Objective:To evaluate the efficacy of eculizumab in treating hematopoietic stem cell transplantation-associated thrombotic microangiopathy (TA-TMA) .Methods:This retrospective study included 11 patients who developed TA-TMA after allogeneic hematopoietic stem cell transplantation and subsequently received eculizumab treatment at Peking University People′s Hospital between June 2018 and May 2024. The incidence of TA-TMA, treatment details, and clinical outcomes were analyzed.Results:Among the 11 included patients [4 males, 7 females; median age: 29 years (range: 9-56) ], underlying diseases were severe aplastic anemia (SAA) in 5 patients, acute lymphoblastic leukemia (ALL) in 3 patients, and acute myeloid leukemia (AML) in 3 patients. The median time to TA-TMA diagnosis was 48 days post-transplantation (range: 4-213 days), and all patients met the diagnostic criteria for high-risk TA-TMA. The median interval from TA-TMA diagnosis to the initiation of eculizumab treatment was 12 days (range: 1-56 days). Patients received a median of 3 doses of eculizumab (range: 1-14). Ten of the 11 patients were assessed as having no response (NR) to eculizumab at the end of treatment or at death. One patient achieved a partial response (PR) but subsequently died after TA-TMA relapsed due to infection. At the last follow-up, all patients were either lost to follow-up or had died. The median follow-up duration was 88 days (range: 33-326 days), and the median time from TA-TMA diagnosis to the last follow-up was 31 days (range: 21-113 days) .Conclusion:Eculizumab demonstrated poor efficacy in this TA-TMA cohort. This might be attributable to the critical and complex condition of the patients, delayed initiation of eculizumab treatment, and insufficient dosage.

Objective:To evaluate the efficacy of eculizumab in treating hematopoietic stem cell transplantation-associated thrombotic microangiopathy (TA-TMA) .Methods:This retrospective study included 11 patients who developed TA-TMA after allogeneic hematopoietic stem cell transplantation and subsequently received eculizumab treatment at Peking University People′s Hospital between June 2018 and May 2024. The incidence of TA-TMA, treatment details, and clinical outcomes were analyzed.Results:Among the 11 included patients [4 males, 7 females; median age: 29 years (range: 9-56) ], underlying diseases were severe aplastic anemia (SAA) in 5 patients, acute lymphoblastic leukemia (ALL) in 3 patients, and acute myeloid leukemia (AML) in 3 patients. The median time to TA-TMA diagnosis was 48 days post-transplantation (range: 4-213 days), and all patients met the diagnostic criteria for high-risk TA-TMA. The median interval from TA-TMA diagnosis to the initiation of eculizumab treatment was 12 days (range: 1-56 days). Patients received a median of 3 doses of eculizumab (range: 1-14). Ten of the 11 patients were assessed as having no response (NR) to eculizumab at the end of treatment or at death. One patient achieved a partial response (PR) but subsequently died after TA-TMA relapsed due to infection. At the last follow-up, all patients were either lost to follow-up or had died. The median follow-up duration was 88 days (range: 33-326 days), and the median time from TA-TMA diagnosis to the last follow-up was 31 days (range: 21-113 days) .Conclusion:Eculizumab demonstrated poor efficacy in this TA-TMA cohort. This might be attributable to the critical and complex condition of the patients, delayed initiation of eculizumab treatment, and insufficient dosage.

Objective:To summarize the clinical features associated with respiratory syncytial virus (RSV) infection in patients following the hematopoietic stem cell transplant (HSCT) and exploring the risk factors for death.Methods:Patients who had RSV infection after undergoing HSCT from October 2023 to January 2024 in the hematology department of Peking University People’s Hospital were enrolled in the study. The clinical characteristics of the participating patients were summarized. The clinical characteristics of the surviving and the dying patients were compared, and the risk factors of death were analyzed by binary logistic regression.Results:Among the 43 RSV-positive HSCT patients, 20 (46.5%) were hypoxemic, six (14.0%) were admitted to the ICU for further treatment, four (9.3%) required tracheal intubation assisted ventilation, and seven patients (16.3%) died. A comparison of the clinical features of the surviving patients and the deceased patients demonstrated that the deceased patients had a lower PLT when infected with RSV [74.5 (8.0-348.0) ×10 9/L vs 15.0 (10.0-62.0) ×10 9/L, P=0.003], a higher incidence of simultaneous bacterial infections (85.7% vs 41.7%, P=0.046), and a higher rate of hematological recurrence (71.4% vs 13.9%, P=0.004). Hematological recurrence ( OR=15.500, 95% CI 2.336-102.848, P=0.005), influenza A viral infection ( OR=14.000, 95% CI 1.064-184.182, P=0.045), and low PLT at the time of RSV infection ( OR=0.945, 95% CI 0.894-0.999, P=0.048) were the factors associated with death following HSCT. Conclusion:Patients infected with RSV after undergoing HSCT have a poor prognosis, and active prevention and treatment of RSV in the autumn and winter requires urgent attention.

Objective:To investigate the utilization of health management services and its influencing factors among new urban population.Methods:It is a cross-sectional study. From July 2020 to March 2021, a stratified random sampling method was used to extract 1978 new urban population in Jining city, and an anonymous self-administered questionnaire survey was conducted using a self-made questionnaire ′Residents Health Questionnaire′. The survey included general demographic characteristics, personal behavior lifestyle and medical care status. The χ2 test and binary logistic regression were used to analyze the factors influencing the utilization of health management services by new urban population. Results:The overall utilization of health management services in the new urban population was 47.22%. There were significant differences in utilization of health management services among new urban population with different gender, age, education level, occupation and monthly income. Binary logistic regression analysis showed that female ( OR=1.354, 95% CI: 1.094-1.676), people aged over 60 years ( OR=1.873, 95% CI: 1.413-2.483), people with a mean monthly income over 3 000 yuan ( OR=1.498, 95% CI: 1.123-1.997), people engaged in light manual labor ( OR=1.596, 95% CI: 1.003-2.539), people who exercise regularly( OR=2.400, 95% CI: 2.028-2.841) and people having social basic medical insurance ( OR=2.633, 95% CI: 2.042-3.394) had better utilization of health management projects. People who sat more than 3 hours a day ( OR=0.630, 95% CI: 0.532-0.745) had lower utilization of health management care. Conclusion:The utilization of health management projects in the new urban population is low. Gender, age, monthly income, physical exercise, sedentary time, daily labor intensity and social basic medical insurance status are the main influencing factors.

Objective To study the analysis of auditory rehabilitation outcomes of patients with cochlear nerve canal stenosis after cochlear implantation(CI).Methods A cohort of 30 patients with bilateral profound senso-rineural hearing loss who were diagnosed with cochlear neural canal stenosis by high-resolution CT were tested with evoked compound action potential (ECAP)and evoked auditory brainstem response (EABR)during and 3 ,6 , 9 months after CI.Audiometry in sound field was also assessed before and 3 ,6 ,9 months after CI.Among the co-hort,1 7 patients over 3 years old underwent postoperative speech recognition rate test.All the auditory rehabilita-tion outcomes were analyzed.Results ① For all 30 patients,there were no obvious differences of ECAP and EABR waveforms tested in 3,6 and 9 months after CI.②The thresholds in sound field in 3,6,9 months after CI were 65 ±8 dB HL,62 ±4 dB HL and 61 ±7 dB HL,respectively.The thresholds in sound field were significantly im-proved after than before CI (100 ±5 dB HL).③ The single vowel recognition rates of 17 patients in 3 ,6 and 9 months after CI were 55%±7%,56%±8% and 80%±4%,respectively.The single vowel recognition rate was significantly improved in 9 months after than before CI(52%±8%).The single consonant recognition rates of 17 pa-tients in 3 ,6 and 9 months after CI were 9%±3%,8%±4% and 9%±2%,respectively.The single consonant recognition rates were not significantly improved after than before CI (8%±2%).Conclusion ① For patients with bi-lateral cochlear neural canal stenosis,neither ECAP nor EABR waves were produced during or after CI.The language com-munication of patients is limited as a result of their poor subjective thresholds in sound field and speech recognition rates.

OBJECTIVE: To investigate the feasibility of the round window stimulation electrical evoked auditory brainstem response (EABR) test, and optimize the parameters of recording and stimulation electrodes positions. METHOD: Ten healthy Hartley guinea pigs (20 ears) were used for the EABR test. The positive stimulation electrodes were placed into the round window niche, the animals were divided into three group according to the negative electrodes position, group A: the electric field was parallel with the projection of cochlear modiolus on the tympanic membrane, group B: the electric field was perpendicular to modiolus projection toward to the mastoid, group C: the electric field was perpendicular to modiolus projection toward to the zygomatic process. A series of optimized recording and stimulation parameters were uesed to reduce the electrical artifact. RESULT: All the 20 ears were normal in the ABR testing, and EABR waves were stable and well-differentiated in the EABR tests out of cochlea. But EABR waves of group A were more stable and differentiated than those of group B and C. In group A, the threshold of EABR was (0.54 ± 0.11) mA, and latency of wave III was (1.71 ± 0.05) ms when the stimulus intensity was 0.8 mA. In group B, the threshold of EABR was (0.62 ± 0.12) mA, and latency of wave III was (1.77 ± 0.03) ms. In group C, the threshold of EABR was (0.70 ± 0.14) mA, and latency of wave III was (1.86 ± 0.04)ms. The threshold of EABR and latency of wave III were significantly different among the three groups by statistic analysis. CONCLUSION EABR waves were stable and well-differentiated in the EABR tests out of cochlea. The EABR waves were recorded more stably and differentiated when the stimulating electrode and recording electrode were paralleled with the projection of modiolus on the tympanic membrane.
Animals ; Cochlea ; physiology ; Electric Stimulation ; Electrodes ; Evoked Potentials, Auditory, Brain Stem ; Guinea Pigs ; Round Window, Ear ; Tympanic Membrane

Objective To find out the influence of different suffix vowel(abbreviation:SV) on the duration of/s/(a voiceless fricative) with acoustic detection method. Methods Two men and two women who were fluent in Mandarin Chinese were recruited in this study. The words included /s/and the suffix vowel /a/,/u/or/i/.The CSL4150 software from Kay Co. was used to analyze the words. Then we compared the durations of/s/when the suffix vowel was /a/,/i/and/u/,respectively. The influence of difference suffix vowel on the duration was exam_ined, and gender effects were also studied on the duration. ResuIts As a voiceless fricative, when the suffix vowel was /a/,the duration for males was 200. 2, and 276. 8 ms for females, respectively. When the suffix vowel was /i/, the duration for males was 218. 2, and 326. 5 ms for females, respectively. When the suffix vowel was /u/,the dura_tion for males was 214. 5, and 302. 9 ms for females, respectively. ConcIusion When the suffix vowel is different, the duration of stop, resistance and in number is different, and also affected by gender.

Child ; Child, Preschool ; Cochlea ; surgery ; Cochlear Implantation ; methods ; Female ; Humans ; Infant ; Male ; Ossification, Heterotopic ; surgery

OBJECTIVE: To review the classification of cochlear modiolus deficiency and decision on surgical approach for above case,in order to provide mastery for cochlear implant (CI) indication. METHOD: Basing on temporal bone HRCT pre-operation, CI subjects with modiolus deficiency were defined as following groups: (1) deficiency caused by cochlear dysplasia (Mondini malformation); (2) deficiency caused by dysplasia of cochlear and vestibule (Common cavity malformation); (3) deficiency caused by absence of internal acoustic meatus fundus (IP-III malformation). Three types of surgical approach were utilized: type I, electrode array was introduced through facial recess, enlarged the round window, type II, opened the surface of chchlea, electrode array was introduced through facial recess, fenestration on posterior promontory and then inserted around lateral wall of inner-cochlear cavity. type III, electrode array was introduce through fenestration of lateral semicircular canal and then placed close to the bony wall of common cavity. RESULT: One hundred and sixty-six cochlear modiolus deficiency cases were identified into 3 groups as following: 135 Mondini malformation cases into group a, 18 common cavity malformation cases into group b, and 13 IP-III malformation cases into group c. Surgical approach: type I were used in 136 cases (123 Mondini cases and 13 IP-III cases), while approach type II in 12 cases (12 Mondini cases), and approach type III in 18 cases (18 common cavity cases). Income post-operation of CI: For group a (Mondini malformation), post-activation mean hearing threshold in sound field was 65 dB, speech recognition score is 95% (single finals test) and 25% (signal initials test), while it was 80 dB, 60% and 0 for group b (Conmon cavity malformation), and it was 55 dB, 100% and 45% for group c (IP-III malformation). CONCLUSION The income of speech recognition score for cochlear modiolus deficiency was relatively poor, group b was worst and group c was best, while group a moderate.
Cochlea ; abnormalities ; surgery ; Cochlear Implantation ; methods ; Cochlear Implants ; Ear ; Ear, Inner ; abnormalities ; Female ; Humans ; Male ; Postoperative Period ; Round Window, Ear ; surgery ; Semicircular Canals ; surgery ; Speech Perception ; Temporal Bone ; Vestibule, Labyrinth ; abnormalities

OBJECTIVE: The purpose of this study is to report surgical skills for CI cases with modiolus ossification and to investigate the relation between post-operational electroneurophysilogical test result and speech recognition result. Further more, we also attempt to confirm indications for CI in this specific population. METHOD: Based on temporal bone HRCT, 7 subjects were identified as modiolus ossification from 101 cases with cochlear ossification. Modiolus ossification is confirmed by CT scan if CT value in modiolus reaches or exceeds 900 HU with the exception of congenital modiolus ossification or modiolus seal off. Electroneurophysiological test was conducted intra- and pos-operationally speech tests were applied for 7 subjects. RESULT: Normal impedance value was observed by intro-operational measurement in 7 subjects. EABR test was conducted and negtive response was observed in only 1 subject, while other 6 subjects were confirmed with atypical EABR waves which were observed in apical and middle turn region. Hearing threshold test (in sound field) was applied, no auditory response was recorded for the subject without EABR waveform, while hearing threshold in average for the other 6 subjects was 75 dB. Results of speech tests (Mandarin) were followed as 0 for the one without EABR wave, while 100% (simple finals test) and 30% (simple initials test) for the other 6 subjects. CONCLUSION Optimal multichannel CI surgery that inserting and locating electrode array spirally is very frequently interrupted by ossification,which was indentified with atypical EABR wave and relative poor speech recognition results, especially in modiolus ossification case. A post-operative negative EABR response may indicate surgical failure following cochlear implantation.
Adolescent ; Adult ; Child ; Child, Preschool ; Cochlea ; pathology ; Cochlear Implantation ; Evoked Potentials, Auditory, Brain Stem ; physiology ; Female ; Humans ; Infant ; Male ; Middle Aged ; Ossification, Heterotopic ; surgery ; Postoperative Period ; Young Adult