Objective: To explore the clinical characteristics and diagnosis and treatment plans of neurofibromatosis type 1 (NF1), and to provide references for clinical diagnosis and treatment. Methods : The clinical manifestations and treatment of an 8-year-old female patient with NF1 was reported. A literature review was conducted to summarize the clinical characteristics and therapeutic strategies of NF1. Multiple NF1s occurred on the right cheek, orbit, and eyelid, and recurred after surgical resection. The tumor caused ptosis, incomplete closure, and vision loss in the upper eyelid of the right eye. After a multidisciplinary assessment determined that radical resection was not feasible, selumetinib sulfate targeted therapy was adopted (25 mg, Po, bid), 28 days constitute one treatment course, and 14 courses have been completed, combined with symptomatic ocular treatments, such as Befusu. Result: The follow-up showed that the tumor volume did not continue to increase (stable disease), the uncorrected vision of the right eye improved (0.05 vs 0.1), and no drug-related adverse reactions occurred during the treatment period. The literature review summarizes the diverse clinical manifestations of NF1, with café-au-lait macules, multiple neurofibromas, and Lisch nodules being hallmark features. Currently, surgical intervention remains the most commonly employed and primary therapeutic approach for NF1; however, for patients who do not meet the criteria for surgery, alternative treatment strategies should be considered. MEK inhibitors, such as selumetinib, demonstrate significant efficacy in inhibiting the growth of NF1-associated plexiform neurofibromas, with tumor volume reductions of at least 20% observed in 70% of pediatric patients in the SPRINT clinical trial. Furthermore, these inhibitors exhibit favorable long-term safety profiles. Conclusion Café-au-lait macules, multiple neurofibromas, and Lisch nodules are hallmark features of NF1. Selumetinib is safe and effective for NF1 in the head and neck of children, and it is the preferred treatment option for patients who are not suitable for surgery. Long-term follow-up monitoring of tumor changes and drug safety is required.

Objective:To investigate the distribution law of TCM syndrome types and the differences in influencing factors among different syndrome types in unstable angina pectoris (UA), so as to provide an objective basis for TCM syndrome differentiation and treatment of UA.Methods:A retrospective study chose 1 684 inpatients in the Department of Cardiovascular Medicine of the First Affiliated Hospital of Henan University of Chinese Medicine from August 2015 to April 2019. Epidata 3.0 software was used to input general information of patients [gender, age, length of hospital stay, BMI, systolic blood pressure (SBP), diastolic blood pressure (DBP)], laboratory indicators[TC, TG, HDL-C, LDL-C, fibrinogen (FIB), thrombin time (TT), D-dimer (D-dimer), HbA1c], TCM syndrome types (qi and yin deficiency syndrome, phlegm turbidity and obstruction syndrome, qi deficiency and blood stasis syndrome, heart blood stasis syndrome, qi stagnation and blood stasis syndrome, heart and kidney yang deficiency syndrome) and other information. A database was established to analyze the distribution of TCM syndrome types and the relationship with the general information of patients, blood lipids, blood sugar and coagulation indexes. Logistic regression was used to analyze the influencing factors of different syndrome types.Results:The distribution of syndrome types in 1 684 UA patients was as follows: qi and yin deficiency syndrome (44.7%), phlegm turbidity and obstruction syndrome (35.3%), qi deficiency and blood stasis syndrome (7.4%), heart blood stasis syndrome (5.3%), qi stagnation and blood stasis syndrome (4.6%), heart and kidney yang deficiency syndrome (2.6%); more men than women ( P<0.05); there were significant differences in the distribution of gender, age, BMI, TC, and HDL-C among the 6 syndrome types ( P<0.05); the age of patients with phlegm turbidity and obstruction syndrome was younger than that of qi and yin deficiency syndrome and heart blood stasis syndrome ( P<0.05); the age of patients with qi stagnation and blood stasis syndrome was younger than that of qi and yin deficiency syndrome, heart blood stasis syndrome, and heart kidney yang deficiency syndrome ( P<0.05); BMI of patients with phlegm turbidity and obstruction syndrome was higher than that of qi and yin deficiency syndrome and qi stagnation and blood stasis syndrome ( P<0.05); the level of TC in patients with phlegm turbidity and obstruction syndrome was lower than that of qi and yin deficiency syndrome and qi deficiency and blood stasis syndrome ( P<0.05); the level of HDL in patients with qi and yin deficiency syndrome was lower than that in qi deficiency and blood stasis syndrome and qi stagnation and blood stasis syndrome. Binary Logistic regression analysis found that TC [ OR(95% CI)=0.761(0.592, 0.978)] and HDL-C [ OR(95% CI)=2.131(1.145, 3.966)] were independent influencing factors for predicting qi deficiency and blood stasis syndrome ( P<0.05); age[ OR(95% CI)=1.017 (1.008, 1.026)], length of hospital stay [ OR(95% CI)=1.019 (1.001, 1.038)], DBP [ OR(95% CI)=0.984(0.975, 0.993)] and HDL-C [ OR(95% CI)=0.984(0.975, 0.993)] were independent influencing factors for predicting qi and yin deficiency syndrome ( P<0.05); age [ OR(95% CI)=0.965 (0.946, 0.985)], and HDL-C [ OR(95% CI)=2.329(1.206, 4.500)] were independent influencing factors for predicting qi stagnation and blood stasis syndrome ( P<0.05); age [ OR(95% CI)=0.982 (0.973, 0.991)], length of hospital stay [ OR(95% CI)= 0.978 (0.958, 0.997)], BMI [ OR(95% CI)=1.048 (1.015, 1.082)], DBP [ OR(95% CI)=1.014 (1.004, 1.024)] and HDL-C [ OR(95% CI)=0.505 (0.351, 0.726)] were independent influencing factors for predicting phlegm turbidity and obstruction syndrome ( P<0.05); age [ OR(95% CI)=1.031(1.003, 1.060)] and DBP [ OR(95% CI)=1.028(1.001, 1.056)] were independent influencing factors for predicting heart kidney yang deficiency syndrome ( P<0.05). Conclusion:The distribution of TCM syndrome types in UA shows a certain regularity, among which qi and yin deficiency syndrome and phlegm turbidity and obstruction syndrome are more common. Gender, age, BMI, TC, HDL-C are different among TCM syndrome types, which can provide some reference for UA TCM syndrome differentiation and treatment.

The spectrum of biopsy-confirmed kidney disease varies with regions and periods. We describe the distribution of pathological types and epidemiological characteristics of kidney diseases in Northwest China due to regional differences in geographical environment, social economy, and dietary habits. Methods: Kidney biopsy cases from 2005 to 2020 in Xijing Hospital were retrospectively analyzed. Pathological characteristics of patients in different periods were analyzed using the t test or chi-square test. Joinpoint regression was used to analyze trends in pathological types and disease spectrum. Results: A total of 10,528 eligible patients were included. Primary glomerular disease (PGD) accounted for the majority of the cases and exhibited an obvious downward trend, whereas secondary glomerular disease (SGD) showed an obvious upward trend. Among PGD, immunoglobulin A nephropathy (IgAN) remained the most common pathological type, and the detection rate of membranous nephropathy (MN) was significantly increased. Among SGD, Henoch-Schönlein purpura nephritis (HSPN) was the most common pathological type and may present a significant characteristic of Northwest China. Diabetic nephropathy (DN) exhibited the most obvious upward trend in the whole process, whereas the fastest growth since 2012 was in hypertensive nephropathy. Conclusion: The proportion of SGD increased whereas PGD declined. IgAN remained the most common PGD, and HSPN was the most common SGD. MN and DN showed the most obvious upward trend among PGD and SGD, respectively. Changes in the spectrum of kidney disease, especially the constituent ratio of SGD, pose a great challenge to public health.

Objective:To explore whether the electroacupunture stimulation (ES) at acupoint Zusanli (ST36) can inhibit the bone loss caused by Staphylococcus aureus (SA) infection and its mechanism in a model of SA osteomyelitis.Methods:Twelve male C57 BL/6 mice aged 10 to 12 weeks were randomly divided into 2 even groups ( n=6) for SA infection + ES or SA infection only. After ES at ST36 was conducted for 4 weeks in the model of SA osteomyelitis, samples were harvested from the femora and tibiae. Micro-CT reconstruction was performed to detect trabecular bone volume fraction (BV/TV), trabecular number (Tb.N), trabecular thickness (Tb.Th), trabecular separation (Tb.Sp), connectivity density (Conn.Dn) to analyze changes in bone mass. Leptin receptor (LEPR) staining was performed to detect osteoblasts. Tartrate resistant acid phosphatase (TRAP) staining was used to detect the changes in osteoclasts. The changes in plasma inflammatory factors were detected by enzyme-linked immunosorbent assay (ELISA). Results:Micro-CT results showed that the BV/TV, Tb.N, Tb.Th, and Conn.Dn in the cancellous bone in the target areas in the SA + ES group were all higher than those in the SA group, LEPR immunofluorescence results indicated that the number of osteogenic precursor cells in the ES group was larger than that in the SA group, and serum ELISA indicated a decrease in inflammatory factors in the blood in the SA+ES group compared with the SA group. There were significant differences in the comparisons above ( P<0.05). There was no significant difference in the number of osteoclasts on the surface of trabecular bone between the 2 groups in TRAP staining. Conclusion:ES may slow down infectious bone destruction by inhibiting the inflammatory response induced by SA infection and by inducing aggregation and differentiation of mesenchymal stem cells into trabecular bone.

Objective: To investigate the effects of insulin-like growth factor 1 receptor (IGF-1R) inhibitor on tubulopathy in diabetic kidney disease (DKD) mice. Methods: C57BL/6J male mice were randomly divided into normal control group (n=10) and DKD model group (n=30), by giving a single intraperitoneal injection of STZ 150 mg/kg to establish a DKD model. After established successfully, the mice in DKD model group were randomly divided into DKD group (n=10), benazepril group (n=10) and IGF-1R inhibitor group (n=10). IGF-1R inhibitor group was given intraperitoneal injection of IGF-1R inhibitor (30 mg·kg^-1·d^-1) and benazepril group was given intraperitoneal injection of benazepril (30 mg·kg^-1·d^-1). Normal control group and DKD group were given an equal amount of normal saline. After 8 weeks of feeding, mice were euthanatized. Body weight and kidney weight were recorded. Blood, urine and kidney samples were collected. Biochemical tests such as blood glucose and urine albumin were measured by automatic biochemical instruments and albumin excretion rate was calculated. Pathological changes of mice were observed by hematoxylin-eosin staining (HE) and periodic acid-schiff staining (PAS). Phosph (p) IGF-1R expression level was determined by immunohistochemistry and Western blotting. Results: Compared with the normal control group, blood glucose, kidney weight/body weight and urinary albumin excretion rate were significantly higher in DKD group (all P<0.01). In DKD mice, glomerular expansion, tubular stenosis, tubular swelling and tubular atrophy were significantly detected. Meanwhile, the number of proximal tubular epithelial (PTE) cells was decreased, and the renal tubular injury scores, the average glomerular volume, and pIGF-1R protein expression were increased (all P<0.05). Compared with the DKD group, albumin excretion rate was significantly reduced (P<0.01), the above pathological changes were alleviated and the effect of IGF-1R inhibitor was more significant. Compared with the DKD group, the pIGF-1R protein expression was reduced in IGF-1R inhibitor group (P<0.05). Compared with the benazepril group, the pIGF-1R protein expression was reduced in IGF-1R inhibitor group (P<0.05). Conclusion IGF-1R inhibitor has better effect than benazepril on alleviating the tubulopathy of DKD mice.

BACKGROUND/AIMS: Neurotensin is a gut-brain peptide with both inhibitory and excitatory actions on the colonic musculature; our objective was to understand the implications of this for motor patterns occurring in the intact colon of the rat. METHODS: The effects of neurotensin with concentrations ranging from 0.1-100 nM were studied in the intact rat colon in vitro, by investigating spatio-temporal maps created from video recordings of colonic motility before and after neurotensin. RESULTS: Low concentration of neurotensin (0.1-1 nM) inhibited propagating long distance contractions and rhythmic propagating motor complexes; in its place a slow propagating rhythmic segmental motor pattern developed. The neurotensin receptor 1 antagonist SR-48692 prevented the development of the segmental motor pattern. Higher concentrations of neurotensin (10 nM and 100 nM) were capable of restoring long distance contraction activity and inhibiting the segmental activity. The slow propagating segmental contraction showed a rhythmic contraction—relaxation cycle at the slow wave frequency originating from the interstitial cells of Cajal associated with the myenteric plexus pacemaker. High concentrations given without prior additions of low concentrations did not evoke the segmental motor pattern. These actions occurred when neurotensin was given in the bath solution or intraluminally. The segmental motor pattern evoked by neurotensin was inhibited by the neural conduction blocker lidocaine. CONCLUSIONS: Neurotensin (0.1-1 nM) inhibits the dominant propulsive motor patterns of the colon and a distinct motor pattern of rhythmic slow propagating segmental contractions develops. This motor pattern has the hallmarks of haustral boundary contractions.
Absorption ; Animals ; Baths ; Colon* ; In Vitro Techniques ; Interstitial Cells of Cajal ; Lidocaine ; Myenteric Plexus ; Neural Conduction ; Neurotensin* ; Peristalsis ; Rats* ; Receptors, Neurotensin ; Video Recording