title: [Chronic intestinal pseudoobstruction due to visceral myopathy].
abstract: A case is reported of a chronic intestinal pseudoobstruction with lethal outcome in a 6-year-old boy. The clinical symptoms and radiology examination showed ileus without mechanical obstruction. During the observation the patient developed left sided mydriasis and grand mal seizures with lactacidosis. He was treated conservatively which included total parenteral nutrition, fluid-sodium supplements, intravenous erythromycin and somatostatin, correction of acidosis. On the 48th day he died suddenly of cardiac failure at the intensive care unit. The gastrointestinal and neurologic symptoms with lactacidosis suggested the possibility of mitochondrial myopathy. Postmortem histopathology showed visceral myopathy. Molecular genetic analysis could not confirm the presence of the mDNA mutation.
authors.affiliation: Petz Aladár Megyei Oktató Kórház, Csecsemo- és Gyermekgyógyászati Osztály, Gyor, Hungary. kovacmarti@freemail.hu
authors.forename: Márta
authors.initials: M
authors.lastname: Kovács
authors.forename: Gábor
authors.initials: G
authors.lastname: Veres
authors.forename: László
authors.initials: L
authors.lastname: Szônyi
authors.forename: Antal
authors.initials: A
authors.lastname: Dezsôfi
authors.forename: Hedvig
authors.initials: H
authors.lastname: Bodánszky
authors.forename: György
authors.initials: G
authors.lastname: Illyés
authors.forename: Zsuzsa
authors.initials: Z
authors.lastname: Schaff
authors.forename: András
authors.initials: A
authors.lastname: Arató
mesh_terms: D000140:Acidosis, Lactic / Q000209:etiology; D002648:Child; D002908:Chronic Disease; D016757:Death, Sudden, Cardiac / Q000209:etiology; D003937:Diagnosis, Differential; D004830:Epilepsy, Tonic-Clonic / Q000209:etiology; D017809:Fatal Outcome; D005769:Gastrointestinal Motility; D006801:Humans; D007415:Intestinal Obstruction / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D007418:Intestinal Pseudo-Obstruction / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D015878:Mydriasis / Q000209:etiology; D011859:Radiography