Lowe et al. have described a disease characterized by organic aciduria, decreased renal ammonia production, hydrophthalmos, and mental retardation, this disease is now called as Lowe's syndrome or oculo-cerebro-renal syndrome which manifests growth plates, metabolic acidosis, and characteristic eye changes, including bilateral cataract, glaucoma and nystagmus. It is a congenital hereditary affectation. Most of the patients with this syndrome are male sex. We report a new case of Lowe's syndrome in a 5-year old girl.
Acidosis ; Ammonia ; Cataract ; Child ; Female ; Glaucoma ; Growth Plate ; Humans ; Hydrophthalmos ; Intellectual Disability ; Male

This study was performed to investigate the effect of endotoxin to the CCl4-induced liver injury. Twelve Sprague-Dawley rats were intraperitoneally injected 1.6 g/kg CCl4 as control group. Another 24 rats were orally administrated 300 mg/kg of neomycin at 16 and 3 hours prior to CCl4 injection as experimental group. Twelve among them were intraperitoneally infected 1.0 mg/kg of endotoxin(E-Coli, 0111:B4, No L-2630, lipopolysaccharide, Sigma, USA) and CCl4 simultaneously for offsetting neomycin effect. The rats were sacrificed at 1, 4, 10, and 24 hours after CCl4 injection. The liver tissues from all experimental groups were observed by light and electron microscopy. The results obtained were summarized as follows: In the CCl4 only group, the hepatocytes revealed sweling of ER and mitochondria with many lipid droplet in the cytoplasm. Focal cellular necrosis was seen at the later phase. The Kupffer cells were activated and showed many cytoplasmic processes, secondary lysosomes, and vaculoles. The endothelial cells were edematous. Several neutrophils, platelets, and microthrombi were scattered in the sinusoid. In the neomycin-CCl4-endotoxin administrated group, both hepatocytic destruction and intrasinusoidal microthrombi formation were more pronounced. In the neomycin pretreated group, the hepatocytes revealed mild cellular destruction without necrosis. There is no intrasinusoidal microthrombi. According to these results, it would be concluded that the small dosage of gastrointestinal tract-derived endotoxin affects to the liver injury caused by CCl4. The synergistic effects of CCl4 and gastrointestinal tract-derived endotoxin which can not be detoxified by damaged Kupffer cells, may be more important in the pathogenesis of CCl4-induced liver injury.
Rats ; Animals

No abstract available.
Erythropoietin* ; Humans* ; Renal Dialysis*

BACKGROUND: Surfactant protein C(SP-C) is a hydrophobic 5,000 dalton molecule. SP-C has the primary roles in accelerating surface spreading of a surfactant phospholipid. The filter hypbridization and solution hybridization assays are both rapid and sensitive and can be used to measure the RNAs complementary to any cloned DNA sequence. METHODS: The authors measured the SP-C mRNA levels quantitatively using solution hybridization and filter hybridization assays to obtain a standard curve equation to quantify the mRNA of unknown samples comparatively. RESULTS: 1. The minimum level of the specimens by solution hybridization was 3 pg for SP-C mRNA. 2. The standard curve equation of the solution hybridization assay between the counts per minute(Y) and the SP-C mRNA transcript input(X) was Y=6.46 X+244. The correlation coefficient was 0.9. 3. The minimum detection level of specimens by filter hybridization was 0.1 ng for SP-C mRNA. 4. The standard curve equation of the filter hybridization assay between the counts per minute(Y) and SP-C mRNA transcript input(X) is Y=2541.6 X+252.7. The correlation coefficient was 0.99. CONCLUSION: A comparison of CPM/filter in the linear range allowed an accurate and reproducible estimation of the SP-C mRNA copy number. Filter hybridization and solution hybridization assays are both rapid and sensitive and can be used to measure the RNAs complementary to any cloned DNA sequence. It is ideally suited to situations where accurate quantitation of multiple samples is required.
Animals ; Base Sequence ; Clone Cells ; Protein C* ; Rats* ; RNA ; RNA, Messenger*

Reconstruction of soft tissue dejects on the lower leg and foot remains a difficult challenge for surgeons. If the deject was large and complicated by bone defects, an excellent result could be obtained with free tissue transfer. In case of no bony problem, no major infection and relatively small defect, a local flap is more Convenient and economic than free tissue transfer because of its simple, one-stage and reliable operation. The vascularization of the subcutaneous tissue reveals the predominance of the vascular network in this layer with regard to the dermal or fascial plane. The dermal vascular network at the donor site is sufficient to let the skin survive without its underlying subcutaneous vascular support. Distally based adipofascial flaps, nourished by the lower perforator originating from the major vessel as link pattern were. used successfully for reconstruction of the bone and/or tendon exposure of the lower leg and foot in 10 patients. Between February 1992 and December 1995, ten cases underwent this procedure to reconstruct soft tissue defect on the lower leg and foot. The average age of the patients was 50.6 years (range 5 - 73years). Follow-up was from 10 months to 38 months (mean 21 months). The average time of the operation was about 2 hours 18 minutes. The length to width ratio of adipofascial flap was 2.4 - 5.0 : 1 (mean 3.4 : 1). The advantages of this method are easy dissection, short operation, preservation of the major vascular pericles of the lower limb, skin preservation at the donor site, thus preserve the shape of the limb and minimize donor site scar, and versatility (it is supple and can adapt to every surface, and it can be grafted on the deep or the superficial side). In conclusion, this technique is an useful and alternative method for reconstruction of soft tissue defects on the lower leg and foot in selected cases.
Cicatrix ; Extremities ; Follow-Up Studies ; Foot* ; Humans ; Leg* ; Linear Energy Transfer ; Lower Extremity ; Skin ; Subcutaneous Tissue ; Tendons ; Tissue Donors ; Transplants

No abstract available.
Adult* ; Humans ; Magnetic Resonance Imaging*

BACKGROUND: A newly-found mitochondrial toxin, 3-nitropropionic acid (3-NP), has been proved to induce apoptosis in the striatum. Although striatal lesions produced by 3-NP could develop through an excitotoxic mechanism, the exact relationship between apoptosis induction and excitotoxicity after 3-NP treatment is still not clear. We investigated the role of excitotoxicity and oxidative stress on apoptosis induction within the striatum following intra-peritoneal injection of 3-NP. METHODS: 3-NP was injected for 5 days intra-peritoneally in three month-old mice. One day after the last injection, animals were decapitated. To confirm the presence of apoptosis, we performed in-situ detection of DNA fragmentation by using TUNEL technique and agarose gel elctrophoresis after DNA extraction from striatum. To examine the effect of frontal cortex removal on 3-NP-indeced apoptosis, we removed left frontal cortex by aspiration. For excitotoxicity, NMDA-receptor antagonist-MK 801, non-NMDA antagonist-NBQX, and saline were injected intraperitoneally before 3-NP treatment To detect superoxide, we administered hydroethidium (HEt: 200 ul; 1mg/ml) into the jugular vein 2 days after 3-NP, and the density of oxidized HEt in samples were examined under flouscent microscope. We performed caspase staining to test immunoreactivity of caspase 3 in samples. RESULTS: The TUNEL positive cells were not observed in the striatum ipsilateral to the frontal cortex-removed side, but found in the contralateral striatum. Superoxide radicals measured by using HEt and caspase immunoreactivity were also significantly weaker in the striatum ipsilateral to the frontal cortex-removed side than the contralateral striatum. TUNEL staining revealed less apoptotic changes in the striatum of MK801-treated group than NBQX-or saline-treated groups. DNA laddering on agarose gel electrophoresis was observed in the striatum of NBQX- or saline-treated mice, but not found in MK 801-treated group. CONCLUSION: We demonstrated that removal of the corticostriatal glutamate pathway reduced superoxide production as well as apoptosis induced by 3-NP and NMDA receptor antogonist, but not non-NMDA antagonist, prevented 3-NP-induced apoptosis in the striatum. These results suggest that NMDA-mediated glutamatergic excitotokicity plays an important role in 3-NP related striatal damage.
Animals ; Apoptosis ; Caspase 3 ; DNA ; DNA Fragmentation ; Electrophoresis, Agar Gel ; Glutamic Acid ; In Situ Nick-End Labeling ; Injections, Intraperitoneal* ; Jugular Veins ; Mice* ; Mitochondria ; N-Methylaspartate ; Oxidative Stress ; Sepharose ; Superoxides

Sternocostoclavicular hyperostosis is a benign ossifying diathetic disorder characterized by hyperostosis and soft tissue ossification of the clavicles, anterior portion of the first ribs, and manubrium, with variable hyperostosis or ankylosis in the spine and sacroiliac joints. A review of the literature and our own case describes the clinical findings and its characteristic features in RI, CT, and plain film.
Ankylosis ; Clavicle ; Hyperostosis ; Hyperostosis, Sternocostoclavicular* ; Manubrium ; Ribs ; Sacroiliac Joint ; Spine

PURPOSE: In the case of well pneumatized sphenoid sinus, magnetic susceptibility artifact can be visualized at the brainstem and especially at the pons on echo-planar imaging (EPI) diffusion-weighted imaging. Fast spin-echo periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) is a novel imaging method that can reduce these artifacts. In 3.0T MR, we first evaluate the degree of the relationship of pneumatization of the sphenoid sinus with the occurrence of magnetic susceptibility artifacts (MSA) on the echo planar imaging (EPI) diffusion-weighted imaging (DWI), and we evaluated using PROPELLER-DWI for cancellation of MSAs of the pons in the patients who had MSAs on the EPI-DWI. MATERIALS AND METHODS: Sixty subjects (mean age: 58 years old and there were 30 men) who were classified according to the two types of sphenoid sinus underwent EPI-DWI. The two types of sphenoid sinus were classified by the degree of pneumatization on the sagittal T2-weighted image. The type-1 sphenoid sinus was 0% to less than 50% aeration of the bony sellar floor, and type-2 was 50% or more aeration of the boney sellar floor. Each of 10 subjects (n=20/60, mean age: 53) of the two types had PROPELLER and EPI-DWI performed simultaneously. We first evaluated the absence or presence of MSAs at the pons in the two types, and we compared EPI and PROPELLER-DWI in the subjects who underwent the two MR sequences simultaneously. We used 3.0T MR (Signa VHi, GE, MW, U.S.A.) with a standard head coil. All the MR images were interpreted by one neuroradiologiest. RESULTS: For the type-1, two (6.7%) cases had MSAs and 28 (93.7%) cases did not have MSAs on the EPI-DWI. For the type-2, twenty-seven (90%) cases had MSAs and 3 (10%) cases did not have MSAs on the EPI-DWI. The degree of pneumatization of the sphenoid sinus was related with the occurrence of MSAs of the pons, according to the chi-square test (p=0.000). All twenty cases who had PROPELLER-DWI performed had no MASs at the pons regardless of the type of sphenoid sinus. But all ten cases of type-2 produced MASs on the EPI-DWIs CONCLUSION: For EPI-DWI, a well aerated sphenoid sinus can induce MASs at the pons, and we should recognize this phenomenon to differentiate it from true infarcted lesion. PROPELLER DWI can be an optional tool to use for canceling this artifact.
Artifacts* ; Brain Stem ; Echo-Planar Imaging ; Head ; Humans ; Magnetic Resonance Imaging* ; Middle Aged ; Pons ; Sphenoid Sinus*

No abstract available.
Gemfibrozil* ; Humans ; Peritoneal Dialysis, Continuous Ambulatory*