Refractory hyperammonemia on CRRT: Overwhelming hyperammonemia requiring multiple renal replacement therapy modalities

 

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Refractory hyperammonemia on CRRT: Overwhelming hyperammonemia requiring multiple renal replacement therapy modalities

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Samir
Gautam
Dayana Abilmona dayana.abilmona@medstar.net The MedStar Health (Baltimore) Internal Medicine Baltimore United States -
Fizza Naqvi fnaqvi3@jhmi.edu Johns Hopkins School of Medicine Internal Medicine/Nephrology Baltimore United States -
Samir Gautam sgautam6@jh.edu Johns Hopkins School of Medicine Internal Medicine/Nephrology Baltimore United States *
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Hyperammonemia syndrome (HS) is a rare but often fatal condition that occurs in immunosuppressed individuals, affecting approximately 4% of lung transplant recipients. It is characterized by progressively rising serum ammonia levels that overwhelms the liver’s clearance capacity. Ammonia readily crosses the blood-brain barrier and, together with glutamate, is converted to glutamine within astrocytes. Glutamine acts as an active osmolyte, and as ammonia levels quickly rise, the body’s ability to maintain intracellular osmolality becomes overwhelmed. This imbalance leads to astrocyte swelling, cerebral edema and ultimately death. Acute hyperammonemia requires rapid management through extracorporeal ammonia removal. Continuous renal replacement therapy (CRRT) serves as a critical intervention for rapidly and effectively reducing ammonia levels. We present a case of refractory hyperammonemia syndrome requiring multiple renal replacement therapy intervention, eventually double CRRT to normalize the hyperammonemia.

Case report


Hyperammonemia syndrome secondary to disseminated Ureaplasma infection is often refractory to treatment. Ureaplasma hydrolyzes urea to ammonia and carbon dioxide. Overwhelming the liver’s capacity to clear ammonia, neurological sequelae can be detrimental with life threatening cerebral edema. Along with antimicrobial treatment, extracorporeal clearance ammonia remains cornerstone in time sensitive management of those patients. Generally CRRT or intermittent hemodialysis are able to correct the hyperammonemia. While, intermittent hemodialysis is efficient in clearance of hyperammonemia, there is rebound phenomenon, so CRRT is generally preferred, especially in unstable patients. The usual recommended dose to start is between 50 ml/kg/hr and if needed can go upto 80 ml/kg/hr. In this case however, we had to continue to increase the support to the point of requiring two CVVHD machines in parallel to correct the hyperammonemia.

Kewords