{"orpha":90051,"name":"Sepsis in premature infants","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare systemic condition affecting neonates born at less than 37 weeks gestational age and characterized by life-threatening organ dysfunction caused by a dysregulated host response to an infection, which may have been acquired shortly before or during birth (resulting in early-onset neonatal sepsis during the first 72 hours of life), or after birth (leading to late-onset neonatal sepsis between 72 hours and three months). Prematurity constitutes one of the primary risk factors for neonatal sepsis. The clinical picture may develop gradually with signs and symptoms like irritability, lethargy, or poor feeding, or progress rapidly to respiratory distress, fever, hypothermia, hypotension, shock, and multiple organ failure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90051/","orphanet_url":"https://www.orpha.net/en/disease/detail/90051","names":{"fr":{"name":"Sepsis chez le nouveau-né prématuré","definition":"Maladie systémique rare affectant les nouveau-nés dans des cas de naissance prématurée à moins de 37 semaines d'âge gestationnel caractérisée par un dysfonctionnement organique potentiellement létal dû à une réponse déréglée de l'hôte à une infection, qui peut avoir été contractée peu avant la naissance ou pendant l'accouchement (entraînant une septicémie néonatale à début précoce pendant les 72 premières heures de vie), ou après la naissance (entraînant une septicémie néonatale à début tardif entre 72 heures et trois mois de vie). La prématurité constitue l'un des principaux facteurs de risque de septicémie néonatale. Le tableau clinique peut s'installer de façon progressive avec des signes et des symptômes tels qu'une irritabilité, une léthargie ou des difficultés à s'alimenter, ou évoluer rapidement vers une détresse respiratoire, une fièvre, une hypothermie, une hypotension, un choc et une défaillance multiviscérale."},"de":{"name":"Sepsis bei Frühgeborenen","definition":"Eine seltene systemische Erkrankung, die Neugeborene betrifft, die in einem Gestationsalter von weniger als 37 Wochen geboren wurden, und die durch eine lebensbedrohliche Organdysfunktion gekennzeichnet ist, die durch eine dysregulierte Abwehrreaktion des Patienten auf eine Infektion verursacht wird. Diese Infektion kann kurz vor oder während der Geburt erworben worden sein (was zu einer früh einsetzenden neonatalen Sepsis in den ersten 72 Stunden des Lebens führt) oder nach der Geburt (was zu einer spät einsetzenden neonatalen Sepsis zwischen 72 Stunden und drei Monaten führt). Die Frühgeburtlichkeit ist einer der Hauptrisikofaktoren für die neonatale Sepsis. Das klinische Bild kann sich allmählich mit Anzeichen und Symptomen wie Reizbarkeit, Lethargie oder schlechter Nahrungsaufnahme entwickeln oder rasch zu Atemnot, Fieber, Hypothermie, Hypotonie, Schock und Multiorganversagen fortschreiten."},"es":{"name":"Sepsis en neonatos prematuros","definition":"Es una enfermedad sistémica poco frecuente que afecta a los neonatos con edad gestacional inferior a 37 semanas y que se caracteriza por una disfunción orgánica potencialmente mortal causada por una respuesta desregulada del hospedador a una infección, que puede haber sido adquirida poco antes o durante el parto (dando lugar a sepsis neonatal de inicio precoz durante las primeras 72 horas de vida), o después del nacimiento (dando lugar a una sepsis neonatal de inicio tardío entre las 72 horas y los tres meses de edad). La prematuridad constituye uno de los principales factores de riesgo de la sepsis neonatal. El cuadro clínico puede desarrollarse gradualmente con signos y síntomas como irritabilidad, letargia o la mala alimentación, o progresar rápidamente a dificultad respiratoria, fiebre, hipotermia, hipotensión, shock y fallo multiorgánico."},"it":{"name":"Sepsi nei neonati prematuri"},"nl":{"name":"Sepsis bij premature zuigeling","definition":"Een zeldzame systemische aandoening bij neonaten die geboren worden op een gestatieleeftijd van minder dan 37 weken, gekenmerkt door levensbedreigende orgaandisfunctie als gevolg van een ontregelde gastheerrespons op een infectie, die werd verworven kort voor of tijdens de geboorte (resulterend in vroeg optredende neonatale sepsis tijdens de eerste 72 uren na de geboorte), of na de geboorte (resulterend in laat optredende neonatale sepsis tussen 72 uren en drie maanden). Prematuriteit is een van de primaire risicofactoren voor neonatale bloedvergiftiging. Het klinische beeld kan geleidelijk ontwikkelen, met verschijnselen en symptomen als prikkelbaarheid, lethargie of voedingsproblemen, of snel evolueren naar ademnood, koorts, hypothermie, hypotensie, shock, en multiorgaanfalen."},"pt":{"name":"Sépsis de apresentação tardia em lactentes prematuros","definition":"Doença sistémica rara que afeta recém-nascidos com menos de 37 semanas de idade gestacional e caracterizada por disfunção orgânica com risco de vida causada por uma resposta desregulada do hospedeiro a uma infeção, que pode ter sido adquirida pouco antes ou durante o nascimento (resultando em sepsis neonatal de início precoce durante as primeiras 72 horas de vida) ou após o nascimento (resultando em sepsis neonatal tardia entre 72 horas e três meses). A prematuridade constitui um dos principais fatores de risco para sepsis neonatal. O quadro clínico pode evoluir gradualmente com irritabilidade, letargia ou má alimentação, ou progredir rapidamente para desconforto respiratório, febre, hipotermia, hipotensão, choque e falência de multiorgânica."},"pl":{"name":"Sepsa u wcześniaków","definition":"Rzadka choroba ogólnoustrojowa występująca u noworodków urodzonych przed 37 tygodniem ciąży, charakteryzująca się zagrażającą życiu dysfunkcją narządów wskutek nieprawidłowej odpowiedzi organizmu pacjenta na infekcję, która mogła zostać nabyta krótko przed lub w trakcie porodu (prowadząc do wczesnej posocznicy noworodków w ciągu pierwszych 72 godzin życia) lub po urodzeniu (prowadzące do późnej posocznicy noworodków w okresie pomiędzy 72 godziną a 3 miesiącem życia). Wcześniactwo jest jednym z głównych czynników ryzyka sepsy noworodków. Obraz kliniczny może rozwijać się stopniowo z podmiotowymi i przedmiotowymi objawami obejmującymi drażliwość, senność lub problemy w karmieniu, lub szybko przechodzić w niewydolność oddechową, gorączkę, hipotermię, niedociśnienie, wstrząs i niewydolność wielonarządową."},"cs":{"name":"Sepse u nedonošených"},"uk":{"name":"Сепсис у недоношених дітей","definition":"A rare systemic condition affecting neonates born at less than 37 weeks gestational age and characterized by life-threatening organ dysfunction caused by a dysregulated host response to an infection, which may have been acquired shortly before or during birth (resulting in early-onset neonatal sepsis during the first 72 hours of life), or after birth (leading to late-onset neonatal sepsis between 72 hours and three months). Prematurity constitutes one of the primary risk factors for neonatal sepsis. The clinical picture may develop gradually with signs and symptoms like irritability, lethargy, or poor feeding, or progress rapidly to respiratory distress, fever, hypothermia, hypotension, shock, and multiple organ failure."},"zh":{"name":"早产儿败血症"},"tr":{"name":"Prematüre bebeklerde sepsis"}},"xrefs":[{"source":"ICD-10","code":"P36.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"P36.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"P36.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"P36.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"P36.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"P36.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"P36.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"P36.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"KA60","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"UMLS","code":"C5681350","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":182222,"name":"Rare systemic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":32.0,"area":"Europe","status":"Validated","source":"European Medicines Agency[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2020-02-14","source":"28709162[PMID]_27589549[PMID]_30207217[PMID]_28434651[PMID]_29382648[PMID]","terms":[{"hpo":"HP:0010978","label":"Abnormality of immune system physiology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0040187","label":"Neonatal sepsis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001622","label":"Premature birth","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001518","label":"Small for gestational age","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001649","label":"Tachycardia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031602","label":"Abnormal mucociliary clearance","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011410","label":"Caesarian section","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004325","label":"Decreased body weight","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001875","label":"Decreased total neutrophil count","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012719","label":"Functional abnormality of the gastrointestinal tract","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002579","label":"Gastrointestinal dysmotility","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002045","label":"Hypothermia","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:0001942","label":"Metabolic acidosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001319","label":"Neonatal hypotonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000980","label":"Pallor","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032169","label":"Severe infection","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001892","label":"Abnormal bleeding","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002795","label":"Abnormal respiratory system physiology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000236","label":"Abnormality of the anterior fontanelle","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001903","label":"Anemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001662","label":"Bradycardia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000961","label":"Cyanosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001410","label":"Decreased liver function","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004387","label":"Enterocolitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001265","label":"Hyporeflexia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030863","label":"Nasal flaring","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100520","label":"Oliguria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000967","label":"Petechiae","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002686","label":"Prenatal maternal abnormality","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000979","label":"Purpura","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004713","label":"Reversible renal failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005968","label":"Temperature instability","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011880","label":"Acute disseminated intravascular coagulation","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031696","label":"Disseminated viral infection","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0020101","label":"Invasive fungal infection","frequency":"Very rare (<4-1%)","criteria":null}]}}