Materiały źródłowe

• #1 Hemolytic Uremic Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK556038/

  Hemolytic uremic syndrome (HUS) is a rare but potentially serious condition, often due to infection and less commonly to genetic predisposition or other underlying conditions. Prompt recognition of HUS’s varied etiologies and manifestations is essential for timely diagnosis and intervention, optimizing patient outcomes. This activity describes the evaluation of hemolytic uremic syndrome and how to differentiate HUS from other forms of thrombotic microangiopathies. […] The diagnosis of HUS requires a high index of suspicion based on symptoms, travel history, laboratory data, and dietary history. Initial tests should include a complete blood count with differential and comprehensive blood metabolic panel. Elevated LDH and indirect bilirubin, as well as low haptoglobin and elevated plasma hemoglobin, are diagnostic of hemolytic anemia, as are schistocytes on peripheral smear. A Coombs test should be negative, with the exception of HUS caused by S pneumonia. […] All of the above tests have therapeutic value, but if clinical suspicion is high, treatment should not be delayed while waiting for all testing results, as early treatment initiation is associated with improved outcomes.

• #1 Hemolytic-Uremic Syndrome (HUS) – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/hemolytic-uremic-syndrome-hus

  Hemolytic-uremic syndrome is suspected in patients with suggestive symptoms, thrombocytopenia, microangiopathic anemia, and signs of acute kidney injury. […] If the disorder is suspected, urinalysis, peripheral blood smear, reticulocyte count, serum lactic dehydrogenase (LDH), haptoglobin, renal function tests, serum bilirubin (direct and indirect), PT, PTT, fibrinogen, and direct antiglobulin test are done. […] The diagnosis of HUS is suggested by thrombocytopenia and anemia, fragmented red blood cells on the blood smear indicative of microangiopathic hemolysis, evidence of hemolysis, negative direct antiglobulin test, absence of use of a medication that can cause thrombotic microangiopathy, and normal ADAMTS13 activity. […] Stool testing is done in children with diarrhea and also adults who had a prodrome of bloody diarrhea; however, the organism and toxin may have cleared by the time of presentation. […] In atypical cases, that is, in patients who have not had preceding infection or who have recurrent disease, testing for complement factor gene mutations is recommended.

• #1 Haemolytic uraemic syndrome – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/470

  1st tests to order include FBC, peripheral blood smear, renal function/creatinine, serum electrolytes, prothrombin time (PT), PTT, LDH, haptoglobin, stool culture on sorbitol-MacConkey agar to detect Shiga toxin-producing Escherichia coli, and polymerase chain reaction (PCR) to detect Shiga toxin 1/Shiga toxin 2.

• #1 Hemolytic-uremic syndrome laboratory findings – wikidoc

  https://www.wikidoc.org/index.php/Hemolytic-uremic_syndrome_laboratory_findings

  The classic laboratory findings in hemolytic uremic syndrome (HUS) include anemia, thrombocytopenia and acute renal injury. Anemia is microangiopathic hemolytic anemia which low hemoglobin often 8g/dl, high reticulocyte count and LDH, low haptoglobin level as well as fragmanted RBC’s and schistiocytes on peripheral blood smear (PBS). Platelets are frequently less than 60,000 without active bleeding usually and renal damage is seen in form of high creatinine, BUN, and electrolyte abnormalities. […] Following Lab findings are seen in HUS: […] Microangiopathic hemolytic anemia with features as follows: Low hemoglobin level typically 10g/dl, High reticulocyte count, Increased LDH level, Low haptoglobin level, Negative Coombs test, Peripheral blood smear shows fragmented RBC’s (Schistiocytes, Helmet, and Burr cells), PT and aPTT are normal (differentiating feature from Disseminated Intravascular Coagulation).

• #1 Hemolytic-uremic syndrome laboratory findings – wikidoc

  https://www.wikidoc.org/index.php/Hemolytic-uremic_syndrome_laboratory_findings

  Thrombocytopenia Below 150,000 although typically less than 60,000. In spite of low platelet count, active bleeding is rarely seen. […] Serum chemistry abnormalities High BUN, High creatinine, Electrolyte abnormalities may include hyponatremia, hyperkalemia, hyperphosphatemia, hypocalcemia, and acidosis (resulting from diarrhea, dehydration and renal failure), Increased Bilirubin and aminotransferases, High uric acid levels. […] Urinalysis – may show any of following Blood / Red blood cells, Protein, Bilirubin, WBC, Casts. […] Stool Testing Stool culture on Sorbitol MacConkey’s agar or detection of Shiga toxin with serological testing. […] Genetic Testing Done if suspicion of genetic or complement-mediated HUS/ recurrent HUS. However, results take weeks-month thus have no role in the acute management of disease and treatment should not be delayed while awaiting results. Low C3 and C4 may indicate complement mediated HUS. Genetic testing/screening for cobalamine metabolic defects in neonates presenting with HUS. […] Cultures Blood, spinal, organ/tissue cultures may be needed in case of suspicion of other sources of HUS for example pnemococcal infection.

• #1 Misconceptions Regarding Hemolytic Uremic Syndrome | Pediatric Emergency Medicine

  https://www.acep.org/pediatrics/education/education-articles/hemolytic-uremic-syndrome

  Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy that can be seen after diarrheal illness in young children and consists of the triad of thrombocytopenia, nonimmune hemolytic anemia, and azotemia. […] Hematological bloodwork in HUS reveals an elevated total bilirubin, reticulocytes, lactate dehydrogenase, and decreased haptoglobin. Coombs test is negative. […] The diagnosis of STEC must include both culture and nonculture-based assays such as polymerase chain reaction (PCR) to detect STEC toxins or genes. By the time HUS is diagnosed, stool cultures are less likely to be positive for E. coli. Negative immunoassay tests should be confirmed by PCR. PCR can detect the Shiga toxin gene up to 20 days after onset of abdominal symptoms. […] Children diagnosed with HUS should be transferred to a pediatric facility to be evaluated by a pediatric nephrologist and hospitalized. Management is supportive and includes intravenous (IV) fluid hydration, blood pressure management, red blood cell transfusions, and dialysis.

• #1 Hemolytic–uremic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hemolytic%E2%80%93uremic_syndrome

  Hemolyticuremic syndrome (HUS) is a syndrome characterized by low red blood cells, acute kidney injury (previously called acute renal failure), and low platelets. Initial symptoms typically include bloody diarrhea, fever, vomiting, and weakness. Kidney problems and low platelets then occur as the diarrhea progresses. […] Diagnostic method: Blood tests (to monitor levels of platelets, red blood cells, and white blood cells), stool tests (especially to check for microscopic or macroscopic levels of fresh or old blood), urinalysis (to help monitor kidney function, like blood urea nitrogen, or BUN, levels, pH, and for blood in the urine- hematuria). […] The similarities between HUS, aHUS, and TTP make differential diagnosis essential. All three of these systemic TMA-causing diseases are characterized by thrombocytopenia and microangiopathic hemolysis, plus one or more of the following: neurological symptoms (e.g., confusion, cerebral convulsions, seizures); renal impairment (e.g., elevated creatinine, decreased estimated glomerular filtration rate [eGFR], abnormal urinalysis); and gastrointestinal (GI) symptoms (e.g., diarrhea, nausea/vomiting, abdominal pain, gastroenteritis). […] Diagnostic work-up supports the differential diagnosis of TMA-causing diseases. A positive Shiga-toxin/EHEC test confirms a cause for STEC-HUS, and severe ADAMTS13 deficiency (i.e., 5% of normal ADAMTS13 levels) confirms a diagnosis of TTP.

• #1 Overview of hemolytic uremic syndrome in children – UpToDate

  https://www.uptodate.com/contents/overview-of-hemolytic-uremic-syndrome-in-children

  The hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. It is one of the main causes of acute kidney injury in children. Although all pediatric cases exhibit the classic triad of findings that define HUS, there are a number of various etiologies of HUS that result in differences in presentation, management, and outcome. […] This topic will provide an overview of the different causes, evaluation, and initial management of HUS in children. […] Thrombotic microangiopathy describes a specific pathologic lesion in which abnormalities in the vessel wall of arterioles and capillaries lead to microvascular thrombosis. […] Classification — In the past, HUS had been divided into diarrhea-positive and diarrhea-negative HUS. The former, also referred to as typical HUS, primarily resulted from Shiga toxin-producing Escherichia coli (STEC) infections, and less frequently from Shigella dysenteriae type 1 infection. All other causes of HUS were referred to as atypical HUS or assigned to the diarrhea-negative HUS, even though some patients with non-STEC-associated HUS also presented with diarrhea. […] However, ongoing research has provided a better understanding of the underlying causes of HUS, especially those due to genetic mutations in the alternative pathway of complement.

• #1 Hemolytic Uremic Syndrome: An Emerging Health Risk | AAFP

  https://www.aafp.org/pubs/afp/issues/2006/0915/p991.html

  Hemolytic uremic syndrome is caused primarily by Shiga toxinproducing Escherichia coli O157:H7. […] Early laboratory testing is important to diagnose and manage this syndrome. Obtaining a complete blood count and stool culture and performing Shiga toxin testing are the first of a series of tests that may help diagnose hemolytic uremic syndrome. […] Hemolytic uremic syndrome cannot be diagnosed without evidence of hemolytic anemia. Hematologic findings include destruction and fragmentation of erythrocytes that result in microangiopathic hemolytic anemia. This develops in all patients within a day or so of contamination and may result in respiratory and cardiovascular compromise. […] Laboratory testing can be used to secure a diagnosis of hemolytic uremic syndrome. Findings of hemolysis and thrombocytopenia on a complete blood count are required to establish the diagnosis. […] Hemolytic uremic syndrome is a reportable disease; therefore, local public health officials should be notified.

• #1 Hemolytic uremic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4523989/

  The diagnosis is made by clinical and laboratory findings of microangiopathic hemolytic anemia, thrombocytopenia and acute renal damage and demonstration of the disruption in regulation of the complement system. Decreases serum C3 level is a warning finding. C4 is normal in all patients. The definite diagnosis is made by serological and genetic tests related with the complement system. Serum CFH, CFI and CFB levels, anti-factor H antibody measurement and genetic mutations of the complement proteins are the tests performed for this objective. However, these tests are not widely used and easily applicable.

• #1 Hemolytic uremic syndrome (HUS) – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/symptoms-causes/syc-20352399

  Hemolytic uremic syndrome (HUS) is a condition that can occur when small blood vessels become damaged and inflamed. […] Hemolytic uremic syndrome can lead to kidney failure, which can be life-threatening. […] Hemolytic uremic syndrome is serious. But treating it in time leads to a full recovery for most people, especially young children. […] The most common cause of hemolytic uremic syndrome is infection with certain strains of E. coli bacteria. […] Other causes of hemolytic uremic syndrome can include: Other infections. This can include infection with pneumococcal bacteria, human immunodeficiency virus (HIV) or a flu virus. […] Seek emergency care if you or your child doesn’t urinate for 12 hours or more. […] Hemolytic uremic syndrome can cause life-threatening complications, including: Kidney failure, which can be sudden, called acute, or happen over time, called chronic. […] Hemolytic uremic syndrome caused by E.coli can occur if you: Eat meat, fruit or vegetables with the bacteria. […] The risk of getting hemolytic uremic syndrome is highest for: Children 5 or younger.

• #1 Atypical Hemolytic Uremic Syndrome (aHUS): Essential Aspects of an Accurate Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-essential-aspects-of-an-accurate-diagnosis/

  Atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA), is a rare, life-threatening, systemic disease. When unrecognized or inappropriately treated, aHUS has a high degree of morbidity and mortality. aHUS results from chronic, uncontrolled activity of the alternative complement pathway, which activates platelets and damages the endothelium. […] Clinical identification of a TMA requires documentation of microangiopathic hemolysis accompanied by thrombocytopenia. […] aHUS can be distinguished from TTP on the basis of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity, with a severe decrease characteristic of TTP. This test, as both an activity assay and an inhibitor assay, should be ordered before the initiation of plasma therapy in any patient presenting with a TMA. Finally, it is important to recognize that aHUS remains a clinical diagnosis, but in complex scenarios, tissue biopsy may be a useful adjunct in diagnosis.

• #1 Atypical Hemolytic Uremic Syndrome (aHUS): Essential Aspects of an Accurate Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-essential-aspects-of-an-accurate-diagnosis/

  The importance of rapid diagnosis of patients with aHUS cannot be stressed more highly. […] In an uncomplicated case—ie, one not associated with an ongoing complement-activating disease state that has “unmasked” aHUS—a platelet count greater than 30,000/mm³ and/or a creatinine greater than 2.3 mg/dL is highly indicative of aHUS rather than TTP. […] Two-thirds of aHUS cases are associated with an identifiable complement-activating condition. These conditions include autoimmune diseases, infection, malignant hypertension, pregnancy, organ or tissue transplant, and treatment with chemotherapeutic or anti-GVHD medications. In order to distinguish aHUS unmasked by those conditions from the laboratory and clinical signs of a TMA that simply reflects the pathology of those conditions, one must treat the complement-activating condition, and assess whether the TMA has resolved. If not, and ADAMTS13 is greater than 5% to 10% and cobalamin C deficiency has been excluded, consider that the primary cause of pathology is aHUS and treat accordingly. […] Biopsy of any highly vascular site, including the skin, gingiva, rectum, or kidney, with IHC or IFA for C5b-9 deposition on microvessels can be a useful adjunct to diagnosis in difficult cases.

• #1 Hemolytic-Uremic Syndrome Workup: Approach Considerations, Laboratory Studies, Histologic Findings

  https://emedicine.medscape.com/article/201181-workup

  Hemolytic-uremic syndrome (HUS) is primarily a clinical diagnosis, confirmed by laboratory studies showing a microangiopathic hemolytic anemia. […] Biopsy findings pathologically establish the diagnosis of HUS. However, kidney biopsy is not required in children. In adults, kidney biopsy is rarely required. […] In patients with atypical hemolytic-uremic syndrome (aHUS), Kidney Disease: Improving Global Outcomes (KDIGO) recommends genetic testing to identify an underlying hereditary abnormality. […] Laboratory findings in patients with hemolytic-uremic syndrome (HUS) may include the following: […] On complement serology testing, a decrease in both complement factor B (CFB) and CH50 may offer important support for the diagnosis of atypical HUS. […] The characteristic pathologic findings of hemolytic-uremic syndrome (HUS) are occlusive lesions of the arterioles and small arteries and consequent tissue microinfarctions. […] As a rule, changes in kidney function and the course of kidney failure correlate well with the pathologic findings in the kidney.

• #1 Hemolytic Uremic Syndrome (HUS): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/16470-hemolytic-uremic-syndrome

  Hemolytic uremic syndrome (HUS) is a condition that damages blood vessels in your kidneys. […] Treatment includes transfusions and medications. […] Hemolytic uremic syndrome (HUS) can affect anyone. […] Contact a healthcare provider if you have bloody diarrhea or diarrhea that lasts longer than three days. Without treatment, a severe case of HUS can be deadly. […] A healthcare provider will diagnose hemolytic uremic syndrome by: reviewing your medical history, asking about your family medical history, conducting a physical examination. […] If they suspect you have HUS, theyll also order tests. […] A healthcare provider may order the following tests to diagnose hemolytic uremic syndrome: pee test (urinalysis), blood test, stool (poop) test, genetic testing, kidney biopsy. […] Tests look for the presence of different things that indicate hemolytic uremic syndrome: pee tests look for blood or protein, blood tests detail your red blood cell levels and platelet levels, stool tests check for the presence of E. coli O157 and other bacteria that may cause HUS, a kidney biopsy shows damage to your kidney and sometimes the cause of the damage.

• #1 Atypical Hemolytic Uremic Syndrome (aHUS): Making the Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-making-the-diagnosis/

  Atypical hemolytic uremic syndrome (aHUS) is a major thrombotic microangiopathy (TMA). A TMA is recognized by the laboratory signs of microangiopathic hemolysis, as indicated by schistocytes, elevated lactate dehydrogenase, low haptoglobin, and low hemoglobin, plus thrombocytopenia and accompanying signs and symptoms of organ system involvement. […] Clinically, aHUS is often indistinguishable from the other TMAs: Shiga toxin–producing Escherichia coli (STEC) hemolytic uremic syndrome and thrombotic thrombocytopenic purpura (TTP). […] In nearly all patients, aHUS can be distinguished from TTP on the basis of an ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) enzyme activity measurement. […] It is essential that aHUS and TTP be differentiated quickly, as they require markedly divergent treatments.

• #1 Hemolytic Uremic Syndrome (HUS): Symptoms, Treatment, Causes, Prognosis

  https://www.medicinenet.com/hemolytic_uremic_syndrome/article.htm

  Uremia: Kidney function can be measured by testing the level of waste products in the blood normally filtered by the kidney. BUN (blood urea nitrogen) and creatinine are measures of this kidney function. When levels rise, it is an indication of kidney failure or uremia in which the kidneys cannot clear the waste products of metabolism from the body. […] Abnormal urine findings: Blood and protein may be found in the urine when normally they are not present. […] Stool cultures: Since E. coli O157:O7 is the most common cause of HUS in children; attempts are made to culture the bacteria from stool samples. The body usually clears the bacteria from the stool within a week, so a negative test does not exclude the disease. A positive test helps confirm HUS and will be reported to public health authorities to try to determine the source of the infection. In 2011, outbreaks in Europe were traced by public health authorities to tainted sprouts.

• #1 Hemolytic uremic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4523989/

  Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by the triad of thrombotic microangiopathy, thrombocytopenia, and acute kidney injury. […] In recent years, better understanding of the HUS, especially those due to genetic mutations in the alternative complement pathway have provided an update on the terminology, classification, and treatment of the disease. […] The diagnosis is made clinically with hemolytic anemia, thrombocytopenia and renal damage which occur suddenly in a patient with a history of diarrhea in the last two weeks. For a definite diagnosis STEC infection should be proven (demonstration of Stx with stool serologic tests or stool cultures). Renal biopsy is not necessary for making a definite diagnosis. […] The differential diagnosis should be made with the other conditions causing to TMA.

• #1 Hemolytic Uremic Syndrome (HUS): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/16470-hemolytic-uremic-syndrome

  You must get hemolytic uremic syndrome treatment at a hospital. […] If HUS causes kidney failure, you may need a kidney transplant. […] Yes, you can recover from hemolytic uremic syndrome without permanent damage to your health. […] With proper diagnosis and care, many people with HUS make a full recovery without any permanent damage to their health. […] Severe cases of HUS can be fatal without treatment, especially if HUS affects other organs like your brain. […] With proper diagnosis and treatment, more than 90% of people survive hemolytic uremic syndrome.

• #2 Hemolytic Uremic Syndrome (HUS): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/16470-hemolytic-uremic-syndrome

  Hemolytic uremic syndrome (HUS) is a condition that damages blood vessels in your kidneys. […] Treatment includes transfusions and medications. […] Hemolytic uremic syndrome (HUS) can affect anyone. […] Contact a healthcare provider if you have bloody diarrhea or diarrhea that lasts longer than three days. Without treatment, a severe case of HUS can be deadly. […] A healthcare provider will diagnose hemolytic uremic syndrome by: reviewing your medical history, asking about your family medical history, conducting a physical examination. […] If they suspect you have HUS, theyll also order tests. […] A healthcare provider may order the following tests to diagnose hemolytic uremic syndrome: pee test (urinalysis), blood test, stool (poop) test, genetic testing, kidney biopsy. […] Tests look for the presence of different things that indicate hemolytic uremic syndrome: pee tests look for blood or protein, blood tests detail your red blood cell levels and platelet levels, stool tests check for the presence of E. coli O157 and other bacteria that may cause HUS, a kidney biopsy shows damage to your kidney and sometimes the cause of the damage.

• #2 Hemolytic–uremic syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Hemolytic%E2%80%93uremic_syndrome

  Hemolyticuremic syndrome (HUS) is a syndrome characterized by low red blood cells, acute kidney injury (previously called acute renal failure), and low platelets. Initial symptoms typically include bloody diarrhea, fever, vomiting, and weakness. Kidney problems and low platelets then occur as the diarrhea progresses. […] Diagnostic method: Blood tests (to monitor levels of platelets, red blood cells, and white blood cells), stool tests (especially to check for microscopic or macroscopic levels of fresh or old blood), urinalysis (to help monitor kidney function, like blood urea nitrogen, or BUN, levels, pH, and for blood in the urine- hematuria). […] The similarities between HUS, aHUS, and TTP make differential diagnosis essential. All three of these systemic TMA-causing diseases are characterized by thrombocytopenia and microangiopathic hemolysis, plus one or more of the following: neurological symptoms (e.g., confusion, cerebral convulsions, seizures); renal impairment (e.g., elevated creatinine, decreased estimated glomerular filtration rate [eGFR], abnormal urinalysis); and gastrointestinal (GI) symptoms (e.g., diarrhea, nausea/vomiting, abdominal pain, gastroenteritis). […] Diagnostic work-up supports the differential diagnosis of TMA-causing diseases. A positive Shiga-toxin/EHEC test confirms a cause for STEC-HUS, and severe ADAMTS13 deficiency (i.e., 5% of normal ADAMTS13 levels) confirms a diagnosis of TTP.

• #2 Hemolytic Uremic Syndrome (HUS)

  https://www.health.ny.gov/diseases/communicable/e_coli/fact_sheet.htm

  HUS cannot be diagnosed with one single test or indicator, but rather a panel of tests and evaluations. These tests and evaluations include a medical and family history, physical exam, urine test, blood test, stool test, and/or kidney biopsy. […] 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.

• #2 Hemolytic uremic syndrome – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/470

  1st tests to order include CBC, peripheral blood smear, renal function/creatinine, serum electrolytes, prothrombin time (PT), PTT, LDH, haptoglobin, stool culture on sorbitol-MacConkey agar to detect Shiga toxin-producing Escherichia coli, polymerase chain reaction (PCR) to detect Shiga toxin 1/Shiga toxin 2, and proteins involved in complement regulation.

• #2 Hemolytic-uremic syndrome laboratory findings – wikidoc

  https://www.wikidoc.org/index.php/Hemolytic-uremic_syndrome_laboratory_findings

  Thrombocytopenia Below 150,000 although typically less than 60,000. In spite of low platelet count, active bleeding is rarely seen. […] Serum chemistry abnormalities High BUN, High creatinine, Electrolyte abnormalities may include hyponatremia, hyperkalemia, hyperphosphatemia, hypocalcemia, and acidosis (resulting from diarrhea, dehydration and renal failure), Increased Bilirubin and aminotransferases, High uric acid levels. […] Urinalysis – may show any of following Blood / Red blood cells, Protein, Bilirubin, WBC, Casts. […] Stool Testing Stool culture on Sorbitol MacConkey’s agar or detection of Shiga toxin with serological testing. […] Genetic Testing Done if suspicion of genetic or complement-mediated HUS/ recurrent HUS. However, results take weeks-month thus have no role in the acute management of disease and treatment should not be delayed while awaiting results. Low C3 and C4 may indicate complement mediated HUS. Genetic testing/screening for cobalamine metabolic defects in neonates presenting with HUS. […] Cultures Blood, spinal, organ/tissue cultures may be needed in case of suspicion of other sources of HUS for example pnemococcal infection.

• #2 Hemolytic-Uremic Syndrome (HUS) – Hematology and Oncology – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/hemolytic-uremic-syndrome-hus

  Hemolytic-uremic syndrome is suspected in patients with suggestive symptoms, thrombocytopenia, microangiopathic anemia, and signs of acute kidney injury. […] If the disorder is suspected, urinalysis, peripheral blood smear, reticulocyte count, serum lactic dehydrogenase (LDH), haptoglobin, renal function tests, serum bilirubin (direct and indirect), PT, PTT, fibrinogen, and direct antiglobulin test are done. […] The diagnosis of HUS is suggested by thrombocytopenia and anemia, fragmented red blood cells on the blood smear indicative of microangiopathic hemolysis, evidence of hemolysis, negative direct antiglobulin test, absence of use of a medication that can cause thrombotic microangiopathy, and normal ADAMTS13 activity. […] Stool testing is done in children with diarrhea and also adults who had a prodrome of bloody diarrhea; however, the organism and toxin may have cleared by the time of presentation. […] In atypical cases, that is, in patients who have not had preceding infection or who have recurrent disease, testing for complement factor gene mutations is recommended.

• #2 Hemolytic-uremic syndrome laboratory findings – wikidoc

  https://www.wikidoc.org/index.php/Hemolytic-uremic_syndrome_laboratory_findings

  The classic laboratory findings in hemolytic uremic syndrome (HUS) include anemia, thrombocytopenia and acute renal injury. Anemia is microangiopathic hemolytic anemia which low hemoglobin often 8g/dl, high reticulocyte count and LDH, low haptoglobin level as well as fragmanted RBC’s and schistiocytes on peripheral blood smear (PBS). Platelets are frequently less than 60,000 without active bleeding usually and renal damage is seen in form of high creatinine, BUN, and electrolyte abnormalities. […] Following Lab findings are seen in HUS: […] Microangiopathic hemolytic anemia with features as follows: Low hemoglobin level typically 10g/dl, High reticulocyte count, Increased LDH level, Low haptoglobin level, Negative Coombs test, Peripheral blood smear shows fragmented RBC’s (Schistiocytes, Helmet, and Burr cells), PT and aPTT are normal (differentiating feature from Disseminated Intravascular Coagulation).

• #2

  https://journals.lww.com/gjtm/fulltext/2021/06010/diagnosis_and_management_of_hemolytic_uremic.20.aspx

  A diagnosis of probable pHUS is made in patients, usually younger than 2 years, with sepsis, pneumonia, or meningitis, and positive DAT without features of DIC. […] The final diagnosis was confirmed after the identification of uncovered T-antigen on the patient’s red blood cells by a simple agglutination technique using an indigenously lectin panel. […] We endorse from our first case that prompt starting of TPE along with immunosuppressive agents could be life saving for HUS patients with anti-CFH antibodies and the message we would like to convey through the discussion of our second case is that identification of T-antigens should be integrated in pHUS diagnostic algorithm. […] Finally, we conclude that a well-equipped transfusion medicine department could change the outcome of HUS in children by facilitating early diagnosis and prompt management.

• #2 Hemolytic Uremic Syndrome (HUS): Symptoms, Treatment, Causes, Prognosis

  https://www.medicinenet.com/hemolytic_uremic_syndrome/article.htm

  Uremia: Kidney function can be measured by testing the level of waste products in the blood normally filtered by the kidney. BUN (blood urea nitrogen) and creatinine are measures of this kidney function. When levels rise, it is an indication of kidney failure or uremia in which the kidneys cannot clear the waste products of metabolism from the body. […] Abnormal urine findings: Blood and protein may be found in the urine when normally they are not present. […] Stool cultures: Since E. coli O157:O7 is the most common cause of HUS in children; attempts are made to culture the bacteria from stool samples. The body usually clears the bacteria from the stool within a week, so a negative test does not exclude the disease. A positive test helps confirm HUS and will be reported to public health authorities to try to determine the source of the infection. In 2011, outbreaks in Europe were traced by public health authorities to tainted sprouts.

• #2 Atypical Hemolytic Uremic Syndrome (aHUS): Making the Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-making-the-diagnosis/

  Atypical hemolytic uremic syndrome (aHUS) is a major thrombotic microangiopathy (TMA). A TMA is recognized by the laboratory signs of microangiopathic hemolysis, as indicated by schistocytes, elevated lactate dehydrogenase, low haptoglobin, and low hemoglobin, plus thrombocytopenia and accompanying signs and symptoms of organ system involvement. […] Clinically, aHUS is often indistinguishable from the other TMAs: Shiga toxin–producing Escherichia coli (STEC) hemolytic uremic syndrome and thrombotic thrombocytopenic purpura (TTP). […] In nearly all patients, aHUS can be distinguished from TTP on the basis of an ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) enzyme activity measurement. […] It is essential that aHUS and TTP be differentiated quickly, as they require markedly divergent treatments.

• #2 EM@3AM – Hemolytic Uremic Syndrome – emDocs

  https://www.emdocs.net/em3am-hemolytic-uremic-syndrome/

  Treatment & Disposition: Fluid resuscitation. […] Primary HUS => treated with eculizumab: monoclonal anti-C5 antibody that targets a downstream mediator in the complement cascade. […] The differential diagnosis of HUS should include: vasculitis, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, and non-microangiopathic etiologies of hemolytic anemia.

• #2 Hemolytic-Uremic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/779218-workup

  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. […] Initial therapy is similar for these conditions. Plasma exchange is the initial treatment of choice in all adult patients with HUS that is not associated with Shiga-like toxin (atypical HUS). Two complement inhibitors, eculizumab and ravulizumab, are approved for the treatment of pediatric and adult patients with atypical HUS. […] HUS is classified into two main categories, depending on whether it is associated with Shiga toxin (Stx) or not. […] Typical HUS (Shiga toxin-associated HUS [Stx-HUS]) is the classic, primary or epidemic, form of HUS. Stx-HUS is largely a disease of children younger than 2-3 years and often results in diarrhea (denoted D+HUS). One fourth of patients present without diarrhea (denoted D-HUS). 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.

• #2 Haemolytic uraemic syndrome – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://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. […] 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. […] Key diagnostic factors include diarrhoea, especially bloody diarrhoea, and childhood, especially age 5 years. […] Other diagnostic factors include known community outbreak of Shiga toxin-producing E coli, history of ingestion of food that may have been contaminated with Shiga toxin-producing E coli, and unusual adverse effect following treatment with ciclosporin, some chemotherapy agents, targeted cancer agents, and quinine.

• #2 Atypical Hemolytic Uremic Syndrome (aHUS): Making the Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-making-the-diagnosis/

  The standard treatment for TTP is plasma exchange, a therapy that has no role for patients with a diagnosis of aHUS established by ADAMTS13 activity levels. […] The three conditions noted above—TTP, STEC-HUS, and aHUS—are collectively referred to as the major thrombotic microangiopathies (TMAs). Clinically, they are often indistinguishable. […] The fact that the TMAs are rare is a further impediment to an accurate diagnosis. […] Yet the high morbidity and mortality associated with TTP and aHUS, and the markedly divergent treatments they require, mandate swift recognition of a TMA, as well as the ability to distinguish the two conditions. […] This review highlights the differences in pathophysiologic and diagnostic criteria among TMA subtypes. […] Four standard initial steps can be used to reach a specific TMA diagnosis: 1. A TMA must first be recognized. This involves examining the principle laboratory criteria for microangiopathic hemolysis: schistocytes on peripheral blood smear, low haptoglobin levels, and a decline in baseline hemoglobin.

• #2 Haemolytic uraemic syndrome (HUS): symptoms and treatments

  https://www.kidneyresearchuk.org/conditions-symptoms/haemolytic-uraemic-syndrome/

  HUS is usually diagnosed through: […] Blood tests which may confirm you have anaemia and a reduction in platelets […] Blood and urine tests which may show a reduction in your kidney function […] A kidney biopsy (a medical procedure to take small samples of the kidney to look at under the microscope) may also be required to make an accurate HUS diagnosis […] Other specialist tests may help to confirm the cause of HUS and can include Blood and stool sample tests which may confirm the presence of E.coli O157 and Shiga-toxin.

• #2 Hemolytic Uremic Syndrome (HUS) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/hemolytic-uremic-syndrome

  Hemolytic uremic syndrome (HUS) is a rare condition that can lead to kidney failure in children. […] HUS is a serious illness and potentially fatal. […] In addition to a complete medical history and physical examination, diagnostic procedures for hemolytic may include: Blood tests: to check blood cell counts, electrolytes and kidney function, Urine tests: to check for blood and protein, Stool tests: to check for blood, Abdominal X-ray: a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

• #2 HUS: Diagnosis & Management – EM Board Bombs Podcast

  https://www.emboardbombs.com/study-guide/2021-7-18-hus-its-whats-for-dinner-hemolytic-uremic-syndrome-mzx2s/

  Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by an acute kidney injury with associated microangiopathic hemolytic anemia and thrombocytopenia. Infection with Shiga toxin-producing E. coli (also known as STEC) is the most common cause of HUS in the pediatric population, accounting for up to 90% of all cases in children under the age of five. […] Its important for us to cover this acquired HUS, as its often misdiagnosed. In fact, one study showed that in children with STEC, 1 in 7 developed HUS within a median of 3 days, and ~30% of those who had HUS were first sent home. […] The first step is a good history suspect STEC infection in kids with acute onset of bloody diarrhea and abdominal pain or acute onset diarrhea with known exposure to STEC patient/outbreak. Initial labs: CBC, electrolytes (including creatinine), urinalysis, stool specimen for culture or Shiga toxin test.

• #2 Hemolytic Uremic Syndrome | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/22741

  Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. […] Prompt recognition of HUS’s varied etiologies and manifestations is essential for timely diagnosis and intervention, optimizing patient outcomes. […] The diagnosis of HUS requires a high index of suspicion based on symptoms, travel history, laboratory data, and dietary history. […] Initial tests should include a complete blood count with differential and comprehensive blood metabolic panel. […] A stool sample should be collected to test for Shiga toxin whenever diarrhea is present. […] Low complement levels are suggestive of, but not specific to aHUS, as typical HUS also causes immune system abnormalities. […] Genetic testing can also be sent to evaluate for genetic causes of aHUS. […] All of the above tests have therapeutic value, but if clinical suspicion is high, treatment should not be delayed while waiting for all testing results, as early treatment initiation is associated with improved outcomes.

• #2 Misconceptions Regarding Hemolytic Uremic Syndrome | Pediatric Emergency Medicine

  https://www.acep.org/pediatrics/education/education-articles/hemolytic-uremic-syndrome

  A good history is key to determine need for bloodwork to evaluate for early HUS in young children with abdominal symptoms, altered mental status, and evidence of dehydration on physical examination. Early diagnosis and initiation of IV fluids decreases dialysis and long-term renal complications. Hospitalization and close monitoring for dialysis needs is mandatory.

• #2 Atypical Hemolytic Uremic Syndrome (aHUS): Making the Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-making-the-diagnosis/

  A specific, FDA-approved, complement-based therapy for aHUS is now available. Once an ADAMTS13 activity of >5–10% (depending on the assay used) has been documented in the setting of a TMA, and thus the diagnosis of aHUS has been made, plasma therapy should be discontinued and appropriate treatment instituted. […] If a putative TTP or other TMA patient is not responding to plasma therapy, according to generally accepted measures of hematologic, clinical, and organ system changes, or requires prolonged plasma exchange to effect and/or maintain a partial remission, it is necessary to re-evaluate the diagnosis and consider aHUS.

• #2 Department of Health | Communicable Disease Service | Hemolytic Uremic Syndrome

  https://www.nj.gov/health/cd/topics/hus.shtml

  Report within 24 hours of Diagnosis to the Local Health Department. […] Hemolytic uremic syndrome (HUS) is a serious illness that affects the kidneys and blood cells. […] All patients with HUS must be in the hospital until kidney function returns to normal.

• #2 Hemolytic Uremic Syndrome (HUS) – Symptoms, causes, treatment | National Kidney Foundation

  https://www.kidney.org/kidney-topics/hemolytic-uremic-syndrome-hus

  When HUS causes mild kidney failure, fluid volume management may be all that is necessary for adequate treatment. When it causes severe kidney failure (when kidney function is less than 10 percent of normal), dialysis may be needed to do the work of the kidneys. […] 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. […] Most HUS patients that require dialysis ultimately recover their kidney function.

• #3 Haemolytic uraemic syndrome

  https://dermnetnz.org/topics/haemolytic-uraemic-syndrome

  Haemolytic uraemic syndrome is diagnosed on the following findings: […] The cause and complications of HUS should be investigated.

• #3 Atypical Hemolytic Uremic Syndrome (aHUS): Making the Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-making-the-diagnosis/

  2. These laboratory changes must be accompanied by involvement of at least one organ system. […] 3. aHUS and TTP can be distinguished from STEC-HUS by PCR, or culture-based assays for the Shiga-toxin producing E. coli, using stool or a rectal swab. […] 4. Finally, in the majority of cases, TTP typically responsive to PE can be distinguished from aHUS, for which plasma therapy does not have a role, on the basis of ADAMTS13 activity levels. […] In most settings, it takes from 48 hours to 1 week to obtain results from the tests, which are generally performed by only specialty laboratories. […] Assessment of complement levels or currently identifiable complement regulatory protein mutations cannot be used to rule out a diagnosis of aHUS. […] Unlike TTP, plasma therapy has no role in the treatment of aHUS.

• #3 Hemolytic-Uremic Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/779218-workup

  Non-Stx-HUS, or atypical HUS, is less common than Stx-HUS and accounts for 5-10% of all cases. As the name indicates, non-Stx-HUS does not result from infection by Stx-producing bacteria. […] Overall, patients with non-Stx-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. […] 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.

• #3 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hemolytic-Uremic-Syndrome-Investigation-and-Diagnosis.aspx

  Investigations of these patients are similar to the above. However there are certain additional risk factors whose presence needs to be looked for – these are mostly associated with alternative complement pathway dysregulation. […] Laboratory tests to distinguish between these two categories include: […] It is important to distinguish complement-mediated forms of atypical HUS from the second category because only the first type is likely to respond to the administration of the humanized monoclonal anti-complement antibody, eculizumab.

• #3 Hemolytic Uremic Syndrome | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/22741

  Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. […] Prompt recognition of HUS’s varied etiologies and manifestations is essential for timely diagnosis and intervention, optimizing patient outcomes. […] The diagnosis of HUS requires a high index of suspicion based on symptoms, travel history, laboratory data, and dietary history. […] Initial tests should include a complete blood count with differential and comprehensive blood metabolic panel. […] A stool sample should be collected to test for Shiga toxin whenever diarrhea is present. […] Low complement levels are suggestive of, but not specific to aHUS, as typical HUS also causes immune system abnormalities. […] Genetic testing can also be sent to evaluate for genetic causes of aHUS. […] All of the above tests have therapeutic value, but if clinical suspicion is high, treatment should not be delayed while waiting for all testing results, as early treatment initiation is associated with improved outcomes.

• #4 Hemolytic Uremic Syndrome: An Emerging Health Risk | AAFP

  https://www.aafp.org/pubs/afp/issues/2006/0915/p991.html

  Hemolytic uremic syndrome is caused primarily by Shiga toxinproducing Escherichia coli O157:H7. […] Early laboratory testing is important to diagnose and manage this syndrome. Obtaining a complete blood count and stool culture and performing Shiga toxin testing are the first of a series of tests that may help diagnose hemolytic uremic syndrome. […] Hemolytic uremic syndrome cannot be diagnosed without evidence of hemolytic anemia. Hematologic findings include destruction and fragmentation of erythrocytes that result in microangiopathic hemolytic anemia. This develops in all patients within a day or so of contamination and may result in respiratory and cardiovascular compromise. […] Laboratory testing can be used to secure a diagnosis of hemolytic uremic syndrome. Findings of hemolysis and thrombocytopenia on a complete blood count are required to establish the diagnosis. […] Hemolytic uremic syndrome is a reportable disease; therefore, local public health officials should be notified.

• #4 Atypical Hemolytic Uremic Syndrome (aHUS): Making the Diagnosis – Hematology & Oncology

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-making-the-diagnosis/

  A specific, FDA-approved, complement-based therapy for aHUS is now available. Once an ADAMTS13 activity of >5–10% (depending on the assay used) has been documented in the setting of a TMA, and thus the diagnosis of aHUS has been made, plasma therapy should be discontinued and appropriate treatment instituted. […] If a putative TTP or other TMA patient is not responding to plasma therapy, according to generally accepted measures of hematologic, clinical, and organ system changes, or requires prolonged plasma exchange to effect and/or maintain a partial remission, it is necessary to re-evaluate the diagnosis and consider aHUS.

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