New Phototherapy Device to Treat Patients With Crigler-Najjar Disease
New Phototherapy Device to Treat Patients With Crigler-Najjar Disease
Evaluating the efficacity of a new device phototherapy by comparing it with conventional
 phototherapy. Jaundice occurs in many newborns, and is, in most cases benign, However, owing
 to the potential neurotoxicity of unconjugated bilirubin, newborns must be monitored to
 identify those who might develop severe hyperbilirubinemia an, in rare cases, acute bilirubin
 encephalopathy or kernicterus. Treatment of jaundice in newborn relies on phototherapy,
 exposing their skin to light of a specific wavelength . Fluorescent tubes or halogen lamps
 have been used as light sources for phototherapy for many years. Light-emitting diodes (LEDs)
 are more recent sources which are power efficient, have a longer life and are portable with
 low heat production. Several technologies and devices are developed using LEDs and specially
 a compact system.
Crigler-Najjar (CN) syndrome is a congenital inborn error of hepatic bilirubin metabolism
 caused by the deficiency of bilirubin uridinediphosphate glucuronosyltransferase activity CN
 patients are at permanent risk of life-threatening bilirubin encephalopathy (kernicterus).
 Treatment of CN disease relies on daily prolonged phototherapy (10-12 h/day). To date, liver
 transplantation is the only curative treatment available. To date, no phototherapy device
 exists for teenagers and adults. CN patients use homemade systems or add several neonatal
 systems together to reach a better efficacy.

 A new device has been designed by weaving optical fibers into a sheet connected to LEDs. This
 new technology, marketed by several manufacturers of phototherapy devices, is used in
 pediatric wards for treating jaundiced newborns. A prototype of 80× 100cm was thus developed,
 which combines numerous advantages : important surface of treatment, easy use and
 transportation, user-friendliness.

 Before going to a therapeutic trial for assessing the benefits of this new device in
 ambulatory current practice, it is essential to make sure of both its feasibility and
 efficiency on the control of bilirubinemia, under medical supervision.

 Hypothesis: the new phototherapy sheet is as effective as, and better tolerated than the
 usual devices used in CN patients.
Inclusion Criteria:

 -  genetic diagnosis of Crigler Najjar disease

 -  child whose height is between 60cm and 100cm

 -  patients with follow-up in reference center,

 Exclusion Criteria:

 -  opposition of parents

 -  no social security insurance