Thrombocytopenia 2 (THC2) is an autosomal dominant hematologic disorder caused by germline mutations in the ANKRD26 gene, characterized primarily by persistent thrombocytopenia and a predisposition to myeloid neoplasms. Owing to nonspecific clinical presentation and limited disease awareness, THC2 is frequently underdiagnosed or misdiagnosed, potentially leading to inappropriate interventions. This article systematically outlines the clinical manifestations, pathogenesis, and strategies for the precise diagnosis and treatment of THC2, aiming to provide a theoretical basis and practical guidance for its clinical management and for the in-depth investigation of its pathogenesis.

Objective:To investigate the current situation of nuclear medicine practices, determine the number of nuclear medicine staff, conduct internal exposure monitoring and dose estimation for nuclear mecidine staff engaged in 131I treatment in Zhejiang province. Methods:A survey was conducted over all the 22 hospitals involved in 131I treatment in the province. The 131I activity in thyroid of 96 stafff in 131I treatment workplaces were measured by means of direct method. At the same time, the effective doses from internal exposure were estimated and the influencing factors were analyzed. Results:131I activity in thyroids was found to be above the detection limit for 49 staff (51.04%) in nineteen hospitals. The maximum value of 131I activity was 629.18 Bq. There was no statistically significant difference in 131I detection rate in thyroid of 131I treatment staff between different positions, different genders and different levels of hospitals ( P>0.05). Comparisons of 131I activity of thyroid of nuclear medicine staff for theatment of thyroid cancers had shown that the highest was for nurses, followed by technicians and doctors, and the lowest was for cleaning staff ( H=6.39, P<0.05). The estimated committed effective dose to the nuclear medicine staff ranged from 0.05 to 2.37 mSv, with those below 1 mSv accounting for 93.88% of the total. Logistic regression analysis showed that nursing position was the risk factor contributing to the committed effective dose ( OR=2.805, 95% CI 1.076-7.314). Conclusions:In Zhejiang province, the committed effective dose to thyroid of nuclear medicine staff from 131I internal exposure was not in excess of the dose limit. However, the staff performing iodine therapy still need to strengthen protection against internal exposure and take scientific and effective protective measures to reduce the risk of health hazards from internal exposure.

Objective To investigate the effect of orthosis design parameters on correction of scoliosis and orthosis-trunk interface pressure.Methods A finite element model of scoliosis was constructed to simulate the assembly effect of the orthosis.The orthosis was divided into four loading areas(left rib,right rib,anterior-left and posterior-right area)to simulate six modification conditions.In Models 1,2 and 3,a fixed modification of 20 mm was applied on the anterior left and posterior right areas,while the displacement loads of 20,25 and 30 mm were applied on both the left rib and right rib areas.In Models 4,5 and 6,a fixed modification of 25 mm was applied on left rib and right rib areas,with the displacement loads of 15,20 and 25 mm applied on both anterior left and posterior right areas.The Cobb angle,apical vertebral rotation(AVR)and interface pressure were calculated.Results The correction of Cobb angle in Models 1,2 and 3 was 8.94°,15.62° and 17.91°,respectively,with AVR correction of 7.53°,6.69° and 5.87°,respectively.In Models 4,5 and 6,the correction of Cobb angle was 14.55°,15.62° and 16.09°,with AVR correction of 5.25°,6.69° and 8.63°,respectively.In Model 6,the correction rate of Cobb angle and AVR was 45.48%and 41.22%,respectively,with a maximum pressure of 26.51 kPa on orthosis-trunk interface,achieving the most significant outcome.Conclusions The modification of orthosis has a significant effect on the correction of Cobb and AVR angles.The loading on the left rib and right rib areas mainly affect the Cobb angle,while the loading on the anterior left and posterior right areas mainly affect the spinal axial-rotation.A modification of 25 mm on all loading areas achieves the optimal spinal correction.This study provides the quantitative data for orthosis design.

Objective:To investigate the current situation of nuclear medicine practices, determine the number of nuclear medicine staff, conduct internal exposure monitoring and dose estimation for nuclear mecidine staff engaged in 131I treatment in Zhejiang province. Methods:A survey was conducted over all the 22 hospitals involved in 131I treatment in the province. The 131I activity in thyroid of 96 stafff in 131I treatment workplaces were measured by means of direct method. At the same time, the effective doses from internal exposure were estimated and the influencing factors were analyzed. Results:131I activity in thyroids was found to be above the detection limit for 49 staff (51.04%) in nineteen hospitals. The maximum value of 131I activity was 629.18 Bq. There was no statistically significant difference in 131I detection rate in thyroid of 131I treatment staff between different positions, different genders and different levels of hospitals ( P>0.05). Comparisons of 131I activity of thyroid of nuclear medicine staff for theatment of thyroid cancers had shown that the highest was for nurses, followed by technicians and doctors, and the lowest was for cleaning staff ( H=6.39, P<0.05). The estimated committed effective dose to the nuclear medicine staff ranged from 0.05 to 2.37 mSv, with those below 1 mSv accounting for 93.88% of the total. Logistic regression analysis showed that nursing position was the risk factor contributing to the committed effective dose ( OR=2.805, 95% CI 1.076-7.314). Conclusions:In Zhejiang province, the committed effective dose to thyroid of nuclear medicine staff from 131I internal exposure was not in excess of the dose limit. However, the staff performing iodine therapy still need to strengthen protection against internal exposure and take scientific and effective protective measures to reduce the risk of health hazards from internal exposure.

Objective To investigate the effect of orthosis design parameters on correction of scoliosis and orthosis-trunk interface pressure.Methods A finite element model of scoliosis was constructed to simulate the assembly effect of the orthosis.The orthosis was divided into four loading areas(left rib,right rib,anterior-left and posterior-right area)to simulate six modification conditions.In Models 1,2 and 3,a fixed modification of 20 mm was applied on the anterior left and posterior right areas,while the displacement loads of 20,25 and 30 mm were applied on both the left rib and right rib areas.In Models 4,5 and 6,a fixed modification of 25 mm was applied on left rib and right rib areas,with the displacement loads of 15,20 and 25 mm applied on both anterior left and posterior right areas.The Cobb angle,apical vertebral rotation(AVR)and interface pressure were calculated.Results The correction of Cobb angle in Models 1,2 and 3 was 8.94°,15.62° and 17.91°,respectively,with AVR correction of 7.53°,6.69° and 5.87°,respectively.In Models 4,5 and 6,the correction of Cobb angle was 14.55°,15.62° and 16.09°,with AVR correction of 5.25°,6.69° and 8.63°,respectively.In Model 6,the correction rate of Cobb angle and AVR was 45.48%and 41.22%,respectively,with a maximum pressure of 26.51 kPa on orthosis-trunk interface,achieving the most significant outcome.Conclusions The modification of orthosis has a significant effect on the correction of Cobb and AVR angles.The loading on the left rib and right rib areas mainly affect the Cobb angle,while the loading on the anterior left and posterior right areas mainly affect the spinal axial-rotation.A modification of 25 mm on all loading areas achieves the optimal spinal correction.This study provides the quantitative data for orthosis design.

Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.

Objective:To explore the short-term outcomes of 3D-printing stand-alone artificial vertebral body(AVB)in the surgical procedure of anterior cervical corpectomy and fusion(ACCF).Methods:Following the proposal of IDEAL(idea,development,exploration,assessment,and long-term follow-up)framework,we designed and conducted this single-armed,retrospective cohort study.The patients with cervical spondylotic myelopathy were recruited,and these patients exclusively received the surgical procedure of single-level ACCF in our single center.After the process of corpectomy,the size was tailored using different trials and the most suitable stand-alone AVB was then implanted.This AVB was manufactured by the fashion of 3D-printing.Two pairs of screws were inserted in an inclined way into the adjacent vertebral bodies,to stabilize the AVB.The participants were regularly followed-up after the operation.Their clinical data were thoroughly reviewed.We assessed the neurological status according to Japanese Orthopedic Association(JOA)scale.We determined the fusion based on imaging examination six months after the operation.The recorded clinical data were analyzed using specific software and they presented in suitable styles.Paired t test was employed in comparison analysis.Results:In total,there were eleven patients being recruited eventually.The patients were all followed up over six months after the operation.The mean age of the cohort was(57.2±10.2)years.The mean operation time was(76.1±23.1)min and the median bleeding volume was 150(100,200)mL.The postoperative course was uneventful for all the cases.Dysphagia,emergent hematoma,and deterioration of neurological func-tion did not occur.Mean JOA scores were 13.2±2.2 before the operation and 16.3±0.8 at the final follow-up,which were significantly different(P＜0.001).The mean recovery rate of neurological func-tion was 85.9％.By comparing the imaging examinations postoperatively and six months after the opera-tion,we found that the average subsidence length was(1.2±1.1)mm,and that there was only one ca-ses(9.1％)of the severe subsidence(＞3 mm).We observed significant improvement of cervical lor-dosis after the operation(P=0.013).All the cases obtained solid fusion.Conclusion:3D-printing stand-alone AVB presented favorable short-term outcome in one-level ACCF in this study.The fusion rate of this zero-profile prosthesis was satisfactory and the complication rate was relatively low.