Chinese expert guidance on overall application of lenvatinib in hepatocellular carcinoma / 中华消化外科杂志

Lenvatinib mesylate is an oral receptor tyrosine kinase inhibitor against targets of vascular endothelial growth factor receptors 1-3, fibroblast growth factor receptors 1-4, platelet-derived growth factor receptor α, stem cell growth factor receptor, and rearranged during transfection, et al. Lenvatinib has been approved by the National Medical Products Administration of China on September 4,2018, for the first-line treatment of patients with unresectable hepatocellular carcinoma who have not received systematic treatment before. Up to February 2023, Lenvatinib has been listed in China for more than 4 years, accumulating a series of post-marketing clinical research evidences. Based on the clinical practice before and after the launch of lenvatinib and referring to the clinical experience of other anti-angiogenesis inhibitors, domestic multidisciplinary experts and scholars adopt the Delphi method to formulate the Chinese Expert Guidance on Overall Application of Lenvatinib in Hepatocellular Carcinoma after repeated discussions and revisions, in order to provide reference for reasonable and effective clinical application of lenvatinib for clinicians.

A historical review of dental schools founded by dentist Situ Bo in modern time / 中华口腔医学杂志

About 8 dental schools were founded by Chinese dentists during modern times (1909-1949) in China. Only one still worked after the founding of the People's Republic of China, which was one of the three dental schools founded by dentist Situ Bo. There were no systematic researches on Situ Bo's participation in dentistry education. This paper analyzes the founder's experience in dentistry and in the two schools he founded at the beginning, focusing on the background and process of the schools' construction, management, teaching, teachers and students training, etc. The results show that the establishment of the first two dental schools have laid important foundation for the establishment and development of Shanghai Dental College by the reserve of trained professional teachers and accumulated valuable experience in running schools. The systematic review of this history will help us better understand the establishment and development of Shanghai Dental College and the efforts in dentistry education made by dentists growed up in modern China, as well as the early development process of stomatology.

Diagnosis and treatment of malignant mesothelioma of the tunica vaginalis testis: a series of 7 cases / 中华外科杂志

Objective: To investigate the clinical features, diagnosis, prognosis of malignant mesothelioma of the tunica vaginalis testis (MMTVT). Methods: The clinicopathological data of 7 patients with MMTVT who treated at Sun Yat-sen University Cancer Center between January 2010 and October 2022 were retrospectively reviewed. Cases were first diagnosed at (M (IQR)) 49 (23) years old (range: 27 to 64 years old). The main clinical manifestations were scrotal enlargement (7 cases) and hydrocele (2 cases). Results: Three patients underwent radical orchiectomy as initial treatment, 2 cases underwent hydrocelectomy due to diagnosis of hydrocele, followed by radical orchiectomy at Sun Yat-sen University Cancer Center, and 2 cases underwent transscrotal orchiectomy. Common tumor markers of testicular cancer were not significantly elevated in MMTVT. The expression of tumor PD-L1 was positive in 2 out of the 3 cases. One patient received adjuvant chemotherapy and 2 patients received first-line chemotherapy after tumor recurrence. Chemotherapy regimens used include cisplatin+pemetrexed. Up to October 2022, 3 cases relapsed, of which 2 cases died. The median overall survival was 35 months (range: 4 to 87 months) and the median progression-free survival was 6 months (range: 2 to 87 months). Conclusions: MMTVT at early stage should be treated with early radical orchiectomy and followed up closely after surgery. The cisplatin+pemetrexed regimen is a common option for the treatment of metastatic MMTVT, while whether immune checkpoint inhibitors could serve as a second-line treatment option deserves further research.

Population structure of food-borne Staphylococcus aureus in China / 中华流行病学杂志

Objective: To understand the population structure of food-borne Staphylococcus (S.) aureus in China. Methods: Whole genome sequencing was used to analyze 763 food-borne S. aureus strains from 16 provinces in China from 2006 to 2020. Multilocus sequence typing (MLST), staphylococcal protein A gene (spa) typing, and staphylococcal chromosome cassettemec (SCCmec) typing were conducted, and minimum spanning tree based on ST types (STs) was constructed by BioNumerics 7.5 software. Thirty-one S. aureus strains isolated from imported food products were also included in constructing the genome phylogenetic tree. Results: A total of 90 STs (20 novel types) and 160 spa types were detected in the 763 S. aureus isolates. The 72 STs (72/90, 80.0%) were related to 22 clone complexes. The predominant clone complexes were CC7, CC1, CC5, CC398, CC188, CC59, CC6, CC88, CC15, and CC25, accounting for 82.44% (629/763) of the total. The STs and spa types in the predominant clone complexes changed over the years. The methicillin-resistant S. aureus (MRSA) detection rate was 7.60%, and 7 SCCmec types were identified. The ST59-t437-Ⅳa (17.24%, 10/58), ST239-t030-Ⅲ (12.07%, 7/58), ST59-t437-Ⅴb (8.62%, 5/58), ST338-t437-Ⅴb (6.90%, 4/58) and ST338-t441-Ⅴb (6.90%, 4/58) were the main types in MRSA strains. The genome phylogenetic tree had two clades, and the strains with the same CC, ST, and spa types clustered together. All CC7 methicillin sensitive S. aureus strains were included in Clade1, while 21 clone complexes and all MRSA strains were in Clade2. The MRSA strains clustered according to the SCCmec and STs. The strains from imported food products in CC398, CC7, CC30, CC12, and CC188 had far distances from Chinese strains in the tree. Conclusions: In this study, the predominant clone complexes of food-borne strains were CC7, CC1, CC5, CC398, CC188, CC59, CC6, CC88, CC15, and CC25, which overlapped with the previously reported clone complexes of hospital and community-associated strains in China, suggesting that close attention needs to be paid to food, a vehicle of pathogen transmission in community and food poisoning.

Study of the urban-impact on microbial communities and their virulence factors and antibiotic resistance genomes in the Nandu River, Haikou / 中华流行病学杂志

Objective: To explore the changes in bacterial community structure, antibiotic resistance genome, and pathogen virulence genome in river water before and after the river flowing through Haikou City and their transmission and dispersal patterns and to reveal anthropogenic disturbance's effects on microorganisms and resistance genes in the aquatic environment. Methods: The Nandu River was divided into three study areas: the front, middle and rear sections from the upstream before it flowed through Haikou City to the estuary. Three sampling sites were selected in each area, and six copies of the sample were collected in parallel at each site and mixed for 3 L per sample. Microbial community structure, antibiotic resistance, virulence factors, and mobile genetic elements were analyzed through bioinformatic data obtained by metagenomic sequencing and full-length sequencing of 16S rRNA genes. Variations in the distribution of bacterial communities between samples and correlation of transmission patterns were analyzed by principal co-ordinates analysis, procrustes analysis, and Mantel test. Results: As the river flowed through Haikou City, microbes' alpha diversity gradually decreased. Among them, Proteobacteria dominates in the bacterial community in the front, middle, and rear sections, and the relative abundance of Proteobacteria in the middle and rear sections was higher than that in the front segment. The diversity and abundance of antibiotic resistance genes, virulence factors, and mobile genetic elements were all at low levels in the front section and all increased significantly after flow through Haikou City. At the same time, horizontal transmission mediated by mobile genetic elements played a more significant role in the spread of antibiotic-resistance genes and virulence factors. Conclusions: Urbanization significantly impacts river bacteria and the resistance genes, virulence factors, and mobile genetic elements they carry. The Nandu River in Haikou flows through the city, receiving antibiotic-resistant and pathogen-associated bacteria excreted by the population. In contrast, antibiotic-resistant genes and virulence factors are enriched in bacteria, which indicates a threat to environmental health and public health. Comparison of river microbiomes and antibiotic resistance genomes before and after flow through cities is a valuable early warning indicator for monitoring the spread of antibiotic resistance.

Comparison study of clinical features between persistent hyperplastic primary vitreous and congenital fibrovascular pupillary membrane / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To analyze the similarities and differences of the clinical features between persistent hyperplastic primary vitreous(PHPV)and congenital fibrovascular pupillary membrane(CFPM).METHODS: Retrospectively analyze the ocular biometric parameters, clinical features and morphological changes in children with PHPV(PHPV group)and CFPM(CFPM group)who received surgery at the department of ophthalmology, Xijing Hospital from March 2006 to December 2021.RESULTS: The study included 56 cases(61 eyes)of PHPV and 24 cases(25 eyes)of CFPM. There were no differences on the gender and age of onset between PHPV and CFPM, and both of them were mainly unilaterally affected, with the ratio of 91% and 96%. Children with PHPV and cataract combined with other complications and ocular developmental abnormalities. CFPM was mainly presented different degrees of blockage and morphological abnormalities of pupillary area. In unilateral cases of PHPV and CFPM, the anterior chamber depth(ACD)of affected eyes was smaller than that of the fellow eyes, and in subgroups with age of operation ≤24mo, the axial length(AL)of affected eyes was smaller than that of the fellow eyes(P<0.05). The corneal diameter(CD)of the affected eyes in PHPV group was smaller and the intraocular pressure(IOP)was higher than those of the fellow eyes(all P<0.05); while there were no significant differences on CD and IOP between affected eyes and the fellow eyes in CFPM group(P>0.05). The ACD of affected eyes in PHPV group was significantly smaller than that of CFPM group(P<0.05). The fibrovascular membrane in PHPV group was located in the posterior part of the lens and vitreous cavity; while the fibrovascular membrane in CFPM group was located between the iris and the anterior capsule of the lens, rarely involving the lens.CONCLUSION: PHPV and CFPM had the similar clinical features, suggesting that they may belong to the different variants of persistent fetal vasculature(PFV). However, PHPV had a wider range of lesions and more complex conditions.

Efficacy comparison of Jensen and augmented Hummelsheim procedure in the treatment of complete paralytic esotropia / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To compare the efficacy of Jensen and augmented Hummelsheim procedures in the treatment of complete paralytic esotropia.METHOD: A total of 35 patients(44 eyes)who were diagnosed with complete paralytic esotropia from October 2016 to October 2020 were retrospectively analyzed, of which 15 cases(21 eyes)underwent Jensen procedure combined with recession of antagonist muscle(Jensen procedure group), and 20 cases(23 eyes)received augmented Hummelsheim procedure combined with recession of antagonist muscle(Hummelsheim procedure group). The operation time, preoperative and postoperative esotropia deviation, degree of abduction paralysis, recession of medial rectus muscle and cure rate were observed.RESULTS: Clinical data and operation time of the patients in two groups were not statistically significant(P >0.05). During the last follow-up, the esotropia deviation of Jensen procedure group decreased from 102.33±41.70PD to 3.93±4.82PD(P<0.001), and it decreased from 94.75±33.03PD to 2.85±5.96PD in Hummelsheim procedure group(P<0.001), while the degree of abduction paralysis were significantly improved from -4.81±0.40 to -1.57±0.51 in the Jensen procedure group(P<0.001)and from -4.91±0.29 to -1.22±0.42 in Hummelsheim procedure group(P<0.001). Besides, there was no statistical difference in postoperative esotropia deviation between the two groups(P>0.05), but the degree of postoperative abduction paralysis in the Hummelsheim procedure group was significantly better than that of Jensen procedure group(P<0.05). The recession of medial rectus muscle of the two groups were 7.16±2.07 and 6.37±2.34 mm, respectively(P>0.05). During the last follow-up, in the Jensen procedure group, 2 patients were undercorrection(+10PD and +12PD respectively)and 13 cases(87%)were cured. In the Hummelsheim procedure group, 1 patient was undercorrection(+25PD)and 19 patients were cured(95%), and there was no statistical significance in cure rates of the two groups(P=0.565).CONCLUSIONS: Both Jensen procedure and augmented Hummelsheim procedure can effectively treat complete paralytic esotropia, and the latter is more effective in improving the abduction paralysis.

Homeostatic medicine: new strategy and concept of health maintenance as well as diagnosis and treatment of diseases / 中华口腔医学杂志

Homeostasis is a dynamic balance process of self-regulating. Biological systems remain stable through adapting to changing external conditions to maintain normal life activities. Homeostatic medicine is the science of studying homeostasis of human molecules, cells, organs and the whole body. It is a comprehensive discipline based on maintaining homeostasis to keep human health and assist for diseases prevention and diagnoses. Homeostatic medicine focuses on the whole body and on the role of homeostasis in health and disease, which is expected to provide new ideas and strategies for maintaining health as well as diagnosing and treating diseases. Nitric oxide (NO) plays an important role in the control of multisystem homeostasis. Nitrate is an important substance in regulating NO homeostasis through the nitrate-nitrite-NO pathway. Sialin, nitrate transporter which is located in the cell membrane and cytoplasm, mediates multiple cellular biological functions. The nitrate-nitrite-NO pathway and sialin-mediated biological functions play an important role in the regulation of body homeostasis.

Efficacy of partial nephrectomy in patients with localized renal carcinoma: a 20-year experience of 2 046 patients in a single center / 中华外科杂志

Objectives: To analyze the long-term survival of patients with localized renal cell carcinoma after partical nephrectomy. Methods: The clinicopathological records and survival follow-up data of 2 046 patients with localized renal cell carcinoma, who were treated with partial nephrectomy from August 2001 to February 2021 in the Department of Urology, Sun Yat-sen University Cancer Center, were retrospectively analyzed. There were 1 402 males and 644 females, aged (M(IQR)) 51 (19) years (range: 6 to 86 years). The primary end point of this study was cancer-specific survival. Survival curves were estimated using the Kaplan-Meier method, and the difference test was performed by Log-rank test. Univariate and multivariate Cox analysis were fitted to determine factors associated with cancer-specific survival. Results: The follow-up time was 49.2 (48.0) months (range: 1 to 229 months), with 1 974 patients surviving and 72 dying. The median cancer-specific survival time has not yet been reached. The 5- and 10-year cancer specific survival rates were 97.0% and 91.2%, respectively. The 10-year cancer-specific survival rates for stage pT1a (n=1 447), pT1b (n=523) and pT2 (n=58) were 95.3%, 81.8%, and 81.7%, respectively. The 10-year cancer-specific survival rates of patients with nuclear grade 1 (n=226), 2 (n=1 244) and 3 to 4 (n=278) were 96.6%, 89.4%, and 85.5%, respectively. There were no significant differences in 5-year cancer-specific survival rates among patients underwent open, laparoscopic, or robotic surgery (96.7% vs. 97.1% vs. 97.5%, P=0.600). Multivariate analysis showed that age≥50 years (HR=3.93, 95%CI: 1.82 to 8.47, P<0.01), T stage (T1b vs. T1a: HR=3.31, 95%CI: 1.83 to 5.99, P<0.01; T2+T3 vs. T1a: HR=2.88, 95%CI: 1.00 to 8.28, P=0.049) and nuclear grade (G3 to 4 vs. G1: HR=2.81, 95%CI: 1.01 to 7.82, P=0.048) were independent prognostic factors of localized renal cell carcinoma after partial nephrectomy. Conclusions: The long-term cancer-specific survival rates of patients with localized renal cancer after partial nephrectomy are satisfactory. The type of operation (open, laparoscopic, or robotic) has no significant effect on survival. However, patients with older age, higher nuclear grade, and higher T stage have a lower cancer-specific survival rate. Grasping surgical indications, attaching importance to preoperative evaluation, perioperative management, and postoperative follow-up, could benefit achieving satisfactory long-term survival.

Drug resistance and genomic characteristics of a strain of O139 Vibrio cholerae isolated from human bloodstream infection / 中华预防医学杂志

Objective: To analyze the drug resistance and genomic characteristics of a strain of serogroup O139 Vibrio cholerae producing cholera toxin isolated from the bloodstream of a person with bacteremia. Methods: The broth dilution method and automatic drug sensitivity analyzer were used to determine the antibiotic sensitivity of the strain. The complete genome sequence of the strain was obtained by using second-generation gene sequencing and nanopore sequencing. BLAST software was used for comparison and analysis with CARD, Resfinder, ISfinder, VFDB, and other databases. The drug-resistant genes, insertion sequences and virulence genes carried by the strain were identified. MEGA 5.1 software was used to construct a genetic phylogenetic tree based on the core genomic single nucleotide polymorphisms. Results: V. cholerae SH400, as the toxigenic strain, carried multiple virulence-related genes and four virulence islands. The strain was resistant to streptomycin, tetracycline and cotrimoxazole, carrying corresponding drug-resistant genes. The strain also carried IncA/C plasmid with the size of 172914 bp and contained 10 drug-resistant genes. Combined with the genomic evolutionary relationship, this study found that the drug-resistant genes and drug-resistant plasmids carried among strains showed certain aggregation. The traditional ST type of strain SH400 was ST69, and the cgMLST type was a new type highly similar to cgST-252. Conclusion: This strain of serogroup O139 V. cholerae carries the ctxAB gene, multiple drug-resistant genes and IncA/C plasmid, and there are multiple drug-resistant islands.

Research progress of laboratory biomarker for colorectal cancer / 中华预防医学杂志

Colorectal cancer (CRC) has become a major medical and public health threat to human life and health. At present, the clinical diagnosis and treatment of CRC mainly depends on the laboratory tests. With the increasing demand for treatment and prognosis, screening methods for CRC are emerging. In order to provide a reference for reasonable selection of laboratory diagnostic biomarkers, and further improve the accuracy and reliability of colorectal cancer screening, auxiliary diagnosis, efficacy monitoring, as well as prognostic evaluation, this article reviews the laboratory screening and diagnostic methods for CRC, and makes outlook for the future detection markers of CRC.

Establishment and evaluation of a method for phages enrichment by ferric trichloride-polyvinylidene fluoride membrane filter / 中华预防医学杂志

Objective: To establish and evaluate a method of enriching bacteriophages in natural water based on ferric trichloride-polyvinylidene fluoride (FeCl3-PVDF)membrane filter. Methods: Based on the principle of flocculation concentration, the method of recovering bacteriophage from water sample was established by using iron ion flocculation combined with membrane filter. The titer of phage was determined by Agar double layer method. The recovery efficiency of phage was detected by phage fluorescence staining and real-time fluorescence PCR reaction. Water samples from different sources were collected for simulation experiment to evaluate the enrichment effect. At the same time, the sewage discharged from hospitals was taken as the actual water sample, and the common clinical drug-resistant bacteria were used as the host indicator bacteria to further analyze the enrichment effect of FeCl3-PVDF membrane filter rapid enrichment method on the bacteriophage in natural water samples. Results: The method of enrichment of bacteriophages in natural water by iron ion concentration 50 mg/L and PVDF membrane filter was established. The recovery rate of this method for bacteriophage was 93%-100%. Under the multi-functional microscope, it was found that the bacteriophage of the enriched water sample increased significantly and the fluorescence value of the enriched water sample determined by the enzyme labeling instrument was about 13 times as high as that before enrichment. After concentration of the actual water samples from the hospital drainage, the positive rate of bacteriophage isolation in the concentrated group and the non-concentrated group was 23% and 4%, and the fluorescence value in the concentrated group was 2-24 times as high as that of the non-concentrated group. Conclusion: The method of FeCl3-PVDF membrane filter is a simple, efficient and rapid method for enriching bacteriophages in different water samples.

Research progress on the correlation between visual impairment and cognitive impairment / 国际眼科杂志(Guoji Yanke Zazhi)

As aging societies enter worldwide, the elderly population is increasing year by year, and visual and cognitive impairment have thus become important global issues, imposing a significant socioeconomic burden worldwide. In recent years, a large number of studies have shown a strong association between visual and cognitive impairments, with patients with visual impairment being more likely to experience cognitive decline, especially dementia. In addition, there is also evidence that improving visual acuity has a significant effect on cognitive function, and that by improving visual function, the development of cognitive decline and dementia can be delayed, which provides new ideas for public health efforts to delay and mitigate cognitive decline and dementia by improving visual function. The possible mechanisms underlying the association between visual and cognitive impairment remain unclear and need to be studied further.

Clinical characteristics and treatment of patients with acute acquired concomitant esotropia in different refractive status / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To analyze the clinical characteristics and treatment of patients with acute acquired concomitant esotropia(AACE)in different refractive status.METHODS: A retrospective analysis was conducted on 110 patients with non-type I AACE treated from January 2020 to January 2022. The non-myopic group(30 cases, spherical equivalent&#x003E;-0.5D)and the myopic group(80 cases, spherical equivalent≤-0.5D)were divided according to the refractive status. The degree of deviation, accommodative convergence and accommodation ratio(AC/A), visual function, and surgical methods were observed. RESULTS: The non-myopic group had no difference in the degree of near deviation [(47.13±23.54)△] and the degree of distant deviation [(48.90±22.59)△](P&#x003E;0.05); near deviation [(40.49±26.09)△] of myopic group was less than distant deviation [(50.09±25.41)△](P&#x003C;0.001); and there was no difference in the same distance between the two groups(P&#x003E;0.05). AC/A in the non-myopic group(5.40±2.23)was higher than that in the myopic group(3.14±3.10; P&#x003C;0.05). Patients in the myopic group had better near stereopsis than the non-myopic group(P&#x003C;0.05). The non-myopic group had a variety of surgical methods, while the myopic group mostly used lateral rectus resection or/and medial rectus recession.CONCLUSION: AACE can occur in different refractive status. Non-myopic patients have the same degree of distant and near strabismus, high AC/A, and varied surgical methods. However, myopic patients have less degree of near deviation than distant deviation and have normal AC/A and better near stereopsis, and lateral rectus resection or/and medial rectus recession are commonly used.

Clinical practice guidelines and real-life practice in hepatocellular carcinoma: A Chinese perspective

Liver cancer is the fourth most prevalent and the second most lethal cancer in China. Hepatitis B virus (HBV) infection represents a major risk factor for hepatocellular carcinoma (HCC). Liver ultrasonography plus alpha-fetoprotein every 6 months continues to be the predominant surveillance modality. The age-Male-ALBI-Platelets score was recommended in the recent 2022 Chinese guidelines to predict HCC occurrence. The Chinese liver cancer (CNLC) staging system proposed in the 2017 guidelines continues to be the standard model for staging with modifications in the treatment allocations. Considering the aggressive nature of HBV-associated HCC, multimodal and high-intensity strategies like the addition of immunotherapy-based systemic treatment to local therapies, including resection, ablation, and intra-arterial therapies, have been adopted in real-life practices in China. The latest Chinese guidelines recommend atezolizumab plus bevacizumab, suntilimab plus a bevacizumab analog, lenvatinib, sorafenib, donafenib, and FOLFOX (folinic acid, fluorouracil, and oxaliplatin) chemotherapy as first-line treatment without priority. Regorafenib, apatinib, camrelizumab, and tislelizumab have been added as second-line systemic therapies for patients who progressed on sorafenib. Systemic therapies adopted in real-life practice are sophisticated with various combination modalities and different sequences.

Research progress of macrophage polarization in the treatment of aortic dissection / 中国胸心血管外科临床杂志

@#Aortic dissection is one of the most devastating cardiovascular diseases. One of the most important pathological features of aortic dissection is local inflammatory response, including the infiltration of inflammatory cells, extracellular matrix degradation, and smooth muscle cell phenotype switch. Macrophages which are the core of the inflammatory response play an extremely pivotal role in the progression of inflammation and tissue remodeling. Macrophages can be artificially divided into M1 and M2 types, of which the M1-type promotes inflammation while the M2-type is associated with the regression of inflammation and tissue healing. Mastering the switch of phenotypic transformation of macrophages may be of great help in inhibiting the inflammation of aortic tissue and facilitating tissue healing, as well as the treatment of aortic dissection. In this paper, we focus on the polarization of macrophages and discuss the role of macrophages in aortic dissection, the polarization pathway and the effect of related polarizing agents on the treatment of aortic dissection.

Clinical analysis of a family with autosomal recessive spastic paraplegia caused by IBA57 gene mutations / 中华神经科杂志

Objective:To investigate the clinical, imaging and gene variation characteristics of hereditary spastic paraplegia type 74 caused by mutations in IBA57 gene. Methods:A retrospective analysis was performed on 2 cases of autosomal recessive spastic paraplegia caused by mutations in IBA57 gene who visited the Department of Neurology, the Affiliated Wuxi Children′s Hospital of Nanjing Medical University in 2010 and 2021, and the patients′ clinical data were collected. Results:The 2 patients were siblings with onset age of 4 years and 7 months, 1 year and 3 months, respectively. The same compound heterozygous mutations in IBA57 gene were found in the sibling patients [c.473G>C (p.R158P) and c.697C>T (p.R233X)]. Both patients were diagnosed as spastic paraplegia type 74. They had mild to moderate gait abnormalities, optic atrophy, decreased vision, and leukodystrophy with periventricular white matter abnormality, but no obvious growth and mental retardation in developmental assessment. Conclusions:Cases of spastic paraplegia type 74 caused by compound heterozygous mutations in IBA57 gene mainly manifested as childhood onset and slowly progressive inferior spasmodic weakness. The patients did not display significant cognitive impairment, and imaging examinations showed obvious leukodystrophy.

Clinical efficacy and safety of secukinumab in the treatment of 13 cases of refractory localized pustular psoriasis / 中华皮肤科杂志

Objective:To evaluate clinical efficacy and safety of secukinumab in the treatment of patients with localized pustular psoriasis irresponsive or intolerant to conventional treatment.Methods:Clinical data were collected from 13 patients with refractory localized pustular psoriasis, who received secukinumab treatment in Department of Dermatology, Xijing Hospital from December 2019 to April 2022. Efficacy was evaluated by comparing palmoplantar pustulosis (PPP) area and severity index (PPPASI) score and physician global assessment (PGA) score in PPP patients before and after the treatment, and by comparing clinical global impression (CGI) score in patients with acrodermatitis continua of Hallopeau (ACH). Adverse events were recorded during the treatment.Results:Among the 13 patients with refractory localized pustular psoriasis, 6 were diagnosed with PPP, 3 with ACH, and 4 with PPP complicated by ACH. There were 3 males and 10 females, and their age was 33.2 ± 14.6 years. After 12-week treatment, the PPPASI score decreased from 13.88 ± 3.62 points at baseline to 6.81 ± 2.31 points in 10 patients presenting with PPP lesions, 4 achieved 75% improvement in the PPPASI score (PPPASI75), and 5 achieved PGA0/1; at the same time, 6 of the 7 patients presenting with ACH lesions achieved moderate or marked improvement in the CGI score, and 4 of the 6 patients achieved marked improvement. Two patients with PPP discontinued the treatment after 3- and 5-week treatment respectively due to poor response, and 1 patient with ACH achieved mild improvement in the CGI score after 12-week treatment. No severe adverse events were reported during the treatment. However, inflammatory follicular papules occurred in 1 patient, and eczematoid lesions occurred in another 1 patient, which both regressed after symptomatic treatment.Conclusion:Secukinumab was effective and safe in the treatment of refractory localized pustular psoriasis, and may serve as a new treatment option for refractory PPP and ACH.

Efficacy and safety of ultra rapid lispro in the treatment of type 2 diabetes mellitus: a randomized controlled clinical trial / 中华内科杂志

Objective:To evaluate and compare the efficacy and safety of ultra-rapid lispro insulin (URLi) and humalog lispro (HL) in the treatment of type 2 diabetes mellitus.Methods:This was an international multicenter, double-blind, randomized controlled study. From May 2019 to January 2021, a total of 481 patients with type 2 diabetes mellitus, who had been using insulin for at least 90 days and had poor glycemic control, were included. These patients were recruited from 34 research centers in China, including Shanghai Jiao Tong University School of Medicine Affiliated Sixth People′s Hospital. They were assigned to either the URLi group (319 patients) or the HL group (162 patients) using stratified blocked randomization. The primary endpoint was the change in hemoglobin A 1c (HbA 1c) relative to baseline after 26 weeks of treatment. Secondary endpoints included the proportion of patients who achieved HbA 1c<7.0% and ≤6.5% after 26 weeks of treatment, 1-h postprandial glucose (1hPG) or 2-h postprandial glucose (2hPG) excursions during a mixed meal tolerance test at week 26, as well as safety parameters. Continuous variables were compared using mixed model repeated measures or analysis of covariance, and categorical variables were compared using logistic regression or Fisher′s exact test. Results:Data based on the Chinese subgroup showed that there were no statistically significant differences between the URLi and HL groups in terms of male percentage [56.1% (179/319) vs. 56.2% (91/162); P=0.990], age [(59.5±8.4) vs. (59.6±9.3) years; P=0.839] and other baseline characteristics. Regarding the change in HbA 1c relative to baseline, the URLi group was non-inferior to the HL group (-0.59%±0.05% vs. -0.66%±0.06%; P=0.312). There were no statistically significant differences between the URLi and HL groups in proportion of patients who achieved HbA 1c<7.0% [47.3% (138/292) vs. 45.2% (70/155); P=0.907] and≤6.5% [27.7% (81/292) vs. 27.7% (43/155); P=0.816]. The excursions in 1hPG [(6.20±0.21) vs. (6.90±0.25) mmol/L; P=0.001] and 2hPG [(8.10±0.27) vs. (9.30±0.31) mmol/L; P<0.001] were lower in the URLi group than the HL group, with statistically significant differences. In terms of safety, there were no statistically significant differences in the percentage of subjects who reported treatment-emergent adverse events between the URLi and HL groups [49.8% (159/319) vs. 50.0% (81/162); P=1.000]. The event rate of nocturnal hypoglycemia was lower in the URLi group than the HL group, with statistically significant differences [(0.53±0.10) vs. (0.89±0.16) events per patient -year; P=0.040]. Conclusions:With good glycemic control, URLi showed non-inferiority for HbA 1c improvement versus HL and was superior to HL for postprandial glucose excursion control. Meanwhile the rate and incidence of nocturnal hypoglycemia were lower in the URLi group than the HL group.

Management of primary adrenal lymphoma with intracranial invasion / 中华内分泌代谢杂志

Primary adrenal lymphoma (PAL) is a rare extranodular lymphoma. It is subject to misdiagnosis due to atypical clinical and imaging features. Histopathology is required to establish the diagnosis. Patients often present to the Endocrinology Department upon revelation of adrenal incidentalomas by imaging. PAL is often accompanied by invasion of other tissues and organs, with a high mortality rate and a poor prognosis. In this paper, the clinical features of a patient with primary adrenal lymphoma and intracranial invasion were summarized, and the pathogenesis, clinical manifestations, diagnosis and treatment of this disease were reviewed.