Humans ; Acute Disease ; Pancreatitis/diagnosis* ; Follow-Up Studies ; Patient Discharge ; Disease Progression

Conventional periodontal regenerative surgery has limited effect on tooth with severe periodontitis-related alveolar bone defects. This article reported a case of regenerative treatment in severe distal-bone defect of mandibular first molar. The treatment involved applying 3D printing, advanced/injectable platelet-rich fibrin, and guided tissue-regeneration technology. After the operation, the periodontal clinical index significantly improved and the alveolar bone was well reconstructed.
Humans ; Platelet-Rich Fibrin ; Follow-Up Studies ; Digital Technology ; Furcation Defects/drug therapy* ; Periodontitis ; Guided Tissue Regeneration, Periodontal

OBJECTIVE: To evaluate the effect of uncemented total hip arthroplasty(THA) on treatment of traumatic arthritis caused by intramedullary nailing interfixation of intertrochanteric fractures. METHODS: Total of 22 patients treated with THA due to traumatic arthritis caused by intramedullary nailing interfixation of intertrochanteric fractures from January 2012 to January 2017 were studied retrospectively, including 10 males and 12 females with a mean age of (72.5±9.8) years old ranging from 61 to 84 years old. Initial internal fixation method:14 patients were treated with Gamma nails and 8 patients were treated wit PFNA.The time from internal fixation surgery to THA was 10 to 68 months with an average of (32.2±21.3) months.Harris scores of the hip joint before and after surgery were compared, and the position of the prosthesis through postoperative imaging at 3, 6, 12 months and the last follow-up were evaluated. RESULTS: One patient was died due to heart failure 1 year after operation. Two patients was died to advanced tumor 2 years after operation. The other 19 patients were followed up for 36 to 64 months with an average of (48.5±11.9) months. At final follow up, 14 patients regained the ability to walk independently, 4 patients needed support of a cane, 1 patient needed assistance of a walker. No serious complications such as joint dislocation, periprosthetic fracture and deep venous thrombosis occurred during follow-up. There were no signs of loosening and subsidence of the prosthesis at the final follow-up. Mean Harris hip score increased from (29.2±12.9) points preoperatively to (74.2±11.2) points at the final follow up(P<0.05);the score was excellent in 9 patients, good in 7 and fair in 3. CONCLUSION Uncemented total hip arthroplasty for traumatic arthritis after intramedullary nail fixation of femoral intertrochanteric fracture can significantly improve hip function and effectively avoid bone cement implantation syndrome. The medium-term effect is satisfactory.
Male ; Female ; Humans ; Middle Aged ; Aged ; Aged, 80 and over ; Arthroplasty, Replacement, Hip/methods* ; Follow-Up Studies ; Treatment Outcome ; Retrospective Studies ; Bone Nails ; Hip Fractures/surgery* ; Fracture Fixation, Intramedullary/adverse effects* ; Arthritis/surgery*

OBJECTIVE: To evaluate the effect of denosumab on bone mineral density around proximal femoral prosthesis after total hip arthroplasty(THA) in the postmenopausal osteoporotic patients. METHODS: Fifty-four consecutive patients underwent unilateral primary THA were included in this retrospective study. Twenty-five patients received denosumab for osteoporosis as the treatment group, and the twenty-nine without denosumab were the control group. At 1 week, 3month, 6 months, and 12 months after THA, bone turnover markers and proximal femoral periprosthetic bone mineral density (BMD) were measured. RESULTS: At 3, 6 and 12 months after operation, the level of TRACP-5b in the control group was significantly higher than that in the treatment group (P<0.05);the level of bone-specific alkaline phosphatase (BALP) between two groups showed significant difference in 12 months after operation (control group was higher than treatment group, P<0.05). The BMD of Gruen 1 and Gruen 7 decreased at 3, 6 and 12 months after operation compared with 1 week after operation. Comparing the treatment group and the control group, the differences of the the decrease of BMD in Gruen 1 and Gruen 7 were no significant at 3 months after surgery. In Gruen 1, Gruen 7 at 6 months after operation and Gruen 1, Gruen 7 at 12 months after operation, the decrease of BMD in the control group was significantly higher than that in the treatment group(P<0.05). It is suggested that desudumab could inhibit the loss of BMD after 6 months, and continuously show a protective effect on bone mass at 12 months after operation. CONCLUSION After THA in postmenopausal patients with osteoporotic femoral neck fracture, Desuzumab can reduce the loss of BMD around the proximal femoral prosthesis and effectively inhibit bone resorption.
Humans ; Arthroplasty, Replacement, Hip ; Bone Density ; Denosumab/therapeutic use* ; Retrospective Studies ; Postmenopause ; Absorptiometry, Photon ; Bone Remodeling ; Follow-Up Studies ; Hip Prosthesis

Hip firearm injuries are rare injuries that could lead to serious complications, such as posttraumatic hip arthritis and coloarticular fistula. We report a case of a 25-year-old male who sustained a pelvic injury caused by a single bullet which led to a bilateral acetabular fracture, concomitant with a colon injury treated on an emergency basis by a diverting colostomy; acetabular fractures were treated conservatively by traction. After the patient recovered from the abdominal injury, he was presented with bilateral hip pain and limited motion; plain radiographs showed bilateral hip arthritis with proximal migration of the femoral head and bilateral acetabular defect classified as Paprosky type ⅢA. Reconstruction of the hips was performed using the same technique: impaction bone grafting for acetabular defect reconstruction and a reversed hybrid total hip arthroplasty (THA) 6 months apart. The patient presented with loosening of the left THA acetabular cup 3 years later, which was revised; then he presented with a discharging sinus from the left THA with suspicion of coloarticular fistula, which was confirmed using CT with contrast material. A temporary colostomy and fistula excision were performed, and a cement spacer was applied to the hip. After clearing the infection, a final revision THA for the left hip was performed. Treating post-firearm hip arthritis by THA is challenging, especially in the situation of neglected cases with the presence of an acetabular defect. Concomitant intestinal injury increases the risk of infection with the possibility of coloarticular fistula formation, which could present later. Working with a multidisciplinary team is paramount.
Male ; Humans ; Adult ; Arthroplasty, Replacement, Hip ; Firearms ; Wounds, Gunshot/surgery* ; Acetabulum/injuries* ; Hip Fractures/surgery* ; Arthritis/surgery* ; Spinal Fractures/surgery* ; Reoperation ; Fistula/surgery* ; Treatment Outcome ; Follow-Up Studies ; Prosthesis Failure ; Retrospective Studies

OBJECTIVES: To investigate genotype-phenotype characteristics and long-term prognosis of neonatal carbamoyl phosphate synthetase 1 (CPS1) deficiency among children through newborn screening in Zhejiang province. METHODS: The clinical and follow-up data of children with CPS1 deficiency detected through neonatal screening and confirmed by tandem mass spectrometry and genetic testing in Zhejiang Province Newborn Disease Screening Center from September 2013 to August 2023 were retrospectively analyzed. RESULTS: A total of 4 056 755 newborns were screened and 6 cases of CPS1 deficiency were diagnosed through phenotypic and genetic testing. Ten different variations of CPS1 genewere identified in genetic testing, including 2 known pathogenic variations (c.2359C>T and c.1549+1G>T) and 8 unreported variations (c.3405-1G>T, c.2372C>T, c.1436C>T, c.2228T>C, c.2441G>A, c.3031G>A, c.3075T>C and c.390-403del). All patients had decreased citrulline levels (2.72-6.21 μmol/L), and varying degrees of elevated blood ammonia. The patients received restricted natural protein intake (special formula), arginine and supportive therapy after diagnosis, and were followed-up for a period ranging from 9 months to 10 years. Three patients experienced hyperammonemia, and one patient each had attention deficit hyperactivity disorder, transient facial twitching and increased muscle tone. One patient died, while the other five surviving patients had normal scores of the Ages & Stages Questionnaires (ASQ) and Griffiths Development Scales up to the present time; 4 cases had combined height or weight lag and one case was normal in height and weight. CONCLUSIONS Low citrulline levels and hyperammonemia are common in CPS1 deficiency patients in Zhejiang. Most gene variants identified were specific to individual families, and no hotspot mutations were found. Early diagnosis through newborn screening and following standardized treatment can significantly improve the prognosis of the patients.
Child ; Humans ; Infant, Newborn ; Carbamoyl-Phosphate Synthase I Deficiency Disease/therapy* ; Neonatal Screening ; Follow-Up Studies ; Hyperammonemia ; Citrulline/genetics* ; Retrospective Studies ; Mutation

OBJECTIVES: To retrospectively analyze the variation and characteristics of phenylalanine hydroxylase (PAH) gene, and to observe the long-term treatment effect and follow-up of newborns with PAH deficiency. METHODS: Clinical data, treatment and follow-up results of 198 patients with PAH deficiency diagnosed by newborn screening in Jinan from 1996 to 2021 were collected. The genetic analysis of 55 patients with PAH deficiency diagnosed by newborn screening in Jinan and 213 patients referred from the surrounding areas of Jinan were summarized. Gene variations were checked by a customized Panel gene detection method. Blood phenylalanine-concentration and physical development indicators including height and weight were regularly monitored. Intellectual development was assessed using a neuropsychological development scale for patients aged 0-6 years and academic performance, and brain injury in patients was assessed using brain magnetic resonance imaging. RESULTS: c.728G>A, c.158G>A, c.721C>T, c.1068C>A, c.611A>G variations were common in PAH gene. The genotype of c.158G>A variation is compound heterozygous variation, with mainly a mild hyperpheny-lalaninemia. 168 patients with PAH deficiency who were followed-up regularly had normal physical development without dwarfism or malnutrition. Among the 33 preschool patients who underwent mental development assessment, 2 were mentally retarded and the initial treatment age was older than 6 months. Nine patients with an average age of (17.13±2.42) years completed brain magnetic resonance imaging, one case was normal, and 8 cases were abnormal. There were patchy or patchy hyperintense foci near the bilateral lateral ventricles on T₂WI, and the intellectual development was normal. Compared with the other eight patients, the blood phenylalanine concentration of the normal child was better and stably controlled within the ideal range. CONCLUSIONS c.728G>A, c.158G>A, c.721C>T, c.1068C>A, c.611A>G variations were common in PAH gene. After standardized treatment, most patients with PAH deficiency diagnosed by screening can obtain normal growth and intellectual development in adolescence, but there are different degrees of organic lesions in the cerebral white matter.
Child ; Child, Preschool ; Adolescent ; Humans ; Infant, Newborn ; Young Adult ; Adult ; Neonatal Screening ; Follow-Up Studies ; Retrospective Studies ; Phenylketonurias/genetics* ; Phenylalanine Hydroxylase/genetics* ; Phenylalanine/therapeutic use* ; Mutation

Sinonasal inverted papilloma（SNIP） is a kind of benign tumor originating from the nasal cavity and paranasal sinuses, accounting for 70% of papillomas. The incidence of the disease is more common in males, with an average age of 50-60 years. It is most likely to occur in unilateral maxillary sinus and ethmoid sinus, followed by sphenoid sinus and frontal sinus.It has the characteristics of local invasion, high recurrence rate and malignant tendency, and most malignant transformation into squamous cell carcinoma. Endoscopic nasal resection and appropriate adjuvant therapy can help to reduce the recurrence rate and inhibit further deterioration. We report the results of a 10-year follow-up of a SNIP patient, including the clinical manifestations, recurrence course and treatment plan during the 10 years. The patient underwent multiple nasal endoscopic surgeries, and had a recurrence of multiple focal attachment pattern, and finally had direct invasion and distant metastasis. Tumor recurrence and further deterioration persisted despite the use of a comprehensive treatment.
Male ; Humans ; Middle Aged ; Papilloma, Inverted ; Follow-Up Studies ; Neoplasm Recurrence, Local ; Head and Neck Neoplasms ; Frontal Sinus

Objective: Murine CD19 chimeric antigen receptor T-cell (CAR-T) products have been approved for the treatment of refractory/relapsed (R/R) B-cell acute lymphocytic leukemia (B-ALL) ; moreover, humanized products are also undergoing clinical trials. This study aimed to explore the differences in safety and short- and long-term follow-up efficacy between humanized and murine CD19 CAR-T-cells for treating relapsed and refractory B-ALL. Methods: Clinical data of 80 patients with R/R B-ALL treated with CD19-targeted CAR-T-cells at the Union Hospital of Tongji Medical College of Huazhong University of Science and Technology between May 2016 and March 2023 were analyzed, which included 31 patients with murine CAR-T and 49 with humanized products. Results: The proportion of patients with cytokine-release syndrome (CRS) in the murine and humanized groups was 63.1% and 65.3%, respectively. Moreover, a higher proportion of patients suffered from severe CRS in the murine group than in the humanized CAR-T group (19.4% vs 8.2%, P=0.174). Furthermore, one patient per group died of grade 5 CRS. The incidence of grade 1-2 immune effector cell-associated neurotoxicity syndrome (ICANS) was 12.9% and 6.1%, respectively; severe ICANS were not observed. Among patients receiving murine CAR-T-cells, an overall response (OR) was observed in 74.2%. Conversely, the OR rate of patients receiving humanized CAR-T-cells was 87.8%. During the median follow-up time of 10.5 months, the median recurrence-free survival (RFS) of patients with murine CAR-T-cells was 12 months, which was as long as that of patients with humanized CAR-T-cells. The median overall survival (OS) were not reached in both groups. Of the 45 patients with a bone marrow burden over 20% at baseline, humanized CAR-T therapy was associated with a significantly improved RFS (43.25% vs 33.33%, P=0.027). Bridging transplantation was an independent factor in prolonging OS (χ(2)=8.017, P=0.005) and PFS (χ(2)=6.584, P=0.010). Common risk factors, such as age, high proportion of bone marrow blasts, and BCR-ABL fusion gene expression, had no significant effect on patients' long-term follow-up outcomes. Three patients reached complete remission after reinfusion of humanized CAR-T-cells. However, one patient relapsed one month after his second infusion of murine CAR-T-cells. Conclusions: The results indicate that humanized CAR-T therapy showed durable efficacy in patients with a higher tumor burden in the bone marrow without any influence on safety. Moreover, it could overcome immunogenicity-induced CAR-T resistance, providing treatment options for patients who were not treated successfully with CAR-T therapies.
Animals ; Humans ; Mice ; Antigens, CD19 ; Burkitt Lymphoma/drug therapy* ; Cell- and Tissue-Based Therapy ; Follow-Up Studies ; Immunotherapy, Adoptive ; Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy* ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Receptors, Chimeric Antigen

INTRODUCTION: Despite the challenges related to His bundle pacing (HBP), recent data suggest an improved success rate with experience. As a non-university, non-electrophysiology specialised centre in Singapore, we report our experiences in HBP using pacing system analyser alone. METHODS: Data of 28 consecutive patients who underwent HBP from August 2018 to February 2019 was retrospectively obtained. The clinical and technical outcomes of these patients were compared between two timeframes of three months each. Patients were followed up for 12 months. RESULTS: Immediate technical success was achieved in 21 (75.0%) patients (mean age 73.3 ± 10.7 years, 47.6% female). The mean left ventricular ejection fraction was 53.9% ± 12.1%. The indications for HBP were atrioventricular block (n = 13, 61.9%), sinus node dysfunction (n = 7, 33.3%) and upgrade from implantable cardioverter-defibrillator to His-cardiac resynchronisation therapy (n = 1, 4.8%). No significant difference was observed in baseline characteristics between Timeframe 1 and Timeframe 2. Improvements pertaining to mean fluoroscopy time were achieved between the two timeframes. There was one HBP-related complication of lead displacement during Timeframe 1. All patients with successful HBP achieved non-selective His bundle (NSHB) capture, whereas only eight patients had selective His bundle (SHB) capture. NSHB and SHB capture thresholds remained stable at the 12-month follow-up. CONCLUSION Permanent HBP is feasible and safe, even without the use of an electrophysiology recording system. This was successfully achieved in 75% of patients, with no adverse clinical outcomes during the follow-up period.
Humans ; Female ; Middle Aged ; Aged ; Aged, 80 and over ; Male ; Bundle of His ; Follow-Up Studies ; Stroke Volume ; Retrospective Studies ; Treatment Outcome ; Cardiac Pacing, Artificial/adverse effects* ; Electrocardiography ; Ventricular Function, Left/physiology*