Zespół hemolityczno-uremicznego
Objawy
Zespół hemolityczno-uremiczny (HUS) to złożone schorzenie charakteryzujące się triadą: mikroangiopatyczną niedokrwistością hemolityczną, małopłytkowością oraz ostrym uszkodzeniem nerek. Typowa postać (STEC-HUS) jest związana z zakażeniem bakteriami produkującymi toksynę Shiga, najczęściej Escherichia coli O157:H7, i manifestuje się po 2-4 dniach inkubacji objawami gastroenterologicznymi, takimi jak biegunka krwista, ból brzucha, wymioty i gorączka. Objawy HUS pojawiają się zwykle 5-10 dni po wystąpieniu biegunki i obejmują bladość, zmęczenie, wybroczyny, żółtaczkę oraz objawy niewydolności nerek, takie jak oligoanuria/anuria, krwiomocz, nadciśnienie tętnicze i obrzęki. Schistocyty w rozmazie krwi obwodowej potwierdzają mikroangiopatyczną niedokrwistość hemolityczną. Około 55-70% pacjentów z STEC-HUS rozwija ostre uszkodzenie nerek, z 70-85% odzyskującą funkcję nerek. Atypowy HUS (aHUS) ma etiologię związaną z dysfunkcją układu dopełniacza i charakteryzuje się gorszym rokowaniem, z 50% ryzykiem progresji do schyłkowej niewydolności nerek i 25% śmiertelnością w fazie ostrej.
- Wprowadzenie do zespołu hemolityczno-uremicznego
- Początkowe objawy infekcji poprzedzającej HUS
- Rozwój i manifestacja kliniczna HUS
- Różnice między typowym (STEC-HUS) a atypowym (aHUS)
- Grupy szczególnego ryzyka
- Czynniki prognostyczne w zespole hemolityczno-uremicznym
- Przebieg choroby i potencjalne powikłania
- Rokowanie w zespole hemolityczno-uremicznym
- Podsumowanie
Wprowadzenie do zespołu hemolityczno-uremicznego
Zespół hemolityczno-uremiczny (Hemolytic uremic syndrome, HUS) to rzadkie, ale poważne schorzenie charakteryzujące się triadą objawów: mikroangiopatyczną niedokrwistością hemolityczną, małopłytkowością oraz ostrym uszkodzeniem nerek. Choroba prowadzi do uszkodzenia małych naczyń krwionośnych, powodując tworzenie się zakrzepów w naczyniach całego organizmu, które mogą uszkadzać nerki i inne narządy12. Zespół hemolityczno-uremiczny jest najczęstszą przyczyną ostrego uszkodzenia nerek u dzieci3.
Wyróżnia się dwie główne postaci zespołu hemolityczno-uremicznego: postać typową (STEC-HUS), związaną z zakażeniem bakteriami wytwarzającymi toksynę Shiga (najczęściej Escherichia coli O157:H7) oraz postać atypową (aHUS), która może być spowodowana zaburzeniami genetycznymi lub nabytymi, aktywującymi alternatywną drogę układu dopełniacza45.
Początkowe objawy infekcji poprzedzającej HUS
W przypadku typowej postaci zespołu hemolityczno-uremicznego (STEC-HUS), objawy kliniczne zazwyczaj pojawiają się po okresie inkubacji wynoszącym 2-4 dni od zakażenia bakteriami E. coli6. Pierwszymi objawami są dolegliwości ze strony przewodu pokarmowego, które mogą trwać od 1 do 15 dni78:
- Biegunka, często z domieszką krwi
- Ból brzucha i kurczowe dolegliwości
- Nudności i wymioty
- Gorączka i dreszcze
- Bóle głowy
Biegunka krwista pojawia się zazwyczaj 1-3 dni po początkowych objawach zakażenia11. Typowo, objawy HUS rozwijają się po 5-10 dniach od wystąpienia biegunki, choć w niektórych przypadkach okres ten może wynosić od 2 do nawet 14 dni612.
W przypadku atypowego HUS (aHUS), początkowe objawy są często niespecyficzne i obejmują zmęczenie, bladość skóry czy senność. Pacjenci czasami zgłaszają wystąpienie „czynnika wyzwalającego” w postaci drobnej infekcji lub biegunki134.
Rozwój i manifestacja kliniczna HUS
Objawy hematologiczne
W miarę rozwoju choroby toksyny uwalniane w jelitach zaczynają niszczyć krwinki czerwone, co prowadzi do niedokrwistości hemolitycznej14. Charakterystyczne objawy hematologiczne obejmują:
- Bladość skóry (utrata koloru)
- Znaczne zmęczenie i osłabienie
- Łatwe siniaczenie się
- Nietypowe krwawienia, np. z nosa i jamy ustnej
- Wybroczyny (drobne, czerwone lub fioletowe plamki na skórze)
- Żółtaczka (zażółcenie skóry i białkówek oczu)
W badaniu krwi obwodowej można zaobserwować schistocyty (fragmenty zniszczonych erytrocytów), które są charakterystyczne dla mikroangiopatycznej niedokrwistości hemolitycznej1819.
Objawy nerkowe
Uszkodzone krwinki czerwone oraz inne czynniki mogą blokować drobne naczynia krwionośne w nerkach, co prowadzi do uszkodzenia tkanki nerkowej i zaburzenia funkcji nerek20. Objawy niewydolności nerek w przebiegu HUS to:
- Zmniejszone wydalanie moczu (oliguria) lub całkowity brak produkcji moczu (anuria)
- Krwiomocz (krew w moczu)
- Nadciśnienie tętnicze (często ciężkie)
- Obrzęki, szczególnie kończyn dolnych, stóp i kostek, czasem również twarzy i rąk
- Uogólnione gromadzenie się płynu w tkankach (obrzęk)
Oligoanuria (zmniejszone wydalanie moczu lub jego brak) występuje w większości przypadków HUS. Zazwyczaj funkcja wydalnicza nerek powraca po około tygodniu, jednak czas trwania oligoanurii może być różny – od 2-3 dni do nawet miesiąca lub dłużej23. Około 55-70% pacjentów z STEC-HUS rozwija ostre uszkodzenie nerek, przy czym do 70-85% z nich odzyskuje funkcję nerek3.
Objawy pozanerkowe
Zespół hemolityczno-uremiczny może również powodować objawy związane z uszkodzeniem innych narządów2. Manifestacje pozanerkowe obejmują:
Objawy neurologiczne
- Drażliwość i zmiany zachowania
- Splątanie
- Drgawki (występują u około 20-30% dzieci z HUS, częściej u najmłodszych)
- Zmniejszony poziom świadomości
- Niedowład połowiczy
- Udar mózgu
Objawy sercowo-naczyniowe
- Niedokrwienie mięśnia sercowego
- Niewydolność serca
Objawy z przewodu pokarmowego
- Zapalenie trzustki
- Zapalenie pęcherzyka żółciowego
- Martwica jelit
- Perforacja jelita
- Wgłobienie jelita
Objawy endokrynologiczne
- Cukrzyca
Różnice między typowym (STEC-HUS) a atypowym (aHUS)
Istnieją pewne różnice w przebiegu klinicznym między typową a atypową postacią zespołu hemolityczno-uremicznego:
Typowy STEC-HUS:
- Zazwyczaj występuje po prodromalnej biegunce krwistej i objawach gastroenterologicznych
- Objawy HUS pojawiają się średnio 5-10 dni po wystąpieniu biegunki
- Częściej występuje u dzieci poniżej 5 roku życia
- Rokowanie jest zazwyczaj dobre – większość dzieci w pełni wraca do zdrowia
Atypowy aHUS:
- Może, ale nie musi, być poprzedzony infekcją
- Początek objawów jest często mniej gwałtowny niż w typowym HUS
- Może występować zarówno u dzieci, jak i dorosłych
- Rokowanie jest gorsze – do 50% pacjentów może rozwinąć schyłkową niewydolność nerek, a do 25% umiera w ostrej fazie choroby
Grupy szczególnego ryzyka
Niektóre grupy osób są bardziej narażone na rozwój ciężkiej postaci zespołu hemolityczno-uremicznego oraz związanych z nim powikłań:
- Dzieci poniżej 5 roku życia – HUS występuje najczęściej w tej grupie wiekowej i stanowi najczęstszą przyczynę ostrego uszkodzenia nerek u dzieci10
- Dzieci poniżej 1 roku życia lub powyżej 5 lat – większe ryzyko zgonu24
- Osoby starsze – również zwiększone ryzyko ciężkiego przebiegu choroby i zgonu24
Czynniki prognostyczne w zespole hemolityczno-uremicznym
Istnieją czynniki, które mogą wskazywać na gorsze rokowanie w przebiegu HUS31:
- Oliguria utrzymująca się ponad 10 dni
- Anuria trwająca dłużej niż 5 dni
- Odwodnienie w ostrej fazie
- Wysoka leukocytoza (powyżej 20 000/mm³)
- Hematokryt powyżej 23%
- Wystąpienie objawów neurologicznych
- Niska liczba płytek krwi
- Niska liczba neutrofilów
Dodatkowo, niekorzystnym czynnikiem prognostycznym jest długotrwała potrzeba dializoterapii, co zwiększa ryzyko długoterminowych problemów nerkowych32.
Przebieg choroby i potencjalne powikłania
Zespół hemolityczno-uremiczny może prowadzić do różnych powikłań, zarówno ostrych, jak i długoterminowych15:
- Ostra niewydolność nerek – występuje u 55-70% pacjentów z HUS3
- Przewlekła choroba nerek – może rozwinąć się u około 30% pacjentów30
- Schyłkowa niewydolność nerek – wymaga długoterminowej dializoterapii lub przeszczepu nerki
- Nadciśnienie tętnicze – może utrzymywać się przez wiele lat po epizodzie HUS33
- Powikłania neurologiczne – od subtelnych zaburzeń, takich jak niezgrabność, słaba koordynacja ruchowa drobna, nadaktywność i dekoncentracja, po ciężkie uszkodzenie mózgu23
- Zaburzenia krzepnięcia – prowadzące do krwawień15
- Problemy sercowe – w tym niedokrwienie mięśnia sercowego i niewydolność serca15
Rokowanie w zespole hemolityczno-uremicznym
Rokowanie w zespole hemolityczno-uremicznym zależy od wielu czynników, w tym od typu HUS, wieku pacjenta, czasu rozpoczęcia leczenia oraz wystąpienia powikłań.
Typowy STEC-HUS
W przypadku typowego STEC-HUS rokowanie jest zazwyczaj korzystne:
- Ponad 85% pacjentów z typowym HUS odzyskuje pełną funkcję nerek233
- Śmiertelność wynosi około 3-5%19
- U około 30% pacjentów mogą rozwinąć się długoterminowe problemy nerkowe, w tym nadciśnienie tętnicze i przewlekła choroba nerek30
- Dzieci z HUS mają lepsze rokowanie niż dorośli34
Atypowy aHUS
W przypadku atypowego HUS rokowanie jest gorsze:
- Do 50% pacjentów rozwija schyłkową niewydolność nerek lub nieodwracalne uszkodzenie mózgu3
- Do 25% pacjentów umiera w ostrej fazie choroby3
- Wprowadzenie nowych metod leczenia, jak inhibitory układu dopełniacza (ekulizumab, rawulizumab), znacznie poprawiło rokowanie – zmniejszyło progresję do schyłkowej niewydolności nerek lub zgonu u dzieci z 30-50% do 9%, a u dorosłych z 60% do 15%35
Podsumowanie
Zespół hemolityczno-uremiczny (HUS) jest poważnym schorzeniem, charakteryzującym się triadą objawów: mikroangiopatyczną niedokrwistością hemolityczną, małopłytkowością oraz ostrym uszkodzeniem nerek. Najczęstsza postać, STEC-HUS, zazwyczaj występuje po infekcji przewodu pokarmowego bakteriami produkującymi toksynę Shiga, najczęściej E. coli O157:H7.
Choroba zwykle rozpoczyna się od biegunki, często krwistej, bólów brzucha, wymiotów i gorączki. Po kilku dniach rozwijają się objawy związane z uszkodzeniem krwinek czerwonych (bladość, zmęczenie, łatwe siniaczenie się) oraz niewydolnością nerek (zmniejszone wydalanie moczu, obrzęki, nadciśnienie). Mogą również wystąpić objawy uszkodzenia innych narządów, w tym ośrodkowego układu nerwowego.
Mimo że zespół hemolityczno-uremiczny jest poważnym schorzeniem, większość dzieci z typową postacią HUS wraca do pełnego zdrowia. Jednak u części pacjentów mogą rozwinąć się długoterminowe powikłania, takie jak przewlekła choroba nerek, nadciśnienie tętnicze czy zaburzenia neurologiczne. Gorsze rokowanie obserwuje się w przypadku atypowego HUS oraz u pacjentów z czynnikami ryzyka, takimi jak długotrwała oliguria lub anuria, objawy neurologiczne czy wysoka leukocytoza.
Wczesne rozpoznanie objawów i natychmiastowe wdrożenie odpowiedniego leczenia są kluczowe dla poprawy rokowania w zespole hemolityczno-uremicznym.
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Materiały źródłowe
- #1 Hemolytic uremic syndrome (HUS) | Altru Health Systemhttps://www.altru.org/health-library/conditions/hemolytic-uremic-syndrome-hus
Hemolytic uremic syndrome (HUS) is a condition that can occur when small blood vessels become damaged and inflamed. This damage can cause clots to form in the vessels all through the body. The clots can damage the kidneys and other organs. Hemolytic uremic syndrome can lead to kidney failure, which can be life-threatening. […] The symptoms of hemolytic uremic syndrome vary, depending on the cause. The first symptoms of hemolytic uremic syndrome caused by E. coli bacteria might include: Diarrhea, which is often bloody. Pain, cramping or bloating in the stomach area. Fever. Vomiting. […] All forms of hemolytic uremic syndrome damage blood vessels. This damage causes red blood cells to break down, called anemia. The condition also causes blood clots to form in the blood vessels and, in turn, damage the kidneys.
- #2 Hemolytic Uremic Syndrome (HUS) – Symptoms, causes, treatment | National Kidney Foundationhttps://www.kidney.org/kidney-topics/hemolytic-uremic-syndrome-hus
Hemolytic uremic syndrome (HUS) damages blood vessels, causing low platelets, anemia, and kidney failure. It can also harm other organs like the brain and heart. […] HUS generally occurs in children who have had an illness involving diarrhea (usually bloody). Most children fully recover from their bowel illness without developing HUS. However, a small percentage become pale and have less energy due to HUS progression. Their urine output may also decrease, but a loss of color in the skin is the most striking symptom. […] In HUS, the tiny filter units in the kidneys known as glomeruli become clogged with platelets and damaged red blood cells. This leads to problems with the kidney’s ability to filter and eliminate waste products. […] More than 85 percent of patients with the most common form of HUS recover complete kidney function. However, even with full recovery, there is the chance for high blood pressure or other kidney problems in the years ahead. In general, the outlook in HUS is related to the severity of involvement of other organs such as the brain, pancreas, liver, or heart.
- #3 Hemolytic-Uremic Syndrome: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/779218-overview
Hemolytic-uremic syndrome (HUS) is a clinical syndrome characterized by progressive kidney failure that is associated with microangiopathic (nonimmune, Coombs-negative) hemolytic anemia and thrombocytopenia. HUS is the most common cause of acute kidney injury in children and is increasingly recognized in adults. […] Acute kidney injury occurs in 55-70% of patients, but they have a favorable prognosis, and as many as 70-85% of patients recover kidney function. […] Overall, patients with nonStx-HUS have a poor outcome, with as many as 50% progressing to end-stage renal disease (ESRD) or irreversible brain damage. Up to 25% of patients die during the acute phase. […] For nonStx-HUS, patients have poor outcomes, with up to 50% progressing to ESRD or irreversible brain damage. As many as 25% die during the acute phase. […] Patients collectively have a poor prognosis, and as many as 50-60% progress to ESRD (50% in those with the sporadic forms and 60% in those with the familial forms) or develop irreversible brain damage. About 25% die during the acute phase.
- #4 Hemolytic Uremic Syndrome – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK556038/
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. HUS is most commonly caused by Shiga toxin (typical HUS) or, less commonly, infections or genetic abnormalities activating the alternative complement pathway (atypical HUS). […] The initial clinical manifestation of aHUS typically involves nonspecific symptoms like fatigue, pallor, or somnolence. These symptoms can progress to signs of acute kidney injury (AKI), including oliguria, uremia, and fluid overload. The risk of progression to stage 3 or 4 chronic kidney disease (CKD) and end-stage renal disease (ESRD) in aHUS is high. In contrast to typical HUS, patients with aHUS often fail to regain kidney function without treatment. Untreated, approximately 50% of aHUS cases progress to dialysis dependency, with a mortality rate of 25%.
- #5 Haemolytic uraemic syndrome – Symptoms, diagnosis and treatment | BMJ Best Practicehttps://bestpractice.bmj.com/topics/en-gb/470
Haemolytic uraemic syndrome (HUS) is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia, and acute kidney injury. […] Most cases of HUS occur in children and are diarrhoea-associated (D+ HUS). Diarrhoea-associated HUS is usually caused by Shiga toxin-producing Escherichia coli. […] Acute kidney injury necessitating dialysis develops in approximately half of children with diarrhoea-associated HUS. […] Atypical HUS can occur due to genetic or acquired abnormalities in the alternative complement regulatory pathway. HUS can also occur as a secondary phenomenon due to medications, cancer, and other systemic diseases.
- #6 Haemolytic Uraemic Syndrome (Causes, Symptoms and Treatment)https://patient.info/doctor/haemolytic-uraemic-syndrome-pro
Haemolytic uraemic syndrome (HUS) was first described in 1955. It is is a triad of: Microangiopathic haemolytic anaemia (Coombs’ test negative), Thrombocytopenia, Acute kidney injury (acute renal failure). Haemolytic uraemic syndrome is the most common cause of acute kidney injury in children and its incidence appears to be growing worldwide. Some children may develop partial or incomplete HUS with thrombocytopenia, with or without anaemia, but the serum creatinine concentration remains normal. Typical (or infection-induced) HUS is most commonly associated with Escherichia coli with somatic (O) antigen 157 and flagella (H) antigen 7 – hence the designation O157:H7. The development of HUS may occur up to two weeks after the initial onset of symptoms and may develop after apparent recovery from the initial acute illness. About 10% of HUS cases are atypical and are not caused by Shiga toxin-producing bacteria or streptococci. The classical presenting feature is profuse diarrhoea that turns bloody 1-3 days later. Most adults infected with E. coli O157 remain asymptomatic. Early clinical signs of haemolytic uraemic syndrome may not be specific and it is recommended that if there is any suspicion of HUS (eg, any case of bloody diarrhoea) then assessment should include: evidence of haemolysis, anaemia and thrombocytopenia. Raised white cell count and low platelet count are early indicators of development of HUS. Typical HUS with a diarrhoeal prodrome usually has a good prognosis. Fatality is highest in infants, small children and the elderly. Atypical HUS often has a poorer prognosis, with death rates reportedly as high as 25% and progression to end-stage kidney disease in 50%. […] The incubation period for STEC is 2-4 days. HUS usually arises within seven days after exposure to VTEC but this interval may be up to 14 days.
- #7 Hemolytic Uremic Syndrome in Children – Stanford Medicine Children’s Healthhttps://www.stanfordchildrens.org/en/topic/default?id=hemolytic-uremic-syndrome-in-children-90-P03089
Hemolytic uremic syndrome (HUS) occurs when damaged red blood cells block the kidney’s filtering system which can lead to kidney failure in children. HUS causes red blood cells to clog the tiny blood vessels in the kidneys. It may cause damage to the kidney tissues. These problems make it harder for them to work and to remove waste and extra fluid from the blood. HUS is a serious illness and can be fatal. But most children with the most common form of HUS recover with full kidney function. […] Symptoms can occur a bit differently in each child. The first symptoms of HUS often last from 1 to 15 days and may include: Belly (abdominal) pain, Bloody or watery diarrhea, Vomiting, Fever. […] Severe problems in the bowel and colon may develop in some children, even after the above symptoms are gone. In these cases, a child may have symptoms such as: Irritability, Tiredness, Small bruises in the lining of the mouth, Pale skin, Signs of extra water loss (dehydration).
- #8 Hemolytic Uremic Syndrome in Children | Children’s Hospital of Philadelphiahttps://www.chop.edu/conditions-diseases/hemolytic-uremic-syndrome-children
HUS is a rare condition that can lead to kidney failure in children. Kidney failure develops as a result of destruction of the small, functional structures and vessels inside the kidney. HUS is a serious illness and potentially fatal. However, more than 85 percent of patients with the most common form of HUS recover complete kidney function. […] The initial symptoms of HUS frequently last from one to 15 days and may include symptoms in the digestive tract, such as the following: Abdominal pain, Bloody or watery diarrhea, Vomiting, Fever. […] Severe problems in the bowel and colon may develop in some cases. In these cases, even if the digestive symptoms are no longer present, a child may still exhibit the following symptoms: Irritability, Fatigue, Small, unexplained bruises visible in the lining of the mouth, Pale skin, Dehydration.
- #9 Hemolytic Uremic Syndrome (HUS): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/16470-hemolytic-uremic-syndrome
Hemolytic uremic syndrome (HUS) is a condition that damages blood vessels in your kidneys. In most cases, symptoms include diarrhea, abdominal pain, high blood pressure and peeing less than usual. […] Hemolytic uremic syndrome affects your kidneys and typically cause abdominal symptoms, pale skin, bruises and headaches. Hemolytic uremic syndrome typically causes abdominal symptoms, including diarrhea. Severe HUS can damage your kidneys. […] The most common symptoms of hemolytic uremic syndrome include: Diarrhea, usually bloody. Abdominal pain. Nausea and vomiting. Headache. Chills and fever. […] In severe cases of HUS, damaged red blood cells can cause blood clots that damage your organs, especially your kidneys. This affects how well your kidneys can filter and eliminate waste from your body. If your kidneys cant filter and eliminate waste, it can lead to acute kidney injury (AKI). AKI symptoms include: Blood in your pee (hematuria). Increased blood toxin levels, which can make you feel generally sick. High blood pressure. Shortness of breath (dyspnea). Swelling (edema), especially in your legs, feet or ankles. Peeing less than expected (oliguria).
- #10 Hemolytic Uremic Syndrome (HUS)https://healthweb-back.health.ny.gov/diseases/communicable/e_coli/hus.htm
HUS is a rare but serious disease that affects the kidneys and blood clotting functions of infected people. Infection with HUS causes destruction of red blood cells, which can then cause kidney failure. HUS occurs as a complication of a diarrheal infection (usually E.coli O157:H7 infection). The disease occurs more commonly in children under 5 years of age than in other age groups. […] Symptoms of HUS include vomiting, bloody diarrhea (loose stool/poop), stomach pain, fever, chills, and headache. As infection progresses, persons may experience fatigue, weakness, fainting, bruising, and paleness. Complications may occur leading to high blood pressure, seizures, blood-clotting problems, kidney disease, stroke, or coma. […] Treatment for HUS infection typically involves hospitalization, and may include special diets, blood transfusions, and kidney dialysis. Most people recover completely from HUS, but in some cases, it can be fatal.
- #11 Haemolytic uraemic syndromehttps://dermnetnz.org/topics/haemolytic-uraemic-syndrome
Haemolytic uraemic syndrome (HUS), a form of thrombotic microangiopathy, is defined by the clinical triad of thrombocytopenia, anaemia, and acute kidney damage. […] Haemolytic uraemic syndrome presents with: Pallor, Acute kidney failure oedema, nausea and vomiting, reduced urine output, high blood pressure, Extrarenal manifestations such as shortness of breath and neurological symptoms and signs (irritability, confusion, seizures, decreased level of consciousness). […] Onset of abdominal pain and bloody diarrhoea, which can be severe, 47 days after ingestion of contaminated food or water. […] Abrupt onset of HUS symptoms and signs 210 days later. […] Usually triggered by a respiratory or gastrointestinal febrile illness. […] Onset of HUS manifestations usually less abrupt than in typical HUS. […] Haemolytic uraemic syndrome can cause acute and/or end-stage renal failure. Most patients with Shiga toxin-related HUS recover fully. Poor prognostic signs include neurological symptoms, low neutrophil count, low platelet count, and prolonged anuria.
- #12 Hemolyticâuremic syndrome – Wikipediahttps://en.wikipedia.org/wiki/Hemolytic%E2%80%93uremic_syndrome
Early: Bloody diarrhea, vomiting, fever Later: Low platelets, low red blood cells, kidney failure. […] Initial symptoms typically include bloody diarrhea, fever, vomiting, and weakness. […] Kidney problems and low platelets then occur as the diarrhea progresses. […] Complications may include neurological problems and heart failure. […] HUS typically develops about 5 to 10 days after the first symptoms, but can take up to 3 weeks to manifest, and occurs at a time when the diarrhea is improving. […] Related symptoms and signs include lethargy, decreased urine output, blood in the urine, kidney failure, low platelets, and destruction of red blood cells. […] Failure of neurologic, cardiac, renal, and gastrointestinal organs, as well as death, can occur unpredictably at any time, either very quickly or following prolonged symptomatic or asymptomatic disease progression. […] Acute renal failure occurs in 55-70% of people with STEC-HUS, although up to 70-85% recover renal function. […] Patients with aHUS generally have poor outcomes, with up to 50% progressing to end-stage renal disease (ESRD) or irreversible brain damage; as many as 25% die during the acute phase.
- #13 Haemolytic uraemic syndrome (HUS): symptoms and treatmentshttps://www.kidneyresearchuk.org/conditions-symptoms/haemolytic-uraemic-syndrome/
Haemolytic uraemic syndrome (HUS) is a rare kidney condition. Common features of HUS include: Anaemia â a shortage of red blood cells. In HUS this is caused by the destruction of red blood cells. A low platelet count. Platelet cells are involved with blood clotting and a shortage of these cells can cause bruising on your skin and excessive bleeding. Kidney failure. This is caused by damage to tiny blood vessels within your kidneyâs filtering system which become blocked by blood clots. Severe HUS can also affect the brain, also the gut, heart, liver and pancreas, although when it does this it begins to look more like a related condition known as TTP (thrombotic thrombocytopenic purpura). HUS typically develops a few days after a bout of bloody diarrhoea. Regardless of the type of HUS, the symptoms of HUS are similar and can include: Reduced urine production, Tiredness, Poor appetite, Swelling, Bruising. The outlook for patients with STEC-HUS is generally good and for most patients, there is recovery with little evidence of kidney damage. However, HUS is a serious disease and some people can have severely damaged kidneys or die as a result of HUS. After recovery, you may be left with mild permanent kidney damage, but sometimes it can be more severe. In the most severe cases, other organs may be damaged too. The outlook for patients with atypical HUS used to be poor, with many patients needing long-term dialysis with a high risk of the disease returning if transplanted. New treatments have become available that have improved outcomes in recent years. […] In STEC-HUS the first likely symptoms are the same as those caused by severe gastroenteritis: Bloody diarrhoea, Vomiting (sometimes), Severe abdominal pain. In atypical HUS, patients sometimes report a âtriggerâ such as minor illness or diarrhoea.
- #14 Hemolytic Uremic Syndrome in Children – NIDDKhttps://www.niddk.nih.gov/health-information/kidney-disease/children/hemolytic-uremic-syndrome
Hemolytic uremic syndrome, or HUS, is a kidney condition that happens when red blood cells are destroyed and block the kidneys’ filtering system. […] Hemolytic uremic syndrome is the most common cause of acute kidney injury in children. […] A child with hemolytic uremic syndrome may develop signs and symptoms similar to those seen with gastroenteritis such as vomiting, bloody diarrhea, abdominal pain, fever and chills, and headache. […] As the infection progresses, the toxins released in the intestine begin to destroy red blood cells. […] When the red blood cells are destroyed, the child may experience the signs and symptoms of anemia a condition in which red blood cells are fewer or smaller than normal, which prevents the body’s cells from getting enough oxygen. […] As the damaged red blood cells clog the glomeruli, the kidneys may become damaged and make less urine.
- #15 Hemolytic uremic syndrome (HUS) | Altru Health Systemhttps://www.altru.org/health-library/conditions/hemolytic-uremic-syndrome-hus
Symptoms of these changes include: Loss of color in the skin. Extreme tiredness. Easy bruising. Unusual bleeding, such as bleeding from the nose and mouth. Decreased urinating or blood in the urine. Swelling, called edema, of the legs, feet or ankles. Swelling occurs less often in the face, hands, feet or entire body. Confusion, seizures or stroke. High blood pressure. […] See a member of your health care team right away if you or your child has bloody diarrhea or several days of diarrhea followed by: Urinating less. Swelling. Bruising. Unusual bleeding. Extreme tiredness. […] Hemolytic uremic syndrome can cause life-threatening complications, including: Kidney failure, which can be sudden, called acute, or happen over time, called chronic. High blood pressure. Stroke or seizures. Coma. Clotting problems, which can lead to bleeding. Heart problems. Digestive tract problems, such as problems with the intestines, gallbladder or pancreas.
- #16 Hemolytic-uremic syndrome – UF Healthhttps://ufhealth.org/conditions-and-treatments/hemolytic-uremic-syndrome
STEC-HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. People with this condition may urinate less than normal. Urine output may almost stop. […] Red blood cell destruction leads to symptoms of anemia. […] Early symptoms: Blood in the stools, Irritability, Fever, Lethargy, Vomiting and diarrhea, Weakness. […] Later symptoms: Bruising, Decreased consciousness, Low urine output, No urine output, Pallor, Seizures — rare, Skin rash that looks like fine red spots (petechiae).
- #17 Hemolytic Uremic Syndrome (HUS) Mnemonic for USMLEhttps://pixorize.com/view/7127/image
HUS, or hemolytic uremic syndrome, is a disorder characterized by hemolysis and uremia. HUS is caused by shiga-like toxins created by the EHEC bacteria. The consumption of platelets to form microthrombi leads to thrombocytopenia. These microthrombi in small vessels can shear passing red blood cells to induce hemolysis, in what is formally known as microangiopathic hemolytic anemia, or MAHA for short. Microthrombi can also block flow to the small vessels of the kidney, leading to acute kidney injury. Other symptoms to look out for include mucocutaneous bleeding and a history of bloody diarrhea. On laboratory testing, patients with HUS will usually have decreased platelet and red blood cell counts and increased bleeding times. Schistocytes may be seen on peripheral blood smear. […] Classically seen in children. […] Triad of: Thrombocytopenia, Microangiopathic hemolytic anemia (MAHA), Acute kidney injury. […] History of bloody diarrhea associated with EHEC or Shigella. […] Mucocutaneous bleeding: Gingival bleeding, menorrhagia, petechiae, epistaxis.
- #18https://step2.medbullets.com/heme/120243/hemolytic-uremic-syndrome-hus
A 7-year-old boy recently had several episodes of bloody diarrhea. Desperate, his parents called his primary care physician, who reassured them that the infection is self-limited. Still concerned, his parents bought some diarrhea pills (anti-motility agents). A week later, he presents to the ER with jaundice, abdominal pain, and easy bruising. Lab results come back with low platelet count, anemia, and increased creatinine. A peripheral blood shows schistocytes. He is started on IV hydration. […] Symptoms include prodromal gastrointestinal illness, abdominal pain, bloody diarrhea, nausea, vomiting, fatigue (anemia), and oliguria/anuria (renal failure). […] Physical exam findings may include pallor (anemia), jaundice (hemolysis), petechiae/purpura (uncommon), and hepatosplenomegaly. […] The classic triad of HUS consists of thrombocytopenia (first), nonimmune microangiopathic hemolytic anemia (second), and acute renal failure (hence, uremia in title) (third).
- #19https://step1.medbullets.com/hematology/109081/hemolytic-uremic-syndrome-hus
A 4-year-old girl is admitted to the floor with a several day history of bloody diarrhea. Other members of the daycare she attends also had bloody diarrhea. She is irritable and lethargic. Her skin has also turned slightly yellow. Her arms have multiple petechiae. Lab results show creatinine of 4.0 mg/dL, platelet of 40,000/mm3, and hemoglobin of 7 g/dL. A peripheral blood smear shows schistocytes. […] Symptoms include prodromal gastrointestinal illness, abdominal pain, bloody diarrhea, nausea, vomiting, and fatigue (anemia). […] Physical exam may reveal pallor (anemia) and jaundice (hemolysis). […] Evaluation includes complete blood count showing anemia and thrombocytopenia, peripheral smear showing schistocytes (helmet cells), serum creatinine, bleeding time, and normal PT/PTT. […] Prognosis includes 3-5% mortality and 5% severe sequelae such as end-stage renal failure. […] Complications may include chronic kidney disease and electrolyte abnormalities.
- #20 Hemolytic Uremic Syndrome | Children’s Wisconsinhttps://childrenswi.org/medical-care/nephrology/conditions/hemolytic-uremic-syndrome
HUS is a rare condition that can lead to kidney failure in children. Kidney failure develops as a result of destruction of the small, functional structures and vessels inside the kidney. HUS is a serious illness and potentially fatal. […] The following are the most common symptoms of HUS. However, each child may experience symptoms differently. […] The initial symptoms of HUS frequently last from one to 15 days and may include symptoms in the digestive tract such as the following: Abdominal pain, Bloody or watery diarrhea, Vomiting. […] Severe problems in the bowel and colon may develop in some cases. In these cases, even if the digestive symptoms are no longer present, a child may still exhibit the following symptoms: Irritability, Fatigue, Small, unexplained bruises visible in the lining of the mouth, Pale skin, Dehydration.
- #21 Hemolytic Uremic Syndrome in Children – NIDDKhttps://www.niddk.nih.gov/health-information/kidney-disease/children/hemolytic-uremic-syndrome
When hemolytic uremic syndrome causes acute kidney injury, a child may have the following signs and symptoms: edema, albuminuria, decreased urine output, hypoalbuminemia, blood in the urine. […] Most children who develop hemolytic uremic syndrome and its complications recover without permanent damage to their health. […] However, children with hemolytic uremic syndrome may have serious and sometimes life-threatening complications, including acute kidney injury, high blood pressure, blood-clotting problems that can lead to bleeding, seizures, heart problems, chronic kidney disease, stroke, coma. […] If necessary, a health care provider will treat acute kidney injury with dialysis the process of filtering wastes and extra fluid from the body with an artificial kidney. […] Some children may sustain significant kidney damage that slowly develops into chronic kidney disease.
- #22 Pediatric hemolytic uremic syndrome (HUS) â Childrenâs Health Nephrologyhttps://www.childrens.com/specialties-services/conditions/hemolytic-uremic-syndrome
Pediatric hemolytic uremic syndrome (hemolytic – uremic – uremia) or HUS occurs when tiny tubes in the kidneys become clogged with damaged red blood cells, impairing kidney function. […] Hemolytic uremic syndrome is a condition that causes damaged red blood cells to clog tiny tubes in the kidney, called glomeruli. In healthy kidneys, glomeruli, separate waste and clean the blood. When they become clogged, they do not function properly, which can lead to kidney injury. […] The first symptoms of hemolytic uremic syndrome are vomiting and diarrhea. After five to 10 days, children will show additional signs of HUS, as bacteria continues to damage the blood. […] These symptoms include: Bloody diarrhea, Decreased or no urination, Fever, Irritability, Pale skin, Seizures (rare symptom), Swelling of the face, hands, feet or any other part of the body as the body retains fluid, Unexplained bruising or bleeding, Weakness and lethargy (extreme exhaustion).
- #23 Treatment for HUS | Marler Clarkhttps://marlerclark.com/foodborne-illnesses/hemolytic-uremic-syndrome/hus-treatment
Reduced or absent urine output (oligoanuria) occurs in most cases of HUS. […] While urinary output usually rebounds after a week, the duration of oligoanuria can be variableas brief as two to three days, or up to a month or longer. […] Seizures are the most dramatic central nervous system (CNS) manifestation of HUS; they are more likely to occur in children. […] The incidence is greater in toddlers (30%) than in older children (15%). […] Unfortunately, structural damage to the brainthrough stroke or swellinghas not decreased over time. […] If cerebral edema becomes severe, an increase in intracranial pressure can strangulate the brainstem, causing rapid death. […] Fortunately, most children recover normally from HUS and do not have long-term complications from CNS involvement. […] Studies have shown that children discharged without neurologic injury do not demonstrate an increased risk of subclinical problems with learning behavior or attention; however, some children with major neurologic symptoms have exhibited subtle neurologic sequelae, including clumsiness, poor fine motor coordination, hyperactivity, and distractibility.
- #24 Haemolytic uraemic syndrome | Better Health Channelhttps://www.betterhealth.vic.gov.au/health/conditionsandtreatments/haemolytic-uraemic-syndrome
Children under five years of age are at greatest risk of developing HUS. The onset of illness usually starts with abdominal pains and bloody diarrhoea, which generally lasts about a week. After this time, the child becomes lethargic and passes decreasing amounts of urine. It usually takes between two and eight days after the bacteria are taken in by mouth for the first symptoms to appear. Small numbers of bacteria can cause illness. […] Other characteristic features of HUS include: Hypertension (high blood pressure), Oliguria (less urine production), lasting for about one week. Some children actually stop producing urine for a short time, Jaundice, Seizures these affect about 20 per cent of children who develop HUS, Bleeding into the skin. […] HUS can lead to: Mild, chronic kidney damage occurs in about 50 per cent of cases. Kidney failure occurs in five per cent of cases. Stroke HUS is a significant cause of stroke in infants and young children. […] About five per cent of children who develop HUS will die. Children under one year or older than five years are at the greatest risk of death. The elderly, if affected, are also at serious risk of death.
- #25 Hemolytic Uremic Syndrome: An Emerging Health Risk | AAFPhttps://www.aafp.org/pubs/afp/issues/2006/0915/p991.html
Acute renal failure results when micro-thrombi are deposited in kidney parenchyma. This manifests in the form of hypertension associated with oliguria and anuria, which are early signs of acute renal failure. […] Approximately 10 percent of patients with hemolytic uremic syndrome develop central nervous system problems and subsequent coma, hemiparesis, or stroke.
- #26 Hemolytic Uremic Syndrome – Core EMhttps://coreem.net/core/hemolytic-uremic-syndrome/
Clinical triad of thrombocytopenia, microangiopathic hemolytic uremia, and acute kidney injury […] Patients commonly have a history of gastroenteritis symptoms, including watery diarrhea that subsequently becomes bloody […] HUS usually occurs 1 week after diarrhea, with oligoanuric acute kidney injury […] Other symptoms include hypertension (owing to fluid retention and edema) and manifestations of thrombocytopenia and anemia […] Extrarenal manifestations: Neurologic: including encephalopathy, seizures, coma […] Cardiovascular: ischemia, cardiac failure […] Endocrine: diabetes […] Gastrointestinal: pancreatitis, cholecystitis, necrosis, perforation, stricture formation […] Mortality rates are up to 10%, and up to 40% of children may develop long-term complications such as hypertension, proteinuria, and renal complications (including ESRD).
- #27 Hemolytic Uremic Syndrome: An Emerging Health Risk | AAFPhttps://www.aafp.org/pubs/afp/issues/2006/0915/p991.html
Hemolytic uremic syndrome is caused primarily by Shiga toxinproducing Escherichia coli O157:H7. The most common cause of acute renal failure in children, hemolytic uremic syndrome also can occur in adults. Characteristic features of the syndrome are microangiopathic anemia, thrombotic thrombocytopenia, and renal failure. Although the presentation of this syndrome is diverse, the classic prodromal illness is bloody diarrhea following ingestion of hamburger meat contaminated with E. coli O157:H7, the most common mode of infection in the United States. Children with hemolytic uremic syndrome generally present with gastro-enteritis complaints (e.g., abdominal pain or tenderness, nausea or vomiting, fever, anemia); affected adults may be asymptomatic. Complications from hemolytic uremic syndrome can include intussusception, chronic renal failure, and seizures in severe cases.
- #28 Hemolytic-Uremic Syndrome (HUS) – Hematology and Oncology – Merck Manual Professional Editionhttps://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/hemolytic-uremic-syndrome-hus
Hemolytic-uremic syndrome (HUS) is an acute, fulminant disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. HUS usually occurs in children following an infection, typically with Shiga toxin-producing bacteria (eg, Escherichia coli O157:H7), but may also occur in adults. […] Children with Shiga toxin-related hemolytic-uremic syndrome (HUS) usually have a prodrome of vomiting, abdominal pain, and diarrhea (frequently bloody) and often a history of exposure to infection. […] About a week after the prodrome, manifestations of hemolytic anemia, thrombocytopenia, and acute kidney injury develop. […] Manifestations of ischemia develop with varying severity in multiple organs. Neurologic manifestations occur in about one quarter of patients and include weakness, confusion, and seizures. Kidney injury may produce hematuria, decreased urination or anuria, and/or hypertension.
- #29 Haemolytic uraemic syndrome (STEC HUS) | Kidney Care UKhttps://kidneycareuk.org/kidney-disease-information/kidney-conditions/haemolytic-uraemic-syndrome-stec-hus/
Haemolytic uraemic syndrome (HUS) is a rare condition that damages the small blood vessels inside the kidneys. […] STEC HUS can occur after a stomach infection caused by the bacteria Shiga toxin-producing Escherichia coli (STEC), a type of E.coli. […] STEC HUS affects around 1 in 100,000 people in the UK. There are about 100 new cases a year. It usually affects children under the age of ten. […] The first symptoms of STEC HUS are linked to gastroenteritis (commonly called a stomach bug) and include: diarrhoea that lasts for several days, urinating less than normal or not at all, feeling and/or being sick, stomach pain, high temperature (over 38C), often with excessive sweating and/or shivers. […] Blood may appear in the poo after a few days of diarrhoea. […] Excessive tiredness and anaemia can develop after about a week.
- #30 Frontiers | Short- and Long-Term Renal Outcome of Hemolytic-Uremic Syndrome in Childhoodhttps://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2018.00220/full
Hemolytic-uremic syndrome (HUS) is defined by a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury and belongs to the heterogeneous group of thrombotic microangiopathy (TMA) (1â3). HUS is a rare disease with the highest incidence in young childhood (6/100,000 children <5 years) in Western Europe and USA (2, 4), where it is a common cause for intrarenal acute kidney injury (AKI) (5). Most cases occur sporadically, but epidemic outbreaks are seen from time to time (e.g., Germany, 2011) (6). Traditionally, HUS was classified by clinical presentation in prodromal phase as Diarrhea-positive (D+) or typical HUS and Diarrhea-negative (Dâ) or atypical HUS. The former primarily resulted from Shigatoxin-producing Escherichia coli (STEC) infections and accounts for more than 90% of HUS cases in childhood and adolescence (7). All other cases were summarized as atypical, Diarrhea-negative HUS, even though some of these patients also presented with diarrhea. A novel etiology-based classification has evolved (8), which was recently adapted to our national guideline. STEC-HUS is usually caused by an infection with STEC or, less frequent, Shigella dysenteriae Type 1. Typically, affected children are between 2 and 5 years of age. Patients with STEC-HUS present with diarrhea, often bloody, in the prodromal phase. Renal impairment occurs usually 7â14 days after infection. Extrarenal complications occur more frequently in central nervous system, but are also seen in pancreas, gastrointestinal tract and cardiorespiratory system (4, 9, 10). Mortality in the acute phase is low (<5%) (15), but renal impairment persists in up to 30% of patients with STEC-HUS (12, 16). Although renal outcome has improved over the investigated last decades, patients with HUS still face a high risk of permanent renal damage. These findings underline the importance of a consequent long-term follow-up in HUS-patients. Renal sequelae persisted more often in the group 1976â1995 (39.3%) than in the group 1996â2014 (28.3%), but more than 50% of all patients were lost to follow-up.
- #31 Azthena logo with the word Azthenahttps://www.news-medical.net/health/Hemolytic-Uremic-Syndrome-Management-and-Complications.aspx
Acute renal failure in young children is most commonly linked to hemolytic uremic syndrome (HUS). In most cases, HUS is self-limited, and 95% of children recover with effective supportive treatment. The symptoms of prodromal gastroenteritis may be agonizing, yet this is usually self-limited. In atypical HUS, sometimes caused by genetic mutations in 5% of children, the illness is the result of abnormal complement system activation. Oliguria or anuria usually occurs, with marked shifts in the fluid-electrolyte balance, leading to a requirement for renal replacement in 50-70% of patients. 5% of patients may die, most commonly during the acute renal injury phase. Factors which predict the onset of complications and death include: Oliguria persisting beyond 10 days, Anuria for more than 5 days, Dehydration in the acute phase, High total count above 20 000 per mm3, Hematocrit over 23%. This is found in 20-50% of children with HUS. Many patients may have permanent neurologic damage. In up to a quarter of patients, complications persist for life, such as hypertension and chronic renal failure, occurring even 20 years after an apparently complete recovery.
- #32 Haemolytic uraemic syndrome (HUS) | healthdirecthttps://www.healthdirect.gov.au/haemolytic-uraemic-syndrome-hus
If you have HUS, you may need intensive care in hospital, generally for about 1 to 2 weeks. You may need intravenous fluids (IV) and a blood transfusion. If your kidneys aren’t working properly, you may need dialysis. […] Long-term complications of HUS can include kidney failure, high blood pressure and seizures. If you have not passed urine for around 10 to 14 days, or need a long period of dialysis, you have a greater risk of long-term kidney problems.
- #33 Hemolytic Uremic Syndrome (HUS): Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/16470-hemolytic-uremic-syndrome
With proper diagnosis and care, many people with HUS make a full recovery without any permanent damage to their health. But you have an increased chance of developing high blood pressure or other conditions that may affect your kidneys in the future. Severe cases of HUS can be fatal without treatment, especially if HUS affects other organs like your brain. […] More than 85% of people with HUS recover complete kidney function. But, even if you make a full recovery, you may have high blood pressure or other kidney problems in the future.
- #34 Hemolytic Uremic Syndrome – familydoctor.orghttps://familydoctor.org/condition/hemolytic-uremic-syndrome/
Most people who become infected with E. coli wonât get HUS. If you or your child do, you will need to go to the hospital. Your doctor will want to watch your kidney function and make sure you keep enough fluids in your body. […] Most people who are diagnosed with HUS make a full recovery. But itâs a serious illness. If you or your child have significant damage to your kidneys, you may need regular transfusions or dialysis. Left untreated, HUS can cause death. Children with HUS tend to have better outcomes than adults.
- #35 Hemolytic Uremic Syndrome – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK556038/
HUS symptoms include renal symptoms, such as anuria, oliguria, and fluid overload, and symptoms related to anemia, such as syncope, fatigue, and pallor. Petechiae and easy bleeding may be notable due to thrombocytopenia. […] Extrarenal manifestations are common with both typical and atypical HUS. Neurologic and cardiac symptoms are often responsible for much of the morbidity and mortality of HUS. […] The course of aHUS has traditionally been much less benign than typical HUS; however, the advent of eculizumab treatment has decreased progression to ESRD or death in children from 30% to 50% down to 9% and in adults from 60% to 6% down to 15%.