Colitis eosinofílica en pacientes pediátricos intervenidos de enfermedad de Hirschsprung / Eosinophilic colitis in children after surgery for Hirschsprung disease

Introducción: la colitis eosinofílica suele debutar con signos de enterocolitis, aunque hay casos descritos en los que se diagnostica tras episodios de obstrucción intestinal subaguda. Presentamos nuestra experiencia en niños diagnosticados de colitis eosinofílica que han sido intervenidos previamente de enfermedad de Hirschsprung. Resultados: 7 de los 44 pacientes intervenidos por enfermedad de Hirschsprung fueron diagnosticados de colitis eosinofílica. Mediana de edad de cirugía de enfermedad de Hirschsprung: tres meses; mediana de edad de colitis eosinofílica: 21 meses. Debutaron con diarrea mucosanguinolenta seis casos. Seis de siete niños presentaron cuadro de enteritis previo al diagnóstico de colitis eosinofílica y uno de siete rectorragia. El cribado infeccioso fue negativo en todos los casos. El diagnóstico de colitis eosinofílica mediante biopsia en todos los casos. Un paciente sin tratamiento, tres con fórmula elemental y solo dos con esteroides sistémicos (uno de ellos requirió cirugía finalmente). En todos los niños tratados se resolvió el cuadro, salvo en un caso que precisó instauración de nutrición parenteral y finalmente resección colónica con ileostomía. Conclusiones: en nuestra serie, hemos encontrado asociación entre enfermedad de Hirschsprung y colitis eosinofílica. En todos los casos se ha encontrado relación temporal entre ambas patologías, siendo siempre el diagnóstico de colitis eosinofílica posterior a la intervención quirúrgica por enfermedad de Hirschsprung. La exclusión de las proteínas de la leche de vaca y el empleo de antiinflamatorios han sido efectivos en la mayoría de los casos (AU)

Introduction: eosinophilic colitis tends to appear with signs of enterocolitis, although cases have been described after episodes of subacute intestinal obstruction. We present our experience in children diagnosed with eosinophilic colitis who had been previously operated for Hirschsprung disease. Results: 7 out of44 patients who underwent Hirschsprung disease were diagnosed with eosinophilic colitis. Median age of surgery for Hirschsprung disease was 3 months and the median age of diagnosis of eosinophilic colitis 21 months. Six cases debuted with diarrhea with blood. Six of seven children had enteritis prior to diagnosis of eosinophilic colitis and one of seven rectal bleeding. The infectious screening was negative in all cases.The diagnosis was performed by biopsy in all cases, compatible with eosinophilic colitis. Treatment: one patient did not require treatment, three children needed elemental formula and two patients systemic steroids (one of them finally required surgery). In all treated children the clinical picture was resolved, except in one case in which, given the persistence and impact of symptoms, required parenteral nutrition and finally colonic resection with ileostomy. Conclusions: In our series, we found some association between Hirschsprung disease and eosinophilic colitis. In all cases it was found a certain temporal relationship between the two diseases, always eosinophilic colitis after surgery for Hirschsprung disease. The exclusion of cow's milk proteins and the use of anti-inflammatory drugs have been effective in most cases (AU)

Enfermedad de Crohn metastásica en pediatría / Metastatic Crohn’s disease in pediatrics

Introducción: la enfermedad de Crohn metastásica (ECM) constituye una manifestación extraintestinal de la enfermedad de Crohn, siendo fundamental la biopsia para su diagnóstico. Existen referencias escasas a ECM en la edad pediátrica, y en adultos se estima una incidencia del 0,5-1%. No hay consenso sobre su abordaje terapéutico. Nuestro objetivo es describir nuestra experiencia diagnóstica y terapéutica en ECM. Caso clínico: se describen 4 casos de ECM en seguimiento en una Unidad de Gastroenterología Infantil en un hospital pediátrico de tercer nivel. Edades al diagnóstico entre 7 y 13 años. Las lesiones aparecieron antes del diagnóstico de enfermedad de Crohn (EC) en tres de ellos y durante la evolución de la enfermedad en otro. Localización genital en tres pacientes y en región pretibial bilateral en el otro. Todos demostraron granulomas no caseificantes en la biopsia. Dos pacientes precisaron únicamente nutrición enteral exclusiva, observándose resolución completa, mientras que otros dos recibieron terapias combinadas (corticoides, azatioprina, tacrolimus, infliximab y adalimumab) por recurrencia. Solo un caso requirió cirugía por mal control clínico. Discusión: la ECM es una entidad rara aunque siempre debemos incluirla en el diagnóstico diferencial de las lesiones cutáneas en enfermedad de Crohn, teniendo en cuenta que puede ser el debut de la enfermedad. Nos basaremos en la biopsia en cualquier caso para el diagnóstico definitivo. En esta serie se verifica la región genital como la más habitual en niños. El abordaje terapéutico no difiere del manejo de la afectación intestinal (AU)

Introduction and objectives: Metastatic Crohn’s disease (MCD) is an extraintestinal manifestation of Crohn’s disease, with biopsy as fundamental diagnostic tool. There are few references to MCD in children, with a 0.5-1% estimated incidence in adults. There is no consensus about its therapeutic approach. We describe our diagnostic and therapeutic experience in MCD. Results: Four cases of MCD are described in our Pediatric Gastroenterology Unit in a tertiary care hospital. The age at diagnosis was between 7 and 13 years. Lesions appeared before the diagnosis of Crohn’s disease in three of them, and during the course of the disease in another one, with genital location in three patients and bilateral pretibial region in the other. All four cases demonstrated non-caseificant granulomas on biopsy. Only two patients used exclusive enteral nutrition therapy with complete resolution, while other two cases received a combination of therapies (corticosteroids, azathioprine, tacrolimus, infliximab and adalimumab) because of recurrence. Only one case required surgery after poor clinical control. Conclusion: The MCD is infrequent but must always be included in the differential diagnosis of cutaneous lesions in Crohn’s disease, considering it could be the debut of the disease. We will rely on biopsy anyway for definitive diagnosis. In this series the genital region is verified as the most commonly affected in children. The therapeutic approach does not differ from the management of intestinal involvement (AU)

Metastatic Crohn's disease in pediatrics.

INTRODUCTION AND OBJECTIVES: Metastatic Crohn's disease (MCD) is an extraintestinal manifestation of Crohn's disease, with biopsy as fundamental diagnostic tool. There are few references to MCD in children, with a 0.5-1% estimated incidence in adults. There is no consensus about its therapeutic approach. We describe our diagnostic and therapeutic experience in MCD. RESULTS: Four cases of MCD are described in our Pediatric Gastroenterology Unit in a tertiary care hospital. The age at diagnosis was between 7 and 13 years. Lesions appeared before the diagnosis of Crohn's disease in three of them, and during the course of the disease in another one, with genital location in three patients and bilateral pretibial region in the other. All four cases demonstrated non-caseificant granulomas on biopsy. Only two patients used exclusive enteral nutrition therapy with complete resolution, while other two cases received a combination of therapies (corticosteroids, azathioprine, tacrolimus, infliximab and adalimumab) because of recurrence. Only one case required surgery after poor clinical control. CONCLUSION: The MCD is infrequent but must always be included in the differential diagnosis of cutaneous lesions in Crohn's disease, considering it could be the debut of the disease. We will rely on biopsy anyway for definitive diagnosis. In this series the genital region is verified as the most commonly affected in children. The therapeutic approach does not differ from the management of intestinal involvement.

Esophageal multichannel intraluminal impedance and pH testing in the study of apparent life threatening episode incidents in infants.

INTRODUCTION: The conventional 24-hour pH monitoring is the gold standard for the diagnosis of gastro-esophageal reflux (GER), a possible cause of Apparent Life Threatening Episodes (ALTE). However, multichannel intraluminal impedance (MII) may provide advantages. OBJECTIVES: Comparison of the results of MII and pH monitoring in patients undergoing MII-pH monitoring in the 3-year study period because of having suffered from ALTE. MATERIAL AND METHODS: Prospective study of MII-pH monitoring performed in our unit to infants < 12 months of ageadmitted for ALTE for a 3-year period. RESULTS: Thirty nine patients studied. 2,692 pH monitoring episodes, with median of 24 (IQ: 15-44) episodes/patient, 1.30 (IQ: 0.80-2.60) reflux/hour, 1 (IQ: 0-4) reflux episode > 5 min per patient and clearance of 1.20 (IQ: 0.70-2.20) min/reflux. With pH monitoring analysis, 14 children (35.9 %) could have been diagnosed as GER (8 mild, 4 moderate and 2 severe) based on the classical criteria. MII identified a total of 8,895 events; only 3,219 among them were refluxes, with a median of 75 (IQ: 54-111) per patient, 1.30 (IQ: 1.3-2.6) episodes/hour). With MII-pH monitoring combination there were 21.60 (SD 15.21) acid reflux episodes, 67.33 weekly acid (SD 32.09) and 3.34 (SD 7.23) non-acid, being finally diagnosed 33 patients as GER. CONCLUSIONS: The association of pH monitoring and MII provides additional information that improves GER diagnostic performance without posing any additional risk to the infant patient. The non-acid/weekly acid refluxes, not detected by pH monitoring, account for a high percentage of episodes, this may have diagnostic and therapeutic significance, especially in infants. Further studies are needed to assess the normality of MMI in pediatric patients.

Impedanciometría esofágica multicanal y pHmetría en el estudio del episodio de riesgo vital aparente del lactante / Esophageal multichannel intraluminal impedance and pH-testing in the study of apparent life threatening episode incidents in infants

Introducción: la pHmetría convencional de 24 horas constituye el patrón de oro para el diagnóstico de episodios de riesgo vital aparente (ALTE). La impedanciometría intraluminal multicanal (IIM) puede aportar ventajas en el lactante. Objetivos: comparación de los resultados de IIM y pHmetría en los pacientes sometidos a IIM-pHmetría en el periodo de estudio por causa de estudio de ALTE. Material y métodos: estudio retrospectivo de IIM-pHmetrías realizadas en nuestro servicio a lactantes < 12 meses de edad ingresados por ALTE durante tres años. Resultados: 39 pacientes estudiados. La pHmetría registró 2.692 reflujos, con medianas de 24 (IQ: 15-44) reflujos/paciente, índice de reflujo de 1,30 (IQ: 0,80-2,60) reflujos/hora, 1 (IQ: 0-4) episodio de reflujo > 5 min por paciente y aclaramiento de 1,20 (IQ: 0,70-2,20) min/reflujo. Por pHmetría 14 niños (35,9 %) fueron diagnosticados de RGE. Mediante IIM se registraron 8.895 eventos; 3.219 fueron reflujos, con mediana de 75 (IQ: 54-111) reflujos/ paciente, 1,30 (IQ: 1,3-2,6) reflujos/hora. Mediante IIM-pHmetría, la media de reflujos ácidos por paciente fue de 21,60 (DE 15,21), débilmente ácidos 67,33 (DE 32,09) y no ácidos 3,34 (DE 7,23), pudiéndose diagnosticar finalmente a 33 pacientes de RGE. Conclusiones: la asociación de IMM y pHmetría proporciona información adicional que mejora el rendimiento diagnóstico, sin suponer ningún riesgo adicional para el paciente. Los reflujos no ácidos/débilmente ácidos, los cuales no son detectados mediante pHmetría, suponen un porcentaje elevado, sobre todo en lactantes; esto tiene gran importancia diagnóstica en el estudio del ALTE. Aún son necesarios estudios para valorar la normalidad en IIM en pacientes pediátricos (AU)

Introduction: The conventional 24-hour pH monitoring is the gold standard for the diagnosis of gastro-esophageal reflux (GER), a possible cause of Apparent Life Threatening Episodes (ALTE). However, multichannel intraluminal impedance (MII) may provide advantages. Objectives: Comparison of the results of MII and pH monitoring in patients undergoing MII-pH monitoring in the 3-year study period because of having suffered from ALTE. Material and methods: Prospective study of MII-pH monitoring performed in our unit to infants < 12 months of age admitted for ALTE for a 3-year period. Results: Thirty nine patients studied. 2,692 pH monitoring episodes, with median of 24 (IQ: 15-44) episodes/patient, 1.30 (IQ: 0.80-2.60) reflux/hour, 1 (IQ: 0-4) reflux episode > 5 min per patient and clearance of 1.20 (IQ: 0.70-2.20) min/reflux. With pH monitoring analysis, 14 children (35.9 %) could have been diagnosed as GER (8 mild, 4 moderate and 2 severe) based on the classical criteria. MII identified a total of 8,895 events; only 3,219 among them were refluxes, with a median of 75 (IQ: 54-111) per patient, 1.30 (IQ: 1.3-2.6) episodes/hour). With MII-pH monitoring combination there were 21.60 (SD 15.21) acid reflux episodes, 67.33 weekly acid (SD 32.09) and 3.34 (SD 7.23) non-acid, being finally diagnosed 33 patients as GER. Conclusions: The association of pH monitoring and MII provides additional information that improves GER diagnostic performance without posing any additional risk to the infant patient. The non-acid/weekly acid refluxes, not detected by pH monitoring, account for a high percentage of episodes, this may have diagnostic and therapeutic significance, especially in infants. Further studies are needed to assess the normality of MMI in pediatric patients (AU)

Successful treatment for ulcerative proctitis with rectal tacrolimus in an 8-year-old girl with intolerance to mesalamine.

Ulcerative colitis (UC) is defined as a chronic inflammatory condition causing continuous mucosal inflammation of the colon without granulomas on biopsy. It affects the rectum, and, to a variable extent, the colon in continuity and is characterized by a relapsing and remitting course. Oral 5-aminosalicylic acid (5-ASA) regimens are recommended as first-line induction therapy for mild to moderately active pediatric UC and for maintenance of remission regardless of other initial treatments. In large clinical trials in adults, mesalamine intolerance was found in 2-5 % of the patients. We present a case of an 8-year-old female patient with intolerance to mesalamine and proctitis resistant to conventional therapy who responded to rectal tacrolimus treatment. The patient started with a dose of 2 mg/day at night with an excellent response. She reported feeling better than any of the previously prescribed treatments and without feeling the discomfort of previously administered enemas. After four weeks of treatment, the dose was reduced to 2 mg/week with no relapses. Tacrolimus suppositories were very well tolerated, and no adverse effects have been reported. Although only very little data has been published, rectal tacrolimus seems to be safe and of efficacy in ulcerative proctitis resistant to standard therapy.

Efficacy and safety of adalimumab in the treatment of Crohn’s disease in children / Eficacia y seguridad del adalimumab en el tratamiento de la enfermedad de Crohn infantil

Objectives: to describe the efficacy and safety of adalimumab (ADA) in inducing clinical remission and reducing inflammation of intestinal mucosa in children with Crohn’s disease (CD). Methods: we carried out a descriptive, observational study with all patients diagnosed with CD and treated with ADA between January 2007 and March 2013. Disease activity was determined using the Pediatric Crohn’s Disease Activity Index (PCDAI), and the degree of mucosa inflammation by fecal calprotectin (FC). Results: sixteen patients were included. Mean age at diagnosis was 10.6 ± 2.5 years, with a mean age at start of ADA treatment of 12.4 ± 1.8 years, and a median of 1.4 years (IQR 0.5-3) duration from CD diagnosis to start of treatment. Twelve patients were naïve to anti-TNF-a. The PCDAI score at start of ADA treatment was significantly reduced at 12 weeks of follow-up (31.25 IQR 26.8-37.5 vs. 1.2 IQR 0.0-5.0; p = 0.001). Similarly, the FC level decreased at 12 weeks (749 μg/g IQR 514-898 vs. 126 μg/g IQR 67.7-239.2; p = 0.02). Surgery was performed in 4 patients. Adverse events were reported in 4 patients. One patient developed lymphoma at 4 years of ADA treatment in monotherapy. Conclusions: ADA has been shown to be effective in children with moderate-to-severe CD. Treatment benefits should be weighed against side effects. Multicenter longitudinal studies with longer follow- up periods are required to determine the true efficacy and safety of long-term ADA treatment (AU)

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Efficacy and safety of adalimumab in the treatment of Crohn´s disease in children.

OBJECTIVES: to describe the efficacy and safety of adalimumab (ADA) in inducing clinical remission and reducing inflammation of intestinal mucosa in children with Crohn´s disease (CD). METHODS: we carried out a descriptive, observational study with all patients diagnosed with CD and treated with ADA between January 2007 and March 2013. Disease activity was determined using the Pediatric Crohn´s Disease Activity Index (PCDAI), and the degree of mucosa inflammation by fecal calprotectin (FC). RESULTS: sixteen patients were included. Mean age at diagnosis was 10.6 ± 2.5 years, with a mean age at start of ADA treatment of 12.4 ± 1.8 years, and a median of 1.4 years (IQR 0.5-3) duration from CD diagnosis to start of treatment. Twelve patients were naïve to anti-TNF-a. The PCDAI score at start of ADA treatment was significantly reduced at 12 weeks of follow-up (31.25 IQR 26.8-37.5 vs. 1.2 IQR 0.0-5.0; p = 0.001). Similarly, the FC level decreased at 12 weeks (749 µg/g IQR 514-898 vs. 126 µg/g IQR 67.7-239.2; p = 0.02). Surgery was performed in 4 patients. Adverse events were reported in 4 patients. One patient developed lymphoma at 4 years of ADA treatment in monotherapy. CONCLUSIONS: ADA has been shown to be effective in children with moderate-to-severe CD. Treatment benefits should be weighed against side effects. Multicenter longitudinal studies with longer follow-up periods are required to determine the true efficacy and safety of long-term ADA treatment.