No abstract available.
Tetany*

Sjögren-Larsson syndrome(SLS) is a rare hereditable disease characterized by congenital ichthyosis, spastic diplegia and mental retardation. Along with the typical triad of symptoms, many patients with this disease have short stature, kyphosis and glistening dots in the retina of the eye. The pathogenesism is unknown but recent studies suggest that SLS might be, at least in part, a disorder of fatty acid metabolism. We describe a patient with a pathognomonic finding in the fundus and with the classic features of SLS.
Cerebral Palsy ; Humans ; Ichthyosis ; Intellectual Disability ; Kyphosis ; Metabolism ; Retina ; Sjogren-Larsson Syndrome*

Nevus lipomatosus cutaneous superficialis(NLCS) is a rare nevoiri anomaly characterized by ectopic adipose tissue in the dermis without predilection. In this disease two clinical types are distinguished: The classic type consists of asymptomatic linear or zosteriform coliection of soft, flesh colored to yellowish, papules or nodules that often coalesce into plaque. It is usually located in the pelvic girdle and lumbar area but has been repeted on the abdomen, thorax, thigh, and scalp. The solitary form usually appears later in life and can occur at any site. There is no sexurl irredilection or hereditary trend. There are no assoeiated abnor malities. The authors experienced three cases of typical NLCS including thia silitary type that had several solitary nodules irregularly. The first case was a 24-year-old female which has had multiple, flesh to dark brown-colored, confluent nodules over the left upper thigh for 5-years. The second case is the solitary type that a 56-year-old female has had discrete bean sized to what sized nodules on the right buttock for 13-years. The third case was a 23-year-old female who had soft, yellowish skin colored, cerebriform tumor on the right buttock for 10-yesrs. Diagnos was confirmed by clinical and histopathologic findings.
Abdomen ; Adipose Tissue ; Buttocks ; Dermis ; Female ; Humans ; Middle Aged ; Nevus* ; Scalp ; Skin ; Thigh ; Thorax ; Young Adult

No abstract available.
Emergencies* ; Emergency Service, Hospital* ; Humans ; Statistics as Topic*

No abstract available.
Knee*

PURPOSE: As a new strategy to modulate drug resistance in the treatment of relapsed or refractory non-Hodgkin's lymphoma(NHL), continuos infusion of drugs has been incorporated into the chemotherapy. We conducted a phase II study to determine the activity and safety of EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide, prednisolone) chemotherapy, in which the natursl products are administered as a continuous infusion, for previously treated NHL's of intermediate grade. MATERIALS AND METHODS: EPOCH chemotherapy (etoposide 50 mg/m2/day 24 hour- continuous infusion, days 1~4, vincristine 0.4 mg/m2/day 24 hour-continuous infusion, days 1~4, doxorubicin 10 mg/m2/day 24 hour-continuous infusion, days 1~4, cyclophosphamide 750 mg/m2 i.v., day 5, prednisolone 60 mg/m2/day p.o. days 1-5) was given to eligible patients every 3 weeks and we assessed response and toxicity of the regimen. RESULTS: Between June 1993 and December 1995, total 56 patients entered this trial and 49 were evaluable. The complete response rate was 41%(95% C.I.: 27-55%). After follow up of 9~50(median 38) months, progression free survival was 0~39+(median 7) months and the overall survival was 1~44+(median 14) months. The prognostic factor analyses showed that B symtoms and serum LDH level before treatment and response to previous treatment affected complete response rate, and patients' performance status and response to previous treatment affected progression free survival and overall survival. Toxicities of EPOCH regimen were leukopenia, stomatitis, nausea/vomiting and neurotoxicity, but they were tolerable. There was 1 case of treatment-related death due to sepsis. CONDUSION: EPOCH chemotherapy was safe and effective for the patients with relapsed NHL. However, the results of patients with NHL refractory to previous treatment were so poor that more intensive, novel treatment would be needed for this category of patients.
Cyclophosphamide ; Disease-Free Survival ; Doxorubicin* ; Drug Resistance ; Drug Therapy* ; Follow-Up Studies ; Hodgkin Disease* ; Humans ; Leukopenia ; Lymphoma, Non-Hodgkin ; Prednisolone ; Sepsis ; Stomatitis ; Vincristine*

BACKGROUND: Spontaneous pneumothorax patients with blebs or bullae are considered to be good candidates for operation, and various objective diagnostic modalities have been performed for detection of blebs and bullae. This study was performed to compare the efficacy of thoracoscopic examination with using a minimally invasive 2 mm thoracoscope with high-resolution computed tomography (HRCT) for treating primary spontaneous pneumothorax. MATERIAL AND METHOD: From June 2001 to March 2002, 34 patients with spontaneous pneumothorax underwent study with 2 mm video-thoracoscopic examination and HRCT. We regarded a bleb larger than 5 mm in diameter as significant. Standard thoracoscopic wedge resection was performed in 18 patients with significant bleb via a 2 mm video-thoracoscopic examination. 1 patient incurred bleeding, and the remaining 15 patients were treated with pleural drainage. RESULT: Multiple or single bleb lesions were detected by 2 mm video-thoracoscope in 52.9% (18/34) of the patients with primary pneumothorax. For a total of 19 patients who were operated on, the diagnostic accuracy of the 2 mm video-thoracoscopic examination for bullae and bleb was 94.7% (18/19), which was superior to that of HRCT (73.7%, 14/19). At a mean follow-up of 30+/-3 months, no recurrence occurred in both the operative group and the non-operative group. CONCLUSION: 2 mm video-thoracoscopic examination under local anesthesia has higher diagnostic accuracy than HRCT, and it is a useful alternative for determining the operative indications for spontaneous pneumothorax.
Anesthesia, Local ; Blister ; Drainage ; Follow-Up Studies ; Hemorrhage ; Humans ; Pneumothorax ; Recurrence ; Thoracoscopes ; Thoracoscopy

PURPOSE: Drug resistance is one of the major obstacles to treatment of cancer. Multidrug resistance (MDR) caused by overexpression of p-glycoprotein (Pgp) in cancer cell membrane is a well-known mechanism of drug resistance in in vitro system and was reported to be a significant mechanism of resistance in non-Hodgkins lymphoma (NHL). Verapamil, a calcium channel blocker, is proven in vitro to overcome the MDR caused by Pgp. We performed a phase II trial of verapamil in patients with NHL refractory to EPOCH regimen (etoposide, prednisolone, vincristine, cyclophosphamide, and doxorubicin) to overcome the MDR caused by Pgp. MATERIALS AND METHODS: Verapamil was administered via intravenous route from 1 hour before to 12 hour after the 96-hour infusion of etoposide, doxorubicin, and vincristine which were known to be substrates of Pgp in EPOCH regimen. The dose of verapamil was 0.15 mg/Kg in bolus and 0.2 mg/Kg/hr in infusion at the beginning and escalated by 0.05 mg/Kg/hr every 24 hours if there was no dose-limiting toxicities such as 2nd or 3rd degree AV block, hypotension, or congestive heart failure. Plasma verapamil concentrations were measured every 24 hour by gas chromatography. Mdrl expression level in tumor tissues was measured by RT-PCR. RESULTS: From Feb. to Nov. 1994, 14 patients were treated with this protocoL However, poor tolerability and no response in these patients led to early closure of the study at this 1st stage of patient accrual according to Gehans method. Among 14 patients, 12 experienced 2nd or 3rd degree AV block and/or hypotension and required temporary cessation of infusion and reduction of verapamil dose. However, there was no congestive heart failure or treatment-related death. The peak concentrations of verapamil were 0.29-1.94 pM (mean 0.93 pM) and mean concentrations during the 4-day infusion were 0.22-1.21 pM (mean 0.6 pM). Mdrl expression levels measured in 6 patients were 0.99-14.43 U (median 4.39). CONCLUSION: These results suggest that verapamil in this dose and schedule was neither tolerable nor effective for the reversal of drug resistance in NHL patients.
Appointments and Schedules ; Atrioventricular Block ; Calcium Channels ; Cell Membrane ; Chromatography, Gas ; Cyclophosphamide ; Doxorubicin ; Drug Resistance* ; Drug Resistance, Multiple ; Etoposide ; Heart Failure ; Hodgkin Disease* ; Humans ; Hypotension ; Lymphoma, Non-Hodgkin ; P-Glycoprotein ; Plasma ; Prednisolone ; Verapamil* ; Vincristine

Autophagy is a series of catabolic process mediating the bulk degradation of intracellular proteins and organelles through formation of a double-membrane vesicle, known as an autophagosome, and fusing with lysosome. Autophagy plays an important role of death-survival decisions in neuronal cells, which may influence to several neurodegenerative disorders including Parkinson's disease. Chebulagic acid, the major constituent of Terminalia chebula and Phyllanthus emblica, is a benzopyran tannin compound with various kinds of beneficial effects. This study was performed to investigate the autophagy enhancing effect of chebulagic acid on human neuroblastoma SH-SY5Y cell lines. We determined the effect of chebulagic acid on expression levels of autophagosome marker proteins such as, DOR/TP53INP2, Golgi-associated ATPase Enhancer of 16 kDa (GATE 16) and Light chain 3 II (LC3 II), as well as those of its upstream pathway proteins, AMP-activated protein kinase (AMPK), mammalian target of rapamycin (mTOR) and Beclin-1. All of those proteins were modulated by chebulagic acid treatment in a way of enhancing the autophagy. Additionally in our study, chebulagic acid also showed a protective effect against 1-methyl-4-phenylpyridinium (MPP+) - induced cytotoxicity which mimics the pathological symptom of Parkinson's disease. This effect seems partially mediated by enhanced autophagy which increased the degradation of aggregated or misfolded proteins from cells. This study suggests that chebulagic acid is an attractive candidate as an autophagy-enhancing agent and therefore, it may provide a promising strategy to prevent or cure the diseases caused by accumulation of abnormal proteins including Parkinson's disease.
1-Methyl-4-phenylpyridinium ; Adenosine Triphosphatases ; AMP-Activated Protein Kinases ; Autophagy* ; Cell Line ; Humans ; Lysosomes ; Negotiating ; Neuroblastoma ; Neurodegenerative Diseases ; Neurons ; Neuroprotective Agents* ; Organelles ; Parkinson Disease ; Phyllanthus emblica ; Sirolimus ; Terminalia

STUDY DESIGN: Retrospective study. OBJECTIVES: To evaluate the clinical and radiological outcomes by the method of three-level anterior cervical discectomy and fusion with cervical plate. SUMMARY OF LITERATURE REVIEW: The arthrodesis rate and outcome for patients having three-level anterior cervical discectomy and fusion procedures is disappointing. The success of arthrodesis depends on several factors including bone graft type, size, and additional anterior plate fixation. MATERIALS AND METHODS: Five patients (average age, 69 years; all males) were observed. All had a anterior discectomy, placement of autogenous tricortical iliac bone graft at three-levels and application of a Orion plate. Clinical and radiologic results of bony union, cervical lordosis and intervertebral disc height were assessed. RESULTS: All clinical symptoms of patients had been resolved after operation. The postoperative scores by the criteria of Odom are 2 in excellent and 3 in good. The bony unions were achieved in all cases in the average 12 weeks after surgery (minimum 8 weeks, maximum 20 weeks). The sum of three-level intervertebral disc height in average was increased from 14.8 mm preoperatively to 25.4 mm postoperatively. The average angle of cervical lordosis was corrected from 18o preoperatively to 27o postoperatively. CONCLUSIONS: The three-level anterior cervical discectomy and fusion with Orion plate and autogenous tricortical iliac bone graft results in effective surgical treatment, which produces good clinical outcome, early and solid bony union, restoration of the normal cervical lordosis and disc space height.
Animals ; Arthrodesis ; Diskectomy* ; Humans ; Intervertebral Disc ; Lordosis ; Retrospective Studies ; Transplants