital de Pediatría del Centro Médico Nacional del IMSS se investigó la presencia de azúcares reductoras en heces. En 1968 se establecieron pautas para el diagnóstico y tratamiento de la intolerancia a la lactosa en niños con gastroenteritis y se diseñaron fórmulas con harina de soya, carne de pollo y caseinato de calcio para sustituir la fórmula láctea. Se confirmó la utilidad de las cintas y tabletas reactivas y curvas de tolerancia a la lactosa en el diagnóstico de intolerancia a la lactosa en el diagnóstico de intolerancia a los carbohidratos. En 1979 se realizó por primera vez la determinación de hidrógeno espirado como prueba diagnóstica de intolerancia a la lactosa y se realizaron diversos estudios metabólicos, uno de ellos comparando proteínas de soya y garbanzo demostrándose igual valor nutricio. Se comenta sobre las ventajas de las fórmulas lácteas sin lactosa