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| 1 | Treatment of primary sclerosing cholangitis in children显示文摘Primary sclerosing cholangitis(PSC) is a rare disease of stricturing and destruction of the biliary tree with a complex genetic and environmental etiology.Most patients have co-occurring inflammatory bowel disease. Children generally present with uncomplicated disease, but undergo a variable progression to endstage liver disease. Within ten years of diagnosis, 50% of children will develop clinical complications including 30% requiring liver transplantation.Cholangiocarcinoma is a rare but serious complication affecting 1% of children.Ursodeoxycholic acid and oral vancomycin therapy used widely in children as medical therapy, and may be effective in a subset of patients. Gamma glutamyltransferase is a potential surrogate endpoint for disease activity, with improved survival in patients who achieve a normal value. Endoscopic retrograde cholangiopancreatography is a necessary adjunct to medical therapy to evaluate mass lesions or dominant strictures for malignancy, and also to relieve biliary obstruction. Liver transplantation remains the only option for patients who progress to end-stage liver disease. We review special considerations for patients before and after transplant, and in patients with inflammatory bowel disease. There is presently no published treatment algorithm or guideline for the management of children with PSC. We review the evidence for drug efficacy, dosing, duration of therapy, and treatment targets in PSC, and provide a framework for endoscopic and medical management of this complex problem. | Trevor J Laborda M Kyle Jensen Marianne Kavan Mark Deneau | 2019 | World Journal of Hepatology2019，11，1： | 2 |
| 2 | Self-administration of psychoactive substances by the monkey显示文摘 | Gerald Deneau Tomoji Yanagita M. H. Seevers | 1969 | Psychopharmacologia1969，，1： | 1 |
| 3 | Classification of chron- ic blepharitis显示文摘 | McCulley JP Dougherty JM Deneau DG | 1987 | Ophthalmology1987，89，： | 1 |
| 4 | Outcome after discontinuation of immunosuppression in children with auto- immune hepatitis, a population - based study显示文摘 | DENEAU M BOOK LS GUTHERY SL | 2014 | J Pediatr2014，164，4： | 1 |
| 5 | The concentrations and distributions of phytic acid phosphorus and other mineral nutrients in wild-type and low phytic acid Js-12-LPA wheat (Triticum aestivum) grain parts显示文摘 | Deneau A K Peterson Joyce C Ockenden I Raboy V and Lott John N A | | 0，，12： | 1 |
| 6 | Primary scler0sing chol- angitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history 显示文摘 | Deneau M Jensen MK Holmen J | 2013 | Hepatology2013，58，： | 1 |
| 7 | Cholangiocarcinoma in a 17-year-old boy with primary sclerosing cholangitis and inflammatory bowel disease显示文摘 | Deneau M Adler DG Schwartz JJ | 2011 | Pediatr Gastroenterol Nutr2011，52，5： | 1 |
| 8 | Self-administration of psychoactive substances by the monkey显示文摘 | Deneau G Yanagita T Seevers MH | 1969 | Psychopharmacologia1969，16，1： | 1 |
| 9 | Classification of chronic blepharitis 显示文摘 | McCulley JP Dougherty JM Deneau DG | 1982 | Ophthalmology1982，89，： | 1 |
| 10 | Outcome after discon- tinuation of immunosuppression in children with autoimmune hepatitis: a population-based study显示文摘 | Deneau M Book LS Guthery SL | 2014 | J Pediatr2014，164，71： | 1 |
| 11 | Classification of chronic blepharitis 显示文摘 | McCulley JP Dougherty JM Deneau DG | 1982 | Ophthalmology1982，89，10： | 1 |
| 12 | Insulin regulation of GnRH gene expression through MAP kinase signaling pathways显示文摘 | KIM H H DIVALL S A DENEAU R M | 2005 | Mol Cell Endocrinol2005，242，12： | 1 |
| 13 | Neonatal cholestasis and hepatosplenomegaly caused by congenital dyserythropoietic anemia type 1: A case report显示文摘BACKGROUND Congenital dyserythropoietic anemia type 1(CDA1)is an autosomal recessive disorder of ineffective erythropoiesis,resulting in increased iron storage.CDA1 is usually diagnosed in children and adolescents but can rarely present in the neonatal period with severe anemia at birth.There are no prior reports of neonatal liver histologic findings of CDA1.We report a case of CDA1 in a newborn presenting with severe anemia,cholestasis and liver failure,where liver biopsy helped confirm the diagnosis.CASE SUMMARY A term infant,born via emergency Cesarean section,presented with cholestasis,hepatosplenomegaly,multiorgan failure and severe anemia at birth.A prior pregnancy was significant for fetal demise at 35 wk without autopsy or known etiology for the fetal demise.Parents are both healthy and there is no history of consanguinity.On further evaluation,the patient was found to have severe ferritin elevation and pulmonary hypertension.An extensive infectious and metabolic work-up was negative.Salivary gland biopsy was negative for iron deposition.At 2 wk of age,a liver biopsy showed findings consistent with CDA1.A genome rapid sequencing panel revealed novel variants in the CDAN1 gene.The patient’s liver dysfunction,cholestasis and organomegaly resolved,however she remains transfusion-dependent.CONCLUSION We report liver pathology findings of CDA1 with a novel genetic mutation for the first time in a newborn. | Catalina Jaramillo Anna K Ermarth Angelica R Putnam Mark Deneau | 2019 | World Journal of Hepatology2019，11，5： | 1 |
| 14 | Natural killer cell lymphoma in a pediatric patient with inflammatory bowel dis- ease显示文摘 | Deneau M Wallentine J Guthery S etal | 2010 | Pediatrics2010，126，： | 1 |
| 15 | Self-administration of psychoactive substances by the monkey显示文摘 | Deneau G Yanagita T Seevers MH | 1969 | Psychopharmacologia1969，16，1： | 1 |
| 16 | Natural killer cell lymphoma in a pediatric patient with inflammatory bowel disease 显示文摘 | DENEAU M WALLENTINE J GUTHERY S | 2010 | Pediatrics2010，126，4： | 1 |
| 17 | Classification of chronic blepharitis显示文摘 | McCulley JP Dougherty JM Deneau DG | 1982 | Ophthalmology1982，89，： | 1 |
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