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.
Animals ; Epithelium* ; Mice* ; Oocytes* ; Oviducts* ; Phenobarbital*

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

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

When the tumor is advanced with distant metastasis or unresectable initially, preoperative chemotherapy could be applied in the treatment of Wilms' tumor We experienced 6 cases of favorable type of Wilms' tumor, 1 case of clear cell sarcoma and 1 case of renal cell carcinoma. They were treated with preoperative chemotherapy and underwent CT Scans before and after the therapy. Pathologic changes after chemotherapy in Wilms' tumor were known from previous reports as subtotal hemorrhagic necrosis, cystic change, clusters of foamy histiocytes, granulation tissue formation, primitive nephrogenic tissues and peripherally remained focal areas of blastemal infiltration. Changes of CT findings after chemotherapy were internal necrosis(6/6), decrease in size(5/6), decrease and absence of regional lymph node enlargement(4/6) and improved or disappeared metastatic lesions(3/3). Although our study had some limitations such as small numbers of cases and all cases were favorable types, we thought that there were good correlations between change of CT findings and subtotal hemorrhagic necrosis after preoperative chemotherapy in Wilms' tumor.
Carcinoma, Renal Cell ; Drug Therapy* ; Granulation Tissue ; Histiocytes ; Lymph Nodes ; Necrosis ; Neoplasm Metastasis ; Sarcoma, Clear Cell ; Tomography, X-Ray Computed ; Wilms Tumor

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