Journal article
A Severe Reaction After Phototherapy in a Neonate With X-Linked Protoporphyria
Pediatrics (Evanston), Vol.154(2), e2023064724
08/01/2024
DOI: 10.1542/peds.2023-064724
PMCID: PMC13046223
PMID: 39054943
Abstract
Protoporphyria is a subtype of porphyria characterized primarily by painful phototoxic skin reactions after light exposure at specific wavelengths. Historically, phototherapy is not contraindicated in patients with protoporphyria since there have not been any reports of phototoxic reactions. However, patients with protoporphyria are advised to avoid direct sunlight. In this case report, we describe a neonate not known to have X-linked protoporphyria who received phototherapy for 1 to 2 hours. Within hours after initiation of phototherapy, this neonate developed a life-threatening reaction consisting of rash over the distribution of phototherapy, acute liver failure with coagulopathy, diffuse hypotonia with diaphragmatic failure, and subsequent acute respiratory failure that required mechanical ventilation. As in this case, patients with protoporphyria-related acute liver failure can have signs and symptoms similar to that of an acute hepatic porphyria attack. Neither neonatal reactions to phototherapy nor liver failure temporally associated with phototherapy have been reported in patients with X-linked protoporphyria. Early recognition of this entity is crucial in light of potential life-threatening complications. Therefore, providers must react quickly when neonates have abnormal reactions to phototherapy and consider protoporphyria in the differential diagnosis.Protoporphyria is a subtype of porphyria characterized primarily by painful phototoxic skin reactions after light exposure at specific wavelengths. Historically, phototherapy is not contraindicated in patients with protoporphyria since there have not been any reports of phototoxic reactions. However, patients with protoporphyria are advised to avoid direct sunlight. In this case report, we describe a neonate not known to have X-linked protoporphyria who received phototherapy for 1 to 2 hours. Within hours after initiation of phototherapy, this neonate developed a life-threatening reaction consisting of rash over the distribution of phototherapy, acute liver failure with coagulopathy, diffuse hypotonia with diaphragmatic failure, and subsequent acute respiratory failure that required mechanical ventilation. As in this case, patients with protoporphyria-related acute liver failure can have signs and symptoms similar to that of an acute hepatic porphyria attack. Neither neonatal reactions to phototherapy nor liver failure temporally associated with phototherapy have been reported in patients with X-linked protoporphyria. Early recognition of this entity is crucial in light of potential life-threatening complications. Therefore, providers must react quickly when neonates have abnormal reactions to phototherapy and consider protoporphyria in the differential diagnosis.
Details
- Title: Subtitle
- A Severe Reaction After Phototherapy in a Neonate With X-Linked Protoporphyria
- Creators
- Jeremy A Sandgren - University of IowaMoon Ley Tung - University of IowaKristen G Berrebi - University of IowaDimah N Saade - University of IowaJennifer R Bermick - University of Iowa, NeonatologyStephanie S Lee - University of Iowa, NeonatologyAmy H Stanford - University of Iowa, Stead Family Department of Pediatrics
- Resource Type
- Journal article
- Publication Details
- Pediatrics (Evanston), Vol.154(2), e2023064724
- DOI
- 10.1542/peds.2023-064724
- PMID
- 39054943
- PMCID
- PMC13046223
- NLM abbreviation
- Pediatrics
- ISSN
- 1098-4275
- eISSN
- 1098-4275
- Publisher
- AMER ACAD PEDIATRICS; Itasca
- Language
- English
- Electronic publication date
- 07/26/2024
- Date published
- 08/01/2024
- Academic Unit
- Dermatology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Medical Genetics and Genomics; Neurology (Pediatrics); Neonatology
- Record Identifier
- 9984687781302771
Metrics
32 Record Views