Multi-center analysis of calcinosis in children with juvenile dermatomyositis]

OBJECTIVES: To reveal the frequency and the clinical characteristics of dystrophic calcification that occurs in children with juvenile dermatomyositis, multi-center analysis was constructed. METHOD: Fifty children with JDM were enrolled, and 14 of them (28.0%) were complicated with calcinosis. Clinical symptoms and laboratory tests at onset, initial therapy and disease course were compared in children with and without calcinosis. RESULTS: The mean age of the onset of calcinosis was 4.78 +/- 3.33 years, and it was younger than those of children without calcinosis (8.66 +/- 3.85 years) (P = 0.0017). No differences of clinical manifestation except Gower's sign were observed. The frequency of positive anti-nuclear antibody was 7.1% in children with calcinosis and 52.9% without calcinosis (P = 0.0112). The initial therapy of methylprednisolon pulses gave no effects on prognosis of calcium deposition. The calcinosis appeared in 1.56 +/- 1.91 year after the onset of the disease. The various types of calcium deposition including large tumorous clumps, subcutaneous plaques or nodules, sheet-type calcification were deserved. They appeared over knee joints (64.3%), elbow joint (64.3%), and hip processes (50.0%). Calcinosis affecting the subcutaneous tissues frequently resulted in painful superficial ulceration of the overlying skin (42.9%), local infection (50.0%), and limitation of joint movement (14.3%). Although aluminum phosphate was effective in 2 children among 7, no other effective treatment was recommended. In 5 cases, surgical removal of tumorous clumps was operated. Thus, juvenile dermatomyositis is frequently complicated with calcinosis. This type of calcinosis was found to be unlikely to resolve completely, and resulted in severe disability in children.     

Invasion of the intracranial space by a malignant odontogenic mixed tumor: Report of a case

A case of an odontogenic tumor which invaded the intracranial space from the mandible is reported. Judging from the radiographic images it was similar to a malignant tumor. The patient died 17 years after the first visit. According to the final pathological diagnosis, it was malignant odontogenic mixed tumor of low grade which did not belong to any of the WHO classification.     

Venous hemangioma of the mesoappendix: Report of a case and a brief review of the Japanese literature

We present herein the rare case of a 48-year-old man in whom an abdominal mass, revealed by celiotomy to be a solid tumor of the mesoappendix, was histologically diagnosed as having a venous hemangioma. To our knowledge, only 18 cases of mesenteric hemangioma have been reported in Japan, including the present case. However, establishing a correct diagnosis preoperatively is extremely difficult despite advanced imaging techniques. In fact, a mesenteric mass was diagnosed preoperatively in only 3 of these 18 cases. Complete excision with or without bowel resection was performed in 16 cases. Interestingly, the histological diagnosis of all the previous cases was cavernous hemangioma, confirming that this report decuments the first case of venous hemangioma of the mesentery in the Japanese literature.     

Interstitial hyperthermia of malignant brain tumors using implant heating system (IHS)

We have developed an implant heating system (IHS) for interstitial hyperthermia of brain tumors. IHS consists of three compartments: ferromagnetic implant with low Curie point, induction coil and generator to produce high frequency magnetic field. The device works as follows: It is heated up to a Curie temperature (Tc) by Eddy current under the magnetic field. Heat generated in the implant is conducted to the tumor tissue into which it has been implanted. To evaluate the effect of this hyperthermia, a brain tumor model was produced by innoculation of VX2 tumor cells and treated either by hyperthermia with IHS alone, chemotherapy with ACNU alone, or with a combination of both. The longest survival was obtained by the combined treatment, and significant prolongation of survival was found in the single treatment groups. In the Phase I clinical trial, one or several implants (1.8 mm X 15 mm, Tc = 68 degrees C) made of Fe-Pt alloy were placed in the tumor by CT guided stereotactic procedure, or manually during craniotomy. Hyperthermia of above 42 degrees C for 30 to 60 minutes twice a week was brought about in ten cases of malignant brain tumor. CT evaluation was made in nine cases treated for more than ten times in this way. Five out of the nine cases responded to this hyperthermia with irradiation. In conclusion, a safe, repeated and longterm treatment was possible without significant side effects. The hyperthermia with IHS may also be applicable to benign intracranial tumors and neoplasms in other part of body as well.     

Augmentation of intrarenal angiotensin II levels in uninephrectomized aldosterone/salt-treated hypertensive rats; renoprotective effects of an ultrahigh dose of olmesartan.

Recent studies have suggested that aldosterone plays a role in the pathogenesis of renal injury. In this study, we investigated whether local angiotensin II (Ang II) activity contributes to the progression of renal injury in aldosterone/salt-induced hypertensive rats. Uninephrectomized rats were treated with 1% NaCl in a drinking solution and one of the following combinations for 6 weeks: vehicle (2% ethanol, s.c.; n=9), aldosterone (0.75 mug/h, s.c.; n=8), aldosterone+Ang II type 1 receptor blocker olmesartan (10 mg/kg/day, p.o.; n=8), or aldosterone+olmesartan (100 mg/kg/day, p.o.; n=9). Aldosterone/salt-treated hypertensive rats exhibited severe proteinuria and renal injury characterized by glomerular sclerosis and tubulointerstitial fibrosis. Aldosterone/salt-induced renal injury was associated with augmented expression of angiotensin converting enzyme and Ang II levels in the renal cortex and medullary tissues. Renal cortical and medullary mRNA expression of transforming growth factor-beta (TGF-beta) and connective tissue growth factor (CTGF) as well as the collagen contents were increased in aldosterone/salt-treated hypertensive rats. Treatment with olmesartan (10 or 100 mg/kg/day) had no effect on blood pressure but attenuated proteinuria in a dose-dependent manner. Olmesartan at 10 mg/kg/day tended to decrease renal cortical and medullary Ang II levels, TGF-beta and CTGF expression, and collagen contents; however, these changes were not significant. On the other hand, an ultrahigh dose of olmesartan (100 mg/kg/day) significantly decreased these values and ameliorated renal injury. These data suggest that augmented local Ang II activity contributes, at least partially, to the progression of aldosterone/salt-dependent renal injury.     

Intracellular free magnesium of red blood cells in patients with renal disease.

While serum magnesium (Mg) level is increased in patients with end-stage renal disease (ESRD), it is decreased in renal transplant recipients (TR) receiving ciclosporin. This study was performed to examine the cation metabolism of red blood cells (RBC) in these patients. Intracellular free Mg was measured with 31P-nuclear magnetic resonance spectrometry, and ouabain-sensitive sodium (Na) efflux rate (Eos) was measured from the increase in RBC-Na concentration when RBC were incubated in the presence of ouabain. The ouabain-sensitive Na efflux rate constant (ERCos) was obtained by dividing Eos by RBC-Na concentration. RBC free Mg and ERCos were significantly higher in the TR group than in the ESRD group. There was a significant correlation between RBC free Mg and ERCos (r = 0.474, p less than 0.01). These results support the views that the regulation mechanism for intracellular free Mg is different from that for extracellular Mg in patients with renal disease, and intracellular free Mg modulates Na pump activity of RBC.     

A clinico-pathological study of resected lung cancer cases accompanied by pleural dissemination

A total of 26 lung cancer cases accompanied by pleural dissemination were resected between June 1977 and June 1988. Of these 16 cases were male and 10 cases were female. Their age was 34-78, and the average age was 56.7 years. The histologic type was adenocarcinoma in 23 cases, 1 was large cell carcinoma, 1 was combined adenosquamous cell carcinoma, and 1 was combined adeno-small cell carcinoma. Of these 23 adenocarcinoma cases, 15 were well differentiated, 7 were moderately differentiated, and 1 was poorly differentiated. There was no correlation between tumor size and pleural dissemination. Pleural effusion was observed in 8, 5 had bloody effusion and the other 3 had yellow effusion. Exact preoperative diagnosis and evaluation of extent was very difficult in pleural dissemination cases except for the pleural effusion cases. Concerning the operation method in these cases pleuropneumonectomy was performed in 10, pleurolobectomy in 6, and lobectomy in 10. Prognosis of cases of resected pleural dissemination was very poor. The median survival time was 16 months, The 1-year survival rate was 56.3%, the 2-year survival rate was 23.2%, the 3-year survival rate was 15.4%, and the 4-year survival rate was 7.7%. There was no 5-year survivor in lung cancer cases of this group. Malignant pleural effusion cases had a poorer prognosis, with 6 of 8 cases dying within 1 year from operation. No remarkable therapeutic effects were achieved by adjuvant chemotherapy. In the single case of preoperative hyperthermia, histological therapeutic effect (Ef 2) was recognized. These results suggest that there is no indication of operation in malignant pleural effusion cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of metastatic optic nerve head and retinal carcinoma with vitreous seeds

Metastasis directly to the optic nerve head without choroidal involvement is rare. We report a case of metastatic adenocarcinoma of the optic nerve head and retina with vitreous seeds which was observed in a 45-year-old Japanese male. The primary site of the lesion was clinically the upper lobe of the left lung. Tumor emboli were observed in the central retinal vein. Vitreous seeds appeared to have arisen from a necrotic area and papillary projections of the tumor.