Pretreatment of enteral nutrition with sodium polystyrene sulfonate: effective, but beware the high prevalence of electrolyte derangements in clinical practice.

BACKGROUND: Current treatment options for chronic hyperkalemia in children with chronic kidney disease include dietary restrictions or enteral sodium polystyrene sulfonate (SPS); however, dietary restrictions may compromise adequate nutrition and enteral SPS may be limited by palatability, adverse effects and feeding tube obstruction. A potentially safer alternative is to pretreat enteral nutrition (EN) with SPS prior to consumption. The purpose of this study was to evaluate the efficacy and safety of pretreating EN with SPS in pediatric patients with hyperkalemia. METHODS: We performed a retrospective cohort study between September 2012 and May 2016 at the Children's Hospital of Philadelphia. In all, 14 patients (age range 0.5-53.2 months) who received 19 courses of SPS pretreatment of EN were evaluated. Serum electrolytes were evaluated at baseline and within 1 week of initiating therapy. The primary endpoint was mean change in potassium at 7 days. Secondary endpoints included the mean change in serum sodium, chloride, bicarbonate, calcium, phosphorous and magnesium, as well as the percentage of patients who developed electrolyte abnormalities within the first week of treatment. RESULTS: Serum potassium levels decreased from 6.0 to 4.4 mmol/L (P < 0.001) and serum sodium levels increased from 135.8 to 141.3 mmol/L (P = 0.008) 1 week after initiating SPS pretreatment. No significant differences in mean serum calcium or magnesium levels were noted. Nevertheless, more than half of the courses resulted in at least one electrolyte abnormality, with hypokalemia (31.6%), hypernatremia (26.3%) and hypocalcemia (21.1%) occurring most frequently. CONCLUSIONS: Pretreatment of EN with SPS is an effective method for treating chronic hyperkalemia in pediatric patients; however, close monitoring of electrolytes is warranted.

Neonatal atypical hemolytic uremic syndrome from a factor H mutation treated with eculizumab.

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) results from an inherited dysregulation of the alternative complement pathway leading to thrombotic microangiopathy consisting of hemolytic anemia, thrombocytopenia, and renal injury. The complement inhibitor eculizumab is an approved treatment, but its reported use in neonates - who have an inherently high risk of infection - is limited. CASE DIAGNOSIS/TREATMENT: A 28-day-old female presented with gross hematuria and hypertension. aHUS was suspected based on anemia with schistocytes, thrombocytopenia, low C3, and acute kidney injury requiring peritoneal dialysis. A septic work-up initiated on day 2 for hypothermia and respiratory failure was negative. There was no improvement after 6 days of plasma therapy. Despite being < 6 weeks old she was vaccinated with pneumococcal-13 conjugate, meningococcal (groups C and Y) polysaccharide, and Haemophilus b tetanus toxoid conjugate vaccines and started on penicillin prophylaxis. After 1 dose of eculizumab 300 mg, dialysis was discontinued and her hematological parameters improved. Genetic testing revealed a complement factor H mutation. After 11 months of follow-up, she remains on eculizumab and penicillin without recurrence of aHUS or any infectious complications. CONCLUSIONS: Eculizumab is a safe and effective treatment option for aHUS even in neonates at high risk for infection.