No abstract available.
Ichthyosiform Erythroderma, Congenital*

No abstract available.
Pancreatic Pseudocyst*

The purpose of this study is to compare the quality of intraoperative salvaged blood with that of Citrate Phosphate Dextrose(CPD) stored blood in 32 patients having cardiac surgery involving cardiopulmonary bypass. 20 patients in the control group received one week cild CPD stored blood and 12 patients received autologous intraoperative salvaged blood which prepared and processed with the Hemonetics Cell Saver Plus autotransfusion device. 1) Salvaged blood had a higher hemoglobin concentration(l7.6 vs 13.1 g/dl, P<0.005), a higher 2.3-diphosphoglycerate concentration(3.1 vs 0.6 mmol/L, P<0.005), higher pH(7.6 vs 6.8, P<0.005) than CPD stored blood group. Salvaged blood had a lower ATP concentration was(47.7 vs 67.7 pmol/dl, P<0.005) than stored blood group. 2) Platelet count was l3.4 X 10(9)/L and WBC count was 5.09 X 10(9)/L in salvaged blood. 3) Salvaged blood volume was 1411+/-487.9 ml(range 580 to 2325 ml). Intraoperative salvaged blood had a superior oxygen carrying and delivery capacity, but modification of washing is required to lessen the platelet and white blood cell count in the salvaged blood.
2,3-Diphosphoglycerate* ; Adenosine Triphosphate* ; Blood Platelets ; Blood Transfusion, Autologous ; Blood Volume ; Cardiopulmonary Bypass ; Citric Acid ; Erythrocytes* ; Humans ; Leukocyte Count ; Oxygen ; Platelet Count ; Thoracic Surgery

Membranous lipodystrophy is a peculiar type of fat necrosis, present in patients with various types of skin diseases. It is charaeterized by the presence of microcysts and macrocysts lined by amorphous eosinophilic material with a crenelated arabesque appearance and microgranules in the subcutis with massive fat necrosis. The eosinophilic lining and microgranules stain positively with periodic acid-Schiff, are resistant to diastase, and also stain with Sudan black B. We report three cases of subcutanous membranous lipodystrophy in patients with erythema induratum, posttraumatic panniculitis and morphea with typical clinical and histopathologic findings.
Amylases ; Eosinophils ; Erythema Induratum ; Fat Necrosis ; Humans ; Lipodystrophy* ; Panniculitis ; Scleroderma, Localized ; Skin Diseases ; Sudan

No abstract available.
Lithotripsy* ; Shock*

No abstract available.
Hernia, Diaphragmatic* ; Stomach Volvulus*

No abstract available.
Animals ; Epithelium* ; Mice* ; Oocytes* ; Oviducts* ; Phenobarbital*

Netherton Syndrome is a rare autosomal recessive inherited disorder, characterized by triad of congenital ichthyosiform dermatosis, trichorrhexis invaginata and atopy diathesis. It has been found to be due to mutations in SPINK5, a gene encoding LEKTI (lympho-epithelial Kazal-type related inhibitor). LEKTI is a serine proteinase inhibitor that is expressed in epithelial and lymphoid tissues, and may be important in the keratinocyte terminal differentiation and T and B cell maturation. We herein report two cases of Netherton Syndrome in sisters who had ichthyosis linearis circumflexa, trichorrhexis invaginata and atropic dermatitis.
Dermatitis ; Disease Susceptibility ; Genes, vif ; Humans ; Ichthyosis ; Keratinocytes ; Lymphoid Tissue ; Netherton Syndrome* ; Serine Proteases ; Siblings* ; Skin Diseases

Leiomyosarcoma of the rectum is a extremely rare disease without well documented report on its management and prognosis. The most complicated problem lies on the correct diagnosis. Many pathologic and histologic criteria have been proposed to make it clear. The treatment of rectal leiomyosarcoma is controversial. Some authors recommand wide local excison for low-grade tumors as much as 2 cm in diameter. However, radical abdominoperineal resection is the procedure of choice. Leiomyosarcoma of the rectum is resistant to radiotherapy, and no single effective chemotherapeutic drug has been found yet, although adriamycin is effective in one third of all cases. The local recurrence rate was much higher in patients receiving wide local excision and the overall 5-year or 10-year survival rate is similar. We report a case of rectal leiomyosarcoma and review the literature.
Diagnosis ; Doxorubicin ; Humans ; Leiomyosarcoma* ; Prognosis ; Radiotherapy ; Rare Diseases ; Rectum* ; Recurrence ; Survival Rate

Microsatellites are short nucleotide repeat sequences present throughout the human genome. Alterations of microsatellites, comprising extra or missing copies of these se quences, have been termed microsatellite instability(MSI, genetic instability, replication errors, RER(+) phenotype). To date, at least four genes involved in DNA mismatch repair, hMSH2, hMLH1, hPMS1 and hPMS2, are thought to account for the observation of microsatellite instability in tumor from Hereditary nonpolyposis colorectal cancer (HNPCC) patients. The genetic defect responsible for the MIN+ phenotype in sporadic colorectal cancer, however, has yet to be clearly delineated. The purpose of this study was to determine the presence of MSI in sporadic cancer and to correlate its occurrence with clinicopathological parameters, we have studied six microsatellite loci by use of polymerase chain reaction amplification and denaturing polyacrylamide gel electrophoresis. We found that 20%(9 of 46 cases) sporadic colorectal cancers showed RER at two or several loci(RER+). Microsatellite instability was associated with location of the tumor in the proximal colon 66%(6 of 9 cases) and with poorly differentiated tumor phenotype 56%(5 of 9 cases). In order to better understand the role of somatic alterations within hMSH2 in the process of colorectal tumorigenesis, we examined the most conserved regions(codon 598~789) of this gene in nine patients with MIN spotadic colorectal cancer. 6 patient of RER(+) colorectal ca. patients had a polymorphism which was a T to C base change in the intron sequence at -6 position of the splice acceptor site at the 5'end of exon 13. This particular sequence variation is a polymorphism rather than a mutation which increase cancer susceptability. These data suggest that the genetic instability is detect ed in some colorectal cancers and play an important role in the pathogenesis of sporadic colorectal cancer.
Carcinogenesis ; Colon ; Colorectal Neoplasms* ; Colorectal Neoplasms, Hereditary Nonpolyposis ; DNA Mismatch Repair ; Electrophoresis, Polyacrylamide Gel ; Exons ; Genome, Human ; Humans ; Introns ; Microsatellite Instability* ; Microsatellite Repeats* ; Phenotype ; Polymerase Chain Reaction ; RNA Splice Sites