Paroxysmal nocturnal hemoglobinuria (PNH) could be a cause of acute renal failure in pediatric patients, according to a case report abstract published in a special edition of The American Journal of the Medical Sciences.
The study was presented at the 2023 Southern Regional Meeting that took place in New Orleans, Louisiana, from February 2 to 4.
The authors reported the case of a 15-year-old female patient who presented to the pediatric emergency department with persistent nausea and vomiting. Two weeks before, she experienced fever, vomiting, and respiratory symptoms, leading to the diagnosis of influenza A. The patient also reported having a history of rhabdomyolysis.
Upon physical exam, the patient was hypertensive and dehydrated. The laboratory results indicated renal failure with elevated blood urea nitrogen (138) and creatinine (15.09), as well as normocytic anemia (hemoglobin 8.3, MCV 87). The urine analysis revealed hematuria with a total of 12 red blood cells.
Read more about PNH complications
Kidney biopsy results suggested a hemolysis-associated hemoglobin cast nephropathy. Laboratory results, aside from elevated levels of lactate dehydrogenase (LDH), did not indicate hemolysis. In consultation with pediatric hematologists, the study authors performed a PNH flow cytometry that eventually confirmed the diagnosis.
Next, the doctors placed a dialysis catheter for hemodialysis and administered amlodipine for the treatment of hypertension.
When the patient’s renal function stabilized, hemodialysis was no longer indicated and the dialysis catheter was removed. Hypertension was resolved, hence amlodipine could be discontinued. The patient was followed up at the department of pediatric hematology after discharge from the hospital.
As PNH may negatively affect the complement system and increase the risk of infection with encapsulated organisms, the patient was immunized with Menactra and Bexsero and started on prophylactic PenVK. A bone marrow aspirate and biopsy, obtained to evaluate the risk of bone marrow failure, revealed no myelodysplasia. Moreover, the patient was started on ravulizumab with significant clinical improvement.
“Paroxysmal nocturnal hemoglobinuria is rare in pediatric patients, and the diagnosis may be challenging. Our patient’s previous diagnosis of rhabdomyolysis was believed to be a subtler episode of PNH. Accurate diagnosis is crucial for proper management and prevention of complications,” Tiller and colleagues wrote.
The onset of PNH is typically associated with adults aged 30 to 40 years. As the condition presents with nonspecific symptoms such as fatigue, dark urine, or renal insufficiency, the diagnosis is often delayed.
Reference
Tiller E, Lewis H, Harrison VA. Paroxysmal nocturnal hemoglobinuria: a rare cause of acute renal failure in a pediatric patient. Am J Med Sci. Published online January 27, 2023. doi:10.1016/S0002-9629(23)00263-X
