The role of peroral video cholangioscopy in patients with IgG4-related sclerosing cholangitis

Background

The cholangioscopic features of IgG4-related sclerosing cholangitis (IgG4-SC) remain undefined. The aim of this study was to clarify these endoscopic features using peroral video cholangioscopy (PVCS) in IgG4-SC patients.

Methods

PVCS was performed in 33 patients: IgG4-SC (n = 13); primary sclerosing cholangitis (PSC; n = 5); and cholangiocarcinoma (n = 15), which included hilar cholangiocarcinoma (HCCA; n = 5) and distal cholangiocarcinoma (DCCA; n = 10).

Results

The most frequent findings on PVCS in the IgG4-SC patients were dilated (62 %) and tortuous (69 %) vessels, and absence of partially enlarged vessels. The incidence of dilated and tortuous vessels was significantly higher in IgG4-SC patients than in PSC patients (p = 0.015). Scarring and pseudodiverticula were found significantly more often in PSC patients than in IgG4-SC patients (p = 0.001 and p = 0.0007, respectively). The incidence of partially enlarged vessels was significantly higher in DCCA patients than in IgG4-SC patients (p = 0.004). In contrast, the incidence of dilated vessels was significantly higher in IgG4-SC patients than in HCCA patients (p = 0.015). PVCS performed after corticosteroid therapy showed resolution of bile duct stenosis and dilated, tortuous, or partially enlarged vessels, as well as resolution of friability in all patients with IgG4-SC.

Conclusion

Cholangioscopy was useful in differentiating IgG4-SC from PSC. In addition, monitoring the patterns of proliferative vessels on PVCS may be useful to differentiate IgG4-SC from cholangiocarcinoma.     

Factors affecting the prognosis of patients with hepatocellular carcinoma invading the portal vein--a retrospective analysis using 952 consecutive HCC patients

BACKGROUND/AIMS: Hepatocellular carcinoma with portal venous invasion has a very poor prognosis. The aim of this study is to clarify the factors contributing to the survival of hepatocellular carcinoma patients with portal venous invasion. METHODOLOGY: Out of 952 patients with hepatocellular carcinoma admitted to Tokyo University hospital and its affiliated hospitals from 1987 to 1999, 53 patients developed portal venous invasion until December 2000. The main portal vein was invaded in 33 patients, and the first branch was invaded in the 20 patients. The factors contributing to the prognosis of hepatocellular carcinoma patients with portal venous invasion were determined by univariate and multivariate analyses using 19 clinicopathological parameters. RESULTS: Overall survival rates of the 53 patients at 6 months, and 1 and 2 years were 40%, 18%, and 12%, respectively. Univariate analysis indicated that the serum albumin level, Child classification, number of tumor foci, portal venous invasion-targeted irradiation, and percutaneous tumor ablation of the parenchymal main tumor were significant. Multivariate analysis showed that percutaneous tumor ablation (P = 0.033; risk ratio = 0.28) was the most important factor contributing to a favorable prognosis followed by number of tumor foci (P = 0.048; risk ratio = 0.41). CONCLUSIONS: This study showed the significance of treatment for the parenchymal main tumor in addition to portal venous invasion in patients with hepatocellular carcinoma involving portal venous invasion. Therefore, the efficacy of combined therapy using portal venous invasion-targeted irradiation and percutaneous tumor ablation for the parenchymal main tumor on survival of hepatocellular carcinoma patients with portal venous invasion is suggested.     

A novel 203 kD aberrant BCR-ABL product in a girl with Philadelphia chromosome positive acute lymphoblastic leultaemia

Summary We report a girl with Ph¹-positive ALL with the aberrant BCR-ABL product. In this case. bcr exon 3 jointed not to ordinal abl exon 2 but to exon 3 resulting in the production of a 20 3 kD BCR-ABL fusion protein with marked tyrosine kinase activity. To our knowledge, this is the first report of an aberrant BCR-ABL product in childhood. This case was characterized with younger age and low leucocyte count at the onset, but relapsed early like the typical Ph^l-positive ALL, suggesting the diversity in the clinicopathogenesis of Ph¹-positive ALL.     

Review of sick house syndrome

'Sick house syndrome' (SHS) is a health issue that closely resembles sick building syndrome (SBS) that had occurred in European countries. The aim of this review is to clarify the characteristics of SHS by reviewing previous reports rigorously. We propose the definition of SHS as "health impairments caused by indoor air pollution, regardless of the place, causative substance, or pathogenesis". Cases of SBS are reported to occur predominantly in offices and sometimes schools, whereas those of SHS are usually found in general dwellings. In many cases, SHS is caused by biologically and/or chemically polluted indoor air. Physical factors might affect the impairments of SHS in some cases. It is considered that symptoms of SHS develop through toxic, allergic and/or some unknown mechanisms. Psychological mechanisms might also affect the development of SHS. It is still unclear whether SBS and SHS are very close or identical clinical entities, mostly because a general agreement on a diagnostic standard for SHS has not been established. Previous research gradually clarified the etiology of SHS. Further advances in research, diagnosis, and treatment of SHS are warranted with the following measures. Firstly, a clinical diagnostic standard including both subjective and objective findings must be established. Secondly, a standard procedure for assessing indoor air contamination should be established. Lastly, as previous research indicated multiple causative factors for SHS, an interdisciplinary approach is needed to obtain the grand picture of the syndrome.     

Factors predisposing to poorly differentiated hepatocellular carcinoma and its recurrence

BACKGROUND/AIMS: We investigated the clinical factors predisposing moderately or poorly differentiated hepatocellular carcinoma and analyzed which clinical and histological factors are associated with poorly differentiated hepatocellular carcinoma (HCC) recurrence. METHODOLOGY: Percutaneous fine-needle biopsy was taken from the liver tumor of 191 consecutive patients between January 1994 and September 1996. The histological degree of differentiation of hepatocellular carcinoma at the first time of initial treatment and at the time of second recurrence was classified according to the criteria of Edmondson and Steiner. RESULTS: At the time of the first therapy, 86 patients, 81, 24, and 0 patients had liver tumors classified as Edmondson (Ed), 1, 2, 3, and 4, respectively. The prognosis of patients with Ed-3/4 HCC was worse than and the tumor sizes were larger than that of Ed-1/2 HCC patients. Of the 167 patients classified as Ed-1/2 at the time of first therapy, HCC recurred in 95 of the patients during the mean follow-up period of 3.4 years. Multivariate analysis revealed that only tumor size (P=0.035) and TACE therapy (P=0.0009) were independently significant factors in predicting future Ed-3/4 or multiple HCC recurrence. CONCLUSIONS: Tumor size and TACE therapy were clinical predisposing factors for Ed-3/4 or multiple HCC recurrences.     

Rhythm and amplitude of rhythmic masticatory muscle activity during sleep in bruxers – comparison with gum chewing

Objectives: The aim of this study was to elucidate characteristics of rhythmic masticatory muscle activity (RMMA) during sleep by comparing masseteric EMG (electromyogram) activities of RMMA with gum chewing.

Method: The parts of five or more consecutive phasic bursts in RMMA of 23 bruxers were analyzed. Wilcoxon signed-rank test for matched pairs and Spearman’s correlation coefficient by the rank test were used for statistical analysis.

Results: Root mean square value of RMMA phasic burst was smaller than that during gum chewing, but correlates to that of gum chewing. The cycle of RMMA was longer than that of gum chewing due to the longer burst duration of RMMA, and variation in the cycles of RMMA was wider.

Discussion: These findings suggest that the longer but smaller EMG burst in comparison with gum chewing is one of the characteristics of RMMA. The relation between size of RMMA phasic bursts and gum chewing is also suggested.     

Can malocclusion provide clinicians with information for differential diagnosis of temporomandibular joint diseases?: A review

The aim of this literature review was to summarize the clinical characteristics and symptoms of temporomandibular joint diseases, and to discuss the associations between temporomandibular joint diseases and categorization of malocclusion. Electronic literature searches were performed using the PubMed database. The authors established a differential diagnostic method for temporomandibular joint diseases related to malocclusion. A literature search using PubMed yielded 213 texts, of which based on exclusion criteria, 28 were included in this study. Malocclusions were categorized into 5 types. The authors suggested a diagnostic tree of temporomandibular joint diseases based on the types of malocclusion and 4 variables in clinical characteristics and symptoms. Clinicians treating malocclusions must attempt to clarify the cause of the occlusal condition. If caused by temporomandibular joint disease, it is important to make a proper differential diagnosis at first, and not to overlook the causative disease. Further clinical knowledge of associations between temporomandibular joint diseases and malocclusions should be accumulated, and the diagnostic tree should be improved based on new information.     

Combination therapy of intraarterial 5-fluorouracil and systemic interferon-alpha for advanced hepatocellular carcinoma with portal venous invasion

BACKGROUND: Hepatocellular carcinoma (HCC) with portal venous invasion (PVI) has a very poor prognosis, with a median survival of 3 months and virtually no survival at 1 year. The combination of intraarterial 5-fluorouracil (FU) and systemic interferon-alpha (IFNalpha) was recently reported to be effective against HCC with PVI, but these were small pilot studies. METHODS: One hundred and sixteen patients with HCC with PVI received IFNalpha (5,000,000 U intramuscularly on Days 1, 3, and 5 of each week of treatment) and 5-FU (500 mg into hepatic artery on Days 1-5 of the first and second week of each 4-week cycle). The therapy was either terminated at the end of the first cycle in cases with progressive disease, or continued for at least 3 cycles, when responses to treatment were evaluated by Eastern Cooperative Oncology Group criteria. The survival rate was compared with that of historical controls (n = 40). RESULTS: Nineteen (16%) patients showed complete response and another 42 (36%) showed partial response. Adverse events were limited to nausea and appetite loss. The survival rates at 12 and 24 months among overall patients were 34% and 18%, respectively, in contrast to 15% and 5% among the historical controls. Survival rates at 12 and 24 months were 81% and 59% among complete responders, respectively, and 43% and 18% among partial responders. CONCLUSION: The combination therapy with 5-FU and IFN was safe, and substantially improved the survival rate among the complete responders. These results provide a rationale for future randomized controlled trials.     

Improvement of image quality of time-domain diffuse optical tomography with l sparsity regularization

An l(p) (0 < p ≤ 1) sparsity regularization is applied to time-domain diffuse optical tomography with a gradient-based nonlinear optimization scheme to improve the spatial resolution and the robustness to noise. The expression of the l(p) sparsity regularization is reformulated as a differentiable function of a parameter to avoid the difficulty in calculating its gradient in the optimization process. The regularization parameter is selected by the L-curve method. Numerical experiments show that the l(p) sparsity regularization improves the spatial resolution and recovers the difference in the absorption coefficients between two targets, although a target with a small absorption coefficient may disappear due to the strong effect of the l(p) sparsity regularization when the value of p is too small. The l(p) sparsity regularization with small p values strongly localizes the target, and the reconstructed region of the target becomes smaller as the value of p decreases. A phantom experiment validates the numerical simulations.