Objective: To evaluate the response of oral melphalan plus high-dose dexamethasone (MDex) for patients with primary light chain amyloidosis (pAL). Methods: Clinical data, hematological and organ responses, and survival of 76 patients with pAL who had received MDex from January 2009 to July 2017 were retrospectively analyzed. Results: Of 76 patients (47 males and 29 females with the median age of 56 [range, 20-74] years old), 19.70% patients were defined as Mayo 2004 stage 3, involvement of more than or two organs was presented in 65 (85.53%) patients. Among 60 response evaluable patients, overall hematological response was 48.33% with complete response of 20.00% and very good partial response of 20.00%, respectively. The median time to the hematological response was 5 (range, 1-15) months. 36.67% patients achieved organ response. After the median follow up of 23(range, 1-113) months for surviving patients, median progression-free survival (PFS) and overall survival (OS) were 34 and 43 months, respectively. In a three months landmark analysis, the median rates of PFS and OS were 46 and 65 months, respectively. The median OS rates of patients with Mayo 2004 stage 3 and non Mayo 2004 stage 3 were 5 and 65 months (P=0.001), respectively. Conclusions: MDex was an effective treatment for patients with early stage pAL, but was not suitable for those with severe cardiac involvement.
Adult ; Aged ; Amyloidosis/drug therapy* ; Dexamethasone/administration & dosage* ; Drug Combinations ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Male ; Melphalan/administration & dosage* ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult

To cultivate high-quality medical talents,Shanghai Jiao Tong University School of Medicine established a multidisciplinary and comprehensive basic medical practical integration curriculum Medical Functional Experiment.Over several years' exploration and renovation,insisting on student-centered,this course has established a modular experiment teaching system with emphasis on foundation,combina-tion with clinical and focusing on innovation.Integration curriculum adopts four-stage teaching pattern which is suitable for medical students,and implements diversified teaching method and evaluation system.And meanwhile,the paper explores the ideas and approaches to further deepen the construction of teaching staff,expand experimental teaching techniques,strengthen the construction of curriculum materials,and strengthen the cultivation of teachers' innovative ability.

Objective To evaluate the sensitivities of various biopsy methods for the diagnosis of systematic amyloidosis (SA). Methods The clinical data and biopsy results of 194 SA patients who were treated in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed. Results The highest sensitivity was achieved by biopsy of affected organs,with renal biopsy 97.4%,heart biopsy 95.0% and liver biopsy 87.5%. Among non-invasive biopsy methods,tongue biopsy was found to be 75% sensitive,followed by gingiva biopsy at 57%,abdominal fat pad aspiration at 57%,rectum biopsy at 16%,and bone marrow examination at 8%. Combination of tongue and abdominal fat pad biopsy yielded a detection rate of 93.1%. Conclusions Biopsy of the involved organ has the highest sensitivity. However,combination of multiple non-invasive biopsy methods may has sensitivity comparable to organ biopsy and is safer and more convenient.
Adipose Tissue ; pathology ; Amyloidosis ; diagnosis ; Biopsy ; methods ; Biopsy, Needle ; Humans ; Retrospective Studies ; Sensitivity and Specificity ; Tongue ; pathology

BACKGROUNDThe cochlear hydrops analysis masking procedure (CHAMP) is a new diagnostic technique for Meniere's disease (MD). But its value has not been well proven. This study aimed to evaluate the diagnostic value of CHAMP for MD.

METHODSCHAMP test was taken in three populations using the Auditory Evoked Potential system delivered by Bio-logic Systems Corporation: (1) otologically normal subjects; (2) patients clinically diagnosed with definite MD; (3) patients clinically diagnosed with probable and possible MD.

RESULTSAccording to the comparison between the normal and definite MD group, if the abnormal criterion of CHAMP was defined as latency delay less than 0.3 ms, then the corresponding sensitivity was only 52%. However, if the abnormal criterion was defined as latency delay between 0.6 and 3.8 ms, then a sensitivity of 93% and a specificity of 100% can be achieved. The complex amplitude ratio showed a significant overlap between normal and definite MD group. If the abnormal criterion was defined as a complex amplitude ratio less than 0.95, the corresponding specificity was only 50%. However, if the abnormal criterion was defined as less than 0.80, the corresponding sensitivity was 60%, and the specificity was 97%. If the abnormal criterion of CHAMP was defined as latency delay less than 0.6 ms or the complex amplitude ratio less than 0.80, CHAMP result can be obtained in all subjects with good sensitivity and specificity.

CONCLUSIONSCHAMP can differentiate patients with Meniere's disease from otologically normal subjects with high sensitivity and specificity. The recommended criterion of abnormal CHAMP was a latency delay less than 0.6 ms or a complex amplitude ratio less than 0.80.

Adolescent ; Adult ; Aged ; Audiometry, Evoked Response ; Endolymphatic Hydrops ; diagnosis ; physiopathology ; Female ; Humans ; Male ; Meniere Disease ; diagnosis ; physiopathology ; Middle Aged ; Young Adult

Objective To study the epidemiological characteristics on the clustering nature of pandemic (H1N1) 2009 in China.Methods Time and place distribution of pandemic (H1N1) 2009on the nature of clustering through data from Public Health Emergency Management Information System were described.Results As of August 10,2010,2773 pandemic (H1N1) 2009 clusters,a total of 77363 cases (including 20 deaths) were reported in the mainland of China.The most reported number of clusters was from schools and kindergartens with the total number of 2498 (accounted for 90.08％ of the total number).Middle schools appeared the have the most clusters (1223,accounting for 48.96％ ).The number of clusters reported in the southern provinces (cities) accounted for 77.03％ of the total,and was more than that in the northern provinces (cities).Two reported peaks in the southern provinces (cities) were in June and November,2009,respectively.There was only one reported peakin the northern provinces in September,2009.Conclusion Time and place distribution characteristics on the clusters of pandemic (H1N1) 2009 were similar to the seasonal influenza,but the beginning of winter peak was much earlier and intensity of reporting was much higher on the clusters of pandemic (H1N1 ) 2009 than that of seasonal influenza.

Objective To investigate whether patients presenting with self-reported olfactory disorders demonstrates significant side difference in odour recognition by measuring separately for each nostril.Methods One hundred and four patients with chief complaint of hyposmia were evaluated by medical history,physical examination,T&T olfactory testing and medical imaging.Smell was tested using T&T olfactometry in each nostril separately.Results Based on the history and results from the clinical examination,unilateral sinonasal diseases and abnormal nasal structure were excluded.In 14 patients (13.5％) a side difference was detected.Especially,six of the patients were identified with lateralized hyposmia.Conclusions Olfactory testing could be performed in each nostril separately.The above findings suggested that testing each nostril separately was necessary so as not to miss unilateral hyposmia as a special clinical manifestation of olfactory disorder.

Congresses as Topic ; Hearing Tests ; Humans ; Infant, Newborn ; Neonatal Screening

Auditory Cortex ; Auditory Perceptual Disorders ; diagnosis ; Hearing Tests ; Humans ; Infant, Newborn ; Neonatal Screening

Objective Auditory brainstem responses (ABR) evoked by tone burst is an important method of hearing assessment in referral infants after hearing screening. The present study was to compare the thresholds of tone burst ABR with filter settings of 30 - 1500 Hz and 30 - 3000 Hz at each frequency,figure out the characteristics of ABR thresholds with the two filter settings and the effect of the waveform judgement, so as to select a more optimal frequency specific ABR test parameter. Methods Thresholds with filter settings of 30 - 1500 Hz and 30 -3000 Hz in children aged 2 -33 months were recorded by click,tone burst ABR. A total of 18 patients ( 8 male / 10 female), 22 ears were included. Results The thresholds of tone burst ABR with filter settings of 30 - 3000 Hz were higher than that with filter settings of 30 - 1500 Hz. Significant difference was detected for that at 0. 5 kHz and 2.0 kHz ( t values were 2.238 and 2. 217, P ＜ 0. 05 ), no significant difference between the two filter settings was detected at the rest frequencies tone evoked ABR thresholds. The waveform of ABR with filter settings of 30 - 1500 Hz was smoother than that with filter settings of 30 - 3000 Hz at the same stimulus intensity. Response curve of the latter appeared jagged small interfering wave. Conclusions The filter setting of 30 - 1500 Hz may be a more optimal parameter of frequency specific ABR to improve the accuracy of frequency specificity ABR for infants' hearing assessment.

Objective To study the characteristic of the cochlear hydrops analysis masking procedure ( CHAMP) in normal adults, and to evaluate the diagnostic values of its parameters for logic auditory evoked potential system: clicks presented alone (unmasked condition) and clicks presented with ipsilateral pink noise high-pass filtered at 8 , 4, 2, 1, and 0. 5 kHz respectively. Results The wave V latency of ABR to the high-pass masking pink noise clicks were longer than ABR to clicks alone. The latency delays of wave V for clicks presented with ipsilateral pink noise high-pass filtered at 8 , 4, 2, 1, and 0.5 kHz compared to clicks alone were (0.30 ±0.18), (0.97 ±0.43), (1.65 ±0.64), (3.21 ±0.56),(4. 66 ± 0. 37 ) ms respectively. The complex amplitude ratio between ABR to click + 0. 5 kHz high-pass noise and click alone was 0.95 ±0.11. Conclusions CHAMP is a promising diagnostic method for membranous labyrinth hydrops, and the latency delay of wave V might be used as the normal criterion. The specificity of the complex amplitude ratio need furthur evaluation in clinical work.