Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.

Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.

OBJECTIVETo explore the efficacy of partial resection of puborectalis combined with mutilation of internal anal sphincter(IAS) in the treatment of puborectalis syndrome with high anal pressure.

METHODSTwenty-five cases of puborectalis syndrome with high anal resting pressure in the preoperative examination received the operation of partial resection of puborectalis combined with mutilation of IAS in Zhongnan Hospital of Wuhan University between January 2013 and May 2015. The position of puborectalis was confirmed by touching with the exposure under the transfixion device, and a transverse incision was made by electrotome between 3 and 5 o'clock direction of puborectalis, then partial puborectalis was lifted by vessel clamp at 5 o'clock direction, and about 0.5 cm of muscular tissue was resected. Between 8 to 10 o'clock direction of anal tube, about 1 cm length of transverse incision was made by electrotome, then partial IAS was lifted by vessel clamp and cut off. Preoperative and postoperative 3-month anorectal manometry and defecography were carried out. Wexner constipation score and Cleveland Clinic incontinence score were implemented before surgery and 3, 6, 12 months after operation. This study was registered in the Chinese Clinical Trial Registry (registration number: ChiCTR-ORB-16007695).

RESULTSOf the 25 cases, 18 were male and 7 were female, the average age was 55 years old and the average course of disease was 9 years. Compared with pre-operation, the postoperative 3-month anal resting pressure and maximal squeeze pressure were significantly decreased [(53.56±9.05) mmHg vs. (92.44±7.06) mmHg, (142.80±20.35) mmHg vs. (210.88±20.56) mmHg, respectively, both P=0.000]; anorectal angulation at resting state and forced defecation state increased significantly [(102.32±4.96)degree vs. (95.88±4.01)degree, (117.88±5.95)degree vs. (89.52±3.25)degree, respectively, both P=0.000]. Wexner constipation score of postoperative 3-month, 6-month, 12-month (8.28±3.91, 7.40±3.64 and 8.04±4.74) was significantly lower than the preoperative score (16.00±3.69, all P<0.05), while the score was not significantly different among 3 time points after operation (P>0.05). Cleveland Clinic incontinence score was 0 at postoperative 6 and 12 months, and revealed 20 cases were effective among all the surgical patients(80%).

CONCLUSIONPartial resection of puborectalis combined with mutilation of internal anal sphincter can effectively reduce anal pressure and improve symptoms of outlet obstruction, which is an effective method in the treatment of puborectalis syndrome with high anal pressure.

Anal Canal ; physiopathology ; surgery ; Constipation ; surgery ; Defecation ; Defecography ; Digestive System Surgical Procedures ; methods ; Female ; Gastrointestinal Diseases ; surgery ; Humans ; Male ; Manometry ; Middle Aged ; Muscle Hypertonia ; surgery ; Pelvic Floor ; physiopathology ; surgery ; Pressure ; Treatment Outcome

OBJECTIVETo evaluate the clinical outcomes of ileal D-pouch anal anastomosis in the treatment of ulcerative colitis (UC) and familial adenomatous polyposis (FAP).

METHODSClinical and follow-up data of 6 UC patients and 5 FAP patients undergoing proctocolectomy and D-ileum pouch anal anastomosis between October 2014 and March 2015 were retrospectively analyzed. End-to-side anastomosis was firstly performed in ileal cutting end and ileum, then side-to-side anastomosis was operated in closing amphi-loop to construct the D-ileum pouch.

RESULTSThe mean age of the patients was 42 years (range 18 to 61 years), 5 patients were female. The duration of surgery was (225±23) min, the operation time to D-ileum pouch was (18±4) min, the volume of D-ileum pouch was (175±15) ml, the blood loss was (110±30) ml. There was no procedure-related death, however rectum perforation occurred in 1 male UC patient during operation. The postoperative hospital stay was 8 to 11 days (mean 8.5 days). The follow-up period was 2 to 7 months (median 3 months). One female FAP patient suffered from anal anastomosis vagina fistula 21 days after operation. No pouch-related fistula, anastomosis or input loop stricture fecal incontinence, and severe pouchitis were recorded. The defecation frequency was 4 to 6 times per day (UC) and 3 to 5 times per day (FAP) 6 months after operation. No night-time fecal leakage was complained in those patients. Wexner incontinence score was 3±2 and GQLI was 114±11 one month after operation. Clinical outcome in the first month was excellent in 10 patients and good in 1 patient.

CONCLUSIONSAfter total colorectal resection for UC and FAP patients, application of D-ileum pouch can clear ileal stump of pouch and avoid the pouch-associated complications effectively.

Adenomatous Polyposis Coli ; Adolescent ; Adult ; Anastomosis, Surgical ; Colitis, Ulcerative ; Colonic Pouches ; Fecal Incontinence ; Female ; Humans ; Ileum ; Male ; Middle Aged ; Pouchitis ; Proctocolectomy, Restorative ; Rectal Fistula ; Retrospective Studies ; Vagina ; Young Adult

Objective To investigate the diagnosis and treatment of malignant fibrous histiocytoma (MFH) in abdominal cavity. Methods The clinical manifestations, imaging examination, pathology, methodology, effect of treatment and prognosis of 28 MFH patients were retrospectively analyzed. Results Tumors were in the retroperitoneal space in 17 cases, within abdominal cavity in 6, in mesentery in 3 cases, and in the abdominal wall in 2 cases. The clinical symptoms were body weight loss, abdominal pain and mass. B ultrasonography and CT scanning showed mass in the abdomen. 24 cases were with multiplefoci,4 cases were with a single mass. Seventeen cases underwent complete tumor resection,11 cases with partial resection, 16 cases received postoperative chemotherapy and 14 cases with postoperative radiotherapy. The overall survival rate of 1-, 3- and 5- year was 76.9%,26.9% and 3.9%,respectively. Conclusion MFH is most often located in retroperitoneal space, surgical resection was the treatment of choice. The prognosis in patients with MFH might be improved by complete resection combined with chemotherapy or/ and radiotherapy.