{"orpha":90069,"name":"Systemic monochloroacetate poisoning","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Systemic monochloroacetate poisoning is a rare, life-threatening intoxication with monochloroacetic acid (mainly through the skin, but also by inhalation or ingestion). It is characterized by vomiting, diarrhea and central nervous system (CNS)-excitability (disorientation, delirium, convulsions) as early signs of systemic poisoning, followed by CNS-depression, coma and cerebral edema. Additional signs include heart involvement (severe myocardial depression, shock, arrhythmias, nonspecific myocardial damage), severe metabolic acidosis, hypokalemia, hypocalcemia and progressive renal failure leading to anuria. Myoglobinemia and leukocytosis may occur. Manifestations may be delayed for 1-4 hours.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90069/","orphanet_url":"https://www.orpha.net/en/disease/detail/90069","names":{"fr":{"name":"Intoxication systémique au monochloroacétate","definition":"L'intoxication systémique au monochloroacétate est une intoxication rare par l'acide monochloroacétique (surtout par la voie cutanée, mais aussi par inhalation ou ingestion), mettant en jeu le pronostic vital. Elle se caractérise par des vomissements, une diarrhée et une excitabilité du système nerveux central (SNC) (désorientation, délire, convulsions) comme signes avant-coureurs de l'empoisonnement systémique. Ils sont suivis par une dépression du SNC, un coma et un oedème cérébral. Les manifestations additionnelles incluent une atteinte cardiaque (sévère dépression myocardique, choc, arythmies, trouble myocardique non spécifique), une acidose métabolique sévère, une hypokaliémie, une hypocalcémie et une insuffisance rénale progressive conduisant à une anurie. Une myoglobinémie et une leucocytose peuvent survenir. Les manifestations peuvent être retardées d'une à quatre heures."},"de":{"name":"Monochloracetat-Vergiftung, systemische","definition":"Die systemische Monochloracetatvergiftung ist eine seltene, lebensbedrohliche Vergiftung mit Monochloressigsäure (hauptsächlich über die Haut, aber auch durch Einatmen oder Verschlucken). Sie ist gekennzeichnet durch Erbrechen, Durchfall und Erregbarkeit des Zentralnervensystems (ZNS) (Desorientierung, Delirium, Krämpfe) als frühe Zeichen einer systemischen Vergiftung, gefolgt von ZNS-Depression, Koma und Hirnödem. Weitere Anzeichen sind Herzbeteiligung (schwere Myokarddepression, Schock, Arrhythmien, unspezifische Myokardschäden), schwere metabolische Azidose, Hypokaliämie, Hypokalzämie und fortschreitendes Nierenversagen mit Anurie. Myoglobinämie und Leukozytose können auftreten. Die Manifestationen können um 1-4 Stunden verzögert sein."},"es":{"name":"Intoxicación sistémica por monocloroacetato","definition":"La intoxicación sistémica por monocloroacetato es una intoxicación poco frecuente potencialmente letal por ácido monocloroacético (principalmente a través de la piel, aunque también por inhalación o ingesta). Los síntomas precoces de la intoxicación sistémica consisten en vómitos, diarrea y excitabilidad del sistema nervioso central (SNC) (desorientación, delirio, crisis), seguidos por depresión del SNC, coma y edema cerebral. Otros signos adicionales incluyen afectación cardíaca (depresión miocárdica grave, hipotensión grave con shock, arritmias, daño miocárdico inespecífico), acidosis metabólica grave, hipopotasemia, hipocalcemia e insuficiencia renal progresiva con anuria. Puede presentarse con mioglobinemia y leucocitosis. Las manifestaciones pueden retrasarse entre 1-4 horas."},"it":{"name":"Avvelenamento sistemico da monocloroacetato","definition":"L'avvelenamento sistemico da monocloroacetato è un'intossicazione rara e potenzialmente letale da acido monocloroacetico (di solito per via cutanea, ma anche per inalazione o ingestione). I segni di esordio dell'avvelenamento sistemico sono il vomito, la diarrea e l'eccitabilità del sistema nervoso centrale (SNC) (disorientamento, delirio, convulsioni), seguiti dalla depressione del SNC, dal coma e dall'edema cerebrale. Altri segni clinici sono l'interessamento cardiaco (grave depressione miocardica, shock, aritmie, danno miocardico aspecifico), l'acidosi metabolica grave, l'ipokaliemia, l'ipocalcemia e l'insufficienza renale progressiva che esita nell'anuria. Possono associarsi mioglobinemia e leucocitosi. I sintomi possono presentarsi dopo una-quattro ore."},"nl":{"name":"Systemische monochloroacetaatvergiftiging","definition":"Systemische monochloroacetaatvergiftiging is een zeldzame, levensbedreigende vergiftiging met monochloroacetaat (vooral door de huid, maar ook door inademing of ingestie). De ziekte wordt gekarakteriseerd door braken, diarree en prikkelbaarheid van het centrale zenuwstelsel (CZS) (desoriëntatie, delirium, stuiptrekkingen) als vroege symptomen van systemische vergiftiging, gevolgd door depressie van het CZS, coma en hersenoedeem. Bijkomende symptomen omvatten betrokkenheid van het hart (ernstige myocardiale depressie, shock, hartritmestoornissen, niet-specifieke myocardiale schade), ernstige metabole acidose, hypokaliëmie en progressief nierfalen dat leidt tot anurie. Myoglobinemie en leukocytose kunnen voorkomen. Manifestaties kunnen een vertraging van 1-4 uren hebben."},"pt":{"name":"Envenenamento sistémico por monocloroacetato"},"pl":{"name":"Ogólne zatrucie kwasem monochlorooctowym"},"cs":{"name":"Systémová otrava monochlóracetátem"},"uk":{"name":"Системне отруєння монохлорацетатом","definition":"Systemic monochloroacetate poisoning is a rare, life-threatening intoxication with monochloroacetic acid (mainly through the skin, but also by inhalation or ingestion). It is characterized by vomiting, diarrhea and central nervous system (CNS)-excitability (disorientation, delirium, convulsions) as early signs of systemic poisoning, followed by CNS-depression, coma and cerebral edema. Additional signs include heart involvement (severe myocardial depression, shock, arrhythmias, nonspecific myocardial damage), severe metabolic acidosis, hypokalemia, hypocalcemia and progressive renal failure leading to anuria. Myoglobinemia and leukocytosis may occur. Manifestations may be delayed for 1-4 hours."},"zh":{"name":"全身性氯乙酸中毒"},"tr":{"name":"Sistemik monokloroasetat zehirlenmesi"}},"xrefs":[{"source":"ICD-10","code":"T60.3","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"NE61","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019545","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707283","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":108999,"name":"Rare disorder due to toxic effects"},"parents":[{"orpha":556508,"name":"Rare disorder due to poisoning","classifications":[204]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2004[OTHER]"}],"phenotypes":null}