Myopathy in primary aldosteronism in relatively rare disease in Korea. A 42-year-old woman with hypokalemic periodic paralysis, proximal muscle weakness and hypertension was found to have myopathy associated with adenoma in the right adrenal gland. She showed marked elevation of muscle enzymes and myopathic pattern in EMG.
Adenoma ; Adrenal Glands ; Adult ; Female ; Humans ; Hyperaldosteronism ; Hypertension ; Hypokalemic Periodic Paralysis ; Korea ; Muscle Weakness ; Muscular Diseases* ; Rare Diseases

AIM: To identify the novel nuclear export signal by analyzing the DNA sequences and detecting the cell localization of different adenovirus ElA - associated protein BS69 isoforms. METHODS:BS69 DNA sequences in Emsebl database were blasted and the sequence of amino acids was aligned with the typical nuclear export signal. Different BS69 isoform fragments were cloned into pcDNA3.1 vector and transfected into Cos7 cells. The BS69 localization was observed by immunostaining and the function was verified by Western blotting. RESULTS: A novel nuclear export signal was found in BS69 isoform 2 but not in isoform 1.The isoform 2 was localized in cytoplasm and isoform 1 in nucleus, which was also consistent with the DNA sequence. The isoform 2 was involved in LMP1 - activated JNK phosphorylation whereas the isoform 1 was not. CONCLUSION: Different BS69 isoforms have different cellular localization. BS69 isoform 2 is localized in cytoplasm, interacting with Epstein - Barr virus latent membrane protein 1 and may be involved in nasopharyngeal carcinoma development.However, the isoform 1 is localized in nucleus and plays important roles in transcription.

Objective To evaluate the clinical outcome of external bracket fixation in the treat- ment of complex tibia diaphysis fracture involving intra-articular fractures.Methods Forty-two cases of complex tibia diaphysis fracture with proximal and distal intra-articular fractures treated surgically in our hospital from January 1999 to January 2004 were analyzed.The complex tibia diaphysis fractures were categorized according to the AO classification as type C2 (multiple segments fracture) and type C3 (ir- regular fracture),proximal and distal intra-articular fractures in 23 and 19 cases,respectively.Definite operation was done within one week.Twenty-two cases were treated with simple external fixator,and 20 cases treated with screws and external fixator.Results All the 42 cases were followed-up regularly. According to AO evaluation of the knee and ankle joint movement,83% (35/42 cases) of the cases gained satisfactory functional outcome,14% (6/42 cases) had quite satisfactory results and 2% (1/42 case) had unsatisfactory functional outcome.Conclusion External bracket fixation can obtain outcome of relative length of the tibia and fibula,tube structure reconstruction,smoothness of the articular surface and the parallel and symmetric relation of knees and ankles for complex tibia diaphysis fracture with proxi- mal and distal intra-articular fracture.The arthritis resulting in pain in movement and restriction of func- tion is considered to be the most important factor affecting the joint function.Early functional exercise is important for best recovery of knee and ankle function.

Objective To study the radiation dose level and dose distribution of leakage and scattering radiation in a helical tomotherapy (TOMO) room,and to identify the dose distribution characteristic which is different from the conventional radiotherapy so as to provide scientific data for the radiation protection of TOMO.Methods A helical tomotherapy facility typed Tomotherapy Hi-Art was employed as the radiotherapy device.The air kerma from leakage radiation and scattering radiation distributed on the couch plane and in TOMO room were detected mainly by GR-200A TLDs of LiF(Mg,Cu,P),while 100 Gy were being accumulated under the condition of simulation radiation treatment.Then,the leakage radiation ratio and scattering-leakage radiation ratio (the ratio of site radiation dose to isocenter standard output dose) were calculated.Results The radiation level and dose distribution of leakage radiation and scattering radiation in the TOMO treatment room were in bilateral symmetry to the rotation isocenter and the treatment couch longitudinal axis,and the radiation level in front of the radiotherapy device was significantly higher than that of the rear of the TOMO equipment.In the plane of treatment couch,the maximum leakage radiation ratio at the position 100 cm away from the isocenter was only 1.3 × 10-4,and the mean value of the leakage radiation ratio at the position 300 cm away from the isocenter was lower than 2.0 × 10-5.The scattering radiation at 200-300 cm away from the isocenter was accounted for 25％-30％ of the leakage radiation from the responding sites,and the indoor scattering radiation decreased faster than leakage radiation with the distance from the isocenter.Conclusions The radiation level of leakage and scattering in a TOMO room is significantly lower than that in a conventional radiotherapy room.

No abstract available.
Humans

OBJECTIVETo investigate the diagnosis, surgical procedure and clinical outcome of cervical intraspinal benign neoplasm.

METHODSTwenty-two cases confirmed pathologically were reviewed included 16 males and 6 females with an average age of 43.8 years old ranging from 18 to 56 years. Symptoms mainly included neck pain, numbness and weakness of the extremities, urinary and fecal incontinence, pyramid signs, etc. All underwent X-ray, CT and MRI, 11 cases were diagnosed as neurilemomas, 4 neurofibromas, 3 spinal meningiomas, 2 intraspinal cysts and 2 lipomas. The tumors were removed completely in 18 cases, above 60% in 3 cases and 25% with biopsy in 1 case. Cervical stability in 11 cases was restored after removal of tumors.

RESULTSAll were followed-up for from 6 to 84 months(means 12.5 months). Symptoms improved in 20 cases and stable in 1 case and worsened in 1 case postoperatively according to JOA scoring system. Two patients recurred and were treated with second surgery. JOA scoring was (6.52 +/- 1.10) before operation, (11.42 +/- 0.79) after operation, there was statically significance (t = 21.38, P < 0.001). The average recover ratio of total was (46.7 +/- 2.46)%, cervical stability was (62.37 +/- 3.58)%, the other methods was (41.21 +/- 4.63)%. The results of JOA were excellent in 18 cases, good in 2, fair in 1 and poor in 1.

CONCLUSIONThe surgical exairesis for cervical intraspinal benign neoplasm has low post-operative recurrence. The main reason of recurrence is not removed the tumor completely. MRI is regarded the effective method which is helpful in differential diagnosis and surgery guidance.

Adolescent ; Adult ; Cervical Vertebrae ; pathology ; physiopathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Spinal Neoplasms ; pathology ; physiopathology ; surgery ; therapy ; Treatment Outcome ; Young Adult

OBJECTIVE To observe the effect of baicalin on Aβ25-35 induced learning and memory deficits and changes in autophagy-related genes in mice so as to explore the related mechanisms of Alzheimer disease (AD) treatment . METHODS C57 mice were administered with 3μL Aβ25-35 3 mmol·L-1 by intracerebroventricular injection to establish an AD model. Baicalin was given by intracerebroventricular injection at the dose of 25, 50 and 100 mg · kg-1 for 15 d, respectively. The total distance and the central grid residence time were measured in the open-field test. The escape latency and the time to reach the platform were monitored in the Morris water maze trial. The autophagic vacuoles in the hippocampus of the mice were observed by transmission electron microscopy before the protein expressions of microtu?bule-associated protein 1 light chain 3 (LC3) and Beclin1 in brain tissue were analyzed by Western blot?ting assay. RESULTS Intracerebroventricular injection of Aβ25-35 could reduce the total distance from (3984±321)cm to (2790±306)cm and extend central grid residence time from (3.6±1.2)s to (8.8±2.9)s in the open-field test. The escape latency of water maze also increased from (22.0 ± 1.9)s to (38.8 ± 2.2)s. Autophagic vacuoles or late autophagic vacuoles and increased Beclin1 and LC3 and protein level were observed in the hippocampus after Aβ25-35 injection. Intraperitoneal injection of Baicalin 50 and 100 mg · kg-1 for fifteen consecutive days extended the total distance in open-field test to (3705 ± 337)cm and (3968 ± 448)cm, respectively, while the central grid residence time was reduced to (5.6 ± 1.8)s and (3.9±1.5)s, respectively. The total time taken to reach the platform in water maze test was reduced to (28.6± 1.9)s, (22.9 ± 1.7)s. Mitochondrial swelling, vacuolar membrane structure or autophagic vacuoles were visible in the hippocampus. LC3 and Beclin1 protein expression was significantly up-regulated(P<0.01). CONCLUSION Baicalin shows protective effect against Aβ25-35 induced learning and memory deficits, and this effect may be related to the activation of autophagy in the mouse hippocampus.

OBJECTIVETo prevent chronic severe hepatitis, even more fulminant hepatic failure (FHF) occurrence in patients with chronic hepatitis B of severe degree using steroid.

METHODS120 patients were randomized into conventional supporting treatment and steroid treatment groups. The latter, 62 patients were given intravenously hydrocortisone sodium succinate at the dose of 150 mg to approximately 200 mg everyday plus support care.

RESULTSThe rate of deteriorating to chronic severe hepatitis in steroid treatment group was significantly lower than that of conventional group (22% vs 48%, x(2) =7.60, P<0.01). 53.6% (15/28) patients with chronic severe hepatitis in conventional group died, while only 28.6% (4/14) in steroid treatment group succumbed to terminal liver disease (x(2)=0.02, P>0.05). There was no difference between the two groups regarding to complications incidence: gastrointestinal bleeding and infections except for some controllable serious reverse events, such as candidiasis, diabetes, herpes zoster and pulmonary tuberculosis found in some patients in steroid-treated group.

CONCLUSIONThese results suggest that steroid administration with improved support care not only is likely to prevent chronic severe hepatitis occurrence in patients with chronic viral hepatitis of severe degree, but also shows some efficacy for FHF, which warrant further investigation.

Adult ; Female ; Glucocorticoids ; therapeutic use ; Hepatitis B, Chronic ; complications ; drug therapy ; Humans ; Liver Failure ; prevention & control ; Male

BACKGROUND AND PURPOSE: The receptor for advanced glycation end products (RAGE) may contribute to the development of diabetic neuropathy. To assess its relevance in humans, this study examined the expression of RAGE in the skin biopsy samples of patients with diabetes mellitus, and investigated its correlation with intraepidermal nerve-fiber density (IENFD) and clinical measures of neuropathy severity. METHODS: Forty-four patients who either had type 2 diabetes or were prediabetes underwent clinical evaluation and a 3-mm skin punch biopsy. The clinical severity of their neuropathy was assessed using the Michigan Diabetic Neuropathy Score. IENFD was measured along with immunohistochemical staining for RAGE in 29 skin biopsy samples. The expression of RAGE was also quantified by real-time reverse-transcription PCR in the remaining 15 patients. RESULTS: RAGE was localized mostly in the dermal and subcutaneous vascular endothelia. The staining was more intense in patients with a lower IENFD (p=0.004). The quantity of RAGE mRNA was significantly higher in patients with severe neuropathy than in those with no or mild neuropathy (p=0.003). The up-regulation of RAGE was related to dyslipidemia and diabetic nephropathy. There was a trend toward decreased sural nerve action-potential amplitude and slowed peroneal motor-nerve conduction with increasing RAGE expression. CONCLUSIONS: The findings of this study demonstrate up-regulation of RAGE in skin biopsy samples from patients with diabetic neuropathy, supporting a pathogenic role of RAGE in the development of diabetic neuropathy.
Biopsy* ; Diabetes Mellitus ; Diabetic Nephropathies ; Diabetic Neuropathies* ; Dyslipidemias ; Glycosylation End Products, Advanced* ; Humans ; Michigan ; Polymerase Chain Reaction ; Prediabetic State ; Rage ; RNA, Messenger ; Skin* ; Sural Nerve ; Up-Regulation* ; Advanced Glycosylation End Product-Specific Receptor