Clinical Remission of Delta-Aminolevulinic Acid Dehydratase Deficiency Through Suppression of Erythroid Heme Synthesis

Hepatology. 2019 Jul;70(1):434-436. doi: 10.1002/hep.30543. Epub 2019 Jun 6.

Authors

Rochus A Neeleman¹, Eduard J van Beers², Edith C Friesema¹, Rita Koole-Lesuis¹, Willem L van der Pol³, J H Paul Wilson¹, Janneke G Langendonk¹

Affiliations

¹ Porphyria Center Rotterdam, Center for Lysosomal and Metabolic Disease, Department of Internal Medicine, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands.
² Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
³ Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, the Netherlands.

No abstract available

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Erythroid Cells / drug effects*
Erythroid Cells / metabolism
Heme / biosynthesis
Humans
Hydroxyurea / pharmacology
Hydroxyurea / therapeutic use*
Infant, Newborn
Male
Nucleic Acid Synthesis Inhibitors / pharmacology
Nucleic Acid Synthesis Inhibitors / therapeutic use*
Porphobilinogen Synthase / deficiency*
Porphyrias, Hepatic / drug therapy*

Substances

Nucleic Acid Synthesis Inhibitors
Heme
Porphobilinogen Synthase
Hydroxyurea

Supplementary concepts

Porphyria, Acute Hepatic

Grants and funding

SLO Foundation for Liver and Gastrointestinal Research/International