What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

The diagnosis of cystic liver disease has made great progress with the advent of enhanced imaging techniques. At the same time, its management has gradually improved over the past few decades, providing the basis for the development of appropriate diagnostic and treatment guidelines. To this end, the European Association for the Study of the Liver has developed clinical guidelines for the diagnosis and treatment of non-infectious cystic liver disease. This guideline put forward recommendations based on an in-depth review of the relevant literature for addressing clinical issues, including the diagnosis and treament of hepatic cysts, hepatic mucocystic tumors, biliary hamartomas, polycystic liver disease, Caroli disease or Caroli syndrome, biliary hamartomas, and peribiliary cyst.
Humans ; Liver Diseases/pathology* ; Cysts/pathology* ; Caroli Disease/diagnosis* ; Liver Neoplasms/therapy* ; Hamartoma

In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued the consensus on the diagnosis and management of cholestatic liver diseases. In the past years, more data have emerged from clinical practice. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 22 recommendations on clinical practice of cholestatic liver diseases. The guidelines aim to provide a working reference for the management of cholestatic liver diseases.
Autoimmune Diseases/diagnosis* ; Cholestasis/therapy* ; Consensus ; Gastroenterology ; Humans ; Liver Diseases/therapy*

Hepatic osteodystrophy is frequent complication in patients with chronic liver disease, particularly with chronic cholestasis. We report a male infant with congenital hepatoblastoma, who had osteodystrophy complicated by multiple bone fractures despite adequate supplementation of fat-soluble vitamins including vitamin D. He was born by Caesarean section because of a 7 cm–sized abdominal mass detected by prenatal ultrasonography. The pathologic diagnosis was hepatoblastoma, PRETEXT staging III or IV. Whole body bone scan at the time of diagnosis showed no abnormal uptake. Oral vitamin D3 of 2,000 IU/day was administered with other fat-soluble vitamins. Serum direct bilirubin level gradually increased up to 28.9 mg/dL at postnatal 6 days and was above 5 mg/dL until 110 days of age. Bony changes consistent with rickets became apparent in left proximal humerus since 48 days of age, and multiple bone fractures developed thereafter. With resolving cholestasis by chemotherapy, his bony lesions improved gradually after add-on treatment of bisphosphonate and parenteral administration of vitamin D with calcium. High level of suspicion and prevention of osteodystrophy is needed in patients with hepatoblastoma, especially when cholestasis persists.
Bilirubin ; Calcium ; Cesarean Section ; Cholecalciferol ; Cholestasis ; Diagnosis ; Drug Therapy ; Female ; Fractures, Bone ; Hepatoblastoma ; Humans ; Humerus ; Infant ; Liver Diseases ; Male ; Pregnancy ; Rickets ; Ultrasonography, Prenatal ; Vitamin D ; Vitamins

Due to advances in the treatment and diagnosis of cancer, many women survive long after treatment, and therefore express concerns about the impact of estrogen deficiency on their quality of life. Cancer treatment can induce menopause through surgical removal of the ovaries, chemotherapy, or radiation. Women who undergo induced menopause usually experience more sudden and severe menopausal symptoms, including vasomotor symptoms, psychological symptoms, genitourinary symptoms, cardiovascular disease, and osteoporosis. Menopausal hormone therapy (MHT) is especially important in women younger than 40. In this review, we consider the role of MHT after the diagnosis of breast, gynecologic, colorectal, stomach, liver, lung, and hematologic cancers. MHT is advantageous in endometrial cancer type I, cervical squamous cell carcinoma, colorectal cancer, hepatocellular carcinoma, and hematologic malignancies. However, MHT is not recommended for use in breast cancer, endometrial stromal sarcoma, hormone receptor–positive gastric cancer, and lung cancer survivors because it is linked to an increased risk of cancer recurrence. Depending on the type of cancer, clinicians should recommend that cancer survivors receive appropriate MHT in order to reduce vasomotor symptoms and to benefit from its positive effects on the cardiovascular and skeletal systems.
Breast ; Breast Neoplasms ; Carcinoma, Hepatocellular ; Carcinoma, Squamous Cell ; Cardiovascular Diseases ; Colorectal Neoplasms ; Diagnosis ; Drug Therapy ; Endometrial Neoplasms ; Estrogens ; Female ; Hematologic Neoplasms ; Humans ; Liver ; Lung ; Lung Neoplasms ; Menopause ; Osteoporosis ; Ovary ; Quality of Life ; Recurrence ; Sarcoma, Endometrial Stromal ; Stomach ; Stomach Neoplasms ; Survivors

PURPOSE: Hepatocellular carcinoma (HCC) is a rarely occurring disease in the pediatric population. We report our center's experience of management of HCC in children and adolescents. METHODS: From 1996 to 2012, 16 patients aged 18 or younger were diagnosed with HCC at our center. The medical records of these 16 patients were retrospectively reviewed. RESULTS: There were 9 boys and 7 girls. Median age at diagnosis of HCC was 14.5 years. All patient had pathologically confirmed diagnosis of HCC. Three patients had distant metastasis at the time of HCC diagnosis. Eight patients were surgically managed, including 4 liver resections, 3 liver transplantations, and 1 intraoperative radiofrequency ablation. The remaining 8 patients received systemic chemotherapy. Overall, 6 patients are alive at median 63.6 months after diagnosis of HCC. All survivors were surgically managed patients. CONCLUSION: HCC is a rare disease occurring in childhood. Patients with systemic disease have poor outcome. Liver transplantation may be a good option for treatment of pediatric HCC.
Adolescent* ; Carcinoma, Hepatocellular* ; Catheter Ablation ; Child* ; Diagnosis ; Drug Therapy ; Female ; Humans ; Liver ; Liver Transplantation ; Medical Records ; Neoplasm Metastasis ; Pediatrics ; Rare Diseases ; Retrospective Studies ; Survivors

BACKGROUND/AIMS: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that typically presents in the form of skin manifestations with or without lymph node and bone marrow involvement. Given its rarity and recent recognition as a distinct pathological entity, no standard of treatment exists for this aggressive disease and its prognosis is particularly dismal. METHODS: We retrospectively analyzed clinical features and treatment outcomes of patients who were diagnosed with BPDCN between 2000 and 2014. RESULTS: Ten patients had a median age at diagnosis of 41 years (range, 18 to 79), and seven patients were male. Sites of disease involvement were the skin (n = 7), lymph node (n = 5), bone marrow (n = 2), liver (n = 2), spleen (n = 2), and soft tissue (n = 1). Intensified chemotherapy regimens such as hyperCVAD regimen (cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, cytarabine), and VPDL (vincristine, methylprednisolone, daunorubicin, L-asparaginase) were used as a first-line treatment. Although all patients treated with intensified chemotherapy showed an objective response (five patients with complete response) with median progression-free survival of 11.2 months (range 6.2 to 19.4), complete remission was not sustained for more than 2 years in any case. The response was relatively long-lived compared with previously reported CHOP (doxorubicin, cyclophosphamide, vincristine, prednisone)-like regimens, but the above regimens do not result in long-term remission. CONCLUSIONS: All patients treated with hyperCVAD or VPDL showed an objective response, but the duration of response was relatively short. Thus, the development of more effective induction as well as consolidation treatment strategy should be warranted to improve this rare disease entity.
Bone Marrow ; Cyclophosphamide ; Daunorubicin ; Dendritic Cells* ; Dexamethasone ; Diagnosis ; Disease-Free Survival ; Doxorubicin ; Drug Therapy ; Hematologic Neoplasms ; Humans ; Korea* ; Liver ; Lymph Nodes ; Male ; Methotrexate ; Methylprednisolone ; Prognosis ; Rare Diseases ; Retrospective Studies ; Skin ; Skin Manifestations ; Spleen ; Vincristine

PURPOSE: The purpose of this study was to understand patient treatment patterns, outcomes, and healthcare resource use in cases of metastatic and/or locally recurrent, unresectable gastric cancer (MGC) in South Korea. MATERIALS AND METHODS: Thirty physicians reviewed charts of eligible patients to collect de-identified data. Patients must have received platinum/fluoropyrimidine first-line therapy followed by second-line therapy or best supportive care, had no other primary cancer, and not participated in a clinical trial following MGC diagnosis. Data were summarized using descriptive statistics. Kaplan-Meier analysis was used to describe survival. RESULTS: Of 198 patients, 73.7% were male, 78.3% were diagnosed with MGC after age 55 (mean, 61.3 years), and 47.0% were current or former smokers. The majority of tumorswere located in the antrum/pylorus (51.5%). Metastatic sites most often occurred in the peritoneum (53.5%), lymph nodes (47.5%), and liver (38.9%). At diagnosis, the mean Charlson comorbidity indexwas 0.4 (standard deviation, 0.6). The most common comorbidities were chronic gastritis (22.7%) and cardiovascular disease (18.7%). Most patients (80.3%) received second-line treatment. Single-agent fluoropyrimidine was reported for 22.0% of patients, while 19.5% were treated with irinotecan and a fluoropyrimidine or platinum agent. The most common physician-reported symptoms during second-line treatment were nausea/vomiting (44.7%) and pain (11.3%), with antiemetics (44.7%), analgesics (36.5%), and nutritional support (11.3%) most often used as supportive care. Two-thirds of inpatient hospitalizations were for chemotherapy infusion. Outpatient hospitalization (31.6%) and visits to the oncologist (58.8%) were common among second-line patients. CONCLUSION: Most patients received second-line treatment, although regimens varied. Understanding MGC patient characteristics and treatment patterns in South Korea will help address unmet needs.
Analgesics ; Antiemetics ; Cardiovascular Diseases ; Comorbidity ; Delivery of Health Care ; Diagnosis ; Drug Therapy ; Gastritis ; Hospitalization ; Humans ; Inpatients ; Kaplan-Meier Estimate ; Korea* ; Liver ; Lymph Nodes ; Male ; Nutritional Support ; Observational Study ; Outpatients ; Peritoneum ; Platinum ; Republic of Korea ; Stomach Neoplasms*

Some patients have ascites without having liver disease, so it is important to analyze the cause of these ascites. Tuberculous peritonitis is an infectious disease characterized by lymphocyte-dominant exudative ascites. In contrast, myxedema ascites is a very rare disease characterized by a high serum/ascites albumin gradient (SAAG) with hypothyroidism. We herein report a case involving a 48-year-old woman with both diseases simultaneously. She was hospitalized because of massive ascites, generalized edema, and a puffy face. Hypothyroidism was confirmed by thyroid function tests. Her ascitic fluid had a high SAAG; no other specific findings were identified by cytology, culture, or computed tomography. Three months after initiating drug therapy for the hypothyroidism, the patient's systemic edema improved but the ascites recurred. Accordingly, diagnostic laparoscopy was performed, and tuberculous peritonitis was confirmed. As seen in this case, when myxedema ascites is associated with tuberculous peritonitis, an accurate diagnosis may be challenging.
Ascites* ; Ascitic Fluid ; Communicable Diseases ; Diagnosis ; Drug Therapy ; Edema ; Female ; Humans ; Hypothyroidism ; Laparoscopy ; Liver Diseases ; Middle Aged ; Myxedema* ; Peritonitis, Tuberculous* ; Rare Diseases ; Thyroid Function Tests

Along with the aging process, the spectrum of liver disease changes greatly. Nonalcoholic fatty liver disease (NAFLD) in elderly people lead to low liver function and is also the major cause of extrahepatic diseases, such as cardiovascular disease and malignant tumor. This review provides an overview of the morphological structure and function of the liver in aged people, and discusses the characteristics of weakness, malnutrition and limited movement in the elderly, as well as the current status of multiple diseases and multiple drug use. Finally, this article puts forward some appropriate regimens for the diagnosis and treatment of NAFLD in elderly people to provide a reference for clinical practice.
Aged ; Cardiovascular Diseases ; Humans ; Liver ; pathology ; Malnutrition ; Neoplasms ; Non-alcoholic Fatty Liver Disease ; diagnosis ; pathology ; therapy ; Risk Factors