Advantages of an alternate-day glucocorticoid treatment strategy for the treatment of IgG4-related disease: A preliminary retrospective cohort study.

Alternate-day glucocorticoid (GC) therapy is a treatment option that can reduce GC-associated adverse events. We investigated the safety and efficacy of alternate-day GC therapy in patients with immunoglobulin G4-related disease (IgG4-RD). Medical records of patients with IgG4-RD who were followed for at least one year at St. Luke's International Hospital, Tokyo, Japan, from 2004 to 2020 were reviewed. Patients who fulfilled comprehensive IgG4-RD diagnostic criteria were divided into alternate-day or daily GC treatment groups based on their treatment protocol. The effect of alternate-day GC therapy on glucocorticoid toxicity index (GTI) score was evaluated using multilinear analysis with adjustments for cumulative GC doses until each assessment point and propensity scores (PS) for alternate-day GC therapy. Kaplan-Meier curves and Cox proportional hazard models were used to assess the efficacy of alternate-day GC therapy for disease control. Among the 67 patients with IgG4-RD, patients with alternate-day (n = 13) and daily (n = 31) GC treatments were analyzed after excluding 23 ineligible patients. The median (interquartile range) age was 64 (60-70) years, 29 (65.9%) were male patients, 26 (59.1%) patients had positive biopsy results, and the median follow-up period was 1643 days. Significantly more patients with alternate-day GC treatment used concomitant immunosuppressants (11 [84.6%] vs 11 [35.5%]; P = .007). The alternate-day strategy significantly lowered the GTI score after adjusting for cumulative GC dose until the assessment and PS (adjusted coefficient: -29.5 [-54.3, -4.8], P = .021 at 12 months; -20.0 [-39.8, -0.1], P = .049 at 24 months). Serious infections were numerically less frequent in the alternate-day group (incidence ratio [95% confidence interval [CI]: 0.45 [0.05, 3.63], P = .45). Most patients (92.3%) in the alternate-day GC treatment group and all patients in the daily GC treatment group showed treatment responses in the remission induction therapy. The PS-adjusted hazard ratio of alternate-day GC treatment for disease flares was not significant (1.55 [0.53, 4.51]; P = .43). The alternate-day treatment strategy significantly reduced GC-related adverse events regardless of the cumulative GC dose. Alternate-day GC treatment is a feasible option for patients with IgG4-RD, without a significant increase in disease flares particularly when combined with immunosuppressants.

Image quality and radiologists' subjective acceptance using model-based iterative and deep learning reconstructions as adjuncts to ultrahigh-resolution CT in low-dose contrast-enhanced abdominopelvic CT: phantom and clinical pilot studies.

PURPOSE: In contrast-enhanced abdominopelvic CT (CE-APCT) for oncologic follow-up, ultrahigh-resolution CT (UHRCT) may improve depiction of fine lesions and low-dose scans are desirable for minimizing the potential adverse effects by ionizing radiation. We compared image quality and radiologists' acceptance of model-based iterative (MBIR) and deep learning (DLR) reconstructions of low-dose CE-APCT by UHRCT. METHODS: Using our high-resolution (matrix size: 1024) and low-dose (tube voltage 100 kV; noise index: 20-40 HU) protocol, we scanned phantoms to compare the modulation transfer function and noise power spectrum between MBIR and DLR and assessed findings in 36 consecutive patients who underwent CE-APCT (noise index: 35 HU; mean CTDIvol: 4.2 ± 1.6 mGy) by UHRCT. We used paired t-test to compare objective noise and contrast-to-noise ratio (CNR) and Wilcoxon signed-rank test to compare radiologists' subjective acceptance regarding noise, image texture and appearance, and diagnostic confidence between MBIR and DLR using our routine protocol (matrix size: 512; tube voltage: 120 kV; noise index: 15 HU) for reference. RESULTS: Phantom studies demonstrated higher spatial resolution and lower low-frequency noise by DLR than MBIR at equal doses. Clinical studies indicated significantly worse objective noise, CNR, and subjective noise by DLR than MBIR, but other subjective characteristics were better (P < 0.001 for all). Compared with the routine protocol, subjective noise was similar or better by DLR, and other subjective characteristics were similar or worse by MBIR. CONCLUSION: Image quality, except regarding noise characteristics, and acceptance by radiologists were better by DLR than MBIR in low-dose CE-APCT by UHRCT.

Nonhepatocytic malignant mixed tumor of the liver in adults: report of a long surviving case.

A primary nonhepatocytic malignant mixed tumor in the liver contains both epithelial and mesenchymal components, and the incidence in adults is extremely rare. A 45-year-old female was admitted because of abdominal fullness. Abdominal imaging studies revealed a huge cystic tumor with a mural nodule in the right lobe. A right trisegmentectomy and an invaded partial diaphragm resection were performed. Diagnosis was established after surgery. The patient is still alive 11 years after surgery, and to our knowledge is the longest surviving patient with a primary nonhepatocytic malignant mixed primary tumor of the liver.

A case of isolated pancreatic metastasis of gastric cancer presenting problematic discrimination from gastropancreatic double cancer.

We experienced a case of isolated pancreatic metastasis caused by gastric cancer which showed high levels of the tumor markers relevant to pancreatic cancer. The patient was a 59-year-old man who had tumors in both the gastric antrum and pancreatic head. He was diagnosed as having double cancer of the stomach and pancreas on account of the high values of pancreatic cancer-associated markers, and underwent operation. This resulted in non-curable resection attributable to broad lymph node metastasis. The resected specimen lacked continuity between the pancreas tumor and gastric tumor, and the pancreatic tumor was histologically diagnosed as metastasis from the gastric cancer. Moreover, the advanced lymph vessel invasion suggested possible metastasis through the lymph ducts. The present case is a rare metastatic form because there has been no report of lymphogenous isolated pancreatic metastasis of gastric cancer. In advanced gastric cancer, the possibility of pancreatic metastasis should be always borne in mind, and pancreatic biopsy may be taken into account at times. Radical operation may be performed corresponding to the degree of each stage of the double cancer of stomach and pancreas. However, considering the low possibility of curable resection for pancreatic metastasis of gastric cancer, other treatments than surgery should also be considered.

Indications for extended hepatectomy in the management of stage IV hilar cholangiocarcinoma.

HYPOTHESIS: In operations for hilar cholangiocarcinoma, simultaneous extended hepatectomy and removal of extrahepatic bile ducts are considered curative resection. However, the effect of extended operations for stage IV hilar cholangiocarcinoma on survival is still unclear. DESIGN: Retrospective review of the treatment of hilar cholangiocarcinoma from 1981 to 2001. PATIENTS AND METHODS: Fifty-seven patients with stage IVA or IVB hilar cholangiocarcinoma were enrolled. Thirty-three of these patients underwent extended hepatectomy to achieve macroscopic radical resection (surgical group). A self-expandable metallic biliary stent (EMBS) was implanted in 24 patients (EMBS group) in whom radical treatment was judged to be impossible. Main Outcome Measure Survival in patients with stage IV hilar cholangiocarcinoma treated by means of extended operation or stenting. RESULTS: Survival was 25.7 +/- 40.9 months in the surgical group vs 6.5 +/- 5.8 months in the EMBS group (P =.03). In the surgical group, radical resection results were macroscopically and histologically successful in 21 patients (64%). In patients with stage IVB disease, survival did not differ between the surgical and EMBS groups. CONCLUSIONS: In patients with stage IVA disease, radical extended hepatectomy should be performed after excluding patients who have extensive invasion of the hepatic artery or portal vein. However, in patients with stage IVB disease with carcinomatous peritonitis or distant metastasis, there is little possibility of achieving long-term survival with surgery, and stent implantation should be the first choice.

Fructose-1,6-Bisphosphate inhibits excess activation of Kupffer cell function induced by endotoxin.

The effect and mechanism of action of fructose-1,6-bisphosphate (FBP) on Kupffer cell activation were studied in vitro. Kupffer cell was activated by isolation procedure alone from the hepatic tissue. In cultured rat Kupffer cells stimulated by endotoxin, treatment with 5-20 mM FBP not only preserved phagocytic activity, but also inhibited secretion of cytokines (tumor necrosis factor-a and interleukin-1beta) and production of nitric oxide (NOx). Moreover, treatment with 10 mM FBP suppressed the elevation in the intracellular Ca2+ concentration on Kupffer cells stimulated by phorbol 12-myristate 13-acetate, which suggested that this effect may be one of the agents that limit the activation of Kupffer cells. The administration of FBP was effective in the prevention of endotoxin-induced hepatopathy, and we suggest that this may have useful clinical applications.

Storage solution containing fructose-1,6-bisphosphate inhibits the excess activation of Kupffer cells in cold liver preservation.

BACKGROUND: In liver transplantation, the activation of Kupffer cells at the time of cold preservation and reperfusion is considered to play an important role. In the present study, the usefulness of cold storage solution containing fructose-1,6-bisphosphate (FBP) was compared with University of Wisconsin (UW) solution in the function of Kupffer cells. METHODS: Kupffer cells were separated from rat liver stored at 4 degrees C in each storage solution. Four kinds of storage solutions were used: UW, simplified UW without FBP (0-FBP), and solutions with 10 or 20 mM FBP (10-FBP, 20-FBP). Lipopolysaccharide (LPS) labeled by fluorescein was loaded after 12 or 24 hr of cold preservation in each solution. The rates of cells uptaking LPS as phagocytic ability were measured using flow cytometry. Tumor necrosis factor-alpha, cytokine-induced neutrophil chemoattractant, and nitric oxide (NO) were measured in the supernatant. RESULTS: Tumor necrosis factor-alpha values in the 20-FBP group were significantly lower than those in the UW group. Cytokine-induced neutrophil chemoattractant values at 60 min after loading LPS were significantly lower in the 20-FBP group than in the UW group. NO values at 24 hr after loading LPS were significantly lower in the 20-FBP group compared with the UW group. The 20-FBP group was highest in the rates of cells uptaking LPS after 24-hr cold preservation. CONCLUSIONS: The storage solution containing FBP controlled the secretion of cytokines and NO from Kupffer cells and maintained phagocytic ability. This solution was considered to be more useful than UW solution for Kupffer cell protection.