Downloaded 12 times
Uploaded byRivindu Wickramanayake
Hemolytic uremic syndrome
Hemolytic-uremic syndrome (HUS) is a disease characterized by hemolytic anemia, low platelet count, and kidney failure. It is predominantly seen in children and can be typical (caused by E. coli or Shigella infection) or atypical (caused by complement abnormalities or other infections). Treatment involves supportive care, antibiotics for infections, and plasma therapy for complement abnormalities to replace deficient factors. With aggressive treatment, over 90% of patients survive the acute phase, though some may have long term kidney or other organ damage.
EducationâŠ
More Related Content
Similar to Hemolytic uremic syndrome
More from Rivindu Wickramanayake
Hemolytic uremic syndrome
- 1. BY: DR NAJIBULLAHSUHRABY FMR FIRSTYEAR Hemolytic-Uremic Syndrome Bhavya Koganti Group no. 03a 6th Year 2nd Semester â 2020 September Tbilisi State Medical University, Georgia
- 2. Definition ï HUS, isa disease characterized by : ï§ Hemolytic anemia ï§ Uremia ï§ Low platelet count ï It predominantly, but not exclusively, affects children.
- 3. Types - HUS ïTypical HUS ïAtypical HUS ïHUS due to Complement abnormalities
- 4. Classification of HUS/TTPaccording to Etiopathogenesis âą Shiga toxin producing E.coli/Shigella âą Pneumococcal infection âą HIV âą Other viral or bacterialinfections ï Type of HUS / TTP Specific Cause ï Infection related Typical âą Complement factorabnormality Factor H deficiency Factor I deficiency âą Miscellaneous -Atypical CTD Drugs Malignancy
- 5. Etiopathogenesis ï Typical/Diarrhea associated/ShigaToxin associated HUS ï Enterohaemorrhagic E. coli ï Shigella dysenteriae type 1 ï Rarely, HUS can occur with E. coliUTI
- 6. Continued; ï The commonserotype of Ecoli:0157:H7 ï However, only about 10-15% patients with E. coli 0157:H7 infection will develop HUS ï Sources of infection are : ï Milk and animal products (incompletely cooked beef, pork, poultry,lamb) ï Human feco-oral transmission ï Vegetables, salads and drinking water may be contaminated by bacteria shed in animal wastes
- 8. Can this feedingtransmit?
- 9. Atypical/non-diarrhea related HUS ïPneumococcal HUS ï HUS due to Complement abnormalities ï Miscellaneous Causes of HUS / TTP ïŒ Abnormalities in intracellular vitamin B12 metabolism ïŒ HIV ïŒ Systemic lupus erythromatosus ïŒ Malignancies ïŒ Radiation ïŒ Certain drugs
- 10. Other Infections AssociatedWith HUS ï Include viruses like : ï Influenza ï Cytomegalovirus ï Infectious mononucleosis ï Bacteria like: ï Streptococcii ï Salmonella
- 11. Continued; ï The typicalpathophysiology involves the shiga-toxin binding to proteins on the surface of glomerular endothelium and inactivating a metalloproteinase called ADAMTS13, which is also involved in the closely related TTP ï The arterioles and capillaries of the body become obstructed by the resulting complexes of activated platelets which have adhered to endothelium via large multimeric vWF. ï The growing thrombi lodged in smaller vessels destroy RBCs as they squeeze through the narrowed blood vessels, forming schistocytes, or fragments of sheared RBCs.
- 12. ï The consumptionof platelets as they adhere to the thrombi lodged in the small vessels typically leads to mild or moderatethrombocytopaenia ï However, in comparison to TTP, the kidneys tend to be more severely affected in HUS, and the central nervous system is less commonly affected Continued;
- 13. Clinical Features ï Thecommonest clinical presentationof HUS is : ï Acute pallor ï Oliguria ï Diarrhea ordysentery ï It occurs commonly in children between 1-5years of age ï HUS develops about 5-10 days after onset of diarrhea ï Hematuria and hypertension arecommon. ï Complicationsof fluid overload may presentwith: ï Pulmonaryedema ï Hypertensiveencephalopathy ï Despite thrombocytopenia, bleeding manifestations are rare ï Neurological symptomslike: ï Irritability ï Encephalopathy ï Seizures
- 14. Investigations ï CBC ï Peripheralblood smears ï Reticulocyte count ï LDH ï Bili unconjugated ï Cr & BUN ï Urine analysis ï Hemoglobinuria ï Hematuria ï Proteinuria
- 15.
- 16. Investigations to IdentifyCause ï In patients with dirrhea, the identificationof pathogenic EHEC or Shigella is performedby: ï Stool culture ï Further serotyping by agglutination or enzyme immunoassay ï Rarely HUS can occur withE. coli UTI: ï Urine cultures are indicated in non-diarrhealpatients ï Bacteriological cultures of body fluidsare indicated in suspected pneumococcal disease. ï Sputum ï CSF ï Blood ï Pus
- 17. Diagnosis ï Clinically, HUScan be very hard to distinguish from TTP ï The laboratory features are almost identical, and not every case of HUS is preceded bydiarrhea ï HUS is characterized by the triadof: ï Hemolytic anemia ï Thrombocytopenia ï Acute renal failure ï The only distinguishing feature is that in TTP fever and neurological symptoms are often present, but this is not always the case ï A pericardial friction rub can also sometimes be heard on auscultation ï The two conditions are sometimes treated as a single entity called TTP/HUS.
- 18. Management ï Supportive Therapy ïAntibiotics ï Plasma Therapy ï Miscellaneous
- 19. ï In allpatients, supportive treatment is primary. ï Close clinical monitoring of : ï Fluid status ï Blood pressure ï Neurological ï Ventilatory parameters ï Blood levels of glucose, electrolytes, creatinine and hemogram need frequent monitoring ï The use of antimotility therapy for diarrhea has been associated with a higher risk of developing HUS ï With the onset of acute renal failure : ï Fluid restriction ï Diuretics Supportive Therapy
- 20. Antibiotics ï E. coli ïShigellosis ï pneumococcal HUS Plasma Therapy ï In a HUS due to: ï complement factor abnormality ï ADAMTS13 deficiency ï The replacement of the deficient factor with FFP ï Daily plasma infusions (10 to 20mL/kg/day) ï Exchange of 1.5 times plasmavolume ( 60 to 75 mL/kg/day) using FFP
- 21. Miscellaneous ï In infantswith HUS associated withcobalamin abnormalities: ï Treatment with hydroxycobalamin ï Oral betaine ï Folic acid ï Normalizes the metabolic abnormalities can help to prevent furtherepisodes. ï In patients with persistentADAMTS13 antibodies and poor response to plasma exchange: ï Immunosuppressive therapy with high dose steroids/cyclophosphamide/ cyclosporin/rituximab ï Splenectomy
- 22. Prognosis ï With aggressivetreatment, more than 90% survivethe acute phase. ï About 9% may develop end stage renal disease ï About one-third of persons with HUS have abnormal kidney function many years later, and a few require long- term dialysis. ï Another 8% of persons with HUS have other lifelong complications, such as : ï High blood pressure ï Seizures ï Blindness ï Paralysis
- 24. Key Messages ï Goodsanitation and maintenance of food hygienecan prevent diarrhea associated HUS. ï Supportive care with early dialysis support remains the cornerstone of management. ï Non-infective atypical HUS should be treated rapidly with plasma therapy. ï Efforts should be made to make an etiological diagnosis in cases of atypical HUS as treatment and prognosis is affected.