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Liver transplantation (LT) predisposes to metabolic derangements and increases the risk for cardiovascular disease. We conducted a national cross-sectional study of all pediatric recipients who underwent LT between 1987 and 2007. We measured serum levels of noncholesterol sterols (surrogate markers of cholesterol synthesis and intestinal absorption) and fibroblast growth factor 21 (FGF21) in 49 patients (74% of survivors) at a median of 10 years posttransplant and in 93 controls matched for age and gender. Although serum cholesterol levels were similar in patients and controls, patients displayed increased whole-body synthesis and decreased intestinal absorption of cholesterol compared with controls (lathosterol to cholesterol ratio 129 ± 55 vs. 96 ± 41, respectively, p < 0.001; campesterol to cholesterol ratio 233 ± 91 vs. 316 ± 107, respectively; p < 0.001). Azathioprine (r =–0.383, p = 0.007) and low-dose methylpredisolone (r =–0.492, p < 0.001) were negatively associated with lathosterol/sitosterol ratio reflecting a favorable effect on cholesterol metabolism. FGF21 levels were higher in patients than in controls (248 pg/mL vs. 77 pg/mL, p < 0.001). In healthy controls, FGF21 was associated with cholesterol metabolism, an association missing in LT recipients. Normal serum lipids are achievable in long-term survivors of pediatric LT, but changes in cholesterol metabolism and increased FGF21 levels may explicate later cardiovascular risk.

In children, optimal timing of liver transplantation (LT) is crucial, but reliable prognostic tools for chronic liver diseases are scarce. We assessed the predictive value of galactose half-life (Gal½) for LT or death. A retrospective search of hospital database 2003-2010 revealed 92 consecutive children with chronic liver disease (36 biliary atresia) whose liver function was assessed with Gal½ measurement. Gal½, follow-up data, and liver biochemistry were recorded and pediatric/model for end-stage liver disease (P/MELD) scores calculated. Patients listed for LT or those who died within 1 year of the Gal½ measurement (Group 1) were compared to those surviving without listing (Group 2). Predictive value of Gal½ and P/MELD for listing for LT was assessed with area under the receiver operating characteristic curve (AUROC) analysis. Group 1 had markedly increased median Gal½ [17.0 (interquartile range 12.5-28.5) min] and higher P/MELD [13 (-1-23)] compared with group 2, [10.5 (9.5-12.5) min and -1 (-8-8); P < 0.001 for both]. Both Gal½ and P/MELD (P < 0.001) predicted listing or death with respective AUROCs of 0.808 (95% CI 0.704-0.913) and 0.780 (0.676-0.890), and 85% sensitivity and 69% specificity for Gal½≥12.0 min. Gal½ is a useful tool when evaluating 1-year prognosis in children with chronic liver disease.

Unresectable malignant liver tumors may be treated by LTx. We evaluated the results of LTx for HB and HCC. All patients transplanted for HB or HCC between 1990 and 2007 were included. Effects of histologic tumor type, primary tumor resection, disease staging, and serum AFP levels at diagnosis and at transplantation on disease recurrence and survival were evaluated. Twelve patients with median age of five (range, 2-16) were transplanted and followed for a median of 11 (2-18) yr. Six patients had HB and six had HCC. At diagnosis, eight patients were staged as PRETEXT III and four patients as PRETEXT IV. Two patients had pulmonary metastases. All patients received neoadjuvant chemotherapy. Median time from diagnosis to LTx was seven (2-133) months. At LTx, none of the patients had radiological evidence of extrahepatic disease, and the median AFP level was 85 (6-15 180) microg/L. No routine chemotherapy after LTx was used.The overall one-, five-, and 10-yr cumulative survival rates were 100%, 80%, and 67%, respectively. Survival was comparable between the two tumor types (4/6 for both). Two deaths occurred secondary to tumor recurrence, one of each tumor type. Both of these patients had an AFP response of

Background Despite improved survival rates of patients with pediatric intestinal failure (IF), data on health-related quality of life (HRQoL) of IF patients are still scarce. We hypothesized that I) continued parenteral nutrition, underlying intestinal motility disorder, abdominal pain and problematic bowel function would be associated with poorer HRQoL and higher parental stress levels, and II) the time intervals since the latest bowel operation, the latest episode of sepsis, and the latest inpatient care episode would be associated with better HRQoL and lower parental stress. Methods Patients with pediatric onset IF and their parents answered questionnaires on HRQoL, parental stress, and bowel-related symptoms. Clinical data were gathered by chart review. Controls matched for age and sex were randomly chosen by the Population Register Centre of Finland. Results Thirty-six (73%) IF patients participated at a median age of 9years. Overall HRQoL was similar to healthy peers, and frequent abdominal pain was the only factor associated with poorer HRQoL. Abdominal pain and stool frequency >3 times per day were associated with higher levels of parental stress, whereas longer time intervals since the latest bowel operation or hospitalization were associated with lower parental stress levels. Conclusion Long-term HRQoL of pediatric IF patients is comparable to that of healthy peers. Time often eases parental stress, but frequent abdominal pain presents a challenge to the well-being of some patients and requires medical attention.

Hepatic artery thrombosis (HAT) after liver transplantation (LT) increases patient morbidity and mortality. Early HAT is considered to occur within the first month after LT, whereas late HAT occurs after the first month. Few studies have addressed late HAT after LT, especially in pediatric patients. Between 1987 and 2007, 99 patients (age 18 years) underwent deceased donor LT. Thirty-four of 66 eligible patients (52%) underwent magnetic resonance imaging (MRI) according to protocol. On the basis of MRI findings, the patients were grouped as those who experienced late HAT and those who did not. Additionally, potential risk factors for late HAT were analyzed retrospectively. P values were adjusted for multiplicity. The median age at LT was 1.7 years [interquartile range (IQR) = 1.0-9.6 years], and the median follow-up time at MRI was 9.5 years (IQR = 4.0-16.4 years). Late HAT was diagnosed in 15 of the 34 patients [44%, 95% confidence interval (CI) = 29%-61%] undergoing MRI and in 3 of these patients with angiography preceding MRI. Ultrasonography revealed late HAT in 6 of these 15 patients with a sensitivity of 40% (95% CI = 20%-64%). The donor/recipient weight ratio remained significantly higher for the patients with late HAT versus the patients without late HAT after P values were adjusted (5.4 versus 1.9, P = 0.03). No marked differences were observed in laboratory or liver histology parameters between the groups. In conclusion, late HAT is common after pediatric LT. The donor/recipient weight ratio was higher for patients with late HAT, and this was attributable to the lower weight of the recipients. No salient features of late HAT were observed with respect to laboratory or histological parameters, at least in terms of our study's cross-sectional period.

Controversy remains about the role of protocol liver biopsy for symptom-free recipients and about the long-term use of low-dose steroids after pediatric liver transplantation (LT). We conducted a national cross-sectional study of pediatric recipients who underwent LT between 1987 and 2007. Liver biopsy samples were taken from 54 patients (82% of survivors) after a median posttransplant follow-up of 11 years, and they were reviewed by 2 pathologists blinded to the clinical data. Biopsy samples from 18 patients (33%) showed nearly normal histology with no inflammation, fibrosis, or steatosis. Portal inflammation was detected in 14 samples (26%), showed no correlation with anti-nuclear antibodies, and was less frequent in the 35 patients whose immunosuppression included steroids (14% versus 47% of patients not using steroids, P = 0.008). Fibrosis was present in 21 biopsy samples (39%). According to the Metavir classification, 16 were stage 1, 3 were stage 2, and 2 were stage 3. The fibrosis stage correlated negatively with serum prealbumin levels (r = -0.364, P = 0.007) and positively with chronic cholestasis (cytokeratin 7 staining; r = 0.529, P0.001) and portal inflammation (r = 0.350, P = 0.01). Microvesicular steatosis was found in 23 biopsy samples (43% of patients in 5%-80% of hepatocytes), and it correlated with the body mass index (r = 0.458, P0.001) but not with steroid use. The age of the allograft (donor age plus follow-up time) correlated with higher serum gamma-glutamyltransferase (r = 0.472, P0.001) and conjugated bilirubin levels (r = 0.420, P = 0.002) as well as chronic cholestasis (r = 0.299, P = 0.03). The biopsy findings led to treatment changes in 10 patients (19%), whereas only 1 complication (subcapsular hematoma) was encountered. In conclusion, continuing low-dose steroids indefinitely after pediatric LT may have a positive effect on the long-term histological state of the liver graft. Allograft aging may lead to chronic cholestasis and thus contribute to the development of liver fibrosis.

Effects of caseload and organization of care on outcomes of biliary atresia (BA) remain unclear. We compared outcomes before and after national centralization of BA treatment in Finland with a population of 5.4 million people and 60,000 live births/year.All children born in Finland from 1987 to 2010 with BA were included. Complete patient identification was ascertained from the national Register of Congenital Malformations. Hospital records were reviewed for confirmation of the diagnosis, treatment, and follow-up data. Clearance of jaundice (serum bilirubin ≤ 20 μmol/l) and survival modalities were compared before and after centralization from five centers to Helsinki.The incidence of BA was 1 in 20,100 live births. A total of 72 BA patients of whom 64 had undergone surgery for BA were identified. After centralization, the median caseload per center increased from 0 (range, 0-3) to 4 (2-5) patients/year (p0.001), clearance of jaundice rate increased from 27% to 75% (p = 0.001), 2-year jaundice-free native liver survival from 25% to 75% (p = 0.002), transplant-free survival from 27% to 75% (p = 0.005), and overall survival from 64% to 92% (p = 0.082). Baseline patient characteristics including type of BA and age at portoenterostomy remained unaltered. In a logistic regression analysis including treatment era, operating center, BA splenic malformation syndrome, and age at portoenterostomy as variables, only treatment in Helsinki after centralization predicted clearance of jaundice (odds ratio 4.2; 95% confidence interval 1.05-16.5; p = 0.043).In small countries, BA treatment should be centralized to appointed multidisciplinary teams allowing high quality results with a median of four cases/year.

The long-term impact of pediatric liver transplantation (LT) and its complications on general health, health-related quality of life (HRQoL) and sexual health were assessed. We conducted a national cross-sectional study of all pediatric recipients who underwent LT between 1987 and 2007. Of 66 survivors, 57 participants (86%) were compared to randomly chosen healthy controls (n = 141) at 10.7 ± 6.6 years posttransplant. PedsQL4.0, SF-36, DISF-SR and AUDIT questionnaires for appropriate age groups were used. Patients and controls

Vaginal laceration is rare following a water sport accident. Several cases have been reported in water-skiers and personal watercraft passengers. If a patient presents with vaginal bleeding after such an accident, careful examination under general anesthesia is recommended to detect deep trauma. We present a case of vaginal laceration from a water slide in a middle-aged healthy woman.

Unresectable malignant liver tumors may be treated by LTx. We evaluated the results of LTx for HB and HCC. All patients transplanted for HB or HCC between 1990 and 2007 were included. Effects of histologic tumor type, primary tumor resection, disease staging, and serum AFP levels at diagnosis and at transplantation on disease recurrence and survival were evaluated. Twelve patients with median age of five (range, 2-16) were transplanted and followed for a median of 11 (2-18) yr. Six patients had HB and six had HCC. At diagnosis, eight patients were staged as PRETEXT III and four patients as PRETEXT IV. Two patients had pulmonary metastases. All patients received neoadjuvant chemotherapy. Median time from diagnosis to LTx was seven (2-133) months. At LTx, none of the patients had radiological evidence of extrahepatic disease, and the median AFP level was 85 (6-15 180) microg/L. No routine chemotherapy after LTx was used.The overall one-, five-, and 10-yr cumulative survival rates were 100%, 80%, and 67%, respectively. Survival was comparable between the two tumor types (4/6 for both). Two deaths occurred secondary to tumor recurrence, one of each tumor type. Both of these patients had an AFP response of99%. Six of eight patients with primary LTx survived, when compared to two of four transplanted after primary resection. PRETEXT tumor staging had no effect on survival. LTx even without post-transplantation chemotherapy is an effective treatment option for unresectable HB and HCC with comparable survival. Incomplete AFP response to chemotherapy and primary tumor resection were associated with decreased survival.

Background and Aims: Mopeds and scooters have become increasingly popular among Finnish teenagers. The aim of this retrospective study was to assess incidence of and injury patterns associated with moped and scooter accidents in adolescents. Materials and Methods: All 222 patients treated for moped and scooter-related injuries at Helsinki Children's Hospital and Töölö Trauma Centre from January 2002 to December 2007 were included. Information was drawn from patient records and compared with nation-wide Finnish data gathered from public data-bases. Results: The annual number of patients at our centres increased from 14 to 76 and the proportion of girls increased from 7% to 25%. A similar trend was found on a national level. In our material, collisions between mopeds and other motorized vehicles accounted for 52% of accidents, and 33% of patients were injured from falling. Seventy-five percent of patients were hospitalized, and 50% needed at least one procedure requiring general anaesthesia. Five percent of the patients were under the influence of alcohol. Trauma of the head occurred in 22%; helmets did not protect against severe trauma. On a national level the proportion of 15- to 17-year-old road traffic victims has doubled in five years. Among this age group, more than half of all road-traffic accidents involve mopeds and scooters. Conclusions: Over a time span of six years, moped accidents among adolescents have become very common. Our results suggest that measures should be taken to diminish the number of moped and scooter accidents and to improve driver safety.

Biliary atresia is the most common indication for childhood liver transplantation. The effects of successful portoenterostomy (PE) on native liver histology remain unclear.We assessed changes in native liver histology after a successful PE in relation to liver function and clinical outcomes.In total, 70 native liver biopsies of 44 biliary atresia patients were obtained at PE (n=30), 4.2 years after successful PE (n=23) and 1.1 years after failed PE (n=17), and reviewed for cholestasis, fibrosis, inflammation, and cytokeratin 7 (CK7) immunopositivity (chronic cholestasis). Ten transplant donor livers served as controls.After a successful PE [serum bilirubin 11 (2 to 35) μmol/L at biopsy], histologic native liver cholestasis completely resolved in 83% of the patients and portal inflammation significantly decreased. Nevertheless, enhanced fibrosis [Metavir stage 2 (1-4) vs. 4 (1-4)], bile duct proliferation [grade 2 (1-2) vs. 1 (0-2)], and periportal CK7 immunostaining [grade 1 (0-2) vs. 1 (0-4)] persisted in 100%, 87%, and 61% of subjects, respectively. Metavir fibrosis stage corresponded cirrhosis (stage 4) in 52% of the patients, associated with the presence of portal hypertension, and correlated with serum-conjugated bilirubin (r=0.601, P=0.002), bile duct proliferation (r=0.657, P=0.001), and CK7 positivity (r=0.657, P=0.001). Aspartate transferase to platelet ratio index predicted native liver fibrosis and development of esophageal varices. The degree of fibrosis and portal inflammation at PE were unrelated to native liver survival.Despite resolution of cholestasis and decreasing inflammation, bile duct proliferation, periportal CK7 immunostaining, and fibrosis persist after successful PE. Fibrosis is associated with biochemical cholestasis, bile duct proliferation, CK7 immunopositivity (chronic cholestasis), and development of portal hypertension.