The epidemiology and natural history of pediatric primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC), and autoimmune hepatitis (AIH) are not well characterized. Using multiple, overlapping search strategies followed by a detailed records review, we identified all cases of pediatric PSC, ASC, AIH, and inflammatory bowel disease (IBD) in a geographically isolated region of the United States. We identified 607 cases of IBD, 29 cases of PSC, 12 cases of ASC, and 44 cases of AIH. The mean age at diagnosis was 13.0 years for PSC, 11.3 years for ASC, and 9.8 years for AIH. The incidence and prevalence of PSC, ASC, and AIH were 0.2 and 1.5 cases, 0.1 and 0.6 cases, and 0.4 and 3.0 cases per 100,000 children, respectively. The mean duration of follow-up was 5.9 years. The probability of developing complicated liver disease within 5 years of the diagnosis of liver disease was 37% [95% confidence interval (CI) 5 21%-58%] for PSC, 25% (95% CI 5 7%-70%) for ASC, and 15% (95% CI 5 7%-33%) for AIH. The 5-year survival rates with the native liver were 78% (95% CI 5 54%-91%) for PSC, 90% (95% CI 5 47%-99%) for ASC, and 87% (95% CI 5 71%-95%) for AIH. Cholangiocarcinoma developed in 2 of the 29 PSC patients (6.9%). PSC occurred in 9.9% of patients with ulcerative colitis (UC) and in 0.6% of patients with Crohn's disease (CD). ASC occurred in 2.3% of UC patients and 0.9% of CD patients. AIH occurred in 0.4% of UC patients and in 0.3% of CD patients. Liver disease occurred in 39 of 607 IBD patients (6.4%) overall. Conclusion: Immune-mediated liver diseases are important sources of morbidity in children. Using a populationbased design, this study quantifies the burden and natural history of immune-mediated liver disease in children. (HEPATOLOGY 2013;58:1392-1400

SummarySerum a-fetoprotein (AFP) levels were monitored in 32 normal babies consecutively from 2 to 3 days, 2 wk, and 2 and 4 months after birth. In addition, serum AFP concentration was also measured in 116 random specimens from infants with normal liver enzymes and 10 infants born immaturely. Results were combined to establish normal AFP levels for infants at various ages. Serum AFP disappeared rapidly after birth. We found that it was not until 8 months of age that the normal AFP level in infants approached adult level. The half-lives of AFP degradation were estimated to be 5.5 days between birth and 2 wk, 11 days between 2 wk to 2 months, and 33 days between 2 and 4 months of age. In contrast to earlier belief, we felt that some AFP synthesis still exists after birth; however, the rate of synthesis may also decrease with age. SpeculationOur results favor the view that some a-fetoprotein (AFP) synthesis exists after birth. The rate of synthesis is slowly decreased with time. Any factor affecting the rate of synthesis may influence the serum AFP level. The wide range of variation in serum AFP levels in infants of various ages indicates that there must be more than one unknown factor affecting the AFP serum level. The normal range will be narrower if these factors become known. By using our age-dependent normal AFP serum levels, it would be interesting to see whether differential diagnosis of neonatal h e p atitis from biiary atresia can be improved and whether infants do have higher and more frequent elevation of serum AFP with hepatic disorders.In adults, the measurement of serum a-fetoprotein (AFP) level is useful in the diagnosis and treatment of hepatoma, teratoblastoma, and a number of liver diseases (3. 10). However, because of the lack of reliable normal values for infants, the diagnosis of similar abnormalities in these patients is more difficult. At birth, the average AFP level of cord serum is several thousandfold higher than are adult levels. These levels progressively decline as the infant matures (7, 11, 12) However, it is not precisely known at what age the serum AFP of infants reaches adult level. In addition, the rate at which the level declines with age, as well as the various normal levels during that period, are also unknown.Several investigators have attempted to distinguish infants with neonatal hepatitis from those with biliary atresia by measuring their serum AFP level (2, 7. 12). In this regard, a single normal AFP level was used, although the infants varied by several months of age. It is possible that this differential diagnosis may be significantly improved if more reliable age-discriminated normal AFP levels were available. In our study, we monitored the serum AFP levels of 32 normal babies consecutively from birth to 4 months of age. In addition. AFP levels were determined in 116 random serum specimens from supposedly normal children with normal serum liver enzyme activities to establish acceptable upper normal limits for these older infants and to determine at what age they fall to...

No abstract

No abstract