What is the efficacy of phototherapy for the treatment of Crigler-Najjar syndrome type 1?

Updated: May 21, 2019
  • Author: Hisham Nazer, MBBCh, FRCP, DTM&H; Chief Editor: BS Anand, MD  more...
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Answer

Phototherapy causes the formation of water-soluble bilirubin isomers that can be secreted in bile without conjugation.

Patients with Crigler-Najjar syndrome type 1 generally need 10-16 hours of treatment per day. Monitor the intensity of light to keep it at a level of at least 4-10 µW/cm2/nm. The appropriate wave length is in the blue-green spectrum at 425-475 nm.

The efficacy of phototherapy is dose dependent; therefore, the response to phototherapy increases when the dose is increased. Efficacy of phototherapy can be increased by increasing the intensity of light, by increasing exposure of the body surface, and by using reflecting surfaces (eg, mirrors). Double-surface phototherapy has also been used in some cases to improve the outcome. Oral calcium phosphate may be a useful adjuvant to phototherapy in Crigler-Najjar syndrome type 1.

The effectiveness of phototherapy decreases after age 3-4 years, because the ratio of skin surface area to body mass is reduced. [71]

Problems associated with phototherapy include restriction of activity and play, poor compliance, inability of the patient to travel or take vacations, irritation from the eye shades, difficulties in temperature maintenance, tanning of the skin, embarrassment from the need to be nearly nude during phototherapy, and difficulty in procuring phototherapy lamps. Long-term phototherapy may lead to developmental delay, impaired weight gain, and possible psychological disturbances.

More recent studies indicate that conventional and intensive phototherapy are also associated with DNA damage in term infants with hyperbilirubinemia. [72]

Newer methods of delivering phototherapy, such as sit-up phototherapy units, may reduce phototherapy time by 50% while maintaining its effectiveness and, thus, may allow a child to attend school.


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