Objective To study the clinical characteristics by using CT, MRI and biochemistry test on the X linked adrenoleukodystrophy (X ALD) Methods The clinical,neuro imaging and determinations of plasma very long chain fatty acids (VLCFAs) of 12 cases with X ALD were analysed Results The main clinical features consisted of the childhood onset,progressive auditory, visual and/or intelligent impairment, behavioural changes,epileptic seizures and melanodermia. CT or MRI scans demonstrated that the demyelinating lesions were located on the bilateral white matter in occipital,posterior parietal and temporal lobes.The levels of hexacosanoic acid (C 26:0 ) and the ratio of C 26:0 to docosanoic acid (C 22:0 ) were increased. Conclusions ALD is an inherited disease as an X linked trait.The childhood X ALD is characteristiced by progressive auditory, visual and intelligent deterioration.The abnormalities of CT or MRI scan and the levels of the plasma VLCFAs are crucial to the diagnosis of X ALD.

Objective: To observe the effects of electroacupuncture (EA) at Tianshu (ST25) on pro- and anti-inflammatory cytokines in sodium taurocholate-induced severe acute pancreatitis (SAP) in rats. Methods: Sixty-six male Sprague-Dawley rats were randomly divided into three groups: sham-operation group, SAP group and EA group. An SAP model was established by injecting 3.5% sodium taurocholate at a dose of 1 mL/kg through puncturing the pancreatic duct. In the EA group, EA at ST25 was performed with sparse-dense wave (2/100 Hz, 2 mA for 30 min) immediately after sodium taurocholate injection and 30 min before sacrifice. Rats were sacrificed at 3 (n=7), 6 (n=7) and 12 h (n=8) after operation. Levels of tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6) and IL-10 in serum were estimated by enzyme-linked immunosorbent assay. Pathological changes of pancreatic tissues were examined by hematoxylin and eosin staining and observed under a microscope and also scored. Results: The levels of serum TNF-α and IL-6 of SAP rats were significantly higher than those of sham-operation rats at 3, 6 and 12 h. The level of IL-10 in serum of SAP rats was significantly higher than that of sham-operation rats at 3 and 6 h (P<0.05). EA at ST25 down-regulated the levels of TNF-α and IL-6 in serum and up-regulated the level of IL-10 and the ratio of IL-10/TNF-α, also attenuated the morphological damages at 6 and 12 h (P<0.05) in rats with SAP. The pathological scores of pancreatic tissues in the EA group were significantly less than those in the SAP group at 6 and 12 h (P<0.05). Conclusion: EA at ST25 may have a therapeutic effect on rats with SAP by down-regulating the levels of TNF-α and IL-6 and up-regulating the level of IL-10 to re-establish the balance of pro- and anti-inflammatory cytokines, and to ameliorate the inflammatory response of SAP.

Objective:To investigate the imaging and clinical feature of leukoencephalopathy with hearing loss first complaint.Method:The head MRI and clinical feature of 7 cases leukoencephalopathy with hearing loss first complaint were retrospectively analysed,including their place,shape,range and blood plasma very-long-chain fatty acid(VLCFA).Result:The head MRI of 2 cases shows symmetry distributed long T_1 and T_2 signal like butterfly aliform in white matter beside both cornu posterious ventriculi lateralis.The head MRI of 5 cases shows abnormal long T_1 and T_2 signal in different part in Alba.The pathological changes often located in white matter of frontal lobe and secondly beside both eornu posterious ventriculi lateralis.The shape shows little patching in 7 cases.The range only shows beside cornu posterious ventriculi lateralis in 2 cases.The range only shows in frontal lobe in 1 cases.Two or more than two parts shows in the rest 5 cases.The result of VLCFA is normal in 3 cases.The rest 4 cases refused to have examination of VLCFA. Conclusion:The patient who firstly complained of hearing loss should be routinely done head MRI.The head MRI imaging could clearly show abnormal white matter in order to avoid omitting existent pathological changes.

Experimental studies on the myocardial ischemia and reperfusion injury in 16 anaethetized SDrats,of which,8 animals were pretreated with morphine(5 mg/kg,i.p.)for preventing of arrhyth-mias,were studied immunocytochemically with anti-muscle actin specific monoclonal antibody (HHF_(35)),8 shan-operated rats were used as control.With HHF_(35) ABC immunocytochemical method,the area of myocardial ischemia and reperfusion injury(without morphine)showed decrease or ab-sence of staining,large areas of staining loss were also seen.In the group with morphine,only smallfoci of staining absence were shown.The myocardium in control animals showed evenly positive stain-ing.No change were seen with HE staining in all groups.The results obtained with HHF_(35) stainingsupport its important value in studying on myocardic reperfusion injury,and indicated that the degreeof myocardic damage may be relative to the arrhythmias in myocardial reperfusion injury.

OBJECTIVETo compare the clinical effective differences between acupuncture-moxibustion and Chinese herbs for functional dyspepsia with emotional disorder.

METHODSEighty patients were randomly divided into an observation group and a control group, 40 cases in each one. In the observation group, based on the basic treatment Zusanli (ST 36), Yinlingquan (SP 9), Zhongwan (CV 12), Danzhong (CV 17), Neiguan (PC 6), Taichong (LR 3), Ganshu (BL 18), Geshu (BL 17) and Danshu (BL 19) were selected once every day or every other day. The treatment was given 14 times. In the control group, based on the basic treatment individual therapy of Chinese herbs according to syndrome differentiation was applied,one dose a day. Patients were treated for 4 weeks in the two groups. The scores of self-rating depression scale (SDS), the main symptoms and satisfied degree by self-rating for treatment were observed before and after 4-week treatment in the two groups. Also, the rate and the average time of return visit were compared in 6 months after treatment. Results After treatment, the SDS scores and the main symptoms scores were all improved compared with those before treatment in the two groups (all P< 0. 05). After 4-week treatment, the improvement of the SDS score in the observation group was better than that in the control group (48. 9±8. 5 vs 53. 1±8. 0, P<0. 05). After 1-week treatment and 4-week treatment, the improvements of the main symptoms in the observation group were superior to those in the control group (2. 7 ± 1. 0 vs 3. 3±0. 9, 1. 5±0. 9 vs 2. 3±1. 1, both P<0. 05), and the satisfied degree scores by self-rating were better than those in the control group (3. 0±1. 1 vs 3. 8±1. 3, 2. 0±1. 4 vs 2. 9±1. 5, both P<0. 05). In 6 months after treatment,the return visit rate in the observation group was 42. 5% (17/40), and it was lower than 70. 0% (28/40) in the control group (P<0. 05). The average time of return visit in the observation group was less than that in the control group (1. 0±0.8 vs 1. 9±0. 7, P<0. 05).

CONCLUSIONThe effect of acupuncture-moxibustion or functional dyspepsia with emotional disorder is better than that of Chinese medicine.

Acupuncture Points ; Acupuncture Therapy ; Adult ; Aged ; Dyspepsia ; psychology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Moxibustion ; Treatment Outcome

ObjectiveTo study how to diagnose thrombosis of intracranial venous sinus and cerebral venous thrombosis(CVT). Methods6 cases with intracranial venous sinus and CVT were analysed by clinical features and imaging signs. ResultsMost patients had symptoms and signs of intracranial hypertension. Some patients manifested symptoms of whole brain or focal neurological deficits. Magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) play important roles for the diagnosis of intracranial venous sinus and CVT, however, digital subtraction angiography (DSA) is one of the most reliable method for early diagnosis of the above diseases.Conclusions According to clinical features and imaging signs, intracranial venous sinus and CVT could be diagnosed accurately .

OBJECTIVE: To investigate the imaging and clinical feature of leukoencephalopathy with hearing loss first complaint. METHOD: The head MRI and clinical feature of 7 cases leukoencephalopathy with hearing loss first complaint were retrospectively analysed, including their place, shape, range and blood plasma very-long-chain fatty acid (VLCFA). RESULT: The head MRI of 2 cases shows symmetry distributed long T, and T2 signal like butterfly aliform in white matter beside both cornu posterior ventriculi lateralis. The head MRI of 5 cases shows abnormal long T1 and T2 signal in different part in Alba. The pathological changes often located in white matter of frontal lobe and secondly beside both cornu posterior ventriculi lateralis. The shape shows little patching in 7 cases. The range only shows beside cornu posterior ventriculi lateralis in 2 cases. The range only shows in frontal lobe in 1 cases. Two or more than two parts shows in the rest 5 cases. The result of VLCFA is normal in 3 cases. The rest 4 cases refused to have examination of VLCFA. CONCLUSION The patient who firstly complained of hearing loss should be routinely done head MRI. The head MRI imaging could clearly show abnormal white matter in order to avoid omitting existent pathological changes.
Adolescent ; Child ; Child, Preschool ; Female ; Hearing Loss ; diagnosis ; etiology ; Humans ; Leukoencephalopathies ; complications ; diagnosis ; Magnetic Resonance Imaging ; Male ; Retrospective Studies ; Tomography, X-Ray Computed

OBJECTIVETo observe the clinical efficacy differences between electroacupuncture (EA) and regular treatment for severe acute pancreatitis accompanied with paralytic ileus.

METHODSThis was a prospective pragmatic randomized controlled trial. A total of 140 cases of severe acute pancreatitis accompanied with paralytic ileus were randomly assigned into an EA group and a regular treatment group, 70 cases in each one. The patients in the regular treatment group were treated with regular treatment, including intensive care, gastrointestinal decompression, fasting, blood capacity supplement, acid suppression treatment, internal environment maintenance, infection prevention, inhibition of pancreatic exocrine secretion, etc. Based on the regular treatment, patients in the EA group were treated with EA at Zusanli (ST 36) and Zhigou (TE 6), 30 min for each treatment, once a day for totally 5 days. The VAS-based abdominal distension severity scale and abdominal pain severity scale were compared before and during treatment in the two groups, moreover, the number of patients who transferred to surgery or ICU was compared.

RESULTS(1) After the 1st EA, the abdominal pain and distension severity scales were both improved in the EA group, which were superior to those of the regular treatment group (all<0.05); afterwards, the abdominal distension and pain severity scales of each day in the EA group were all significantly superior to those of the regular treatment group (all<0.05). (2) The number of patients who transferred to surgery or ICU was not significantly different between the two groups (>0.05).

CONCLUSIONSEA at Zusanli (ST 36) and Zhigou (TE 6) can significantly reduce the abdominal distension and pain severity scales in patients of severe acute pancreatitis accompanied with paralytic ileus, indicating positive clinical significance; in addition, EA is safe and can be recommended to the treatment of severe acute pancreatitis in combination with treatment plan of integrated Chinese and western medicine.

Chaperone-mediated autophagy (CMA) is a lysosome-dependent selective degradation pathway implicated in the pathogenesis of cancer and neurodegenerative diseases. However, the mechanisms that regulate CMA are not fully understood. Here, using unbiased drug screening approaches, we discover Metformin, a drug that is commonly the first medication prescribed for type 2 diabetes, can induce CMA. We delineate the mechanism of CMA induction by Metformin to be via activation of TAK1-IKKα/β signaling that leads to phosphorylation of Ser85 of the key mediator of CMA, Hsc70, and its activation. Notably, we find that amyloid-beta precursor protein (APP) is a CMA substrate and that it binds to Hsc70 in an IKKα/β-dependent manner. The inhibition of CMA-mediated degradation of APP enhances its cytotoxicity. Importantly, we find that in the APP/PS1 mouse model of Alzheimer's disease (AD), activation of CMA by Hsc70 overexpression or Metformin potently reduces the accumulated brain Aβ plaque levels and reverses the molecular and behavioral AD phenotypes. Our study elucidates a novel mechanism of CMA regulation via Metformin-TAK1-IKKα/β-Hsc70 signaling and suggests Metformin as a new activator of CMA for diseases, such as AD, where such therapeutic intervention could be beneficial.