Paraplegia caused by spinal cord injury is a serious neurological complication, for which surgery is currently the main treatment method. Due to different surgical approaches, patients are usually expected to maintain a passive prone position for a long time or switch between the supine and prone positions. Affected by multiple factors such as neurogenic sensory disorders, pathological changes in muscle tone and operative duration, the risk of intraoperative acquired pressure injury (IAPI) is significantly increased. Current clinical prevention strategies for IAPI in these patients predominantly focus on localized pressure relief during positioning, lacking systematic, standardized comprehensive prevention protocols or evidence-based guidelines. To address it, Department of Nursing, Orthopedics Branch, China International Exchange and Promotive Association for Medical and Health Care, Spinal Trauma Professional Committee, Orthopedics Branch, Chinese Medical Doctor Association, Nursing Group of Spine and Spinal Cord Professional Committee of Chinese Association of Rehabilitation Medicine organized experts in relevant fields to formulate Guideline for the prevention of intraoperative acquired pressure injury in paraplegic patients with spinal cord injury ( version 2025), based on evidence-based medical evidence and latest research results and clinical practice at home and abroad. Eleven recommendations were put forward from the aspects of preoperative risk assessment, intraoperative prevention strategies, postoperative handover and monitoring, and supportive mechanisms for IAPI prevention, aiming to standardize the prevention measures and management strategies of IAPI in paraplegic patients with spinal cord injury and accelerate the recovery of patients and improve the therapeutic effect.

BACKGROUND: Generalized pustular psoriasis (GPP), a rare and recurrent autoinflammatory disease, imposes a substantial burden on patients and society. Awareness of GPP in China remains limited. METHODS: This cross-sectional survey, conducted between September 2021 and May 2023 across 14 hospitals in China, included GPP patients of all ages and disease phases. Data collected encompassed demographics, clinical characteristics, economic impact, disease severity, quality of life, and treatment-related complications. Risk factors for GPP recurrence were analyzed. RESULTS: Among 127 patients (female/male ratio = 1.35:1), the mean age of disease onset was 25 years (1st quartile [Q1]-3rd quartile [Q3]: 11-44 years); 29.2% had experienced GPP for more than 10 years. Recurrence occurred in 75.6% of patients, and nearly half reported no identifiable triggers. Younger age at disease onset ( P  = 0.021) and transitioning to plaque psoriasis ( P  = 0.022) were associated with higher recurrence rates. The median diagnostic delay was 8 months (Q1-Q3: 2-41 months), and 32.3% of patients reported misdiagnoses. Comorbidities were present in 53.5% of patients, whereas 51.1% experienced systemic complications during treatment. Depression and anxiety affected 84.5% and 95.6% of patients, respectively. During GPP flares, the median Dermatology Life Quality Index score was 19.0 (Q1-Q3: 13.0-23.5). This score showed significant differences between patients with and without systemic symptoms; it demonstrated correlations with both depression and anxiety scores. Treatment costs caused financial hardship in 55.9% of patients, underscoring the burden associated with GPP. CONCLUSIONS The substantial disease and economic burdens among Chinese GPP patients warrant increased attention. Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk.
Humans ; Male ; Female ; Psoriasis/pathology* ; Adult ; Cross-Sectional Studies ; Adolescent ; Child ; Young Adult ; Quality of Life ; Middle Aged ; China/epidemiology* ; Recurrence ; Risk Factors ; Surveys and Questionnaires ; East Asian People

Paraplegia caused by spinal cord injury is a serious neurological complication, for which surgery is currently the main treatment method. Due to different surgical approaches, patients are usually expected to maintain a passive prone position for a long time or switch between the supine and prone positions. Affected by multiple factors such as neurogenic sensory disorders, pathological changes in muscle tone and operative duration, the risk of intraoperative acquired pressure injury (IAPI) is significantly increased. Current clinical prevention strategies for IAPI in these patients predominantly focus on localized pressure relief during positioning, lacking systematic, standardized comprehensive prevention protocols or evidence-based guidelines. To address it, Department of Nursing, Orthopedics Branch, China International Exchange and Promotive Association for Medical and Health Care, Spinal Trauma Professional Committee, Orthopedics Branch, Chinese Medical Doctor Association, Nursing Group of Spine and Spinal Cord Professional Committee of Chinese Association of Rehabilitation Medicine organized experts in relevant fields to formulate Guideline for the prevention of intraoperative acquired pressure injury in paraplegic patients with spinal cord injury ( version 2025), based on evidence-based medical evidence and latest research results and clinical practice at home and abroad. Eleven recommendations were put forward from the aspects of preoperative risk assessment, intraoperative prevention strategies, postoperative handover and monitoring, and supportive mechanisms for IAPI prevention, aiming to standardize the prevention measures and management strategies of IAPI in paraplegic patients with spinal cord injury and accelerate the recovery of patients and improve the therapeutic effect.

Clouston syndrome (OMIM #129500), also known as hidrotic ectodermal dysplasia type 2, is a rare autosomal dominant skin disorder. To date, four mutations in the GJB6 gene, G11R, V37E, A88V, and D50N, have been confirmed to cause this condition. In previous studies, the focus has been mainly on gene sequencing, and there has been a lack of research on clinical manifestations and pathogenesis. To confirm the diagnosis of this pedigree at the molecular level and summarize and analyse the clinical phenotype of patients and to provide a basis for further study of the pathogenesis of the disease, we performed whole-exome and Sanger sequencing on a large Chinese Clouston syndrome pedigree. Detailed clinical examination included histopathology, hair microscopy, and scanning electron microscopy. We found a novel heterozygous missense variant (c.134G>C:p.G45A) for Clouston syndrome. We identified a new clinical phenotype involving all nail needling pain in all patients and found a special honeycomb hole structure in the patients' hair under scanning electron microscopy. Our data reveal that a novel variant (c.134G>C:p.G45A) plays a likely pathogenic role in this pedigree and highlight that genetic testing is necessary for the diagnosis of Clouston syndrome.
Humans ; Connexin 30/genetics* ; Connexins/genetics* ; East Asian People ; Ectodermal Dysplasia/pathology* ; Phenotype

OBJECTIVE：To establish a method for simultaneous determination of the contents of codonopatin ，syringin， atractylenolide Ⅰ，atractylenolide Ⅱ and atractylenolide Ⅲ，and to compare the contents of above 5 components in different varieties and harvesting periods of Codonopsis Radix. METHODS ：HPLC method was used. The column was Inertsil ODS- 3 with mobile phase consisted of acetonitrile-water （gradient elution ）at the flow rate of 0.8 mL/min. The detection wavelengths were 210 nm （codonopatin），220 nm （syringin，atractylenolide Ⅱ ，atractylenolide Ⅲ），276 nm （atractylenolide Ⅰ）. The column temperature was set at 30 ℃ ，and the sample size was 20 μ L. RESULTS：The linear range of codonopatin ，syringin， atractylenolide Ⅰ，atractylenolide Ⅱ and atractylenolide Ⅲ were 44.30-886.00 μg/mL（r＝0.999 7），6.50-130.03 μg/mL（r＝0.999 6）， 4.47-89.46 μg/mL（r＝0.999 5），2.53-50.50 μg/mL（r＝0.999 4），5.64-112.80 μg/mL（r＝0.999 5）；the limits of quantification were 2.446 0，0.168 0，0.248 1，0.065 7，0.099 8 μg/mL，and detection limits were 1.352 0，0.067 2，0.005 4，0.006 3，0.007 3 μ g/mL；RSDs of precision ，stability（24 h），repeatability and durability tests were all less than 2％；the recoveries were 98.87％-100.62％（RSD＝0.73％，n＝6），98.46％-101.54％ （RSD＝1.15％，n＝6），98.32％-101.12％（RSD＝1.19％，n＝ 96.83％-104.16％（RSD＝2.62％，n＝6），97.87％-100.99％ （RSD＝1.07％，n＝6）. The average contents were 33.78-431.82， 0-20.60，0.44-3.68，0-10.83，0.27-73.40 μ g/g. The content of 1271985629@qq.com codonopatin was in descending order was as follows as Codonopsis pilosula ＞C. tangshen ＞C. pilosula Nannf. var. modesta （Nannf.） L. T. Shen ＞ecotypic variety of C.·1677· tangshen. The content of syringin in descending order was C. pilosula ＞C. pilosula Nannf. var. modesta（Nannf.）L. T. Shen ＞C. tangshen，but it was not detected in ecotypic variety of C. tangshen . The content of atractylenolide Ⅰ in descending order was C. pilosula Nannf. var. modesta（Namf.）L. T. Shen ＞ecotypic variety of C. tangshen ＞C. pilosula ＞C. tangshen . The content of atractylenolide Ⅱ in C. pilosula was higher than C. pilosula Nannf. var. modesta（Nannf.）L. T. Shen ，but was no detected in C. tangshen and ecotypic variety of C. tangshen . The content of atractylenolide Ⅲ in descending order was C. pilosula ＞C. pilosula Nannf. var. modesta（Nannf.）L. T. Shen ＞ecotypic variety of C. tangshen ＞C. tangshen . In Codonopsis Radix collected from Jul. to Oct. ，the content of codonopatin was the highest ；the content of atractylenolide Ⅰ was lower in sample collected from Jun. to Oct.；atractylenolide Ⅱ was not detected in sample collected in Aug. ；the contents of atractylenolide Ⅰ and atractylenolide Ⅱ were the lower in sample collected in Sept. ，and syringin and atractylenolide Ⅱ were not detected in some samples. CONCLUSIONS ： The established HPLC method is simple ，accurate，highly sensitive and reproducible. It can be used to simultaneously determine 5 active ingredients contents of Codonopsis Radix ；there are great difference in contents of 5 active ingredients in different varieties and harvesting periods of Codonopsis Radix.

A case of cytomegalovirus infection with skin lesions as the primary manifestation is reported.A 46-year-old female patient presented with a 3-month history of painful perioral blisters and erosions,and a 6-week history of erythema,blisters and erosions on the left ann.The patient was ever diagnosed with systemic lupus erythematosus and lupus nephritis 12 years prior to the presentation.Systemic lupus erythematosus was exacerbated 5 months prior to this presentation,and glucocorticoids and mycophenolate mofetil were administered.Skin examination revealed irregularly shaped perioral blisters with erosions and crusts,localized patchy erythema with erosion in the center on the flexor aspect of the left forearm,erythema and blisters on the left upper arm,and multiple petechiae and ecchymoses on the abdomen.Histopathological examination of the skin lesion on the left upper limb showed epidermal necrolysis with scattered viral inclusion bodies.Immunohistochemical examination revealed positive staining for cytomegalovirus antigen in giant cells in the necrolytic epidermis.Cytomegalovirus DNA was detected in exudates from lesions.However,cytomegalovirus DNA was not detected in the serum in the initial test,but became positive (8.04 × 103 copies/ml) 1 week later.In addition,anti-cytomegalovirus IgG antibodies were detected in the serum.The patient was diagnosed with cutaneous cytomegalovirus infection.Affter the treatment with both oral and topical ganciclovir,the lesions were improved gradually,followed by severe pulmonary infection,and the patient was finally died of multiple organ failure.

Psoriasis is a chronic inflammatory skin disease.Its pathogenesis remains unclear,and is currently considered to be co-mediated by heredity,environment and immunity.Signal transducer and activator of transcription 3 (STAT3) is an important transcription factor,which is associated with cancer and autoimmune diseases.STAT3 plays a crucial role in the occurrence of psoriasis by participating in the differentiation of T helper 17 cells,hyperproliferation and abnormal differentiation of keratinocytes,interaction with inflammatory cells,hyperplasia of dermal vessels and other important pathological processes.In recent studies,therapies targeting STAT3 and its upstream Janus kinases (JAK) have shown good efficacy and safety in the management of psoriasis.This review summarizes the association between psoriasis and STAT3,so as to provide evidence for the feasibility of the treatment of psoriasis with targeted inhibition of STAT3.

Objective To report a pedigree with tyrosinemia type Ⅱ,and to analyze its causative mutations.Methods Clinical data were obtained from a 10-year-old male proband with tyrosinemia type Ⅱ,and analyzed retrospectively.Blood and urine samples were collected from 19 persons in 3 generations of the pedigree,and the amino acid level was detected in these samples.Genomic DNA was extracted from all of the 19 family members,and mutations in the tyrosine aminotransferase (TAT) gene were detected.Results The patient developed photophobia at 2 months after birth,and the symptom was gradually aggravated after that.At the age of 6 years,ocular pain and photophobia occurred.At the age of 8 years,linear keratotic plaques occurred on his fingertips and soles of both feet,with obvious tenderness.Ophthalmic examination showed no obvious abnormalities in corneal staining or ocular fundus.Skin examination showed multiple linear keratotic plaques on the fingers and soles of both feet.The serum tyrosine level was 825.64 μmol/L,and the level of p-hydroxyphenyllactic acid in urine was 161.4 μmol/L.Genetic testing showed 2 novel mutations,including c.236G ＞ A at position 236 in exon 2 of the TAT gene causing the substitution of glycine by glutamic acid (p.Gly79Glu),and c.1141G ＞ T at position 1141 in exon 10 of the TAT gene leading to the formation of a premature termination codon instead of glutamic acid (p.Glu381*).The proband was the only patient in the family.Some members in the patrilineal family carried the mutation c.1141G ＞ T (p.Glu381*),and some in the maternal family carried the mutation c.236G ＞ A (p.Gly79Glu).Conclusion This is the first case of tyrosinemia type Ⅱ reported in the domestic population,and 2 novel heterozygous mutations were identified in the TAT gene,which may lead to the occurrence of tyrosinemia type Ⅱ in the patient.

Objective To investigate clinical and histopathological features of lichen planopilaris (LPP).Methods The clinical and histopathological findings in 3 cases of LPP were analyzed.Results All the 3 patients were female,and their average age was 49 years.One patient presented with large-area patchy hair loss on the frontal,parietal and occipital region,and 2 patients presented with irregular patchy hair loss on the scalp and skin atrophy.Besides the hair loss,the eyebrows and axillary hairs also lost in 1 patient.Histopathological examination showed liquefaction degeneration of basal cells in the walls of hair follicles and infiltration of lymphocytes.Infiltration of a small number of lymphocytes was also observed around blood vessels and appendages.Conclusions LPP may only affect the scalp,or involve the other sites all over the body.LPP commonly manifests as patchy hair loss on the scalp and skin atrophy,and is pathologically characterized by liquefaction degeneration of basal cells in hair follicles and infiltration of lymphocytes.

Objective: To explore the effects of sodium arsenite (NaAsO₂) exposure on the activation and extracellular matrix secretion of human hepatic stellate cells, and to provide a theoretical basis for the mechanism study of arsenic induced hepatic fibrosis. Methods: Different doses of NaAsO₂ (0.0, 0.1, 1.0, 10.0, 50.0, 100.0 μmol/L) were exposed to human hepatic stellate cell line (Lx-2) for 24, 48 and 72 huors. CCK-8 assay was used to measure cell viability and IC₅₀ of NaAsO₂ on Lx-2 was then calculated; According to IC₅₀ results, 0.000, 1.875, 3.750, 7.500, and 15.000 μmol/L of NaAsO₂ were exposed to Lx-2 cells for 24 hours, besides, 7.500 μmol/L of NaAsO₂ was exposed to Lx-2 cells for 0, 12, 24, 48, and 72 hours, then collected cells and culture supernatant; HSC activation-related protein, including α-smooth muscle actin (α-SMA), and transforming growth factor-β1 (TGF-β1) expression levels were detected by Western blot analysis, the main extracellular matrix including laminin (LN) , hyaluronic acid (HA), collagen Ⅳ (COL-Ⅳ) and procollagen Ⅲ(P Ⅲ NP) secretion level was detected by Elisa assay. Results: CCK-8 assay showed that the cell viability of Lx-2 cells were increased obviously at low doses (≤1.0 μmol/L) of arsenic exposure, especially at 48 and 72 h. In contrast, with the increasing doses of arsenic exposure, the survival rate of Lx-2 cell was decreased gradually, and the survival rate of the high-dose (50, 100 μmol/L) arsenic exposure group at 24, 48 and 72 h were significantly lower than 0.0 μmol/L group, P<0.05. The IC₅₀ of NaAsO₂ on Lx-2 cells at 24, 48, 72 h were calculated as 72.75, 48.19 and 29.95 μmol/L, respectively; The expression levels of HSC activation-related protein showed that, after treated with 1.875, 3.750, 7.500, 15.000 μmol/L NaAsO₂ for 24 h, α-SMA and TGF-β1 protein level were higher than 0.000 μmol/L group. The increased expression of α-SMA and TGF-β1 protein were most significant in 7.500 μmol/L NaAsO₂ group (P<0.05). In addition, the expression levels of α-SMA and TGF-β1 also showed a time-dependent increasing in Lx-2 cells after treated with 7.500 μmol/L NaAsO₂ for 0, 12, 24, 48 and 72 h; Elisa assay showed that after treated with 1.875, 3.750, 7.500, 15.000 μmol/L NaAsO₂ for 24 h, the secretion levels of HA, LN, COL-Ⅳ and PⅢNP were obvious higher than 0.000 μmol/L group (P<0.05). Moreover, the secretion levels of HA, LN, COL-Ⅳ and P Ⅲ NP also showed a time-dependent increased manner in Lx-2 cells after exposed to 7.500 μmol/L NaAsO₂ for 0, 12, 24, 48 and 72 h (P<0.05). Conclusion NaAsO₂ exposure to Lx-2 cells can upregulate the expression level of HSC activation-related proteins, induce its further activation, then increase ECM secretion level.