Autoimmune Cholangitis: Is It an Antimitochondrial Antibody Negative Primary Biliary Cirrhosis?

Autoimmune cholangitis/cholangiopathy (AIC) is a recently described disease characterized by chronic cholangitis resembling primary biliary cirrhosis (PBC) with a high frequency of antinuclear antibodies (ANA) and with sero-negativity for antimitochondrial antibodies (AMA). Whether AIC is a disease entity distinct from PBC and autoimmune hepatitis (AIH) or whether it is an AMAnegative variant of PBC or a cholangiopathic variant of AIH have so far been controversial. We recently examined the specificities of AMA and ANA in Japanese patients with AIC, PBC and AIH by immunofluorescence, immunoblotting and enzyme inhibition assays using various mitochondrial and nuclear autoantigens including 2-oxoacid dehydrogenase complex, Sp100, gp210, and p62, and found that AIC and PBC had similar patterns of immunoreactivity. However, this duo is of interest because, usually, among sets of autoimmune syndromes, differences in serological targetting are matched by differences in clinical presentation: AIC and PBC seem to be an exception to this rule. While it is true that a single etiological agent can produce a wide range of disease expression, it is possible that seemingly similar clinicopathological features can be induced by pathogenetic mechanisms caused by a diversity of etiological agents

高齢脳卒中リハビリテーション患者におけるMini Nutritional Assessment Short-FormとGeriatric Nutritional Risk Indexの併存的および予測的妥当性

Background: Malnutrition might worsen the clinical outcomes in stroke patients, although few nutritional screening tools have assessed their validity. Methods: We assessed clinical data of consecutive stroke patients aged ≥65 years in rehabilitation hospital from 2015 to 2017 using the Mini Nutritional Assessment Short-Form (MNA-SF) and the Geriatric Nutritional Risk Index (GNRI) for index testing. The European Society for Parenteral and Enteral Nutrition diagnostic criteria for malnutrition (ESPEN-DCM) was used as a reference standard. The receiver-operating characteristics curve was illustrated by the sensitivity (Se) and specificity (Sp). The Youden index was used to define the cut-off value for malnutrition detection or screening. The Functional Independence Measure (FIM) and discharge destination were compared for verifying predictive validity. Results: We enrolled 420 patients for the analysis. Of them, 125 patients were included in malnutrition group (mean age: 80 years) and 295 in non-malnutrition group (mean age: 77 years) by the ESPEN-DCM. The area under the curve of the MNA-SF and the GNRI were 0.890 and 0.865, respectively. Se and Sp cut-off values to detect or screen malnutrition were 5 (Se: 0.78; Sp: 0.85) and 7 (Se: 0.96; Sp: 0.57) for the MNA-SF and 92 (Se: 0.74; Sp: 0.84) and 98 (Se: 0.93; Sp: 0.50) for the GNRI, respectively. The GNRI were associated with discharge destination, whereas no correlation was observed between the MNA-SF and outcomes by multivariable analysis. Conclusions: The MNA-SF and GNRI have fair concurrent validity if appropriate cut-off values were used. The GNRI exhibits good predictive validity in stroke patients

The sperm mitochondria-specific translocator has a key role in maternal mitochondrial inheritance.

The mechanism of maternal mitochondrial inheritance in animals involves the selective elimination of sperm mitochondria by the elimination factor of the egg and the sperm mitochondria-specific factor. In vitro fertilization using sperm from isogenic mice incorporating heterospecific mitochondrial DNA (mtDNA) showed that the number of PCR positives of sperm mtDNA in two-cell embryos was significantly increased following sperm incubation with anti-tetratricopeptide repeat-containing protein involved in spermatogenesis (tpis) protein, anti-translocator of mitochondrial outer membrane (Tom) 22 and anti-Tom40 antibodies. The treatment of fertilized eggs with EGTA and other endonuclease inhibitors increased the sperm mtDNA levels. We conclude that the elimination factor, which is probably an endonuclease, is selectively received by the tpis protein of the sperm mitochondrial outer membrane within the egg. It is then transported into the sperm mitochondria by Tom22 and Tom40, where it destroys the sperm mtDNA, establishing the maternal inheritance of mtDNA.The Version of Record (VoR) is available at http://www.cellbiolint.or

Endoscopic Polypectomy of Esophageal Leiomyomas; Report of Two Cases

We describe esophageal leiomyomas in two young patients (aged 35 and 32 years), who complained of dysphagia and epigastralgia, which were successfully treated by endoscopic polypectomy. Upper endoscopy showed a pedunculated polyp beneath the normal mucosa located at 28 cm from the incisor in the first case and 1 cm sessile 2.1 cm semipedunculated polypoid lesion in the lower esophagus just above the esophageal-gastric junction in the second case. Both lesions were resected by snare polypectomy without any complication. Light microscopic examination and immunohistochemistry of the tumor tissue confirmed the diagnosis of leiomyoma. Endoscopic polypectomy of esophageal leiomyoma is safe and should be considered as an optional treatment modality whenever possible

A Case of Autoimmune Hepatitis Associated with Idiopathic Thrombocytopenic Purpura and Chronic Thyroiditis

Autoimmune hepatitis (AIH) is frequently associated with extrahepatic autoimmune disorders such as rheumatoid arthritis, Sjogren\u27s syndrome, and chronic thyroiditis, but the association with idiopathic (immune) thrombocytopenic purpura (ITP) is rare. We report a 46-year-old Japanese woman who presented with severe thrombocytopenia, elevated levels of aminotransferases, immunoglobulin (Ig) G, and platelet-associated IgG (PAIgG), positive anti-nuclear antibody, and hypothyroidism. After a diagnosis of coexisting AIH, ITP, and chronic thyroiditis, the patient was treated with 30 mg/day of prednisolone orally. The patient responded to such treatment: showing an increase in the number of platelets and decrease of serum levels of aminotransferases, IgG, and PAIgG to within normal ranges. Discrimination of ITP from liver cirrhosis as a cause of severe thrombocytopenia seen in chronic liver disease is important because complications and therapy are quite different. Prednisolone as a treatment for All should be also effective for ITP, and therefore, ITP should be considered when liver dysfunction is accompanied by severe thrombocytopenia, particularly in the autoimmune types of liver diseases

Response to Urinary Trypsin Inhibitor Therapy in Ulcerative Colitis is Associated With a Decrease in Mast Cell Count in the Colonic Mucosa

BACKGROUND: Urinary trypsin inhibitor (UTI, ulinastatin (R)) inhibits proteinases and has been used for the treatment of ulcerative colitis (UC). We investigated the therapeutic effect of UTI in patients with UC and correlated this effect to mast cell (MC) and macrophages (M) counts in the colonic mucosal wall. DESIGN: Patients with UC resistant to corticosteroids (n=16) and normal control subjects (n=10) were included in this study. Biopsy specimens obtained from the sigmoid colon of patients before and after UTI therapy were immunostained with antibodies to tryptase (AA1, MC) and CD68 (M). The number of MC and M in the lamina propria (LP) was determined and expressed per mm2 of LP. RESULTS: Nine patients with UC responded to UTI treatment. The mean number of MC in the upper part of LP in responders(440ﾂｱ51/mm2)was higher than nonresponders (312ﾂｱ76/mm2)and normal controls(200ﾂｱ47/mm2). MC counts in the lower part of the LP were not different in responders and nonresponders, although the counts in both groups were significantly higher than control. The number of M in the lower part of LP was similar in responders and nonresponders, but were higher than control subjects. M counts in the upper part of LP were similar in both groups of patients and control. Effective treatment with UTI in responders was associated with a significant fall in the number of MC in the upper layer of LP but not in M. CONCLUSION. Our results showed that UTI is an effective therapy in steroid-resistant UC. Our results also showed effective therapy with UTI was associated with a reduction in MC counts in the colonic mucosa, suggesting that the control of these cells may mediate, at least in part, the therapeutic effects of UTI in UC

Dietary intake, mental status, physical activity, and lifestyle affecting bowel movement frequency and stool texture in young Japanese women

Constipation is a symptom-based disorder, and its definition is mainly subjective. Patients are more concerned with ease ofpassage and consistency rather than frequency of bowel movement. Studies on bowel movement frequency and stool texture inthe general population are sparse, especially in young women. In this cross-sectional study, data obtained from self-administered questionnaires, including age, height, body weight, lifestyle, food habits, anxiety, depressive status, frequency of bowel movements,stool texture, and defecation-related symptoms were analyzed in 245 female Japanese university students. An establishedsemiquantitative questionnaire available for clinical investigation (FFQg) was used to obtain a detailed assessment of food intake and physical activity levels. Of the participants, 21.4% had bowel movements ?3 times per week and 33.3% had hard or lumpy stools ?25% and loose (mushy) or watery stools <25% of bowel movements. There was a positive association between infrequent bowel movements and hard or lumpy stools. These two situations both caused similar symptoms such as a sensation of incomplete evacuation and straining. There was no association of bowel movement frequency and stool texture with any specificnutrients and foods, dietary intake, mental status, or physical activity. Several lifestyle factors such as regular bowel movements and hesitation with evacuation were associated with bowel movement frequency and stool texture. Several lifestyle factors, but not mental, physical, or dietary intake factors, were associated with bowel movement frequency and stool texture in young Japanese women

Protein-Energy Malnutrition in Patients with Liver Cirrhosis

Protein-energy malnutrition (PEM) is frequently seen in patients with liver cirrhosis. This condition is associated with a poorprognosis and reduced survival. We investigated the protein and energy metabolic status, including serum albumin concentration,and resting energy expenditure (REE) and respiratory quotient (RQ) measured by indirect calorimetry in 23 patients withliver cirrhosis (8 men and 15 women; mean age, 60.3 years). The median value of %REE (measured REE / predicted REE)was highest in Child-Pugh grade A and lowest in grade C, and the range of RQ tended to be highest in Child-Pugh grade Aand lowest in grade C, although there were not statistically significant (p=0.871 and 0.664, respectively). Serum triglycerideconcentration was significantly lower in patients who had an RQ less than 0.85 than in patients who had an RQ of 0.85 ormore, and free fatty acid tended to be higher in patients who had an RQ less than 0.85 than in patients who had an RQ of 0.85or more. Of the 23 patients, 78.3% were in a state of protein and/or energy malnutrition and 47.8% had PEM. Our results suggestthat %REE and RQ were not significantly associated with liver function, but the oxidation rate of fat was increased in advancedliver cirrhosis. A longitudinal study in a large population is needed to determine the efficacy of %REE and RQ measurementsfor adequate nutritional treatment and improvement of patient outcome