Myoepitheliomas of the soft palate: helical CT findings in two patients.

We describe the enhancement patterns of myoepithelioma in two patients with a soft palate mass. In the first case, helical CT revealed a faintly enhancing mass. Histologically, the tumor was composed of plasmacytoid cells in a background of rich myxoid stroma. Immunostaining for CD34 showed scanty blood vessels. In the second case, helical CT revealed an intensely enhancing mass. Histologically, the mass was a cellular tumor with fibrous stroma. Immunostaining for CD34 also showed frequent blood vessels.     

Kidney retrieval conditions influence damage to renal medulla: evaluation by proton nuclear magnetic resonance (NMR) pectroscopy.

In the present investigation, the influence of retrieval condition on medullary damage in kidneys was assessed. The isolated perfused pig kidney was used to assess initial renal function from multiorgan donors or single organ donors after cold flush and 24 h cold storage preservation with two preservation solutions: Euro-Collins and University of Wisconsin solutions. Kidneys flushed with cold heparinized saline and immediately perfused were used as a control group. Kidneys were perfused for 90 min at 37.5 degrees C and renal perfusion flow rate, glomerular filtration rate, tubular reabsorption of Na+ and lactate dehydrogenase and N-acetyl-beta-D-glucosaminidase excretion were determined. Ischaemia reperfusion impairment was also determined by 1H NMR (proton nuclear magnetic resonance) spectroscopy. Renal function was significantly decreased in experimental groups when compared to the control group, but there was no significant difference between experimental groups after 24 h cold storage. The release of lactate dehydrogenase in the effluent and the urinary excretion of N-acetyl-beta-D-glucosaminidase were not significantly different after 24 h cold storage. The most relevant resonances determined by 1H NMR spectroscopy were citrate, trimethylamine-N-oxide, lactate, acetate and amino acids. Excretion of these markers was significantly different when compared to biochemical markers. A resonance P (Peak) detected particularly in Euro-Collins solution multiorgan donors after 24 h cold storage was identified and well correlated to renal dysfunction. N-acetyl-beta-D-glucosaminidase spectroscopy, which is a non-invasive and non-destructive technique, is more efficient to assess renal damage than conventional histology and biochemical analysis.     

Nephron Sparing Surgery for De Novo Renal Cell Carcinoma in an Allograft Kidney: A Case Report

De novo renal cell carcinoma in a renal allograft is rare and has special implications in renal transplant recipients. We describe a patient with a renal allograft who developed a de novo renal cell carcinoma in the functioning renal allograft 258 months after transplantation. The patient underwent enucleation of the tumor because preoperative MRI showed it was well-encapsulated. A DNA banding study showed that the tumor originated from the donor. Indications for conservative renal surgery in renal cell carcinoma have been increasing. Accordingly, 1 option in the treatment of de novo renal cell carcinoma in a functioning renal allograft is enucleation as a method of nephron sparing surgery.     

RETT's syndrome in Korea--report of two cases

Rett's syndrome(RS) is a progressive neurodegenerative disorder characterized by exclusive occurrence in females, autistic behavior, dementia, gait ataxia, loss of purposeful use of the hands with stereotypic hand movement, and seizures. Initially RS was considered to be very rare; however, recent reports suggest that the prevalence is considerably higher and occurrence is world-wide. Because the pathophysiological process remains unknown, the diagnosis of RS is based mainly on its characteristic clinical features and course. We experienced two cases of RS which, to our knowledge, are the first reported in Korea. It is quite possible that many patients with RS not yet being diagnosed in Korea.     

HLA-DQA1*0101 haplotypes and disease outcome in early onset pauciarticular juvenile rheumatoid arthritis.

To further investigate a clinical impression that patients with early onset pauciarticular juvenile rheumatoid arthritis (EOPA-JRA) who carry HLA-DQw1 have more severe arthritis, we subtyped HLA-DQw1 in American midwestern patients with EOPA-JRA. The HLA-DQA1*0101 subtype was present in 10 of 19 patients who developed persistent polyarticular erosive disease compared with 18 of 92 healthy controls (chi 2 = 9.13, p = 0.003, RR = 4.6), and occurred more frequently in this polyarticular group than in patients without polyarticular erosive disease (chi 2 = 4.11, p = 0.040, RR = 3.0). The presence of HLA-DQA1*0101 was significantly lower in patients with chronic iridocyclitis than in patients without chronic iridocyclitis (chi 2 = 7.07, p = 0.008, RR = 0.21). In HLA-DQA1*0101 positive patients, DNA sequences of the beta-1 domain of the HLA-DQ alpha and HLA-DQ beta genes (HLA-DQA1*0101, HLA-DQB1*0501 and HLA-DQB1*0503) were identical to those in controls. In this midwestern EOPA-JRA population, HLA-DQA1*0101 or genes in linkage disequilibrium with it, are associated with a cohort of patients with EOPA-JRA with distinct clinical characteristics.     

Distraction vs remodeling surgery for craniosynostosis

Objects We designed several distraction devices and applied these instruments in 14 patients with varying types of craniosynostosis. The aim of this report is to clarify the advantages and disadvantages of these surgical methods and to discuss current concepts for the surgical strategy in the treatment of craniosynostosis. Methods From January 2000 to July 2005, 28 patients with craniosynostosis were retrospectively analyzed. Surgical treatment was performed on 14 patients using the distraction method with internal distraction devices that we designed, in which 5 patients had plagiocephaly, 3 brachycephaly, and 6 scaphocephaly. All patients underwent preoperative and postoperative evaluations, which included the patient’s neurological state, and three-dimensional CT. Results With distraction devices, the time required for the surgery could be shortened almost 3 1/3 h; the bleeding during the surgery was decreased with reduced requirement of more than 200 ml of blood transfusion as compared with remodeling surgery. Postoperatively achieved distraction distances varied from 30.0 to 47.5 mm (mean, 42.99 mm). The average increased volume percent of cranium in distraction surgery group was 20.9% (range, −11.5 to 58.9%) after full distraction. Conclusion With distraction surgery, satisfactory cranial volume expansion and aesthetically pleasing morphological states were achieved in all cases, and the efficacy was statistically significantly high as compared with remodeling method.