ICD-10: D59.3

Hemolytic-uremic syndrome

Additional Information

Description

Hemolytic-uremic syndrome (HUS) is a serious condition characterized by a triad of symptoms: hemolytic anemia, acute renal failure, and thrombocytopenia. The ICD-10 code D59.3 specifically refers to this syndrome, which can arise from various underlying causes, including infections, particularly those caused by certain strains of Escherichia coli (E. coli), as well as other factors such as genetic predispositions and certain medications.

Clinical Features of Hemolytic-Uremic Syndrome

1. Hemolytic Anemia

Hemolytic anemia in HUS results from the destruction of red blood cells, leading to symptoms such as fatigue, pallor, and shortness of breath. Laboratory findings typically show low hemoglobin levels and elevated levels of lactate dehydrogenase (LDH), along with the presence of schistocytes (fragmented red blood cells) on a blood smear.

2. Acute Renal Failure

Acute renal failure is a hallmark of HUS, often presenting with oliguria (reduced urine output) or anuria (absence of urine output). Patients may experience fluid overload, electrolyte imbalances, and uremic symptoms, which can include nausea, vomiting, and confusion. Renal function tests typically reveal elevated serum creatinine and blood urea nitrogen (BUN) levels.

3. Thrombocytopenia

Thrombocytopenia, or low platelet count, is another critical feature of HUS. This can lead to increased bleeding tendencies, such as petechiae (small red or purple spots on the body) and easy bruising. Platelet counts are often significantly reduced in affected individuals.

Etiology and Pathophysiology

HUS can be classified into two main types:

  • Typical HUS: Often associated with infections, particularly those caused by Shiga toxin-producing E. coli (STEC), which is commonly linked to outbreaks from contaminated food or water. The toxin damages endothelial cells in the kidneys, leading to the clinical manifestations of HUS.

  • Atypical HUS: This form is not typically associated with infections and may be due to genetic mutations affecting the complement system, leading to uncontrolled complement activation and subsequent endothelial damage. Atypical HUS can also be triggered by certain medications, pregnancy, or other medical conditions.

Diagnosis

Diagnosis of HUS is primarily clinical, supported by laboratory findings. Key diagnostic tests include:

  • Complete blood count (CBC) to assess hemoglobin and platelet levels.
  • Peripheral blood smear to identify schistocytes.
  • Renal function tests to evaluate creatinine and BUN levels.
  • Stool cultures may be performed to identify STEC in cases of typical HUS.

Treatment

Management of HUS focuses on supportive care, including:

  • Fluid management: Careful monitoring and management of fluid intake and output to prevent overload.
  • Dialysis: May be necessary in cases of severe renal failure.
  • Transfusions: Red blood cell transfusions may be required for severe anemia, and platelet transfusions may be considered in cases of significant bleeding.
  • Eculizumab: For atypical HUS, eculizumab, a complement inhibitor, has been shown to be effective in reducing the frequency of HUS episodes and improving renal outcomes.

Conclusion

ICD-10 code D59.3 encapsulates the critical aspects of hemolytic-uremic syndrome, a condition that requires prompt recognition and management due to its potential for serious complications, particularly acute renal failure. Understanding the clinical features, etiology, and treatment options is essential for healthcare providers to effectively manage patients with this syndrome. Early intervention can significantly improve outcomes and reduce the risk of long-term renal damage.

Clinical Information

Hemolytic-uremic syndrome (HUS) is a serious condition characterized by a triad of symptoms: hemolytic anemia, acute renal failure, and thrombocytopenia. This syndrome can arise from various etiologies, including infections, particularly those caused by certain strains of Escherichia coli, as well as other infectious agents and non-infectious causes. Below is a detailed overview of the clinical presentation, signs, symptoms, and patient characteristics associated with HUS, particularly in the context of the ICD-10 code D59.3.

Clinical Presentation

Triad of Symptoms

  1. Hemolytic Anemia: This is characterized by a decrease in red blood cells due to their destruction. Patients may present with fatigue, pallor, and jaundice. Laboratory findings typically show elevated levels of lactate dehydrogenase (LDH), low haptoglobin, and the presence of schistocytes on a peripheral blood smear[1][2].

  2. Acute Renal Failure: Patients often experience a sudden decline in kidney function, which may manifest as oliguria (reduced urine output) or anuria (absence of urine output). Laboratory tests may reveal elevated serum creatinine and blood urea nitrogen (BUN) levels, indicating impaired renal function[1][3].

  3. Thrombocytopenia: A significant drop in platelet count is common, which can lead to increased bleeding tendencies. Patients may present with petechiae, purpura, or more severe bleeding complications[2][4].

Additional Symptoms

  • Gastrointestinal Symptoms: Many patients, especially those with infectious HUS, may present with gastrointestinal symptoms such as abdominal pain, diarrhea (often bloody), and vomiting. These symptoms are particularly common in cases associated with Shiga toxin-producing E. coli (STEC) infections[3][5].
  • Neurological Symptoms: In some cases, neurological manifestations such as seizures, confusion, or altered mental status may occur, particularly in severe cases or in children[2][6].

Signs and Symptoms

Common Signs

  • Pallor: Due to anemia.
  • Jaundice: Resulting from hemolysis.
  • Edema: Particularly in the lower extremities, due to fluid overload from renal failure.
  • Hypertension: Can occur due to fluid retention and renal impairment[1][3].

Laboratory Findings

  • Complete Blood Count (CBC): Shows anemia and thrombocytopenia.
  • Peripheral Blood Smear: May reveal schistocytes, indicating microangiopathic hemolytic anemia.
  • Renal Function Tests: Elevated creatinine and BUN levels.
  • Coagulation Studies: May show normal coagulation parameters, distinguishing HUS from thrombotic thrombocytopenic purpura (TTP) which typically presents with more severe coagulopathy[4][5].

Patient Characteristics

Demographics

  • Age: HUS is most commonly seen in children, particularly those under the age of 5, but it can also occur in adults. The incidence is higher in young children due to the prevalence of infections from contaminated food sources[3][6].
  • Gender: There is no significant gender predisposition, although some studies suggest a slight male predominance in certain age groups[2][4].

Risk Factors

  • Infectious Etiologies: Recent gastrointestinal infections, particularly those caused by STEC, are significant risk factors. Outbreaks are often linked to undercooked beef, unpasteurized dairy products, and contaminated vegetables[5][6].
  • Underlying Health Conditions: Patients with compromised immune systems or pre-existing renal conditions may be at higher risk for developing HUS following an infectious trigger[1][3].

Conclusion

Hemolytic-uremic syndrome (ICD-10 code D59.3) presents a complex clinical picture characterized by a triad of hemolytic anemia, acute renal failure, and thrombocytopenia. Understanding the clinical features, signs, symptoms, and patient demographics is crucial for timely diagnosis and management. Early recognition and intervention can significantly improve outcomes, particularly in pediatric populations where the syndrome is most prevalent. If you suspect HUS in a patient, prompt laboratory evaluation and supportive care are essential to mitigate the risks of severe complications.

Approximate Synonyms

Hemolytic-uremic syndrome (HUS), classified under ICD-10 code D59.3, is a serious condition characterized by a triad of symptoms: hemolytic anemia, acute renal failure, and thrombocytopenia. This syndrome can arise from various causes, particularly infections, and is most commonly associated with Shiga toxin-producing Escherichia coli (STEC) infections. Below are alternative names and related terms associated with this condition.

Alternative Names for Hemolytic-Uremic Syndrome

  1. HUS: The most common abbreviation for hemolytic-uremic syndrome.
  2. Shiga Toxin-Associated Hemolytic-Uremic Syndrome: Specifically refers to HUS caused by Shiga toxin-producing bacteria, particularly E. coli O157:H7.
  3. Post-Diarrheal Hemolytic-Uremic Syndrome: This term highlights the syndrome's association with gastrointestinal infections leading to diarrhea.
  4. Atypical Hemolytic-Uremic Syndrome (aHUS): Refers to a variant of HUS that is not associated with infections and is often linked to genetic factors or complement system dysregulation.
  1. Thrombotic Microangiopathy: A broader category that includes HUS and other conditions characterized by small blood vessel damage, leading to thrombosis and organ damage.
  2. Acute Kidney Injury (AKI): A common complication of HUS, where the kidneys suddenly become unable to filter waste from the blood.
  3. Thrombocytopenia: A condition of low platelet count, which is a hallmark of HUS.
  4. Hemolytic Anemia: Refers to the breakdown of red blood cells, which is a key feature of HUS.
  5. Renal Failure: Often used interchangeably with acute kidney injury in the context of HUS, indicating the kidneys' inability to function properly.

Clinical Context

Understanding these alternative names and related terms is crucial for healthcare professionals when diagnosing and treating patients with HUS. The condition can have various etiologies, and recognizing the specific type of HUS can guide appropriate management strategies. For instance, atypical HUS may require different therapeutic approaches compared to the more common post-diarrheal form associated with STEC infections.

In summary, hemolytic-uremic syndrome (ICD-10 code D59.3) is known by several alternative names and is associated with various related terms that reflect its clinical manifestations and underlying mechanisms. Recognizing these terms can enhance communication among healthcare providers and improve patient care outcomes.

Diagnostic Criteria

Hemolytic-uremic syndrome (HUS) is a serious condition characterized by a triad of symptoms: hemolytic anemia, acute renal failure, and thrombocytopenia. The ICD-10 code D59.3 specifically refers to hemolytic-uremic syndrome, and its diagnosis involves several clinical criteria and laboratory findings. Below is a detailed overview of the criteria used for diagnosing HUS, particularly in the context of the ICD-10 classification.

Clinical Criteria for Diagnosis

1. Clinical Presentation

  • Hemolytic Anemia: Patients typically present with signs of hemolytic anemia, which may include fatigue, pallor, and jaundice. Laboratory tests will show a decreased hemoglobin level and elevated levels of lactate dehydrogenase (LDH).
  • Thrombocytopenia: A significant drop in platelet count is observed, often below 150,000 platelets per microliter of blood. This can lead to increased bleeding tendencies.
  • Acute Renal Failure: Patients may exhibit signs of acute kidney injury, such as decreased urine output, elevated serum creatinine, and urea levels. This renal impairment is a hallmark of HUS.

2. Laboratory Findings

  • Peripheral Blood Smear: The presence of schistocytes (fragmented red blood cells) on a blood smear is indicative of microangiopathic hemolytic anemia, which is common in HUS.
  • Coagulation Studies: Normal coagulation studies (PT and aPTT) help differentiate HUS from other thrombotic microangiopathies like Thrombotic Thrombocytopenic Purpura (TTP).
  • Renal Function Tests: Elevated creatinine and blood urea nitrogen (BUN) levels confirm renal impairment.

3. Exclusion of Other Conditions

  • It is crucial to rule out other causes of hemolytic anemia and acute renal failure, such as TTP, disseminated intravascular coagulation (DIC), and infections (e.g., those caused by Shiga toxin-producing E. coli).

Diagnostic Codes and Guidelines

ICD-10 Code D59.3

  • The ICD-10 code D59.3 is specifically designated for hemolytic-uremic syndrome. This code is used in clinical settings to document the diagnosis for billing and statistical purposes. It is essential for healthcare providers to ensure accurate coding based on the clinical criteria outlined above.

Additional Considerations

  • Epidemiological Context: HUS can be associated with certain infections, particularly those caused by Shiga toxin-producing bacteria. Understanding the epidemiological context can aid in diagnosis and management.
  • Follow-Up and Monitoring: Patients diagnosed with HUS require close monitoring for potential complications, including chronic kidney disease, which can develop following acute episodes.

Conclusion

The diagnosis of hemolytic-uremic syndrome (ICD-10 code D59.3) relies on a combination of clinical symptoms, laboratory findings, and the exclusion of other similar conditions. Accurate diagnosis is critical for effective management and treatment of this potentially life-threatening syndrome. Healthcare providers must be vigilant in recognizing the signs and symptoms to ensure timely intervention and improve patient outcomes.

Treatment Guidelines

Hemolytic-uremic syndrome (HUS), classified under ICD-10 code D59.3, is a serious condition characterized by hemolytic anemia, acute renal failure, and thrombocytopenia. The management of HUS can vary based on its etiology, with the most common forms being typical HUS, often associated with Shiga toxin-producing Escherichia coli (STEC), and atypical HUS (aHUS), which is often linked to genetic factors or complement dysregulation. Below, we explore standard treatment approaches for HUS, focusing on both typical and atypical forms.

Treatment Approaches for Hemolytic-Uremic Syndrome

1. Supportive Care

Supportive care is the cornerstone of HUS management, regardless of its type. This includes:

  • Fluid Management: Careful monitoring and management of fluid intake and output are crucial to prevent fluid overload, especially in patients with acute renal failure.
  • Electrolyte Monitoring: Regular monitoring of electrolytes, particularly potassium, is essential due to the risk of hyperkalemia associated with renal impairment.
  • Blood Transfusions: Transfusions may be necessary for patients with severe anemia or significant thrombocytopenia, although they are used cautiously due to the risk of further hemolysis.

2. Treatment of Typical HUS

Typical HUS, often triggered by infections, particularly those caused by STEC, generally requires:

  • Antibiotics: The use of antibiotics is controversial in typical HUS, as they may increase the risk of hemolysis and worsen the condition. Therefore, they are typically avoided unless there is a clear indication for their use.
  • Plasma Exchange: In severe cases, especially when there is a rapid decline in renal function, plasma exchange may be considered to remove circulating toxins and inflammatory mediators.

3. Treatment of Atypical HUS

Atypical HUS is often associated with genetic mutations or complement dysregulation and may require more specialized treatment:

  • Complement Inhibitors: Medications such as eculizumab, a monoclonal antibody that inhibits the complement system, have shown efficacy in treating aHUS. This treatment is particularly beneficial for patients with complement-mediated aHUS.
  • Plasma Exchange: Similar to typical HUS, plasma exchange can be beneficial in aHUS, especially in acute settings or when renal function is severely compromised.
  • Renal Replacement Therapy: In cases of acute kidney injury, dialysis may be necessary until renal function recovers.

4. Long-term Management

Long-term management of HUS, particularly aHUS, may involve:

  • Genetic Counseling: For patients with aHUS, genetic counseling may be recommended to understand the hereditary nature of the condition and its implications for family members.
  • Regular Monitoring: Ongoing monitoring of renal function and blood parameters is essential to detect any recurrence or complications early.

Conclusion

The management of hemolytic-uremic syndrome (ICD-10 code D59.3) requires a tailored approach based on the underlying cause. Supportive care remains fundamental, while specific treatments such as complement inhibitors for atypical HUS and careful management of fluid and electrolytes are critical. Early recognition and intervention can significantly improve outcomes for patients suffering from this serious condition. As research continues, treatment protocols may evolve, emphasizing the importance of staying updated with the latest clinical guidelines and evidence-based practices.

Related Information

Description

  • Serious condition characterized by triad of symptoms
  • Hemolytic anemia, acute renal failure, thrombocytopenia
  • Destruction of red blood cells leading to fatigue and shortness of breath
  • Acute renal failure presenting with oliguria or anuria
  • Thrombocytopenia leading to increased bleeding tendencies
  • Damage to endothelial cells in kidneys by Shiga toxin
  • Uncontrolled complement activation causing endothelial damage

Clinical Information

  • Hemolytic anemia with red blood cell destruction
  • Acute renal failure with oliguria or anuria
  • Thrombocytopenia with low platelet count
  • Gastrointestinal symptoms like abdominal pain and diarrhea
  • Neurological symptoms in severe cases
  • Pallor due to anemia
  • Jaundice from hemolysis
  • Edema in lower extremities
  • Hypertension due to fluid retention
  • Schistocytes on peripheral blood smear
  • Elevated lactate dehydrogenase (LDH) levels
  • Low haptoglobin levels

Approximate Synonyms

  • HUS
  • Shiga Toxin-Associated HUS
  • Post-Diarrheal HUS
  • Atypical Hemolytic-Uremic Syndrome (aHUS)
  • Thrombotic Microangiopathy
  • Acute Kidney Injury (AKI)
  • Thrombocytopenia
  • Hemolytic Anemia

Diagnostic Criteria

  • Hemolytic anemia
  • Acute renal failure
  • Thrombocytopenia
  • Schistocytes on peripheral blood smear
  • Normal coagulation studies
  • Elevated creatinine and BUN levels
  • Exclusion of TTP, DIC, and infections

Treatment Guidelines

  • Fluid management to prevent overload
  • Electrolyte monitoring for hyperkalemia
  • Blood transfusions for severe anemia
  • Avoid antibiotics in typical HUS
  • Plasma exchange for toxin removal
  • Complement inhibitors for aHUS
  • Dialysis for acute kidney injury
  • Genetic counseling for family members
  • Regular monitoring of renal function

Coding Guidelines

Code Also

  • acute kidney failure (N17.-)
  • , if applicable, any associated:
  • chronic kidney disease (N18.-)

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