Current understanding of primary biliary cholangitis

Primary biliary cholangitis (PBC) causes chronic and persistent cholestasis in the liver, eventually resulting in cirrhosis and hepatic failure without appropriate treatment. PBC mainly develops in middle-aged women, but it is also common in young women and men. PBC is considered a model of autoimmune disease because of the presence of diseasespecific autoantibodies, that is, antimitochondrial antibodies (AMAs), intense infiltration of mononuclear cells into the bile ducts, and a high prevalence of autoimmune diseases such as comorbidities. Histologically, PBC is characterized by degeneration and necrosis of intrahepatic biliary epithelial cells surrounded by a dense infiltration of mononuclear cells, coined as chronic non-suppurative destructive cholangitis, which leads to destructive changes and the disappearance of small- or medium-sized bile ducts. Since 1990, early diagnosis with the detection of AMAs and introduction of ursodeoxycholic acid as first-line treatment has greatly altered the clinical course of PBC, and liver transplantation-free survival of patients with PBC is now comparable to that of the general population.

Autoimmune Hepatitis: 2019 Update

Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease, characterized by the elevation of aminotransferases, presence of anti-nuclear antibody or anti-smooth muscle antibody, elevated immunoglobulin G (IgG), and interface hepatitis/plasma-lymphocytic inflammation based on histology. Recent epidemiological studies have indicated an increasing trend in the prevalence of AIH worldwide, especially in male patients; this trend may suggest the alteration of environmental triggers of disease onset over time. As no disease-specific biomarker or histological finding is currently available, AIH requires a clinical diagnosis, and a validated diagnostic scoring system with acceptable specificity and sensitivity has been proposed. Regarding treatment, corticosteroids and azathioprine are recommended, and in those who exhibit an incomplete response or those who are intolerant to these drugs, second-line therapy, such as mycophenolate mofetil, is considered. Overall, the long-term outcome is excellent in patients with complete biochemical responses, while life-long maintenance treatment may be required since the cessation of immunosuppressive agents frequently leads to the relapse of the disease. Acute-onset AIH does occur, and the diagnosis is very challenging due to the lack of serum autoantibodies or elevated IgG. The unmet needs include earlier diagnosis, intervention with disseminated clinical practice guidelines, and recognition and improvement of patients’ health-related quality of life with the development of novel corticosteroid-free treatment regimens.

IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis

Sclerosing cholangitis (SC) is defined as a condition with progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis and currently includes three categories: primary sclerosing cholangitis (PSC), secondary cholangitis, and IgG4-related sclerosing cholangitis (IgG4-SC). SC categories share similar clinical features, such as cholestasis. Patients with SC present with cholestatic symptoms, including jaundice and pruritus, and blood tests reveal elevation of cholestatic enzymes. Cholangiography, endoscopic or magnetic, is inevitably required for making a diagnosis. Although the presentation of IgG4-SC and PSC are similar, the comorbidities, treatment response, and outcomes differ significantly, and therefore, it is strongly advisable to be familiar with these two diseases to make a correct diagnosis. Differentiation of cholangiocarcinoma from IgG4-SC and PSC is also extremely important. In this review, the clinical characteristics, comorbidities, treatment and outcomes of IgG4-SC and PSC will be outlined based on experience mainly from Japan.

A Case of Pyogenic Spondylodiscitis Caused by Campylobacter fetus for Which Early Diagnosis by Magnetic Resonance Imaging Was Difficult

The purpose of this case report was to report a rare case of pyogenic spondylodiscitis caused by Campylobacter fetus. A 37-year-old male presented with fever and low back pain. By lumbar magnetic resonance imaging (MRI), no abnormal finding was observed at the first presentation. However, low back pain was aggravated, and fever did not improve. Thus, lumbar MRI was repeated on the 26 day after the onset of symptoms, showing abnormal signals at vertebrae and disc spaces, and pyogenic spondylitis was diagnosed. The possibility of pyogenic spondylodiscitis should be taken into account if a patient presents with low back pain and fever, and areas of low signal intensity on a T1-weighted MRI should be carefully examined. When initial MRI does not reveal abnormal findings, repeated MRI after one or two weeks or, more favorably, immediate gadolinium enhancement MRI, are important for patients who have persistent low back pain and fever.

The Significance of the Synthetic Personality Inventory for Selecting Residents / 医学教育

Since 1998 the Synthetic Personality Inventory (SPI), a well-established aptitude test, has been used to select residents at St. Luke's International Hospital. The aims of our study were to analyze the association of the SPI with several characteristics of residents, e. g., intellectual capability, results of examinations in medicine, and evaluation during 2 years of residency, and to clarify the significance of the SPI for selecting residents. General mental ability measured with the SPI was strongly correlated with the intellectual capability of residents but was not correlated with results of examinations in medicine. High scores for general mental ability, for activity, and for tendency of extroversion were correlated with high evaluations of residents for 2 years, whereas a cooperative personality, reasoning ability, and practicality were related to an improvement in grades from the first to second years. These results demonstrate that the results of the SPI are correlated with several characteristics of residents.

Coronary Artery Revascularization in Chronic Hemodialysis Patients / 日本心臓血管外科学会雑誌

We controlled the hemodilution and electrolyte levels during coronary artery revascularization in chronic hemodialysis patients by hemofiltration during the period of extracorporeal circulation. Subjects comprised 7 chronic hemodialysis patients (males, average age 53) undergoing coronary artery revascularization in our department from January 1988 to December 1989. All patients had been undergoing hemodialysis for chronic renal failure and in one patient, after admission, continuous ambulatory peritoneal dialysis (CAPD) was additionally performed. During surgery, the dialyzer was equipped with an extracorporeal circulation circuit and the electrolyte level and hemodilution were adjusted using transfusion (1, 270±372ml). A large infusion volume (12, 657± 3, 966ml) was maintained and removal of water was carried out by ultrafiltration. After surgery, all patients underwent hemodialysis twice or more by the 3rd day of recovery. Concentrations of electrolytes were maintained at appropriate levels throughout the day of surgery except for one case of postoperative hypokalemia, but no marked changes in hemodynamics were observed during and after surgery. Hemofiltration during extracorporeal circulation is safe and useful in coronary surgery because it is simpler and requires less time than hemodialysis.