My son had typical HUS at age 5 in 1998. He is now 23 yr old, so 18 yrs since HUS. He was anuric and on peritoneal dialysis for 2-2 1/2 weeks. He had CNS complications (cerebellar signs told were mini-strokes) for 1 1/2 days, resolved with plasmapheresis ? He was accidentally fluid overloaded prior to the onset of CNS symptoms. He did not have hypertension during acute illness. He has no known neurologic sequelae. At discharge his nephrologist recommended: avoid dehydration get IV fluids if has vomiting and diarrhea or can’t tolerate liquids (we did this 3 times) avoid NSAID’s, get regular follow up even if he has a period where he appears to have recovered. He has had 2 episodes of severe abdominal pain (“can’t stand up straight due to pain”) over the past year. When he went in to ER to rule out appendicitis his symptoms resolved after being NPO for 8 hr and given IV fluids. He had marked tenderness in the epigastric area with no rebound. A CT scan with contrast was discussed. He did not get one since he was improving. I have now added IV contrast to the list of what to avoid unless absolutely neccessary and he is well hydrated prior. His last adult nephrologist told him he was fully recovered and if he has any kiney problems in the future it would most likely be do to something other than his past HUS. She did not have his complete records at the time. My impression is that the incidence of proteinuria and hypertension increases after 10 years. He has had 500mg protein in urine for the past year (increased from prior years), and his blood pressure was 130/83 last visit (it had usually been 60-65 diastolic). His Creatinine was 1.23, increased from 1.0. He weighs 136lb and is 5’10” tall. He has 2-3 bowel movements a day and on 1 occasion may have had fatty stools. He was not evaluated for pancreatitis when he was in the ER to rule our appendicitis. My questions are: Is pancreatic insufficiency or pancreatitis known to be associated with HUS? Could it develop 18 yr after the acute HUS? He is going to be evaluated for this. I still think the original recommendations given by the doctor who discharged him make sense but it is hard to make that case given what his last nephrologist said. I am going to have another nephrologist see him but this is the second nephrologist to say he is “recovered”. How much water should he drink a day? He is always dehydrated at his yearly checkups. He makes some attempts to drink water frequently. I try to not nag him about it now that he is recovered? He gets fever blisters and when I read about acyclovir and it cautions about using it with decreased kidney function. He has not taken it so far. Would it be OK for him to take it if he was sure to be well hydrated? I am obviously concerned that he is not actually recovered. My husband and I have been careful to not show our concern and have told him he is doing great and if he keeps doing what he has been it should stay that way. Now that he is an adult he will be handling this with his nephrologist and I hope that he gets accurate information going forward, with good advice about how to follow up in the future.

Hemolytic Uremic Syndrome (HUS) that occurs in childhood may cause residual kidney damage but it is unlikely to recur unless he has some type of genetic predisposition such as a complement deficiency or other genetic defect in his complement system.  I agree that he should be tested for kidney damage with blood and urine testing at least annually.  I do not think pancreatitis is a cause of HUS.  Routine follow up with his primary care physician (PCP) should be adequate to monitor for any new or residual kidney disease that may occur.  I do not recommend genetic testing unless he has another attack of acute kidney injury or abnormal blood counts such as new onset of anemia or thrombocytopenia.

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