Objective:To investigate the clinical features and therapeutic efficacy of patients with hereditary leiomyomatosis and renal cell carcinoma(RCC) syndrome-associated RCC (HLRCC-RCC) in China.Methods:The clinical data of 119 HLRCC-RCC patients with fumarate hydratase (FH) germline mutation confirmed by genetic diagnosis from 15 medical centers nationwide from January 2008 to December 2021 were retrospectively analyzed. Among them, 73 were male and 46 were female. The median age was 38(13, 74) years. The median tumor diameter was 6.5 (1.0, 20.5) cm. There were 38 cases (31.9%) in stage Ⅰ-Ⅱand 81 cases (68.1%) in stage Ⅲ-Ⅳ. In this group, only 11 of 119 HLRCC-RCC patients presented with skin smooth muscle tumors, and 44 of 46 female HLRCC-RCC patients had a history of uterine fibroids. The pathological characteristics, treatment methods, prognosis and survival of the patients were summarized.Results:A total of 86 patients underwent surgical treatment, including 70 cases of radical nephrectomy, 5 cases of partial nephrectomy, and 11 cases of reductive nephrectomy. The other 33 patients with newly diagnosed metastasis underwent renal puncture biopsy. The results of genetic testing showed that 94 patients had FH gene point mutation, 18 had FH gene insertion/deletion mutation, 4 had FH gene splicing mutation, 2 had FH gene large fragment deletion and 1 had FH gene copy number mutation. Immunohistochemical staining showed strong 2-succinocysteine (2-SC) positive and FH negative in 113 patients. A total of 102 patients received systematic treatment, including 44 newly diagnosed patients with metastasis and 58 patients with postoperative metastasis. Among them, 33 patients were treated with tyrosine kinase inhibitor (TKI) combined with immune checkpoint inhibitor (ICI), 8 patients were treated with bevacizumab combined with erlotinib, and 61 patients were treated with TKI monotherapy. Survival analysis showed that the median progression-free survival (PFS) of TKI combined with ICI was 18 (5, 38) months, and the median overall survival (OS) was not reached. The median PFS and OS were 12 (5, 14) months and 30 (10, 32) months in the bevacizumab combined with erlotinib treatment group, respectively. The median PFS and OS were 10 (3, 64) months and 44 (10, 74) months in the TKI monotherapy group, respectively. PFS ( P=0.009) and OS ( P=0.006) in TKI combined with ICI group were better than those in bevacizumab combined with erlotinib group. The median PFS ( P=0.003) and median OS ( P=0.028) in TKI combined with ICI group were better than those in TKI monotherapy group. Conclusions:HLRCC-RCC is rare but has a high degree of malignancy, poor prognosis and familial genetic characteristics. Immunohistochemical staining with strong positive 2-SC and negative FH can provide an important basis for clinical diagnosis. Genetic detection of FH gene germ line mutation can confirm the diagnosis. The preliminary study results confirmed that TKI combined with ICI had a good clinical effect, but it needs to be confirmed by the results of a large sample multi-center randomized controlled clinical study.

In March 2024, organized by Chinese Society of Parenteral and Enteral Nutrition (CSPEN), experts in the fields of general surgery, critical care medicine, gastroenterology, geriatrics, clinical nutrition, clinical pharmacy and evidence-based medicine developed the guidelines for the clinical application of micronutrients in Chinese adult patients (2024 edition) on the basis of latest clinical evidence. The guidelines is of great value in guiding the clinical application of micronutrients. Here, we intended to interpret the guidelines, summarize the differences between domestic and foreign guidelines, and thus enhance our understanding.

Objective:To evaluate the efficacy of first-line tyrosine kinase inhibitors (TKI) plus immune checkpoint inhibitors (ICI) in metastatic fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC).Methods:The data of 87 metastatic FH-deficient RCC patients from West China Hospital ( n=44), Renji Hospital ( n=27) and Sun Yat-sen University Cancer Center (n=16) from Mar 2019 to Aug 2022 were retrospectively analyzed. The median age was 37(30, 47) years, the male to female ratio was 1.9∶1. The median size of tumor was 7.5(5.0, 10.0) cm. Sixty-one patients (70.1%) had germline FH mutations, and 26 patients (29.9%) had somatic FH mutations. Forty-nine patients (56.3%) metastasis disease at initial diagnosis, and 38 patients (43.7%) had metachronous metastasis. The most common site of metastasis was lymph node (41/87, 47.1%), followed by bone (33/87, 37.9%), liver (22/87, 25.3%), and lung (14/87, 16.1%). Fifteen patients (17.2%) had weak expression of FH protein and 59 patients (67.8%) had positive PD-L1 expression. The most common treatments were sintilimab plus axitinib (52/87, 59.8%), followed by pembrolizumab plus cabozantinib (7/87, 8.0%), tirelizumab plus axitinib (6/87, 6.9%), pembrolizumab plus axitinib (5/87, 5.7%), and toripalimab plus axitinib (4/87, 4.6%). Thirteen patients (13/87, 14.9%) received other ICI plus TKI combination treatments. Statistical analysis was conducted using R 4.2.3 software. Kaplan Meier survival curve was used to evaluate survival data, and log-rank test was used to compare differences between treatment groups. Results:The overall objective response rate (ORR) and disease control rate (DCR) of first-line TKI + ICI were 39.1% and 89.7%, respectively. The median progression-free survival (PFS) and overall survival (OS) were 16.5 months and 71.0 months, respectively. For first-line sintilimab plus axitinib, the ORR and DCR were 44.2% and 92.3%, respectively. The median PFS was 17.3 months and the median OS was not reached for this combination treatment. The efficacy of first-line tirelizumab plus axitinib was inferior to other treatment strategies (median PFS: 4.0 vs. 16.6 months, P<0.001; median OS: 22.0 vs. 71.0 months, P=0.043). Subgroup analyses further showed that the efficacy of ICI+ TKI combination therapy was consistent in patients with different clinicopathologic and genomic features. However, patients with liver metastasis had shorter OS than those without liver metastasis (median OS: 26.3 vs. 71.0 months, P=0.021). Conclusion:First-line TKI + ICI is effective for metastatic FH-deficient RCC and can significantly prolong the survival of the patients.

Most hospitalized patients infected with coronavirus disease 2019 (COVID-19) are in severe or critical condition, and malnutrition is a key factor contributing to adverse outcomes. The basic principles of medical nutrition therapy have been determined in the recently released tenth edition of the National Diagnosis and Treatment Protocol. The principles have promoted nutritional risk assessment, emphasized the preferred method of enteral nutrition, and recommended the daily intake of calories at 25 to 30 kcal/kg and protein at > 1.2g/kg. Parenteral nutrition should be also added when necessary. Based on the above principles, Beijing Hospital has refined the medical nutrition therapy measures to facilitate the implementation in clinical practice, in order to improve healthcare quality and decrease the mortality in COVID-19 patients.

Objective:To study the association of frailty status with nutritional risk and the effect on clinical outcomes among elderly surgical inpatients.Methods:Elderly inpatients from the surgery department of Beijing Hospital were enrolled from January to June 2021. Frail scale and nutritional risk screening 2002 (NRS 2002) were used for frailty evaluation and nutrition risk screening. The influence of frailty and associated nutrition risk in elderly surgical inpatients was analyzed.Results:487 elderly surgical patients were included, of whom 131 cases were in the non-frailty group, 279 cases were in the pre-frailty group and 77 cases were in the frailty group, according to the Frail scale score. 146 cases were at nutritional risk, of whom 8 (6.1% of 131) were in the non-frailty group, 87 (31.2% of 279) in the pre-frailty group and 51 (66.2% of 77) were in the frailty group. According to univariate/multivariate logistic regression analysis of frailty in elderly surgical patients, a higher NRS 2002 score, older age, and the presence of multiple concurrent diseases (≥ 5) were significantly associated with frailty ( P < 0.001). The Frail scale score was positively correlated with NRS 2002 score ( r = 0.448, P < 0.01). Multiple comparisons showed that frailty had statistically significant effects on hospital stay and medical costs in elderly surgical patients ( P < 0.05). Conclusions:The prevalence of frailty is higher in elderly surgical patients, and the prevalence of nutritional risk increases with the progression of frailty. Frailty can lead to prolonged hospital stays and increased hospital costs in elderly surgical patients.

Objective:To investigate the prevalence of malnutrition in elderly patients with neurological diseases and the of nutrition, and to explore their association with clinical outcomes.Methods:A retrospective study was conducted to analyze 566 elderly patients with neurological diseases in the database of the "National Multicenter Survey on the Dynamic Changes of Nutritional Status of Hospitalized Patients" by using the Global leadership Initiative on Malnutrition(GLIM)criteria and subjective global assessment(SGA). The two diagnostic tools for malnutrition were compared to explore the correlation between malnutrition and clinical outcomes.Results:Based on the GLIM criteria, 83 cases were diagnosed with malnutrition and the incidence of malnutrition was 14.66%(83/566), with 14.72%(48/326)in men and 14.58%(35/240)in women.Patients with moderate malnutrition accounted for 8.30%(47/566)and patients with severe malnutrition accounted for 6.36%(36/566). According to the SGA, the incidence of moderate malnutrition(SGA Grade B)was 15.55%(88/566), the incidence of severe malnutrition(SGA Grade C)was 1.94%(11/566), and all cases of malnutrition(SGA Grade B+ C)accounted for 17.49% of the participants(99/566). The total length of hospital stay was(15.46±6.49)days in the malnutrition group and(13.55±5.09)days in the non-malnutrition group, with a statistical difference between the two groups( t=-3.02, P<0.01). The body weight of the malnutrition group was significantly lower than non-malnutrition group[(52.0±8.5)kg vs.(65.2±9.6)kg, t=12.92, P<0.01]. There were also statistically significant differences in BMI(19.1±2.7 kg/m 2vs.23.9±2.6 kg/m 2, t=15.48, P<0.01), upper arm circumference[(22.3±2.5)cm vs.(28.3±3.9)cm, t=7.01, P<0.01], and lower leg circumference[(28.9±3.4)cm vs.(32.5±3.3)cm, t=6.81, P<0.01]between the two groups.Laboratory tests showed that there were significant differences in lymphocytes[(5.0±8.5)×10 9/L vs.(9.4±11.8)×10 9/L, t=3.61, P<0.01]and albumin[(38.5±4.4)g/L vs.(40.7±5.1)g/L, t=3.18, P<0.01]between the malnutrition group and the non-malnutrition group.The correlation between GLIM and SGA was good, and the consistency was reasonable(AUC=0.711). Conclusions:The incidence of malnutrition in elderly patients with neurological diseases is relatively high; The GLIM criteria are suitable for the diagnosis of malnutrition in elderly patients with neurological diseases, and the diagnostic results have a good correlation with those of SGA.Malnutrition is associated with anthropometric measurements, laboratory indicators, and clinical outcomes.

Objective:To study the correlation between nutritional status and length of hospital stay in patients with digestive disorders.Methods:The data were collected from the database of a multi-center investigation on the dynamic changes of nutritional status of hospitalized patients in China, a project led by the Geriatric Nutrition Support Group, Society of Parenteral and Enteral Nutrition, Chinese Medical Association. The enrolled patients were screened for malnutrition and possible sarcopenia using Global Leadership Initiative on Malnutrition criteria, and the dynamic changes of serum biochemical indexes during hospital stay and the effects of malnutrition and possible sarcopenia on the length and cost of hospital stay were analyzed.Results:A total of 1 180 patients were enrolled, with an average age of (56.3±16.1) years, the average height of (164.65±8.29) cm, and the average weight of (62.12±12.12) kg. There were significant differences in body weight, body mass index, calf circumference, lymphocyte count, triglyceride, hemoglobin, albumin and total protein between at discharge and at admission ( P<0.001). There might be a correlation between post-admission malnutrition and sarcopenia. There was neither significant difference in the proportion of patients with malnutrition at admission among different age groups ( P=0.438), nor in that at discharge among different age groups ( P=0.439). The proportion of patients with malnutrition showed no significant difference between subgroups with patients<65 years old and ≥ 65 years old, at admission and discharge ( P>0.05). However, comparison of the proportion of patients with sarcopenia between subgroups with patients<65 years old and ≥65 years old displayed significant differences at admission and discharge ( P<0.001), but not the comparison of the proportion of patients with possible sarcopenia ( P>0.05). The length of hospital stay in patients with malnutrition was significantly longer than that in patients without malnutrition [(13.22±6.24) days vs. (12.08±5.25) days, P<0.001]. The length of hospital stay of patients with and without sarcopenia was also significantly different [(12.87±5.93) days vs. (12.02±5.22) days, P<0.001). Patients with concurrent malnutrition and sarcopenia had longer hospital stay [(14.57±7.15) days vs. (12.07±5.22) days, P<0.001], and higher medical cost [(2.78±2.19) ten thousand Chinese Yuan vs. (2.24±2.33) ten thousand Chinese Yuan, P<0.05)] compared with those without concurrent malnutrition and sarcopenia. Conclusions:A large proportion of patients with digestive disorders were diagnosed with malnutrition and/or possible sarcopenia during hospitalization. There is possible correlation between malnutrition and possible sarcopenia, and both can lead to a longer hospital stay and higher medical cost.

Micronutrients (MNs), including vitamins and trace elements, play an indispensable role in human metabolism, immune function and other aspects. Due to the chronic microinflammation and long-term chemoradiotherapy, patients with malignant tumors often suffer from malnutrition, resulting in different degrees of MNs deficiency. In severe cases, MNs deficiency is closely related to the adverse clinical outcomes. Therefore, reasonable MNs supplementation is of great significance in improving the prognosis and quality of life of patients with tumors. Recently, multiple guidelines have made recommendations on the application of MN supplementation in various clinical settings, providing evidence for the standardized MN supplementation in patients with malignant tumors.

Objective:To investigate the prevalence of malnutrition in hospitalized patients with lung cancer in China using the global leadership initiative on malnutrition (GLIM) criteria.Methods:A retrospective analysis was performed with 623 lung cancer patients selected from the national multi-center prospective investigation database in 2014. The diagnosis of malnutrition was according to GLIM criteria with muscle mass reduction not included. The prevalence of malnutrition in patients with lung cancer was investigated and the correlation between malnutrition diagnosis per GLIM criteria and anthropometry as well as laboratory indexes was explored.Results:Among the 623 lung cancer patients, 33.5% (209) were at nutritional risk as per Nutritional Risk Screening 2002, 17.8% (111) were malnourished as per GLIM criteria. There were significant differences in age, body weight, body mass index, arm circumference, leg circumference, grip strength, hemoglobin, albumin, total protein and prealbumin between malnutrition and non-malnutrition groups as diagnosed with GLIM criteria ( P<0.05). Conclusions:The nutritional risk and malnutrition prevalence in lung cancer patients are relatively high. The nutritional risk screening of lung cancer patients should be standardized and the GLIM criteria can be used for the diagnosis of malnutrition.

Objective:To summarize the surgical treatment experience of extended thymectomy for myasthenia gravis(MG), and to explore the surgical treatment of MG.Methods:Retrospectively analyzed the clinical data of 527 MG patients undergoing extended thymectomy in our hospital from June 1996 to October 2017, including 242 males and 285 females, aged 5 to 77 years, with a mean age of(52.6±13.7) years old. The course of illness was 12 days to 18 years. There were 22 cases of hyperthyroidism, 7 cases of pure red blood cell aplastic anemia, 1 case each of hypothyroidism, irritable bowel disease, rheumatoid arthritis, ankylosing spondylitis and thrombocytopenia syndrome. There were 272 cases of MG in Osserman Ⅰ, 72 cases inⅡa, 78 cases inⅡb, 81 cases in Ⅲ, and 24 cases in Ⅳ, respectively. The muscle fatigue test and neostigmine test of all patients were positive, and the diagnosis was confirmed by chest CT examination. Meanwhile, summarize the perioperative data and postoperative follow-up.Results:3 cases died during the postoperative period, all of which were thymoma with MG, including 2 cases of Osserman Ⅲ MG and 1 case of Ⅳ MG; 15 cases of postoperative myasthenia crisis, including 2 OssermanⅡb cases, 11 Osserman Ⅲ cases and 2 Osserman Ⅳ cases, also including 7 cases of tracheotomy; 70 cases of plasma exchange, of whom 2 cases of hypotonic syndrome and 2 cases of lower extremity venous thrombosis. The postoperative pathological types were followed by thymic hyperplasia 293 cases(55.60%), thymoma 207 cases(39.28%), thymic cyst 24 cases(4.55%) and thymic atrophy 3 cases(0.57%) in descending order. 378 cases were followed up with an average follow-up of(85.9±58.5)months; MG with complete remission, partial remission, no change and deterioration accounted for 135(35.71%), 192(50.79%), 41(10.85%) and 10(2.65%)cases, respectively. Complete remission rate ranked as Osserman typeⅠ>Ⅱa>Ⅳ>Ⅱb>Ⅲ, the deterioration rate from high to low was Osserman type Ⅲ>Ⅳ>Ⅰ. 18 OssermanⅠcases showed no effects, whose preoperative course of disease> 5-10 years; 1 OssermanⅠcase was worsening who initially was diagnosed with ophthalmic MG and underwent video-assisted thoracoscopic thymectomy. The left thymus lobe was left unresected and developed 6 years later. Another operation was performed to remove the left lobe thymus, confirming the compensatory hypertrophy of the left lobe thymus. Among the worsening patients, 6 died, all of them were thymoma with MG, including 5 cases of type Ⅲ and one case of type Ⅳ. The cause of death was omyasthenia crisis(3 cases), sudden respiratory arrest after 3 months of rapid stopping of brompistigmine(2 cases)and cholinergic crisis(1 case).Conclusion:Standardized extended thymectomy is an effective method for the treatment of MG. Earlier surgery for ocular muscle type MG can effectively reduce the risk of generalization. MG with Osserman Ⅱb or higher is prone to myasthenia crisis. Comprehensive treatments should be taken to reduce MG-related risks. Myasthenia crisis can occur repeatedly in severe patients in the long term after surgery, requiring regular medication and comprehensive MG treatments.