Isaacs' syndrome associated with myasthenia gravis, showing remission after cytoreductive surgery of pleural recurrence of thymoma

We report a patient with Isaacs' syndrome associated with myasthenia gravis and pleural recurrence of thymoma, who showed severe limb pain attributed to hyperexcitability of sensory nerves. Myokymia and severe pain were successfully treated with cytoreductive surgery and intraoperative hyperthermic intrathoracic perfusion chemotherapy, but neither pharmacotherapy nor plasma exchange showed obvious clinical effects. Pleural thymoma in our patient may have caused Isaacs' syndrome, probably by unconfirmed humoral immune mechanisms. Cytoreductive treatment for recurrent thymoma should be actively considered as a potent therapeutic option in refractory patients with disabling neuromyotonia symptoms.     

Effect of low-intensity pulsed ultrasound stimulation on callus remodelling in a gap-healing model: evaluation by bone morphometry using three-dimensional quantitative micro-CT

We evaluated the effect of low-intensity pulsed ultrasound stimulation (LIPUS) on the remodelling of callus in a rabbit gap-healing model by bone morphometric analyses using three-dimensional quantitative micro-CT. A tibial osteotomy with a 2 mm gap was immobilised by rigid external fixation and LIPUS was applied using active translucent devices. A control group had sham inactive transducers applied. A region of interest of micro-CT was set at the centre of the osteotomy gap with a width of 1 mm. The morphometric parameters used for evaluation were the volume of mineralised callus (BV) and the volumetric bone mineral density of mineralised tissue (mBMD). The whole region of interest was measured and subdivided into three zones as follows: the periosteal callus zone (external), the medullary callus zone (endosteal) and the cortical gap zone (intercortical). The BV and mBMD were measured for each zone. In the endosteal area, there was a significant increase in the density of newly formed callus which was subsequently diminished by bone resorption that overwhelmed bone formation in this area as the intramedullary canal was restored. In the intercortical area, LIPUS was considered to enhance bone formation throughout the period of observation. These findings indicate that LIPUS could shorten the time required for remodelling and enhance the mineralisation of callus.     

Central nervous system primitive neuroectodermal tumor of spinal cord developing 20 years after curative treatment of pineal tumor

A 65-year-old male who had previously received curative treatment for a pineal tumor presented with an extremely rare case of primary central nervous system (CNS) primitive neuroectodermal tumor (PNET) of the spinal cord manifesting as progressive tetraparesis. Although the histology was not verified, highly radiosensitive tumor was suspected because of the benign clinical course for over 20 years after only radiation therapy. Magnetic resonance imaging demonstrated an intramedullary tumor extending from C5 to T1. He underwent partial resection and histological examination revealed blue tumor with undifferentiated small round cells. Immunohistochemically, c-kit was negative but CD99 was strongly and diffusely positive. Therefore, rearrangement of the Ewing sarcoma gene was examined to determine the presence of peripheral type of PNET. The results were negative and systemic workup revealed no other disease. These findings led to the diagnosis of primary intramedullary CNS PNET of the spinal cord, and suggested that the spinal cord tumor occurred independently of the prior pineal disease. The residual tumor was controlled by postoperative local radiation therapy.     

Optimum initial dose of silodosin for treatment of lower urinary tract symptoms associated with benign prostatic hyperplasia

We investigated the optimum initial dose and timing of administration of α1A-adrenoceptor antagonist silodosin for treatment of lower urinary tract symptoms associated with benign prostatic hyperplasia (BPH/LUTS). Ninety-eight patients were given a 4 mg dose after breakfast (group A), 4 mg after supper (group B), or 4 mg after breakfast and after supper (group C). At baseline, 4, 8 and 12 weeks after treatment, we assessed International Prostate Symptom Score (IPSS) and quality of life (QOL) index. Twenty-five percent or less improvement of total IPSS and no improvement of QOL index compared with baseline were defined as treatment failure at each evaluation point. Otherwise treatment was considered effective. In group A and group B, patients with treatment failure at 4 or 8 weeks after treatment, the dose of silodosin was increased to 8 mg daily. At the end of the study, 83 patients were evaluable. At 12 weeks after treatment, 20 of the 31 patients in group A and 22 of the 29 patients in group B remained on the 4 mg dose ; silodosin was effective in 65 and 76% of the patients, respectively. When patients with dose escalation were included, silodosin was effective in 81 and 90% of the patients, respectively. Silodosin was effective in 18 of the 23(78%) patients in group C, although improvement of total IPSS and voiding symptom score of IPSS at 12 weeks after treatment was better in group C than in group A or group B, the difference was not significant. In patients with IPSS less than 20, the degree of improvement of IPSS was similar among the 3 groups. In contrast, in patients with IPSS of 20 or greater the degree of improvement was better in group C than in group B or group C, but the difference was not significant. Storage symptom score of IPSS was significantly improved in all 3 groups without any significant difference among the 3 groups. Three patients (52, 59 and 76 years old) experienced abnormal ejaculation. In conclusion, 4 mg of silodosin daily showed effectiveness against BPH/LUTS, but 8 mg of silodosin daily might be better for patients with severe LUTS.     

Lung carcinoma producing carbohydrate antigen 19-9; report of a case

A 58-year-old female was admitted to our hospital for investigation of serum elevation of carbohydrate antigen (CA 19-9). Computed tomography of the chest revealed a spiculated pulmonary nodule with the longest diameter of 3.7 cm in the right lower lobe. The diagnosis of lung adenocarcinoma was made. The patient underwent right lower lobectomy with lymphnode dissection. Histological examination revealed acinar type adenocarcinoma. The tumor was classified as stage IB with T2aN0M0. Immunohistochemically, the tumor cells stained positively for CA19-9. The serum CA19-9 level returned to a normal level after operation, but increased again with mediastinal lymphnode metastasis and brain metastasis. She died after an operation in 16 months.     

Enzymatic synthesis of no-carrier-added 6-[18F]fluoro-L-dopa with beta-tyrosinase.

An enzymatic synthesis of nca 6-[18F]fluoro-L-dopa has been developed. The process consists of a chemical synthesis of 4-[18F]fluorocatechol and its enzymatic reaction with beta-tyrosinase. The 4-[18F]fluorocatechol was prepared by nucleophilic aromatic substitution of the NO2 group on 6-nitroveratoraldehyde with [K/222]+18F-, followed by decarbonylation with tris(triphenylphosphine) rhodium(I) chloride and hydrolysis with hydroiodic acid. By C18 Sep-Pak purification, 4-[18F]fluorocatechol was obtained in ethanol with a radiochemical yield of 9.2%. An enzymatic reaction of 4-[18F]fluorocatechol, ammonium and pyruvate catalyzed by beta-tyrosinase in an ethanolic Tris-HCl buffer (pH 9.0) containing ascorbate gave within 5 min 6-[18F]fluoro-L-dopa with an approximate radiochemical yield of 60% without any isomers. The deproteinized reaction mixture was applied to a preparative reverse phase column, and the radiochemically and enantiomerically pure 6-[18F]fluoro-L-dopa was obtained with a radiochemical yield of 2.0% based on [18F]F- (decay-corrected). The synthesis time was 150 min from the EOB and the specific activity was > 200 GBq/micromol.     

Neutralization of blood group A-antigen by a novel anti-A antibody: overcoming ABO-incompatible solid-organ transplantation

BACKGROUND: The major barrier to ABO-incompatible solid-organ transplantation is acute humoral rejection. It is known to be triggered by antidonor blood group A/B antibodies, which might bind to A/B-antigen on the endothelium of the graft. Various strategies to reduce antiblood group antibody by overcoming ABO-incompatible transplantation have been tried. However, antigen-suppressing procedures have not been performed. METHODS: We produced a novel anti-A antibody (K7508) by immunizing mice with salivary mucin of a blood group A individual, thereby clarifying that blood group A-antigen is expressed in endothelial cells of the liver. We investigated whether K7508 can mask A-antigen on the cells in vitro. Next, we immunized mice with A-antigen-expressing cells coated with K7508 or its Fab fragment, and measured anti-A antibody production in the mice. RESULTS: Blood group A-antigen-expressing cells, such as blood group A-red blood cells (A-RBCs) and A431 cells, coated with K7508 were not recognized by another anti-A antibody in flow cytometry, indicating that A-antigen was masked by K7508 in vitro. The A-antigen on the paraffin-embedded liver tissue was also masked by K7508. Furthermore, the production of anti-A antibody in mice immunized with A-antigen-expressing cells coated with K7508 or its Fab fragment was significantly suppressed compared to that in mice immunized with non-coated cells alone, indicating that A-antigen was neutralized by K7508 in vivo. CONCLUSIONS: The neutralization of blood group antigen by antiblood group antibody and especially its Fab fragment might represent one strategy to overcome ABO-incompatible organ transplantation.     

Biliary complications in right lateral sector graft live donor liver transplantation.

Biliary complications remain the most challenging issue in adult living donor liver transplantation (LDLT) and to the best of our knowledge, no study has focused on the biliary complications in LDLT with right lateral sector graft (RLSG), a graft consisting of segments VI and VII according to Couinaud's nomenclature for liver segmentation. Between January 1996 and October 2006, 310 LDLTs were performed for adult recipients at our institution. Among them, 20 patients received RLSG. The incidence of biliary complications during follow-up in these patients with RLSG was retrospectively analyzed. Follow-up period after transplantation ranged from 1 to 87 months (median 58 months). The 3-year and 5-year graft survival rates following the use of RLSGs in LDLT were 90% and 90%, respectively. Biliary complications were encountered in altogether nine patients. Two patients (10%) were complicated with bile leakage requiring surgical intervention. Seven patients (35%) were complicated with bile duct stenosis, which occurred with a median interval of 26 months (range: 6-51 months) after LDLT. Four were treated surgically and the other three were treated by endoscopic approach. Outcomes of the interventions were satisfactory in all cases. The incidence and severity of biliary complications after LDLT using RLSG was within an acceptable range with excellent graft survival. Accordingly, it is concluded that RLSG is a technically feasible option that may effectively expand the donor pool. Further application of RLSG is warranted.     

Acceptable definition of peripheral early lung cancer

This study was conducted to evaluate the outcome of 88 patients who underwent surgical resection for peripheral non-small cell lung cancers less than 20 mm in diameter. Twenty-one cases with lesions smaller than 10 mm had no lymph node metastasis, intrapulmonary metastasis, pleural dissemination, or distant metastasis. The 5-year survival rate of them was 100%. However, 67 patients with tumors larger than 10 mm showed lymph node metastasis in 14 cases, intrapulmonary metastasis in 3, pleural dissemination in 2 and distant metastasis in 1. And the 5-year survival rates of patients with tumor dimensions of < or = 15 mm and < or = 20 mm were 77.9% and 74.4%, respectively. In addition, patients having adenocarcinoma categorized A and B by Noguchi's classification had no lymph node metastasis, intrapulmonary metastasis, pleural dissemination, or distant metastasis, and showed 100% of 5-year survival rate. To be defined as early cancers in terms of curability, it is thought that the 5-year survival rate of the patients with them is over 95%. Therefore, these results suggest that tumors smaller than 10 mm or adenocarcinoma less than 20 mm in diameter diagnosed as Noguchi's A and B are considered as peripheral early lung cancers.