Urinary and gastrointestinal malakoplakia in a 12-year-old girl.

Malakoplakia is a rare chronic inflammatory disease that was originally described in the urinary bladder but can involve many other organs and soft tissues. It is believed to be caused by an alteration in the bacterial phagocytic system. Clinically, it is described as single or multiple tumors that can appear in any part of the body. Histologically, the presence of Michaelis-Gutmann bodies is pathognomonic. Malakoplakia in children is rare. Few pediatric cases in the urinary tract, kidney, or gastrointestinal tract have been published. We present a case of urinary and gastrointestinal malakoplakia in a 12-year-old girl.

Safety and efficacy of granulocyte and monocyte adsorption apheresis in paediatric inflammatory bowel disease: a prospective pilot study.

OBJECTIVE: Selective granulocyte-monocyte adsorption (GMA) apheresis is a safe technique that has shown efficacy in inflammatory bowel disease (IBD), especially in adult steroid-dependent and steroid-refractory ulcerative colitis. GMA apheresis is performed with Adacolumn, a direct blood perfusion system that selectively adsorbs circulating granulocytes and monocytes. Studies on efficacy of GMA apheresis in paediatric IBD are scarce. Our aim was to evaluate efficacy, safety, and tolerability of GMA apheresis in paediatric IBD patients followed for 1 year. PATIENTS AND METHODS: Nine patients with a mild to moderate flare-up (6 boys, 3 girls; 5 ulcerative colitis [UC], 4 Crohn disease [CD]) were included. Mean age at inclusion was 13 years and 9 months, and mean disease duration before inclusion was 28 months. All of our patients with UC were steroid-dependent; patients with CD had been unsuccessfully treated with other therapies. GMA apheresis consisted of 5 consecutive weekly sessions lasting 60 minutes each. RESULTS: After the 5 sessions, 4 of 5 patients with UC and 1 of 4 patients with CD achieved remission. This remission was maintained in 2 of 4 patients with UC and in the single patients with CD. Patients taking steroids could begin to taper their daily doses after the second apheresis, and 3 of 5 of these patients reached the end of the study steroid-free. GMA apheresis was well tolerated and no severe side effects related to the technique were observed. CONCLUSIONS: GMA apheresis is a safe, well-tolerated technique in paediatric IBD. As previously reported, we have observed a better efficacy in promoting and maintaining remission, and reducing conventional drugs in patients with UC than in patients with CD.

Solitary rectal ulcer syndrome in childhood: a rare, benign, and probably misdiagnosed cause of rectal bleeding. Report of three cases.

Solitary rectal ulcer syndrome is an uncommon and often underdiagnosed condition that usually presents with hematochezia, mucous discharge, and tenesmus. Its etiology is unknown but it seems related to excessive straining with defecation. Prolonged efforts force the anterior rectal mucosa into the anal canal with strangulation and appearance of congestion, edema, and ulceration. Histologic findings (fibromuscular obliteration of lamina propria and disorientation of muscle fibers) are characteristic, which helps to differentiate these lesions from other rectal entities. Although solitary rectal ulcer syndrome is rarely reported in children, it must be suspected in patients with rectal discharge of blood and mucus and previous disorders of evacuation. We present three children (aged 9, 10, and 14 years) with solitary rectal ulcer syndrome that had presented with rectal bleeding. A careful inquiry about evacuation habits and a high index of suspicion in children presenting with hematochezia helps to diagnose this possibly unrecognized or misdiagnosed entity in children. Endoscopy and histologic examination confirms this condition.

Infliximab in paediatric inflammatory bowel disease.

Infliximab has been widely used in paediatric Crohn's disease, mainly in luminal and fistulous disease refractory to standard treatment and for extraintestinal manifestations. Moreover, there is growing experience with its use in refractory ulcerative colitis. Infliximab has shown similar efficacy and safety in children as in adult population. It is postulated that its early use in paediatric inflammatory bowel disease, as a bridging treatment until the onset of action of other immunomodulators, could reduce the use of steroids and change the natural history of the disease as well. The effect of infliximab on mucosal healing could also contribute to the normal growth and sexual maturation in these patients.

Rectocolitis a proteïnes de llet de vaca / Rectocolitis a proteínas de leche de vaca / Proctocolitis secondary to intolerance to cow's milk protein

Introducció. La proctocolitis és una causa de rectorràgia en lactants inclosa dins les reaccions adverses a proteïnes alimentàries. L’objectiu d’aquest treball és revisar de manera prospectiva l’evolució en un grup de lactants amb aquesta sospita clínica. Mètode. Estudiar de manera prospectiva un grup de lactants visitats en el nostre centre amb clínica de rectorràgia i sospita de rectocolitis analitzant clínica, analítica i endoscòpia. Resultats. Vam visitar un total de nou pacients, dos dels quals van ser diagnosticats de colitis infecciosa i colitis ulcerosa, respectivament. La clínica es va manifestar amb rectorràgies acompanyades de bon estat general. La majoria dels restants (5/7) prenien lactància materna en el moment del debut. A tots se’ls va fer un estudi analític bàsic i una cerca de gèrmens en el coprocultiu. No van presentar anèmia (excepte un pacient), ni eosinofília perifèrica, ni hipoalbuminèmia. El diagnòstic es va fer sobre la base d’una bona història clínica i una hipòtesi diagnòstica. També se’ls va fer rectoscòpia amb presa de biòpsies en les quals es mostraven unes lesions característiques en la histologia (>20 eosinòfils per camp), però no patognomòniques. Conclusions. La rectocolitis a proteïnes de llet de vaca constitueix una entitat clínica pròpia que el pediatre general ha de reconèixer i incloure dins del diagnòstic diferencial dels lactants amb rectorràgia. Té una evolució clínica i una resolució excel•lents, i la rectoscòpia i la presa de biòpsies són necessàries per fer-ne la confirmació (AU)

Introducción. La proctocolitis es una causa de rectorragia en los lactantes, cuya naturaleza se encuentra dentro de las reacciones adversas a proteínas alimentarias. El objetivo del presente trabajo es revisar de forma prospectiva la evolución en un grupo de lactantes con sospecha de esta entidad. Método. Estudiar prospectivamente a un grupo de lactantes llegados a nuestro servicio con clínica de rectorragia y sospecha de rectocolitis analizando clínica, analíticas y endoscopia. Resultados. Visitamos a un total de nueve pacientes, dos de los cuales fueron diagnosticados de colitis infecciosa y colitis ulcerosa, respectivamente. La clínica se manifestó con rectorragias y buen estado general. La mayoría de los restantes (5/7) recibían lactancia materna en el momento del debut. En todos se realizó estudio analítico básico y búsqueda de gérmenes en los coprocultivos. No presentaron anemia (excepto un paciente), ni eosinofilia periférica, ni hipoalbuminemia. El diagnóstico se realizó en base a una buena historia clínica y una hipótesis diagnóstica. En todos se realizó rectoscopia con toma de biopsias, mostrando unas lesiones características en la histología (>20 eosinófilos por campo), pero no patognomónicas. Todos realizaron una dieta de exclusión materna de proteínas vacunas y suplementos con leches especiales (tres con hidrolizado y tres con fórmula elemental). Sólo uno mejoró con dieta de exclusión materna. La reintroducción se realizó sin problemas a partir del año de edad. Conclusiones. La rectocolitis a proteínas de leche de vaca constituye una entidad clínica propia que el pediatra general debe reconocer e incluir dentro del diagnóstico diferencial de los lactantes con rectorragia. Su evolución clínica y su resolución es excelente, y es conveniente y necesaria la rectoscopia y la toma de biopsias para su confirmación (AU)

Background. Proctocolitis is one of the causes of rectal bleeding in infants, and it is probably related to food protein intolerance. We prospectively reviewed the clinical course and outcome of a group of infants with rectal bleeding and suspected cow's milk protein intolerance. Method. We performed a prospective evaluation of a group of patients with rectal bleeding. We analyzed the clinical presentation, laboratory evaluation, and colonoscopy findings. Results. The group includes 9 infants; two of them were eventually diagnosed with infectious colitis and ulcerative colitis and were excluded from further analysis. Clinical presentation was characterized by rectal bleeding and a good general condition. Five of the 7 patients were breastfed at the time of presentation. Infectious etiology was excluded in all patients. One patient had mild anemia, and none of the patients presented with eosinophilia or hypoproteinemia. All patients underwent a rectoscopy with biopsy, and pathology showed eosinophilic infiltrates, suggestive of protein intolerance. All patients responded well to maternal restriction of cow protein and 6 patients also required supplementation with hydrolyzed (3 patients) or elemental (3 patients) formulas. After 12 months, cow's milk reintroduction was performed successfully in all cases. Conclusions. Food protein intolerance is one of the most common causes of rectal bleeding in infants, and this diagnosis should be included in the differential diagnosis of infants with bloody stools. Colonoscopy and biopsy are necessary for the diagnosis. Clinical course and outcome are excellent after exclusion of the offending protein (AU)

Implicació del reflux gatroesofàgic en els episodis aparentment letals / Gastroesophageal reflux and apparently lethal episodes

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El reflux gastroesofágic

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