ObjectiveTo describe the MR features of Rasmussen encephalitis (RE).Methods The MRI of 10 pathologic confirmed patients (7 male,3 female,mean age 11 ± 4 years) with RE were retrospectively analyzed in this study.Routine axial,sagittal and coronal (perpendicular to the oblique long axis of the hippocampus) scans were obtained for T1WI,T2WI and fluid-attenuated inversion recovery (FLAIR)images. The location and degree of cerebral atrophy,gray matter signal changes,and the evolution of these findings were evaluated. Results Brain atrophy included the enlargement of lateral ventricle(8/10),temporal horn (9/10)and lateral fissure (9/10); widened sulci and small gyri in the isolateral hemisphere (7/10) ; atrophy in caudate and putamen nucleus (6/10).The cortical atrophy was extensive at late stage of the RE,and usually was hemispheric or involved more than two lobes.The signal changes included hyperintensity involving extensive cortical and/or subcortical regions (9/10). The follow-up MR study demonstrated the progression of brain atrophy and extensive signal changes.Conclusions RE usually presents in pediatric patients. The imaging findings included progressive unilateral brain atrophy,enlargement of lateral ventricle,lateral fissure and sulci,and small gyri with or without cortical T2hyperintensity.Deep nucleus atrophy may be involved in RE.

Objective To obtain suitable hearing screening methods for preschool children.Methods A total of 2025 children aged 2~6 years old in 30 kindergartens in Huangshi City were selected by random sampling method.Acoustic impedance and otoacoustic emission tests(transiently evoked otoacoustic emissions and distortion product otoacoustic emissions) were performed in two stages of preliminary hearing screening.The children who failed the hearing screening needed to do the re-creening with the same methods;the children who failed the rescreening needed to receive audiological tests including ABR, ASSR examination and imaging examinations.Results The total screening pass rate was 94.02%, of which 1 842 passed the preliminary hearing screening(90.96%, 1 842/2 025).The 183 children who failed the preliminary hearing screening received the re-screening, 62 children passed the re-screening(33.88%,62/183).121 children failed the re-screening(5.98%,121/2 025), and finally 72 children(3.56%,72/2 025)were diagnosed with hearing loss, including 47 cases of otitis media,22 cases of sensorineural hearing loss(8 cases were moderate, 4 cases were severe hearing loss,10 cases were profound);18 cases were unilateral hearing loss while 4 cases were bilateral hearing loss.Conclusion Acoustic impedance and otoacoustic emission tests can be used for hearing screening in preschool children.The hearing problems of preschool children in Huangshi City were concentrated mainly in the middle ear secretory otitis media and different degree of sensorineural hearing loss.

Objective To explore the correlations of the MRI findings and its pathological typing in the focal cortical dysplasia (FCD) .Methods MR images of 74 patients with FCD confirmed by operation and histopathologic examination were analysed retro‐spectively .MRI findings with FCD were divided into three subtypes including radial band type ,hyperintensity type and mild type . The correlation of the FCD MRI findings and pathological typing is analysed .Results In 74 patients with FCD ,there were radial band type in 12 cases ,hyperintensity type in 32 cases ,and mild type in 30 cases respectively .M RI finding of radial band type FCD showed a tail of increased T2WI/FLAIR signal tapering down to the lateral ventricle .Hyperintensity type FCD showed increased T2 WI/FLAIR signal in the cortex and subcortical white matter ,accompanied with focal cortical thickening .Mild type FCD showed T2 WI/FLAIR subtle hyperintense signal in cortex with or without focal cortical thickening ,but there was no hyperintense signal in subcortical white matter .Most of radial band type FCD were ⅡB in pathology .Most of hyperintensity FCD were ⅡA and ⅡB .Mild type FCD was more found to beⅠA orⅠB .Conclusion Analysing MRI features would improve the accurate diagnosis of FCD and help to infer the pathological type .

Objective To determine MR manifestations and pathologic types of benign meningiomas and their relationship with tumor recurrence.Methods There were 218 patients (160 females,58 males; age range 4-79 years) with benign meningiomas in the study,including 31 recurrent meningiomas (recurrence group)and 187 primary meningiomas (primary group).All patients were proved by postoperative pathology.Differences of pathological types and MRI manifestations between the recurrence group and the primary group were evaluated by using x2 test and rank sum test.Logistic regression analysis was performed by taking tumor recurrence as the dependent variable,and age,gender,vital structures involvement and pathologic types as independent variables.The recurrent time intervals were compared by rank sum test.Results There were 30 patients with intracranial vital structures involvement or extreintracranial communication tumors in the recurrent group,which was obviously higher than that of the primary group (61 patients).The difference was statistically significant (x2 =57.672,P =0.001).The tumors located in the skull-base and juxtasinus in the recurrent group were obviously more than those in the primary group,and difference was statistically significant (x2 =10.990,P =0.001).Multi-logistic regression analysis showed that the recurrent risk of benign meningiomas was elevated significantly only with vital structure involvement or extre-intracranial communication tumors (wald x2 =31.863,OR =3.820,P =0.001).The recurrent risk of dural sinus involvement was 3.820 times of cerebral artery trunk and cranial nerves involvement,and the risk of the latter was 3.820 times of the non-involved.There was no statistical difference between the two groups in pathology type,location,peritumoral edema,tumor morphology and tumor size.The relapse time of dural sinus involvement and cerebral artery trunk involvement in the recurrent group was 24(13 to 180) and 126(12 to 187) months,respectively.There was significant difference (Z =2.197,P =0.028).Conclusions It is more common that the recurrent benign meningiomas located in the skull base and juxtasinus.The recurrent risk significantly increases when benign meningiomas with vital intracranial structure involved or with extra-intracranial communication tumor.The relapse time of dural sinus involvement is possibly shorter than that of cerebral artery trunk involvement.MRI plays an important role in predicting tumor recurrence and prognosis of benign meningiomas.

Objective To investigate the MRI classifications and imaging findings of dysembryoplastic neuroepithelial tumor(DNET). Methods MR images of 34 patients with pathologic confirmed DNET of Beijing Sanbo Brain Hospital were retrospectively reviewed in this study. The classification was made according to the number of pseudocysts, scope of involvement, morphology and location. Results MRI appearances of DNET were divided into three subtypes: cystic‐like, polycystic‐like and diffuse type. Twelve cases had cystic cortical, including front lobe (5 cases), temporal lobe (5 cases), parietal lobe (2 cases). These cases presented quasi‐circular or oval shape, with hypointense on T1WI and strongly hyperintense on T2WI. T2‐FLAIR was observed hyperintense ring sign in the tumor periphery and the cystic content was close to CSF but having the largest difference to that of CSF, which signal was higher than CSF. Twenty cases were polycystic‐like, front lobe (7 cases), temporal lobe (7 cases), parietal lobe (5 cases), occipital lobe (1 case). In these 20 cases, they had slightly hypointense on T1WI and strongly hyperintense on T2WI. Located in the cortex and subcortical matter, with wedge shape, gyriform or triangle shape.On T2‐FLAIR, internal septation and hyperintense"ring sign"were observed. Two cases were diffuse type, bilateral (1 case), unilateral (1 case). In these 2 cases, diffuse lesions involving multiple areas with hyperintense ring and internal septation on FLAIR, including subcortical white matter, deep nucleus and periventricular area. Conclusions The MR appearances of DNET are variable. Understanding the MR imaging type of DNET might improve the MR diagnosis of DNET.

Objective: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. Method: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A 'very high risk’ group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4. Results: Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients. The median age at diagnosis was 57 (20-84) years, and 22 patients (64.7%) were male. All 34 patients were diagnosed with cardiac involvement, multi-organ involvement was observed in 15 patients (44.1%) , and 27 (81.8%) had New York Heart Association Class Ⅲ or Ⅳ. Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 μg/L, 11 733 ng/L, and 403 mg/L, respectively. Eight (24.2%) had more than 10% plasma cell on the bone marrow aspirate. Sixteen (47.1%) patients received bortezomib based chemotherapy and overall hematologic response rate was 58.3%. Median overall survival (OS) was 4 months. The estimated OS at 3, 6, 12, and 24 months was 51.3%, 44.0%, 35.2%, and 29.6%, respectively. Fourteen (41.2%) patients died within 3 months after the diagnosis. The estimated 1-year survival rate for the patients who got hematologic response, without hematologic response, and palliative treatment was 90.9%, 11.1%, and 0, respectively (P<0.001) . Conclusion Patients with very high risk pAL had very poor prognosis and the early death rate remained high. Those patients who obtained hematologic remission would have significantly better outcomes.

OBJECTIVETo evaluate the usage of Mayo staging system in Chinese patients with primary light chain (LC) amyloidosis.

METHODClinical data, treatment and outcome of 162 primary LC amyloidosis patients with Mayo Clinic staging in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed.

RESULTSThe median age of 162 patients with Mayo Clinic 2004 stage was 57 (20-81) y, of them 62.3% were male. The number of patients with stage I to III were 44 (27.2%), 69 (42.6%), and 49 (30.2%), respectively. The median overall survival was not reached, 23 months and 12 months in patients with Mayo Clinic 2004 stage I, II, and III, respectively (P<0.001). Among 128 patients with Mayo Clinic 2012 stage, 48 patients (37.5%), 32 patients (25.0%), 32 patients (25.0%) and 16 patients (12.5%) were staged as Mayo Clinic 2012 stage 1 to 4, and the median OS was not reached, not reached, 13 months and 3 months, respectively (P<0.001).

CONCLUSIONMayo Clinic staging systems had important prognostic value in patients with primary LC amyloidosis.

Adult ; Aged ; Aged, 80 and over ; Amyloidosis ; diagnosis ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Young Adult

Breast milk is the best source of nutrition for infants, and the microbial communities contained in breast milk can regulate the colonization of bacteria in the intestines of newborns and enhance their immunity.As microbial culture, isolation and identification techniques continue to evolve, more and more breast-milk-derived probiotics are being discovered.Common probiotics in breast milk include Lactobacillus, Bifidobacteria, Philococcus, Streptococcus thermophilus, etc.A large number of studies have proved that breast milk-derived probiotics have a certain role in the treatment of infantile colic, functional constipation, diabetes, mastitis and other diseases, and the natural source of breast milk probiotics, adapted to the milk matrix and other characteristics make it stand out from a group of probiotics.The discovery of probiotics isolated from breast milk and the exploration of their probiotic functions can not only provide an experimental basis for the addition of breast milk-derived probiotics to infant formula, but also provide new prospects for the treatment of various diseases.

Thyroid nodule is a common endocrine system disease in clinic. Its onset is mostly related to affect-mind internal damage. The core pathogenesis is dysfunction of the liver and liver-qi stagnation, and the main pathological changes are qi stagnation, phlegm turbidity and blood stasis. Constitution is inherited from the natural factors and nourished from the postnatal factors, which is closely related to the occurrence of diseases, the transformation of syndromes and the transmission of diseases. Chaihu constitution refers to the type of constitution suitable for long-term use of Bupleuri Radix and prescriptions about Bupleuri Radix. The characteristics of patients with Chaihu constitution are similar to those of patients with thyroid nodules in clinic, and the risk of developing thyroid nodules is higher. Chaihu constitution is the internal basis of thyroid nodules. When treating thyroid nodule patients with Chaihu constitution, the dominant position of Bupleuri Radix should be highlighted, and Bupleuri Radix and prescriptions about Bupleuri Radix should be used flexibly. The dosage of Bupleuri Radix is mostly between 6-15 g, and according to the change of pathogenesis, it is often combined with Chinese materia medica with the efficacy of soothing liver and promoting qi, resolving phlegm and removing turbidity, promoting blood circulation and removing blood stasis, in order to prevent the occurrence and development of thyroid nodules.

Objective To explore the occurrence of fetal chromosomal abnormalities among pregnant women with an adverse reproductive history using traditional karyotyping and single nucleotide polymorphism microarray(SNP-array)technology.Methods Totally 94 in 2 163(4.35%)cases of singleton pregnant women with an adverse reproductive history were performed amniocentesis in Jinhua Maternal and Child Health Care Hospital from June 2015 to June 2017. Traditional karyotyping and SNP-array were employed simultaneously for prenatal diagnosis,and the detection rates of the two methods were compared. Results All of the 94 specimens were successfully analyzed, 11 cases were found with chromosomal anomaly, the overall detection rate was 11.7%(11/94). Seven (7.4%,7/94) abnormalities cases were detected by karyotyping,and 7(7.4%)by SNP-array.The karyotyping results of trisomy 21,and 45,X and the deletion of chromosome 13 were consistent with SNP-array.Only 3(3.2%,3/94)microdeletion/duplications (the sizes of duplications and deletions were between 422.4-1 708.4 kb)and 1(1/4)loss of heterozygosity were detected by SNP-array,but were missed by karyotyping.Furthermore, 2 cases′copy number variation were found pathogenic gene related, while the other 2 were considered benign or variant of uncertain significance. Four cases(4/7)of abnormalities were detected by karyotyping, while confirmed balanced translocation and inversion by SNP-array.All patients were informed and chosen to continue the pregnancy.Conclusions The rate of abnormal fetal chromosomes in pregnant women with an adverse reproductive history is still high.SNP-array is a new molecular genetic technique,and combined with use of traditional karyotyping,it could improve the detection rate of fetal chromosomal abnormalities and reduce abortion rate, thus providing a basis for genetic counseling and prenatal diagnosis.