Improvement of action myoclonus by an administration of 5-hydroxytryptophan and carbidopa in a child with muscular subsarcolemmal hyperactivity

We report a 3-year-11-month-old boy who manifested action myoclonus only. Histochemical analysis of the quadriceps muscle revealed subsarcolemmal hyperactivity. The administration of 5-hydroxytryptophan and carbidopa dramatically improved the action myoclonus and reduced an amplitude of giant somatosensory evoked potentials. A nosological relation of this case with "essential myoclonus" and mitochondrial encephalomyopathy was discussed.     

Evaluation of conventional weaning criteria in patients with acute respiratory failure

We evaluated the reliability of conventional weaning criteria from a ventilator during 33 weaning trials on 25 patients with acute respiratory failure (ARF). Of 13 criteria, a ratio of maximal voluntary ventilation to minute ventilation (MV) 2, a vital capacity 12ml·kg^–1, a spontaneous respiratory rate 25 breaths·min^–1, and a MV 10l·min^–1 appeared to be useful for predicting successful weaning outcome. However, even using those criteria, there were many falsely-negative cases. The alveolar-arterial P_{O 2} gradient 350mmHg at an Fi _{O 2} 1.0 was not useful as a predictor of weaning outcome. The present study demonstrates that conventional criteria are frequently inaccurate for predicting weaning outcomes and suggests that the use of some of these criteria may unnecessarily prolong the length of ventilator support. Since ventilation of most patients with poor oxygenation can be successfully discontinued by placing them on a continuous positive airway pressure system, these results suggest that the improvement of oxygenation is not an indispensable prerequisite for weaning from mechanical ventilators.(Okamoto K, Iwamasa H, Dogomori H, et al.: Evaluation of conventional weaning criteria in patients with acute respiratory failure. J Anesth 4: 213–218, 1990)     

Therapeutic effect of a CDDP-epirubicin-Lipiodol emulsion on advanced hepatocellular carcinoma

Prior injection of an anticancer agent and Lipiodol mixture is a key point for the treatment of hepatocellular carcinoma (HCC). We therefore prepared a new, improved emulsion of Lipiodol containing a high dose ofcis-diamminedichloroplatinum (CDDP) and epirubicin by replacing the ionic contrast medium (Urografin 67) with a nonionic contrast medium (Iopamidol; Iopamiron 300) and adding phosphatidyl choline. This CDDP-epirubicin-Lipiodol emulsion (CELE) was examined pharmacologically and chemically with the following results. The size of these particles is less than 10 m (diameter) for up to 24 h; the release of 28%–34% of the CDDP and 80%–90% of the epirubicin was estimated in the dissolution test, and 85% of the CDDP and 35% of the epirubicin was retained in the organs in the moment calculation. CELE was injected into 58 HCC patients via a celiac angiographic catheter. In 36 of these patients, the CELE injection was followed by transcatheter arterial embolization (TAE) therapy. Following the administration of CELE as one-shot injection therapy for stage IV HCC, the 1-year survival rate was 59% and the 2-year survival rate was 27%. Moreover, in patients (stage II, 12; stage III, 8; stage IV, 16) who received CELE and subsequently underwent TAE therapy, the 1-year survival rate was 90% and the 2-year survival rate was 67%. The nonionic contrast medium with Lipiodol forms finer emulsified particles, and these particles are more capable of penetrating into the tumor. In addition, the greater pharmacological stability of these particles provides a slow-release effect and prolonged stability of their shape. Finally, theoretically, the use of two major anticancer agents such as CDDP and epirubicin showed a greater clinical effect in the treatment of HCC than either our earlier suspension or a single anticancer agent.Work presented at the Third International Symposium on Treatment of Liver Cancer, Seoul, Korea, 12–13 February 1993     

Recurrent breast cancer at 21 years after resection detected by serum tumor markers and manifested as dermatomyositis

A 52-year-old Japanese woman developed dermatomyositis. She had undergone a standard radical mastectomy for left breast cancer 21 years earlier. Though no physical sign of recurrent breast cancer appeared clinically, levels of tumor markers were abnormally elevated. Therefore, tamoxifen and CAF therapy were given. Further, the clinical course of dermatomyositis almost paralleled the level of serum tumor markers and the clinical course of her recurrent breast cancer. These markers were useful for detecting the recurrence, following the metastatic disease, and monitoring her response to therapy.     

Ocular findings in Japanese women with nevus of Ota

Background: Nevus of Ota is common in Japanese women, but most patients are not examined ophthalmologically. Methods: We performed ophthalmologic examinations on 16 Japanese women who had had bluish pigmentation in the periorbital region, sclera, and conjunctiva since birth. Results: Fifteen patients had unilateral involvement, and one had bilateral lesions. The visual acuities were good, and the intraocular pressures were within normal range. All patients had a negative family history. Three patients had light pigmentation in the optic disc in the affected eye. Conclusion: We believe that optic disc pigmentation associated with nevus of Ota, as found in these three patients, may be common but have been rarely described.     

A patient of successful renal transplantation using donor kidney with congenital abnormalities of the inferior vena cava

At the time of donor nephrectomy, congenital abnormalities of the inferior vena cava was discovered. A 56-year-old woman was worked up preoperatively and no abnormalities were found. Abdominal aortography was normal. At the time of operation, the inferior vena cava was located to the left and anterior to the abdominal aorta. In the renal hilus, the location of the renal artery and vein was reversal. We thought the ideas of operation and could successfully performed renal transplantation using donor kidney with congenital abnormalities of the inferior vena cava.     

Tissue inhibitors of metalloproteinases (TIMP-1 and TIMP-2) stimulate osteoclastic bone resorption

As both tissue inhibitor of metalloproteinases-1 (TIMP-1) and TIMP-2 have been reported to inhibit bone resorption, we examined whether TIMP-1 or TIMP-2 in fetal calf serum (FCS), with which culture media were supplemented, affected osteoclastic bone resorption in vitro. Contrary to our expectation, almost complete suppression of osteoclastic bone resorption was observed when both TIMP-1 and TIMP-2 were removed from the FCS. Bone resorption was, however, almost fully restored by the addition of recombinant TIMPs. TIMPs stimulate bone resorption at significantly lower concentrations (∼ng/ml) than those (∼μg/ml) required to inhibit bone resorption. To understand the mechanism of TIMP-dependent bone resorption, we counted and compared the number of tartrate-resistant acid phosphatase-(TRAP-) positive and multinuclear cells in cultures containing either 10% FCS or TIMP-1-free and/or TIMP-2-free FCS. There was essentially no difference in number among these, suggesting that the TIMP role seems to be related to the functional expression of osteoclasts. Metallo-proteinase inhibitors, either BE16627B[l-N-(N-hydroxy-2-isobutylsuccinynamoyl)-seryl-l-valine] or R94138 {N-methyl-(3S)-2-[(2R)-2-hydroxycarbamoylmethylundecanoyl] hexahydropyridazine-3-carboxamide}, could not replace TIMPs, suggesting that the osteoclast-stimulating activity of TIMPs cannot be ascribed to merely their inhibitory effect on matrix metalloproteinases. Received: Oct. 15, 1998 / Accepted: April 5, 1999     

Significance of platelet-derived microparticles and activated platelets in diabetic nephropathy

We measured levels of platelet-derived microparticles (PMP), which have coagulative activity and are produced by platelet activation or physical stimulation, and CD62P/CD63-positive platelets in patients with diabetes mellitus to determine their clinical significance and effects on complications of diabetes including diabetic nephropathy. We also compared these levels before and after administration of the antiplatelet drug cilostazol. Plasma PMP and CD62P/CD63-positive platelet levels were significantly higher in patients with diabetes mellitus than normal controls. CD62P-positive platelet levels were significantly higher in patients with nephropathy than in patients without complications. After administration of cilostazol, PMP and CD62P/CD63-positive platelet levels were significantly decreased. The increases in platelet activity and its related procoagulant activity appear to account in part for the hypercoagulability observed in diabetes mellitus. Our findings suggest that activated platelets might play a role in the development of diabetic nephropathy. Furthermore, antiplatelet therapy with cilostazol for diabetic patients may be useful as antithrombin therapy including antiplatelet therapy, since it suppresses the production of intrinsic coagulants produced by platelet activation.     

Total Esophagectomy versus Proximal Esophagectomy for Esophageal Cancer at the Cervicothoracic Junction

To investigate the adequate extent of esophagectomy and lymphadenectomy for an esophageal cancer localized at the cervicothoracic junction, the mortality and morbidity rates, survival rates, and patterns of recurrence were retrospectively analyzed in two groups—14 patients who underwent total esophagectomy with or without laryngectomy and 15 patients who underwent proximal esophagectomy with or without laryngectomy—at Kurume University Hospital from 1981 to 1996. Proximal esophagectomy with or without laryngectomy resulted in a lower hospital mortality rate and better overall survival for patients who underwent curative esophagectomy compared with total esophagectomy with or without laryngectomy. Multivariate analysis indicated that the extent of esophagectomy (total esophagectomy versus proximal esophagectomy) was not a prognostic factor. The incidence of recurrence was not different between the two groups. Lymph node metastasis or recurrence from such esophageal cancers was localized to the neck and upper mediastinum. For an esophageal cancer localized at the cervicothoracic junction, therefore, proximal esophagectomy with or without laryngectomy and with cervical and upper mediastinal lymphadenectomy could be better indicated for preselected patients.     

Evaluation of the pressure transfer system in the intracranial cavity by coherency

Coherency provides a method to evaluate model linearity. The characteristics of pressure wave transmission in the intracranial cavity were studied by coherency in 16 cats with hydrostatic pressure loading to assess the linearity of the system, which is an assumption for use of the transfer function. Linearity was observed in only the fundamental waves of the respiration-induced component and the cardiac-induced component of intracranial pressure oscillation, and in the second harmonic wave of the latter. Linearity at the other frequencies was close to zero. The pressure transfer system in the intracranial cavity was basically a non-linear system. As intracranial pressure rose, the increase in the pressure transfer efficiency was largest in the low-frequency domain and smallest in the high-frequency domain, indicating that the cerebral blood vessels are characterized by inferior transmission of high frequency due to increased intracranial pressure. In addition, the correlation between the coherencies of the cardiac-induced fundamental wave component and intracranial pressure, and between those of the cardiac-induced second harmonic wave component and intracranial pressure, showed that the slope of the straight line was greater between 45 and 70 mmHg than between 10 and 45 mmHg. This suggests that there is a break point, located between 45 and 70 mmHg, where the increase in the coherency values is accelerated, caused by an increase in the intracranial elastance, as well as an increase in the cerebrovascular compliance due to the reduced vascular transmural pressure.