Abstract: PUB636
A Case of Severe Hyponatremia Managed with Prolonged 3% Saline in a Patient with Acute Intermittent Porphyria
Session Information
Category: Nephrology Education
- 1302 Fellows and Residents Case Reports
Authors
- Ahmed, Sayed Shayan, McGovern Medical School at UTHealth, Houston, Texas, United States
- Waguespack, Dia Rose, UTHealth, Houston, Texas, United States
Background
Acute Intermittent Porphyria (AIP) results from partial deficiency of heme biosynthetic enzyme porphobilinogen deaminase. Among various clinical manifestations, severe hyponatremia is a possible clinical presentation. Hyponatremia can occur from variety of entities including syndrome of inapprpriate antiduretic hormone (SIADH), losses from the gastrointestinal tract and renal sodium wasting. We report a case of severe hyponatremia in patient with AIP managed with aggressive and prolonged 3% saline replacement.
Methods
A 21 year old woman presented with generalized body pain. She had a history of rhabdomyolysis one month ago. During that admission she was managed with intravenous fluid and pain control. This admission she presented with similar symptoms, however her creatinine phosphokinase level was normal. Nephrology was consulted for severe hyponatremia (serum sodium level of 116 meq/L) that was consistent with SIADH on laboratory work up. She was initially treated with 3% saline boluses. Despite this her sodium level continued to decline. She was then transitioned to a continous 3% saline infusion at 25-30 ml/hr. The treatment goal was to maintain goal sodium correction of 6 meq/24hrs. During this time confirmatory tests for the diagnosis of AIP returned. She was started on directed treatment of AIP. Over next several days hypertonic saline therapy was discontinued as sodium level stabilized between 130-140 meq range with the treatment of her AIP.
Conclusion
This case was notable because of continued use of 3% saline over approximately 2 week period due to SIADH/salt wasting etiology. This case also demonstrates refractory hyponatremia in AIP requiring aggressive and prolonged repletion with 3% saline and eventual stabilization of sodium level after treating underlying etiology.