To evaluate changes in the clinical presentation of celiac disease in southeastern Wisconsin.

Failure to thrive, at the time of presentation, is indicative of a final diagnosis of CD or IC, not UC. Linear growth failure is a common finding in patients with early onset CD. A high proportion of patients with CD have failure to thrive, colonic, and perianal disease. The IBD serology panel is of limited clinical relevance in providing definitive diagnostic information in this pediatric population.

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Improved neonatal medical care and renal replacement technology has improved the long term survival of patients with autosomal recessive polycystic kidney disease (ARPKD). Ten year survival of those surviving the first year of life is reported to be 82% and is continuing to improve further. However, despite increases in overall survival and improved treatment of systemic hypertension and other complications of their renal disease, nearly 50% of survivors will develop end stage renal disease (ESRD) within the first decade of life 1, 2. In addition to renal pathology, patients with ARPKD develop ductal plate malformations with cystic dilation of intra- and extra-hepatic bile ducts resulting in congenital hepatic fibrosis (CHF) and Caroli syndrome. Many patients with CHF will develop portal hypertension with resulting esophageal varices, splenomegaly, hypersplenism, protein loosing enteropathy and gastrointestinal bleeding3,4,5. Management of portal hypertension may require endoscopic band ligation of esophageal varices or porto-systemic shunting. Complications of hepatic involvement can include ascending cholangitis, cholestasis with malabsorption of fat soluble vitamins, and rarely benign or malignant liver tumors. Patients with ARPKD who eventually reach end-stage renal disease, and ultimately require kidney transplantation, present a unique set of complications related to their underlying hepato-biliary disease. In this review, we focus on new approaches to these challenging patients, including the indications for liver transplantation in ARPKD patients with severe chronic kidney disease awaiting kidney transplant. While survival in patients with ARPKD and isolated kidney transplant is comparable to that of age-matched pediatric patients who have received kidney transplants due to other primary renal diseases, 64–80% of the mortality occurring in ARPKD kidney transplant patients is attributed to cholangitis/sepsis which is related to their hepato-biliary disease6,7 Recent data demonstrate that surgical mortality among pediatric liver transplant recipients is decreased to less than 10% at 1 year8,9. The immunosuppressive regimen used for kidney transplant recipients is adequate for most liver transplant recipients. We therefore suggest that in a select group of ARPKD patients with recurrent cholangitis or complications of portal hypertension, combined liver-kidney transplant is a viable option. Although further study is necessary to confirm our approach, we believe that combined liver-kidney transplantation can potentially decrease overall mortality and morbidity in carefully selected ARPKD patients with ESRD and clinically significant CHF.