Objective To observe the natural outcomes of Graf type Ⅱ a hip dysplasia aged 6 weeks to 3 months,and to explore the indications of treatment.Methods A prospective study was conducted to collect children aged from 6 to 12 weeks with Graf type Ⅱ a dysplasia (50°≤α angle＜60°) but clinically stable hips according to the Early Screening of Developmental Dysplasia of the Hip in Tianjin project from July 2012 to July 2014.Those hips with history of treatment or neuromuscular disorders were excluded.All infants had no treatment initially following with ultrasound surveillance each 2 weeks and radiograph evaluation each at 3,4.5 and 6 months of age.Patients received Pavlik harness or abduction brace treatment if hip dysplasia turned to be type Graf Ⅱ c or worse,or clinical unstable before 6 months;also if hip dysplasia was persistent in radiograph at 6 months.All infants were routinely followed up at 12 and 24 months of age to detect the late cases.The difference of initial α angle,hip abduction,acetabulum index and acetabulum margin morphology (sharp,round or defect) in the pelvic radiograph at 3 month were compared between the treated and untreated groups.Results A total of 238 children (285 hips) were enrolled in the present study,of which there were 25 males and 213 females,193 left and 92 right hips.The average age was 9 weeks (range 6 to 12 weeks).No hip turned to be type Ⅱ c or worse,or clinically unstable.One hundred patients (120 hips) received treatment for persistent dysplasia at 6 months.There had statistically significant difference in initial α angle between the treated and untreated groups (respectively 54.5°±3.3° and 55.6°±2.9°,t=-2.749,P=0.004).In the treated group,there were 47 hips (39.2％,47/120) with limited abduction initially,and 52 hips (43.3％,52/120) with poor acetabular morphology at 3 months.The differences were statistically significant comparing with the untreated group (x2=4.010,P=0.045;x2=14.143,P=0.000).Logistic multivariate regression analysis showed that patients with Graf Ⅱ a-hips (OR=2.908) and poor acetabular morphology hips (OR=2.822) were more likely to receive treatment.Thirty-eight patients (47 hips) received treatment among Graf Ⅱ a+ hips (α angle ≥55°),of which 21 hips (44.7％,21/47)had limited abduction and 31 hips (66.0％,31/47) poor acetabular morphology.The differences were statistically significant comparing with untreated cases (x2=12.073,P=0.001;x2=35.879,P=0.000).Logistic multivariate regression analysis showed that Graf Ⅱ a+ hips with limited hip abduction (OR=4.145) and poor acetabular morphology (OR=10.117) were more likely to receive treatment.Conclusion Graf Ⅱ a dysplasia with clinically stable hips should be treated if α angle ＜55° at 6 weeks or if α angle ≥55° with limited hip abduction or poor acetabular morphology (round or defect) at 3 months.

Objective To evaluate the outcome of Titanium Elatic Intramedullary Nail (TEINs) for treatment of lengthunstable femoral shaft fracture of children.Methods Retrospectively analyze the data of total of 21 children with length-unstable femoral shaft fractures,from December 2011 to June 2014,were treated with TEINs and end caps.This study involved 15 boys and 6 girls,with age ranged from 5 years and 6 months to 10 years and 10 months,whose average age was 7 years and 5 months.Firstly children were treated with traction for 3-7 days,with traction weight of 1/4 to 1/6 of the weight.After that,shortening and angular deformity were significantly corrected.Then surgeries of close reduction and internal fixation with TEINs were done in general anesthesia.TEINs were supported with double nail C shape.The lateral tip of TEIN located near the epiphyseal plate of greater trochanter,and the medial tip near the epiphyseal plate of femur head.Cut the tail of TEINs,set and screw the end caps into cortex,keeping the tail of TEINs and fracture fixed.Spica casting was used for 4 weeks after operation.The last follow-up time was 2 years,and the follow up results were analyzed.Results 21 children were followed up ranged from 22 to 26 months,with the mean follow up 23.4 months.Remove the spica casting and bear loading at 4 weeks after operation.All fractures healed,with healing time of 5 to 8 weeks (an average of 6.2 weeks).At the last follow-up,the limb force line was good with no angular deformity.Femur overgrew 4.2-21.1 mm,with an average of 6.1 mm.At the last follow-up,the patients were evaluated with Harris score,and the excellent and good rate was 100％.No case of TEINs push-out,limited hip mobility,fracture nonunion,delayed healing or other complications was found.Only 1 case got a mild lameness.All parents were satisfied with their outcome.Conclution TEINs and end caps are effective methods for the length-unstable femoral shaft fracture in children.These results indicated that the risk of TEINs push-out can be reduced.

Objective To compare the early treatment results of Pavlik harness and closed reduction plus spica cast?ing in treating developmental dislocation of the hips (DDH). Methods The patients with GrafⅢ/Ⅳtype DDH diagnosed by ul?trasound in our hospital were analyzed retrospectively from January 2009 to December 2013. The subjects with intact clinical and radiologic data and more than 2 years follow?up were included. The present study included 257 patients (298 hips), 31 male and 226 female, with the average age of 74.1±39.2 d (from 30 to 159 d). Pavlik harness underwent in 190, while 67 cases were treated with closed reduction plus spica casting. Acetabular index (AI), Wiberg OE angle (point O was the middle point of proximal me?taphyseal border) and Smith instability index (SI, SI?c:the lateral displacement index, SI?h:the vertical displacement index) on the pelvic X?ray film at 2-3 years of age after successful early treatment were recorded. Femoral head avascular necrosis (AVN) was diagnosed according to the Salter criteria. Results The success rate of reduction of closed reduction was significantly higher than Pavlik harness (97.5% vs. 69.9%, χ2=0.353, P=0.000). There was no significant difference in the incidence of AVN be?tween the closed reduction and Pavlik harness (6.6% vs. 4.6%, χ2=0.106, P=0.745). For GrafⅢ type DDH, the success rate of reduction of Pavlik harness and closed reduction were 80.7% and 98.4% respectively (χ2=11.248, P=0.001), while the Pavlik harness group had significantly worse results of AI, OE and SI?c at the age of 2-3 years (Pavlik harness: 22.9°±3.7°, 16.5°± 6.3° and 0.74 ± 0.06; closed reduction: 21.4° ± 3.4° , 18.9° ± 3.6° and 0.72 ± 0.03; P<0.05). For GrafⅣ type DDH, the success rate of reduction of Pavlik harness was much less than closed reduction (25.6% vs. 94.4%, χ2=24.231, P=0.001), with signifi?cantly worse results of OE at the age of 2-3 years (16.0°±4.1° vs. 18.6°±4.5°, t=-2.141, P=0.038). Conclusion Pavlik har?ness has limitations in treating GrafⅢ/Ⅳtype DDH, with lower success rate of reduction for GrafⅣtype DDH. Closed reduction plus spica casting has better clinical results than Pavlik harness in success rate of reduction and subsequent hip development with? out the additional risk of AVN.

Objective: To explore the changes in ribosomal DNA copy number in peripheral blood among patients with pneumoconiosis and its influencing factors, so as to provide insights into prevention and treatment of pneumoconiosis. Methods: Eighty-eight patients with pneumoconiosis who visited a designated hospital and 71 community residents with no history of pneumoconiosis or dust exposure were selected as the pneumoconiosis group and control group, and age, smoking history, drinking history and cumulative years of exposure to dust were collected through questionnaire surveys. The copy number of 45S rDNA and 5S rDNA was detected using real-time fluorescence quantitative PCR, and the differences between the two groups were compared. Factors affecting the copy number of 45S rDNA and 5S rDNA were identified by a multiple linear regression model. Results: The pneumoconiosis group had a median age of 56.00 (interquartile range, 15.25) and a mean cumulative dust exposure duration of (12.40±8.08) years, with 56.82% smoking and 62.50% drinking. The control group had a median age of 64.00 (interquartile range, 37.00) years, with 32.39% smoking and 26.76% drinking. The median copy number of 45S rDNA in the pneumoconiosis group was 1.29 (interquartile range, 0.59), which was lower than 2.10 (interquartile range, 1.88) in the control group; the median copy number of 5S rDNA in the pneumoconiosis group was 5.33 (interquartile range, 0.85), which was higher than 4.66 (1.34) in the control group (both P<0.05). Multiple linear regression analysis identified age (β=-0.034) and pneumoconiosis (β=-1.595) as factors affecting 45S rDNA copy number, age (β=-0.013) as a factor affecting 5S rDNA copy number, and age (β=0.018) as a factor affecting 5S rDNA copy number in the pneumoconiosis group (all P<0.05). Conclusions Compared with community residents with no history of pneumoconiosis or dust exposure, the copy number of 45S rDNA in peripheral blood among patients with pneumoconiosis is reduced and the copy number of 5S rDNA is increased.

Objective:To analyze the risk factors of avascular necrosis (AVN) after closed reduction and spcia casting in treating developmental dysplaisa of the hip (DDH).Methods:The patients with DDH who received closed reduction in our department from January 2016 to December 2017 were retrospectively reviewed. The inclusion criteria included aged at reduction ≥6 months, achieving successful reduction, having complete data with more than 2 years follow-up. A total of 48 cases with 54 hips were included in the present study. Among them, there were 2 males and 46 females, 41 unilateral hips and 13 bilateral hips. The mean age at closed reduction was 16.4±3.8 months (range 6-24 months). The mean follow-up duration was 2.9±1.8 years (range 2.3-4.1 years). Closed reduction was conducted under general anesthesia followed with a spcia cast immobilization. The abduction angle of the cast was recorded. The stability of reduction was evaluated by Ramsey safety zone. The maximum abduction and re-dislocation abduction were recorded. The quality of reduction was evaluated by the medial gap and femoral head coverage on intraoperative arthrography and post-reduction MRI. AVN was diagnosed according to Salter criteria. The risk factors of AVN were analyzed by univariate and binary logistic regression analysis.Results:AVN occurred in 12 hips (22.2%) of 54 hips. International Hip Dysplasia Institute (IHDI) grade and the difference between maximum abduction and cast abduction (Max-Cast abduction) were related to the occurrence of AVN in univariate analysis. The incidence of AVN in hips of IHDI grade 4 (42.9%, 9/21) was significantly higher than that in hips of IHDI grade 3 (9.7%, 3/31) (χ 2=6.007, P=0.018). However, the hips of IHDI grade 3 and 2 (0%, 0/2) presented a similar incidence of AVN (χ 2=0.000, P=1.000). The Max-Cast abduction was -0.7°±5.9° in the AVN group and 6.1°±7.6° in the AVN group ( t=2.125, P=0.038). Finally, IHDI grade ( OR=8.256, P=0.015) and Max-Cast abduction ( OR=0.832, P=0.047) were both independent factors of AVN in multivariate analysis. Conclusion:Most of the hips with AVN are IHDI grade 4 after closed reduction for DDH. The abduction angle in a spica cast could not be significantly related to the occurrence of AVN. However, the risk of AVN might be increased when the cast abduction is close to or beyond the maximum abduction. Safe abduction in the cast should be 5 to 10 degrees less than maximum abduction at least.

Objective:Course of evolution observed relatively early Perthes disease in children surgically treated and conservative, explore the significance of early surgical treatment of Perthes disease.Methods:From January 1997 to December 2017, 632 children with Perthes disease were admitted. According to the inclusion and exclusion criteria, a total of 67 children were included in this study. Surgical group: 35 cases, 32 males and 3 females, age ranged from 7.0 to 11.8 years, with an average 8.3±1.0 years old, 21 cases on the left side and 14 cases on the right side. Conservative group: 32 cases, 24 males and 8 females, age ranged from 7.1 to 12.0 years old, with an average of 8.4±1.4 years old, 22 cases on the left side and 10 cases on the right side. The pelvic orthosis and frog position were taken every 3 months, and the evolution of Perthes disease was evaluated by referring to the modified Waldenstr?m staging. In the series of observations, no modified Waldenstr?m IIb stage was found to skip the fragmentation stage. After the healing period, the pelvic orthosis and frog position were evaluated according to the Stulburg classification results, and the ratio of the width of the metaphyseal end of the affected side to the contralateral side and the height of the femoral head epiphysis were measured.Results:The time required to enter stage IIIa, surgical group: range from 0.1 to 1.5 years, with an average of 0.58±0.33 years, conservative group: range from 0.4 to 1.8 years, with an average of 0.96±0.30 years, it takes both to enter the repair phase The time difference was statistically significant ( t=5.259, P<0.05); children whose disease course skipped the fragmentation stage, surgical group: 11 cases (31%), conservative group: 5 cases (16%), the difference is statistically significant ( χ2=22.626, P<0.05). The ratio of the affected side to the uninfected side of the metaphysis: surgical group: range 101%-123%, with an average of 108.0%±6.0%. Conservative group: range 101%-148%, with an average of 115.8%±11.2%. The difference in the ratio between the affected side and the healthy side of the metaphysis was statistically significant ( t=3.450, P<0.05). The ratio of the affected side to the healthy side of the height of the femoral head epiphysis: surgical group: range 61%-96%, with an average of 82.5%±8.2%. Conservative group: range 33%-92%, with an average of 74.7%±1.3%. There was a statistically significant difference in the ratio of the height of the femoral head epiphysis between the affected side and the healthy side ( t=2.921, P<0.05). Stulberg classification, surgical group: Type I hip joints 29 hips, Type II hip joints 6 hips, Type III hips 0 hips; conservative group: Type I hips 16 hips, Type II hips 10 hips, Type III hips 6 hips. There was a statistically significant difference in the Stulberg classification after the two entered stage IV ( Z=3.386, P<0.05). Conclusion:Early surgery changed the natural evolution of Perthes disease. Early surgical treatment shortens the fragmentation period of Perthes disease, so that it enters the repair phase ear-lier, and has the opportunity to skip the late fragmentation period and change its natural course. After healing, the morphological change of the proximal femur is closer to normal.

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 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 genetic basis for a rare case of acute B-lymphocytic leukemia (B-ALL) with double Philadelphia chromosomes (Ph) and double derivative chromosome 9s [der(9)]. METHODS: A patient with double Ph and double der(9) B-ALL who presented at Shanghai Zhaxin Intergrated Traditional Chinese and Western Medicine Hospital in June 2020 was selected as the subject. Bone marrow morphology, flow cytometry, G-banding karyotyping, fluorescence in situ hybridization (FISH), genetic testing and chromosomal microarray analysis (CMA) were used to analyze bone marrow samples from the patient at various stages. RESULTS: At initial diagnosis, the patient's bone marrow morphology and flow immunotyping have both supported the diagnosis of B-ALL. G-banded karyotyping of the patient indicated double Ph, in addition with hyperdiploid chromosomes involving translocations between chromosomes 9 and 22. BCR-ABL1 fusion gene was positive. Genetic testing at the time of recurrence revealed presence of a heterozyous c.944C>T variant in the kinase region of the ABL1 gene. FISH showed a signal for ABL1-BCR fusion on both chromosome 9s. CMA showed that the mosaicism homozygosity ratio of chromosome 9 was about 40%, and the mosaicism duplication ratio of chromosome 22 was about 43%. CONCLUSION Since both der(9) homologs were seen in 40% of cells, the possible mechanism for the double der(9) in this patient may be similar to that of double Ph, which might have resulted from non-disjunction during mitosis in the Ph chromosome-positive cell clone.
Humans ; Philadelphia Chromosome ; In Situ Hybridization, Fluorescence/methods* ; China ; Chromosome Aberrations ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics* ; Translocation, Genetic ; Fusion Proteins, bcr-abl/genetics* ; Chromosomes, Human, Pair 9/genetics*

Objective:Comparing with the external fixator technique, investigate the clinical effect of fixator-assisted plating technique for treatment distal femoral valgus deformity in adolescent.Methods:A retrospective analysis of the relevant data of 22 patients (25 limbs) with acute correction of distal femoral valgus deformities from July 2015 to June 2019, according to the difference of the final fixation, they were divided two groups. The fixator-assisted plating group, including 8 patients (9 limbs), 5 boys and 3 girls, 5 left and 2 right, 1 bilateral, the physis were closed in 4 cases and opened in 4 cases, age 14.04±1.99 years (11.7-18 years). The external fixator group, including 14 patients (16 limbs), 6 boys and 8 girls, 8 left and 4 right, 2 bilateral, the physis were closed in 9 cases and opened in 5 cases, age 13.33±1.88 years (10.1-16.5 years). Measuring the mechanical axis deviation (MAD) and the mechanical lateral distal femur angle (mLDFA) in full length standing AP view X-ray of the lower limb pre and post operation. Recording the changed of limb length discrepancy (LLD) before and after surgery and the knee range of motion at 6 weeks, 3 months, 6 months and the last follow up post operation. The Kolcaba comfort status scale was used to evaluate the comfort of two groups at 2 weeks, 6 weeks, 3 months, 6 months and the last follow up. Meanwhile recorded the healing time of osteotomy sites and the complications.Results:The difference in general information between the two groups was not statistically significant. The fixator-assisted plating group followed up mean 26 months (range, 12-40 months), the healing time were 4.00±0.66 months, the mLDFA mean 73.33°±4.12° before surgery and 87.89°±1.69° after surgery and there was significant difference ( t=10.582, P<0.05). The external fixator group followed up mean 36 months (range, 22-42 months), the healing time were 4.00±0.66 months, the mLDFA mean 73.31°±3.95° before surgery and 87.31°±1.54° after surgery and there was significant difference ( t=14.118, P<0.05). The MAD were in the normal range in all patients after surgery, and there were no significant difference about healing time of the osteotomy sites and postoperative mLDFA between the two groups ( t=1.514, P=0.150; t=0.845, P=0.411). Comparing with the knee function, the fixator-assisted plating group was better at 6 weeks, 3 months, 6 months after surgery but there was no difference at the last follow up. Also, the fixator-assisted plating group felt more comfortable at 2 weeks, 6 weeks, 3 months, 6 months and there was no difference at the last follow up. All the patients with external fixator have mild pin sites infection and there were no obvious complications in patients with plate. Conclusion:The fixator-assisted plating technique can accurately correct the valgus deformity with satisfactory healing of the osteotomy in distal femoral compare with the external fixator technique, but the patients feel more comfortable and the knee joint function recovers faster and no risk of pin site infection, the clinical results was satisfactory with fewer complication.