Xeroderma pigmentosum(XP)is a rare autosomal recessive disorder related to XP gene variation,often causing secondary malignant tumors.This article reports a case of a young child with secondary squamous cell carcinoma of the lower lip caused by xero-derma pigmentosum.Through a review of relevant literature,the clinical characteristics,pathogenesis,clinical pathological manifesta-tions,and prognosis of XP are discussed.

OBJECTIVE To investigate the application value of narrow-band CE-Chirp ASSR(NB CE-Chirp ASSR)and modulated acoustic ASSR in the assessment of hearing threshold in children with hearing impairment.METHODS Forty-eight children with sensorineural hearing loss were tested by pure tone audiometry(PTA),NB CE-Chirp ASSR and modulated acoustic ASSR.According to the results of pure tone audiometry,they were divided into mild to moderate group(48 ears)and severe to profound group(48 ears).The difference and correlation between pure tone hearing threshold and ASSR response threshold of different stimuli were compared between 500-4 000 Hz.RESULTS The absolute differences between the NB CE-Chirp ASSR response threshold and the PTA threshold in 48 children were all smaller than the differences between the modulated acoustic ASSR response threshold and the PTA threshold,and the differences were statistically significant(P<0.001).Under the same stimulus sound,the absolute difference between ASSR response threshold and pure tone hearing threshold in the mild to moderate group was higher than that in the severe to very severe group.The correlation coefficients between NB CE-Chirp ASSR threshold and pure tone hearing threshold are higher than those between modulated sound ASSR threshold and pure tone hearing threshold at 500-4 000 Hz.The test time of NB CE-Chirp ASSR[(20.92±9.33)min]was significantly shorter than that of modulated acoustic ASSR[(33.68±10.97)min](P=0.004).CONCLUSION NB CE-Chirp ASSR can more accurately assess the hearing threshold of children with different degrees of hearing loss than modulated acoustic ASSR.

Objective To explore the characteritics of spontaneous brain functional activities for patients with hand dysfunction after ischemic stroke using resting state functional magnetic resonance imaging(rs-fMRI).Methods From August,2024 to June,2025,23 patients with hand dysfunction after ischemic stroke were as patients group,and ten age-matched healthy adults were recruited as healthy control group.The patients group was as-sessed with Fugl-Meyer Assessment-Upper Extremities(FMA-UE),Wolf Motor Function Test(WMFT)and modified Lindmark Scale.All subjects underwent rs-fMRI examinations,and the amplitude of low frequency fluctuations(ALFF),fractional amplitude of low frequency fluctuations(fALFF)and regional homogeneity(Re-Ho)were compared between two groups.The resting state functional network was constructed based on indepen-dent component analysis,and the functional connectivity(FC)were analyzed.The rs-fMRI indicators of the pa-tients and the clinical scale scores were analyzed.Results Compared with the healthy control group,the zALFF value decreased in the precentral gyrus(P<0.05,FWE corrected)in the patient group,as well as the values of zfALFF in Cerebelum-Crus2-R(P<0.05,FWE correct-ed),and ReHo values in the putamen(P<0.05,corrected by Alpha Sim).FC of the inferior frontal gyrus(IF-Goperc.R)in the executive control network enhanced(P<0.05,Alpha Sim corrected),while FC of the middle oc-cipital gyrus(MOG.L)in the dorsal attention network(DAN)weakened(P<0.05,Alpha Sim corrected).There was a positive correlation between FC of MOG.L in the patient's DAN and the scores of FMA-UE(r=0.439,P=0.036),WMFT(r=0.516,P=0.012),and modified Lindmark Scales(r=0.425,P=0.043).Conclusion The resting state spontaneous activity of some brain areas of the cerebrum,cerebellum and basal ganglia is reduced in patients with hand dysfunction after ischemic stroke,and some brain network connections are abnor-mal.FC of MOG.L may serve as an objective imaging marker for evaluating hand dysfunction.

Xeroderma pigmentosum(XP)is a rare autosomal recessive disorder related to XP gene variation,often causing secondary malignant tumors.This article reports a case of a young child with secondary squamous cell carcinoma of the lower lip caused by xero-derma pigmentosum.Through a review of relevant literature,the clinical characteristics,pathogenesis,clinical pathological manifesta-tions,and prognosis of XP are discussed.

Objective To explore the characteritics of spontaneous brain functional activities for patients with hand dysfunction after ischemic stroke using resting state functional magnetic resonance imaging(rs-fMRI).Methods From August,2024 to June,2025,23 patients with hand dysfunction after ischemic stroke were as patients group,and ten age-matched healthy adults were recruited as healthy control group.The patients group was as-sessed with Fugl-Meyer Assessment-Upper Extremities(FMA-UE),Wolf Motor Function Test(WMFT)and modified Lindmark Scale.All subjects underwent rs-fMRI examinations,and the amplitude of low frequency fluctuations(ALFF),fractional amplitude of low frequency fluctuations(fALFF)and regional homogeneity(Re-Ho)were compared between two groups.The resting state functional network was constructed based on indepen-dent component analysis,and the functional connectivity(FC)were analyzed.The rs-fMRI indicators of the pa-tients and the clinical scale scores were analyzed.Results Compared with the healthy control group,the zALFF value decreased in the precentral gyrus(P<0.05,FWE corrected)in the patient group,as well as the values of zfALFF in Cerebelum-Crus2-R(P<0.05,FWE correct-ed),and ReHo values in the putamen(P<0.05,corrected by Alpha Sim).FC of the inferior frontal gyrus(IF-Goperc.R)in the executive control network enhanced(P<0.05,Alpha Sim corrected),while FC of the middle oc-cipital gyrus(MOG.L)in the dorsal attention network(DAN)weakened(P<0.05,Alpha Sim corrected).There was a positive correlation between FC of MOG.L in the patient's DAN and the scores of FMA-UE(r=0.439,P=0.036),WMFT(r=0.516,P=0.012),and modified Lindmark Scales(r=0.425,P=0.043).Conclusion The resting state spontaneous activity of some brain areas of the cerebrum,cerebellum and basal ganglia is reduced in patients with hand dysfunction after ischemic stroke,and some brain network connections are abnor-mal.FC of MOG.L may serve as an objective imaging marker for evaluating hand dysfunction.

Objective:To explore the clinical features of programmed cell death-1 (PD-1) inhibitor-associated hypophysitis and improve the understanding of the disease.Methods:For the present retrospective case series study, the clinical data of patients with PD-1 inhibitor-associated hypophysitis who were treated at the Affiliated Hospital of Hebei University and the 3rd Hospital of Hebei Medical University from January 2020 to May 2023 were collected for analysis of clinical manifestations and prognosis.Results:Fifteen cases of PD-1 inhibitor-induced hypophysitis were included, with 13 males and 2 females. The mean age of onset was (62.1±7.5) years, and the median time of onset was 6.5 (4.7, 11.6) cycles of PD-1 inhibitor. At diagnosis, 14 patients complained of gastrointestinal symptoms, and 12 patients complained of fatigue. There were 12, 1, 1, 5, and 1 cases of hyponatremia, hypokalemia, hypoglycemia, hypotension, and fever, respectively. Secondary adrenocortical insufficiency occurred in all cases. Moreover, four patients had secondary hypothyroidism, and two patients had secondary hypogonadism. Posterior pituitary hypofunction was not found. Pituitary MRI showed one case each of vacuolar sella turcica, pituitary cystic lesion, pituitary stalk slightly shifted to the left, high metabolism in the sella turcica, and pituitary abnormal signal, while no abnormalities were found in 11 cases. The follow-up time was (47.66±11.93) weeks. At the last follow-up, one patient′s serum levels of adrenocorticotropic hormone and cortisol returned to normal.Conclusions:Hypophysitis associated with PD-1 inhibitors occurs later, and gastrointestinal symptoms and fatigue are the most common clinical manifestations. PD-1 inhibitor-associated hypophysitis mainly manifests as adrenocortical hypofunction, and some cases manifest as hypothyroidism and hypogonadism. In addition, patients with PD-1 inhibitor-associated hypophysitis show no obvious imaging changes in the pituitary gland.

The accurate deduction of postmortem interval is a difficult and extremely critical task in the field of forensic pathology and criminal investigation.In recent years,physics,chemistry,microbiology,immunology,entomology,molecular biology and other disciplines have been on the rise,and special staining,spectroscopy,mass spectrometry,chromatography,radiographic and other techniques have been developed,and methods for postmortem interval inference are increasing,among which immunolabelling techniques have played an important role.In this paper,we systematically reviewed the domestic and foreign relevant studies on the postmortem interval inference using of immunolabelling techniques,including immunohistochemistry,immunoblotting,immunofluorescence,radioimmunoassay,etc.We summarized and analyzed the research progress on these techniques in postmortem interval inference,with the aim of exploring the ideas for the study of postmortem interval inference in forensic pathology,and provide reference for better applying them in forensic practice.

Necrotizing enterocolitis(NEC)is a serious gastrointestinal disease in the neonatal period and one of the main causes of death in premature infants.In recent years, with the advancement of neonatal intensive care, the survival rate of children with NEC has been improved.However, the survivors are often accompanied by poor neurological prognoses, such as periventricular leukomalacia, intraventricular hemorrhage, neurodevelopmental disorders.The pathogenesis of NEC has not been fully elucidated.This review discusses the factors that may influence NEC related brain injury, such as hypoxia and ischemia, inflammatory response, nutrition, and brain-gut axis, in order to provide an overview on the pathogenesis of NEC.

Total volatile organic compounds (TVOCs) are the main indoor pollutants. Long-term exposure to excessive TVOCs will cause acute and chronic adverse health effects. In order to understand current indoor TVOCs pollution in urban residential buildings in China, we searched related literature of indoor TVOCs in urban residential buildings published in CNKI, Wanfang, VIP, Web of Science, and PubMed from 2000 to 2021, and analyzed the pollution characteristics and main sources of indoor TVOCs in urban residential buildings in China. The results showed that the average TVOCs concentration range in urban residential buildings in China was 0.18-1.45 mg·m⁻³, which was widely distributed and exceeded the relevant national standard. The concentrations of TVOCs in bedrooms, study rooms, and kitchens were relatively high among different rooms. Indoor sources such as decoration materials and human activities after moving in were the main sources of TVOCs, and the concentration of TVOCs decreased the most in 4-6 months after the completion of decoration. However, extending the vacancy time after the completion of decoration is not the best method to effectively remove indoor TVOCs, especially for the areas where indoor air pollutants severely exceeding the national limit, it is necessary to control pollution sources to reduce indoor TVOCs concentration. For the study of indoor air TVOCs, future study directions could be the ratio of indoor and outdoor TVOCs concentration and the analysis of indoor human activities and other pollution sources.

Objective:To investigate the relationship between bone mineral density (BMD) and metabolic syndrome (MS) and its components in men.Methods:The cross-sectional study method was used. The subjects were male physical examination population who were examined in the Health Examination Center of the Affiliated Hospital of Hebei University from January 2021 to December 2021. According to the MS diagnostic criteria, they were divided into MS and non MS groups. The BMD of femur was measured by dual energy X-ray bone density analyzer. The prevalence rate and bone mineral density of osteopenia, osteoporosis, metabolic syndrome in different age groups, and the differences of metabolic indicators between MS and non MS groups and the impact of MS on BMD were analyzed. Multivariate linear stepwise regression was used to analyze the risk factors of male bone mineral density.Results:6 191 subjects were included in the study. The prevalence of osteoporosis (OP) was 9.50%(588/6 191) and the prevalence of MS was 31.64%(1959/6 191) in healthy men. The prevalence of age, body mass index (BMI), fasting plasma glucose (FPG), total glyceride (TG), total cholesterol (TC), uric acid, diastolic blood pressure, systolic blood pressure and fatty liver in MS group were higher than those in non-MS group, with statistically significant difference (all P<0.05). The high density lipoprotein cholesterol (HDL-C) in MS group was lower than that in non-MS group, with statistically significant difference (all P<0.05). There were no significant difference in the prevalence of OP and BMD between the MS group and the non-MS group (all P>0.05). There was no statistically significant difference in BMD values among different MS groups, but after adjusting BMI, when the MS group score increased from 0 to 4, the BMD value decreased gradually, and the difference was statistically significant ( P<0.05). Multiple linear regression analysis showed that BMD was positively correlated with BMI and diastolic blood pressure, but negatively correlated with age, systolic blood pressure and prevalence of fatty liver disease (all P<0.05). Conclusions:With the increase of the number of MS components, BMD in men decreased gradually. BMD in men was positively correlated with BMI and diastolic blood pressure, but negatively correlated with age, systolic blood pressure and prevalence of fatty liver disease.