Objective:To evaluate the clinical efficacy, safety, and adverse effects of selumetinib in the treatment of children with plexiform neurofibromas (PNF).Methods:A retrospective analysis was conducted on the clinical data of 11 children with PNF who were treated with oral selumetinib for at least 9 months at Xuzhou Children′s Hospital from January 2024 to February 2025. The dosage was 25 mg/m2 twice daily. General patient information was collected, and clinical efficacy parameters and adverse events were compared before treatment, at 6 months, and at 9 months post-treatment. Tumor volume changes were assessed using magnetic resonance imaging (MRI) according to the Response Evaluation Criteria in Solid Tumours (RECIST). Pain severity and its impact on daily life were evaluated using the Wong-Baker Faces Pain Rating Scale Revision (FPS-R) and Pain Interference Index (PII). Safety and adverse events were monitored via regular clinical examinations, cardiac ultrasound, and ophthalmologic evaluations, with adverse events graded using Common Terminology Criteria for Adverse Events (CTCAE) v5.0.Results:All 11 patients completed follow-up [9.20-13.60 (10.65±1.64) months]. The cohort included 6 males and 5 females, aged 4.00-14.00 (9.35±3.00) years. At 6 months post-treatment, MRI revealed≥20% tumor volume reduction in 2 patients (2/11), meeting RECIST criteria for partial response (PR). The remaining 9 patients showed no significant tumor changes, though 2 exhibited lightening of café-au-lait macules (CALMs). By 9 months, 5 patients (5/11) achieved PR per RECIST, with residual tumors demonstrating volume reductions below PR thresholds. Nine patients showed further lightening of CALMs. Comparisons of FPS-R scores at 6 (5.27±1.01) and 9 months (3.64±0.81) post-treatment with baseline scores (6.72±1.01) showed statistically significant differences ( F=29.059, P<0.001). FPS-R scores at both 6 months post-treatment ( t=3.365, P=0.007) and 9 months post-treatment ( t=7.889, P<0.001) were significantly lower compared to baseline scores. FPS-R scores at 9 months post-treatment were significantly lower than those at 6 months post-treatment ( t=4.175, P=0.002). The PII at 9 months (15.64±2.86) differed significantly from baseline (19.64±2.66, t=3.396, P=0.003) and 6-month scores (18.27±2.45, t=2.316, P=0.031), whereas no significant difference was observed between 6-month and baseline PII scores ( t=1.256, P=0.225). Five of the 11 children experienced adverse reactions after taking the medicine, mainly manifested as acne, rash, paronychia, and vomiting. The CTCAE grades of all adverse reactions were≤2, and all could be relieved after symptomatic treatment. There were no significant changes in the cardiac ejection fraction and electrocardiogram of the 11 children compared with the baseline. Conclusions:Selumetinib effectively alleviated pain and improved daily function in children with PNF. Prolonged treatment significantly reduced tumor volumes, demonstrating durable efficacy. The drug exhibited a favorable safety profile, with manageable adverse events.

Objective:To evaluate the clinical efficacy, safety, and adverse effects of selumetinib in the treatment of children with plexiform neurofibromas (PNF).Methods:A retrospective analysis was conducted on the clinical data of 11 children with PNF who were treated with oral selumetinib for at least 9 months at Xuzhou Children′s Hospital from January 2024 to February 2025. The dosage was 25 mg/m2 twice daily. General patient information was collected, and clinical efficacy parameters and adverse events were compared before treatment, at 6 months, and at 9 months post-treatment. Tumor volume changes were assessed using magnetic resonance imaging (MRI) according to the Response Evaluation Criteria in Solid Tumours (RECIST). Pain severity and its impact on daily life were evaluated using the Wong-Baker Faces Pain Rating Scale Revision (FPS-R) and Pain Interference Index (PII). Safety and adverse events were monitored via regular clinical examinations, cardiac ultrasound, and ophthalmologic evaluations, with adverse events graded using Common Terminology Criteria for Adverse Events (CTCAE) v5.0.Results:All 11 patients completed follow-up [9.20-13.60 (10.65±1.64) months]. The cohort included 6 males and 5 females, aged 4.00-14.00 (9.35±3.00) years. At 6 months post-treatment, MRI revealed≥20% tumor volume reduction in 2 patients (2/11), meeting RECIST criteria for partial response (PR). The remaining 9 patients showed no significant tumor changes, though 2 exhibited lightening of café-au-lait macules (CALMs). By 9 months, 5 patients (5/11) achieved PR per RECIST, with residual tumors demonstrating volume reductions below PR thresholds. Nine patients showed further lightening of CALMs. Comparisons of FPS-R scores at 6 (5.27±1.01) and 9 months (3.64±0.81) post-treatment with baseline scores (6.72±1.01) showed statistically significant differences ( F=29.059, P<0.001). FPS-R scores at both 6 months post-treatment ( t=3.365, P=0.007) and 9 months post-treatment ( t=7.889, P<0.001) were significantly lower compared to baseline scores. FPS-R scores at 9 months post-treatment were significantly lower than those at 6 months post-treatment ( t=4.175, P=0.002). The PII at 9 months (15.64±2.86) differed significantly from baseline (19.64±2.66, t=3.396, P=0.003) and 6-month scores (18.27±2.45, t=2.316, P=0.031), whereas no significant difference was observed between 6-month and baseline PII scores ( t=1.256, P=0.225). Five of the 11 children experienced adverse reactions after taking the medicine, mainly manifested as acne, rash, paronychia, and vomiting. The CTCAE grades of all adverse reactions were≤2, and all could be relieved after symptomatic treatment. There were no significant changes in the cardiac ejection fraction and electrocardiogram of the 11 children compared with the baseline. Conclusions:Selumetinib effectively alleviated pain and improved daily function in children with PNF. Prolonged treatment significantly reduced tumor volumes, demonstrating durable efficacy. The drug exhibited a favorable safety profile, with manageable adverse events.

Xiaoxuming decoction is one of the classical prescriptions in ancient times,mainly used for treating stroke syndrome,with significant therapeutic effects.Recent studies have found that it is also effective for other neurological diseases besides stroke,such as facial paralysis and vertigo,but there are few reports on related achievements,lacking systematic organization and summary.This article systematically summarizes the application practice and mechanism of Xiaoxuming decoction in the above-mentioned diseases,aiming to provide a solid foundation and scientific reference for deepening the exploration of Xiaoxuming decoction in the field of nervous system disease.

The SWOT model of management was used to analyze the advantages(S),disadvantages(W),opportunities(O)and challenges(T)of the transformation of scientific and technological achievements in a large general hospital in Shandong Province.According to the matrix form,four kinds of strategies are formed,including SO strategy using internal advantages to seize opportunities,ST strategy using internal advantages to resist external threats,WO strategy using external opportunities to o-vercome internal weaknesses and WT strategy overcoming internal weaknesses to avoid external threats,and effective strategies to promote the transformation of scientific and technological achievements in large general hospitals are discussed.

The SWOT model of management was used to analyze the advantages(S),disadvantages(W),opportunities(O)and challenges(T)of the transformation of scientific and technological achievements in a large general hospital in Shandong Province.According to the matrix form,four kinds of strategies are formed,including SO strategy using internal advantages to seize opportunities,ST strategy using internal advantages to resist external threats,WO strategy using external opportunities to o-vercome internal weaknesses and WT strategy overcoming internal weaknesses to avoid external threats,and effective strategies to promote the transformation of scientific and technological achievements in large general hospitals are discussed.

Objective:To summarize the clinical features and gene mutation characteristics of a child with mitochondrial enoyl-CoA hydratase short chain 1 deficiency (ECHS1D) caused by enoyl-CoA hydratase short chain 1 ( ECHS1) gene mutation. Methods:The clinical characteristics and genetic test results of a child with ECHS1D who visited the Department of Neurology of Xuzhou Children′s Hospital in January 2021 were retrospectively analyzed, and the clinical features of the disease were also reviewed by searching relevant domestic and foreign literature.Results:The child was a 6 months and 4 days old male, with acute onset, the main clinical manifestation being limb movement disorder after admission. The child had slow motor development, his head was still upright and cannot turn over, the child also cannot sit alone, follow up and make a laugh, and the muscle tension of limbs was increased. The child′s blood lactate was increased to 6.2 mmol/L, which suggested metabolic acidosis, and magnetic resonance imaging (MRI) of the head showed abnormal signals in the basal ganglia on both sides, abnormal enhancement of the meninges of the left cerebral hemisphere. Whole exome sequencing revealed that the child had compound heterozygous mutations in ECHS1 gene, c.563C>T (p.A188V) and c.5C>T (p.A2V), respectively. The child′s father carried c.563C>T mutation, the mother carried c.5C>T mutation, all of which were missense mutations. Conclusions:ECHS1 gene mainly has missense mutations, most of which are compound heterozygous mutations, and a few are homozygous mutations. The ECHS1D caused by ECHS1 gene mutation often affects infants and young children. MRI suggests abnormal signals in the basal ganglia; for cases with the above clinical manifestations and abnormal signals in the basal ganglia on MRI, genetic testing should be considered to confirm the diagnosis.

Objective:To investigate the efficacy of dapagliflozin in patients with diabetic nephropathy.Methods:A total of 82 patients with early diabetic nephropathy who were treated in Wuhu No.1 People′s Hospital from January 2020 to December 2021 were selected as the research objects, and they were divided into the conventional group (41 cases) and the dapagliflozin group (41 cases) according to the random number table method. The patients in the conventional group were given original hypoglycemic treatment and the patients in the dapagliflozin group were given dapagliflozin treatment on the former basis, the patients in the two groups were treated for 12 weeks. The levels renal function index, blood glucose index, glomerular filtration index and the occurrence of adverse reactions during the treatment period were compared between the two groups.Results:After treatment, the total clinical effective rate in the dapagliflozin group was higher than that in the conventional group: 95.12%(39/41) vs. 78.05%(32/41), there was statistical difference ( χ2 = 4.96, P<0.05). The levels of urine albumin/creatinine (UACR), blood urea nitrogen (BUN) , serum creatinine (SCr), fasting blood glucose (FPG), 2 h postprandial blood glucose (2 h PG), glycosylated hemoglobin (HbA 1c), cystatin C (Cys-C) in the dapagliflozin group were lower than those in the conventional group: (49.73 ± 11.65) mg/g vs. (67.26 ± 10.04) mg/g, (6.96 ± 0.54) mmol/L vs. (7.25 ± 0.48) mmol/L, (76.82 ± 2.86) μmol/L vs. (78.59 ± 3.06) μmol/L, (8.58 ± 0.18) mmol/L vs. (8.80 ± 0.32) mmol/L, (8.03 ± 0.42) mmol/L vs. (8.56 ± 0.44) mmol/L, (7.06 ± 0.57)% vs. (7.52 ± 1.06)%, (1.47 ± 0.50) mg/L vs. (1.84 ± 0.55) mg/L, there were statistical differences ( P<0.05). The level of glomerular filtration rate (GFR) between the two groups had no significant differences ( P>0.05). The incidence of total adverse reactions in dapagliflozin group was lower than that in control group: 7.32%(3/41) vs. 24.39%(10/41), there was statistical difference ( P<0.05). Conclusions:Dapagliflozin has a good effect in the treatment of patients with early diabetic nephropathy. It can lower glucose, improve Cys-C, reduce urinary microalbumin and protect renal function.

Objective:To explore the application value of three-dimensional visualization technology in the analysis of anatomic variation of peripancreatic vessels in patients with pancreatic space occupation.Methods:A total of 98 cases in Yijishan Hospital of Wannan Medical College, the First Affiliated Hospital of University of Science and Technology of China, the First Affiliated Hospital of Anhui Medical University, the Second Affiliated Hospital of Anhui Medical University, the First Affiliated Hospital of Bengbu Medical College, Fuyang People's Hospital from June 2018 to December 2019 were retrospectively analyzed. Of 94 patients were enrolled, including 56 males and 38 females, aged (61.2±7.2) years. Abdominal organs and blood vessels were reconstructed by 3D visualization technology, and anatomic variation of peripancreatic vessels was analyzed, including abdominal trunk, hepatic artery system, portal vein system and dorsal pancreatic artery.Results:The three-dimensional reconstruction rate of celiac trunk vessels was 100.0% (94/94). The 60 cases of abnormal celiac trunk were as follows: liver and spleen trunk in 5 cases (5.3%), stomach and spleen trunk in 1 case (1.1%), liver, stomach and spleen mesentery trunk in 3 cases (3.2%), liver, stomach and spleen mesentery trunk in 17 cases (18.1%), celiac trunk with one or more inferior phrenic arteries in 34 cases (36.2%). There were 69 cases (73.4%) of Michels type Ⅰ, 2 cases (2.1%) of Michels type Ⅲ, 1 case (1.1%) of Michels Ⅴ, 2 cases (2.1%) of Ⅷ, and 1 case (1.1%) of type Ⅸ of hepatic artery system. There were 17 cases (18.1%) of accessory left hepatic artery from celiac trunk which did not belong to Michels classification. Left accessory hepatic artery originated from left gastric artery and left hepatic artery originated from superior mesenteric artery in 1 case (1.1%), right accessory hepatic artery originated from celiac trunk combined with proper hepatic artery and right anterior hepatic artery combined with gastroduodenal artery originated from right posterior hepatic artery in 1 case (1.1%). Abnormal walking of dorsal pancreatic artery in 3 cases. The inferior mesenteric vein flowed into the superior mesenteric vein in 38 cases (40.4%), and the inferior mesenteric vein flowed into the splenic vein and superior mesenteric vein in 4 cases (4.3%).Conclusion:Three-dimensional visualization technique is helpful to determine the variation of peripancreatic vessels, and has certain significance for accurate preoperative evaluation and surgical guidance of patients with pancreatic space occupation.

"The Expert Group on Tumor Ablation Therapy of Chinese Medical Doctor Association, The Tumor Ablation Committee of Chinese College of Interventionalists, The Society of Tumor Ablation Therapy of Chinese Anti-Cancer Association and The Ablation Expert Committee of the Chinese Society of Clinical Oncology" have organized multidisciplinary experts to formulate the consensus for thermal ablation of pulmonary subsolid nodules or ground-glass nodule (GGN). The expert consensus reviews current literatures and provides clinical practices for thermal ablation of GGN. The main contents include: (1) clinical evaluation of GGN, (2) procedures, indications, contraindications, outcomes evaluation and related complications of thermal ablation for GGN and (3) future development directions.
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Leigh syndrome (French-Canadian type, LSFC) is a rare autosomal recessive hereditary severe neurological disorder that begins in infancy. Herein we report a case with LSFC in China. The patient was 8 months old, male, whose clinical manifestations included delayed development, low muscle tone, unstable vertical head, inability to sit alone, cognitive impairment, slightly smaller forehead, oblique eyes, epilepsy, etc. Gene sequencing results showed that the LRPPRC gene in the infant had complex heterozygous mutations of c.2989G>A (newly reported) and c.4078G>A. Combined with the clinical manifestations, gene mutations and literatures, the infant was diagnosed as LSFC, and symptomatic rehabilitation was performed. The results of genetic testing can contribute to the early diagnosis and genetic counseling of LSFC patients, and help reduce the burden on the patients and their families.