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Unlabelled: We report on a 3.8-year-old German girl who presented with signs of subacute liver failure based on a 4-month history. Investigations done before admission excluded infectious, metabolic and immunological diseases. Laboratory findings were increased values of aminotransferases, hyperbilirubinaemia, hyperammonaemia and deteriorated plasmatic coagulation. Caeruloplasmin and serum copper concentrations were normal; however, urinary copper excretion was elevated (80 microg/l). Liver biopsy showed a micronodular liver cirrhosis and an extremely high liver copper concentration (1400 microg/g dry weight). Epidemiological investigations revealed an elevated copper concentration (8645 microg/l, normal: <2000) and a low pH value (6.3) of the drinking water supplied by copper pipes. The girl had been exposed to copper-contaminated drinking water since the age of 2 years.Conclusion: Laboratory, histopathological findings and a proven chronic copper intoxication lead to the diagnosis of Indian childhood cirrhosis in a German girl. Whereas this disease is mostly described in patients with increased copper intake in infancy, our patient developed toxic liver cirrhosis with relatively late copper exposure. Indian childhood cirrhosis should be considered in the differential diagnosis of early childhood liver cirrhosis. [ABSTRACT FROM AUTHOR]

Background: Inflammatory bowel disease (IBD) can arise at any age, with peak incidence in adolescence and young adulthood. A registry of pediatric cases of IBD offers the opportunity to document their diagnosis and treatment, with the ultimate aim of improving diagnosis and treatment in the future., Methods: In the German-language CEDATA-GPGE registry, 3991 cases of IBD in patients less than 18 years of age were documented from 2004 to 2014. The 1257 patients who were prospectively included in the registry upon diagnosis and whose further course was documented for at least three months were analyzed in two separate groups--under 10 years old, and 10 years and above--with respect to the type and duration of their symptoms until diagnosis, the completeness of the diagnostic evaluation, the disease phenotype, and the initial treatment., Results: Of the 958 patients for whom full documentation was available, 616 (64.3%) had Crohn's disease (CD), 278 (29%) had ulcerative colitis (UC), 64 (6.7%) had an unclassified IBD, and 23.2% were under 10 years old. The latency to diagnosis was longer for CD than for UC (0.5 versus 0.3 years), regardless of age. 62.5% of the CD patients had ileocolonic involvement, and more than half had involvement of the upper gastrointestinal tract. 71% of the patients with UC had subtotal colitis or pancolitis. Continuous improvement was seen in diagnostic assessment according to published guidelines. For example, in 2004/2005, 69% of patients were evaluated endoscopically with ileocolonoscopy and esophagogastroduodenoscopy; this fraction had risen to nearly 100% by 2013/2014. Similarly, the percentage of patients who underwent a diagnostic evaluation of the small intestine, as recommended, rose from 41.2% to 60.9% over the same period. The most common initial treatments were 5- amino - salicylates (86.8% CD, 100% UC) and glucocorticoids (60.6% CD, 65.6% UC). 32% of the patients with CD received exclusive enteral nutrition therapy., Conclusion: Most of these pediatric patients with IBD, whether in the younger or the older age group, had extensive bowel involvement at the time of diagnosis. The registry data imply that improvement in clinical course may be achieved by shortening the time to diagnosis and by closer adherence to the diagnostic and therapeutic guidelines.

Objectives: To examine predictors of delayed diagnosis of inflammatory bowel disease in children and adolescents., Study Design: A total of 2,436 patients (age 0-18 years) with Crohn's disease, ulcerative colitis, or unclassified colitis were included from 53 pediatric gastroenterologists. Predictors were examined with the proportional hazards model, presented as hazard ratios (HR) with 95% confidence intervals. HR < 1.0 represent factors associated with late diagnosis., Results: Median time to diagnosis was 4 (2-8) months. Crohn's disease (HR 0.62; 0.56-0.68), and within Crohn's disease, ileal disease (HR 0.77, 95% confidence interval 0.67 to 0.89) were associated with delayed diagnosis. Chances for early diagnosis increased with increasing age (HR 1.07 per year of age; 1.06 to 1.08). There was also an effect by center (HR 0.63, 0.52 to 0.67), but not by sex or country (Austria vs Germany). Growth failure was more common in those cases with delayed diagnosis., Conclusions: There is still concern about delays in the diagnosis of inflammatory bowel disease in the very young and in children with small bowel disease. Inequalities of care by region require further investigation., (Copyright © 2011. Published by Mosby, Inc.)

Background: Nutrition of children with end-stage renal disease and peritoneal dialysis (PD) is often difficult. Tube feeding via a gastrostoma is discussed controversially, and some authors consider this as a contraindication because of the risk of peritonitis., Methods: In our centre 16 infants and children with end-stage renal disease were treated with PD and tube feeding over a gastrostoma in the last 12 years. The patients showed dystrophy (mean BMI -1.73 SDS) and were too small (mean body length -4.56 SDS). Seven of them (median age 11 months) received a gastrostoma before insertion of a Tenkhoff-catheter and start of PD. Nine children (median age 5 months) had PD primarily before insertion of the gastrostoma and start of tube feeding., Results: Patients with start of PD while a gastrostoma was already inserted had 15 events with peritonitis in the observation time of 91 months (1.98 per patient year). Patients with primary start of PD had 12 events with peritonitis in a total time of 43 month (3.34 per patient year), after insertion while PD was already running the number of events fell significantly to 25 peritonitis events in a total of 271 months (1.11 per patient year, p < 0.01). The children had a benefit from tube feeding via a gastrostoma in regard of body weight (BMI + 1.61 SDS, p < 0.01) as well as growth (body height + 2.29 SDS, p < 0.05)., Conclusion: Tube feeding via a gastrostoma is a good and safe option for alimentation, even under peritoneal dialysis. A decrease of PD-associated peritonitis under tube feeding was observed while physical development was positively influenced., (Georg Thieme Verlag KG Stuttgart. New York.)