A virus-associated hemophagocytic syndrome is characterized by high fever, liver dysfunction, coagulation abnormalities, pancytopenia, and a benign histiocytic proliferation with prominent hemophagocytosis in bone marrow, lymph node, spleen, and liver. Three phases of disease progression can be defined. In the first week, there is mild leukocytosis and myeloid hyperplasia in the marrow and the fever is resistant to antipyretics and antibiotics. In the second phase, usually corresponding to the 2nd to 3rd week of the illness, the fever persists and jaundice and hepatosplenomegaly may develop and the marrow now reveals the presence of atypical or transformed T lymphocytes and a scattering of histiocytes with hemophagocytosis. In the third phase, the disease progresses to a full-blown hemophagocytic syndrome with coagulopathy and lung infiltrates. The marrow in this stage is usually hypoplastic with florid histiocytic proliferation and hemophagocytosis and the patients usually die within 1-2 months. We describe a pregnant woman with fatal hemophagocytic syndrome. Virologic study strongly suggests that Epstein-Barr virus implicated in the pathogenesis of this patient.
Anti-Bacterial Agents ; Antipyretics ; Bone Marrow ; Disease Progression ; Female ; Fever ; Herpesvirus 4, Human* ; Histiocytes ; Humans ; Hyperplasia ; Jaundice ; Leukocytosis ; Liver ; Liver Diseases ; Lung ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia ; Pregnancy* ; Pregnant Women ; Spleen ; T-Lymphocytes

BACKGROUND: Bone marrow transplantation (BMT) or peripheral blood stem cell transplantation (PBSCT) following high dose chemotherapy has been an important therapeutic option for patients with hematologic malignancies or some solid tumors. The number of progenitor cells in the collection products has been used to determine the optimum time to stop the collections and to predict the hematopoietic engraftment after transplantation. In this study, we investigated the relationship between end-product cell counts measured by different methods and the influence of the infused cell dose on the engraftment rate. METHODS: Twenty five patients receiving autologous PBSCT and 25 patients receiving allogeneic BMT were studied. The number of total nucleated cells (TNC), of mononuclear cells (MNC), of CD34+ cells, and of CFU-GM (colony-forming unit-granulocyte monocyte) colonies were measured in each collection product. The number of days required to achieve an absolute neutrophil count (ANC) of 0.5x109/L with TNC count of 1.0x109/L and platelet count of 20x109/L without transfusions was taken as an arbitrary measure of the engraftment rate. RESLUTS: A close correlation between CD34+ cells/kg and CFU-GM/kg was observed in both collection products (p<0.05). However, MNC/kg also showed significant correlations with CD34+ cells/kg and CFU-GM/kg in allogeneic bone marrow collection products (p<0.05). The CFU-GM amount in the PBSC products was greater than that in the bone marrow collection products (p<0.05). Time to engraftment was a median of 14 (range 9-50) days in autologous PBSCT group, but 29 (range 17-57) days in allogeneic BMT group. In autologous PBSCT, infused CD34+ cells/kg and CFU-GM/kg correlated significantly with ANC recovery (p<0.05). CONCLUSIONS: The number of CD34+ cells was correlated with that of CFU-GM in the collection products, and the infused cell doses showed positive relation to the engraftment rate in autologous PBSCT. These findings suggest that measurement of CD34+ cell counts alone would be a sufficient parameter to predict the engraftment rate in autologous PBSCT.
Bone Marrow Transplantation* ; Bone Marrow* ; Cell Count ; Drug Therapy ; Granulocyte-Macrophage Progenitor Cells ; Hematologic Neoplasms ; Humans ; Neutrophils ; Peripheral Blood Stem Cell Transplantation* ; Platelet Count ; Stem Cells*

Uterine sarcoma is a rare female neoplasm that accounts for about 2-4% of all uterine malignancies. According to the classification of Norris and Taylor, the subgroup of endometrial stromal sarcoma (ESS), comprising approximately 7-15% of all uterine sarcomas, can be divided into low-grade ESS and high-grade ESS and is characterized by proliferations composed of cells with endometrial stromal cell differentiation. We experienced this infrequent malignant tumor in a 52-year old woman and was treated by total abdominal hysterectomy with bilateral salpingo-oophorectomy followed by adriamycin chemotherapy. We present a case of low-grade ESS with a brief review of the literature.
Classification ; Doxorubicin ; Drug Therapy ; Female ; Humans ; Hysterectomy ; Middle Aged ; Sarcoma ; Sarcoma, Endometrial Stromal* ; Stromal Cells

Uterine sarcoma is a rare female neoplasm that accounts for about 2-4% of all uterine malignancies. According to the classification of Norris and Taylor, the subgroup of endometrial stromal sarcoma (ESS), comprising approximately 7-15% of all uterine sarcomas, can be divided into low-grade ESS and high-grade ESS and is characterized by proliferations composed of cells with endometrial stromal cell differentiation. We experienced this infrequent malignant tumor in a 52-year old woman and was treated by total abdominal hysterectomy with bilateral salpingo-oophorectomy followed by adriamycin chemotherapy. We present a case of low-grade ESS with a brief review of the literature.
Classification ; Doxorubicin ; Drug Therapy ; Female ; Humans ; Hysterectomy ; Middle Aged ; Sarcoma ; Sarcoma, Endometrial Stromal* ; Stromal Cells

BACKGROUND: The information on nasal transport and the metabolism of peptides have been obtained from pharmacokinetic investigations in experimental animals. However, there are no transport and metabolic studies of human nasal epithelial cells. In this study, the permeability characteristics and the metabolic properties of in vitro human nasal cell monolayers were investigated. Material and METHODS: Normal human inferior nasal conchal tissue samples were obtained from patients undergoing endoscopic nasal cavitary surgery. The specimens were cultured in a transwell using an air-liquid interface (ALI) culture, and the transepithelial electrical resistance (TEER) value of the blank filter and confluent cell monolayers were measured. To determine the % leakage of mannitol, 4µmol 14C-labelled mannitol was added and the % leakage was measured every 10 minute for 1 hour. RESULT: Human nasal epithelial cells in the primary culture grew to a confluent monolayer within 7 days and expressed microvilli. The tight junction between the cells was confirmed by transmission electron microscopy. The TEER value of the blank filter, fifth day and seventh day reached 108.5 ohm.cm2, 141 ohm.cm2 and 177.5 ohm.cm2, respectively. Transcellular % leakage of the 14C-mannitol at 10, 20, 30, 40, 50 and 60 minutes was 35.67±5.43, 34.42±5.60, 32.75±5.71, 31.76±4.22, 30.96±3.49 and 29.60±3.68 %, respectively. CONCLUSION: The human nasal epithelial monolayer using ALI using techniques is suitable for a transcellular permeability study. The data suggests that human nasal epithelial cells in as ALI culture technique shows some promise for a nasal transport and metabolism study.
Animals ; Culture Techniques* ; Electric Impedance ; Epithelial Cells* ; Humans* ; Mannitol ; Metabolism ; Microscopy, Electron, Transmission ; Microvilli ; Peptides ; Permeability ; Tight Junctions

Methods: Patients with and without TCLH were assigned to groups A and B, respectively. POSEH between the two groups was compared morphometrically and symptomatically. The risk factors for symptomatic and morphometric POSEH in BESS were identified. Results: The morphometric POSEH was greater in group B, and the difference was significant (p =0.019). The incidence of symptomatic POSEH was lower in group A with 4.6% (5/109) than in group B with 9.5% (9/95); however, the rate was not significantly different (p =0.136). The morphometric POSEH was classified into two small (hG1 and hG2) and large (hG3 and hG4) and were compared between groups A and B, and the difference was significant (p =0.02). In the multivariable logistic regression, nonuse of TCLH (p =0.004) and preoperative diagnosis of stenosis (p =0.016) were variables found to be significant risk factors of morphometric POSEH. Conclusions Severe compression of the thecal sac by POSEH is more common in patients without TCLH. The risk of hematoma formation was higher when bilateral decompression was needed and the cut bone surface was more exposed.

Scleroderma renal crisis is defined as the new onset of accelerated arterial hypertension and/or rapidly progressive oliguric renal failure. The pathogenesis is not well understood but there is increasing evidence that renin- angiotensin system is involved. We report an one female patient with diffuse scleroderma and renal crisis. Initial treatment with ACE inhibitor was not effective in controlling blood pressure until the temporal initiation of hemodialysis. Predialysis serum creatinine level was 8.4 mg/dL, but after initiation of hemodialysis, adequate control of blood pressure was achieved with ACE inhibitor alone. This case illustrated many features of the syndrome of scleroderma renal crisis and supported the early use of captopril and emergency hemodialysis if indicated.
Angiotensins ; Blood Pressure ; Captopril ; Cough* ; Creatinine ; Emergencies ; Female ; Humans ; Hypertension ; Renal Dialysis ; Renal Insufficiency ; Scleroderma, Diffuse ; Tic Disorders* ; Tics*

Nonunion of comminuted distal humeral fracture is troublesome problem to orthopedic surgeon. We report a case of 59 years old woman, who suffered nonunion of comminuted distal humeral fracture previously treated by open reduction and internal fixation with plate and screws concomitantly autoiliac bone graft. We reconstructed humeral condyle with fibular inlay graft inside cortical shell of intercondylar bone fragment and obtained excellent result in radiological and functional outcome.
Female ; Fractures, Comminuted ; Humans ; Humeral Fractures ; Humerus ; Inlays ; Orthopedics ; Transplants

OBJECTIVE: To investigate the expression of apoptosis related proteins and apoptotic cells on the human ovarian follicles. MATERIALS AND METHODS: Thirty five Formalin-fixed paraffin-embedded human ovarian tissue blocks were selected from the surgical pathology files of the department of pathology, College of Medicine, Yonsei University, for the period from 1996 to 1998. All specimen were from premenopausal women aged from 32~45. Ovarian tissues were collected from the patients performing hysterectomy for benign uterine diseases. Immunohistochemical staining was performed for the detection of DNA fragmented cell, Bcl-2, Bax, Fas and Fas-ligand. RESULTS: Bcl-2 and bax were not expressed on the surrounding cells and oocyte of the primary, primordial and preantral follicles. Fas and Fas-ligand (Fas-L) were not expressed on the surrounding cells on the primordial and primary follicles. But expressed on the surrounding granulosa cells and oocyte in the primordial and primary follicles. In the healthy follicles, Bcl-2 was expressed on the granulosa cells, however, Bax was not expressed. DNA fragmented cells were expressed on the inner granulosa cell layer of atretic follicles. CONCLUSION: Fas, Fas-ligand, and Bax may be responsible for the follicular atresia and Bcl-2 may be involved in the follicular survival in the human ovary.
Apoptosis* ; DNA ; Female ; Follicular Atresia ; Granulosa Cells ; Humans* ; Hysterectomy ; Oocytes ; Ovarian Follicle* ; Ovary ; Pathology ; Pathology, Surgical ; Uterine Diseases

Subdural empyema of the brain is an uncommon disorder that occurs more frequently in children than in adult. Authors report a very rare of subdural empyema following the subdural hygroma after mild head injury. The exact mechanism of infection is not known. However, we have to consider subdural infection as one of differential diagnosis in elderly patient with subdural hygroma when new abnormal density lesion is developed in the subdural space.
Adult ; Aged ; Brain ; Child ; Craniocerebral Trauma ; Diagnosis, Differential ; Empyema, Subdural ; Escherichia ; Escherichia coli ; Humans ; Subdural Effusion ; Subdural Space