Materiały źródłowe

• #1 Hemolytic uremic syndrome (HUS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/diagnosis-treatment/drc-20352405

  Hemolytic uremic syndrome needs treatment in the hospital. Treatment involves replacing lost fluids and minerals to make up for the kidneys not removing fluids and waste as well as usual. It also might involve getting nutrition through a vein. […] In the hospital, you might receive red blood cells or platelets through a vein, a process called a transfusion. […] Lasting kidney damage from hemolytic uremic syndrome might be treated with a medicine to lower blood pressure. This medicine might prevent or slow more kidney damage. […] For complications or the atypical form of hemolytic uremic syndrome, treatment might include a medicine called eculizumab (Soliris) to help prevent more damage to the blood vessels. […] Depending on the symptoms, the cause of hemolytic uremic syndrome and whether there are complications, treatment might include: Kidney dialysis. Dialysis removes waste and extra fluid from the blood. Dialysis is often done only until the kidneys begin working well again. But people with a lot of kidney damage might need long-term dialysis.

• #1 Hemolytic-Uremic Syndrome Treatment & Management: Medical Care, Consultations, Diet

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

  Specific treatments for Shiga toxin-associated hemolytic-uremic syndrome (Stx-HUS) have not proven of value. Instead, comprehensive supportive therapy is still the mainstay during the acute phase. […] Kidney transplantation is safe and effective for children who progress to end-stage renal disease (ESRD). The recurrence rate in patients who undergo kidney transplantation for HUS is 0-10%. […] The US Food and Drug Administration (FDA) has approved two complement inhibitors for the treatment of atypical HUS: eculizumab and ravulizumab. These monoclonal antibodies inhibit complement-mediated thrombotic microangiopathy. […] Other treatments during the acute phase of the disease, including plasma therapy and use of intravenously infused immunoglobulin (IgG), fibrinolytic agents, antiplatelet agents, corticosteroids, and antioxidants have proved ineffective in controlled clinical trials.

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

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

  HUS is generally treated with medical care in the hospital. Fluid volume management is crucial and may include: […] In about 50 percent of cases, short-term kidney replacement treatment in the form of dialysis is necessary. Most patients who need dialysis will recover kidney function and ultimately discontinue dialysis treatment. At times a special form of treatment called plasmapheresis may also be required. […] 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. Different forms of dialysis may be used depending on each individual’s needs. Most HUS patients that require dialysis ultimately recover their kidney function.

• #1 Hemolytic–uremic syndrome – Wikipedia

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

  Treatment involves supportive care and may include dialysis, steroids, blood transfusions, and plasmapheresis. […] Early IV fluid hydration is associated with better outcomes including shorter hospital stays and reducing the risk of dialysis. […] Empiric antibiotics are not indicated in those who are immunocompetent, and may worsen the HUS. […] Antidiarrheals and narcotic medications to slow the gut are not recommended as they are associated with worsening symptoms, increased risk of HUS in those with STEC infection, and adverse neurologic reactions. […] Platelet transfusions should not be used as they may drive the process of microangiopathy leading to worsening TTP. […] While eculizumab is being used to treat atypical hemolytic uremic syndrome, no evidence as of 2018 supports its use in the main forms of HUS.

• #1 Hemolytic Uremic Syndrome (HUS): Pearls and Pitfalls – emDocs

  https://www.emdocs.net/hus-pearls-and-pitfalls/

  As soon as HUS is suspected, consultation with the pediatric intensivist and nephrologist is warranted. The mainstay of treatment is supportive with resuscitation and constant monitoring. Major aspects of HUS that must be managed acutely include fluid status, anemia, thrombocytopenia, acute kidney injury, hypertension, and neurologic abnormalities. […] Fluid status must be carefully assessed in patients presenting with HUS, as they may present with large volume changes. Patients are often dehydrated due to diarrhea and/or vomiting and may require fluid resuscitation. On the other hand, patients with oliguria or anuria may be fluid overloaded and require fluid restriction and/or dialysis. In addition to fluid resuscitation and monitoring, electrolytes should be monitored and any abnormalities corrected.

• #1 Hemolytic Uremic Syndrome (HUS): Pearls and Pitfalls – emDocs

  https://www.emdocs.net/hus-pearls-and-pitfalls/

  Patients presenting with hemoglobin of 6 g/dL or lower should receive product transfusion, which is required in about 80% of pediatric patients with STEC HUS. A goal hemoglobin between 8-9 g/dL is recommended, as restoring hemoglobin to normal in these patients may lead to heart failure, pulmonary edema, and hypertension. In order to avoid these complications, monitoring for signs of volume overload during transfusion is necessary, and transfusion should be halted if any signs of volume overload occur. […] Platelet transfusions are usually not implicated as platelets rarely fall below 10,000/mm³. Platelet transfusion should be reserved for patients with active bleeding or patients with platelet counts below 30,000/mm³ who are undergoing an invasive procedure. […] In patients who develop renal insufficiency or renal failure, any nephrotoxic agents the patient is taking should be stopped. In addition, dialysis may be required, specifically if severe fluid overload, refractory electrolyte abnormalities, and symptomatic uremia occur. However, early dialysis in patients with HUS has failed to show improved outcomes.

• #1 Hemolytic uremic syndrome – WikEM

  https://wikem.org/wiki/Hemolytic_uremic_syndrome

  Initial management largely supportive with early fluid resuscitation […] Insulin therapy if hyperglycemic and ketones (pancreatic insufficiency complication) […] Antihypertensives: NifedipineER (0.25-0.5 mg/kg/day oral), Labetalol 1-3 mg/kg/day, divided into twice daily dosing (12 mg/kg/day up to 1200 mg/day), Nitroprusside 0.3-0.5 ug/kg/min IV (max 10 ug/kg/min) […] Plasma exchange (plasmapheresis) usually performed if anuria or neurologic sequela […] Eculizumab: Monoclonal anti-C5 antibody that interrupts complement cascade. Shown to be effective in cases of atypical HUS (those not due to shiga toxin forming bacteria) […] Transfusion of RBCs (only severe bleeding) generally only indicated if plasma exchange cannot be performed immediately […] Platelet transfusion is AVOIDED. Only used for life-threatening bleeding or intracranial hemorrhage under guidance from hematologist. Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death

• #1 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hemolytic-Uremic-Syndrome-Management-and-Complications.aspx

  At present, no specific treatment has been developed to intervene in the course of the proinflammatory and prothrombotic changes triggered by the toxin after it enters the bloodstream and binds to Gb3 receptors on the endothelial cells of the kidney, brain and other organs. […] In most cases, HUS is self-limited, and 95% of children recover with effective supportive treatment. […] The best practice in such cases includes: […] Addressing the impaired renal function and correction of metabolic abnormalities by dialysis: this is required in half to three-quarters of patients in the acute phase. […] Nutritional support […] Maintenance of the red cell count by red cell transfusions as appropriate has been found necessary in 80% of patients and is essential in preventing cardiorespiratory complications.

• #1 Treatment for HUS | Marler Clark

  https://marlerclark.com/foodborne-illnesses/hemolytic-uremic-syndrome/hus-treatment

  During the acute phase of HUS, the inflamed colon is usually non-functional for a week or two, and total parenteral nutrition (TPN) may become necessary. […] Reduced or absent urine output (oligoanuria) occurs in most cases of HUS. […] Dialysis is required if a patient is not producing adequate urine, which cleanses the body of uremic toxins and maintains fluid and electrolyte balance. […] Most young children with severe HUS require dialysis via the peritoneal cavity, called peritoneal dialysis, which has fewer negative side effects (such as nausea, vomiting, cramping, and weight gain) than hemodialysis and avoids traumatizing their much-smaller veins. […] Older children and adults are generally treated with hemodialysis, which circulates blood through a filtration machine to remove uremic toxins, normalizes blood chemistries, and corrects any circulatory fluid overload (often appearing as non-dependent edema or anasarca).

• #1 Hemolytic-Uremic Syndrome Treatment & Management: Medical Care, Consultations, Diet

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

  Plasma exchange is the initial treatment of choice in all adult patients with non-Stx-HUS (atypical HUS) or thrombotic thrombocytopenic purpura (TTP) and should be considered as early as possible in the disease course. […] Plasma treatment should be started within 24 hours of the patient’s presentation, to decrease treatment failures. It should be continued once or twice a day for at least 2 days after complete remission. […] Eculizumab (Soliris) was approved for the treatment of non-Stx-HUS (atypical HUS) by the FDA in 2011. […] The safety and effectiveness of eculizumab in non-Stx-HUS were established in two single-arm trials in 37 adults and adolescents and one retrospective study in 19 pediatric and 11 adult patients. […] Ravulizumab (Ultomiris) was approved by the FDA in 2019 for the treatment of aHUS in adult and pediatric patients aged 1 month and older.

• #1 Hemolytic uremic syndrome (HUS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/diagnosis-treatment/drc-20352405

  Plasma exchange. Plasma is the fluid part of blood that helps blood cells and platelets circulate. Sometimes a machine is used to clear the blood of its own plasma and replace it with fresh or frozen donor plasma. […] Kidney transplant. Some people who have severe kidney damage from hemolytic uremic syndrome need a kidney transplant.

• #1

  https://www.indianpediatrics.net/aug2020/aug-744-747.htm

  Hemolytic uremic syndrome is an important cause of acute kidney injury that requires dialysis in children. […] Hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury (AKI) requiring renal replacement therapy. Rapid diagnosis and management is necessary to limit irreversible renal damage. […] Guidelines for treatment of HUS were recently published by the Indian Society of Pediatric Nephrology (ISPN). […] Timely institution of PEX (60-75 mL/kg; fresh frozen plasma as exchange fluid) is most appropriate for patients with suspected aHUS. […] PEX is administered daily until hematological remission (platelets 100,000/mL, schistocytes 2%, LDH less than upper limit of normal for 2 consecutive days) and tapered over 4-6 weeks. […] For patients with anti-FH associated HUS, PEX are most appropriate to achieve the goal of reducing antibody titers.

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

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

  Eculizumab is a recombinant monoclonal antibody that targets the C5 component of complement activation by preventing its cleavage; early eculizumab administration improves its effectiveness. […] 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% down to 6% to 15%. […] Ravulizumab is a new C5 inhibitor approved for use in the USA in 2019 and the EU in 2020. Ravulizumab also binds to C5, preventing cleavage, and it has similar efficacy to eculizumab but with 4 times as long of a half-life. […] Prior to eculizumab, plasma exchange was the standard of care for HUS and is still used if eculizumab is not available or in addition if clinically indicated. […] One ongoing question is how to treat patients who develop ESRD due to aHUS because patients with aHUS often develop renal failure necessitating kidney transplant, and there is a high recurrence rate of aHUS in the transplanted kidney.

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

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-treating-the-patient/

  Mortality from TTP declined from more than 90% to less than 10% with institution of PEx, but the outcome is highly unfavorable in aHUS patients treated with PEx alone. […] If a putative TTP or other TMA patient is not responding to PEx according to generally accepted measures of hematologic, clinical, and organ system changes, or if he or she requires prolonged PEx to effect and/or maintain a remission, it is then important to reevaluate the diagnosis and consider aHUS. […] Eculizumab is the only therapy approved by the US Food and Drug Administration (FDA) for this disease. […] Eculizumab should be initiated as early as possible after making the diagnosis of aHUS, in order to attempt optimal recovery of renal function and prevent an ongoing TMA with extrarenal manifestations. […] Eculizumab is a hybrid of 2 IgG subtypes 2 and 4 and was designed to minimize development of autoantibodies and Fc receptor–mediated functions.

• #1 Hemolytic-Uremic Syndrome (HUS) – Blood Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/blood-disorders/platelet-disorders/hemolytic-uremic-syndrome-hus

  Treatment of HUS is support of key body functions and sometimes hemodialysis, and some people may benefit from medications such as eculizumab. […] About half of children with hemolytic-uremic syndrome temporarily require kidney dialysis, in which a dialysis machine removes waste products from the blood. Most often, the kidneys recover, but some children have permanent kidney damage. […] Eculizumab, ravulizumab, and pegcetacoplan are medications that suppress complement, a component of the immune system. They reduce the rate of kidney damage, and in some people, they may rapidly restore kidney function. People who take eculizumab, ravulizumab, or pegcetacoplan are at higher risk than normal for meningococcal meningitis, so they should receive the meningococcal vaccine to prevent meningococcal infections.

• #1 Treating aHUS Through Research | Children’s Hospital Colorado

  https://www.childrenscolorado.org/advances-answers/recent-articles/atypical-hemolytic-uremic-syndrome-treatment/

  Weve had a great drug for this disorder for many, many years now. But its all about the patient experience, he says. […] The new drug, crovalimab, can be given via a quick injection under the skin rather than an IV infusion, meaning that instead of spending hours at the hospital, Picard can be in and out in a matter of minutes, and her mother, Erica, recently learned to give the injections at home, eliminating the need for frequent hospital visits almost entirely. […] Starting in 2025, Dr. Dixon will begin testing yet another improvement to aHUS this time in adult patients. Instead of injections or IVs, this new option only requires patients to take two pills each day. […] To go from no therapy to one therapy thats good but inconvenient, to lots of options and even something that you can control with oral medication is just, in my mind, a fantastic journey for a rare disease like this, Dr. Dixon says.

• #1 Hemolytic-Uremic Syndrome Treatment & Management: Medical Care, Consultations, Diet

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

  The advent of eculizumab broadened the indications for kidney transplantation in aHUS. Prophylactic eculizumab therapy initiated at transplantation, because most posttransplant recurrence of non-Stx-HUS occurs within the first year, has been used to prevent recurrence and associated graft injury. […] Supportive therapy is as follows: Maintain fluid and electrolyte balance, Adequate blood pressure control and adequate renin-angiotensin blockade, for patients who have chronic kidney disease after an episode of Stx-HUS.

• #1 Management of hemolytic uremic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278190/

  The use of eculizumab in STEC HUS requires further evaluation before its use can be recommended. […] Plasma exchange (PE) until recently has been considered the most effective treatment. […] The effective use of eculizumab in aHUS was first described in 2009, and since then there have been many reports of its use. […] Treatment of aHUS should be started early (as soon as other causes of TMA have been excluded on the basis of clinical and laboratory assessment) since early treatment is associated with a better renal outcome. […] Recurrent disease after transplantation can be treated successfully with eculizumab, and 40% of patients in the recent trials developed disease in a transplanted kidney. […] Eculizumab can also be used prophylactically to prevent recurrence in patients at high risk of recurrence who are undergoing transplantation.

• #1 Hemolytic uremic syndrome (HUS) | Find a specialist & information

  https://www.leading-medicine-guide.com/en/illness/blood-immune-system/hemolytic%E2%80%93uremic-syndrome

  The treatment of choice is a combination of plasmapheresis and/or plasma infusions and administration of fresh frozen plasma. In certain forms of atypical haemolytic uraemic syndrome, eculizumab, which has an inhibitory effect on the dysregulation of the non-specific response, can lead to an improvement in symptoms. […] If the disease is due to a deficiency of a specific protein involved in the innate immune response (so-called complement factor H), a liver transplant may be an option. If end-stage renal failure is also present, a simultaneous kidney transplant is considered. […] Immunosuppression (suppression of the immune system) may also be indicated in some forms of atypical hemolytic uremic syndrome. […] If the cause of the disease is a substance contained in other medications, these should be discontinued.

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

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

  Hemolytic uremic syndrome is the most common cause of acute renal failure in children, and the incidence of this syndrome in children is increasing worldwide. […] Do not treat with antibiotics or antidiarrheals while the patient is in the diarrheal stage. […] Typical hemolytic uremic syndrome is a self-limiting disease with spontaneous recovery, although close monitoring and treatment of symptoms are essential. […] Antibiotics and antimotility agents are not recommended as treatments for hemolytic uremic syndrome during the diarrheal stage of the disease. […] Serial monitoring of the hematocrit and platelet count is important. Currently, platelet transfusion is controversial because it can worsen the thrombotic process. […] Modalities such as plasmapheresis, anti-thrombotic agents, steroids, and Shiga toxin-binding agents have proved ineffective and remain controversial.

• #1 Hemolytic uremic syndrome (HUS) | Find a specialist & information

  https://www.leading-medicine-guide.com/en/illness/blood-immune-system/hemolytic%E2%80%93uremic-syndrome

  There is currently no causal therapy for hemolytic uremic syndrome. Therefore, supportive and, if necessary, intensive medical therapy is usually used. […] As the underlying bacterial infection subsides within 5 to 10 days, no antibiotic treatment should be given in the acute phase (diarrhea phase). Antibiotics are suspected of leading to bacteriolysis (decomposition of the bacteria). This accelerates the release of the disease-causing toxin and thus leads to a worsening of the condition. […] Instead, plasmapheresis can be used to reduce the circulating toxin concentration. This involves removing the blood plasma from the body and replacing it with frozen fresh plasma. Treatment with corticosteroids or H1 and H2 blockers is often carried out in advance to prevent an allergic reaction. […] In severe cases and unsuccessful plasmapheresis, drug therapy with the antibody eculizumab may be indicated. In some cases, plasmapheresis and eculizumab are also administered as a combination therapy.

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

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

  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. […] Although there is still much to do, there has been a significant increase in understanding the underlying cause of HUS and the processes that contribute to kidney damage. Medications designed to prevent the absorption of the E. coli bacterial toxins in children with HUS are currently being studied in clinical treatment trials.

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

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

  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. […] Eculizumab might prevent life-threatening kidney damage and death in some children.

• #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 is serious. But treating it in time leads to a full recovery for most people, especially young children. […] Seek emergency care if you or your child doesn’t urinate for 12 hours or more.

• #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. […] You must get hemolytic uremic syndrome treatment at a hospital. Treatment may include: […] Intravenous (IV) fluids and/or tube feeding. IV fluids help keep you hydrated. Tube feeding (enteral nutrition) ensures you’re getting nutrients. […] Medications. Blood pressure medications (antihypertensives) help reduce your blood pressure and reduce kidney damage. A provider may prescribe eculizumab or ravulizumab if you have atypical hemolytic uremic syndrome. […] Blood transfusions. It’s important that you get red blood cells and platelets if you have HUS. […] If you have severe kidney damage, you may need dialysis to clean your blood while your kidneys heal. 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. […] More than 85% of people with HUS recover complete kidney function.

• #1 Management of hemolytic uremic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278190/

  Hemolytic uremic syndrome (HUS) is a disease characterized by hemolysis, thrombocytopenia, and acute kidney injury, although other organs may be involved. […] Early identification and initiation of best supportive care, with microbiological input to identify the pathogen, result in a favorable outcome in most patients. […] Plasma therapies can reverse defective complement control, and it is now possible to specifically target complement activation. This has led to improved outcomes in patients with atypical forms of HUS. […] Best supportive care (BSC), with particular attention to fluid replacement, renal support, and treatment of neurological manifestations of the disease, is the mainstay of treatment for patients with HUS. […] Evidence for the addition of plasma-based therapy to BSC for the treatment for STEC HUS is limited and it probably has no role.

• #2 Management of hemolytic uremic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278190/

  Hemolytic uremic syndrome (HUS) is a disease characterized by hemolysis, thrombocytopenia, and acute kidney injury, although other organs may be involved. […] Early identification and initiation of best supportive care, with microbiological input to identify the pathogen, result in a favorable outcome in most patients. […] Plasma therapies can reverse defective complement control, and it is now possible to specifically target complement activation. This has led to improved outcomes in patients with atypical forms of HUS. […] Best supportive care (BSC), with particular attention to fluid replacement, renal support, and treatment of neurological manifestations of the disease, is the mainstay of treatment for patients with HUS. […] Evidence for the addition of plasma-based therapy to BSC for the treatment for STEC HUS is limited and it probably has no role.

• #2 Management of hemolytic uremic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278190/

  Most commonly, HUS is due to STEC infection. This can be a severe, life-threatening disease but in most cases is self-limiting and will resolve completely. Treatment is with BSC, and although other treatments, including plasma therapy, eculizumab, and toxin binders, have been proposed, there is very little evidence to support their use outside of clinical trials.

• #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. […] You must get hemolytic uremic syndrome treatment at a hospital. Treatment may include: […] Intravenous (IV) fluids and/or tube feeding. IV fluids help keep you hydrated. Tube feeding (enteral nutrition) ensures you’re getting nutrients. […] Medications. Blood pressure medications (antihypertensives) help reduce your blood pressure and reduce kidney damage. A provider may prescribe eculizumab or ravulizumab if you have atypical hemolytic uremic syndrome. […] Blood transfusions. It’s important that you get red blood cells and platelets if you have HUS. […] If you have severe kidney damage, you may need dialysis to clean your blood while your kidneys heal. 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. […] More than 85% of people with HUS recover complete kidney function.

• #2 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Hemolytic-Uremic-Syndrome-Management-and-Complications.aspx

  Despite the steep fall in the platelet count, platelet transfusions are dangerous and may exacerbate the formation of microthrombi in vulnerable vascular beds and are therefore avoided, unless in situations such as an active hemorrhagic episode or an invasive procedure (such as insertion of a central venous catheter or dialysis catheter) in presence of a low platelet count. […] Fluid-electrolyte balance: firstly the fluid volume should include the amount and type of fluid that is essential to maintain adequate nutrition. […] In atypical HUS, sometimes caused by genetic mutations in 5% of children, the illness is the result of abnormal complement system activation. Plasma exchange is the best mode of treatment in this subset of patients and should be started as soon as indicated. […] The thrombotic microangiopathy in complement-mediated atypical HUS is responsive to the complement inhibitor eculizumab. It is a recombinant molecule which consists of humanized monoclonal anti-C5 antibody. It prevents the cleavage of this factor, and thereby inhibits formation of the membrane attack complex, hindering cellular destruction. This therapy has been shown to perform better than plasma exchange in both preventing and treating atypical HUS in some trials. In children below the age of 2 years, as well, it may be considered the first line of treatment since plasma exchange is not technically feasible in all centers for them.

• #2 Hemolytic uremic syndrome (HUS) – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hemolytic-uremic-syndrome/diagnosis-treatment/drc-20352405

  Plasma exchange. Plasma is the fluid part of blood that helps blood cells and platelets circulate. Sometimes a machine is used to clear the blood of its own plasma and replace it with fresh or frozen donor plasma. […] Kidney transplant. Some people who have severe kidney damage from hemolytic uremic syndrome need a kidney transplant.

• #2 Hemolytic Uremic Syndrome (HUS): Pearls and Pitfalls – emDocs

  https://www.emdocs.net/hus-pearls-and-pitfalls/

  Patients presenting with hemoglobin of 6 g/dL or lower should receive product transfusion, which is required in about 80% of pediatric patients with STEC HUS. A goal hemoglobin between 8-9 g/dL is recommended, as restoring hemoglobin to normal in these patients may lead to heart failure, pulmonary edema, and hypertension. In order to avoid these complications, monitoring for signs of volume overload during transfusion is necessary, and transfusion should be halted if any signs of volume overload occur. […] Platelet transfusions are usually not implicated as platelets rarely fall below 10,000/mm³. Platelet transfusion should be reserved for patients with active bleeding or patients with platelet counts below 30,000/mm³ who are undergoing an invasive procedure. […] In patients who develop renal insufficiency or renal failure, any nephrotoxic agents the patient is taking should be stopped. In addition, dialysis may be required, specifically if severe fluid overload, refractory electrolyte abnormalities, and symptomatic uremia occur. However, early dialysis in patients with HUS has failed to show improved outcomes.

• #2 Management of hemolytic uremic syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4278190/

  The use of eculizumab in STEC HUS requires further evaluation before its use can be recommended. […] Plasma exchange (PE) until recently has been considered the most effective treatment. […] The effective use of eculizumab in aHUS was first described in 2009, and since then there have been many reports of its use. […] Treatment of aHUS should be started early (as soon as other causes of TMA have been excluded on the basis of clinical and laboratory assessment) since early treatment is associated with a better renal outcome. […] Recurrent disease after transplantation can be treated successfully with eculizumab, and 40% of patients in the recent trials developed disease in a transplanted kidney. […] Eculizumab can also be used prophylactically to prevent recurrence in patients at high risk of recurrence who are undergoing transplantation.

• #2

  https://link.springer.com/article/10.1007/s40272-022-00555-6

  Atypical hemolytic uremic syndrome is a thrombotic microangiopathy characterized by hemolysis, thrombocytopenia, and acute kidney injury, usually caused by alternative complement system overactivation due to pathogenic genetic variants or antibodies to components or regulatory factors in this pathway. […] Plasma therapy has been used for atypical hemolytic uremic syndrome treatment with inconsistent results. Complement-blocking treatment changed the outcome and prognosis of patients with atypical hemolytic uremic syndrome. Early administration of eculizumab, a monoclonal C5 antibody, leads to improvements in hematologic, kidney, and systemic manifestations in patients with atypical hemolytic uremic syndrome, even with apparent dialysis dependency. Pre- and post-transplant use of eculizumab is effective in the prevention of atypical hemolytic uremic syndrome recurrence.

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

  https://www.hematologyandoncology.net/supplements/atypical-hemolytic-uremic-syndrome-ahus-treating-the-patient/

  Eculizumab is administered as an intravenous infusion over 30 minutes, and has an estimated half-life of 11 to 12 days. […] Eculizumab appears to be becoming the treatment of choice for aHUS without initial plasma therapy. […] Following a recognized episode of aHUS, 90% of adults relapse within 1 year absent appropriate treatment. […] Eculizumab should be initiated as early as possible after the diagnosis of aHUS is made. […] Eculizumab is the only FDA-approved therapy for aHUS, but it is an expensive drug. […] The duration of treatment with eculizumab is an unresolved issue. […] Eculizumab is indicated for the treatment of patients with aHUS to inhibit complement-mediated thrombotic microangiopathy, including adverse effects on renal function.

• #2 Treating aHUS Through Research | Children’s Hospital Colorado

  https://www.childrenscolorado.org/advances-answers/recent-articles/atypical-hemolytic-uremic-syndrome-treatment/

  For decades, the condition was a death sentence with unreliable treatment options. […] But in 2009, the U.S. Food and Drug Administration (FDA) approved a new drug, eculizumab, that completely rewrote the story. […] That was a life changer, Dr. Dixon says. Patients could come off dialysis. It could suppress the disease from coming back in a transplant. This completely revolutionized the way these patients experience life. […] In 2017, at just 3 years old, Juliette Picard came down with a case of strep. […] Within one visit, Picard was diagnosed with aHUS. At the time, Dr. Dixon was beginning a clinical trial for a new drug to treat the condition, and Picard was almost immediately enrolled. Ravulizumab was designed as a variation of eculizumab, but instead of every two weeks, patients could go four to eight weeks between infusions, depending on their weight. Today, that drug is FDA approved. And since beginning treatment, Picard has been symptom-free.

• #2

  https://link.springer.com/article/10.1007/s40272-022-00555-6

  Recent data favor the restrictive use of eculizumab in carefully selected atypical hemolytic uremic syndrome cases, but close monitoring for relapse after drug discontinuation is emphasized. Prophylaxis for meningococcal infection is important. The long-acting C5 monoclonal antibody ravulizumab is now approved for atypical hemolytic uremic syndrome treatment, enabling a reduction in the dosing frequency and improving the quality of life in patients with atypical hemolytic uremic syndrome. New strategies for additional and novel complement blockage medications in atypical hemolytic uremic syndrome are under investigation.

• #2 Hemolytic uremic syndrome – WikEM

  https://wikem.org/wiki/Hemolytic_uremic_syndrome

  Hemodialysis/Hemoperfusion usually reserved for symptomatic uremia, azotemia (BUN 80 mg/dL), fluid overload or electrolyte abnormalities refractory to medical therapy […] AVOID Antibiotics. May lead to worsening lysis of bacteria and further shiga toxin release […] AVOID Antimotility agents. Leads to prolonged gut exposure to toxins. Risk of toxic megacolon.

• #2 Hemolytic Uremic Syndrome (HUS): Pearls and Pitfalls – emDocs

  https://www.emdocs.net/hus-pearls-and-pitfalls/

  Hypertension, if present, should be managed with calcium channel blockers such as nifedipine or nicardipine. Angiotensin converting enzyme inhibitors may result in decreased renal perfusion and are not recommended in patients presenting with HUS. […] The major neurologic abnormality that requires acute management is seizures. Seizures associated with HUS should receive immediate treatment with antiepileptics that can include a benzodiazepine and second line agents such as fosphenytoin and levetiracetam. […] There is little evidence for the efficacy of other agents in the treatment of HUS, including heparin, prostacyclin, steroids, fibrinolytics, or immunoglobulins. For patients presenting with suspected primary HUS (complement-mediated), plasma exchange may be beneficial. Another treatment for both primary HUS and STEC HUS with CNS involvement is eculizumab (monoclonal antibody to complement factor C5). However, use of these agents should be left up to the pediatric intensivist or inpatient team.

• #2 Management of hemolytic-uremic syndrome in children | IJNRD

  https://www.dovepress.com/management-of-hemolytic-uremic-syndrome-in-children-peer-reviewed-fulltext-article-IJNRD

  Early volume expansion in patients affected by EHEC, implemented within the first 4 days from the onset of diarrhea, can improve renal and general outcomes of D+HUS. […] Current evidence does not support use of fresh frozen plasma infusions or PE in patients with D+HUS. In contrast, PE should be instituted without delay in patients with non-pneumococcal aHUS. […] Current evidence is not sufficient to support the use of eculizumab for patients with D+HUS. […] Comprehensive supportive nutritional and hemodynamic management coupled with peritoneal dialysis or hemodialysis when indicated are associated with best outcomes and minimal mortality.

• #2 Haemolytic Uraemic Syndrome

  https://pch.health.wa.gov.au/for-health-professionals/emergency-department-guidelines/haemolytic-uraemic-syndrome

  To guide Emergency Department (ED) staff with the assessment and management of haemolytic uraemic syndrome. […] Management1,4 […] Establish IV access […] Hypovolaemia should be treated with sodium chloride 0.9% saline boluses, or packed red blood cells, depending on the clinical status (usually sodium chloride 0.9% saline in the ED setting) […] If these develop, immediate discussion should be undertaken with a senior colleague (e.g. ED consultant, renal consultant or Paediatric Critical Care (PCC) consultant) […] Usually secondary to volume overload […] If unresponsive to diuretics, try vasodilator treatment (e.g. Isradipine, clonidine) […] Indicated in pneumonia related HUS. Refer to Acute Respiratory Tract Infection Empiric Guidelines – ChAMP […] Patients needing dialysis (most) will usually be placed on peritoneal dialysis (PD) […] All patients diagnosed with HUS must be admitted […] The paediatric renal team (and general surgical team for PD catheter / line placement) should be consulted early.

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

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

  There is no known treatment that can stop the progression of HUS once it has started. […] Initially, treatment may be supportive, which means that your child’s doctor will treat the symptoms. If your child shows signs of dehydration and electrolyte irregularities, these conditions will be treated first. […] Other therapies may include: […] Intensive care: Close observation and monitoring in an intensive care unit (ICU) may be necessary. […] Blood transfusions: Blood transfusions may be necessary to treat anemia (low red blood cell count). […] Nutrition: Your child may require intravenous nutrition if he or she is having severe digestive tract complications. […] Dialysis: Dialysis may be required in up to half of all children who develop HUS. Dialysis is a medical procedure to remove wastes and additional fluid from the blood after the kidneys have stopped functioning. […] Medication: Medications may be required to treat your child’s blood pressure, which may be elevated.

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