A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with mixed connective tissue disease]

Sweet's syndrome (acute febrile neutrophilic dermatosis) is an unusual condition characterized by fever, polymorphonuclear neutrophil leukocytosis of the blood, thick painful plaques on the face, neck and limbs, and a dense dermal infiltrate of mature neutrophils seen histologically. Recently, this disease has also been reported in association with various malignant neoplasms and chronic inflammatory disorders. In the literature, seven cases of Sweet's syndrome associated with collagen diseases have been reported, but no cases with mixed connective tissue disease (MCTD). The first case of Sweet's syndrome associated with MCTD was herein described and discussed. A 49-year-old man was admitted to our hospital with the complaints of high fever and painful erythema on his face, neck and limbs. Six months ago, MCTD was suspected, with the presence of limited cutaneous sclerosis of the hands, Raynaud's phenomenon, polyarthralgia, an elevation of CPK value and a positive anti-RNP antibody. Just before hospitalization, he suffered a prodromal infection of the upper respiratory tract for two weeks. He was diagnosed as Sweet's syndrome by the clinical and histological features. He began receiving corticosteroid therapy (prednisolone 60 mg/day), and within a week he showed dramatic improvement in the above symptoms.     

Iron in the enameloid of perciform fish.

It is known that a high concentration of iron is deposited in the enameloid of some teleostean fish. Previously, Suga et al. (1989) pointed out that the iron concentration in the enameloid is related to the phylogeny of fish rather than to the feeding habits, according to the results of quantitative iron analyses on the teeth of marine teleost fish of the Tetraodontiformes. In the present study, in order for the previous idea to be verified, quantitative iron analysis was made with an electron microprobe on the enameloid of fish belonging to the Perciformes, which is the largest group of teleostean fish in the world and consists of both marine and freshwater species. The enameloid of all the fish examined (57 species) contained high iron concentrations ranging from 0.2% to 10.2% at the surface or middle layer, whereas that of an advanced suborder, Tetraodontoidei, of the Tetraodontiformes was very low in iron, at a level which could not be discriminated from the background value of the emission intensity. The distribution pattern of iron in the enameloid was classified into at least two types, namely, type A, in which a high iron concentration was observed mainly in the surface layer, and type B, in which iron was deposited throughout the entire layer, although there were differences in concentration. There were some differences in the concentration and distribution of iron in the enameloid for the families; for example, those of the Scaridae had a type A distribution, with about 0.2% iron only at the surface layer, whereas those of the Cichlidae, Centrarchidae, and Acanthuridae, which showed a type B distribution, contained iron ranging from 2.9% to 10.5% at the surface or middle layer of enameloid. Such differences seemed to be associated with the difference in timing of the commencement of the iron deposition into the developing enameloid, which is probably related to the phylogeny of fish. There was no evidence to support the idea that the iron concentration in the enameloid is associated with the feeding habits of fish, as proposed by previous investigators.     

Relationship between left ventricular systolic function and the myocardial gray level distribution in patients with idiopathic dilated cardiomyopathy]

Dilated cardiomyopathy (DCM) is characterized by progressive left ventricular (LV) systolic dysfunction of nonspecific etiology. Fifty-nine DCM patients were serially observed by echocardiography for 4.5 +/- 2.6 years, and 7.3 +/- 3.4 times M-mode and two-dimensional echocardiography was performed during the observation period using SSH-11A (Toshiba). To assess LV systolic function, ejection fraction was calculated by Pombo's method. Myocardial gray level distribution shown by echocardiography was calculated to assess the myocardial tissue character. Two-dimensional echocardiographic images were obtained in the parasternal short-axis view, recorded on U-matic videotape, and transferred to an image processing computer system (MIPRON, Kontron). The images were digitized and stored on the computer. The regions of interest (ROI) were placed in the LV septum, posterior papillary muscle, posterior wall, anterior papillary muscle and entire LV wall. The gray level distributions in each ROI and its quantitative parameters (mean, SD, skewness, excess) were calculated. The corrected myocardial gray level of every ROI (CMD) was also calculated and expressed as the ratio to the mean gray level of the LV cavity. Seven patients exhibited significant decreases in ejection fraction (more than 10%) during the observation period (group A); the remainders showed less change (group B).(ABSTRACT TRUNCATED AT 250 WORDS)     

Long-lasting effect of ceruletide on dyskinesia and monoaminergic neuronal pathways in rats treated with iminodipropionitrile

In a model of dyskinesia induced by the administration of iminodipropionitrile (IDPN) in the rat, we evaluated the effects of ceruletide, an analogue of cholecystokinin, on behavioral abnormalities and monoaminergic neuronal function. Vertical head twitching in the IDPN-treated animals was inhibited for over 5 h following a single subcutaneous dose of 160 micrograms/kg ceruletide. In animals dosed daily for 2 or 3 days, the number of head twitches at 24 h after the last dose was about one-third of the number before treatment. After repeated daily doses of ceruletide for 6 days, the number of head twitches was reduced to low levels and remained significantly below pretreatment levels until the 4th posttreatment day. These results indicate that the inhibition of dyskinesia by ceruletide was long-lasting. Assays of monoaminergic neurotransmitters and their metabolites in various brain regions indicate that an imbalance between dopaminergic and serotonergic neuronal systems plays a major role in the pathogenesis of the IDPN-induced dyskinesia, i.e. the ratio of (DOPAC+HVA)/5-HIAA was significantly greater in the striatum but significantly smaller in the hippocampus of the IDPN-treated vs normal animals. This initially abnormal ratio of (DOPAC+HVA)/5-HIAA in the striatum and hippocampus of IDPN-treated animals returned to normal following treatment with ceruletide, corresponding with the reduction of the head twitching. The alterations in monoaminergic neuronal function induced by repeated administration of ceruletide persisted for at least 3 days, even though its plasma half-life is several minutes. Ceruletide also exerted a marked effect on monoaminergic neuronal function in the IDPN-treated rats, in contrast to only a slight effect in normal animals.(ABSTRACT TRUNCATED AT 250 WORDS)     

Preexpanded distant "super-thin" intercostal perforator flaps for facial reconstruction without the need for microsurgery.

BACKGROUND: Concept of the 'super-thin perforator flap' was introduced in 1994 by authors. Since then, various types of 'super-thin perforator flaps' were applied successfully especially for contour sensitive reconstruction such as face and neck. METHODS: Eleven patients requiring large flaps who presented with extensive disfiguring facial scar (male: seven cases, female: four cases). On the consideration of flaps' colour, texture and thickness requirements, the authors selected 'super-thin' anterior intercostal perforator flaps (AICP, range from 4 x 14 cm to 25 cm x 9 cm) for reconstruction purpose. First, tissue expanders (volume range from 800 cc to 1200 cc) were carefully inserted under the AICP. After the flaps were expanded for 2 months, distant scars were removed and the covering super-thinned flaps were transferred into recipient site. Two weeks later, pedicles in the anterior chest were cut down and flaps were transferred to replace all the left scars. RESULTS: Flap were survived without any complications. The colour, texture and thickness of the transferred flap were satisfactory, shrink of flaps were not observed after long term follow-up. The authors present a method of facial reconstruction that has the advantages of creating a large amount of thin tissue of both good colour and texture, without the need of microsurgery and few disadvantages of donor-site morbidity. The disadvantages are three-staged procedures, complications of tissue expansion and uncomfortable compulsory posture for patients. In our opinion, this is an alternative method of choice for reconstructing all large defects in the lower two-thirds of the face.     

Crossed cerebellar hypoperfusion in unilateral major cerebral artery occlusive disorders.

We evaluated regional blood flow and oxygen metabolism in the cerebral and cerebellar cortices of 15 patients with unilateral major cerebral artery occlusive disorders with PET. These patients showed a cortical blood flow asymmetry in middle cerebral artery distribution. Only subcortical abnormalities were detected on computed tomography. Nine patients showed crossed cerebellar hypoperfusion, a reduction in contralateral cerebellar blood flow, while six did not. No difference in the degree of cerebral blood flow asymmetry existed between the two patient groups. However, oxygen metabolism asymmetry was more pronounced and was more closely matched to blood flow asymmetry in patients with crossed cerebellar hypoperfusion. These findings suggest that a major cause of cerebral cortical blood flow reduction is reduced metabolic demand in patients with crossed cerebellar hypoperfusion. Crossed cerebellar hypoperfusion may have clinical significance as a reflection of the cerebral metabolic state on blood flow images.     

Surgical treatment of native valve endocarditis]

Twenty eight patients with native valve endocarditis (NVE) were subjected to this study. Thirteen patients underwent an operation at the chronic phase, and 15 patients at the active phase. One of the 13 patients at the chronic phase died of cardiac rupture due to myocardial infarction which had occurred preoperatively, and one of 10 patients at active phase without annular infection died of rupture of mycotic cerebral aneurysm early postoperatively. Among 5 patients at the active phase with annular infection, prosthetic valve endocarditis occurred in one patient 1.5 months after supraannular aortic valve replacement, and the second operation with a translocation technique was needed. This patient was lost from low output syndrome. Another patient in this group, who underwent a translocation technique because of mycotic annular abscess, died of intestinal infarction late postoperatively. The other 24 patients went a good postoperative course. Five patients with annular infection at the active phase had a shorter duration from the infectious onset to operation (20 days to 2 months, average 38 days), and the causative microorganisms were streptococcus faecalis, staphylococcus epidermidis and gram-negative coccus. One patient, who died of mycotic cerebral aneurysmal rupture, had candida albicans as a causative microorganism. For patients with NVE, an early aggressive operation is essential before infection extends to the annulus or to other vital organs, especially when these microorganisms are identified.     

Radiation-induced bilateral cystic frontal lobe necroses demonstrating a fluid-blood level--case report.

A 41-year-old male developed radiation-induced bilateral cystic frontal lobe necroses after irradiation for an olfactory neuroblastoma. Computed tomography (CT) and magnetic resonance (MR) imaging revealed the lesions, one containing a fluid-blood level on CT scans and niveau formation on MR images. It was proved to be a coagulated hematoma within the cyst at surgery. Such a fluid-blood level in a radiation-induced cyst has never been reported, although hemorrhage frequently accompanies delayed radiation necrosis. Positron emission tomography with multiple tracers may be useful in differentiating cerebral radiation necrosis from tumor recurrence, because of absence of abnormal tracer accumulation.     

Solitary right ventricle metastasis by renal cell carcinoma.

A 57-year-old man with a history of renal cell carcinoma presented with presyncope. He underwent nephrectomy years earlier followed by HLA-matched allogeneic peripheral-blood stem-cell transplantation. Echocardiographic investigation revealed a solitary right ventricle mass without contiguous vena caval or right atrial involvement. The mass was pathologically confirmed to be metastatic carcinoma in the right ventricular cavity. This case highlights the need to consider an underlying neoplastic syndrome in patients presenting isolated right ventricle mass by echocardiography.