Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.

  Objective  To evaluate the severity and features of pelvic coronal plane tilt in individuals with adolescent idiopathic scoliosis (AIS) who had lumbar curvature during the gait cycle.  Methods  AIS patients with lumbar curvature and patients with microcurvature (Cobb Angle less than 10 degrees) treated in Peking Union Medical College Hospital from September 2020 to February 2023 were retrospectively included. According to PUMC conservative classification system and Spinal Full-length Standing X-ray, AIS patients with lumbar curvature were enrolled. The bilateral iliac crest was used as the bony marker of the pelvic coronal surface, and the bilateral iliac crest height and its changes were measured during the standing position and walking cycle, so as to evaluate the degree of pelvic coronal tilt in AIS patients with lumbar curvature.  Results  A total of 209 AIS patients with lumbar curvature and 36 patients with microcurvature who met the inclusion and exclusion criteria were enrolled. The proportion of AIS patients with lumbar curvature who had a "congruent" relationship between the higher iliac crest and the convex side of the spine in standing position (iliac crest lower on the convex side than on the concave side) was significantly higher in AIS patients with lumbar curvature than patients with microcurvature(58.9% vs. 30.6%, P=0.002). AIS patients with lumbar curvature had statistically different bilateral iliac crest height change values throughout the gait cycle (including minimum, maximum, swing phase minimum, and swing phase maximum) (all P < 0.001), and the iliac crest height change values on the convex side were significantly higher than those on the concave side (all P < 0.05), whereas the patients with microcurvature did not have any statistically significant bilateral iliac crest height change values throughout the gait cycle (all P > 0.05).  Conclusion  The height of the iliac crest on the convex side of the lumbar spine is lower than that on the concave side in the standing position of AIS patients with lumbar curvature, and the value of the change of the iliac crest on the convex side of the pelvis is greater than that on the concave side in walking to maintain the balance of the body, which may provide a new direction for the intervention in the clinical rehabilitation treatment of AIS patients with lumbar curvature.

Objective:To investigate the current situation and influencing factors of the cognition of self-supporting for the elderly in nursing institutions, in order to provide a reference basis for constructing an intervention system of self-supporting in nursing institutions and implementing self-supporting more effectively.Methods:This study was a cross-sectional study. From April to August 2023, 320 elderly people from 7 elderly care institutions in Changsha City were selected by convenience sampling method, and the general information questionnaire, the Barthel Index Scale, the Comprehension Social Support Scale, and the self-developed questionnaire on knowledge belief, and practice of self-supporting for the elderly in nursing institutions were used to investigate. Multiple linear regression was used to analyze the factors influencing self-supporting for older people in nursing facilities.Results:Totally 315 valid questionnaires were recovered including 95 males and 220 females, with the majority aged 80-89 years, being 159. The surveyed elderly people in nursing institutions scored (91.24 ± 11.92) points on the self-supporting knowledge questionnaire, and the mean score of the entries was (3.51 ± 0.46) points. The results of multiple linear regression showed that the total scores of activity frequency, the Barthel index of activities daily living and perceived social support were the main factors affecting self-supporting ( t = 5.02, - 6.67, 9.48, all P<0.05), which could explain 51.8% of the total variation. Conclusions:The self-supporting of the elderly in nursing institutions is at a high level, and relevant personnel can better exert the subjective initiative of the elderly in nursing institutions by publicizing and educating the elderly on self-supporting, organizing regular activities and strengthening social support construction.

Objective:Exploring the effect of recovery-oriented family psychoeducation on the level of social support and family resilience of patients with schizophrenia in remission stage, providing theoretical basis for clinical application.Methods:Using a randomized controlled trial method and convenience sampling method, 60 patients with schizophrenia in remission who visited the outpatient department of the Affiliated Mental Health Center of Shanghai Jiao Tong University School of Medicine from April 2020 to March 2022 were selected as the study subjects, and they were divided into experimental group and control group, with 30 patients in each group according to their residence places. The control group was given routine health education; the experimental group was given the rehabilitation oriented family psychoeducation, before intervention and 3 months after intervention, Social Support Rating Scale and Family Resilience Questionnaire were used to evaluate the intervention effect.Results:A total of 60 patients were included in the control group, including 13 males and 17 females, aged (27.0 ± 6.5)years.In the experimental group, there were 14 males and 16 females, aged (28.0 ± 5.6) years. After intervention, the scores of subjective support, objective support and social support utilization in Social Support Rating Scale of the experimental group were 14.33 ± 3.36, 8.43 ± 1.63 and 9.60 ± 2.49, respectively, the control group were 16.20 ± 3.83, 10.00 ± 2.59 and 8.33 ± 2.28, respectively, and the differences were statistically significant ( t=2.00, 2.80, -2.05, all P<0.05). After intervention, the total score of Family Resilience Questionnaire and the scores of perseverance, support and harmony were 78.77 ± 9.61, 24.90 ± 3.07, 15.10 ± 2.23 and 23.63 ± 3.04, respectively. Higher than the control group 72.67 ± 15.96, 23.43 ± 4.92, 14.03 ± 3.60, 21.13 ± 5.13, the differences were statistically significant ( t values were -2.29--1.37, all P<0.05). Conclusions:Recovery-oriented family psychoeducation can improve the level of social support and family resilience of schizophrenia patients in remission.

Objective:To analyze and compare the practical value of serum cystatin C(Scys C)and serum creatinine(SCr)in the assessment of kidney function in older adults.Methods:A retrospective, cross-sectional study was performed in 2 450 participants who were divided into a non-elderly group(<65 years)and an elderly group(≥65 years).Glomerular filtration rate(GFR), Scys C and SCr were measured by 99mTc-DTPA clearance, particle-enhanced immunoturbidimetry and an oxidase method, respectively.The χ2 test was used to compare increases in percentage of Scys C and SCr at the same GFR level.The screening value of Scys C and SCr for GFR<60 ml·min -1·1.73m -2was evaluated by the area under curve(AUC)of the receiver operating characteristic(ROC)curve.Values of 95% reference ranges were established for Scys C and SCr at different GFR levels. Results:The proportions of the general population with increased Scys C were 82.74%(556/672)and 94.74%(90/95), respectively, for GFR levels between 30~59 ml·min -1·1.73m -2and <30 ml·min -1·1.73m -2, while only 38.24%(257/672)and 75.79%(72/95)had elevated SCr levels( χ2=278.328, 13.571, both P<0.001).For the above GFR intervals, the proportions of older adults with increased Scys C were 84.81%(240/283)and 100.00%(43/43)respectively, and the proportions for non-elderly adults with increased Scys C were 81.23%(316/389)and 90.38%(47/52)( χ2=1.463, 4.364, P=0.226, 0.037), respectively.The screening value of Scys C for GFR<60 ml·min -1·1.73m -2was slightly better than SCr in terms of sensitivity, specificity and the Youden index.However, the sensitivity and specificity of Scys C in older adults were 76.4% and 75.7%, respectively, both lower than 78.7% and 84.0% in non-older adults.The variability of Scys C increased progressively with age.The reference range for Scys C was higher in older adults than in non-older adults at the same GFR level. Conclusions:When screening for GFR<60 ml·min -1·1.73m -2, the sensitivity and specificity of Scys C are slightly better than those of SCr, but are lower in older adults than in non-older adults.Scys C levels are higher and more variable in older adults.Using Scys C to assess GFR may lead to over-diagnosis of chronic kidney disease in older adults.

Objective:To evaluate modified Lanza score (MLS) of gastric mucosa for predicting the prognosis of geriatric patients with sepsis.Methods:Data of 50 patients with sepsis, who were over 60 years old and underwent gastroscopy for suspected gastrointestinal bleeding in the Department of Geriatric Critical Care Medicine of Guangdong Provincial People's Hospital from January 2019 to April 2022, were retrospectively analyzed. Patients were divided into the death group ( n=32) and the survival group ( n=18) according to their regression within 28 days after gastroscopy. Their gastric mucosa was scored by using MLS system, and the mortality of patients with MLS≥1 was calculated, then the patients were further divided into 2 groups, MLS=0-2 ( n=23, less than 2 regions of lesions ) and MLS=3-5 ( n=27, two or more regions of lesions). The relationship between MLS and acute physiology and chronic health status evaluation (APACHE) Ⅱ score, risk factor of death and mortality in each group were compared. The correlation between MLS and mortality was analyzed. The influence of geriatric sepsis risk factors affecting the prognosis of patients within 28 days were analyzed by using logistic regression. Results:Among the 50 geriatric patients with sepsis, those with gastric mucosal lesions, i.e., MLS ≥1, accounted for 68.00% (34/50), including 84.38% (27/32) patients with MLS≥1 in the death group, which was significantly higher than the 38.89% (7/18) patients with MLS≥1 in the survival group ( χ 2=10.593, P<0.001). Patients with MLS=3-5 had significantly higher APACHE Ⅱ scores (26.09±6.47 VS 18.57±7.66, t=3.527, P=0.001) and higher mortality [85.19% (23/27) VS 39.13% (9/23), χ 2=11.434, P=0.001] compared with MLS=0-2. Correlation analysis showed a significant correlation between MLS and mortality ( r=0.886, P=0.019). Multivariate logistic regression analysis showed that MLS=4-5 was an independent risk factor for death in geriatric patients with sepsis ( OR=17.055, 95% CI: 1.387-209.744, P=0.027). Conclusion:MLS presents high sensitivity in predicting 28-day outcomes for geriatric patients with sepsis. Two or more than 2 regions of gastric mucosal lesions can significantly increase the risk of death in geriatric patients with sepsis.

Immunotherapy combined with effective therapeutics such as chemotherapy and photodynamic therapy have been shown to be a successful strategy to activate anti-tumor immune responses for improved anticancer treatment. However, developing multifunctional biodegradable, biocompatible, low-toxic but highly efficient, and clinically available transformed nano-immunostimulants remains a challenge and is in great demand. Herein, we report and design of a novel carrier-free photo-chemotherapeutic nano-prodrug COS-BA/Ce6 NPs by combining three multifunctional components-a self-assembled natural small molecule betulinic acid (BA), a water-soluble chitosan oligosaccharide (COS), and a low toxic photosensitizer chlorin e6 (Ce6)-to augment the antitumor efficacy of the immune adjuvant anti-PD-L1-mediated cancer immunotherapy. We show that the designed nanodrugs harbored a smart and distinctive "dormancy" characteristic in chemotherapeutic effect with desired lower cytotoxicity, and multiple favorable therapeutic features including improved ¹O₂ generation induced by the reduced energy gap of Ce6, pH-responsiveness, good biodegradability, and biocompatibility, ensuring a highly efficient, synergistic photochemotherapy. Moreover, when combined with anti-PD-L1 therapy, both nano-coassembly based chemotherapy and chemotherapy/photodynamic therapy (PDT) could effectively activate antitumor immunity when treating primary or distant tumors, opening up potentially attractive possibilities for clinical immunotherapy.

Objective:To study the clinical characteristics and management strategies of late bleeding after laparoscopic pancreaticoduodenectomy (LPD).Methods:The clinical data of 58 patients with post-pancreaticoduodenectomy hemorrhage (PPH) admitted to the Department of Hepatobiliary Surgery of the Second Hospital of Hebei Medical University from March 2018 to March 2022 were retrospectively analyzed, including 42 males and 16 females, aged (61.88±11.02) years old. According to the occurrence of intra-abdominal erosion factors (e.g., pancreatic fistula, biliary fistula, gastrointestinal anastomotic fistula, intra-abdominal abscess), patients were divided into the erosion group ( n=42) and non-erosion group ( n=16). All patients underwent standard lymphadenectomy. Clinical data including the PPH time-point, occurrence of rebleeding, and treatment outcomes were accessed. The management strategies of PPH in the two groups of patients were analyzed. Results:The PPH time-point in the erosion group and non-erosion patients was 8.00 (5.00, 19.25) d and 21.50 (12.75, 26.75) d, respectively ( P=0.001). PPH can occurred within one month after surgery in both erosion and non-erosion groups. In the erosion group, 31 cases (73.81%, 31/42) were treated by re-operation, two (4.76%, 2/42) by interventional radiology and nine (21.43%, 9/42) with conservative protocol, respectively. In the non-erosion group, five cases (31.25%, 5/16) were treated by re-operation, seven (43.75%, 7/16) by interventional radiology and four (25.00%, 4/16) with conservative protocol, respectively. The incidence of re-bleeding is higher in the erosion group [47.6% (20/42) vs 12.5% (2/16), P<0.05]. Clinical manifestations, sites and severity of bleeding, and treatment outcomes were also different in the erosion and non-erosion groups (all P<0.05). Conclusions:The occurrence of intra-abdominal erosion factors can affect the clinical characteristics and treatment strategy of late bleeding after laparoscopic pancreaticoduodenectomy. Surgery remains the treatment of choice for post-pancreaticoduodenectomy hemorrhage either as an urgent or last resort.

Objective:To explore the effect of comorbidities on the risk of chronic kidney disease with aging.Methods:A total of 103 682 subjects were recruited at the health management center of the First Affiliated Hospital of Nanjing Medical University from January 2018 to January 2020 for a retrospective cross-sectional study.Participants were divided into four groups according to age 18-44(55 055 case), 45-59(31 023 case), 60-74(12 793 case), ≥75(4 811 case)age group.We calculated and compared the prevalences of estimated glomerular filtration rate(eGFR)<60 ml/min/1.73m 2, which was used as a parameter for kidney dysfunction, indifferent age groups and participants with different comorbidities.The association between comorbidities and the prevalence of kidney dysfunction was analyzed by Logistic regression. Results:Levels of blood pressure, body mass index, serum creatinine, eGFR and blood lipids varied with aging, as did the prevalences of hypertension, diabetes, obesity and comorbidities.In all participants and groups based on the types of chronic diseases, the prevalence of kidney dysfunction increased with aging, substantially so in ≥75 age group.After adjustment for age and sex, there was a marked increase in the risk of kidney dysfunction in 45-59 age group adults with hypertension or diabetes( OR=9.163, 95% CI: 3.264-25.727; OR=4.640, 95% CI: 1.028-20.936), and the risk of renal dysfunction increased in people with hypertension+ diabetes or with the coexistence of three diseases, compared with people with a single disease( OR=16.441, 95% CI: 5.325-50.783; OR=15.985, 95% CI: 4.237-60.312); In the 60-74 age group, hypertension alone also significantly increased the risk of renal dysfunction( OR=3.950, 95% CI: 1.911-8.165). With comorbidities, the most significant influence was the coexistence of three diseases( OR=6.245, 95% CI: 2.521-15.468), followed by hypertension+ obesity( OR=5.640, 95% CI: 2.550-12.476)and hypertension+ diabetes( OR=4.330, 95% CI: 1.990-9.421); In ≥75 age group, chronic diseases and comorbidities were associated with a high risk of renal dysfunction, with obesity alone and hypertension obesity posing the highest risk( OR=6.746, 95% CI: 2.193-20.757; OR=6.570, 95% CI: 3.178-13.582), followed by the coexistence of three diseases( OR=4.749, 95% CI: 2.110-10.687). Conclusions:The prevalence of hypertension, diabetes, and obesity varies with aging.The effect of chronic diseases on the risk of chronic kidney disease with reduced renal function in the elderly population is different from that in the non-elderly population.

Objective:To observe the variation rend of serum creatinine(SCr)and estimated glomerular filtration rate(eGFR)with aging, and to explore the warning range of SCr for screening low eGFR of the elderly.Methods:A retrospective cross-sectional study was performed in 10 3513 participants, including 14 221 in 18-29 years old, 29 763 in 30-39 years old, 23 151 in 40-49 years old, 18 838 in 50-59 years old, 10 019 in 60-69 years old, 5 009 in 70-79 years old, 2 512 aged ≥80 years old.We adopted CKD-EPI formula based on Chronic Kidney Disease Epidemiology Collaboration(CKD-EPISCR)formula recommended by the Global Organization for Improving Outcomes in Kidney Disease 2012 to calculate eGFR.The SCr and eGFR levels were calculated according to age to show the trend of these with aging.Further, we used percentage to investigate the distribution of eGFR in population with normal SCr, and the percentile method to establish the warning range of SCr in the older adults.Results:With aging, SCr level showed an elevating trend in women( F=340.80, P<0.001), and gradually increased in men older than 50 years( F=111.05, P<0.001)and eGFR declined with increasing age( F=8 301.60, 9 114.53, P<0.001). In population with normal SCr, the proportions of eGFR<60 and 60-75 ml·min -1·1.73m -2 were 3.83%(661/17 280)and 12.50%(2 160/17 280)in older adults aged 60-74 years respectively, but were 10.39%(488/4 699)and 22.75%(1 069/4 699)in old-older adults aged 75 years and over respectively, and were 0.07%(56/84 145)and 0.91%(762/84 145)in younger adults below 60 years old respectively.In the same distribution of eGFR, SCr levels of older adults aged 60-74 years and old-older adults aged 75 years and over were lower than that of younger population( P<0.001). In old-older adults aged 75 years and over, the warning range of SCr was 98.76-126.03 μmol/L for men and 95.12-130.00 μmol/L for women in individuals with 45 ≤ eGFR<60 ml·min -1·1.73m -2. Conclusions:The SCr level of older adults is lower than younger individuals in same distribution of eGFR.SCr level is still in the reference range in some of older individuals with eGFR-confirmed renal function impairment.The warning range of SCr is needed, especially in older adults, so as to provide reference for the rational use of medicine and disease management.