Short and Long-term Outcomes of HUS

Related Information: Outcomes of hus, effect of HUS

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.

High BP occurs later in nearly 10% of those without oligoanuria, and 33% in those with oligoanuria (exceeding 15 days) and 66% in those with anuria (lasting for 15 days or more). Low GFR and proteinuria, both indicates impaired renal functions along with ongoing hyperfiltration injury. Such a combination exists in utmost 10% of people until there is anuria or oliguria for 5-10 days. Hence it will go up to 15% in people with oliguria greater than 10 days and 40% in case it lasts for greater than 15 days. Such a combination is exhibited in 20% for those with anuria lasting greater than 5 days, 33% in people with anuria lasting greater than 10 days and 66% for greater than 15 days.

In the long term, this leads to ESRD (end-stage renal disease) owing to ongoing hyperfiltration injury, occurring when greater than 50% of nephrons are destroyed (can happen in the acute phase). The remaining good nephrons enlarge to balance the lowered renal population. These nephrons are able to manage the work for several years, but they become overworked at the end. This deficiency will come out as microalbuminuria. This can serve to be a good marker to determine “hyperfiltration injury”. As the value increases, the injury is greater. Microalbuminuria can sometimes lead the beginning of proteinuria by a few years.

In addition, from the age of 30 onwards, there will be a decrease in the volume of nephrons. This places an additional damage to the kidneys. Angiotensin receptor blockers and angiotensin enzyme inhibitors lowers hyperfiltration injury and this can slow the natural process of nephron loss. But finally, if greater than 90% of nephrons are destroyed, ESRD results.

It is strongly recommended to assess the patients many times in the initial stages for determination of BP, serum creatinine, urinalysis and microalbuminuria. This helps in tracking the risk of ESRD well before. Even after the first year, evaluations must be continued. Even after recovery, a medical check every two years is recommended. And monitoring during pregnancies is a must owing to the increased risk of pre-eclampsia and eclampsia.

 

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