OBJECTIVE: To analyze the clinical characteristics, treatment, and prognosis of seven pediatric patients with Acute myeloid leukemia (AML) positive for the t(16;21)(p11;q22) FUS::ERG fusion gene. METHODS: A retrospective analysis was carried out on the clinical data, treatment, and prognosis of seven AML patients with t(16;21)(p11;q22) FUS::ERG fusion gene admitted to Henan Children's Hospital between June 2015 and November 2024. Relevant literature was also reviewed. This study was approved by the Medical Ethics Committee of the Hospital (Ethics No.: 2024-102-001). RESULTS: Among 297 pediatric patients with AML, 7 cases (2.36%) were positive for the t(16;21)(p11;q22) FUS::ERG fusion gene, including 3 males and 4 females, with a median age of 11 years (range: 3 ~ 12 years). According to the FAB classification, these included 1 case of M2, 3 cases of M5, and 3 cases of AML-not otherwise specified (non-M3). All 7 patients were found to harbor the t(16;21)(p11;q22) translocation, with 3 cases showing additional chromosomal abnormalities. Immunophenotyping revealed universal expression of CD13, CD33, CD34, and CD117, with partial expression of CD56, CD4, CD64, CD123, CD15, CD38, CD11b, HLA-DR, cMPO, and CD16. One patient achieved complete remission (CR) after the first course of DAE (cytarabine + daunorubicin + etoposide) induction chemotherapy but relapsed and discontinued the treatment. Six patients received DAH (cytarabine + daunorubicin + homoharringtonine) induction therapy, of whom 2 achieved CR after two courses and underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT), resulting in an overall CR rate of 42.86%. Five children did not receive allo-HSCT and had a median overall survival of 9 months (range: 6 ~ 18 months). Two children who underwent transplantation achieved bone marrow morphological and molecular biological relapse at 6 and 9 months post-transplantation, respectively. After receiving combined chemotherapy and donor lymphocyte infusion, one child failed to achieve remission and died at 22 months post-transplantation, while the other has been followed up to date with positive fusion gene status. Their overall survival was 25 months and 30 months, respectively. CONCLUSION The t(16;21)(p11;q22) FUS::ERG fusion gene is rare in pediatric AML and associated with poor prognosis. Allo-HSCT may mitigate the adverse prognostic impact of the FUS::ERG fusion gene and contribute to prolonged survival.
Humans ; Male ; Child ; Female ; Leukemia, Myeloid, Acute/drug therapy* ; Oncogene Proteins, Fusion/genetics* ; Translocation, Genetic ; Retrospective Studies ; RNA-Binding Protein FUS/genetics* ; Chromosomes, Human, Pair 16/genetics* ; Adolescent ; Child, Preschool ; Chromosomes, Human, Pair 21/genetics* ; Prognosis ; Treatment Outcome

Introduction: Primary aldosteronism (PA) is a common yet underdiagnosed cause of secondary hypertension, associated with increased cardiovascular and renal morbidity. Early detection and subtype-directed management significantly improve outcomes. However, adherence to recommended diagnostic pathways in real-world practice remains variable. Case: We conducted a retrospective audit of patients diagnosed with PA in a Malaysian tertiary centre from January 2018 to December 2025. Data collected included demographics, clinical presentation, biochemical parameters, diagnostic workup (aldosterone-renin ratio [ARR], confirmatory testing, adrenal imaging, and adrenal venous sampling [AVS]), treatment modality, and outcomes. Audit standards were based on established international guidelines. A total of 14 patients were included, with equal gender distribution. The cohort comprised 57% Malay and 43% Chinese. Most patients presented with hypertension (mean ~170/100 mmHg), and hypokalemia was present in approximately 70% of the patients. ARR and adrenal imaging were performed in all patients (100%), while confirmatory testing was conducted in 85% of the patients. However, AVS utilization remained limited (50%). Contributing factors included failed cannulation, technical challenges in overweight patients, preference for medical therapy, refusal of surgery, and limited access to AVS services. The majority had unilateral adrenal adenoma (~75%). Treatment was divided between surgical (55%) and medical (45%) approaches. Post-treatment, hypokalemia resolved in 90% of patients, with a significant reduction in antihypertensive burden and complete hypertension resolution in approximately 35% of the patients. Quality of life improved in most patients. Conclusion This audit demonstrates good adherence to initial screening and imaging in PA but highlights suboptimal utilization of AVS. Barriers to AVS utilization are multifactorial, encompassing technical, patient-related, and system level limitations. Addressing these barriers is essential to optimize subtype-directed management and improve longterm cardiovascular outcomes in patients with PA. Despite this, clinical outcomes were favorable. Strengthening adherence to diagnostic pathways, particularly subtype confirmation, may further improve patient outcomes.
Hyperaldosteronism ; Treatment Outcome ; Retrospective Studies

Introduction: Diabetes mellitus (DM) is a significant comorbidity associated with adverse tuberculosis (TB) treatment outcomes and remains an ongoing clinical challenge. Chronic hyperglycemia impairs host immune responses, particularly macrophage function, leading to delayed bacillary clearance. This contributes to poorer outcomes, including delayed sputum conversion, prolonged treatment duration, relapse, and increased mortality. Poor glycemic control is a key modifiable factor influencing these outcomes. This study aimed to evaluate the impact of glycemic control at diagnosis on TB treatment outcomes in a Malaysian cohort. Methodology: A retrospective cross-sectional audit was conducted using the TB clinic registry at Hospital Sungai Buloh from 2022 to 2025. Adult patients (≥18 years) with confirmed TB (any form) and DM (Type 1 or Type 2) with complete records were included. Of 241 patients screened, 131 were included after exclusions due to incomplete records (29.9%), transfer (29.9%), loss to follow-up (19.9%), and drug-resistant TB (2.9%). Data collected included demographics, hemoglobin A1c (HbA1c) at diagnosis, sputum conversion duration, treatment duration, type of DM therapy, and clinical outcomes. Results: The cohort had a mean age of 53 years, with 97% having Type 2 DM. Mean HbA1c at diagnosis was 10.5%. Poorer glycemic control was associated with delayed sputum conversion. Patients who achieved sputum conversion by 8 weeks had an average HbA1c of 9.6%, compared to 11.8% in those with delayed conversion. Longer treatment duration was associated with higher HbA1c (mean 10.5, 8.6, and 11.8% for 6-, 12-, and 18-month regimens, respectively). Relapse analysis demonstrated a trend towards higher HbA1c with increasing relapse episodes (11.2, 13.75, and 14.5% for one, two, and three relapses, respectively). Overall mortality was 17% (22/131), with markedly higher mean HbA1c in deceased patients compared to survivors (16.0% vs 10.5%). Conclusion Poor glycemic control at TB diagnosis is associated with delayed sputum conversion, prolonged treatment duration, and increased mortality. These findings are consistent with regional evidence, including a South Asian systematic review (Gautam et al., 2021), demonstrating higher mortality and treatment failure in patients with TB and DM. Early and aggressive optimization of glycemic control, alongside standard anti-TB therapy, is essential to improve outcomes.
Humans ; Glycemic Control ; Retrospective Studies ; Diabetes Mellitus ; Treatment Outcome ; Tuberculosis

Wunderlich Syndrome is a rare potentially life-threatening phenomenon that involves spontaneous non-traumatic retroperitoneal hemorrhage. At present, identifying the course of conservative management in these patients, especially in pregnancy, has not been clinically established. Presented here is a known case of Tuberous Sclerosis Complex with a Bilateral, 10cm Angiomyolipoma in a 31-year-old female, initially managed with active surveillance. At 27 weeks of pregnancy, she presented with a sudden onset of left flank pain with a hematocrit of 22%. Anemia was corrected with blood transfusions. A contrast-enhanced MRI of the abdomen showed a large subcapsular perirenal hematoma of the left kidney. Renal angioembolization of the bleeding segmental renal artery was done. The patient was conservatized until 37 weeks of pregnancy and underwent cesarean section delivery.

Four months after angioembolization, she had a recurrence of the left flank pain associated with gross hematuria and hypovolemic shock. The patient underwent emergency renal exploration of the left kidney via a transabdominal approach. Three liters of hemoperitoneum and a large expanding left retroperitoneal hematoma were noted intraoperatively. Early vascular control before nephrectomy of the left kidney was done. The postoperative course was unremarkable and the patient was discharged with improved condition.

This case displays a unique course in the management of a bleeding angiomyolipoma especially during pregnancy. Renal angioembolization can aid in achieving the age of viability in pregnancy. However, close monitoring for rebleeding should be kept in mind. A lower threshold for conservative management should be utilized when patients have a previous history of bleeding.

Human ; Female ; Adult: 25-44 Yrs Old ; Abdomen ; Anemia ; Angiomyolipoma ; Arteries ; Blood ; Blood Transfusion ; Cesarean Section ; Conservative Treatment ; Emergencies ; Female ; Flank Pain ; Hematocrit ; Hematoma ; Hematuria ; Hemoperitoneum ; Hemorrhage ; History ; Hypovolemia ; Insemination, Artificial, Heterologous ; Kidney ; Life ; Nephrectomy ; Pain ; Patients ; Pregnancy ; Recurrence ; Renal Artery ; Research Report ; Sclerosis ; Shock ; Syndrome ; Tuberous Sclerosis ; Watchful Waiting

OBJECTIVE: To analyze the clinical efficacy and safety of microsurgery in patients with Gustilo ⅢC open fractures. METHODS: A total of 64 patients with Gustilo ⅢC open fractures who were admitted to the hospital bewteen September 2018 and March 2022 were included, and divided into the observation group and the control group, with 32 cases in each one. In the observation group, there were 24 males and 8 females, aged from 29 to 42 years with an average of (36.59±3.24) years. The tibial defect ranged from 2 to 5 cm with an average of (3.41±0.61) cm, and the soft tissue defect area ranged from 34 to 76 cm2 with an average of (58.50±9.44) cm2. This group received microsurgical treatment. In the control group, there were 25 males and 7 females, aged from 27 to 44 years with an average of (37.59±3.21) years. The tibial defect ranged from 2 to 6 cm with an average of (3.59±0.80) cm, and the soft tissue defect area ranged from 36 to 78 cm2 with an average of (59.09±9.53) cm2. This group received conventional staged surgery, with plaster or brace fixation after initial debridement and appropriate internal fixation at a later stage. The Anderson scores, motor ability Fugl-Meyer scale, perioperative indicators, rehabilitation outcomes, and complications were compared between the two groups. RESULTS: The patients were followed up for a duration from 2.5 to 5.5 months with an average of (3.15±1.11) months. In the observation group, 19 patients were rated as excellent, 12 patients as good, 1 patient as fair, and no patients as poor. In the control group, 11 patients were rated as excellent, 13 patients as good, 6 patients as fair, and 2 patients as poor. The difference in outcomes between the two groups was statistically significant (P<0.05). The operation time, the hospitalization time, and the hospitalization cost in the observation group(4.39±0.69) h, (30.22±4.58) d, and (23, 500±3, 300) yuan, respectively were significantly lower than those in the control group(5.01±0.75) h, (33.28±3.74) d, and(30, 200±5, 200) yuan, respectively (P<0.05). Revascularization time(134.25±14.76) h, wound healing time(11.34±1.56) d, and fracture healing time(3.09±0.42) months in the observation group were significantly shorter than those in the control group(P<0.05) which were (189.36±22.17) h, (13.22±2.03) d and (4.02±0.57) months respectively. The motor ability(42.91±5.51) points, sensory function(46.19±3.53) points, and total Fugl-Meyer score(89.09±6.08) in the observation group were significantly higher than those in the control group(P<0.05) which were(35.19±4.27), (34.03±3.79), (69.22±6.32) points respectivdy. In the observation group, there were 1 case of refractory wound and 3 cases of complete nerve injury, which were lower than those in the control group (8 cases and 10 cases, respectively, P<0.05). The number of complications in the observation group was lower than that in the control group (P<0.05). CONCLUSION Microsurgical technique can effectively shorten the wound and fracture healing time in patients with Gustilo Ⅲ C open fracture, improve limb movement ability, and reduce the risk of poor prognosis and complications.
Humans ; Male ; Female ; Adult ; Fractures, Open/surgery* ; Microsurgery/methods* ; Treatment Outcome

OBJECTIVE: To explore the early clinical outcomes of 3D printed individualised customised prostheses for in hip revision in patients with combined severe bone defects. METHODS: Twenty-two patients from January 2021 to May 2023 underwent hip revision using 3D printed personalised customised prostheses were retrospective analyzed, including 10 males and 12 females, age 28 to 78 with a mean of (58.9±12.8) years old. All of patients were combined with severe bone defects (Parprosky type Ⅲ). Among of them, 9 patients had periprosthetic infections and 13 patients had aseptic prosthesis loosening. All patients were treated with a 3D printed personalised prosthesis protocol, patients with the periprosthetic infection received a second stage revision after infection control. The operation time, preoperative waiting time, intraoperative and postoperative complications were recorded, and the clinical efficacy were evaluated at the final follow-up using the visual analogue scale (VAS) for pain, the Harris hip score. RESULTS: One patient was lost to follow-up and the remaining 21 patients were followed up for 10 to 15 with a mean of (12.91±1.44) months after surgery. All patients completed surgery as planned, with an operative time of 135 to 390 with a mean of (165.4±39.3) minutes and a preoperative waiting time of 7 to 16 with a mean of (10.5±3.3) days. Regarding patient complications:one patient had a severe intraoperative periprosthetic femoral fracture due to the combination of severe osteoporosis; one patient had an intraoperative greater trochanteric femur fracture. At the latest follow-up, all patients had good position of the custom-made prosthesis and no loosening of the prosthesis;all patients had good wound healing and no local redness or swelling. The total Harris score at the final follow-up (85.86±7.04) was significantly improved compared to the preoperative (44.86±2.36), P<0.001. The VAS at the last follow-up (2.19±0.87) was significantly improved compared with preoperative (7.41±0.96), P<0.001. CONCLUSION The clinical efficacy of 3D-printed personalised customised prosthesis in combined severe bone defect hip revision is satisfactory, but due to the increased preoperative waiting time of the patients and certain risks, certain indications should be mastered when applying in the clinic.
Humans ; Male ; Female ; Printing, Three-Dimensional ; Middle Aged ; Aged ; Adult ; Retrospective Studies ; Hip Prosthesis ; Arthroplasty, Replacement, Hip ; Reoperation ; Prosthesis Design ; Treatment Outcome

OBJECTIVE: To evaluate the mid-term efficacy of talonavicular joint fusion alone combined with navicular stress adjustment in Müller-Weiss disease with significant paradoxical flatfoot deformity. METHODS: Between January 2016 and March 2021, a total of 13 patients diagnosed with Müller-Weiss disease underwent simple talonavicular joint fusion combined with navicular stress adjustmentadjustment.Among them, 5 patients were male and 8 patients were female;age ranged from 42 to 67 years old. The duration of the disease ranged from 8 to 20 years. According to Maceira staging system, 5 patients were in stage III and 8 patients were in stage IV. The American Orthopaedic Foot and Ankle Society (AOFAS) midfoot score, visual analogue scale (VAS), and foot length were evaluated preoperatively and 10 months postoperatively. Additionally, the talonavicular coverage angle, the talus-first metatarsal angle, and the calcaneal inclination angle were assessed preoperatively and 6 months postoperatively. RESULTS: All 13 patients were followed up a period ranging from 24 to 40 months. All patients achieved clinical healing within a period of 3 to 6 months. However, one patient experienced a screw fracture. The VAS decreased from a range of 3 to 7 points preoperatively to a range of 0 to 2 points at the 10 months postoperative. The AOFAS midfoot score improved from a preoperative range of 12 to 66 points to a range of 72 to 100 points at the 10 months postoperative. Based on the AOFAS midfoot score evaluated at 10 months postoperatively, the outcomes were rated as excellent in 3 feet, good in 6 feet, and fair in 4 feet. The talo-navicular coverage angle, the preoperative talus-first metatarsal angle, calcaneal inclination angle were 14.3° to 33.4°, -4.6° to -19.6°, and 3.0°to 16.4° respectively. Six months postoperatively, these angles improved to 2.7°to 9.6°, -8.4°to 1.1°, and 18.8°to 24.9°respectively. Additionally, the foot length increased by 0 to 6 mm at 10 months post-surgery. CONCLUSION Simple talonavicular arthrodesis combined with scaphoid stress adjustment has satisfactory clinical efficacy in Müller-Weiss disease in terms of deformity, foot length, foot function and pain.
Humans ; Male ; Female ; Adult ; Middle Aged ; Arthrodesis/methods* ; Aged ; Flatfoot/physiopathology* ; Tarsal Bones/physiopathology* ; Tarsal Joints/surgery* ; Treatment Outcome

OBJECTIVE: To compare the clinical efficacy of oblique lateral interbody fusion(OLIF) combined with lateral fixation and transforaminal lumbar interbody fusion(TLIF) in patients with lumbar spinal stenosis. METHODS: Totally 47 patients with lumbar stenosis from November 2018 to December 2021 were analyzed retrospectively and were divided into two groups according to the surgical methods. Among them, 21 cases underwent oblique lumbar interbody fusion supplemental anterolateral screw and rod instrumentation, including 5 males and 16 females, with a mean age of (68.19±6.13) years old ranging 55 to 74 years; the other 26 cases underwent posterior pedicle screw fixation and reduction were recorded, including 8 males and 18 females with a mean age of (65.35±7.64) years old ranging 54 to 78 years. Visual analogue scale(VAS) of pain was recorded to evaluate the degree of low back pain and lower extremity pain. The radiographic parameters were collected to evaluate the efficacy of two approaches including disc height, foraminal height, canal sagittal diameter and cross-sectional area. RESULTS: All operations were completed successfully. The wound healed normally and bone fusion was obtained in the last final follow up. No serious complication was occurred in both groups. One case of dural tear occurred in direct compression group. Four cases of left thigh weakness and pain were recorded in indirect decompression group. The average postoperative follow-up was(21.69±4.37)months in direct compression group, while(20.43±4.80)months in another group. There were no significant difference in bone density, body mass index(BMI), hospital stay, Cobb angel(P>0.05). The differences in intra-operative blood loss, operation time, disc height, foraminal height between two groups were statistically significant(P<0.05). The area and sagittal diameter of the spinal canal in the two groups were significantly improved after surgery(P<0.05). CONCLUSION Both two fusion methods have achieved good clinical results in the treatment of lumbar spinal stenosis, with the advantages of good stability, fast recovery and high fusion rate. Compared with TLIF, the advantage of OLIF has greater advantages in less bleeding and less trauma.
Humans ; Male ; Female ; Spinal Stenosis/surgery* ; Spinal Fusion/methods* ; Aged ; Middle Aged ; Lumbar Vertebrae/surgery* ; Retrospective Studies ; Treatment Outcome

OBJECTIVE: To compare clinical efficacy of intermetatarsal incision and lateral soft tissue release of medial incision combined with Scarf osteotomy in treating moderate to severe hallux valgus (HV). METHODS: A retrospective analysis was conducted on clinical data of 42 patients with moderate to severe HV admitted from January 2022 to December 2022. According to different incisions, the patients were divided into medial incision group with 22 patients (22 feet) and intermetatarsal incision group with 20 patients (20 feet). In medial incision group, there were 3 males and 19 females, aged from 40 to 69 years old with an average of (55.0±11.4) years old;body mass index (BMI) ranged from 21 to 29 kg·m-2 with an average of (25.2±2.1) kg·m-2;the courses of disease ranged from 8 to 16 years with average of (12.0±2.2) years;11 patients with moderate deformity and 11 patients with severe deformity. In transplantar incision group, there were 3 males and 17 females, aged from 39 to 68 years old with an average of (53.0±7.5) years old;BMI ranged from 20 to 28 kg·m^-2 with an average of (24.8±1.9) kg·m^-2;the courses of disease ranged from 9 to 17 years with an average of (14.0±3.1) years;9 patients with moderate deformity and 11 patients with severe deformity. Hallux valgus angle (HVA) and the first-second intermetatarsal angle (IMA), American Orthopaedic Foot and Ankle Society (AOFAS) forefoot scores and complications between two groups before operation and 12 months after operation were observed and compared. RESULTS: All patients were successfully completed the surgery and were followed up for 12 to 15 months with an average of (13.52±1.65) months. There were no statistically significant difference in HVA and IMA between two groups before operation and 12 months after operation (P>0.05). AOFAS forefoot scores of medial incision group before operation and 12 months after operation were (45.0±6.8) and (86.0±6.7) respectively, and those of transmetatarsal incision group were (46.0±7.4) and (83.0±7.5) respectively. Postoperative AOFAS forefoot scores between two groups at 12 months were statistically significant compared with those of before operation (P<0.01). According to AOFAS forefoot scores, 8 patients got excellent result, 14 good in medial incision group;while 6 excellent and 14 good in transplantar incision group. At 12 months, postoperative AOFAS forefoot score of functional score of in medial incision group(38.0±2.5), was better than that in transplantar incision group (34.0±2.2), and the difference was statistically significant (P<0.05). One patient in medial incision group occurred HV deformity, mild numbness occurred in 3 toes in transplantar incision group, and 3 patients were dissatisfied with scar. No complications such as infection, nonunion of bones or ischemic necrosis of metatarsal heads occurred in either group. CONCLUSION Both intermetatarsal incision and lateral soft tissue release of medial incision combined with Scarf osteotomy can effectively treat moderate to severe HV. The functional recovery after medial incision is better than that after intermetatarsal incision.
Humans ; Male ; Female ; Hallux Valgus/physiopathology* ; Middle Aged ; Osteotomy/methods* ; Adult ; Aged ; Retrospective Studies ; Treatment Outcome ; Metatarsal Bones/surgery*

OBJECTIVE: To explore short-term clinical efficacy of minimally invasive diagnosis and treatment system for hallux valgus in guiding the treatment of hallux valgus. METHODS: From March 2021 to November 2023, 68 patients (136 feet) with hallux valgus were treated under guidance of minimally invasive diagnosis and treatment system, including 12 males and 56 females;aged from 25 to 68 years old with an average of (42.5±8.5) years old, the course of disease ranged from 3.2 to 15.6 years with an average of (10.3±2.6) years. The changes of hallux valgus angle (HVA) and intermetatarsal angle (IMA), visual analog scale (VAS) and American Orthopaedic Foot Ankle Society (AOFAS) forefoot score were recorded and compared before operation and 12 months after operation. RESULTS: Sixty-five patients (130 feet) were followed up for 12 to 15 months with an average of (13.8±0.5) months, 3 patients (6 feet) were not followed up as required. HVA and IMA improved from (35.5±3.5) ° and (12.5±2.0) ° before operation to (10.5±2.5) ° and (8.5±1.5) °12 months after operation, respectively, with statistically significant differences (P<0.05);VAS decreased from (5.5±1.2) before operation to (1.2±0.5) at 12 months after operation, and the difference was statistically significant (P<0.05);AOFAS forefoot score increased from (50.6±5.1) before operation to (93.8±5.6) at 12 months after operation, with a statistically significant difference (P<0.05). Among them, 102 feet were got excellent result, 24 feet good, and 4 feet fair. Two patients were developed calf intermuscular vein thrombosis, and were cured after 3 months of symptomatic treatment. CONCLUSION Under the guidance of minimally invasive diagnosis and treatment system for hallux valgus, the treatment of HV could obviously improve HVA and IMA, and significantly alleviate pain symptoms, and accelerate functional recovery.
Humans ; Hallux Valgus/diagnosis* ; Male ; Female ; Middle Aged ; Adult ; Aged ; Minimally Invasive Surgical Procedures/methods* ; Treatment Outcome