[Pay attention to clinical research, diagnosis and treatment of mixed cognitive impairment].

The concept of mixed cognitive impairment, which was put forward on the basis of mixed dementia, has wider implications. It emphasizes that cognitive impairment has not progressed to the early clinical stages of dementia. In recent years, the focus of dementia research has shifted to the "pre-dementia stage", and more attention has been paid to the influence of vascular factors on cognitive impairment. To a certain extent, vascular risk factors and cerebrovascular diseases can be intervened. Therefore, paying attention to the early clinical identification of mixed cognitive impairment, and strengthening the research and intervention of vascular factors in cognitive impairment has important clinical significance for the effective prevention and treatment of dementia.

[Morphological and histopathological evaluation of temporomandibular joint osteoarthrosis induced by perforation of articular disc in rats].

Objective: To establish the animal model of temporomandibular joint osteoarthrosis (TMJOA) in rats induced by perforation of articular disc and evaluate the morphological characteristics and histopathological changes of the condyle of temporomandibular joint (TMJ). Methods: Eight male SD rats, purchased from Chongqing Medical University, were marked from No. 1 to No. 8 according to a random number table. The eight numbered rats were then arranged according to their sizes from large to small. The first four rats were divided into the one-week group, while the last four were divided into the four-week group. In order to simulate a TMJ disc perforation, an oblique incision was created along the zygomatic arch of the left side (the model side) making the TMJ superior joint space exposed. The left disc was pulled out and artificially perforated by a dental round bur with 1.5 mm diameter at the postmedian part of TMJ disc. The disc of the right side (the control side) was kept intact. The rats were randomly euthanized respectively at 1 week or 4 weeks (n=4 for each time point) for further study. Morphological observation and imaging examination were used to assess the degree of articular cartilage degradation and the loss of subchondral bone. Immunohistochemicalstaining (IHC), safranin O and fast green staining, tartrate-resistant acid phosphatase (TRAP) staining and haematoxylin staining were performed to evaluate the histopathological changes of condylar cartilage. Results: In the morphological observation, significant redness and swelling were observed on the model side of the 1-week-group and the typical beak-like change of osteoarthropathy was observed on the model side of the 4-week-group. The heights of condylar process on the model sides in the 1-week-group and 4-week-group [(2.73±0.14) and (2.49±0.25) cm, respectively] were significantly lower than that in the control side [(3.30±0.09) and (3.30±0.12) cm, respectively] (P<0.01). Imaging examination showed obvious damage in trabecular microstructure on the model side of the 4-week-group. Safranin O and fast green staining showed that the amounts of proteoglycan on the model sides of the 1-week-group and the 4-week-group were significantly lower than that on the control sides (P<0.01). TRAP staining showed that the numbers of osteoclasts on the model side of the 1-week-group and the 4-week-group was significantly higher than that on the control sides (P<0.01). IHC staining and Haematoxylin staining showed that the mean optical densities of collagen on the model side of the 1-week-group and the 4-week-group was significantly reduced compared with that on the control sides (P<0.01), while the mean optical densities of matrix metalloproteinase increased significantly (P<0.01). Conclusions: After making a articular disc perforation, the condylar cartilage on the model side presented typical TMJOA pathological changes.

Improvement of the Irrigation Scheme in the ORCHIDEE Land Surface Model and Impacts of Irrigation on Regional Water Budgets Over China.

In China, irrigation is widespread in 40.7% cropland to sustain crop yields. By its action on water cycle, irrigation affects water resources and local climate. In this study, a new irrigation module, including flood and paddy irrigation technologies, was developed in the ORCHIDEE-CROP land surface model which describes crop phenology and growth in order to estimate irrigation demands over China from 1982 to 2014. Three simulations were performed including NI (no irrigation), IR (with irrigation limited by local water resources), and FI (with irrigation demand fulfilled). Observations and census data were used to validate the simulations. Results showed that the estimated irrigation water withdrawal ( W ) based on IR and FI scenarios bracket statistical W with fair spatial agreements ( r = 0 . 68 ± 0 . 07 ; p < 0 . 01 ). Improving irrigation efficiency was found to be the dominant factor leading to the observed W decrease. By comparing simulated total water storage (TWS) with GRACE observations, we found that simulated TWS with irrigation well explained the TWS variation over China. However, our simulation overestimated the seasonality of TWS in the Yangtze River Basin due to ignoring regulation of artificial reservoirs. The observed TWS decrease in the Yellow River Basin caused by groundwater depletion was not totally captured in our simulation, but it can be inferred by combining simulated TWS with census data. Moreover, we demonstrated that land use change tended to drive W locally but had little effect on total W over China due to water resources limitation.

MIIP inhibits malignant progression of hepatocellular carcinoma through regulating AKT.

OBJECTIVE: This study was aimed to investigate the expression characteristics of MIIP in hepatocellular carcinoma (HCC), and to further explore whether it can inhibit the malignant progression of this disease via regulating AKT expression. PATIENTS AND METHODS: Real-time quantitative PCR (qRT-PCR) was performed to examine the expression of MIIP in tumor and paracancerous tissue specimens of 39 patients with HCC, and to analyze the interplay between MIIP expression and clinical indicators and prognosis of HCC patients. At the same time, in HCC cell lines, the expression of MIIP was further verified using qRT-PCR. In addition, MIIP overexpression and knockdown models were constructed using lentivirus in HCC cell lines (Bel-7402 and Hep3B), and the influence of MIIP on the biological function of HCC cells was analyzed through CCK-8 and transwell migration assays. Finally, luciferase reporting assay and cell reverse experiments were applied to further explore the potential molecular mechanism and the interaction between MIIP and AKT. RESULTS: The results of qRT-PCR showed that the expression level of MIIP in HCC tissue samples was remarkably lower than that in adjacent ones, with a statistically significant difference. Compared with patients with high expression of MIIP, patients with low MIIP expression had a higher occurrence of distant metastasis and a lower overall survival rate. Similarly, compared with control group, the proliferation and migration ability of HCC cells in MIIP knockdown group (sh-MIIP) was remarkably enhanced, while the opposite result was observed in MIIP overexpression group. In addition, qRT-PCR results also revealed that AKT and MIIP were negatively correlated in HCC tissues. At the same time, the results of luciferase reporter gene assay demonstrated that MIIP can be targeted by AKT through certain binding site. Additionally, cell reverse experiment found that there might exist a mutual regulation between MIIP and AKT, thereby jointly regulating the malignant progression of HCC. CONCLUSIONS: MIIP expression is remarkably decreased both in HCC tissues and cell lines; meanwhile, the low expression of MIIP is positively correlated with the occurrence of distant metastasis and poor prognosis of patients with HCC. In addition, MIIP may be able to inhibit the malignant progression of HCC by modulating AKT expression.

Cardiac iron measurement and iron chelation therapy in patients with ß thalassaemia major: experience from Taiwan.

BACKGROUND: The aims of our study were to evaluate (i) the relationship between cardiac T2* values and cardiac complications in Asian ß-thalassaemia major (TM) patients, and (ii) the association between cardiac T2* values and other parameters currently used to predict cardiac complications as a result of transfusion iron overload. METHODS: We examined the myocardial iron loads of 88 TM patients from Taiwan with cardiac T2* magnetic resonance imaging (MRI) and assessed the correlation between cardiac T2* values and serum ferritin levels, liver iron concentration and left ventricular ejection fraction (LVEF). We also determined the predictive value of these measurements for the development of arrhythmia. RESULTS AND CONCLUSION: In our group of Taiwanese patients, the relative risk for arrhythmia was 10·36 when cardiac T2* values were less than 10 ms (compared with ≥10 ms) and 1·98 when serum ferritin levels increased >2500 ng mL(-1) (compared with ≤2500 ng mL(-1) ). Serum ferritin levels correlated with cardiac T2* values in patients with abnormal myocardial iron loads (T2* < 20 ms, r = -0·48, P = 0·004, n = 34), but LVEF (measured by echocardiography) gave no indication of excess myocardial iron deposition (r = -0·07, P = 0·52) or of the risk of developing arrhythmia.

Isolated right diaphragmatic eventration mimicking congenital heart disease in utero.

Diaphragmatic eventration is the upward displacement of the abdominal viscera secondary to a thin or paralytic diaphragm. Its clinical presentations and radiographic pictures are similar to those of diaphragmatic hernia. Prenatal diagnosis of diaphragmatic eventration is extremely rare. A pregnant woman was referred to us because of abnormal cardiac findings noted at 20 weeks of gestation. A diagnosis of partial anomalous pulmonary venous connection was made on the basis of our findings of right atrial enlargement with an abnormal vascular channel drainage to it. The infant was born via cesarean section at 40 weeks and developed complications of cyanosis immediately after birth. Postnatal imaging studies and surgical findings disclosed right side diaphragmatic eventration with liver and associated vasculature upward displacement into the right pleural cavity. The cardiac structure was otherwise normal. We conclude that when an abnormal vessel tracing and unexplainable cardiac chamber asymmetry is encountered, diaphragmatic eventration should be considered as one of the differential diagnoses. Correct recognition and transferral to the hospital for neonatal assistance may lead to timely and appropriate management of these fetuses.

Prenatal three-dimensional ultrasound and magnetic resonance imaging evaluation of a fetal oral tumor in preparation for the ex-utero intrapartum treatment (EXIT) procedure.

Recent attempts at predelivery management of obstructed fetal airways have focused on the EXIT (ex-utero intrapartum treatment) procedure, which allows sufficient time to secure the fetal airway through preservation of uteroplacental gas exchange. We report a fetus with an exophytic oral tumor noted at 34 weeks of gestation. In this case, three-dimensional (3D) ultrasound allowed a complete and interactive evaluation of the tumor and related facial anatomy, and confirmed that access to the fetal airway was unlikely during delivery. Fetal magnetic resonance imaging (MRI) further demonstrated that the tumor originated in the nasopharynx and obstructed the upper airway. Both imaging results led to a final decision to offer an EXIT procedure for the neonate. At 36 weeks' gestation, a successful EXIT procedure was performed to reduce the risk of respiratory distress immediately after birth. This report highlights the value of 3D ultrasound and MRI as essential prerequisites for optimization of the triage process in selecting EXIT candidates.

Three-dimensional ultrasound diagnosis of Larsen syndrome with further characterization of neurological sequelae.

Larsen syndrome consists of skeletal dysplasia with multiple joint dislocations and a characteristic facies. The basis of this abnormality is a generalized mesenchymal disorder involving connective tissues. We describe our findings in a woman who was referred at 28 weeks' gestation due to multiple fetal anomalies suspected initially at an 18-week ultrasound examination. On three-dimensional (3D) ultrasound we found the fetus had bilateral genu recurvatum. Further 3D examination at 36 weeks confirmed the lower limb anomaly and revealed facial anomalies that led to the diagnosis of Larsen syndrome. An elective Cesarean section was performed at 38 weeks' gestation to minimize neurological sequelae. Magnetic resonance imaging was performed postnatally and showed pachygyria, colpocephaly and agenesis of the corpus callosum. In this case, 3D ultrasound facilitated the prenatal diagnosis of Larsen syndrome. A careful prenatal investigation for other associated anomalies such as those of the cardiovascular or neurological systems is warranted with this diagnosis. These associated lesions are likely to have a greater impact on prognosis than the classic symptoms of Larsen syndrome and a collaborative approach is necessary to optimize delivery and postnatal management of an affected fetus.

Early epileptic encephalopathy with suppression burst electroencephalographic pattern--an analysis of eight Taiwanese patients.

Early epileptic encephalopathy with suppression burst (SB) comprises two distinct epileptic syndromes, early infantile epileptic encephalopathy (EIEE) and early myoclonic encephalopathy (EME). We reviewed etiologies, neurological outcome and clinico-electroencephalographic features of EIEE and EME. Chart records of early epileptic encephalopathy with SB from January 1997 to December 2000 were reviewed. These cases fulfilled the diagnostic criteria of EIEE and EME. Totally eight patients (four females, four males) were enrolled. They consisted of three cases of EIEE and five cases of EME. The follow-up periods ranged from 6 to 30 months. For EIEE, two cases had migrational disorders, and one was cryptogenic; for EME, three cases had non-ketotic hyperglycinemia (NKH), one was pyridoxine dependency and one was cryptogenic. The main initial seizure patterns were tonic spasms in EIEE, and were erratic myoclonus in EME. The age of seizure onset ranged from 26 h to 5 days after birth for EIEE, and 2 h to 7 days of life for EME. The SB pattern in the electroencephalography (EEG) was noted mainly during sleep state in EME, but in both awake and sleep states in EIEE. Asymmetric SB pattern and background activities in EEG were found in migrational disorders. The EEG in all cases of EIEE changed to hypsarrhythmia at 4-6 months of age. In EME, only the EEG in cases of NKH evolved to hypsarrhythmia. Response to anti-convulsants was generally poor. All had severe psychomotor retardation. Although EIEE and EME share several common features, differences in terms of seizure seminology and evolution, EEG patterns and etiologies still exist.

Magnetic resonance cholangiopancreatography diagnosed pancreatitis associated choledochal cyst: report of one case.

This report describes a 6-year-old girl with a choledochal cyst associated with recurrent pancreatitis. A cystic dilatation of the common bile duct was detected by abdominal ultrasound and magnetic resonance cholangiopancreatography (MRCP). She displayed only one of the classic triads: abdominal pain plus pancreatitis. Cyst excision and Roux-en-Y hepaticojejunostomy was indicated in this case. MRCP can be considered as a unique non-invasive tool and the first choice in evaluation of choledochal cyst in pediatric group.

Prenatal depiction of angioarchitecture of an aneurysm of the vein of Galen with three-dimensional color power angiography.

A fetus with a large supratentorial cyst and cardiomegaly was encountered at 33 weeks of gestation. The cyst appeared as an aneurysmal, fluid-filled structure extending posteriorly with a finger-like appendage. Using color flow mapping, we disclosed rapid in-and-out blood flow with marked turbulence within the cyst. For evaluation of its blood supply and venous drainage of the vascular malformation, a three-dimensional reconstruction of the power Doppler image was conducted. The results revealed that the vascular malformation was supplied by a small contralateral aneurysm from the branches of Willis' circle, draining posteriorly into an abnormal falcine sinus and then into the superior sagittal sinus. No other fetal abnormality such as hydrocephalus or hydrops was discovered. The prenatal diagnosis of an aneurysm of the vein of Galen was made on the basis of the gray-scale, color Doppler findings, and also the angioarchitecture obtained by three-dimensional power Doppler imaging. The woman was admitted at 37 weeks of gestation due to labor onset and delivered the baby via the vaginal route without complication. Postnatal angiography and magnetic resonance imaging confirmed the diagnosis of an aneurysm of the vein of Galen, and the angioarchitecture depicted it before birth. We suggest that three-dimensional power Doppler ultrasonography may assist in the diagnosis of an aneurysm of the vein of Galen, and precisely delineate the complicated corresponding vasculature. This may guide postnatal management and predict the prognosis more accurately.

The preoperative and postoperative cerebral blood flow and vasoreactivity in childhood moyamoya disease.

PURPOSE: To report the hemodynamic pattern in childhood moyamoya disease before and after vascular reconstruction. METHODS: We performed the xenon cerebral blood flow (CBF) study in 10 cases of moyamoya disease in last 3 year. In 6 of them, study before and after operation was obtained for comparison. Preoperative study was obtained in 2 cases and postoperative study was obtained in 2 cases. They were 5 males and 5 females with age ranging from 1 to 12 years old. We studied their regional CBF before and after acetazolamide (CCBF) using stable xenon computed tomography. The angiography study was obtained for comparison. RESULT: In the routine study, the CBF and CCBF were poor in the ACA and MCA territory and well correlated to the angiography finding. However, in case of basal ganglion and posterior cerebral artery involvement, xenon CBF study was more sensitive than the angiography. On the postoperative cases, the CBF could increase to a level beyond the level of CCBF shown on the preoperative study. CONCLUSION: Hemodynamic study using stable xenon computed tomography can provide an objective reference for the treatment of moyamoya disease especially in case that surgical management is considered. It is a better modality for the evaluation of surgical outcome than the conventional contrast angiography study.

3-D power Doppler cerebral angiography in neonates and young infants: comparison with 2-D power Doppler angiography.

The aims of this study were to evaluate the ability of 3-dimensional (3-D) power Doppler angiography (3DPDA) to depict the intracranial vasculature in infants, to compare with 2-D power Doppler ultrasonography (2DPDU), and to explore the potential clinical applications of this procedure in young infants with brain disorders. We performed 3DPDA in 27 infants. 2DPDU were completed in both sagittal and coronal directions in 12 of these patients. In the other 15, only right sagittal plane images were available for comparison. Using a grading system and with only vessels with more than half of the length demonstrated included for comparison, we compared the Doppler signals of major vessels. 3DPDA could have good visualization in more than 60% of the internal carotid artery, ophthalmic artery, pericallosal artery, callosomarginal artery, internal cerebral vein, vein of Galen, and straight sinus in the sagittal plane. 3DPDA also could have good demonstration in about 50% of basilar artery in coronal plane, and posterior communicating artery, posterior cerebral artery, and lenticulostriate artery in sagittal plane. 3DPDA was better than 2DPDU in demonstrating all the major intracranial vessels in different planes, except the anterior communicating artery. In the anterior communicating artery, neither can demonstrate more than 30%.

Niemann-Pick disease type C (a cellular cholesterol lipidosis) treated by bone marrow transplantation.

Bone marrow transplantation (BMT) has been used for a wide variety of lysosomal storage diseases with encouraging results. We report a 3-year 5-month-old girl with Niemann-Pick type C disease (NPC) who received an allogeneic BMT. The patient presented with repeated lower respiratory tract infections, hepatosplenomegaly, failure to thrive, and developmental delay. Chest computed tomography (CT) revealed diffuse interstitial lung infiltration. Bone marrow and liver biopsies revealed abundant lipid-filled foamy macrophages. Skin fibroblast sphingomyelinase assay revealed partial deficiency. The ability of her skin fibroblasts to esterify cholesterol was very low, and the cells stained brightly for free cholesterol. She received BMT from a healthy HLA-identical male sibling donor at the age of 2 year 6 months. Full engraftment was evidenced by repeated bone marrow sex chromosome studies. Regression of the hepatosplenomegaly, markedly reduced foamy macrophage infiltration in bone marrow, and decreased interstitial lung infiltration was noted 6 months after BMT. Her neurological status, however, deteriorated. Follow-up magnetic resonance image (MRI) revealed progressive, diffuse brain atrophy. We conclude that resolution occurred in the liver, spleen, bone marrow and lung following successful engraftment. Such a response is remarkable since the underlying problem involves a membrane receptor for cholesterol. This positive response might be due to replacement of the monocyte-phagocytic system or it may imply the existence of cross-correction in the NPC membrane receptor defect by BMT approach. Since BMT did not halt the neurological deterioration, it is unlikely to be an adequate treatment for NPC.