Adolescent idiopathic scoliosis(AIS)is a complex three-dimensional deformity involving coronal,sagittal,and axial planes,with a prevalence that should not be overlooked.With advancements in technology and in-depth research,an increasing number of hospitals and physicians are exploring standardized diagnostic and treatment approaches for AIS.Comprehensive and in-depth understanding is required for AIS,including its etiology,screening and diagnosis,classification,assessment and examination,treatment options,exploration of current focus,and evaluation of quality of life.Such understanding ensures that the diagnostic and treatment are scientific,standardized,and timely.Based on the principles of evidence-based medicine,a consensus on the diagnosis and treatment of AIS is reached after multiple discussions among spinal surgery experts,aiming to provide reference and guidance for clinical practice.

Objective:To discuss the problems existing in the implementation of the Chinese health industry standard WS 523-2019 by testing SPECT device.Methods:Under the WS 523-2019 standards, a total of 10 SPECT devices were tested with regard to their SPECT reconstructed spatial resolution (SRSR), system planar sensitivity (SPS), system spatial resolution (SSR), whole body system spatial resolution (WSSR), intrinsic uniformity (IU), intrinsic count rate performance (ICR), intrinsic spatial resolution (ISR) and intrinsic spatial linearity(ISL).Result:Under the requirements of WS 523-2019 standards for qualified limits, there are 3 devices with ISL unqualified and the rest of the performances qualified. The new standards basically can meet the clinical requirements and reflected the overall performance of SPECT.Conclusions:The distance between the radiation source and the surface of the detector has great influence on the spatial resolution.In the measurement of ISL, there must be a lead grid separately in the x and y directions. The lead grids with the parallel slits shall be positioned on the detector with the center slit centered on the detector. It is suggested to add rotation center in the new standards.

Kidney cancer usually requires multidisciplinary individualized treatments. No matter what kind of treatment, drugs are essential. According to the "six-step process" (prescription legitimacy review, patient basic information evaluation review, treatment protocol review, organ function and laboratory index review, pretreatment review, and unconventional prescription review) in prescription review proposed by the anti-tumor drug prescription review expert group and referring to domestic and foreign kidney cancer guidelines and drug instructions in recent years, this consensus selects 9 targeted drugs and 4 immunotherapeutic drugs that are currently commonly used in China and elaborates the key review points in patient basic information evaluation review, treatment protocol review, and organ function and laboratory index review of kidney cancer drug treatment, in order to provide reference for clinical front-line pharmacists to review prescriptions of kidney cancer patients and promote rational drug use in clinic.

Kidney cancer usually requires multidisciplinary individualized treatments. No matter what kind of treatment, drugs are essential. According to the "six-step process" (prescription legitimacy review, patient basic information evaluation review, treatment protocol review, organ function and laboratory index review, pretreatment review, and unconventional prescription review) in prescription review proposed by the anti-tumor drug prescription review expert group and referring to domestic and foreign kidney cancer guidelines and drug instructions in recent years, this consensus selects 9 targeted drugs and 4 immunotherapeutic drugs that are currently commonly used in China and elaborates the key review points in patient basic information evaluation review, treatment protocol review, and organ function and laboratory index review of kidney cancer drug treatment, in order to provide reference for clinical front-line pharmacists to review prescriptions of kidney cancer patients and promote rational drug use in clinic.

OBJECTIVE: To detect pathological variant in two patients with Chediak-Higashi syndrome (CHS) from a consanguineous family and to explore its genotype-phenotype correlation. METHODS: Clinical data was collected for this pedigree. Genomic DNA was prepared from probands' peripheral leukocytes and their relatives' fingernail. Whole exome sequencing and Sanger sequencing were carried out to detect potential variant of the LYST gene. RESULTS: The proband presented with partial oculocutaneous albinism, immunodeficiency and acidophilic inclusion body in bone marrow and blood smears. A novel homozygous nonsense variant c.8782C>T (p.Gln2928*) was identified in exon 34 of the LYST gene in the sib pair. The same variant was found to be in heterozygous status in 6 unaffected individuals from the pedigree. CONCLUSION Above result enriched the mutational spectrum of CHS and provided a basis for genetic counseling and prenatal diagnosis for this pedigree.
Chediak-Higashi Syndrome ; genetics ; Exons ; Heterozygote ; Humans ; Mutation ; Pedigree ; Sequence Analysis, DNA ; Vesicular Transport Proteins ; genetics ; Whole Exome Sequencing

Objective To investigate the relationship between red blood cell distribution width (RDW) and prognosis in patients with multiple myeloma (MM).Methods The population that studied consisted of 27 patients with multiple myeloma and 30 healthy controls.The RDW was calculated according to the results of blood routine examination and compared between patients and healthy controls.Then,compared the difference between the two groups of RDW.MM patients were treated with international standard staging (ISS),and the differences of RDW in different stages were analyzed.ISS staging was used to draw the receiver operating curve (receiver operating characteristic curve,ROC curve),then take RDW14.65 ％ as the best cut-off point,the MM patients were divided into low RDW group (RDW=14.65 ％) and high RDW group (RDW ＞14.65％).Overall survival (OS) condition were compared between the above two groups.The impacts of RDW on OS were analyzed by Kaplan-Meier and Log-rank test.Results The average RDW value in experimental and controlled were 15.60 ％ ± 2.35 ％ vs 12.72 ％ ±0.61 ％ separately (t=6.201,P＜0.001),with statistical differences.The average RDW value in low ISS(Ⅰ + Ⅱ stage) and high ISS (Ⅲ stage) were 13.99 ％ ± 1.08％ vs 16.55 ％±2.39％ separately (t=3.800,P=0.001).The median survival time of low RDW and high RDW group was 13 months and 8 months respectively,and the difference was statistically significant (x2=6.481,P =0.011).Conclusion RDW increased in patients with MM,the risk stratification higher prognosis is worse.

Objective To report the total skin electron irradiation(TSEI) to treat the mycosis fungoides and the related acute toxicity,short-term efficacy and long-term results.Methods Five patients with pathologically confirmed mycosis fungoides were enrolled from 1990 to 2011,including 4 males and 1 female.Median age was 51 years old(range 14-56 years old).Dose homegeneity within the field were ± 10％ of the prescribed dose with a skin surface dose of about 90％ of the prescribed dose.Four cases received a total dose of about 30 Gy in 30-32 fractions in 8 weeks,and the other case received an electron boost of 5 Gy to the residual tumor after irradiation with 31 Gy within 31 fractions in 8 weeks,who had received 6 cycles chemotherapy before TSEI.Results 4 cases with a follow-up time of more than 15 years achieved complete response 6 months after radiotherapy.For the recently treated case,a great partial response was achieved after radiotherapy.Acute toxicities for all patients were acceptable.Two patients survived after a follow-up of more than 15 years while the other two cases died of visceral involvement at 1 and 3 years later,respectively.The case treated recently survived 2 months later.Conclusions A total TSEI dose of about 30 Gy within 30-32 fractions during 8 weeks could be proved to be very effective and safe in skin lesion control.Visceral involvement is the main failure type.

Objective To observe how brain death affects the hepatic morphology and function of pigs and explore the roles of NF-κB. Methods Under general anaesthesia twelve healthy pigs were allocated randomly to two groups:control group(6 pigs),with non-inflacted Foley balloon catheter placed in the cerebral ventricle for 24 h,and brain death group,6 pigs,with estabhshment of brain death for 24 h.The serum and hepatic tissues in the same locus were taken at 6 h,12 h,and 24 h after the initial conformation of brain death.AST and ALT were determined by automatic biochemistry analyzer.IL-1βwas determined by ELISA.The NF-κB mRNA was determined by Real-time PCR and the NF-κB p65 by immunohistochemistry. Results The AST,ALT,IL-1β in serum,the NF-κB mRNA and the NF-κB p65 in hepatic tissues in brain death group were higher than those in control group and they all increased with the time(P<0.05).In brain death group,hepatocytes were edematous lightly after 12 hours,and the swelling progressively deteriorated after 24 hours,but there were no necrosis. Conclusion The activated NF-κB by brain death promoted the synthesis and release of inflammatory mediators,resulting in the hepatic dysfunction.

Objective To explore the incidence and risk factors of bacterial infection after othtotopic liver transplantation (OLT). Methods Altogether 56 OLT recipients from January 2005 to October 2007 were included in the study. The incidents and the related variables of the infection were analyzed retrospectively. The related variables were evaluated using multivariate logistic regression model to identify the significant risk factors. Results Bacterial infection was confirmed in 29 recipients (51.8%). Among them, the lung infection was the most common site (53.7%). The Gram-positive cocci were 46.3%, while the Gram-negative bacilli were 53.7%. The risk factors for bacterial infection included duration of the operation and detained respirator using. Conclusion Bacterial infection is a major complication following OLT. Surveillance for the risk factors, enhancement the skill of operation, and improving the recovery of respiratory function is the key to decreasing the incidence of bacterial infection after transplantation.