{"orpha":505395,"name":"Ventilator-induced diaphragmatic dysfunction","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["VIDD"],"definition":"A rare respiratory disorder characterized by major reduction of diaphragmatic contractile force together with fiber atrophy in the diaphragm and other respiratory muscles as a consequence of invasive mechanical ventilation. Reduction of diaphragmatic contractile force may be observed even within hours after intubation. The condition can increase weaning time and affects weaning outcome, mortality, and long-term clinical outcomes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-505395/","orphanet_url":"https://www.orpha.net/en/disease/detail/505395","names":{"fr":{"name":"Dysfonction diaphragmatique induite par la ventilation mécanique","definition":"Maladie respiratoire rare caractérisée par une importante diminution de la force de contraction du diaphragme et une atrophie des fibres diaphragmatiques et des fibres d'autres muscles respiratoires, résultant d'une ventilation mécanique invasive. Une diminution de la force contractile du diaphragme peut être observée même dans les heures qui suivent l'intubation. La maladie peut allonger la durée de sevrage, impacter le résultat du sevrage, le taux de mortalité et les résultats cliniques à long terme."},"de":{"name":"Beatmungsinduzierter Zwerchfellschaden","synonyms":["VIDD","Ventilator-induzierter Zwerchfellschaden"],"definition":"Eine seltene Atemwegsstörung, die durch eine starke Verringerung der kontraktilen Kraft des Zwerchfells zusammen mit einer Faseratrophie des Zwerchfells und anderer Atemmuskeln als Folge einer invasiven mechanischen Beatmung gekennzeichnet ist. Die Verringerung der kontraktilen Kraft des Zwerchfells kann sogar innerhalb von Stunden nach der Intubation beobachtet werden. Dieser Zustand kann die Entwöhnungszeit verlängern und wirkt sich auf das Entwöhnungsergebnis, die Sterblichkeit und die langfristigen klinischen Ergebnisse aus."},"es":{"name":"Disfunción diafragmática inducida por la ventilación","synonyms":["DDIV"],"definition":"Es un trastorno respiratorio poco frecuente caracterizado por una reducción importante de la fuerza contráctil diafragmática junto con atrofia de las fibras musculares del diafragma y de otros músculos respiratorios como consecuencia de la ventilación mecánica invasiva. Puede observarse una reducción de la fuerza contráctil diafragmática incluso horas después de la intubación. El trastorno puede prolongar el tiempo de destete y afecta al destete, la mortalidad y al pronóstico clínico a largo plazo."},"it":{"name":"Disfunzione del diaframma indotta dalla ventilazione meccanica"},"nl":{"name":"Disfunctie van diafragma geïnduceerd door kunstmatige beademing","synonyms":["VIDD"],"definition":"Een zeldzame respiratoire aandoening, gekenmerkt door ernstige afname van de contractiekracht van diafragma, in combinatie met atrofie van vezels in diafragma en andere ademhalingsspieren als gevolg van invasieve kunstmatige beademing. Vermindering van de contractiekracht van diafragma kan zelfs al binnen enkele uren na intubatie waargenomen worden. De aandoening kan ontwenning van borst- of flesvoeding vertragen en heeft een effect op de uitkomst van deze ontwenning, op mortaliteit, en op klinische uitkomsten op lange termijn."},"pt":{"name":"Disfunção diafragmática induzida pelo ventilador","definition":"Distúrbio respiratório raro caracterizado por grave redução da força contrátil do diafragma juntamente com atrofia das fibras musculares do diafragma e de outros músculos respiratórios como consequência da ventilação mecânica invasiva. A redução da força contrátil diafragmática pode ser observada mesmo poucas horas após a intubação. A condição pode aumentar o tempo de desmame e afetar o resultado do desmame, a mortalidade e os resultados clínicos a longo prazo."},"pl":{"name":"Mechaniczna wentylacja-wywołanie dysfunkcji przepony","synonyms":["VIDD"],"definition":"Rzadko występująca choroba układu oddechowego charakteryzująca się znacznym zmniejszeniem siły skurczu przepony i zanikiem włókien mięśniowych przepony i innych mięśni oddechowych w wyniku inwazyjnej wentylacji mechanicznej. Zmniejszenie siły skurczu przepony można zaobserwować nawet w ciągu kilku godzin od intubacji. Schorzenie to może wydłużyć czas do momentu odłączenia od wentylacji mechanicznej i wpływać na rezultat odłączenia, śmiertelność i długoterminowe skutki kliniczne."},"cs":{"name":"Dysfunkce bránice vyvolaná ventilátorem","synonyms":["VIDD"]},"uk":{"name":"Діафрагмальна дисфункція, зумовлена ШВЛ","synonyms":["VIDD"],"definition":"A rare respiratory disorder characterized by major reduction of diaphragmatic contractile force together with fiber atrophy in the diaphragm and other respiratory muscles as a consequence of invasive mechanical ventilation. Reduction of diaphragmatic contractile force may be observed even within hours after intubation. The condition can increase weaning time and affects weaning outcome, mortality, and long-term clinical outcomes."},"zh":{"name":"呼吸机诱发的膈肌功能障碍","synonyms":["VIDD"]},"tr":{"name":"Ventilatör kaynaklı diyafram disfonksiyonu","synonyms":["VIDD"]}},"xrefs":[{"source":"ICD-10","code":"J95.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"NE8Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"UMLS","code":"C5680083","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":101944,"name":"Rare pulmonary disease","classifications":[184]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_28887062[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-06-02","source":"30453774[PMID]_28887062[PMID]","terms":[{"hpo":"HP:0000775","label":"Abnormality of the diaphragm","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0009113","label":"Diaphragmatic weakness","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025464","label":"Increased reactive oxygen species production","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005946","label":"Ventilator dependence with inability to wean","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003457","label":"EMG abnormality","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012496","label":"Reduced maximal inspiratory pressure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032169","label":"Severe infection","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011458","label":"Abdominal symptom","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012416","label":"Hypercapnia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000821","label":"Hypothyroidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032341","label":"Reduced forced vital capacity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002747","label":"Respiratory insufficiency due to muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006597","label":"Diaphragmatic paralysis","frequency":"Very rare (<4-1%)","criteria":null}]}}