Publications by authors named "D Wensink"
Article Synopsis
- Erythropoietic protoporphyria (EPP) is a genetic disorder that leads to painful reactions to sunlight, significantly affecting the quality of life (QoL) of both children and adults.
- This study compared QoL scores between children with EPP and matched healthy controls, revealing lower scores in physical and social aspects for the EPP group, though not statistically significant after adjustment.
- While children's overall EPP-QoL scores were similar to those of adults with EPP, they showed significantly lower scores in the disease-specific subdomain, highlighting the urgent need for treatment options and further research for affected children.
Article Synopsis
- Erythropoietic protoporphyria (EPP) significantly affects patients with severe pain from light exposure, negatively influencing their quality of life and social functioning.
- A study conducted in the Netherlands on 121 adult EPP patients found that while many had higher educational levels, their employment rates were low before treatment; however, these rates improved during afamelanotide treatment.
- Results indicated that afamelanotide not only enhanced quality of life and social functioning, but also showed potential for improving employment rates, highlighting the need for further research on the psychosocial effects of EPP.
Background: Erythropoietic protoporphyria (EPP) patients suffer from painful phototoxicity. Sunlight-avoiding behaviour has not yet been quantified objectively in EPP patients.
Objective: To study total white light exposure obtained with an actigraph device, before and during afamelanotide treatment, in EPP patients compared to healthy controls.
Background: In erythropoietic protoporphyria (EPP), which presents with severe painful phototoxicity, progressive deposition of protoporphyrins in hepatocytes and bile canaliculi may result in liver disease. Clinically EPP related liver disease ranges from mildly elevated liver enzymes to cirrhosis and acute cholestatic hepatic failure. The prevalence of liver disease in EPP, and factors predicting the risk of developing liver disease, have not been defined in a large series of unselected EPP patients.
View Article and Find Full Text PDFPurpose: Patients with erythropoietic protoporphyria (EPP), a severe painful photodermatosis, experience prodromal sensations when exposed to sunlight, which are the "warning signals" to exit the sun, as prolonged exposure causes an excruciatingly painful phototoxic attack. The unique prodromal cutaneous sensations are reversible and differ from the severe burning pain attack lasting 2-7 days. Previously, afamelanotide treatment was studied using time to pain or time outside as primary outcome measures.
View Article and Find Full Text PDF