Objective To evaluate the relationship between damages of visual field and retinal nerve fibre layer(RNFL)thickness in primary open angle glaucoma(POAG)with high myopia.Design Retrospective,case-controlled study.Participants POAG group with high myopia(21 eyes of 17 cases),POAG group with non-high myopia(17 eyes of 16 cases),high myopia group without POAG(25 eyes of 20 cases)and normal control group(19 eyes of 17 cases).Methods The static central visual fields were tested with Humphrey 750 automated perimetry and thickness of RNFL was measured with optical coherent tomography(OCT).Main Outcome Measures Mean deviation(MD),pattern standard deviation(PSD)and mean sensitivity at upper,lower,nasal and temporal quadrants in total deviation probability plots.Thickness of RNFL at upper,lower,nasal and temporal quadrants.Results There were more obvious general depression of sensitivity in total deviation probability plots of the early POAG with high myopia than those of POAG without high myopia,and the early visual field defects of glaucoma in pattern deviation probability plots of this group.MD of POAG with high myopia was more than those of others(P＜0.05).The differences of MD,PSD and mean sensitivity between POAG with high myopia and others were significant(P＜0.05).Mean sensitivities in each quadrant of POAG without high myopia were similar to those of high myopia(P＞0.05).The thickness of RNFL of POAG with high myopia was thinner than those of others and the thickness of RNFL of normality was thicker than that of others.The correlationship between mean sensitivity and the thickness of RNFL in each quadrant was significant(P＜0.05).Conclusion Pattern deviation probability plots is an important favor for judgement of the visual field changes in POAG with high myopia.The relationship between RNFL thickness with OCT and visual field damage may provide clinically relevant information in diagnosis of POAG with high myopia

Objective To analyze white matter hyperintensities(WMH)and brain volumes quantitatively in patients with Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy(CADASIL)on conventional MRI and correlate them with clinical parameters.Methods Fifteen patients with CADASIL confirmed by pathological investigation underwent conventional MRI examinations.Involvement of centrum semiovale,posterior limbs of the internal capsule,corpus callosum,external capsules and white matter of the temporal poles was determined by an experienced radiologist.Normalized brain volume(NBV)and the percentage of WMH to brain volume were calculated with softwares and they were correlated with NIHSS and MMSE statistically.Results Involvement of white matter in the brains of patients with CADASIL included:semiovale centrum in 13 cases,white matter of the temporal poles in 10 cases,external capsules in 8 cases,posterior limbs of the internal capsule in 5 cases,corpus callosum in 4 cases.The percentage of WMH to brain volume was(5.7±1.4)%,and the NBV was(1602±58)×103mm3.Spearman test showed a significant relationship between age and NBV(r=-0.555,P＜0.05).There was a significant relationship between NBV and NIHSS(r=-0.624.P＜0.05).The percentage of WMH to brain volume correlated significantly with NIHSS and MMSE(r=0.522,P＜0.05;r=-0.679,P＜0.01).Conclusions The white matter hvperintensities and brain volume in patients with CADASIL can be assessed quantitatively,which showed correlation with severity of the clinical scale.The development of WMH may refleet the degree of cognitive impairment in CADASIL.

Fatal Outcome ; Humans ; Infant, Newborn ; Leukemia ; blood ; congenital ; diagnosis ; Leukocyte Count ; Male

The ultimate goal of post-genome research is to understand a complete set of proteins in a living organism for their expression pattern and biological function,which is called proteomics.One of the major challenges in proteome research is to study the protein-protein interactions,and the emerging bioinformatics approaches present us tremendous advantages when dealing with protein interaction networking and data analysis.Useful bioinformatics tools include protein-protein interaction network mapping,topology of the network,structure of the module and comparison of the network.The technology advancement in this field brings further understandings to the structure and function of cells at the proteome level,which may eventually lead to the discovery of new drug targets and design methods.This paper attempts to review the current researches on protein-protein interaction with an emphasis on bioinformatics intervention,and also summarizes some widely used methods for network analysis.

Objective To investigate the clinical, electrophysiological and pathological features of critical illness polyneuropathy and myopathy (CIPNM). Methods The clinical outcomes, electromyogram Results as well as pathological features in nerves and muscles of 3 patients with CIPNM were investigated and analyzed. Results 3 patients were all provided with assisted respiration after tracheal intubation. 7～10 d after intubation, all the patients emerged muscle strength and tendon reflexes of extremities weakening; while 14 days after that, 2 patients appeared amyotrophy of extremities. Electromyogram showed that the conduction of many motor and sensory nerves for extremities decreased, while the amplitude of compound muscle action potential (CMAP) of part of motor nerves decreased. Biopsy for nerves revealed decreased medullated nerve fibers and regeneration phenomenon of auxiliary fibers; while that for muscles showed neuralgic damage and myopathy-like changes. Conclusion CIPNM can complicate after tracheal intubation. The electrophysiological and pathological examinations for nerves and muscles can be helpful for the diagnosis.

Objective To assess retinal arteriole stenosis in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and to evaluate the relationship between retinal arteriole stenosis with cranial MRI changes and clinical features. Methods Sixteen CADASIL patients (mean age was (43.4±8.1)years, mean duration was(4.7±3.4)years)and sixteen age matched healthy individuals were enrolled. Mini-Mental State Examination (MMSE) and modified Rankin scale scoring were performed in 16 patients. Cranial MRl with Coulthard scores was assessed on CADASIL patients as well. Retinal examinations for arteriole stenosis were done for each subject. Bivariate correlations (Kendall's tau-b) were used to assess the relationship among the grade of retinal stenosis, MMSE, modified Rankin seale, cranial MRI with Coulthard scores and age. Results Retinal arteriole stenosis presented in 15 cases. in whom 4 cases showed mild arterio-venous nicking. while it only presented in 2 controls. Grade 0.Ⅰ, and Ⅲ of retinal arteriole stenosis are noted in 1/16,4/16,7/16 and 4/16cases respectively. Coulthard scores of cerebral MRl were 6.0.19.0.25.1 and 29.8 respectively from Grade 0 toⅢ of retinal arteriole stenosis. The correlation coefficient of retinal arteriole stenosis and cranial MRI scores was 0.743(P＜0.001＝,MMSE-0.429(P＜0.05＝,modified Rankin scale 0.437(P＞0.05).and age 0.299(P＞0.05).Conclusion Retinal arteriole stenosis is significantly correlated with lesion in cranial MRI and dementia.

Objective To analyze the dynamic evolution of brain MRI in patients with mitochondrial myopathy,encephalopathy,lactic acidosis,and stroke-like episodes (MELAS) syndrome.Methods A retrospective study was performed on 58 MELAS cases with pathologically and (or) molecularly confirmed diagnosis.MRI were repeated within 60 days after the onset of stroke-like episodes (SLE) and the evolution changes of cerebral lesions were accessed.Brain atrophy index (BAI) was calculated in the remission stage from 31 patients with MELAS,and the correlation between BAI,age and disease duration was analyzed.Results The proportion of lesions expansion,migration and shrink within 30 days after the onset of SLE was 64.1％ (25/39),10.2％ (4/39),17.9％ (7/39),respectively,and 13％ (3/23),21.7％ (5/23),56.5％ (13/23),between 30-60 days after the onset of SLE respectively.In the recovery stage of SLE,the BAI in 31 patients with MELAS was 15.2％ ±2.8％.The correlation coefficient between BAI and the age,total disease course and duration of encephalopathy was 0.329 (P =0.043),0.405 (P =0.012) and 0.649 (P =0.000).Conclusions Brain atrophy in the studied MELAS patients gradually develops and strokelike lesions shrink with progression of the disease.However,the migration of lesions is persistent.

Objective To report the clinical and pathological characteristics of one patient with glycogen storage disease Ⅳ (Anderson disease). Methods The patient was received detailed clinical examinations, ultrasound, electromyography, head MRI and muscle biopsy. Results The onset of the 22 years old male patient was 7yrs. The main symptoms were intolerance and fatigue in proximal limbs muscular movement, cardiopalmus by chance. Abdominal ultrasound examinations showed cirrhosis, portal hypertension, splenomegaly. Echocardiogram showed left ventricular myohypertrophia, mild mitral and tricuspid valve insufficiency. Electrophysiology study revealed widespread myogenic changes. Cranial MRI, MRA and MRS were normal. Muscle biopsy showed basophilic intracytoplasmic material in a lot of fibers deposits, which was intensively PAS-positive material and partially resistant to diastase digestion. In the electron microscope, the storage material consisted of filamentous and finely granular material. Conclusions There was the first case of glycogen storage disease Ⅳ reported in our country, mainly involved skeletal muscle, liver, spleen and cardiac muscle.

Objective To investigate the pathological changes and pathogenesis of the MELAS syndrome (mitochondrial encephalopathy lactic acidosis strok-like episodes ) by using the method of immunohistochemical staining in the brain biopsy specimens with anti-mitochondrial antibody (AMA).Methods We performed immunohistochemical staining in 3 confirmed MELAS patients' paraffin-imbed brain biopsy specimens.Results Small vessel proliferation and the uneven thickness of the wall were found in the 3 MELAS patients.A lot of brown deposits was shown in the wall of small vessels and also noted in neurons.Conclusions The main pathological change in the MELAS brain biopsy immunohistochemical staining with AMA was the small vessel proliferation,indicating that abnormal mitochondria accumulated in the vascular smooth muscle,endothelial cell and neurons of the lesion sites.This finding was consistent with the electron microscopic discovery and valuable for the diagnosis of MELAS.

Objective To investigate the special expression and diagnostic value of anti-mitochondrial antibody (AMA) in the muscle tissues of patients with mitochondria encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Methods All cases including 10 cases of MELAS, which were diagnosed definitely by clinical, pathological and DNA analysis, and 2 cases of normal control and 3 cases of necrotic muscle fibers, underwent muscle biopsies in order to carry out AMA staining. At the same time the original MGT staining was observed. Results A large number of ragged brown fibers (RBF) were observed under light microscopy with AMA immunohistochemical staining in the muscle tissues of patients with MELAS. But no RBF were observed in control groups. Conclusion RBF might be the specific performance of paraplastic mitochondrial, which was similar to the ragged red fibers, so RBF might be a pathological index in evaluating abnormal mitochondrial function and in diagnosing mitochondriopathy.