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Updated: Jan 20, 2026

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Published on: May 4, 2021
Life-Threatening Extreme Methemoglobinemia during Standard Dose Nitric Oxide Therapy
Roberta Centorrino1, Shivani Shankar-Aguilera2, Silvia Foligno2
1Division of Pediatrics and Neonatal Critical Care, "A. Béclère" Medical Center, South Paris University Hospitals, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France, robertacentorrino@hotmail.it.
Abstract:
We report the first case of life-threatening extreme neonatal-acquired methemoglobinemia that occurred during inhaled nitric oxide (iNO) at the standard 20 ppm dose in a neonate with early onset sepsis and suprasystemic pulmonary hypertension. Life-threatening methemoglobinemia has been efficaciously treated with methylene blue and ascorbic acid, while stopping iNO and starting iloprost and sildenafil. The patient was subjected to various tests (including gene sequencing and hemoglobin electrophoresis) and did not have any known genetic cause or predisposition for methemoglobinemia. Neuroimaging and the 2-year clinical follow-up were completely normal.
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