Atypical HUS

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No cure has been found out in the case of Atypical HUS. In reality, since each and every case varies, treatment cannot be standardized. Children suffering from Atypical HUS are in a greater life threatening situation. However, if the disease is diagnosed and treated early, there are chances for survival.

But the sad thing is that children with Atypical HUS often develop other complications like

? Recurrence

? Seizures/Neurological problems

? Kidney failure

? Supportive treatments will not prove effective

? In spite of kidney transplantation, Atypical HUS can occur again

? Hypertension in the severe form or very high blood pressure.

Causes of HUS

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Research has not yet found the causes for the Hemolytic Uremic Syndrome occurrence in most of the patients. Some of the known things about HUS are

1. The disease is non-contagious.

2. It is not a genetic disease except in some families.

3. It can affect people irrespective of age.

4. It affects women more than men.

5. Patients affected become overweight.

6. Sometimes, HUS occurs during or soon after pregnancy. Since the symptoms are similar to toxemia of pregnancy or preeclampsia (high BP resolved after pregnancy), it is difficult to be diagnosed correctly.

Diagnosis of HUS

Related Information: Diagnosis of hus, HUS diagnosis, hemolytic uremic syndrome symptoms

Hemolytic Uremic Syndrome can be diagnosed well though the symptoms vary among individuals. The healthcare provider or the doctor suspects the patient to have the disease depending upon the history (family or medical) and physical examination. There are some laboratory tests which help to confirm the diagnosis of HUS. They are

? Hemolytic anemia ? a low Red Blood Cells (RBC) count or peripheral blood smear, during the examination of the patient?s blood under a microscope, confirms the damage and destruction of the RBCs. The difference from hemolysis (hemo ? blood and lysis ? cleavage/ destruction) to anemia is that anemia is caused by a lowered production of RBCs from the bone marrow, but here the RBCs are lowered by damage and destruction and not lowered production.

? Thrombocytopenia ? a lower blood platelet count is seen.

? Uremia ? the proper functioning of the kidney can be assessed by determining the amount of waste products found in the blood which is generally removed off or excreted by the kidney. This done by determining the level of creatinine. It is the breakdown product of creatine, a muscle protein. Higher level of creatinine is a signal of uremia or kidney failure.

Symptoms of Hemolytic Uremic Syndrome

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The symptoms of Hemolytic Uremic Syndrome occur in two phases. These phases are similar to that of E. coli infection. E. coli infection, the food-borne illness, is mostly the reason behind HUS. Phase one of HUS includes gastrointestinal illnesses along with any or all of the following symptoms

? Bloody diarrhea

? Painful cramps

? Lethargy

? Fever

? Irritability

Phase two of HUS includes rigorous complications like

? Reduced or no output of urine

? Pale skin or skin rashes (red spots in skin)

? Easy bruising

? Jaundice (yellowing of skin)

? Lower consciousness

? Seizures

HUS Risk Factors

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Hemolytic uremic syndrome (HUS) is a common disease among children of age 10. People with immature or weak or underdeveloped immune systems like very young children or older adults possess the risk of being affected by hemolytic uremic syndrome mostly soon after an infection by E. coli. National Institutes of Health predicts that in almost half the patients who developed hemolytic uremic syndrome, there occurs a sudden kidney failure. Hence in reality, HUS is the best known cause of the acute kidney failure especially in children. The other possible occurrences are high blood pressure, heart problems, coma, stroke, chronic kidney failure apart from acute kidney failure.

Careful observation is very essential to look for any symptoms of hemolytic uremic syndrome soon after a patient is being diagnosed with an infection by shiga-toxin produced by E. coli strain O157:H7 (STEC). STEC has been found to be the leading cause of HUS. Further, it can begin within 3-14 days after bloody diarrhea starts. Almost nearly 3% to 15% of people infected by STEC are sure to develop HUS as well with a higher number of children in the age of 10 years and the elderly, especially the people with weaker immune systems and those who have more contacts with farm animals being the most to be infected.

Pathophysiology of HUS

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HUS frequently is preceded by an infectious disease, mostly diarrhea (90%) and an upper respiratory disease less frequently (10%). Use of anti-motility drugs increases the chances of developing HUS. HUS is especially caused by a toxin which is secreted by Escherichia coli O157:H7. The other agents are Shigella, Yersinia, Salmonella, and Campylobacter species.

Shiga and similar toxins produced by different strains of S. dysenteriae and also E. coli O157:H7. Almost 70% of the HUS cases are due to these toxins in children. Owing to their cytotoxic activity on vero cells, the toxins are known as verotoxins. It is normally transmitted through contaminated food like ground beef, undercooked cattle products, unpasteurized dairy products, etc. The contaminated food does not give any indications like smell, taste or look.

Even contact between persons or water supply contamination also helps in transmission. E. coli bacterium is normal flora which is found in gastrointestinal tracts of many cattle (healthy ones). Transmission into children can easily occur from a cow while petting it.

Plasma Infusion

Related Information: Plasma infusion, HUS treatment therapy, FFP (fresh frozen plasma)

Plasma is the fluid part of blood and contains blood cells, enzymes, hormones and nutrients required to the body. Hemolytic Uremic Syndrome (HUS) is mostly treated by plasma therapy, which involves the addition of fresh plasma to newborns, children inherited or people who acquired HUS. Soon after the detection of HUS in a person, plasma therapy is begun.

For those who inherited HUS, FFP (fresh frozen plasma) is administered intravenously for replacing the missing or altered ADAMTS13 enzyme. For those who had acquired HUS, plasmapheresis or plasma exchange is carried out. This removes the antibodies in the blood which damages ADAMTS13 enzyme. If this is unavailable, FFP is given till it is available.

Treatment with FFP or plasma exchange continues till there is an improvement in symptoms and there are negative blood tests. It can last for days or weeks based on the patient?s condition. Though recovered from HUS, some people experience flare-ups. This can occur immediately or after some time. If this occurs, the treatment needs to be restarted.

FFP represents the plasma separated from a single unit of whole Blood and later frozen to below ?20?C within the next eight hours after collection. FFP includes normal amounts of several coagulation factors and lacks RBCs, WBCs and platelets. It cannot be called a concentrate of the clotting factors. A single unit is roughly 225 ml and needs to be ABO that is compatible with the red cells of the recipient. Rh factor is not a matter of importance.

Prevention of HUS

Related Information: E. coli prevention, Hemolytic Uremic Syndrome Prevention

From research, it has been found that Hemolytic Uremic Syndrome can be caused by mostly E. coli or sometimes by Shigella and hence prevention of E. coli and Shigella needs to be taken of to prevent HUS.

A few simple safety measures can prevent HUS

? Proper cooking of meat. Undercooked foods especially meat at restaurants should be returned off.

Undercooked or partially cooked meat or beef can transmit infection by E. coli. Hence it is essential to eat only well cooked items both at home and restaurant. There is a digital instant-read meat thermometer which can identify the presence of bacteria. The presence of E. coli can be detected by placing the thermometer in numerous sections of the patty, particularly the thickest regions. It should read a minimum of 160? Fahrenheit to indicate the good condition of meat. In case of no thermometer, make sure that the patties are not pinkish in the middle. In case of a restaurant providing you such an item, just return it. Ensure cleanliness in all things like plate, spoon, etc. Do not hesitate in such things.

Serious Complication of HUS: Kidney Failure

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CDC has warned health professionals or doctors that treating patients affected by E. coli infection, food poisoning with antibiotics can even increase the risk and chances of the patient to develop Hemolytic Uremic Syndrome, which in turn can lead to very serious consequences like E. coli kidney failure. This is otherwise stated as renal failure caused by food poisoning by E. coli.

If left untreated or the wrong treatment given for Hemolytic Uremic Syndrome, it can easily result in long lasting damage to kidneys of the patient or even kidney/ renal failure. Most of the Hemolytic Uremic Syndrome cases result only after an infection in digestive system by E. coli strain O157:H7. This bacterium is normally found in food items like undercooked or partially cooked meats or beef, non-pasteurized milk products or juices, etc.

The bacterium stays in digestive system of animals and human beings. It secretes toxins which are capable of entering the blood stream and begin the destruction of red blood cells in the blood. The destructed red blood cells can block the comparatively smaller blood vessels of the kidneys, thus impairing the function of the kidneys to remove off the unwanted substances and excess fluid from blood.

Short and Long-term Outcomes of HUS

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The treatment for D+ HUS has seen a great improvement after the introduction of kidney dialysis along with intensive care facilities. In 1950, the mortality rate due to HUS was 40% and now it has been decreased to 3-5% in the developed world. In spite of this, patients die owing to inability to avoid and treat renal injury. Though damage to brain seems to be the commonest cause for death, multi-organ damage is also common.

Though people survive after treatment, in nearly 3-5% of people, extra-renal damage lasts long and another 3-5% with kidney damage. All this requires chronic dialysis or kidney transplants from the beginning or after a few years. And still others develop future sequelae that can be linked to the presence of anuria and oliguria. Sequelae includes low glomerular filtration rate [GFR], proteinuria, and hypertension. This can vary from mild to severe in people based on the condition of anuria and oliguria.