{"orpha":487809,"name":"Pediatric collagenous gastritis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Childhood-onset collagenous gastritis"],"definition":"A rare gastroenterologic disease characterized by the histopathological finding of a thickened (> 10 µm) gastric subepithelial collagen layer in association with an inflammatory infiltrate in the lamina propria. Patients typically present with upper abdominal pain and severe iron deficiency anemia. The condition is not commonly associated with autoimmune diseases, and involvement of the colon is less frequent than in the adult form. The disease takes a generally benign course with limited long-term morbidity and no increased mortality.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-487809/","orphanet_url":"https://www.orpha.net/en/disease/detail/487809","names":{"fr":{"name":"Gastrite collagène pédiatrique","definition":"Maladie gastroentérologique rare caractérisée par un examen histopathologique mettant en évidence une couche de collagène sous-épithéliale gastrique épaissie (> 10 µm) en association avec un infiltrat inflammatoire dans la lamina propria. Les patients présentent généralement des douleurs dans la partie supérieure de l'abdomen et une anémie sévère due à une carence en fer. Généralement, la maladie n'est pas associée à des maladies auto-immunes, et l'atteinte du côlon est moins fréquente que dans la forme adulte. La maladie évolue généralement de manière bénigne, avec une morbidité limitée à long terme et sans augmentation de la létalité."},"de":{"name":"Gastritis, kollagene, pädiatrische","synonyms":["Kollagene Gastritis des Kindes"],"definition":"Eine seltene gastroenterologische Erkrankung, die durch den histopathologischen Befund einer verdickten (> 10 µm) subepithelialen Kollagenschicht des Magens in Verbindung mit einem entzündlichen Infiltrat in der Lamina propria gekennzeichnet ist. Die Patienten zeigen typischerweise Oberbauchschmerzen und eine schwere Eisenmangelanämie. Die Erkrankung wird nicht häufig mit Autoimmunerkrankungen in Verbindung gebracht, und der Dickdarm ist seltener betroffen als bei der Erwachsenenform. Die Krankheit nimmt im Allgemeinen einen gutartigen Verlauf mit begrenzter Langzeitmorbidität und keiner erhöhten Mortalität."},"es":{"name":"Gastritis colagenosa pediátrica","synonyms":["Gastritis colagenosa de inicio en la infancia"],"definition":"Es una enfermedad gastroenterológica poco frecuente caracterizada por el hallazgo histopatológico de un engrosamiento de la capa de colágeno gástrica subepitelial (> 10 µm) asociado a un infiltrado inflamatorio en la lámina propia. Los pacientes se suelen presentar con dolor abdominal superior y anemia grave por deficiencia de hierro. El trastorno, por lo general, no está asociado a enfermedades autoinmunes y la afectación del colon ocurre con menor frecuencia que en la forma adulta. La enfermedad sigue un curso típicamente benigno con una morbilidad limitada a largo plazo y sin un aumento de la mortalidad."},"it":{"name":"Gastrite collagenosa pediatrica","definition":"Si tratta di una malattia gastroenterologica rara, caratterizzata, dal punto di vista istopatologico, da uno strato ispessito di collagene sotto l'epiteliale gastrico (>10 µm) associato ad un infiltrato infiammatorio nella lamina propria. Di solito i pazienti presentano dolore nella parte superiore dell'addome e grave anemia da deficit di ferro. In genere non si associa a malattie autoimmuni e il coinvolgimento del colon è più raro rispetto alla forma dell'adulto. Di solito la malattia ha un decorso benigno, con una morbilità a lungo termine limitata e nessun aumento della mortalità."},"nl":{"name":"Pediatrische collagene gastritis","synonyms":["Collagene gastritis met aanvang in de kindertijd"],"definition":"Een zeldzame gastro-enterologische aandoening, gekarakteriseerd door de histopathologische bevinding van een verdikte (meer dan 10 micrometer) subepitheliale collageenlaag van maag, geassocieerd met een inflammatoir infiltraat in lamina propria. Patiënten presenteren zich doorgaans met pijn in bovenbuik en ernstige anemie door ijzerdeficiëntie. Associatie met auto-immuunziekten is niet gangbaar, en betrokkenheid van colon komt minder vaak voor dan bij de volwassen vorm. De ziekte kent een overwegend goedaardig verloop met beperkte langdurige morbiditeit en geen verhoogde mortaliteit."},"pt":{"name":"Gastrite colagenosa pediátrica"},"pl":{"name":"Zapalenie kolagenowe żołądka wieku dziecięcego","synonyms":["Zapalenie kolagenowe żołądka o początku w wieku dziecięcym"]},"cs":{"name":"Dětská kolagenní gastritida","synonyms":["Kolagenní gastritida s nástupem v dětství"]},"uk":{"name":"Колагеновий гастрит у дітей","synonyms":["Колагеновий гастрит у дитячому віці"],"definition":"A rare gastroenterologic disease characterized by the histopathological finding of a thickened (> 10 µm) gastric subepithelial collagen layer in association with an inflammatory infiltrate in the lamina propria. Patients typically present with upper abdominal pain and severe iron deficiency anemia. The condition is not commonly associated with autoimmune diseases, and involvement of the colon is less frequent than in the adult form. The disease takes a generally benign course with limited long-term morbidity and no increased mortality."},"zh":{"name":"儿童胶原性胃炎","synonyms":["Childhood-onset collagenous gastritis"]},"tr":{"name":"Pediatrik kollajenöz gastrit","synonyms":["Çocuklukta başlayan kollajenöz gastrit"]}},"xrefs":[{"source":"ICD-10","code":"K29.6","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DA42.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0044624","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680109","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97935,"name":"Rare gastroenterologic disease"},"parents":[{"orpha":101936,"name":"Rare gastroesophageal disease","classifications":[152]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Childhood","Infancy"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":24.0,"area":"Worldwide","status":"Validated","source":"23538318[PMID]_25789098[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_25789098[PMID]"}],"phenotypes":null}