OBJECTIVE: To investigate the effectiveness of posterior lateral perforator flap in lower limb combined with free fibula for maxillary tissue defect repair. METHODS: Between December 2018 and December 2023, 16 patients with the maxillary malignant tumors were admitted. There were 10 males and 6 females, with an average age of 64.3 years (range, 54-75 years). There were 7 cases of maxillary gingival cancer, 5 cases of hard palate cancer, and 4 cases of maxillary sinus cancer. According to the 2017 American Joint Committee on Cancer (AJCC) TNM stage, there were 8 cases of stage Ⅲ, 6 cases of stage Ⅳa, and 2 cases of stage Ⅳb. After resection of the lesion, the remaining maxillary defects were classified into class Ⅱa in 3 cases, class Ⅱb in 5 cases, and class Ⅲb in 8 cases according to Brown's classification. The size of soft tissue defects ranged from 4 cm×3 cm to 8 cm×6 cm. The posterior lateral perforator flap in lower limb in size of 5 cm×4 cm-9 cm×7 cm were harvested to repair soft tissue defects, and free fibula in length of 6-11 cm were used to repair bone defects. The donor sites of the lower limb were sutured directly (6 cases) or repaired with free skin grafting (10 cases). Six patients with positive lymph node pathology were treated with radiotherapy after operation. At 6 and 12 months after operation, the self-assessment was performed by the University of Washington Quality of Survival Questionnaire Form (QUW-4) in five dimensions (facial appearance, swallowing function, chewing function, speech function, and mouth opening), and swallowing function was evaluated by using the Kubota water swallowing test. RESULTS: Postoperative pathological examination showed that all patients were squamous cell carcinoma. One patient who was treated with radiotherapy developed osteomyelitis and 1 patient developed venous crisis of skin flap. The rest of the flaps and all skin grafts survived, and the wounds healed by first intention. All patients were followed up 1-5 years (mean, 2.8 years). Two patients died of local recurrence of the tumor at the 4th and 5th years after operation, respectively. Except for the chewing function score and total score at 6 months after operation, which showed significant differences compared to preoperative scores ( P<0.05), there was no significant difference in other QUW-4 scale scores between different time points ( P>0.05). The patients' swallowing function evaluated by Kubota water swallowing test reached normal in 4 cases, suspicious in 9 cases, and abnormal in 3 cases at 6 months after operation, and 10, 6, and 0 cases at 12 months after operation, respectively. The swallowing function at 12 months was significantly better than that at 6 months ( Z=-2.382, P=0.017). CONCLUSION The posterior lateral perforator flap in the lower limb combined with free fibula to repair maxillary tissue defects can repair soft and hard tissue defects at the same time, so that the patient's facial appearance, swallowing function, chewing function, speech function, and mouth opening are satisfactorily restored and the mid-term effectiveness is good.
Humans ; Middle Aged ; Male ; Female ; Fibula/surgery* ; Aged ; Perforator Flap ; Plastic Surgery Procedures/methods* ; Maxilla/surgery* ; Maxillary Neoplasms/surgery* ; Free Tissue Flaps/transplantation* ; Lower Extremity/surgery* ; Bone Transplantation/methods* ; Treatment Outcome

[This corrects the article DOI: 10.1016/j.apsb.2022.05.019.].

Objective:To evaluate clinical outcomes of occlusion-guided vascularized folded fibula flap reconstruction with delayed implant restoration for mandibular defects after benign tumor resection.Methods:A total of 12 patients with benign mandibular tumors underwent free folded fibula flap reconstruction at the First Affiliated Hospital of Bengbu Medical University between January 2020 and December 2023, including 7 males and 5 females, aged 21-52 years. Six months after mandibular reconstruction, the internal fixation titanium plates were removed, and dental implants were placed using a preoperatively fabricated occlusal guide, followed by second-stage implant surgery and prosthetic restoration. Mandibular CT scans were obtained 6 months after reconstruction to compare the fitting accuracy between the preoperative virtual design and the actual reconstructed mandible. The implant stability quotient (ISQ) was measured 3 months after implant placement. Masticatory efficiency and Enneking lower limb function scores were evaluated at the following time points: before tumor surgery (T1), before implant placement (T2), 6 months (T3) and 9 months (T4) after implant crown restoration. One-way repeated measures ANOVA was used to analyze the masticatory efficiency and lower limb function scores.Results:The free folded fibula grafts were successfully performed via an intraoral approach in all 12 patients, with a 100% of survival rate. Mandibular defects included Brown class I in 6 cases, class II in 2 cases, and class III in 4 cases. A total of 42 implants were placed with successful osseointegration. The ISQ measured at 3 months post-placement was 64.10±4.18. At 6 months postoperatively, morphological analysis comparing the preoperative virtual surgical design with the actual postoperative reconstructed mandible revealed a reconstruction accuracy of 84.27%±4.23%. Significant differences were observed in Enneking scores and masticatory efficiency across all four time points (all P<0.001). Masticatory function showed significant improvement at T4 compared that at T2 [(88.06±3.66)% vs. (65.44±3.82)%, P<0.05]. Conclusion:Occlusal function-guided mandibular reconstruction with vascularized folded fibula flap after removal of benign mandibular tumors is a reliable method, which is associated with minimal donor-site morbidity and enables patients to restore precise occlusion and to achieve favorable masticatory efficiency.

Turning to critical illness is a common stage of various diseases and injuries before death. Patients usually have complex health conditions, while the treatment process involves a wide range of content, along with high requirements for doctor′s professionalism and multi-specialty teamwork, as well as a great demand for time-sensitive treatments. However, this is not matched with critical care professionals and the current state of medical care in China. Telemedicine, which shortens the distance of medical professionals and the gap of disease diagnosis and treatments in various regions through electronic information, can effectively solve the current problem. Therefore, there is an urgent need to develop a standardized, high-quality visualization telemedicine round system .Therefore, experts have been organized to search domestic and foreign literature on telemedicine round for critically ill patients and to form this consensus based on clinical experiences so as to further improve the level of critical care treatments in regions.

Objective:To investigate the clinical and genetic characteristics of patients with amyotrophic lateral sclerosis (ALS) caused by FUS gene mutations. Methods:A retrospective analysis was conducted on 7 families diagnosed with FUS gene related ALS in the Department of Neurology of Henan Provincial People′s Hospital from January 2018 to June 2024. Clinical data and neuroelectrophysiological results of the probands and family members were collected. Next generation sequencing or whole exome sequencing was conducted on the probands. The detected variants of the FUS gene were confirmed by Sanger sequencing. Results:Among the 7 probands, 4 were with familial ALS and 3 with sporadic ALS, including 6 males and 1 female. The average age of onset was 24.6 years (ranging from 21 to 30 years). The onset site included bulbar muscles in 1 case, proximal upper limbs in 3 cases, proximal lower limbs in 2 cases, and both upper and lower limbs in 1 case. Four patients presented both upper and lower motor neurons involvement on examination, and 3 had only lower motor neuron syndrome. Muscle atrophy and fasciculation were observed in 6 patients respectively, and dyspnea in 3 patients. Bilateral muscle strength was asymmetric in 5 patients. Proximal muscle weakness was predominant in 6 of the 7 patients with upper limb weakness, and 3 of the 5 patients with lower limb weakness. Electromyography showed neurogenic damage in all 7 cases. Five heterozygous variants of the FUS gene were detected in 7 patients, including 2 patients with c.1574C>T(p.P525L), 2 with c.1552A>G(p.R518G), 1 with c.1561C>T(p.R521C), 1 with c.1441delC(p.R481Efs *48), and 1 with both c.1574C>T(p.P525L) and c.430_447del(p.G144_Y149del) variants. The variant c.1441delC(p.R481Efs *48) had not been previously reported. During follow-up, 6 patients died of respiratory failure 6-18 months after onset, with an average of 11.8 months. Conclusions:Patients with FUS gene related ALS have an early age of onset, rapid progression, short survival period, asymmetric limb weakness, and more severe involvement of proximal limbs. The c.1574C>T(p.P525L) is a hotspot mutation, and the novel variant c.1441delC(p.R481Efs *48) enriches the mutation spectrum of the FUS gene.

Objective:To investigate the clinical and genetic characteristics of spinal muscular atrophy (SMA) patients with SMN1 gene compound heterozygous mutations. Methods:Three SMA-Ⅲ pedigrees treated in Henan Provincial People′s Hospital from October 2019 to July 2020 were selected. The clinical data of 3 SMA-Ⅲ probands were retrospectively analyzed. Multiplex ligation-dependent probe amplification (MLPA) technology was used to detect the copy number of the SMN gene in the probands and their parents. Polymerase chain reaction amplification combined with microfluidic capillary electrophoresis were used to detect point mutations in the SMN1 gene of the probands. Sanger sequencing was used to validate candidate variant sites. Results:The 3 probands are all male, aged 19, 17 and 12 years, respectively. The main clinical manifestations were symmetrical muscle weakness mainly in the proximal lower limbs, mild to moderate elevation of serum creatine kinase, and neurogenic injury as determined by electromyography or muscle pathology. The genetic testing results showed that all 3 probands had heterozygous deletion in exon 7 of the SMN1 gene, and carried heterozygous variations c.275G>A (p.Trp92 *), c.689C>T (p.Ser230Leu), and c.708dupT (p.Pro237Serfs *19), respectively. The exon deletion and point mutation were inherited separately from their parents. c. 275G>A (p.Trp92 *) and c.708dupT (p.Pro237Serfs *19) variations had not been reported before. Conclusions:The clinical manifestations of SMA-Ⅲ patients are symmetrical muscle weakness, mainly in the proximal extremities of both lower limbs, and electromyography or muscle biopsy suggesting neurogenic lesions. The compound heterozygous variation of point mutation and heterozygous deletion in the SMN1 gene can lead to SMA-Ⅲ. Suspected SMA patients with SMN1 gene heterozygous deletion should take point mutation testing.

Objective:To analyze the clinical manifestations and muscular magnetic resonance imaging (MRI) features of 12 families with facioscapulohumeral muscular dystrophy (FSHD).Methods:Retrospective analysis was conducted on 12 FSHD families diagnosed by genetic testing at the Department of Neurology of Henan Provincial People′s Hospital from January 2017 to June 2021. Clinical data and lower limb muscle MRI results of the probands and related members of the families were collected, and the degree of muscle fatty degeneration shown in the MRI was scored using the modified Mercuri score.Results:There were 21 patients in 12 families, with the age of onset ranged from 10 to 47 years (mean 19.5 years). The course of disease ranged from 1 to 47 years (mean 23.1 years). The onset sites included unilateral upper extremity in 8 cases, bilateral proximal upper extremities in 9 cases, bilateral proximal lower extremities in 2 cases, unilateral proximal lower extremity in 1 case, and simultaneous onset in all 4 limbs in 1 case. Sixteen patients had limb weakness and bilateral asymmetry, and 11/16 cases were more severe on the right side than the left side. Winged scapular and facial muscle weakness were observed in all patients. The creatine kinase range was 85-1 038 U/L (461 U/L on average) in 12/21 cases. There were 10/21 cases of myogenic lesion in electromyography. Myodystroph-like pathological changes were found in 11/21 cases. The fragment length of the 4q35 subtelomere polymorphism EcoRI/p13E-11 was less than 38 kb in 20/21 cases; 1 case was confirmed based on clinical symptoms and family history. Fat infiltration occurred in at least one muscle of lower limbs in 9/10 cases, in thigh muscle in 9/10 cases and in calf muscle in 6/10 cases. The average score of fat infiltration in thigh muscle group was higher than that in calf muscle group. The muscles with higher fat infiltration scores were the vastus intermedius, the long head of the biceps femoris, the vastus medialis, the vastus lateralis, the semitendinosus, the semimembranosus, the vastus lateralis in the thigh (with score ≥2.15), the tibialis anterior, and the medial head of the gastrocnemius in the calf (with score ≥1.11). Fat infiltration in the medial and posterior thigh muscles was more common than in the anterior thigh muscles. There was asymmetry of bilateral muscle fat in 9/10 cases. There were edematous changes in thigh muscles in 1 case and in calf muscles in 3 cases.Conclusions:The age of onset of FSHD patients is mostly ≤30 years. Bilateral asymmetric involvement is the characteristic manifestation of FSHD. The FSHD patients ' muscles most affected by the disease in the thigh are the quadriceps femoris, the long head of the biceps femoris, the vastus medialis, the vastus lateralis, the semitendinosus, the semimembranosus, and the vastus intermedius. In the calf, the muscles most affected are the anterior tibial muscle and the medial head of the gastrocnemius. The MRI pattern of muscle involvement of patients with FSHD is bilateral asymmetrical lesions, with the right side having more severe lesions. The fatization of thigh muscles is more significant than that of calf muscles, and the asymmetry of fatization between bilateral muscles is also present.

Objective:To investigate clinical, lower limb muscle magnetic resonance imaging (MRI) and myopathological features in hereditary caveolinopathy patients.Methods:Clinical data, lower limb muscle MRI and pathological findings of 2 patients with caveolinopathy diagnosed by genetic examination in Henan Provincial People′ s Hospital in January 2020 and August 2022 were retrospectively analyzed. And the characteristics of patients with hereditary caveolinopathy reported in China were analyzed in combination with literature review.Results:Case 1 was a 14-year-old boy. At 4 years old, his right heel did not touch the ground while squatting, and at 11 years old, he experienced weakness in his lower limbs and muscle pain during running, as well as difficulty in lifting his feet while walking. Six months earlier, he underwent surgery for right high-arched foot. Physical examination revealed slight atrophy of both first interosseous muscles, hypertrophy of the left calf, decreased muscle strength in the right distal leg, percussion-induced muscle mounding, postoperation of right high-arched foot, left high-arched foot as well as bilateral ankle contracture, foot drop, and inversion. Case 2 was a 15-year-old girl. At 14 years old, she presented prolonged exercise myalgia. Physical examination showed no positive signs. Both patients had no family history. Creatine kinase was slightly elevated in the 2 patients. Electromyography in the 2 patients showed a myopathic pattern in distal and proximal muscles. T 1-weighted MRI of lower limb muscles showed significant fatty infiltration in the bilateral rectus femoris, bilateral semitendinosus muscles, right tibialis anterior, right peroneus and right gastrocnemius lateral head in case 1. Selective rectus femoris muscle fatty infiltration in the thighs and mild gastrocnemius fatty infiltration in the lower legs were observed in case 2. Short inversion recovery sequence showed edema-like changes in both patients. Muscle pathology of both patients showed dystrophic-like pathological changes. A total of 9 Chinese patients with hereditary caveolinopathy reported in previous literature and in this study had an onset age from early childhood to youth. The main clinical manifestations were slowly progressive distal limb muscle weakness and atrophy accompanied by myalgia or merely presenting as myalgia. The creatine kinase levels were mildly to moderately elevated. Electromyography could show myogenic damage, myogenic and neurogenic mixed damage or normality. The muscle pathology showed myopathy-like or muscular dystrophy-like changes. Conclusions:The clinical manifestations of hereditary caveolinopathy are heterogeneous, and exercise-induced myalgia is frequently the most prominent symptom. The rectus femoris and gastrocnemius muscles tend to show early fatty infiltration in legs on MRI. Muscle pathology lacks specificity.

Objective:To verify the prognostic value of perineural invasion and lymphovascular tumor embolus for patients with gastric cancer undergoing gastrectomy and postoperative chemotherapy, and establish a prognostic prediction nomogram model.Methods:According to 7∶3 radio, 781 gastric cancer patients were randomly divided into training cohort and internal validation cohort. One hundred fifty patients were utilized as the external validation cohort. Univariate and multivariate analysis were performed to evaluate the prognostic value of perineural invasion and lymphovascular tumor embolus, and construct the dynamic nomogram. The concordance index (C-index), net reclassification index and integrated discrimination improvement index, receiver operating characteristic curve, calibration curves and decision curve analysis were used to evaluate the nomogram.Results:Perineural invasion ( HR=1.486, 95% CI: 1.150-1.919, P<0.01) and lymphovascular tumor embolus ( HR=1.321, 95% CI: 1.030-1.693, P<0.05) were independent prognostic risk factors for patients with gastric cancer after gastrectomy and postoperative chemotherapy. C-index (training cohort: 0.734, internal validation cohort: 0.755, external validation cohort: 0.715), net reclassification index (training cohort: 0.228 for 3-year and 0.213 for 5-year OS prediction; internal validation cohort: 0.211 for 3-year and 0.279 for 5-year OS prediction; external validation cohort: 0.220 for 3-year and 0.440 for 5-year OS prediction) and integrated discrimination improvement index (training cohort: 0.051 for 3-year and 0.041 for 5-year OS prediction; internal validation cohort: 0.027 for 3-year and 0.036 for 5-year OS prediction; external validation cohort: 0.063 for 3-year and 0.153 for 5-year OS prediction) indicated that the nomogram performed better than the traditional TNM staging system ( P<0.05). Conclusions:Perineural invasion and lymphovascular tumor embolus are independent prognostic risk factors of gastric cancer patients after postoperative chemotherapy. The novel dynamic nomogram model based on perineural invasion and lymphovascular tumor embolus provides better assistance in evaluating prognosis of gastric cancer patients.

Objective:To investigate the current status of clinical leadership among Interventional Operating Room nurses in Shandong Province and to analyze its influencing factors.Methods:In December 2024, 220 Interventional Operating Room nurses from 46 ClassⅢ Grade A general hospitals in Shandong Province were selected for a cross-sectional survey using convenience sampling. General Information Questionnaire, Clinical Leadership Survey, Chinese version of Wong and Law Emotional Intelligence Scale (WLEIS-C) , and Jefferson Scale of Empathy-Health Professionals (JSE-HP) were used as survey instruments. Multiple linear regression was used to analyze the factors influencing clinical leadership among Interventional Operating Room nurses. Pearson correlation analysis was used to explore the relationship between emotional intelligence, empathy, and clinical leadership.Results:A total of 220 questionnaires were distributed and 204 valid questionnaires were recovered, with a valid recovery rate of 92.73% (204/220) . The clinical leadership scores of the 204 Interventional Operating Room nurses were (66.35±8.74) , and the dimensions scored, in descending order, as encouraging the heart, enabling others to act, modeling the way, inspiring a shared vision, and challenging the process. The mean scores of WLEIS-C and JSE-HP items were (5.88±0.90) and (4.61±0.63) , respectively. Multiple linear regression analysis showed that the nature of employment and whether or not they participated in leadership training were influencing factors for clinical leadership among Interventional Operating Room nurses (partial regression coefficients of -2.831 and -2.999, respectively; P<0.05) . Emotional intelligence and empathy ability among Interventional Operating Room nurses were positively correlated with clinical leadership ( P<0.05) . Conclusions:Clinical leadership of nurses in the Interventional Operating Room of ClassⅢ Grade A general hospitals in Shandong Province is at a moderate to high level. Nurses who participate in leadership training, have staffing, have greater empathy, and have higher emotional intelligence have greater clinical leadership. It is recommended that nursing administrators add leadership-related courses to the training of nurses in the Interventional Operating Room, and take steps to improve nurses' emotional intelligence and empathy ability, thereby promoting clinical leadership among Interventional Operating Room nurses.