Sarcoid myositis with muscle weakness as a presenting symptom

A 54-year-old woman complaining of muscle weakness and weight loss was admitted to our hospital with suspected polymyositis. Muscle biopsy revealed Langhans-type giant cells and noncaseating granulomas. Therefore, sarcoid myositis was diagnosed. The patient was treated with prednisolone, and the symptoms improved gradually. Generally, sarcoidosis is identified clinically in patients with foggy vision or mediastinal lymphadenopathy, but muscular weakness may be an infrequently observed initial symptom. Sarcoidosis should be considered in the differential diagnosis of polymyositis.     

Comparative study of anastomosis in pancreaticogastrostomy and pancreaticojejunostomy after pancreaticoduodenectomy

BACKGROUND/AIMS: The relative advantages of pancreaticojejunostomy and pancreaticogastrostomy after pancreaticoduodenectomy remain to be established. To clarify differences between the two procedures, we examined 66 patients who underwent PD and compared clinical and histological parameters, surgical records and patient outcomes. METHODOLOGY: In this retrospective study, subjects were divided into a pancreaticojejunostomy group (n=48) and a pancreaticogastrostomy group (n=18). Pancreaticogastrostomy and pancreaticojejunostomy were performed using invagination technique and end-to-side anastomosis, respectively. RESULTS: There were no significant differences in patient demographics and surgical records between the two groups. Increases in serum amylase concentrations after operation were significantly greater in the pancreaticogastrostomy group (902 +/- 915 IU/L) than in the pancreaticojejunostomy group (326 +/- 761 IU/L) (p = 0.025). However, there were no significant differences in other postoperative laboratory data or complications between the two groups. CONCLUSIONS: In our study, the clinical and safety data associated with pancreaticojejunostomy and pancreaticogastrostomy procedures for anastomosis after pancreaticoduodenectomy were almost similar. Therefore, the anastomotic procedure used should depend on the surgeon's choice or the distance between the remnant stomach and pancreas.     

Silencing Id-1 inhibits lymphangiogenesis through down-regulation of VEGF-C in oral squamous cell carcinoma

Our previous study demonstrated that overexpression of Id-1 (inhibitor of differentiation/DNA binding) was associated with lymphatic metastasis in human oral squamous cell carcinoma (OSCC). In this study, we further unveiled the association of Id-1 with vascular endothelial growth factor-C (VEGF-C) and peritumoral lymphatic vessel density (PLVD), and the effect of silencing Id-1 on inhibiting lymphangiogenesis in OSCC. We found that Id-1 was associated with VEGF-C (r=0.569, p<0.001) and PLVD (r=0.240, p<0.001) in OSCC. Lentivirus-mediated RNA interference targeting Id-1 in an OSCC cell line Tca8113 resulted in down-regulation of VEGF-C (p=0.003, 0.007). Moreover, when Id-1 was suppressed by injecting Id-1-siRNA-lentivirus into the transplanted tumors in nude mice, VEGF-C was down-regulated (p=0.018) and the PLVD decreased (p=0.001). Our results suggest that Id-1 was correlated with lymphangiogenesis in OSCC. Silencing Id-1 could inhibit lymphangiogenesis through down-regulation of VEGF-C and it might be a promising treatment modality for the lymphatic metastasis of OSCC.     

Long-term ventilation for high-level tetraplegia: a report of 2 cases of noninvasive positive-pressure ventilation

Toki A, Tamura R, Sumida M. Long-term ventilation for high-level tetraplegia: a report of 2 cases of noninvasive positive-pressure ventilation.Ventilator-dependent patients with tetraplegia rarely use noninvasive positive-pressure ventilation (NPPV) for long-term ventilation. We report 2 patients with high-level traumatic tetraplegia who were able to return home after being changed from traditional ventilation to NPPV. When they were referred to our hospital from acute care hospitals 2 to 6 months after injury, both were on tracheostomy ventilation with a cuff inflated 24 hours a day, and tidal volume (Vt) settings were low. In case 1, a man with complete C1 tetraplegia was admitted to our hospital 6 months after injury. We changed ventilator settings to high Vt and introduced NPPV. He was discharged home with NPPV with a volume-setting ventilator. Case 2 involved a man in his late twenties with complete C1 tetraplegia who was discharged home with NPPV. After discharge, he trained in glossopharyngeal breathing by himself, enabling him to breathe up to 1900mL of maximum insufflation capacity. Both have lived nearly 1 year without pulmonary complications in the community. They use visiting nurses 3 times a week and services of visiting caregivers. Further study is needed to determine the usefulness of NPPV for long-term ventilatory management.     

Development and Clinical Application of an Enzyme-Linked Immunosorbent Assay for Oxidized High-Density Lipoprotein

Aims: HDL particles have various anti-atherogenic functions, whereas HDL from atherosclerotic patients was demonstrated to be dysfunctional. One possible mechanism for the formation of dysfunctional HDL is the oxidation of its components. However, oxidized HDLs (Ox-HDLs) remain to be well investigated due to lack of reliable assay systems. Methods: We have developed a novel sandwich enzyme-linked immunosorbent assay (ELISA) for Ox-HDL by using the FOH1a/DLH3 antibody, which can specifically recognize oxidized phosphatidylcholine, a major component of HDL phospholipid (HDL-PL). We defined forced oxidation of 1 mg/L HDL-PL as 1 U/L Ox-HDL. We assessed serum Ox-HDL levels of normolipidemic healthy subjects ( n =94) and dyslipidemic patients ( n =177). Results: The coefficients of variation of within-run and between-run assays were 12.5% and 13.5%. In healthy subjects, serum Ox-HDL levels were 28.5±5.0 (mean±SD) U/L. As Ox-HDL levels were moderately correlated with HDL-PL (r=0.59), we also evaluated the Ox-HDL/HDL-PL ratio, which represents the proportion of oxidized phospholipids in HDL particles. In dyslipidemic patients, Ox-HDL levels were highly variable and ranged from 7.2 to 62.1U/L, and were extremely high (50.4±13.3U/L) especially in patients with hyperalphalipoproteinemia due to cholesteryl ester transfer protein deficiency. Regarding patients with familial hypercholesterolemia, those treated with probucol, which is a potent anti-oxidative and anti-hyperlipidemic drug, showed significantly lower Ox-HDL (16.2±5.8 vs. 30.2±5.4, p ＜0.001) and Ox-HDL/HDL-PL ratios (0.200±0.035 vs. 0.229±0.031, p =0.015) than those without probucol. Conclusion: We have established a novel sandwich ELISA for Ox-HDL, which might be a useful and easy strategy to evaluate HDL functionality, although the comparison study between this Ox-HDL ELISA and the assay of HDL cholesterol efflux capacity remains to be done. Our results indicated that probucol treatment may be associated with lower Ox-HDL levels.     

Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists,neurologists and dermatologists

Although rheumatologists, neurologists and dermatologists see patients with polymyositis (PM) and dermatomyositis (DM), their management appears to vary depending on the physician's specialty. The aim of the present study was to establish the treatment consensus among specialists of the three fields to standardize the patient care. We formed a research team supported by a grant from the Ministry of Health, Labor and Welfare, Japan. Clinical questions (CQ) on the management of PM and DM were raised. A published work search on CQ was performed primarily using PubMed. Using the nominal group technique, qualified studies and results in the published work were evaluated and discussed to reach consensus recommendations. They were sent out to the Japan College of Rheumatology, Japanese Society of Neurology and Japanese Dermatological Association for their approval. We reached a consensus in 23 CQ and made recommendations and a decision tree for management was proposed. They were officially approved by the three scientific societies. In conclusion, a multidisciplinary treatment consensus for the management of PM and DM was established for the first time.     

Rituximab therapy is more effective than cyclophosphamide therapy for Japanese patients with anti‐topoisomerase I‐positive systemic sclerosis‐associated interstitial lung disease

Systemic sclerosis‐associated interstitial lung disease (SSc‐ILD) is the most frequent cause of death for SSc but there is still no sufficient treatment available. Although cyclophosphamide (CYC) therapy is a common treatment which has shown statistical efficacy against SSc‐ILD to date, its effects are temporary and not enough. Rituximab (RTX), the anti‐CD20 monoclonal antibody, has recently shown efficacy in many autoimmune diseases. In SSc‐ILD, RTX is also considered to be one of the novel treatment candidates. However, studies of SSc‐ILD in Japanese treated with RTX have only a few case reports. Therefore, in this study, we retrospectively compared nine patients treated with RTX and 30 patients treated with CYC to investigate the efficacy of RTX treatment for Japanese anti‐topoisomerase I‐positive SSc‐ILD patients. At the 24‐month evaluation, the improvement rates of percent predicted of forced vital capacity and percent predicted of diffusing capacity of the lung carbon monoxide in the RTX‐treated group were significantly higher than those in the CYC‐treated group (20.6 ± 8.8% vs 1.1 ± 3.9%; P < 0.05 and 34.0 ± 6.0% vs ?1.5 ± 2.8%; P < 0.01, respectively). In addition, skin thickness scores also showed a marked improvement from 13.5 points before the start of treatment to 5.8 points after 24 months by RTX therapy (P < 0.05). These results suggest that RTX treatment is more effective for Japanese SSc‐ILD patients than CYC treatment. In the future, it is expected that large‐scale clinical trials will show the usefulness of RTX treatment for SSc‐ILD.     

A case of submandibular malignant rhabdoid tumor transformed from papillary thyroid carcinoma

Malignant rhabdoid tumor (MRT) in the neck region is very rare. We report a case of MRT in a 60-year-old woman who had a history of papillary carcinoma of the thyroid gland 7 years previously. One year before admission, in 1995, thyroid carcinoma recurred, and the tumor contained a small undifferentiated region with rhabdoid features. The tumor in 1996 consisted of round to oval rhabdoid cells with abundant cytoplasm, and the growth pattern was diffuse and infiltrative, with no papillary structures. We therefore concluded that the lesion was MRT, transformed from papillary thyroid carcinoma.