{"orpha":544482,"name":"Infection-related hemolytic uremic syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Infection-related HUS"],"definition":"A rare type of hemolytic uremic syndrome (HUS) characterized by the triad of hemolytic anemia due to generalized thrombotic microangiopathy, thrombocytopenia, and acute kidney injury, and most commonly occurring after acute gastroenteritis due to Shiga toxin-producing enterohemorrhagic <i>Escherichia coli</i> or <i>Shigella dysenteriae</i>. Other infectious causes of HUS include <i>Streptococcus pneumoniae</i>, HIV, <i>Mycoplasma pneumoniae</i>, Histoplasmosis, and Coxsackie virus.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-544482/","orphanet_url":"https://www.orpha.net/en/disease/detail/544482","names":{"fr":{"name":"Syndrome hémolytique et urémique dû à une infection","synonyms":["SHU dû à une infection"],"definition":"Type rare de syndrome hémolytique et urémique (SHU) caractérisé par la triade associant une anémie hémolytique due à une microangiopathie thrombotique généralisée, une thrombocytopénie et une insuffisance rénale aiguë, le plus souvent après une gastroentérite aiguë due à Escherichia coli producteurs de shigatoxines ou à Shigella dysenteriae. Parmi les autres causes du SHU figurent une infection à Streptococcus pneumoniae, au VIH, à Mycoplasma pneumoniae, une histoplasmose et le virus coxsackie."},"de":{"name":"Infektions-assoziiertes hämolytisch-urämisches Syndrom","synonyms":["Infektion-bedingtes HUS"],"definition":"Eine seltene Form des hämolytisch-urämischen Syndroms (HUS), das durch die Trias aus hämolytischer Anämie aufgrund einer generalisierten thrombotischen Mikroangiopathie, Thrombozytopenie und akuter Nierenschädigung gekennzeichnet ist und am häufigsten nach einer akuten Gastroenteritis aufgrund von Shiga-Toxin produzierenden enterohämorrhagischen <i>Escherichia coli</i> oder <i>Shigella dysenteriae</i> auftritt. Andere infektiöse Ursachen für HUS sind <i>Streptococcus pneumoniae</i>, HIV, <i>Mykoplasma pneumoniae</i>, Histoplasmose und Coxsackie-Virus."},"es":{"name":"Síndrome hemolítico urémico asociado a infección","synonyms":["SHU asociado a infección"],"definition":"Es un tipo poco frecuente de síndrome urémico hemolítico (SUH) caracterizado por la tríada de anemia hemolítica debida a microangiopatía trombótica generalizada, trombocitopenia y lesión renal aguda, y que ocurre con mayor frecuencia después de una gastroenteritis aguda causada por <i>Escherichia coli</i> enterohemorrágica productora de toxina Shiga o por <i>Shigella dysenteriae</i>. Otras causas infecciosas del SUH incluyen <i>Streptococcus pneumoniae</i>, VIH, <i>Mycoplasma pneumoniae</i>, histoplasmosis y virus Coxsackie."},"it":{"name":"Sindrome emolitico-uremica correlata ad infezione","synonyms":["SEU correlata alle infezioni"]},"nl":{"name":"Infectie-gerelateerd hemolytisch-uremisch syndroom","synonyms":["Infectie-gerelateerd HUS"],"definition":"Een zeldzaam type van hemolytisch-uremisch syndroom (HUS), gekenmerkt door de triade van hemolytische anemie door gegeneraliseerde trombotische microangiopathie, trombocytopenie, en acuut nierletsel, en doorgaans optredend na acute gastro-enteritis door shigatoxine-producerende enterohemorragische <i>Escherichia coli</i> of <i>Shigella dysenteriae</i>. Andere infectieuze oorzaken van HUS zijn onder meer <i>Streptococcus pneumoniae</i>, HIV, <i>Mycoplasma pneumoniae</i>, histoplasmose, en coxsackievirus."},"pt":{"name":"Síndrome hemolítico-urémico infecção-relacionado","synonyms":["SHU infecção-relacionado"],"definition":"Tipo raro de síndrome hemolítico-urémica (SHU) caracterizada pela tríade de anemia hemolítica devido a microangiopatia trombótica generalizada, trombocitopenia e lesão renal aguda, e ocorre habitualmente após gastroenterite aguda devido a Escherichia coli enterohemorrágica produtora de toxina Shiga ou a Shigella dysenteriae. Outras causas infecciosas de SHU incluem Streptococcus pneumoniae, HIV, Mycoplasma pneumoniae, histoplasmose e vírus Coxsackie."},"pl":{"name":"Zespół hemolityczno-mocznicowy związany z infekcją","synonyms":["Infection-related HUS"],"definition":"Rzadko występujący typ zespołu hemolityczno-mocznicowego (HUS), charakteryzujący się obecnością triady objawów: niedokrwistość hemolityczna spowodowana uogólnioną mikroangiopatią zakrzepową, małopłytkowość i ostre uszkodzenie nerek. Najczęściej występuje jako konsekwencja ostrego zapalenia żołądka i jelit wywołanego zakażeniem jelitowo-krwotocznym typem Escherichia coli wytwarzającym toksynę Shiga lub Shigella dysenteriae. Inne przyczyny infekcyjne HUS obejmują Streptococcus pneumoniae, HIV, Mycoplasma pneumoniae, histoplazmozę i wirus Coxsackie."},"cs":{"name":"Hemolyticko-uremický syndrom související s infekcí","synonyms":["HUS související s infekcí"]},"uk":{"name":"Інфекційний гемолітико-уремічний синдром, повязаний з інфекцією","synonyms":["HUS, повязаний з інфекцією"],"definition":"A rare type of hemolytic uremic syndrome (HUS) characterized by the triad of hemolytic anemia due to generalized thrombotic microangiopathy, thrombocytopenia, and acute kidney injury, and most commonly occurring after acute gastroenteritis due to Shiga toxin-producing enterohemorrhagic <i>Escherichia coli</i> or <i>Shigella dysenteriae</i>. Other infectious causes of HUS include <i>Streptococcus pneumoniae</i>, HIV, <i>Mycoplasma pneumoniae</i>, Histoplasmosis, and Coxsackie virus."},"zh":{"name":"感染性溶血性尿毒症综合征","synonyms":["Infection-related HUS"]},"tr":{"name":"Enfeksiyon-ilişkili hemolitik üremik sendrom","synonyms":["Enfeksiyon-ilişkili HUS"]}},"xrefs":[{"source":"ICD-10","code":"D59.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"3A21.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2048034443"},{"source":"MONDO","code":"0034103","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680165","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":544458,"name":"Hemolytic uremic syndrome","classifications":[188,233]},{"orpha":182047,"name":"Rare acquired hemolytic anemia","classifications":[194]}],"children":[{"orpha":90038,"name":"Shiga toxin-associated hemolytic uremic syndrome","type":"Clinical subtype"},{"orpha":544493,"name":"Streptococcus pneumoniae-associated hemolytic uremic syndrome","type":"Clinical subtype"}],"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"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-06-09","source":"25593915[PMID]_27768015[PMID]_28242109[PMID]_31232894[PMID]_28711159[PMID]","terms":[{"hpo":"HP:0001919","label":"Acute kidney injury","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001878","label":"Hemolytic anemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000980","label":"Pallor","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032169","label":"Severe infection","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032155","label":"Abdominal cramps","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011115","label":"Abnormality of chemokine secretion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011116","label":"Abnormality of interferon secretion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000707","label":"Abnormality of the nervous system","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100282","label":"Acute colitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011037","label":"Decreased urine output","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030783","label":"Increased circulating interleukin 6 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011947","label":"Respiratory tract infection","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100519","label":"Anuria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031864","label":"Bacteremia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025085","label":"Bloody diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002153","label":"Hyperkalemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100735","label":"Hypertensive crisis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002901","label":"Hypocalcemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002902","label":"Hyponatremia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012819","label":"Myocarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012593","label":"Nephrotic range proteinuria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100520","label":"Oliguria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001733","label":"Pancreatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002102","label":"Pleuritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002090","label":"Pneumonia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004372","label":"Reduced consciousness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005208","label":"Secretory diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031691","label":"Severe viral infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030049","label":"Brain abscess","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100704","label":"Cerebral visual impairment","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0005244","label":"Gastrointestinal infarctions","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0007430","label":"Generalized edema","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001269","label":"Hemiparesis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031368","label":"Intestinal perforation","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002576","label":"Intussusception","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011919","label":"Pleural empyema","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003095","label":"Septic arthritis","frequency":"Very rare (<4-1%)","criteria":null}]}}