which reliable administration of phototherapy cannot This study represents a multicenter survey on the be guaranteed. (HEPATOLOGY 1996;24:311-315.) management of patients with Crigler-Najjar syndrome (CNS) type 1. The aim of the survey was to find guiding principles for physicians in the care of these patients. Fifty-seven patients were included. At the time of incluCrigler-Najjar syndrome (CNS) is characterized by unconsion, 21 patients had received a liver transplant (37%). jugated hyperbilirubinemia since birth.1 CNS type 1 results The average age at transplantation was 9.1 { 6.9 years from a complete deficiency of the enzyme bilirubin UDP-(range, 1-23 years); the age of the patients who had not glucuronosyltransferase (UGT), and CNS type 2 results from been transplanted at the time of inclusion was 6.9 { 6.0 a partial deficiency. Bilirubin UGT is a member of the UGT years (range, 0-23 years). Brain damage had developed family, a group of enzymes encoded by the UGT1 and UGT2 in 15 patients (26%). Five patients died, and 10 are alive genes on human chromosomes 2 and 4. UGTs are located in with some degree of mental or physical handicap. In 2 the endoplasmic reticulum of the hepatocyte. UGT isoforms patients, ages 22 and 23 years, early signs of bilirubin are present in various other organs such as intestine, kidney, encephalopathy could be reversed, in 1 by prompt medi-lungs, and adrenals. 3-5 The after retransplantation, 2 improved neurologically, and first report was in 1952 by Crigler and Najjar.1 Before the 4 remained neurologically impaired. The age of 8 pa-advent of phototherapy, the disease was lethal, with death tients with and 13 without brain damage at or before occurring by the age of 15 months. transplantation was 14.3 { 5.9 and 5.9 { 5.4 years (P In CNS type 1, serum bilirubin values of untreated patients õ .01), respectively. Therapy of CNS type 1 consists of are in excess of 350 mmol/L, and bile contains not more than phototherapy (12 h/d), followed by liver transplantation. traces of bilirubin conjugates. 6,7 Kernicterus is a complication Phototherapy, although initially very effective, is so-that can also develop later in life.1,8,9 CNS type 2 is less secially inconvenient and becomes less efficient in the vere, with serum bilirubin values usually not exceeding 350 older age group, thus also decreasing compliance. Cur-mmol/L. The bile of these patients contains bilirubin monorently, liver transplantation is the only effective ther-and diglucuronides in low concentration.6,7 Their serum biliapy. This survey shows that, in a significant number of rubin responds to phenobarbital treatment with a decrease patients, liver transplantation is performed after some by 30% or more.10 Genetically, both diseases result from muform of brain damage has already occurred. From this, tations in the exons of the UGT1 gene.2 CNS types 1 and one must conclude that liver transplantation should be 2 are autosomal recessive diseases because both alleles are performed at a young age, particularly in situations in aff...

Our diagnostic procedure was useful to reduce the rate of surgery. Surgical treatment of chronic osteomyelitis is not always neccessary especially in cases of missing necrosis, joint infection and abscess as demonstrated by the complete recovery of our patients treated solely with antibiotics.

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To improve standardization of a folate bioassay, folate-depleted rats were repleted with a folate-free amino acid-based diet supplemented with 29 levels of folic acid. Growth was the main response variable and body tissue folate concentrations were also assessed. Because a positive correlation was observed between low levels of dietary folic acid and growth and little or no correlation was observed between high levels and growth, six regression models with a steep slope for low levels and a shallow or zero slope for high levels of dietary folic acid were evaluated. The model referred to as the "two-phase regression" or "change-point" model best described the relationship. Depleted rats needed 674 +/- 71 nmol folic acid/kg diet to reach their full growth potential. This value is biologically sensible, and this regression model is well established in the statistical literature. The change-point model is highly recommended to characterize the growth response, because growth is a functional response and, in the range of 226 to 680 nmol folic acid/kg, this response is linear, which is an additional advantage. Linear responses are easier to interpret because of complicated issues of interpretation and confidence intervals with nonlinearities. Linear regressions described serum and liver folate responses, whereas exponentials described whole blood and carcass folate responses. Depleted rats needed 5920 and 5780 nmol folic acid/kg diet to maximize their whole blood and carcass folates, respectively.

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