We summarize the treatment of 20 patients with Crigler-Najjar disease (CND) managed at one center from 1989 to 2005 (200 patient-years). Diagnosis was confirmed by sequencing the UGTA1A gene. Nineteen patients had a severe (type 1) phenotype. Major treatment goals were to maintain the bilirubin to albumin concentration ratio at <0.5 in neonates and <0.7 in older children and adults, to avoid drugs known to displace bilirubin from albumin, and to manage temporary exacerbations of hyperbilirubinemia caused by illness or gallstones. A variety of phototherapy systems provided high irradiance over a large body surface. Mean total bilirubin for the group was 16+/-5 mg/dl and increased with age by approximately 0.8 mg/dl per year. The molar ratio of bilirubin to albumin ranged from 0.17 to 0.75 (mean: 0.44). The overall non-surgical hospitalization rate was 0.12 hospitalizations per patient per year; one-half of these were for neonatal hyperbilirubinemia and the remainder were for infectious illnesses. Ten patients (50%) underwent elective laproscopic cholecystectomy for cholelithiasis. No patient required invasive bilirubin removal or developed bilirubin-induced neurological damage under our care. Visual acuity and color discrimination did not differ between CND patients and age-matched sibling controls. Four patients treated with orthotopic liver transplantation were effectively cured of CND, although one suffered significant transplant-related complications.Conclusions. While patients await liver transplantation for CND, hyperbilirubinemia can be managed safely and effectively to prevent kernicterus. Lessons learned from CND can be applied to screening and therapy of non-hemolytic jaundice in otherwise healthy newborns.
Concerns have been expressed for some time regarding the growth of the cosmetic suntanning industry and the potential harmful effects resulting from these exposures. Recently published work has appeared to confirm a link between sunbed use and skin cancer. A previous survey in Oxford some years ago demonstrated significant output variations, and we have attempted to extend and update that work. Ultraviolet A, UVB and blue-light output measurements were made on 50 sunbeds using a radiometer fitted with broad-band filters and detectors. A number of irradiance measurements were made on each sunbed within each waveband so that the uniformity of the output could also be assessed. UVA outputs varied by a factor of 3, with a mean of 13.5 mW/cm2; UVB outputs varied by a factor of 60, with a mean of 19.2 microW/cm2; and blue-light outputs varied by a factor of 2.5, with a mean of 2.5 mW/cm2. Outputs fall on average to 80% of the central value at either end of the sunbed. Facial units in sunbeds ranged in output between 18 and 45 mW/cm2. Output uniformity shows wide variation, with 16% of the sunbeds having an axial coefficient of variation > 10%. UVB output is highly tube-specific. Eyewear used in sunbeds should also protect against blue light.
A non-contact method for the measurement of a skin ulcer's area and volume has been developed. A commercially available laser displacement sensor is scanned across the ulcer's surface to produce a displacement image. From this image the healthy normal skin's surface is reconstructed and the area and volume found. Results of measurements made with phantom ulcers show that the method has a precision and accuracy +/- 5% of the total size. This compares favourably with results from other non-contact methods that have been published. The method of measurement is simple and reliable. At present, the time taken to produce a displacement image is lengthy but this is due to a limitation in the instrumentation, rather that a failing of the technique. The result of a preliminary measurement made on a patient's leg ulcer is presented.
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