Symptoma

Hemolytic Uremic Syndrome

Gasser Syndrome

Hemolytic uremic syndrome usually begins with diarrhea and vomiting that contains blood. In addition, to these the following signs and symptoms are also present:

In the later stages, affected individuals may also experience seizures, though this is very rare.

  • Easy Bruising

    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. [step2.medbullets.com]

  • Anemia

    It is characterized by hemolytic anemia (anemia, hemolytic); thrombocytopenia; and acute renal failure Use Additional ICD-9-CM Volume 2 Index entries containing back-references to 283.11 : [icd9data.com]

    Typical management of STEC-HUS patients relies on supportive care of electrolyte and water imbalance, anemia, hypertension and renal failure. [ncbi.nlm.nih.gov]

  • Fever

    Fever, tachycardia, a rising white blood cell count, and abdominal pain out of proportion to examination are key findings for which physicians should be vigilant. [ncbi.nlm.nih.gov]

    Some individuals with TTP may have neurological symptoms and fever. [healthcommunities.com]

    When fever and CNS manifestations coexist, the distinction between HUS and thrombotic thrombocytopenic purpura (TTP) can be difficult. Epidemiology HUS has been characterized as either epidemic/endemic (or prodromal) or sporadic (nonprodromal). [doi.org]

  • Fatigue

    Symptoms Signs and symptoms of HUS can include: Bloody diarrhea Decreased urination or blood in the urine Abdominal pain, vomiting and occasionally fever Pallor Small, unexplained bruises or bleeding from the nose and mouth Fatigue and irritability Confusion [mayoclinic.org]

    Symptoms of HUS include extreme irritability and fatigue, paleness of the skin, and a decrease in urine output. [ok.gov]

    Transfusions can relieve symptoms associated with low RBC counts, such as shortness of breath and extreme fatigue. [healthline.com]

    In these cases, even if the gastroenteritis has stopped, a child may still have these symptoms: Irritability Fatigue Small, unexplained bruises or small, clot-sized hemorrhages visible in the mucosal lining of the mouth Paleness The child may produce [hopkinsmedicine.org]

  • HELLP Syndrome

    A pregnancy complicated by typical hemolytic uremic syndrome (HUS) and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome is reported. [ncbi.nlm.nih.gov]

    The question of whether HELLP syndrome is a TMA is relevant because if the link between HELLP syndrome and complement dysregulation is confirmed, complement inhibition may represent a treatment for severe HELLP syndrome. [doi.org]

    HELLP Syndrome. Lab Med 1997; 28:500-502. Kwaan HC, Ganguly P. Introduction: Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome. Sem. In Hematol. 34(2) April 1992, 81-82. Kwaan, Hau C. Soff, Gerald A. [path.upmc.edu]

    syndrome (hemolysis, elevated liver enzymes, and low platelet count). [clinicaladvisor.com]

    HELLP syndrome ( H aemolysis, E levated L iver enzymes, Low P latelet count). An autosomal dominant form of HUS exists with abnormality of the ADAMTS13 gene which encodes the von Willebrand factor. [patient.info]

  • Pallor

    However, eight days post delivery, the patient developed severe nausea and vomiting followed by hematuria, spontaneous bruising, marked pallor, icteric sclera, and lethargy. [ncbi.nlm.nih.gov]

    Symptoms Symptoms of HUS include bloody diarrhea, vomiting, anemia, blood in urine, decreased urinary output, fatigue, lethargy, confusion, pallor, irritability, edema and fever. [symptoma.com]

    […] factors use of antimotility agents in EHEC diarrhea use of antibiotics in EHEC diarrhea Presentation Symptoms prodromal gastrointestinal illness abdominal pain bloody diarrhea nausea vomiting fatigue (anemia) oliguria/anuria (renal failure) Physical exam pallor [step2.medbullets.com]

    Symptoms Signs and symptoms of HUS can include: Bloody diarrhea Decreased urination or blood in the urine Abdominal pain, vomiting and occasionally fever Pallor Small, unexplained bruises or bleeding from the nose and mouth Fatigue and irritability Confusion [mayoclinic.org]

  • Severe Clinical Course

    Moreover, a shorter period between the onset of diarrhea and a diagnosis of D   HUS indicated a more severe clinical course or long-term sequelae, and it should be considered as a risk factor for poor prognosis. [ncbi.nlm.nih.gov]

  • Diarrhea

    Diarrhea-associated hemolytic uremic syndrome (HUS) is characterized by acute kidney injury with microangiopathic hemolytic anemia and thrombocytopenia with a diarrhea prodrome, typically caused by Shiga-like toxin-producing Escherichia coli. [ncbi.nlm.nih.gov]

    Eleven of 45 attendees with diarrhea had positive tests (stool culture or shiga-like toxin assay) for E. coli O157:H7. Two of these 11 (18%) progressed to hemolytic uremic syndrome. [oadoi.org]

    Once the bacteria colonizes, diarrhea followed by bloody diarrhea, hemorrhagic colitis, typically follows. [en.wikipedia.org]

  • Vomiting

    Hemolytic uremic syndrome usually begins with diarrhea and vomiting that contains blood. [symptoma.com]

    However, eight days post delivery, the patient developed severe nausea and vomiting followed by hematuria, spontaneous bruising, marked pallor, icteric sclera, and lethargy. [ncbi.nlm.nih.gov]

    These early symptoms can include diarrhea (which is often bloody), stomach cramps, mild fever, or vomiting that results in dehydration and reduced urine. [en.wikipedia.org]

    Symptoms of HUS include vomiting and diarrhea (often bloody), weakness, lethargy, and bruising (purpura). [medicinenet.com]

  • Abdominal Pain

    Fever, tachycardia, a rising white blood cell count, and abdominal pain out of proportion to examination are key findings for which physicians should be vigilant. [ncbi.nlm.nih.gov]

    Additionally, abdominal pain and tenderness could mimic appendicitis or an acute abdomen. [aafp.org]

    A 50-year-old female, after presenting with diarrhea and abdominal pain, developed pancolitis, acute renal failure, and thrombocytopenia. The patient underwent total abdominal colectomy. [doi.org]

  • Nausea

    However, eight days post delivery, the patient developed severe nausea and vomiting followed by hematuria, spontaneous bruising, marked pallor, icteric sclera, and lethargy. [ncbi.nlm.nih.gov]

    Nausea and vomiting is present in approximately half of the patients. Most patients recover in 7-10 days, but some (6%) go on to have HUS. This is most likely to happen in children and the elderly. [medicinenet.com]

    Adverse Reactions The most frequently reported adverse reactions in the PNH randomized trial ( 10% overall and greater than placebo) are: headache, nasopharyngitis, back pain, and nausea. [alexion.com]

    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. [aafp.org]

    Some of the signs and symptoms of aHUS include: confusion; diarrhea, nausea and vomiting; shortness of breath; fatigue; heart symptoms and kidney symptoms. Certain lab tests can help your doctor determine if you may have aHUS. [enerca.org]

  • Hemorrhagic Diarrhea

    As many as 82% of the household contacts of a child with IStx- E coli HUS (many of whom have hemorrhagic diarrhea) have Stx bound to their PMNs. Despite this binding, such individuals often do not have evidence of renal dysfunction. [emedicine.medscape.com]

    Pathogen and disease overview EHEC was first described in 1983 and has been associated with 2 outbreaks of hemorrhagic diarrhea in 2 cities in the USA; the commonality between these 2 outbreaks was poorly cooked hamburger meat in fast food restaurants [doi.org]

  • Jaundice

    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. [step2.medbullets.com]

    […] the following signs and symptoms are also present: Stools contain blood Fever Lethargy Passing out blood in urine or very less urinary output Weakness Irritability Confusion Pain in the abdomen Development of swelling in the arms, feet and face Pallor Jaundice [symptoma.com]

    In addition to the usual signs of anemia, the patient may also exhibit jaundice. hemolytic component a degree of extravascular hemolysis in association with other types of anemia. hemolytic disease of the newborn see alloimmune hemolytic anemia of the [medical-dictionary.thefreedictionary.com]

     LATER SYMPTOMS: • Bruising • Decreased consciousness • Low urine output • No urine output • Pallor • Seizures -- rare • Skin rash that looks like fine red spots (petechiae) • Yellow skin (jaundice) 10. [slideshare.net]

  • Hypertension

    Overhydration, hypertension, anemia, or dyselectrolytemias sometimes cause cardiovascular impairment in childhood hemolytic-uremic syndrome. [ncbi.nlm.nih.gov]

    […] of Miami, Miami, FL; and 2 Division of Nephrology and Hypertension, Albany Medical College, Albany, NY. [journals.lww.com]

  • Tachycardia

    Fever, tachycardia, a rising white blood cell count, and abdominal pain out of proportion to examination are key findings for which physicians should be vigilant. [ncbi.nlm.nih.gov]

    At that time, she exhibited moderate dehydration, pallor, drowsiness, and a generalized seizure of 10 to 15 min duration, tachycardia, tender and tense abdominal wall, and a history of oligoanuria for the last 48 h. Blood pressure was 128/67 mm Hg. [doi.org]

    Blood cultures were drawn and an antibiotic was given based on a body temperature of 38.9 C and tachycardia. The patient was started at 4 g piperacillin-tazobactam 3 times per day intravenously (i.v.). The cultures did not show any bacterial growth. [bmcresnotes.biomedcentral.com]

    His heart has a regular rhythm with tachycardia and a grade II/VI vibratory systolic ejection murmur at the left sternal border without radiation. No heaves, lifts, thrills, rubs, or gallops are present. His lungs are clear with good aeration. [hawaii.edu]

    The remaining patients from both groups all experienced minor complications, including nausea, hypotension, tachycardia, tachypnoea, dizziness, chills or oedema. [dx.doi.org]

  • Petechiae

    Physical examination is significant for petechiae on his legs and diffuse abdominal tenderness to palpation. Lab-work shows BUN 72 mg/dL, creatinine 8.1 mg/dL, and platelet count 10,000. PT and PTT are within normal limits. [medbullets.com]

    […] diarrhea use of antibiotics in EHEC diarrhea Presentation Symptoms prodromal gastrointestinal illness abdominal pain bloody diarrhea nausea vomiting fatigue (anemia) oliguria/anuria (renal failure) Physical exam pallor (anemia) jaundice (hemolysis) petechiae [step2.medbullets.com]

    In addition, petechia, purpura, and/or active bleeding are uncommon features of HUS, despite the significant low platelet count. [clinicaladvisor.com]

     LATER SYMPTOMS: • Bruising • Decreased consciousness • Low urine output • No urine output • Pallor • Seizures -- rare • Skin rash that looks like fine red spots (petechiae) • Yellow skin (jaundice) 10. [slideshare.net]

    He has no nasal flaring or palatal petechiae. His oral mucosa is moist and his tongue is pale. His neck is supple without adenopathy. [hawaii.edu]

  • Kidney Failure

    Hemolytic uremic syndrome (HUS) is a disease that causes sudden kidney failure due to destruction of the red blood cells (RBCs). The kidney failure is a temporary condition and functioning of the kidneys gets normal after rigorous treatment regime. [symptoma.com]

    […] on, 75 have been hospitalized and 20 have developed hemolytic uremic syndrome (HUS), the form of kidney failure that can be fatal. — David J. [merriam-webster.com]

    It is the most common cause of acute kidney failure in children. Several large outbreaks have been linked to undercooked hamburger meat contaminated with E coli. [nlm.nih.gov]

    "Uremic" refers to the acute kidney failure. Central nervous system problems with seizures and coma can also occur. HUS is the most common cause of acute kidney failure in infants and young children. [medicinenet.com]

    This form is the most common cause of acute kidney failure in children in Western Europe and North America. [kidneyresearchuk.org]

  • Oliguria

    CONCLUSIONS: The age of onset younger than 2 years, oliguria/anuria more than 1 week, and associated with extrarenal complications were predictive factors of poor prognosis. [ncbi.nlm.nih.gov]

    /vegetables animals at petting zoos Risk factors use of antimotility agents in EHEC diarrhea use of antibiotics in EHEC diarrhea Presentation Symptoms prodromal gastrointestinal illness abdominal pain bloody diarrhea nausea vomiting fatigue (anemia) oliguria [step2.medbullets.com]

    […] if oliguria exceeds 15 days. [marlerblog.com]

  • Dark Urine

    You have bloody or dark urine or bowel movements. You have questions or concerns about your condition or care. When should I seek immediate care or call 911? You are urinating less than usual or not at all. You have diarrhea and vomiting. [drugs.com]

    • KIDNEY DIALYSIS • PLASMA EXCHANGE • PLATELET TRANSFUSIONS • RED BLOOD CELL TRANSFUSIONS: Low RBCs result in chills, fatigue, short of breath, rapid heart rate, yellow skin and dark urine. [slideshare.net]

    Two days later, he developed increasing fatigue, and noted dark urine and jaundiced skin and sclerae. There was no history of recent travel or diarrhoea. His dietary history was unremarkable. [doi.org]

The following diagnostic procedures would be done to confirm HUS:

  • Blood tests are carried out to study platelet count, level of creatinine and RBC count. Low levels of RBCs, low platelet count and elevated levels of creatinine all point towards onset of HUS.
  • Peripheral blood smear examination by microscopy will reveal schistocytes as a marker of hemolysis among red blood cells in HUS [8].
  • Stool sample will be analyzed to check for presence of bacteria and further analysis would also be done to determine the pathogenic strain.
  • Urine analysis would determine the presence of blood and protein in urine.
  • A urine protein test is also a necessity to evaluate the levels of protein excreted in urine.

In addition to the above mentioned tests, other tests include a kidney biopsy and blood clotting tests for differential diagnosis.

  • Coombs Negative Hemolytic Anemia

    BACKGROUND: Hemolytic uremic syndrome is characterized by acute renal failure, thrombocytopenia, and Coombs-negative hemolytic anemia. [ncbi.nlm.nih.gov]

    Blood chemistry showed Coombs-negative hemolytic anemia (hemoglobin level 5.5 g/dl) with red blood cell fragmentation and thrombocytopenia (72 10 3 /μl). Serum haptoglobin was Figure 1 ). [doi.org]

    Hemolytic uremic syndrome (HUS) is a disease of nonimmune (Coombs negative) hemolytic anemia, low platelet count, and renal impairment ( 1 ). [jasn.asnjournals.org]

  • Burr Cell

    schistocytes, burr cells, or helmet cells) on peripheral blood smear, AND Renal injury (acute onset) evidenced by either hematuria, proteinuria, or elevated creatinine level (i.e., greater than or equal to 1.0 mg/dL in a child aged less than 13 years [wwwn.cdc.gov]

    Title Other Names: HUS; Acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia associated with distorted erythrocytes ('burr cells') Categories: Hemolytic uremic syndrome (HUS) is a disorder that usually occurs when an E. coli bacterial [rarediseases.info.nih.gov]

    cells, helmet cells Increased L-lactate dehydrogenase level Leukocytosis Negative Coombs’ test Proteinuria on urinalysis Reticulocyte count moderately elevated Stool culture positive for Shiga toxin–roducing Escherichia coli O157:H7 Thrombocytopenia: [aafp.org]

  • Decreased Platelet Count

    This leads to platelet activation, endothelial cell damage, and white blood cell activation, leading to systemic TMA, which manifests as decreased platelet count, hemolysis, damage to multiple organs, and ultimately, death. [en.wikipedia.org]

    platelet count Elevated blood urea nitrogen (BUN) and creatinine due to decreased kidney function Blood and protein in urine Presence of shiga toxin-producing E. coli in stool samples Treatment for hemolytic uremic syndrome Due to its severity, HUS is [davita.com]

  • Haptoglobin Decreased

    decreased hemoglobin, and/or the presence of schistocytes. [en.wikipedia.org]

  • Creatinine Increased

    Increased serum creatinine The condition results when the bacteria lodge in the digestive tract and produce toxins that can enter the bloodstream. [rarediseases.info.nih.gov]

  • Escherichia Coli 0157:H7

    HUS and TTP Associated with Escherichia Coli 0157:H7. Emerg Med News. 2004. Ruggenenti P, Remuzzi G. Treatment of adult hemolytic-uremic syndrome. Adv Nephrol Necker Hosp. 2000. 30:83-94. [Medline]. Safdar N, Said A, Gangnon RE, Maki DG. [emedicine.medscape.com]

    A continuing assessment of risk factors for the development of Escherichia coli 0157:H7-associated hemolytic uremic syndrome. Clin Nephrol 1994 ;42: 85 - 89 27. Cimolai N, Morrison BJ, Carter JE. [dx.doi.org]

  • Colitis

    The most common form of the syndrome (D HUS) occurs in healthy young children ( 6 mo to 5 y of age) and is preceded by watery diarrhea that can evolve to hemorrhagic colitis. [ncbi.nlm.nih.gov]

    Colitis Colitis Colitis Pancreatic colitis [jaoa.org]

    Some studies demonstrated a harmful effect of antibiotic therapy in hemorrhagic colitis. [dx.doi.org]

    Pathology confirmed ischemic colitis with scattered mesenteric microthrombi. Due to mental and respiratory decline, she remained intubated. Continuous venovenous hemodialysis was initiated. [doi.org]

Patients with HUS require immediate hospitalization. The following are the treatment methods administered to stabilize the condition of the patient:

  • Transfusions of red blood cells and platelets.
  • Plasma exchange [9]
  • Dialysis is done as there is acute renal failure. It is a temporary treatment method which is carried out until the kidney restores its normal functioning. If there is severe damage to the kidney or signs of chronic failure are noticed then kidney transplant may be necessary.
  • Patient is given medications such as corticosteroids.
  • Antibiotics may only be used when HUS patients are undergoing septicemia [10].

The prognosis of HUS is very favorable when appropriate treatment regime is initiated. About 70 – 80% children completely recover after treatment. Recovery period in children is faster as compared to adults.

Complications

The possible complications of HUS include the following:

Infections caused by E. coli O157:H7 lead to development of HUS [2]. Such a strain of E. coli causes gastrointestinal infections which precipitate by diarrhea and vomiting in affected children. In addition, other potent strains of E. coli like O111:H8, O103:H2, and O104:H4 have also been linked to HUS [3]. In adults, HUS is known to be triggered by the following factors:

  • Medications like quinine [4], chemotherapy drugs, anti-platelet medications and immunosuppresants can cause HUS.
  • Pregnant women are more prone to develop HUS [5].
  • Individuals with infectious diseases such as H1N1 influenza A virus and HIV/AIDS are likely to suffer from HUS [6]. 
  • Heredity can also play foul in causation of specific type of HUS known as atypical hemolytic uremic syndrome. 

Incidence of HUS peaks during the summer season and majorly affects children. It has been estimated that in United States, for children below 5 years of age, the prevalence of HUS per year has been recorded to be 6.1 cases per 100,000 population.

Hemolytic uremic syndrome is classified under two categories on the basis of its causative agent: Typical HUS and atypical HUS. In the former category, the development of the disease occurs due to toxins produced by E. coli O157:H7 and in the atypical variety, infections are not the cause. It can occur either due to genetic defect or can be sporadic in nature.

Once the pathogenic bacteria E. coli O157:H7 gains entry into the body, it produces Shiga toxin which binds with the epithelial cells lining the gut. Following this event, series of unfavorable reactions are triggered that cause destruction of red blood cells. The destroyed red blood cells then clog the filtering system in the kidney causing acute renal failure. This may further be aggravated by the loss of vascular endothelial growth factor (VEGF) in autoimmune diseases resulting to renal microvascular angiopathies [7].

Food borne cases of HUS can be prevented by following certain simple steps to avoid falling prey to these illnesses. They include:

  • Appropriately cook raw meat and avoid consumption of such high risk foods on open food stalls.
  • Raw food should not share the same platform with cooked food. Keep them separately.
  • Hands should be thoroughly washed prior to cooking and eating.
  • It is also necessary to wash and disinfect all raw produce before cooking them.
  • Sanitization of the cooking surface prior to cooking should be done.

Hemolytic uremic syndrome (HUS) is a disease that causes sudden kidney failure due to destruction of the red blood cells (RBCs). The kidney failure is a temporary condition and functioning of the kidneys gets normal after rigorous treatment regime.

HUS remains to be the most common cause of acute renal failure in children [1]. Children who contract Escherichia coli (E. coli) infection fall prey to HUS. The disease usually begins with bloody diarrhea and finally causes destruction of the red blood cells that eventually block the filtering system. Such a condition causes sudden kidney failure. With prompt diagnosis and appropriate treatment regime, majority of the children completely recover from the condition.

Definition

Hemolytic uremic syndrome (HUS) is characterized by acute renal failure due to destruction of red blood cells usually as a result of severe infection due to E coli O157:H7. Such a condition commonly strikes the children; however it can also affect adults. Contaminated raw produce, water and uncooked meat products are the source of infections.

Cause

HUS occurs due to gastrointestinal infection caused by pathogenic strain of E. coli. In addition, other causes include certain medications, infectious diseases that affect the immune system and heredity factors.

Symptoms

Symptoms of HUS include bloody diarrhea, vomiting, anemia, blood in urine, decreased urinary output, fatigue, lethargy, confusion, pallor, irritability, edema and fever.

Diagnosis

Blood tests to detect levels of RBCs, platelets and total blood count forms the preliminary diagnostic procedures for detecting HUS. Urine analysis and stool culture are some of the additional tests that are done for confirming the disease condition.

Treatment

Patient is hospitalized immediately to ease the signs and symptoms. Acute renal failure is treated through temporary dialysis and the patient is also given transfusions of platelets and red blood cells.

  1. Siegler RL. Management of hemolytic-uremic syndrome. J Pediatr. Jun 1988; 112(6):1014-20.
  2. Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet. Mar 19-25 2005; 365(9464):1073-86.
  3. Frank C, Werber D, Cramer JP, et al. Epidemic profile of Shiga-toxin-producing Escherichia coli O104:H4 outbreak in Germany. N Engl J Med. Nov 10 2011; 365(19):1771-80.
  4. Kojouri K, Vesely SK, George JN. Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: frequency, clinical features, and long-term outcomes. Ann Intern Med. Dec 18 2001; 135(12):1047-51.
  5. Wang YQ, Wang J, Jiang YH, Ye RH, Zhao YY. A case of severe preeclampsia diagnosed as post-partum hemolytic uremic syndrome.Chin Med J (Engl). 2012; 125(6):1189-92 (ISSN: 0366-6999)
  6. Rhee H, Song SH, Lee YJ, Choi HJ, Ahn JH. Pandemic H1N1 influenza A viral infection complicated by atypical hemolytic uremic syndrome and diffuse alveolar hemorrhage. Clin Exp Nephrol. 2011; 15(6):948-52 
  7. Eremina V, Jefferson JA, Kowalewska J, et al. VEGF inhibition and renal thrombotic microangiopathy. N Engl J Med. Mar 13 2008; 358(11):1129-36.
  8. Burns ER, Lou Y, Pathak A. Morphologic diagnosis of thrombotic thrombocytopenic purpura. Am J Hematol. Jan 2004; 75(1):18-21.
  9. Lapeyraque AL, Wagner E, Phan V, et al. Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations. Pediatr Nephrol. Aug 2008; 23(8):1363-6.
  10. McGannon CM, Fuller CA, Weiss AA. Different classes of antibiotics differentially influence Shiga toxin production. Antimicrob Agents Chemother. Sept 2010; 54(9):3790-3798.

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