The relationship of RDW-SD, NLR and FIB with the prognosis of multiple myeloma patients and their predictive value / 中国医师杂志

Objective:To explore the relationship between the standard deviation of red blood cell distribution width (RDW-SD), neutrophil/lymphocyte value (NLR), fibrinogen (FIB) and the prognosis of multiple myeloma (MM) patients and their predictive value.Methods:In this study, a retrospective study method was used to select 120 patients with MM who were initially diagnosed in the department of hematology of the Affiliated Hospital of Jining Medical College from January 2017 to October 2019. The follow-up time was 24 months, including 62 patients who survived (survival group) and 58 patients who died (death group). The RDW-SD, NLR and FIB values of the two groups were compared, and the value of the three indicators in predicting the follow-up outcome of MM patients was analyzed using the receiver operating characteristic (ROC) curve. Logistic regression model was used to analyze the related factors affecting the prognosis of MM patients.Results:Among 120 newly treated MM patients, the RDW-SD, NLR and FIB of the survival group were significantly lower than those of the death group (all P<0.05); The sensitivity, specificity and area under ROC curve (AUC) of RDW-SD+ NLR+ FIB in predicting adverse outcomes of MM patients were 88.96%, 84.50% and 0.919 respectively. Logistic multivariate regression analysis showed that ≥60 years old, International Staging System (ISS) Ⅲ, β2-microglobulin (β2-MG)≥3 500 ng/ml, increased RDW-SD, NLR, and FIB will increase the risk of poor prognosis in MM patients (all P<0.05). Conclusions:The RDW-SD, NLR and FIB have a close relationship with the poor prognosis of newly treated MM patients, and the combined application has certain value in predicting the prognosis of patients.

Advancement on the pathogenesis of hereditary multiple exostoses / 中华骨科杂志

Hereditary multiple exostoses (HME) are benign bone tumors characterized by autosomal dominant inheritance, which can cause skeletal malformation in adolescents, seriously affecting the body's aesthetic and motor functions. Currently, there are no guidelines for diagnosing and treating HME, and the main treatment is surgical treatment to remove the tumor and correct the deformity. However, osteochondroma is multiple and difficult to be completely resected. Therefore, more and more scholars are exploring the method of conservative treatment. However, the current understanding of the pathogenesis of HME is limited, and there are no safe and effective drugs in the clinic. Most hypotheses regarding the pathogenesis of HME are based on genetic mutations. Patients with HME may have EXT tumor suppressor gene mutations and function loss caused by secondary mutations such as loss of gene heterozygosity, which ultimately induce abnormal proliferation and differentiation of cartilage in growth plates. Abnormal EXT gene expression causes a decrease in the level of heparan sulphate (HS), leading to abnormalities in multiple molecular pathways that regulate the development and differentiation of growth plate chondrocytes, which together participate in the entire process of HME development and progression. This paper reviews the relevant studies on the pathogenesis of HME in recent years, in order to better understand the pathological process of HME, provide a theoretical basis for the diagnosis and treatment of HME, and also provide ideas for the development of drugs targeting HME.

Cervical spondylosis of nerve root type with qi stagnation and blood stasis treated with warming needle with different lengths of moxa stick: a randomized controlled trial / 中国针灸

OBJECTIVE@#To compare the clinical efficacy on cervical spondylosis of nerve root type with qi stagnation and blood stasis treated with warming needle with different lengths of moxa stick.@*METHODS@#Six hundred patients with cervical spondylosis of nerve root type with qi stagnation and blood stasis were randomly divided into 4 groups: a 4 cm length group (150 cases, 5 cases dropped off, 2 cases suspended), a 3 cm length group (150 cases, 6 cases dropped off, 2 cases suspended), a 2 cm length group (150 cases, 6 cases dropped off), and a routine acupuncture group (150 cases, 6 cases dropped off). Warming needle with moxa stick in the length of 4 cm, 3 cm and 2 cm was delivered in the 4 cm length group, the 3 cm length group and the 2 cm length group, respectively. In the routine acupuncture group, simple acupuncture was applied. The acupoints selected in the above groups included Dazhui (GV 14) and bilateral Jiaji (EX-B 2) of C5 and C7, Fengchi (GB 20), Jianzhen (SI 9), Quchi (LI 11), Zhongzhu (TE 3), etc. In each group, the intervention was delivered once daily and 5 times a week. One course of intervention was composed of 2 weeks and 2 courses were required. The TCM syndrome score, the score of clinical assessment scale for cervical spondylosis (CASCS), the score of the brachial plexus traction test of the affected upper limb, F wave occurrence rate and conduction velocity of the ulnar nerve, the median nerve and the radial nerve of the affected upper limb were compared before and after treatment in the patients of each group. The levels of serum inflammatory factors, i.e. interleukin-1β (IL-1β), interleukin-6 (IL-6), tumor necrosis factor α (TNF-α) and hypersensitive C-reactive protein (hs-CRP), were measured before and after treatment in the patients of each group. The clinical cfficacy was evaluated in the 4 groups.@*RESULTS@#After treatment, the results of TCM syndrome evaluation, i.e. the scores of neck pain, activity limitation and upper limb numbness and pain, as well as the total scores; and the scores of brachial plexus traction test were reduced when compared with those before treatment in each group (P<0.01, P<0.05). The scores of subjective symptoms and adaptability, and the total scores of CASCS were elevated in comparison with those before treatment in each group (P<0.01, P<0.05). In the 4 cm length group, compared with the other 3 groups, the scores of neck pain and activity limitation for TCM syndrome evaluation, and its total score were lower (P<0.05, P<0.01); and the scores of subjective symptoms and adaptability, and the total score of CASCS were higher (P<0.05, P<0.01). The score of the brachial plexus traction test in the 4 cm length group was lower than that of the routine acupuncture group (P<0.05). After treatment, F wave occurrence rates and conduction velocity of median nerve and radial nerve were increased when compared with those before treatment in each group (P<0.05, P<0.01). F wave occurrence rate and conduction velocity of the radial nerve in the 4 cm length group were higher than those of the other 3 groups (P<0.05), and those of the median nerve were higher when compared with the routine acupuncture group (P<0.05). After treatment, the levels of serum IL-1β, IL-6 and TNF-α were all reduced when compared with those before treatment in each group (P<0.01, P<0.05); the level of serum IL-6 in the 4 cm length group was lower than those of the other 3 groups and serum level of TNF-α was lower compared with that in the routine acupuncture group (P<0.05). The total effective rate of the 4 cm length group was 78.3% (112/143), which was higher when compared with the 3 cm length group (67.6%, 96/142), the 2 cm length group (65.3%, 94/144) and the routine acupuncture group (53.5%, 77/144), respectively (P<0.05).@*CONCLUSION@#Warming needle with moxa stick of 4 cm in length effectively relieves the clinical symptoms of cervical spondylosis of nerve root type with qi stagnation and blood stasis, improves the nerve function of the upper limbs, and reduces the inflammatory responses caused by nerve compression. The clinical efficacy of this therapy with moxa stick of 4 cm in length is superior to the warming needle with moxa sticks of 3 cm and 2 cm, as well as the routine acupuncture.

Current status of metabolomics in pediatric community-acquired pneumonia / 中国小儿急救医学

Community-acquired pneumonia(CAP) is one of the leading causes of death in children under 5 years of age.Early identification and clarification of its severity and appropriate therapeutic measures can improve survival, but there are limitations in the existing laboratory indices applied to diagnose CAP.Therefore, it is still necessary to find new and highly specific biomarkers that can identify the etiology and predict the severity of the disease before it worsens in children, and provides a basis for more effective therapeutic measures.Metabolomics provides a new way to search for biomarkers and pathogenesis through qualitative and quantitative analysis of metabolite changes in biological samples.This review summarized the latest research progress on metabolomics in childhood CAP, hoping to provide ideas for the early diagnosis and treatment of childhood CAP.

Analysis of clinical features of 36 children with necrotizing pneumonia / 中国小儿急救医学

Objective:To summarize the clinical features, diagnosis, treatment, and outcomes of necrotizing pneumonia(NP)in children, so as to improve the understanding of NP.Methods:Children with NP admitted to the Children′s Medical Center of Hunan Provincial People′s Hospital from December 2012 to June 2020 were selected and divided into respiratory support group(nine cases) and non-respiratory support group(27 cases) according to whether they received respiratory support; and they were also divided into pleural effusion group(28 cases) and non-pleural effusion group(eight cases) according to whether combined with pleural effusion.The clinical data of all children were collected, and the differences between different groups were compared.Results:There were thirty-six children with NP, included 14 boys and 22 girls, with a median age of 30(12, 49) months, and the disease duration was 34(25, 42)days.All children had cough, 34 cases had fever, and the fever peak was 39.5(39.1, 40.0) ℃.Laboratory tests(all peaks) showed that blood white blood cell count was 20.77(15.65, 28.35)×10 9/L, neutrophil count was 15.11(8.52, 20.65)×10 9/L, C-reactive protein(CRP) was 104.00(23.45, 146.50)mg/L, D-dimer was 5.12(1.88, 8.04)mg/L, and lactate dehydrogenase(LDH) was 347.95(284.68, 447.81)U/L.The detection rate of pathogens was 58.33%(21/36), and the most common was Staphylococcus aureus(28.57%, 6/21). Eight cases underwent surgical treatment, including five cases of thoracoscopic surgery and three cases of thoracotomy.All patients improved and were discharged from hospital.The differences in hospital stay, white blood cell count, CRP, procalcitonin and LDH levels between respiratory support group and non-respiratory support group were statistically significant, and the median age, white blood cell count, CRP, D-dimer and LDH between pleural effusion group and non-pleural effusion group were statistically significant(all P<0.05). Further multivariate Logistic regression analysis showed that LDH was a risk factor for NP children receiving respiratory support( P<0.05), the area under the ROC curve of LDH was 0.802, whose the cut-off value was 471.21 U/L.There were no statistically significant differences in the indexes between effusion group and non-pleural effusion group. Conclusion:Children with NP are prone to repeated high fever, high inflammatory markers, and a long course of disease.Staphylococcus aureus is the most common pathogen.Serum LDH≥471.21 U/L is an early independent predictor of respiratory support for NP.

Neoadjuvant chemotherapy in the treatment of locally advanced olfactory neuroblastoma in 25 cases / 中华耳鼻咽喉头颈外科杂志

Objective: To evaluate the efficacy of neoadjuvant chemotherapy (NACT) in the treatment of locally advanced olfactory neuroblastoma (ONB), and to explore the factors related to the efficacy of NACT. Methods: A total of 25 patients with ONB who underwent NACT in Beijing TongRen Hospital from April 2017 to July 2022 were retrospectively analyzed. There were 16 males and 9 females, with an average age of 44.9 years (ranged 26-72 years). There were 22 cases of Kadish stage C and 3 cases of stage D. After multiple disciplinary team(MDT) discussion, all patients were treated sequentially with NACT-surgery-radiotherapy. Among them, 17 cases were treated with taxol, cis-platinum and etoposide (TEP), 4 cases with taxol, nedaplatin and ifosfamide (TPI), 3 cases with TP, while 1 case with EP. SPSS 25.0 software was used for statistical analysis, and survival analyses were calculated based on the Kaplan-Meier method. Results: The overall response rate of NACT was 32% (8/25). Subsequently, 21 patients underwent extended endoscopic surgery and 4 patients underwent combined cranial-nasal approach. Three patients with stage D disease underwent cervical lymph node dissection. All patients received postoperative radiotherapy. The mean follow-up time was 44.2 months (ranged 6-67 months). The 5-year overall survival rate was 100.0%, and the 5-year disease-free survival rates was 94.4%. Before NACT, Ki-67 index was 60% (50%, 90%), while Ki-67 index was 20% (3%, 30%) after chemotherapy [M (Q1, Q3)]. The change of Ki-67 before and after NACT was statistically significant (Z=-24.24, P<0.05). The effects of age, gender, history of surgery, Hyams grade, Ki-67 index and chemotherapy regimen to NACT were analyzed. Ki-67 index≥25% and high Hyams grade were related to the efficacy of NACT (all P<0.05). Conclusions: NACT could reduce Ki-67 index in ONBs. High Ki-67 index and Hyams grade are clinical indicators sensitive to the efficacy of NACT. NACT-surgery-radiotherapy is effective for patients with locally advanced ONB.

Analysis of AI image recognition approach for monitoring primary school students outdoor activity time in school / 中国学校卫生

Objective@#To develop a method that can continuously monitor duration of students outdoor activities for a long time, so as to provide data support for the relationship between outdoor activity duration and students health.@*Methods@#From April 28 to July 6, 2022, 1 168 students from a primary school in Shenzhen were selected. Fixed cameras were placed on the top of school classrooms, corridors and critical paths were used to identify student data and duration in the picture. And AI, cloud computing and other methods were used to measure students-non-classroom time instead of outdoor activity time in school.@*Results@#The average length of time spend on outdoor activities in school of the 24 pilot classes were 67.6-113.0 min, and showed a downward trend by grade ( F =42.74, P <0.05). The duration of outdoor activities among students was negatively correlated with the detection rate of myopia and overweight( r =-0.74, -0.45, P <0.05).@*Conclusion@#The data on outdoor activity time calculated by AI image recognition is basically in line with the reality. Monitoring students outdoor activity time at school through AI image recognition is feasible and popularized.

Anti-depression mechanism of Zuojin Pills:based on UHPLC-TOF-MS, network pharmacology, and experimental verification / 中国中药杂志

This study aims to explore the anti-depression mechanism of Zuojin Pills based on the plasma constituents, network pharmacology, and experimental verification. UHPLC-TOF-MS was used for qualitative analysis of Zuojin Pills-containing serum. Targets of the plasma constituents and the disease were retrieved from PharmMapper and GeneCards. Then the protein-protein interaction(PPI) network was constructed and core targets were screened for GO term enrichment and KEGG pathway enrichment. Cytoscape 3.7.2 was employed construct the "compound-target-pathway" network and the targets and signaling pathways of Zuojin Pills against depression were predicted. CUMS-induced depression mouse model was established to verify the key targets. The results showed that a total of 21 constituents migrating to blood of Zuojin Pills were identified, which were mainly alkaloids. A total of 155 common targets of the constituents and the disease and 67 core targets were screened out. KEGG enrichment and PPI network analysis showed that Zuojin Pills may play a role in the treatment of depression through AMPK/SIRT1, NLRP3, insulin and other targets and pathways. Furthermore, the results of animal experiments showed that Zuojin Pills could significantly improve the depression behaviors of depression, reduce the levels of IL-1β, IL-6 and TNF-α in hippocampus and serum, activate AMPK/SIRT1 signaling, and reduce the protein expression of NLRP3. In conclusion, Zuojin Pills may play a role in the treatment of depression by activating AMPK/SIRT1 signaling pathway, and inhibiting NLRP3 activation and neuroinflammation in the hippocampus of mice.

Research progress on application of multi-enzyme-catalyzed cascade reactions in enzymatic synthesis of natural products / 中国中药杂志

As a biocatalyst, enzyme has the advantages of high catalytic efficiency, strong reaction selectivity, specific target products, mild reaction conditions, and environmental friendliness, and serves as an important tool for the synthesis of complex organic molecules. With the continuous development of gene sequencing technology, molecular biology, genetic manipulation, and other technologies, the diversity of enzymes increases steadily and the reactions that can be catalyzed are also gradually diversified. In the process of enzyme-catalyzed synthesis, the majority of common enzymatic reactions can be achieved by single enzyme catalysis, while many complex reactions often require the participation of two or more enzymes. Therefore, the combination of multiple enzymes together to construct the multi-enzyme cascade reactions has become a research hotspot in the field of biochemistry. Nowadays, the biosynthetic pathways of more natural products with complex structures have been clarified, and secondary metabolic enzymes with novel catalytic activities have been identified, discovered, and combined in enzymatic synthesis of natural/unnatural molecules with diverse structures. This study summarized a series of examples of multi-enzyme-catalyzed cascades and highlighted the application of cascade catalysis methods in the synthesis of carbohydrates, nucleosides, flavonoids, terpenes, alkaloids, and chiral molecules. Furthermore, the existing problems and solutions of multi-enzyme-catalyzed cascade method were discussed, and the future development direction was prospected.

Risk factors for herb-induced liver injury / 临床肝胆病杂志

Drug-induced liver injury (DILI) is one of the common adverse drug reactions and is the main cause of withdrawal of drugs after marketing, which has attracted more and more attention of the public, and herb-induced liver injury (HILI) is a special type of DILI. In recent years, the frequent occurrence of HILI not only seriously endangers the health of patients, but also causes the controversy over the safety of traditional Chinese medicine. Therefore, this article reviews the potential risk factors for HILI from the three aspects of "patient", "drug", and "use", so as to provide a basis for the objective identification, prevention, and control of HILI and a reference for the construction of traditional Chinese medicine pharmacovigilance system represented by liver injury.

Regulation of liver fibrosis by matrix metalloproteinase/tissue inhibitor of metalloproteinase and research advances in related therapeutic drugs / 临床肝胆病杂志

Liver fibrosis is the common consequence of various chronic liver injuries and is mainly characterized by the imbalance between the production and degradation of extracellular matrix, which leads to the accumulation of interstitial collagen and other matrix components. Matrix metalloproteinases (MMPs) and their specific inhibitors, i.e., tissue inhibitors of metalloproteinases (TIMPs), play a crucial role in collagen synthesis and lysis. Through a literature review, this article reviews the experimental studies of liver fibrosis based on MMPs/TIMPs, summarizes the components that may exert an anti-liver fibrosis effect by affecting the expression or activity of MMPs/TIMPs, and attempts to clarify the mechanism of MMPs/TIMPs in regulating collagen homeostasis, so as to provide support for the development of anti-liver fibrosis drugs.

Research progress of Netrin-1 in eye diseases / 国际眼科杂志(Guoji Yanke Zazhi)

@#Netrin-1 is a neuronal axon guidance factor, a soluble protein secreted by the cell floor, and is among the most widely studied members of the Netrins family.Netrin-1 has the diversity of receptors, and its binding to different receptors can activate different biological effects, resulting in multiple functions. Recent studies have revealed that Netrin-1 is closely associated with neural axon guidance, inflammatory responses, neovascularization, apoptosis, and other responses. It can act not only in the central nervous system, but also in many systemic diseases such as those of the respiratory and cardiovascular systems, making it an important target for the treatment of these diseases. In the field of ophthalmology, Netrin-1 is closely associated with optic nerve hypoplasia(ONH), corneal disease, diabetic retinopathy(DR), age-related macular degeneration(ARMD), and retinoblastoma(RB), and has very promising applications in the prevention and treatment of these diseases. In this review, we will explore the association of Netrin-1 with ocular diseases and its role.

Mechanism of action of nucleotide-binding oligomerization domain-like receptor protein 3 inflammasome in liver diseases / 临床肝胆病杂志

Inflammasomes play an important role in the innate immunity of the liver; however, the excessive activation of inflammasomes can lead to liver inflammation and injury. The mechanism of nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3) inflammasome-mediated liver injury has been extensively studied. Related studies have shown that the development of various liver diseases may be associated with the excessive activation of inflammasomes, especially NLRP3 inflammasome. This article reviews inflammasomes, the activation mechanism of NLRP3 inflammasome, and the role of NLRP3 inflammasome in different liver diseases, so as to provide a reference for the treatment targets of liver diseases from the perspective of NLRP3 inflammasome.

Captopril related kidney damage: renal afferent arteriolar responses to angiotensin II and inflammatory signaling / 生理学报

Captopril can have nephrotoxic effects, which are largely attributed to accumulated renin and "escaped" angiotensin II (Ang II). Here we test whether angiotensin converting enzyme-1 (ACE1) inhibition damages kidneys via alteration of renal afferent arteriolar responses to Ang II and inflammatory signaling. C57Bl/6 mice were given vehicle or captopril (60 mg/kg per day) for four weeks. Hypertension was obtained by minipump supplying Ang II (400 ng/kg per min) during the second 2 weeks. We assessed kidney histology by periodic acid-Schiff (PAS) and Masson staining, glomerular filtration rate (GFR) by FITC-labeled inulin clearance, and responses to Ang II assessed in afferent arterioles in vitro. Moreover, arteriolar H2O2 and catalase, plasma renin were assayed by commercial kits, and mRNAs of renin receptor, transforming growth factor-β (TGF-β) and cyclooxygenase-2 (COX-2) in the renal cortex, mRNAs of angiotensin receptor-1 (AT1R) and AT2R in the preglomerular arterioles were detected by RT-qPCR. The results showed that, compared to vehicle, mice given captopril showed lowered blood pressure, reduced GFR, increased plasma renin, renal interstitial fibrosis and tubular epithelial vacuolar degeneration, increased expression of mRNAs of renal TGF-β and COX-2, decreased production of H2O2 and increased catalase activity in preglomerular arterioles and enhanced afferent arteriolar Ang II contractions. The latter were blunted by incubation with H2O2. The mRNAs of renal microvascular AT1R and AT2R remained unaffected by captopril. Ang II-infused mice showed increased blood pressure and reduced afferent arteriolar Ang II responses. Administration of captopril to the Ang II-infused mice normalized blood pressure, but not arteriolar Ang II responses. We conclude that inhibition of ACE1 enhances renal microvascular reactivity to Ang II and may enhance important inflammatory pathways.

Clinical effect of Ranibizumab combined with 577nm micropulse laser in the treatment of severe diabetic macular edema / 国际眼科杂志(Guoji Yanke Zazhi)

AIM:To observe the clinical effect of ranibizumab combined with 577nm micropulse laser in the treatment of severe diabetic macular edema(DME). METHODS:There were 52 eyes of 52 patients diagnosed with severe DME who admitted to the People's Hospital of Guangxi Zhuang Autonomous Region from June 2016 to September 2019. The patients were randomly divided into the observation group(26 patients with 26 eyes, treated with ranibizumab combined with 577nm micropulse laser)and the control group(26 patients with 26 eyes, treated with ranibizumab alone). Patients in both groups received intravitreal injection of ranibizumab with “3+PRN” regimen. Followed up at 9mo after treatment to observe the central macular thickness(CMT), the best corrected visual acuity(BCVA)and the times of intravitreal injection of ranibizumab in the two groups.RESULTS:Compared with before treatment, the CMT and BCVA of the two groups were significantly improved at each time point after treatment(all P<0.001), but there was no difference between the two groups(P>0.05). During the follow-up period, the times of vitreous injection of ranibizumabin the observation group was significantly less than that in the control group(5.88±1.24 times vs 7.12±1.24 times, P=0.001). CONCLUSION:Both ranibizumab combined with 577nm micropulse laser and ranibizumab alone are effective in reducing edema and improving vision in patients with severe DME, but the combination therapy reduces the times of injection.

Cornelia de Lange syndrome with recurrent seizures and SMC1A gene c. 2923C > T mutation: a case report and literature review / 中华实用儿科临床杂志

Objective:To explore the characteristics, clinical manifestations and gene mutation types of Cornelia de Lange syndrome (CdLs), and to improve the understanding of the disease.Methods:Clinical data and gene test results of a pediatric case of CdLs diagnosed in the First Affiliated Hospital of Xinxiang Medical University in August 2019 were analyzed retrospectively.Results:A female patient with 2 years and 8 months old presented a special appearance with a low and flat nose, a wide eye distance, audition ears, a downward inclination of the mouth corner, a high arch of the jaw and a small jaw deformity, who had recurrent seizures, speech and mental retardation.The result of gene test showed the mutation of SMC1A gene c. 2923C > T, and thus the patient was diagnosed as type 2 CdLs. Conclusions:CdLs is a rare genetic metabolic disease with special facial features and physical signs.There is only one case of CdLs with gene mutation of SMC1A in China through literature review.The mutation of SMC1A gene c. 2923C>T in CdLs cases has not been reported at home and abroad, which expands the variation spectrum of the SMC1A gene.

Efficacy and safety of ixazomib-based therapy for multiple myeloma / 白血病·淋巴瘤

Objective:To investigate the efficacy and safety of ixazomib-based therapy for multiple myeloma.Methods:The data of 32 patients with multiple myeloma treated with isazomib-based regimen in the Affiliated Hospital of Jining Medical University from December 2020 to December 2021 were retrospectively analyzed. Among 32 patients, 17 cases were relapsed/refractory, and the remaining 15 cases had initial treatment. The treatment regimens included ID (isazomib + dexamethasone), IRD (isazomib + lenalidomide + dexamethasone) and ICD (isazomib + cyclophosphamide + dexamethasone). The short-term curative effect and adverse reactions of relapsed/refractory patients and patients at initial onset were analyzed.Results:The overall response rate (ORR) of relapsed/refractory patients was 52.9% (9/17), of which 6 cases achieved complete remission (CR), 2 cases achieved very good partial remission (VGPR) and 1 case achieved partial remission (PR). The ORR of refractory patients receiving bortezomib therapy was 40.0% (4/10). The ORR of patients at initial onset who could be evaluated the curative effect was 100.0% (14/14), including 9 cases of CR, 2 cases of VGPR and 3 cases of PR. After treatment, 2 patients (6.2%) had grade Ⅲ-Ⅳ adverse events (1 case of herpes zoster and 1 case of thrombocytopenia), and none of the patients had grade Ⅲ-Ⅳ peripheral neuropathy.Conclusion:Isazomib is effective and safe in the treatment of initially treated and relapsed/refractory multiple myeloma.

Primary amoebic meningoencephalitis in children: a case report and literature review / 中华实用儿科临床杂志

A case of primary amoebic meningoencephalitis (PAM) treated in the First Affiliated Hospital of Xinxiang Medical University in March 6, 2022 was reported.The proband was a 14-year-old boy, who was admitted to the hospital because of " fever, headache and vomiting for 2 days" . Metagenomic next-generation sequencing (mNGS) was consequently conducted to examine the pathogens in cerebrospinal fluid.Naegleria infection was detected, so the child was diagnosed with PAM.The disease developed rapidly, and the patient died 29 hours after admission.In the paper, a total of 13 studies were reviewed, and 15 children with PAM were reported.Of the reported cases, only 1 case survived, 14 cases died.PAM had a low incidence, a dangerous condition, and high mortality.Most cases were diagnosed by autopsy or pathogen diagnosis in cerebrospinal fluid.This case is confirmed by mNGS of pathogens, and it is rarely reported at home and abroad.

Cowden syndrome in children caused by a new mutation of PTEN gene: a case report and literature review / 中华实用儿科临床杂志

Objective:To summarize the clinical and gene mutation characteristics of a child with Cowden syndrome and review the literature.Methods:The clinical data of a child with Cowden syndrome treated in the First Affiliated Hospital of Xinxiang Medical University in June 2020 were analyzed retrospectively.Taking " Cowden syndrome" , " PTEN gene" , " hamartoma polyps" , "child" , " Cowden syndrome and child" and " PTEN and child" as key words, literature was retrieved from Chinese databases (China National Knowledge Internet database and Wanfang database) and the PubMed database from the establishment of the database to March 2021. Results:A 13-year-old male had intermittent abdominal pain and abdominal distension for 5 months.Electron microscopic gastroenteroscopy showed multiple polyps, and focal lymphocyte aggregation and infiltration were found in tissue biopsy.Whole exon sequencing revealed a hemizygous mutation of c. 475 (exon5) A>T in PTEN gene, which led to the transformation of the 159 th amino acid from arginine to tryptophan.The prediction results of the tertiary structure of the protein indicated that the variation might affect the spatial structure of the protein and damage the protein function.According to the clinical characteristics, Cowden syndrome was diagnosed.The pedigree confirmed that the variation was inherited from the mother, who had a similar phenotype.No qualified Chinese report was retrieved.Among 41 English studies on PTEN gene mutation in children, there were only two reports related to pediatric Cowden syndrome.The hemizygous mutation of PTEN gene was not reported. Conclusions:The missense mutation of c. 475 (exon5) A>T in PTEN gene in this study is a novel cause of Cowden syndrome, and the case is the first case report in China.

Efficacy and safety of daratumumab-based combined regimens for treatment of multiple myeloma / 白血病·淋巴瘤

Objective:To evaluate the safety and efficacy of daratumumab in the treatment of multiple myeloma (MM).Methods:The clinical data of 19 MM patients treated with daratumumab alone or in combination with chemotherapy regimens from June 2021 to December 2021 in the Affiliated Hospital of Jining Medical College were retrospectively analyzed, of which 2 patients received daratumumab alone, 6 cases received daratumumab combined with lenalidomide+dexamethasone (DRD) regimen, 1 case received daratumumab combined with liposomal doxorubicin+dexamethasone (DVD) regimen, 2 case received daratumumab combined with dexamethasone+cyclophosphamide+etoposide+cisplatin (DECP) regimen, 3 cases received daratumumab combined with isazomib+dexamethasone (ID) regimen, 2 cases received daratumumab combined with bortezomib+dexamethasone (BD) regimen, and 3 cases received daratumumab combined with dexamethasone (DD) regimen. The efficacy and incidence of adverse effects were analyzed.Results:Among the 19 patients, 8 had complete remission (CR), 1 had very good partial remission (VGPR), 5 had partial remission (PR), 1 had stable disease (SD), and 4 had progressive disease (PD). The overall response rate (ORR) was 73.7% (14/19). The median progression-free survival (PFS) time was 10.42 months (95% CI 8.04-12.79 months) and the median overall survival (OS) time was 52.06 months (95% CI 37.85-66.27 months). The main adverse reactions during treatment were grade 3 neutropenia in 3 cases, grade 3 lymphopenia in 3 cases, grade 2 anemia in 5 cases, grade 2 nausea and vomiting in 7 cases, and infusion-related adverse reactions in 7 cases. Conclusions:Daratumumab-based chemotherapy regimens for the treatment of MM patients can achieve great efficacy with good safety and tolerability.