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INTRODUCTION

Hemolytic-uremic syndrome (HUS) was first described by Gasser in a German publication in 1955. Since

1955, thousands of cases have been reported, and hemolytic-uremic syndrome is recognized as the most

common cause of acute renal failure in the pediatric population, occurs primarily in infants and small

children between the ages o 6 months 5 years. It is a heterogeneous group of disorder that are a common

cause of acute renal failure in children. Kidney failure develops as a result of destruction of the small,

functional structures and vessels inside the kidney. HUS is a serious illness and potentially fatal. Although

HUS can cause serious complications and can even be life threatening, most children who develop HUS

recover without permanent damage to their health. HUS is more common during the summer months and

may occur in outbreaks. Outbreaks have been reported in daycare centers, water parks, and fast food

restaurants as a result of inadequately cooked hamburger meat

Healthy red blood cells (left) are smooth and round. In hemolytic uremic syndrome, toxins destroy red blood

cells (right). These misshapen cells may clog the tiny blood vessels in the kidneys. They are characterized by

microangiopathic hemolytic anemia, thrombocytopenia & acute renal insufficiency.



CLASSIFICATION

Studies have revealed distinct subgroups of hemolytic-uremic syndrome and have identified several etiologies

for the disease. Hemolytic-uremic syndrome is classified as

1) D+ or Typical HUS

2) D- or Atypical HUS

i) Within D- hemolytic-uremic syndrome is another subtype, pneumococcal-associated hemolytic-

uremic syndrome (P-hemolytic-uremic syndrome).

1) D+ hemolytic-uremic syndrome is used to describe diarrhea-positive, classic or typical hemolytic-uremic

syndrome, caused by verotoxin- producing E. coli in North America & Europe ; in the Indian subcontinent

Shigella dysenteriae 1 is the chief pathogen.

Cytotoxin mediated injury to the endothelium in the renal

microvasculature leads to localized coagulation & fibrin deposition. As red cell & platelets transverse these

damaged vessels, they are injured & sequestered. Through the brunt of the micro-vascular injury is on the

kidney, other organs especially the brain may be affected.

2) D- hemolytic-uremic syndrome is used to describe diarrhea-negative, non – diarrhea-associated or

atypical hemolytic-uremic syndrome, mediated by abnormalities of the complement system or other heritable

factors. This condition, seen at any age, lacks the prodromal history of diarrhea or dysentery. Onset may be

insidious & occasionally present with rapidly progressive illness

The microangiopathic lesions chiefly affect

interlobular arteries and result in severe hypertension and renal insufficiency. Predisposing factors include

inherited abnormalities in the complement regulatory pathway, infection with neuraminidase-producing

organisms (pneumococci) or HIV, cobalamin deficiency, systemic lupus & drugs ( eg. Cyclosporine,

mitomycin).



i)P-hemolytic-uremic syndrome is used to describe pneumococcal-associated hemolytic-uremic syndrome,

mediated by neuraminidase in the presence of infection with Streptococcus pneumoniae.

CAUSES

The causes of D+ hemolytic-uremic syndrome and D

- hemolytic-uremic syndrome differ.

D+ hemolytic-uremic syndrome

o GI tract infection with E coli (STEC) precedes most cases of typical D+ hemolytic-uremic syndrome.

Stx1 is identical to the Stx produced by Shigella dysenteriae. Stx2 has a 55-60% amino acid homology

This organism is very resilient; viable bacteria has been reported in environments up to 10 months

following initial contamination. Other causes of hemolytic-uremic syndrome include infection by the

following:

S dysenteriae (established as an etiologic agent)

Salmonella typhi (established as an etiologic agent)

Campylobacter jejuni (established as an etiologic agent)

Yersinia species

Pseudomonas species

Bacteroides species

Entamoeba histolytica

Aeromonas hydrophilia

D- hemolytic-uremic syndrome

o Inherited (eg, mutations in the gene for factor H, a complement regulatory protein)

o S pneumoniae (neuraminidase-associated)

o Portillo virus

o Coxsackie virus

http://emedicine.medscape.com/article/970552-overview











o Influenza virus

o Epstein-Barr virus

o Pregnancy: Hemolytic-uremic syndrome or thrombotic thrombocytopenic purpura (TTP) are associated

with pregnancy; pre-eclampsia and HELLP syndrome also have features in common and should be part

of the differential diagnosis.

o Drugs (eg, chemotherapy, oral contraceptives, cyclosporine, tacrolimus)

o Bone marrow or hematopoietic stem cell transplantation

o Malignancy

o Idiopathic

o Systemic lupus erythematosus (SLE)

o Glomerulonephritis, especially membranoproliferative glomerulonephritis

o Malignant hypertension

PATHOPHYSIOLOGY:-

Most affected children have an associated prodrome of gastrointestinal symptoms including bloody

diarrhea, which suggests that an infectious agent may be the cause of hemolytic uremic syndrome.

Nearly all cases are the result of an antecedent infection by shiga’s toxin-producing strains of E. coli.

Primary site of injury appears to be the endothelial lining of the small glomerular arterioles which

become swollen and occluded with deposit of platelets and fibrin clots. RBC are damaged as they

attempt to move through the partially occluded blood vessels. These damage cells are removed by the

spleen, causing acute hemolytic anemia. The platelet aggregation with the damage blood vessels or the

damage & removal of platelets produce the characteristic thrombocytopenia.

















CLINICALFEATURES OF ALL FORMS OF HEMOLYTIC-

UREMIC SYNDROME

Hematology

o Hemolysis occurs in all patients with hemolytic-uremic syndrome. It can proceed rapidly, resulting

in a rapid fall of the hematocrit.

o Platelet counts usually fall below 40,000/mcL. However, the degree of thrombocytopenia does not

correlate with the severity of hemolytic-uremic syndrome, and some children can maintain

relatively normal kidney function despite severe hematologic abnormalities.

o Many patients have petechiae, purpura, and oozing from venipuncture sites.

o Overt bleeding is less common.

CNS

o Patients often present with sudden onset of lethargy and irritability.

o Other findings may include ataxia, coma, seizures, cerebral swelling, hemiparesis, and other focal

neurologic signs.

o CNS changes may be caused by cerebral ischemia from microthrombi, effects of hypertension,

hyponatremia, or uremia.

o D- hemolytic-uremic syndrome tends to be associated with a greater number of neurologic

symptoms than D+ hemolytic-uremic syndrome.

Renal system

o Acute renal insufficiency usually begins with the onset of hemolysis. Although patients have

decreased urine output, frequent diffuse watery stools may mask this sign.

o If renal insufficiency is not recognized and treated, hyponatremia, hyperkalemia, severe acidosis,

ascites, edema, pulmonary edema, and hypertension ensue.

GI tract: D+ hemolytic-uremic syndrome is usually preceded by 3-12 days of watery or bloody

diarrhea. Vomiting and crampy abdominal pain are also common. Note that diarrhea may improve as



the other hemolytic-uremic syndrome symptoms begin (eg, thrombocytopenia, renal insufficiency).

Life-threatening complications include intestinal perforation or necrosis. Even without these

complications, the colitis of hemolytic-uremic syndrome may cause severe abdominal pain which

may persist for several days into the illness.

Infectious signs: Fever is present in 5-20% of patients. The presence of fever, leukocytosis, or both is

a prognostic indicator of the risk of developing more severe hemolytic-uremic syndrome.

Pancreas: Mild pancreatic involvement is common but can be severe on occasion, with necrosis,

pseudocysts, or both, which can leave the patient with insulin-dependent diabetes and, on rare

occasion, exocrine dysfunction.

Cardiovascular: Congestive heart failure may occur.

Physical

Blood pressure may be elevated unless the patient is volume depleted (eg, from diarrhea.)

Child appears ill and pale.

Abdominal pain and tenderness may be present, possibly severe.

Peripheral edema may be present.

Petechiae, purpura, or oozing from venipuncture sites may be present.

Laboratory Studies

Hematology

Classic findings in hemolytic-uremic syndrome (HUS) include anemia and thrombocytopenia, with





fragmented RBCs (eg, schistocytes, helmet cells, burr cells). Peripheral blood smear in hemolytic-

uremic syndrome (HUS) showing many schistocytes and RBC fragments due to hemolysis, and

relatively few platelets reflective of thrombocytopenia.

WBC differential may reveal a left shift (ie, immature WBCs, including bands, myelocytes,

metamyelocytes). Patients with diarrhea-associated hemolytic-uremic syndrome (D+ HUS) may have

extremely high WBC counts, in the range of 50-60,000/mcL.

Coombs test results are negative, except with S pneumoniae – associated hemolytic-uremic syndrome.

Reticulocyte count is elevated.

Levels of serum haptoglobin, which binds hemoglobin, are decreased.

Prothrombin time (PT) and activated partial thromboplastin time (aPTT) are normal.

Fibrin degradation products are increased.

Fibrinogen levels are increased or within reference range.

Serum chemistry testing

BUN and creatinine levels are elevated.

Various electrolyte and ion derangements may be present due to vomiting, diarrhea, dehydration and renal

failure; these may include hyponatremia, hyperkalemia, hyperphosphatemia, hypocalcemia, and

acidosis.Phosphorus concentration is elevated.

Uric acid level may be increased because of acute renal failure, dehydration, and cell breakdown.

Protein (see Serum Protein Electrophoresis) and albumin levels may be mildly decreased.















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Bilirubin and aminotransferase (see Alanine Aminotransferase and Aspartate Aminotransferase) levels are

typically elevated.

Lactate dehydrogenase (LDH) level is elevated. Serial measurements of LDH help track the approximate

level of hemolytic activity.

Urinalysis

Protein

Hem

Bilirubin

RBCs (dysmorphic)

WBCs

Casts - Cellular, granular, pigmented, hyaline

Stool testing

Culture: Usually, culture yield is low after 7 days of diarrhea. The standard method used to detect and

isolate Shigella toxin (Stx) – producing E coli(STEC) involves sorbitol Mac Conkey (SMAC) agar plates

that enable identification of characteristic sorbitol non-fermenting colonies of STEC O157:H7.

E coli 0157:H7 does not grow on agar plates used for routine stool cultures. Notify the laboratory and

request specific testing for this organism when hemolytic-uremic syndrome is suspected.

Even patients with documented bloody diarrhea and other classic features of D+ hemolytic-uremic

syndrome often do not yield a causative organism on stool culture. This reflects the limited sensitivity of

stool culture, not the absence of disease. The diagnosis of hemolytic-uremic syndrome is a clinical one

and is not excluded by a negative stool culture.

Stx may be detected using specific antibody testing, gene studies, and enzyme-linked immunosorbent

assay (ELISA).

Stool leukocytes have little value in detecting E coli 0157:H7. They are absent in approximately 50% of

cases.













Other tests

A test for serum antibodies to STEC 0157:H7 is available, but its clinical use is not well defined.

Complement C3 may be decreased in patients with genetic forms of hemolytic-uremic syndrome.

Genetic testing for complement factor mutations is available from a limited number of laboratories, in

some cases only on a research basis. For a list of some laboratories offering such testing see Special

Concerns. Consult with an expert in this area before ordering such tests.

Genetic tests may take weeks or months to perform, so they are not useful in the immediate management

of a patient with hemolytic-uremic syndrome, and acute treatment decisions should not be delayed while

awaiting results.

Results may be helpful in determining long-term prognosis (eg, the presence of factor H mutations

portends a very poor renal prognosis).

Tests done on a fee basis may be very expensive.

Tests done on a research basis require informed consent. Check with the facility regarding applicable

policies for research testing.

Imaging Studies

Consider performing chest radiography to evaluate for pulmonary congestion or edema, if clinically

indicated.

Renal ultrasound typically reveals nonspecific findings (eg, increased echogenicity) and is of little use.

Ultrasonography may be helpful if the diagnosis is uncertain or if one needs evaluation of blood flow in

the large renal vessels.

Abdominal ultrasonography or CT scanning may help if clinical findings raise suspicion of intestinal

obstruction or perforation.





Non-contrast CT scanning or MRI of the head is indicated in patients with CNS symptoms or acute mental

status changes.[4]

Avoid iodinated contrast or gadolinium in patients with decreased renal function.

Other Tests

Patients with hyperkalemia may require EKG monitoring.

Histologic Findings

Renal biopsy is not usually necessary for diagnosis and may be contraindicated due to thrombocytopenia.

Histologic analysis of kidney specimens reveals thrombotic microangiopathy, with swollen glomerular

endothelial cells and red cells and platelets in the capillaries. Accumulation of fibrinlike material in the

subendothelial space creates a thickened appearance to the capillary walls. Thrombi may be observed in

the glomerular capillaries and arterioles. These findings can progress to acute cortical necrosis involving

both glomeruli and convoluted tubules.

Peripheral blood smear in hemolytic-uremic syndrome (HUS) showing many schistocytes and RBC

fragments due to hemolysis, and relatively few platelets reflective of thrombocytopenia.

Tissue section of the gut shows microangiopathy, with endothelial cell injury, and thrombosis, with

submucosal edema and hemorrhage.

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Microthrombi may be observed in other organs, including the lungs, liver, heart, adrenal glands, brain,

thyroid, pancreas, thymus, lymph nodes, and ovaries.

MANAGEMENT

MEDICAL MANAGEMENT

Successful management of hemolytic-uremic syndrome (HUS) begins with early recognition of the

disease and supportive care. Management includes good control of volume status, electrolyte

abnormalities, hypertension, and anemia. Supportive care measures apply to both diarrhea-associated

hemolytic-uremic syndrome (D+HUS) and non – diarrhea-associated hemolytic-uremic syndrome (D

-

HUS). Additional special considerations for D- hemolytic-uremic syndrome are listed at the end of this

section.

Fluid therapy

o Early and ample hydration with intravenous isotonic saline is associated with a lower risk of progression

to oligoanuric hemolytic-uremic syndrome in patients with diarrhea (see Deterrence/Prevention).[5]

Studies

on fluid therapy in patients with established hemolytic-uremic syndrome are lacking; however, based on

the data above, the authors recommend that patients with hemolytic-uremic syndrome continue to receive

intravenous isotonic saline to maintain a euvolemic state.

o Monitor hydration status closely and frequently. This includes serial and frequent measurements of body

weight, fluid intake and output, heart rate, and blood pressure. Renal function may rapidly decline, so

laboratory test results obtained in the morning may not reflect the patient's renal function or electrolyte

status later in the day. Patients may develop fluid overload or hyperkalemia if not carefully managed.

o Monitor electrolytes. Testing may need to be performed frequently in the early stages of disease or while

children are on dialysis. In children in whom kidney function is stable, testing may be performed daily.

o Use potassium-free fluids until renal function has stabilized. Mild hypokalemia is tolerable and much less

critical than hyperkalemia. Treat severe or symptomatic hypokalemia with very cautious potassium

replacement.



o Once fluid deficits have been replaced, restrict fluid replacement to insensible losses plus actual output.

Management of acute renal failure

o Approximately 50% of patients with D+ hemolytic-uremic syndrome require a period of dialysis. Consider

early dialysis if the patient develops fluid overload, hyperkalemia, acidosis, hyponatremia, or oligoanuria

that is unresponsive to diuretics.

o Any type of dialysis or related technique (eg, hemofiltration) may be used, depending on local availability

and individual patient factors. Suitable techniques include peritoneal dialysis, hemodialysis, or continuous

renal replacement therapies (CRRT).

Peritoneal dialysis is widely used for pediatric patients. Peritoneal dialysis is usually well tolerated, and is

technically easier, especially in small infants.

Hemodialysis is also suitable for children. Hemodialysis may be preferable in patients with severe

abdominal pain, in whom intestinal edema and pain may reduce achievable fill volumes. The intense

visceral inflammation may lead to ultrafiltration failure. Omentectomy and placement of a peritoneal

catheter may worsen their pain and complicate evaluation of continued pain.

Abdominal pain is more complex to assess in patients with a new peritoneal catheter. Pain could be due to

a catheter-related complication, dialysis-associated peritonitis, or critical complications of hemolytic-

uremic syndrome, such as intestinal perforation.

CRRT may be preferable for hemodynamically unstable patients. CRRT allows very precise control of

volume status. CRRT also circumvents the issue of abdominal pain discussed above.

A growing body of evidence from critically ill patients shows that volume overload is a major contributor

to morbidity and mortality. Initiate dialysis promptly if patient has, or is approaching, a state of fluid

overload.

Dialysis does not alter the course of the disease; it only supports the patient while awaiting resolution of

the illness. Early dialysis as a preventive or therapeutic measure is not justified. Current data do not

support a previous theory that peritoneal dialysis could improve outcomes by removal of plasminogen-



activator inhibitor type 1 (PAI-1). However, several studies support early use of dialysis when indicated to

optimize fluid, electrolyte or nutritional status.

o Patients who require dialysis usually need 5-7 days of therapy, although this number widely varies.

Management of hematologic abnormalities

o Most children with hemolytic-uremic syndrome require packed RBC (PRBC) transfusions. PRBCs may be

administered for symptomatic anemia (eg, tachycardia, orthostatic changes in blood pressure or heart rate,

congestive heart failure) or if the hematocrit falls rapidly. The authors try to maintain the hemoglobin at

approximately 7 g/dL, or the lowest amount required to prevent symptomatic anemia. Maintaining a

relatively anemic state keeps the blood less viscous, theoretically helping prevent further thrombus

formation.

o Transfuse platelets if the patient has active bleeding. Other indications for platelet transfusion remain

controversial. Most physicians try to avoid platelet transfusion because it may promote platelet

aggregation and thrombus formation, worsening the disease. A commonly used threshold is to transfuse as

needed to maintain a platelet count near 20,000/mcL. Platelets may also be given just before a surgical or

catheter placement procedure.

Management of hypertension

o A wide range of antihypertensive medications are available, and treatment should be individualized.

o Calcium channel blockers such as amlodipine or isradipine are commonly used in pediatrics.

o ACE inhibitors are very effective but should be used with caution in individuals with a decreased

glomerular filtration rate (GFR) or with hyperkalemia.

o Treatment is covered separately in Hypertension.

Nutritional support

o Providing adequate protein and energy intake enterally or parenterally is important to prevent catabolism

and promote healing. Initiating dialysis, if needed, to provide adequate nutrition is preferred than to

withhold nutrition in the hopes of avoiding the need for dialysis.





o Patients may require intravenous hyperalimentation due to prolonged diarrhea, colitis, abdominal pain,

intestinal ileus, or anorexia.

o Lipid infusion may have to be limited if hypertriglyceridemia is present.

o Patients receiving CRRT may require additional nutrition due to amino acid removal by CRRT. Patients

receiving hyperalimentation while on CRRT may require 3-4 g/kg/d of protein. Consult a dietician with

renal expertise for assistance.

Pain management

o D+ hemolytic-uremic syndrome causes an intense colitis that can be extremely painful. Abdominal pain

may mimic that of an acute abdomen. Severe pain or acute changes in pain should be evaluated as a

surgical emergency just as with any other patient.

o Acetaminophen may be used.

o Avoid nonsteroidal anti-inflammatory drugs (NSAIDs) because of their nephrotoxicity, which is

particularly risky in an acutely injured kidney.

o Many patients will require opioid medication. Observe special precautions when using opioids in patients

with renal insufficiency or failure. Start with a low dose, titrate to effect, and observe carefully for signs of

toxicity.

Fentanyl has no active metabolites and is an excellent choice for patients with renal dysfunction. It has a

rapid onset of action but a relatively short duration.

Hydromorphone has active metabolites but they do not consistently cause symptoms in renal impairment.

Most authors consider hydromorphone to be relatively safe in renal patients, with cautious monitoring for

side effects, most commonly neuro-excitation.

Methadone has metabolites that are excreted primarily through stool. Methadone is a good analgesic in

renal impairment, but due to its slower onset of action and long half-life, it is less suitable for acute pain.

Do not use morphine, codeine, or meperidine in patients with decreased renal function. The human body

converts these drugs into numerous metabolites that have no analgesic function but cause many side



effects. Patients with renal failure cannot excrete these metabolites; thus, they accumulate and cause

nausea, vomiting, altered mental status, hallucinations, and other deleterious effects.

Little data are available on the use of most other opioid analgesics in patients with renal failure. Use other

agents with caution because the drug or its metabolites may have very different effects in patients with

renal failure as opposed to those with normal renal function.

o Patients should receive adequate pain control. Patients with renal disease require special care and

vigilance, but renal failure is not a valid reason to withhold appropriate pain management.

Special considerations for D- hemolytic-uremic syndrome

o Management of D- hemolytic-uremic syndrome is very difficult and remains poorly understood. Clinicians

caring for patients with D

-

hemolytic-uremic syndrome should search recent literature and confer with

physicians with expertise in this disorder.

o Discontinue offending agent if a drug-associated cause is identified.

o Treat bacterial infections (eg, S pneumoniae) promptly and aggressively.

o The role of plasma therapy in pneumococcal hemolytic-uremic syndrome (P-HUS) or neuraminidase-

mediated hemolytic-uremic syndrome remains controversial. Plasma may contain antibodies to the T

antigen, which, in theory, could worsen the hemolytic process. Alternately, plasma exchange may remove

neuraminidase and decrease the amount of circulating anti – T antibody. Some authors advocate plasma

exchange using albumin replacement.

o Plasma therapies form the mainstay of treatment for most forms of D-hemolytic-uremic syndrome. These

therapies use donor plasma products to replace the deficient or abnormal von Willebrand factor (vWF)

metalloproteinase or complement factors.

o No treatment has been found to be more effective than therapeutic plasma exchange (TPE), which is also

called plasmapheresis.

TPE is the most effective therapy for D- hemolytic-uremic syndrome. TPE removes the patient's plasma

and replaces it with fresh frozen plasma (FFP) or a similar product. Albumin should not be used for



replacement because it does not contain the vWF metalloproteinase or complement factors, except in the

case of P-hemolytic-uremic syndrome or neuraminidase mediated hemolytic-uremic syndrome

o Plasma infusion

Plasma infusion consists of simply infusing donor plasma, such as FFP or cryoprecipitate-reduced plasma.

In theory, this delivers the absent or abnormal vWF metalloproteinase or complement factors.

The sole advantage of plasma infusion is its simplicity because it can be performed in almost any medical

facility and does not require specialized equipment, central venous access, or specially trained staff.

Studies have found superior outcomes with TPE.[14]

Infusions typically consist of 20-30 mL of FFP or cryoprecipitate-reduced plasma per kg. One case report

found 40-45 mL/kg infusions necessary.[15]

Volume overload may complicate plasma infusion, especially in patients with reduced renal function. For

example, a 50-kg child receiving 40 mL/kg of plasma would require a 2000 mL infusion, approximately

equal to the entire daily fluid requirement for a patient with normal renal function. The risk of volume

overload may limit the volume administered, reducing the effectiveness of the therapy.

Hyperproteinemia, as shown by elevated serum total protein, has been reported in a patient receiving

chronic plasma infusion.

In theory, one can use exclusively cryoprecipitate-reduced plasma for plasma infusion because the

patient's own coagulation factors are not removed.

Management of end-stage renal disease (ESRD)

o Patients who develop permanent renal failure due to D+ hemolytic-uremic syndrome have a low risk of

recurrence and can proceed to renal transplantation similar to patients with most other renal diseases.

o Renal transplantation in patients with D- hemolytic-uremic syndrome is more difficult because of the high

risk of recurrence and allograft loss, with success rates of only 18-33% reported.



o The risk of recurrence varies with the complement mutation identified; such testing is essential is planning

and counseling patients about transplant options:

Factor H mutation: 80-100% recurrence

Factor I mutation: 80% recurrence

Membrane cofactor protein mutation: 10-20% recurrence

No (known) mutation identified: 30% recurrence

o Combined liver-kidney transplant has been reported in patients with high-risk mutations such as factor H.

Liver transplant alone is an option for patients without renal failure.

Surgical Care

Supportive medical care is the mainstay of treatment of hemolytic-uremic syndrome.

Obtain surgical consultation if the patient has severe abdominal pain or other abdominal findings, which

may be similar to an acute abdomen.

Surgery may also be required for placement of a dialysis catheter.

Medication

Supportive care remains the mainstay of therapy for hemolytic-uremic syndrome (HUS). Medications

such as antihypertensives, diuretics, anticonvulsants, and analgesics are indicated to treat specific

symptoms or complications of hemolytic-uremic syndrome. These medications are have not clearly

demonstrated to alter the disease process.

Eculizumab is the first treatment approved by the US Food and Drug Administration (FDA) in September

2011 for adults and children with atypical hemolytic uremic syndrome (aHUS).

Unfortunately, several agents that in theory should ameliorate hemolytic-uremic syndrome have failed to

do so in clinical trials. These include thrombolytic agents (eg, heparin, urokinase), platelet inhibitors (eg,



aspirin, dipyridamole), and a Shiga toxin (Stx) – binding agent (ie, Synsorb-Pk). Current evidence does not

support use of these medications.

Corticosteroids are not useful in diarrhea-associated hemolytic-uremic syndrome (D+ HUS). They may be

of value in non – diarrhea-associated hemolytic-uremic syndrome (D- HUS) if the patient has an

autoimmune-produced inhibitor of ADAMTS13. Clinical testing for inhibitors is available but has a long

turnaround time. Corticosteroid therapy may be initiated presumptively in patients with unexplained D-

hemolytic-uremic syndrome.

Limited case reports describe using intravenous immune globulin (IVIG) in patients with D- hemolytic-

uremic syndrome associated with organ transplantation. IVIG does not have a role in hereditary D-

hemolytic-uremic syndrome or in D+hemolytic-uremic syndrome.

Plasma therapies are covered in Treatment. They are indicated only for treatment of D- hemolytic-uremic

syndrome, or possibly D+ hemolytic-uremic syndrome with associated CNS involvement.

Studies have shown that antibiotics given to patients with diarrhea due to E coli0157:H7 increase the risk

of developing hemolytic-uremic syndrome. A theory proposed to explain this finding is that antibiotic

therapy causes rapid large-scale bacterial lysis with massive release of Stx, overwhelming host defense

mechanisms. Whether antibiotics affect the course of established hemolytic-uremic syndrome remains

unknown. Patients with E coli 0157 colitis usually clear the infection spontaneously.

Most pediatric nephrologists do not routinely use antibiotics in patients with D+hemolytic-uremic

syndrome, based on a theoretical concern it could exacerbate the disease process. However, antibiotics

should be used when indicated according to clinical judgment. Examples include patients having

suspected or documented bacteremia, urinary tract infection, or sepsis.

PREVENTION

General preventive measures



Avoid ingestion of raw or undercooked meat.

Avoid unpasteurized milk and cheese.

Practice good hand-washing technique, especially during outbreaks of diarrhea.

Wash hands well after touching livestock, farm animals or "petting zoo" animals. Supervise children to

ensure good technique.

Avoid taking antidiarrheal or antimotility agents for diarrhea. Avoid taking antibiotics for diarrhea unless

under the management of a physician.

Seek medical care immediately for bloody diarrhea.

Preventive measures for medical practitioners

Avoid antibiotic treatment of patients with possible GI E coli 0157:H7 infection, unless other clinical

factors require antibiotic therapy.

Use ample parenteral volume expansion with isotonic ("normal") saline in patients with suspected E

coli 0157:H7 infection (eg, those with bloody diarrhea). Early recognition is important.

A study has shown that early and ample rehydration with isotonic saline is associated with a lower risk of

developing oligoanuric renal failure.

Many patients who received this therapy still developed hemolytic-uremic syndrome, but they had a less

severe course, with shorter lengths of stay and fewer patients requiring dialysis.

Ake and colleagues recommend that patients with suspected E coli0157:H7 infection be admitted for

inpatient therapy, using intravenous isotonic saline for both maintenance and replacement fluid

requirements, avoiding use of hypotonic fluids.

The authors of this article concur with this advice.

Trials of oral rehydration, normally an appropriate practice, should be avoided in this situation due to the

risk of prolonged renal hypoperfusion.



Monitor fluid status, intake and output closely because renal function may change rapidly, requiring

adjustments to fluid therapy. Use potassium supplementation with great caution.

COMPLICATIONS

Renal system

Renal insufficiency

Renal failure

Hypertension

CNS

Mental retardation

Seizures

Focal motor deficit

Optic atrophy

Cortical blindness

Learning disability

Endocrine system

Diabetes mellitus

Pancreatic exocrine insufficiency

GI system - Intestinal necrosis

Cardiac system - Congestive heart failure





PROGNOSIS

D+ hemolytic-uremic syndrome

Most patients with D+ hemolytic-uremic syndrome who receive the appropriate treatment have a good

recovery. Recurrence is very rare. Poor prognostic indicators include the following:

Elevated WBC count at diagnosis

Prolonged anuria

Severe prodromal illness

Severe hemorrhagic colitis with rectal prolapse or colonic gangrene

Severe multisystemic involvement

Persistent proteinuria

The long-term prognosis for survivors of childhood D+ hemolytic-uremic syndrome remains unknown. A

five-year follow-up of a cohort of patients showed no difference in blood pressure and slightly higher rates

of microalbuminuria compared with controls.[28]

The patients also had lower glomerular filtration rates

(GFRs) as measured by cystatin C but not as measured by serum creatinine levels. Other studies have

shown similar findings. Continued long-term follow-up studies are needed to help determine whether

survivors have residual subclinical renal injury that could manifest itself later in life. At present, patients

should be counseled on avoiding risk factors for renal disease (eg, tobacco use, obesity, hypertension) and

the importance of continued medical follow-up.

D- hemolytic-uremic syndrome: The prognosis is more guarded than for D

+hemolytic-uremic syndrome.

Patients with D- hemolytic-uremic syndrome typically have frequent relapses and a higher risk of

progression to end-stage renal disease (ESRD).



PATIENT EDUCATION

DIET

Low-salt diet to decrease risk of hypertension

Diet high in iron and folic acid content to help recover from anemia

High-energy diet to help patient regain lost weight

Social worker or psychologist consultation to help the family cope with the illness



SU MITTED TO SU MITTED Y

Mrs NIRUPAM SAHU Ms SHIKHA TIRKEY

LECTURER M SC NURSING 1ST YEAR

CHILD HEALTH NURSING P G COLLEGE OF NURSING



IN EX

S.NO TOPIC PAGE NO.

1 INTRODUCTION

2 CLASSIFICATION

3 CAUSE

4 PATHOPHYSIOLOGY

5 CLINICALMANIFESTATION

6 DIAGNOSTIC EVALUATION

7 MANAGEMENT

8 PREVENTION

9 COMPLICATION

10 PROGNOSIS



BIBLIOGRAPHY:

1. James, Ashwill, Droster; Nursing care of children principles & practice; 2nd Edition; Pg no.

613-614

2. Suraj Gupte; The short textbook of pediatrics; 11th edition; Pg no. 516

3. Richard E. Behrman; Textbook Of pediatrics; 17th edition; Pg no. 1746-1747

4. Marilyn .J. Hockenberry; Essentials Pediatric Nursing; 7 th Edition; Pg no. 999-1000

5. Terri Kyle; Essentials of Pediatric Nursing; Elsevier Publication; Pg no. 742-743

6. Behrman, Klliegman, Jenson; Nelson Textbook of pediatrics; 17th edition; Pg no.1781

7. Waechter, Phillips, Holaday; Nursing Care of Children; 10th Edition; Pg no. 936-937

8. Hunsberger, Wright; Family Centered Nursing Care Of Children; 2nd edition; Pg no. 1545-

1546

9. Adele Pillitter; Child Health Nursing Care Of The Child & Family; Pg no. 796.

WEBSITES-

1. http://en.wikipedia.org/wiki/ hemolytic Uremic Syndrome

2. http://www.elook.org/dictionary/ hemolytic-uremic syndrome

3. http://www.slideshare.net/anitarobins/ hemolytic-uremic syndrome

4. http://www.answers.com/topic/ hemolytic-uremic syndrome

http://en.wikipedia.org/wiki/%20%20hemolytic



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