OBJECTIVETo investigate the current status of research on infantile cholestatic liver disease in China and future research trends.

METHODSA co-word analysis was performed in October 2016. Document retrieval and screening were performed in the Chinese databases CNKI and Wanfang Data using "cholestasis" and "infant" as key words. Excel 2010 was used to establish a co-occurrence matrix of high-frequency key words, and Ucinet 6.0 and Netdraw were used to develop a visualized network of these high-frequency key words.

RESULTSA total of 383 articles were included. The co-occurrence analysis showed that "infant" and "cholestasis" were the core of research in this field, and "infantile hepatitis syndrome", "neonate", "intrahepatic", "biliary atresia", "heredity and metabolism", "hepatitis", "cytomegalovirus", "jaundice", and "conjugated bilirubin" were main research topics. Most of the other articles focused on "parenteral nutrition", "hepatobiliary imaging", "gene mutation", and "liver biopsy". There were relatively few articles on surgical diagnostic techniques and treatment for this disease.

CONCLUSIONSThe research on infantile cholestatic liver disease in China focuses on etiology and differential diagnosis, and genetic diagnosis has become a hot topic in recent years. The research on treatment should be enhanced, and new diagnostic techniques are the research interest in future.

Cholestasis, Intrahepatic ; diagnosis ; etiology ; genetics ; therapy ; Humans ; Infant

Autochthonous hepatitis E virus (HEV) is an emerging pathogen in developed countries, and several cases of acute HEV infection have been reported in South Korea. However, there have been no reports on HEV-associated Guillain-Barré syndrome (GBS) in Korea. We recently experienced the case of a 58-year-old Korean male with acute HEV infection after ingesting raw deer meat. Persistent cholestasis was resolved by the administration of prednisolone. At 2.5 months after the clinical presentation of HEV infection, the patient developed weakness of the lower limbs, and was diagnosed with GBS associated with acute hepatitis E. To our knowledge, this is the second report on supportive steroid therapy for persistent cholestasis due to hepatitis E, and the first report of GBS in a Korean patient with acute HEV infection.
Acute Disease ; Alanine Transaminase/blood ; Antibodies, Viral/blood ; Aspartate Aminotransferases/blood ; Bilirubin/analysis ; Cholestasis/*drug therapy ; Guillain-Barre Syndrome/complications/*diagnosis ; Hepatitis E/*diagnosis/etiology ; Hepatitis E virus/immunology ; Humans ; Immunoglobulin M/blood ; Liver/pathology ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Republic of Korea ; Steroids/*therapeutic use

The purpose of this study was to investigate the etiology, pathological characteristics, management and prognosis of chronic pancreatitis in the Chinese population. The clinical data of 142 patients with chronic pancreatitis were retrospectively studied. All patients were of Chinese nationality and hospitalized from January 2008 to December 2011. Their ages ranged from 14 to 76 years, with a mean of 43 years. Of 142 patients, there were 72 cases of obstructive chronic pancreatitis (50.70%), 19 cases of alcoholic chronic pancreatitis (13.38%), 14 cases of autoimmune pancreatitis (9.86%) and 37 cases of undetermined etiology (26.06%). Pathologically, the average inflammatory mass diameter was 3.8 ± 3.3 cm, biliary obstruction occurred in 36 cases, gall stones in 70 cases, calcification in 88 cases, ductal dilatation in 61 cases, side branch dilatation in 32 cases, ductal irregularity in 10 cases, lymphocytic inflammation in 23 cases, obliterative phlebitis in 14 cases, extra pancreatic lesion in 19 cases and fibrosis in 142 cases. Location of pancreatic lesion in the region of head (n=97), neck (n=16), body (n=12), tail (n=15) and whole pancreas (n=2) influenced the choice of surgical procedures. Ninety-four patients (66.20%) received surgical treatment and 33.80% received other treatments. After operation, 80.85% of 94 patients experienced decreased pain, and 8.51% of 94 showed recovery of endocrine function but with a complication rate of 12.77%. All the operations were performed successfully. According to the pain scale of European Organization for Research and Treatment of Cancer (QLQ-C30) a decrease from 76 ± 22 to 14 ± 18 was observed. Etiology, pathological characteristics, management and prognosis of chronic pancreatitis in the Chinese population vary from others.
Adolescent ; Adult ; Aged ; Autoimmune Diseases ; epidemiology ; therapy ; China ; epidemiology ; Cholestasis ; epidemiology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Pancreatitis, Alcoholic ; epidemiology ; therapy ; Pancreatitis, Chronic ; etiology ; pathology ; therapy ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Young Adult

Cholestasis ; complications ; Consensus ; Humans ; Liver Diseases ; diagnosis ; etiology ; therapy ; Practice Guidelines as Topic

Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by bone fragility and connective tissue manifestations. We report a successful liver transplantation (LT) in an 8-month-old boy with OI and cholestatic biliary cirrhosis. After 4 cycles of intravenous pamidronate, LT was performed under intravenous anesthesia using a left lateral section from his mother without mechanical retractors. The operation time was 420 min and estimated blood loss was 520 mL requiring one unit of RBC transfusion. He was discharged without surgical complications. Therefore, LT should be considered for patients with end stage liver disease and OI under organic multidisciplinary cooperation.
Bone Density ; Bone Density Conservation Agents/therapeutic use ; Cholestasis, Intrahepatic/*diagnosis ; Diphosphonates/therapeutic use ; Fractures, Bone/drug therapy/etiology/radiography ; Humans ; Infant ; *Liver Transplantation ; Living Donors ; Male ; Osteogenesis Imperfecta/complications/*surgery

Biliary Atresia ; diagnosis ; etiology ; therapy ; Bilirubin ; blood ; Biomarkers ; blood ; Calcium-Binding Proteins ; deficiency ; Cholangiopancreatography, Magnetic Resonance ; Cholestasis, Intrahepatic ; diagnosis ; etiology ; therapy ; Citrullinemia ; diagnosis ; etiology ; therapy ; DNA Mutational Analysis ; Humans ; Infant ; Jaundice ; diagnosis ; etiology ; therapy ; Liver Function Tests ; Male ; Mitochondrial Membrane Transport Proteins ; genetics ; Mutation ; Organic Anion Transporters ; deficiency

BACKGROUND/AIMS: Stent migration occurs frequently, but the prevention of complications resulting from covered self-expandable metal stents (C-SEMSs) remains unresolved. We prospectively assessed a newly developed C-SEMS, a modified covered Zeo stent (m-CZS), in terms of its antimigration effect. METHODS: Between February 2010 and January 2011, an m-CZS was inserted into 42 patients (31 initial drainage cases and 11 reintervention cases) at a tertiary referral center and three affiliated hospitals. The laser-cut stent was flared for 1.5 cm at both ends, with a 1 cm raised bank located 1 cm in from each flared end. The main outcome of this study was the rate of stent migration, and secondary outcomes were the rate of recurrent biliary obstruction (RBO), the time to RBO, the frequencies of complications, and overall survival. RESULTS: Of the 31 patients with initial drainage, stent migration occurred in four (12.9%, 95% confidence interval, 5.1% to 29.0%), with a mean time of 131 days. RBO occurred in 18 (58%), with a median time to RBO of 107 days. Following previous C-SEMS migration, seven of 10 patients (70%) did not experience m-CZS migration until death. CONCLUSIONS: m-CZSs with antimigration properties effectively, although not completely, prevented stent migration after stent insertion.
Aged ; Aged, 80 and over ; Alloys ; Carcinoma/*complications ; Cholestasis/etiology/*therapy ; Digestive System Neoplasms/*complications ; Drainage ; Equipment Design ; Feasibility Studies ; Female ; Humans ; Kaplan-Meier Estimate ; Lymphatic Metastasis ; Male ; Middle Aged ; *Prosthesis Failure ; Recurrence ; Reoperation ; *Stents/adverse effects ; Time Factors

OBJECTIVETo analyze the effects of ursodeoxycholic acid (UDCA) on the mRNA expression of multidrug resistance protein 3 (MDR3) and farnesoid X receptor (FXR) in infants with cholestatic hepatitis.

METHODSTwenty-eight infants who were diagnosed with cholestatic hepatitis between July 2008 and July 2010 were included in the study. These patients received treatment with UDCA. The mRNA expression levels of MDR3 and FXR were measured by real-time quantitative RT-PCR with SYBR Green I, before and after treatment with UDCA.

RESULTSAfter treatment with UDCA, the infants with cholestatic hepatitis had significantly decreased serum levels of total bilirubin, direct bilirubin, alanine aminotransferase, and gamma-glutamyltransferase (P<0.05) and significantly increased mRNA expression of MDR3 (P<0.05). No significant change in mRNA expression of FXR was observed, however (P>0.05).

CONCLUSIONSUDCA improves liver function indices in infants with cholestatic hepatitis, which may be related to up-regulated mRNA expression of MDR3.

ATP Binding Cassette Transporter, Sub-Family B ; genetics ; Cholestasis ; complications ; drug therapy ; metabolism ; Female ; Gene Expression Regulation ; Hepatitis ; drug therapy ; etiology ; metabolism ; Humans ; Infant ; Male ; RNA, Messenger ; analysis ; Receptors, Cytoplasmic and Nuclear ; genetics ; Ursodeoxycholic Acid ; pharmacology ; therapeutic use

Animals ; Cholestasis ; complications ; Fibrosis ; etiology ; Gallbladder ; pathology ; Humans ; Kidney ; pathology ; Lipid Metabolism ; Liver Cirrhosis ; etiology ; therapy ; Myocardium ; pathology ; Receptors, Cytoplasmic and Nuclear ; physiology

Hepatitis A virus (HAV) infections occur predominantly in children, and are usually self-limiting. However, 75-95% of the infections in adults are symptomatic (mostly with jaundice), with the illness symptoms usually persisting for a few weeks. Atypical manifestations include relapsing hepatitis, prolonged cholestasis, and complications involving renal injury. Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a severe, drug-induced hypersensitivity reaction characterized by skin rash, fever, lymph-node enlargement, and internal organ involvement. We describe a 22-year-old male who presented with acute kidney injury and was diagnosed with prolonged cholestatic hepatitis A. The patient also developed DRESS syndrome due to antibiotic and/or antiviral treatment. To our knowledge, this is the first report of histopathologically confirmed DRESS syndrome due to antibiotic and/or antiviral treatment following HAV infection with cholestatic features and renal injury.
Acute Kidney Injury/diagnosis ; Anti-Bacterial Agents/*adverse effects/therapeutic use ; Cefotaxime/adverse effects/therapeutic use ; Cholestasis/complications/*diagnosis ; Cytomegalovirus/genetics ; Cytomegalovirus Infections/drug therapy/virology ; DNA, Viral/analysis ; Eosinophilia/etiology ; Exanthema/*chemically induced/pathology ; Ganciclovir/therapeutic use ; Hepatitis A/complications/*diagnosis/drug therapy ; Humans ; Hydrocortisone/therapeutic use ; Immunoglobulins/therapeutic use ; Male ; Syndrome ; Young Adult