Objective:To delineate the surgical methodology and therapeutic paradigm of proximal tibial notch retrograde interlocking intramedullary nailing for ameliorating deformities due to osteofibrous dysplasia (OFD) in a pediatric population.Methods:A retrospective assessment was conducted on the medical records of individuals undergoing orthopedic osteotomy complemented by retrograde interlocking intramedullary nailing for OFD of the tibia from January 2016 to December 2019. The cohort comprised 15 patients, with a follow-up exceeding three years, documenting complete data sets. The patient profile included 8 males and 7 females, with 8 left-side and 7 right-side afflictions. The mean age at the time of surgery was 10.1±2.5 years, ranging from 7.1 to 12.6 years. Parameters measured were preoperative and postoperative imaging findings, which encompassed the scope of the lesion (longitudinal lesion length relative to tibial length), coronal and sagittal limb alignments, and lower limb length discrepancies.Results:The mean follow-up duration was 3.4±1.3 years, ranging from 3 to 6.6 years. Preoperatively, prominent anterior tibial arch deformities and limping were present, with 7 cases reporting fatigue-induced pain and 3 instances of pathological fractures. Post-surgery, pain symptoms were resolved, gait disturbances were improved in 9 patients, and completely resolved in 6. Tibial osteotomy or fracture healing of 15 patients averaged 3.9±0.7 months (range 3-5 months). The lesion range before surgery was 0.41±0.17, immediately after surgery was 0.38±0.17, and at the last follow-up was 0.30±0.16, with no statistical significance ( F=0.101, P=0.904). Lesion range showed no significant change throughout treatment, but radiographic density within the lesion notably increased post-surgery, suggesting bone improvement. The anterior tibial arch Angle was 28.30°±6.62° (range 20°-45°) before surgery, 4.73°±1.53° immediately after surgery, and 6.87°±1.36° at the last follow-up, with statistical significance ( F=159.739, P<0.001). A significant correction in the anterior tibial arch deformity was achieved and maintained postoperatively. There was no significant angular deformity of the tibia in the coronal plane before operation, and the medial proximal tibial angle (MPTA) and lateral distal tibial angle (LDTA) were 87.50°±1.46° and 88.30°±1.62°, 88.40°±1.46° and 88.70°±1.45° immediately after surgery, and 88.00°±1.39° and 89.10°±1.53° at the last follow-up, the differences were statistically significant ( F=1.741, P=0.188; F=1.016, P=0.371), there was no coronal deformity of tibia. The limb length discrepancy (LLD) was 0.60±0.98 cm before surgery, 0.18±0.93 cm at the last follow-up, with statistical significance ( t=0.096, P=0.761). There were no incidents of postoperative complications such as infection. Conclusion:In pediatric cases of tibial deformities attributed to osteofibrous dysplasia, a therapeutic strategy involving osteotomy for lower limb realignment, sans curettage or bone grafting of the lesion, followed by retrograde interlocking intramedullary nailing, yields favorable outcomes. Importantly, this implantation technique does not compromise the integrity of the proximal tibial epiphyseal plate in children and adolescents.

Objective:To explore the clinical effect of plating after lengthening to assist the consolidation of tibial shortening deformity in children.Methods:A retrospective analysis was conducted on 10 children with tibial shortening who were treated with circular external fixator lengthening and replacement with plate-assisted internal fixation (study group) in the Department of Pediatric Orthopedics of Tianjin Hospital from November 2019 to October 2022, and 16 children who were treated by circular external fixator only during the same period were enrolled as the external fixator group (control group). Among the 26 cases, there were 15 males and 11 females, 10 left knees and 16 right knees. The average age at the time of surgery was 8.8±4.7 years (range 5.1-13.8 years). The gender, side, age at the time of surgery were compared between the two groups. The extension length, external fixator index, healing index, Kolcaba comfort scale score, knee and ankle joint range of motion, complications and average total hospitalization costs were recorded.Results:There were no significant differences in gender, side, age at the time of surgery between the study group and control group. The extended lengths were 5.44±1.25 cm and 5.78±1.11 cm respectively without significant difference ( t=0.096, P=0.096). The external fixator index and healing index were 17.86±2.94 d/cm vs. 50.97±7.03 d/cm and 40.94±6.63 d/cm vs. 45.24±5.98 d/cm in study group and the control group with significant differences ( t=13.299, P<0.001; t=1.289, P=0.033). The Kolcaba comfort scale score of the children in the study group was higher than that of the control group, with significant difference ( t=6.821, P=0.001). Comparing the range of motion of the knee and ankle joints between the two groups, there were no significant differences before surgery, at the end of extension surgery, and at the final follow-up. When the extension end was healed, the range of motion of the knee joint was137.89°±4.40° vs. 114.09°±13.60° and ankle joint was 64.35°±5.50° vs. 56.65°±8.86° in the study group and control group with significant difference ( t=17.235, P<0.001; t=7.821, P=0.002). In the study group, 4 cases had pin tract infection, but no refracture occurred; in the control group, pin tract infection occurred in 13 cases, and refracture occurred in 2 cases after removal of the external fixator. The average total hospitalization cost of the study group was higher than that of the control group with significant difference ( t=3.745, P=0.036). Conclusion:The clinical effect of replacing plate-assisted internal fixator during the mineralization period of tibial shortening in children is reliable, and can significantly shorten the time for using external fixator. It is beneficial to the healing of the extended end of the osteotomy and the functional recovery of the knee and ankle joints. Being more comfortable for children with fewer complications, it is applicable for children who need long-distance extension, multiple extensions with limited joint function, poor tolerance for external fixators and low treatment compliance.

Objective:To explore influencing factors of the self-reported brief life quality satisfaction score(Brief-QoL) in patients with acromegaly and understand the persistent low Brief-QoL scores in cases achieving biochemical remission.Methods:This study included 836 acromegaly patients who were hospitalized at Peking Union Medical College Hospital between January 2012 and December 2020. We retrospectively examined how clinical characteristics, biochemical parameters, comorbidities, and symptoms influenced Brief-QoL. Among patients who achieved biochemical remission, differences in clinical symptoms and comorbidities were analyzed between the high and low quality of life groups.Results:Patients with well-controlled biochemical indicators at the last follow-up had generally high Brief-QoL. However, patients with symptoms such as headaches (47.8% in the low-score group vs 14.9% in the high-score group, P<0.001) and joint pain (69.6% in the low-score group vs 19.0% in the high-score group, P<0.001) had low Brief-QoL despite biochemical remission. Receiving combined treatment(52.4% in the low-score group vs 27.5% in the high-score group, P=0.030) and having comorbid diabetes or hyperlipidemia were significant factors leading to decreased quality of life. Conclusion:Brief-QoL is suitable for follow-up of outpatient patients. Early identification of factors affecting quality of life and timely intervention can facilitate the realization of standardized management.

Objective:To explore the clinical efficacy of composite pin-rod external fixation in the treatment of open tibial fractures in children.Methods:The clinical data were retrospectively analyzed of the 21 pediatric patients with open tibial fracture who had been treated at Department of Pediatric Orthopeadics, Tianjin University by composite pin-rod external fixation from January 2019 to December 2021. There were 15 boys and 6 girls, and 9 left sides and 12 right sides. The age at the time of operation was (8.6±4.1) months. According to the Gustilo-Anderson classification, there were 3 cases of type Ⅰ, 12 cases of type Ⅱ, 3 cases of type ⅢA, 1 case of type ⅢB, and 2 cases of type ⅢC. Twenty cases had a concomitant fibular fracture, 3 cases multiple injuries, and 3 cases a segmental fracture. All the operations were performed at the emergency department. After debridement, the fractures were reduced and immobilized with a triangle external fixation of composite pin-rod system. In the patients whose conditions permitted, the wounds were closed at one stage, and no other external fixation was added after operation. Wound healing, fracture healing, frame time, knee and ankle functional results and complications were evaluated.Results:All patients were followed up for (20.7±15.8) months. Primary wound closure was achieved in 18 cases; the wound was covered by vacuum sealing drainage after debridement and reverse skin removal in the other 3 cases, of which 2 healed smoothly, and 1 was complicated with superficial infection of Gustilo type ⅢC treated eventually by an anterolateral thigh flap. Fractures healed satisfactorily in 20 cases, with a frame time of (4.5±1.7) months. At the last follow-up, there was no significant difference in the range of motion of the knee and ankle joints on the affected side compared with the uninjured side ( P>0.05). According to the Johner-Wruhs evaluation criteria, the functional recovery was excellent in 16 cases and good in 4 cases. Fracture nonunion happened in one child after skin flap operation, but fracture occurred again after reoperation for fracture, yielding a poor Johner-Wruhs evaluation. Needle tract infection was found in 9 cases but responded to therapy with no other complications. Conclusions:In the treatment of open tibial fractures in children, the composite pin-rod external fixation may lead to satisfactory clinical outcomes. It is easy in operation, provides stable fixation, and allows early rehabilitation after surgery. It can be used as a terminal treatment for wounds that can be closed at one stage.

Objective:To investigate the efficacy of open exploration, ulnar osteotomy and annular ligament reduction (three-in-one surgery) in the treatment of chronic Monteggia fracture in children.Methods:The data were analyzed retrospectively of the 35 children with chronic Monteggia fracture who had been admitted to Department of Pediatric Orthopaedics, Tianjin University from June 2017 to September 2021. There were 22 boys and 13 girls, and 19 left and 16 right sides, with an age of (6.8±2.5) years. Anterolateral dislocation of the radial head occurred in 30 cases and anterolateral dislocation in 5 ones. The time from injury to operation was (17.9±9.9) months. All patients were treated with three-in-one surgery. The elbow flexion and extension, forearm rotation and The Hospital for Special Surgery (HSS) elbow score were measured and compared in all patients at 12 months after operation. Their complications were also recorded.Results:All children were followed up for 12 months. The osteotomy ends of the ulna achieved bony union 1.5 to 4.0 months after operation. The elbow extension (-1.0°±5.9°) and flexion (128.5°±4.9°) at 12 months after operation were significantly improved compared with those before operation (-9.3°±18.0° and 108.4°±17.3°) ( P<0.05). The forearm pronation (61.5°±19.4°) at 12 months after operation was significantly limited compared with that before operation (72.7°±22.4°) ( P<0.05). There was no significant difference between the forearm supination (86.7°±4.5°) at 12 months after operation and that before operation (81.0°±17.4°) ( P>0.05). The HSS elbow score at 12 months after operation (93.5±5.2) was significantly higher than that (80.6±9.3) before operation ( P<0.05). The efficacy evaluated by the HSS elbow score at 12 months after operation was excellent in 21 cases, good in 12 cases, and fair in 2 cases, giving an excellent and good rate of 94.3% (33/35). Postoperative re-subluxation was observed in one patient. Conclusion:Open exploration, ulnar osteotomy and annular ligament reduction are a safe and effective three-in-one surgery for chronic Monteggia fracture in children, because it may lead to significantly improved elbow flexion and extension after operation, though the forearm pronation may be limited.

Objective:To summarize the clinical characteristics of 4 cases of mixed pituitary adenomas involving growth hormone(GH), prolactin(PRL), and thyroid stimulating hormone(TSH), and explore the standardized management approaches.Methods:The clinical data of four GH/PRL/TSH mixed pituitary adenoma patients diagnosed by Peking Union Medical College Hospital were retrospectively analyzed, including clinical manifestations, biochemical parameters, radiographic characteristics, as well as treatment and prognosis. Then literature review was conducted.Results:Among the 4 patients, 3 were male, with onset ages ranging from 15 to 38 years. All patients presented with coarse facial features as initial symptom. Three patients had visual impairment or visual field defects. All 4 patients had significantly elevated levels of GH and insulin-like growth factor-Ⅰ(IGF-Ⅰ). GH was not inhibited by oral glucose tolerance test. PRL concentration was over 100 ng/mL. Triiodothyronine(T 3)and thyroxine(T 4)were also elevated, while TSH was not inhibited. All pituitary adenomas in four cases were macroadenomas or giant adenomas, all of which were invasive growth, and one case developed pituitary stroke. Except for one patient who did not receive treatment in our hospital due to medical expenses, the remaining three patients underwent a combined treatment of medication and transnasal transsphenoidal pituitary adenoma resection. Among them, one patient had relief of central hyperthyroidism and hyperprolactinemia, but GH/IGF-Ⅰ did not meet the remission criteria. The other two patients had persistent non-resolution of at least 2 hormone axes. Conclusions:Patients with GH/PRL/TSH mixed pituitary adenoma were mainly characterized by coarse facial features, GH/PRL/TSH hyperfunction, large adenoma volume, low biochemical remission after surgery combined with drug treatment, and poor clinical prognosis.

Objective:To retrospectively analyze and evaluate the effect of interlocking intramedullary nail in the treatment of proximalfemoral fibrous dysplasia (FD) and to discuss its application techniques and treatment strategies.Methods:The patients with proximal femoral FD who received treatment with interlocking intramedullary nail in our department from April 2014 to August 2018 were retrospectively reviewed. The inclusion criteria included having complete data with more than 3 years follow-up. A total of 31 cases with 32 thighs were included in the present study. Among them, there were 24 males and 7 females. The mean age at operation was 11.4±2.3 years. The mean follow-up duration was 3.9±1.1 years. The surgical methods were osteotomy or in situ interlocking intramedullary nailing, and femoral neck reconstruction nails pass through the epiphyseal plate in some cases. Preoperative, postoperative and final follow-up radiographic changes, including femoral neck shaft angle, proximal femoral diaphyseal deformity, and femoral alignment were compared. Univariate analysis of the influencing factors of postoperative femoral neck shaft angle loss.Results:In this group, 13 patients had coxa varus deformity, and the angle of the neck shaft was 107.3±7.1°, and 23 patients had the bending deformity of the proximal femur, and angled 29.5°±9.1°. 10 cases of coxa varus combined with proximal femoral diaphyseal deformity received double osteotomy and internal fixation, 17cases of simple coxa varus or diaphyseal deformity received single osteotomy and internal fixation, and 5 cases had no significant deformity, underwent in situ intramedullary nailing due to pathological fractures and pain. Postoperative femoral neck shaft angle was 133.2°±5.2°. Complete correction of proximal femoral flexion deformity was obtained and the diaphyseal axis was within the normal range. At the last follow-up, the neck shaft angle of 7 patients (21.9%, 7/32) decreased from 133.6°±3.9° to 125.7°±3.4°. Univariate analysis showed that the proportion of neck shaft angle loss in cases with proximal femoral locking nails passing through the epiphyseal plate was significantly lower than that in the cases without (0 vs. 43.8%, χ 2=6.58, P=0.010). Conclusion:The application of interlocking intramedullary nails in the treatment of proximal femoral fibrous dysplasia can achieve good results, according to the situation of coxa varus or diaphysis bending deformity, single or double osteotomy can be performed. There is a risk of femoral neck shaft angle loss after surgery, which can be avoided by femoral neck reconstruction locking screw fixation crossing epiphyseal plate.

Objective:To evaluate the feasibility of CT volume rendering technology in the assessment of the physeal bar in children.Methods:A retrospective analysis of the relevant CT data of 20 patients with physeal bar from January 2019 to December 2021, 13 boys and 7 girls, age 9.94±2.91 years. The etiology included 17 cases of trauma, 1 cases of tumor, and 2 cases of unknown. The affected sites included 9 cases of distal femur, 5 cases of proximal tibia, 3 cases of distal tibia, and 3 cases of distal radius. Evaluate using volume rendering technology and traditional surface reconstruction technology respectively and the index include the size and type of physeal bar.Results:All the children were successfully examined at one time, and none of them required sedation. The average exposure time was 3.81±0.83 s. During scanning, only low-dose radiation was performed on the affected joints, and routine radiation protection was performed. Using volume rendering technology to evaluate the proportion of the physeal bar, the results obtained by the three testers were 25.36%±15.36%, 24.75%±16.18%, 26.70%±17.72%, and the intraclass correlation coefficient value ( ICC) was 0.976, the three repeated measurements by one tester were 25.36%±15.36%, 25.41%±15.20%, 25.74%±16.00% ( ICC=0.990). Compared with the traditional curved planar reconstruction technical evaluation, the results obtained by the three testers were 28.36%±16.74%, 23.66%±19.87%, 35.25%±15.92% ( ICC=0.737), the three repeated measurement results by one tester were 28.36%±16.74%, 31.66%±13.06%, 30.89%±12.52% ( ICC=0.875). The volume rendering technology was better than the curved planar reconstruction technique. Paired t test was performed on the measurement results of three evaluators and the three repeated measurements of the same evaluator, and the differences were statistically significant ( P<0.05). The same results were acquired by using volume rendering technology to evaluate the type of phseal bar with three testers; but there were 18 cases had the same results by using curved planar reconstruction technology and 2 cases had differences, showing the volume rendering technology was more accurate. Conclusion:Low-dose CT scanning volume rendering technology is an effective method to evaluate the physeal bar in children. It can more intuitively and accurately evaluate the proportion and type of the bar while reducing the radiation exposure of children. The consistency with inter-group and intra-group is better than traditional curved planar reconstruction technique with good reproducibility and clinical significance.

Objective:Cushing′s disease(CD) is caused by the pituitary adrenocorticotroph hormone(ACTH) secreting adenomas, leading to increased serum cortisol levels and various abnormal metabolic processes. Untreated CD is linked to high mortality, thus it is critical to elucidate its pathogenesis. This study aims to explore the pathogenesis of pituitary ACTH adenomas using whole-genome sequencing analysis.Methods:Fresh tumor tissues and peripheral blood samples were collected in 9 confirmed cases of pituitary ACTH adenomas who underwent surgery. Whole genome sequencing was then performed, followed by analysis and verification of single nucleotide mutations, copy number variation(CNV) and chromosome structure variations.Results:Somatic USP8 mutations(p.Ser718del, p. Ser718Pro, p. Pro720Arg, p. Pro720Gln) were found in 5 patients, with a rate of 55.6%; CNV of USP8 was detected in 1 patient; TP53(p.Cys135Tyr), NF1(p.Val1049Glufs*11) and KMT2C(c.3323+ 1G>A) mutations were identified in 1 patient harboring wild-type USP8. CNV analysis showed a loss of heterozygosity in multiple chromosomes in a wild-type USP8 patient. Structural variations were found in 2 with unknown significance. No germline gene mutations were detected in this study.Conclusion:Somatic USP8 mutations, increased copy number of USP8, variations of tumor-related genes such as TP53 and extensive somatic CNV all contribute to pathogenesis of CD. Chromosomal structure variations may suggest high-risk pituitary ACTH adenomas, and call for frequent follow-up and aggressive treatment.

OBJECTIVE: Psoriasis is a common chronic inflammatory skin disease that is prone to recurrence, and the proinflammatory factor, cysteine-rich protein 61 (Cyr61), is important in its pathophysiology. Long-term clinical practice has shown that Sancao Formula (SC), a Chinese herbal compound, is effective in the treatment of psoriasis, but the precise mechanism remains unknown. In this study, we investigate the mechanism by which SC extract alleviates imiquimod (IMQ)-induced psoriasis. METHODS: The expression of Cyr61 in psoriatic lesions and normal healthy skin was detected using immunohistochemical analysis to investigate the biological role of Cyr61 in models of psoriatic inflammation. A psoriatic mouse model was established by topical application of IMQ, and the effect of topical application of SC extract was evaluated using the psoriasis area and severity index (PASI) score, hematoxylin-eosin staining, and histopathological features of the skin. Next, a HaCaT cell inflammation model was established using interferon-γ (IFN-γ), and the effect of SC extract on the mRNA and protein levels of Cyr61 and intercellular cell adhesion molecule-1 (ICAM-1) was confirmed using Western blot and quantitative real-time polymerase chain reaction analyses. RESULTS: Immunohistochemical staining showed that the expression of Cyr61 in psoriatic lesions was higher than that in normal skin samples (78.26% vs 41.18%, P < 0.05), and the number of Cyr61-positive cells in psoriatic lesions was also significantly higher than in normal skin (18.66 ± 2.51 vs 4.33 ± 1.52, P < 0.05). Treatment in mice with IMQ-induced psoriasis showed that SC extract could significantly improve the inflammatory phenotype, PASI score (10.875 ± 0.744 vs 3.875 ± 0.582, P < 0.05), and pathological features compared with those in IMQ model group; SC treatment was also associated with decreased levels of Cyr61 and ICAM-1. In the IFN-γ-induced inflammatory cell model, the mRNA and protein levels of Cyr61 and ICAM-1 were upregulated, while the SC extract downregulated the levels of Cyr61 and ICAM-1. CONCLUSION The results provide a theoretical basis for the involvement of Cyr61 in the pathogenesis of psoriasis, and suggest that SC should be used to target Cyr61 for the prevention of psoriasis recurrence.
Animals ; China ; Cysteine-Rich Protein 61/metabolism* ; Disease Models, Animal ; Drugs, Chinese Herbal/therapeutic use* ; Imiquimod/adverse effects* ; Inflammation/drug therapy* ; Intercellular Adhesion Molecule-1/genetics* ; Interferon-gamma ; Mice ; Mice, Inbred BALB C ; Psoriasis/pathology* ; RNA, Messenger/therapeutic use*