A retrospective analysis of the clinical data of seven acute B-lymphoblastic leukemia (B-ALL) patients with TCF3-HLF fusion gene-positive admitted to the First Affiliated Hospital of Soochow University from June 2017 to August 2022 was conducted to summarize their clinical features and prognoses. The seven B-ALL patients comprised four males and three females, with a median age of 18 (11-33) years. Five patients tested positive for CD33 expression, and four patients had a normal karyotype. Two patients had hypercalcemia at the initial diagnosis, and one patient developed hypercalcemia at relapse. Six patients presented with coagulation dysfunction at diagnosis. After induction chemotherapy, five out of seven patients achieved complete remission, of which four subsequently relapsed. Two patients did not achieve remission even after two rounds of induction chemotherapy, with one achieving complete remission after treatment with blinatumomab immunotherapy. Three patients underwent chimeric antigen receptor T cell therapy, whereas three patients subsequently underwent hematopoietic stem cell transplantation. Five patients died, while two patients survived with sustained complete remission. TCF3-HLF-positive B-ALL is rare and has a high relapse rate and poor prognosis.

Objective:To investigate the accuracy of magnetic resonance imaging (MRI) cine sequences in determining the range of tumor motion in radiotherapy, providing a basis for the precise delineation of the target volume in motion for radiation therapy.Methods:A modified chest motion phantom was placed in a MRI scanner, and a water-filled sphere was used to simulate a tumor. True fast imaging with steady precession (TrueFISP) MRI cine sequences from Siemens were used to capture the two-dimensional motion images of the simulated tumor. The phantom experiments were divided into three modes: head-foot motion mode, rotation motion mode, and actual respiratory waveform mode. In the head-foot motion mode, respiratory motion period (3, 4, 5, 6, 7 and 8 s), amplitude (5, 10 and 15 mm), and respiratory waveform of the simulated tumor (sin and cos4) were set, resulting in a total of 36 motion combinations. In the rotation motion mode, a cos4 waveform was used for respiration, with respiratory periods of 3, 4, 5, 6, 7 and 8 s, head-foot motion set amplitudes of 5, 10 and 15 mm, and anterior-posterior (AP) and left-right (LR) motion set amplitudes in three combinations ([2.5, 2.5] mm, [2.5, 5.0] mm, [5.0, 5.0] mm), resulting in a total of 54 motion combinations. In the actual respiratory waveform mode, respiratory waveforms of 5 randomly selected patients from Affiliated Cancer Hospital of Zhengzhou University were obtained. Under each motion combination, TrueFISP cine images (30 frames, with an acquisition time of 11 s per frame) were obtained. The code was used to automatically identify the two-dimensional coordinates of the center of the simulated tumor in each image, and sin and cos4 functions were separately employed to fit the tumor position in the motion direction, thereby obtaining the fitted motion period and amplitude. The difference between the maximum and minimum values of the tumor's center coordinates in the head-to-foot direction is taken as the range of movement, referred to as the calculated amplitude. For the actual respiratory waveform, the distance between the measured maximum and minimum positions is used to calculate the amplitude.Results:In the head-foot motion mode, the fitted amplitudes of both sin and cos4 waveforms deviated from the set amplitudes by 0-0.51 mm, with relative deviations of 0%-4.2%. The deviation range between the calculated amplitudes and the set amplitudes of the two waveforms were 0.08-0.94 mm, with relative deviations of 1.1%-6.3%. In the rotation motion mode, the fitted amplitudes deviated from the set amplitudes by 0-0.61 mm, with relative deviations of 0%-6.2%. And the deviation range between the calculated amplitudes and the set amplitudes were 0.16-0.94 mm, with relative deviations of 0%-6.3%. In the actual respiratory waveform motion mode, the deviation range between the calculated amplitudes and the set amplitudes were 0.10-0.48 mm, with relative deviations of 2.2%-8.6%.Conclusion:TrueFISP cine sequences show minimal deviations in determining the range of tumor head-foot motion and effectively captures the tumor's movement state, thereby providing important support for the precise definition of the tumor movement target area during radiotherapy .

Objective:To explore the clinical value of recurrent laryngeal nerve dissection in the surgical treatment for congenital pyriform sinus fistula（CPSF）. Methods:The clinical data of 42 patients with CPSF were retrospectively analyzed. All patients were diagnosed and treated in the First Affiliated Hospital of Guangdong Pharmaceutical University. Results:During the operation, all patients' recurrent laryngeal nerves were dissected successfully, and fistulas were resected completely,no patients had complication of recurrent laryngeal nerve's damage.There were no recurrence cases during the 13 to 48 months of follow-up. Conclusion:The trend of congenital pyriform sinus fistula is closely related to recurrent laryngeal nerve, it's important to dissect the recurrent laryngeal nerve during the operation for congenital pyriform sinus fistula.
Humans ; Neck ; Recurrent Laryngeal Nerve/surgery* ; Retrospective Studies ; Pyriform Sinus/surgery* ; Fistula/surgery*

As conserved enzymes with important functions, aminoacyl-tRNA synthetase are expressed ubiquitously in cells. These include cytoplasmic aminoacyl-tRNA synthetase, mitochondrial aminoacyl-tRNA synthetase and bifunctional aminoacyl-tRNA synthetase. Mitochondrial aminoacyl-tRNA synthetases catalyze the binding of amino acids with its corresponding tRNA in the mitochondria and participate in the translation of 13 subunits of oxidative phosphorylation enzyme complexes encoded by the mitochondrial genome. Mutations in genes encoding mitochondrial aminoacyl-tRNA synthase may cause a variety of genetic disorders. This review has summarized the clinical characteristics, molecular pathogenesis and treatment of genetic diseases caused by mutations of such genes.
Humans ; RNA, Transfer, Amino Acyl ; Genes, Mitochondrial ; Amino Acyl-tRNA Synthetases/genetics* ; Genome, Mitochondrial ; Mitochondria/genetics*

Recent studies have found a special class of macrophages, muscularis macrophages (MMs), in the gastrointestinal tract, which interacts with enteric neuron (EN), interstitial cells of Cajal (ICC), and smooth muscle cells (SMC) to maintain normal intestinal motility. MMs can undergo phenotypic and other changes under altered intestinal microbiota, inflammation, or stress, and act on EN, ICC, or SMC through multiple mechanisms, ultimately affecting gastrointestinal motility. This article reviewed the mechanism of gastrointestinal MMs regulating gastrointestinal motility and role in gastrointestinal motility disorder diseases.

Objective:To discuss a surgical method that reduces the notch tension and assists in suspending and lifting the skin after reposition so as to relieve scar and to prevent alopecia and maintain long-term lifting effect.Methods:After forehead or temporal open incision rhytidectomy, 2-0 absorbable sutures were used to pass through the intradermal at the hairline and skin junction in front of the incision. After that, the sewing needle passed through the whole skin flap vertically from the two points of the suture inlet and outlet, and then sutured on the deep temporal fascia or galea aponeurotica, in order to reduce tension of the main anchor point of the incision and to assist suspending the elevated flap. Afterwards, the width of the skin excision was determined according to the skin relaxation, the subcutaneous relaxation suture of scalp incision was conducted and the incision closed with nailing device.Results:From June 2016 to October 2018, this method was applied in 33 cases of rhytidectomy. In the early postoperative period, the skin around the hairline had a slight appearance of accumulation due to the traction of sutures, which gradually disappeared with time. During the long-term follow-up, 2 patients had hair loss near the incision, and the hair density decreased. The remaining patients had no obvious scar hyperplasia and alopecia, and no long-term skin accumulation. The facial rejuvenation effect was good, and the satisfactory rate of patients was high.Conclusions:The technique of intradermal tension reduction combined with suspension suture redistributes the tension and significantly reduces the scar hyperplasia and baldness at the incision. Meanwhile, the suture also plays the role of accessory suspension, which helps to increase the stability and durability of facial tissue; the whole procedure is simple, safe, and long lasting.

Objective:To explore the method and effect of preventing eyelid ectropion when using expanded skin flap to repair periorbital soft tissue defect.Methods:From April 2015 to December 2019, 176 patients with periorbital soft-tissue defect were treated in the Plastic Surgery Hospital of Chinese Academy of Medical Sciences, including 79 males and 97 females, aged 4-53 years, the defect ranging from 1 cm×1 cm-6 cm×10 cm. According to the location and size of the defect, an appropriate expander was selected before the operation. In the first stage, the expander was embedded, and saline was injected regularly. In the second stage, the expanded flap was transferred to cover the periorbital defect wound, part of the capsule was removed, and the flap was fixed on the periosteum to restore the position and shape of the eyelid and prevent eyelid ectropion. After the operation, elastic headgear was worn for 3-6 months for shaping. The survival and complications of the flaps were followed up.Results:The expander volume was 30-400 ml, and the expansion time was 1-6 months. The size of the expanded flap was 3 cm×3 cm-6 cm×9 cm. All flaps survived after 2 months to 4 years follow-up. Among them, 13 cases had mild lagophthalmus, which was caused by insufficient skin expansion and did not affect the eyelid function, so the patients refused to retreat; 8 cases of lower eyelid ectropion, including 5 cases of scar contracture, 1 case of suture knot falling off, and 2 cases of lower eyelid support tissue relaxation caused by long-term stretching of expander, 5 cases were corrected after skin flap repair, 3 cases were not treated again; the rest were satisfactory. The depression left by the suture fixing flap disappears within 1 to 6 months after surgery.Conclusions:When using an expanded skin flap to repair periorbital soft-tissue defect, preoperative, intraoperative and postoperative interventions were implemented to reduce or disperse the retracting force of expanded skin flap, which could avoid eyelid ectropion and achieve good repair effect.

Sunken upper eyelid is a common eye defect, which seriously affects eye aesthetics and it has become an important issue of periorbital rejuvenation in recent years. This article introduces the mechanism, classification and degree of depression regarding sunken upper eyelid. The correction method of sunken upper eyelid, their indications, postoperative effects as well as the pros and cons of each method were comprehensively summarized and compared, trying to find appropriate corrective method according to the causes and degree of different sunken upper eyelid among patients.

Objective:To study the clinical effect of Microbotox in improving mandibular fat accumulation, lifting mandibular contour and achieving lower face and neck rejuvenation.Methods:From May 2020 to January 2021, patients with mandibular fat accumulation and skin relaxation of mandible and neck were treated in Plastic Surgery Hospital, Chinese Academy of Medical Sciences. Multiple microdroplets of botulinum toxin type A was injected into the dermis or the interface between the dermis and the superficial muscle layer in lower face and neck. Botulinum toxin was diluted to 20 U/ml and injected with 0.6 U per point. The scope of Microbotox was demarcated by the line between earlobe-mouth corner as the upper border, the depressor anguli oris as the anterior border, the front edge of sternocleidomastoid muscle front edge as the posterior border, and the jugular notch as the inferior border. Treatment effect was evaluated postoperatively by the subjective satisfaction of patients and the accessment of a third-party physicians.Results:A total of 25 patients were included, including 10 males and 15 females, with the age of 39.4±3.7 years. The patient subjective satisfaction was 96%(24/25), and 96%(24/25) of patients received improved result , evaluated by a third-party physician. There were no serious complications except one case of local congestion and four cases of local pain and swelling, which could be relieved in a short time.Conclusions:Characterized by improving mandibular fat accumulation, lifting mandibular contour, reducing cervicomental angle, improving skin texture and neck wrinkles, Microbotox is effective and practical.

Objective:To explore the method and effect of preventing eyelid ectropion when using expanded skin flap to repair periorbital soft tissue defect.Methods:From April 2015 to December 2019, 176 patients with periorbital soft-tissue defect were treated in the Plastic Surgery Hospital of Chinese Academy of Medical Sciences, including 79 males and 97 females, aged 4-53 years, the defect ranging from 1 cm×1 cm-6 cm×10 cm. According to the location and size of the defect, an appropriate expander was selected before the operation. In the first stage, the expander was embedded, and saline was injected regularly. In the second stage, the expanded flap was transferred to cover the periorbital defect wound, part of the capsule was removed, and the flap was fixed on the periosteum to restore the position and shape of the eyelid and prevent eyelid ectropion. After the operation, elastic headgear was worn for 3-6 months for shaping. The survival and complications of the flaps were followed up.Results:The expander volume was 30-400 ml, and the expansion time was 1-6 months. The size of the expanded flap was 3 cm×3 cm-6 cm×9 cm. All flaps survived after 2 months to 4 years follow-up. Among them, 13 cases had mild lagophthalmus, which was caused by insufficient skin expansion and did not affect the eyelid function, so the patients refused to retreat; 8 cases of lower eyelid ectropion, including 5 cases of scar contracture, 1 case of suture knot falling off, and 2 cases of lower eyelid support tissue relaxation caused by long-term stretching of expander, 5 cases were corrected after skin flap repair, 3 cases were not treated again; the rest were satisfactory. The depression left by the suture fixing flap disappears within 1 to 6 months after surgery.Conclusions:When using an expanded skin flap to repair periorbital soft-tissue defect, preoperative, intraoperative and postoperative interventions were implemented to reduce or disperse the retracting force of expanded skin flap, which could avoid eyelid ectropion and achieve good repair effect.