To analyze the clinical and genetic features of oculopharyngodistal myopathy (OPDM) patients and compare the phenotypic differences among various causative genes.

This article reports a rare case of thrombotic microangiopathy (TMA) with renal involvement complicated by allogeneic hematopoietic stem cell transplantation (allo-HSCT). The patient appeared increased serum creatinine 20 d after allo-HSCT, and gradually appeared hypertension, oliguria and edema. Despite discontinuing suspected medications, serum creatinine level did not decrease. Treatment with basiliximab and mycophenolate mofetil was initiated to prevent rejection, leading to gradual normalization of urine output and serum creatinine level. However, after stopping mycophenolate mofetil, the patient experienced recurrent increased blood pressure and decreased pulse oximetry, responding well to prednisone but recurring upon cessation, with gradually increased serum creatinine level. Renal pathology indicated that chronic TMA after allo-HSCT caused renal injury, primarily affecting the glomeruli. The renal function achieved long-term stability through low-dose prednisone and symptomatic treatment. By reviewing relevant literature, we discussed the clinical manifestations, laboratory tests, pathological features and treatment strategies of TMA with renal involvement complicated by allo-HSCT.

This paper presents a rare case of renal amyloidosis complicated with primary minimal change disease. The patient initially presented with edema and proteinuria, accompanied by IgG-λ monoclonal immunoglobulinemia, leading to a diagnosis of primary systemic immunoglobulin light chain amyloidosis with renal involvement. Following treatment, the patient achieved both hematologic and renal remission. However, a renal relapse occurred two years later, presenting as nephrotic syndrome without hematologic disease recurrence. A repeat renal biopsy revealed no obvious change in amyloid deposition, but demonstrated markedly enlarged effacement of podocyte foot processes. Based on these findings, a secondary diagnosis of primary minimal change disease was established. The patient exhibited a rapid response to immunosuppressive therapy, achieving sustained long-term remission. This case underscores the importance of remaining vigilant to etiological changes in the treatment of renal diseases and highlights the role of repeated renal biopsy in refining the diagnosis and guiding treatment.

OBJECTIVE: To evaluate the effectiveness of combined anterolateral thigh-ilioinguinal Flow-through flaps for repairing complex lower limb defects. METHODS: A clinical data of 20 patients with complex lower limb injuries admitted between January 2018 and January 2024 was retrospectively analyzed. The cohort included 14 males and 6 females with an average age of 47.3 years (range, 29-65 years). Injury mechanisms comprised heavy-object trauma (n=7), traffic accidents (n=5), machinery crush injuries (n=5), and osteomyelitis (n=3). Defects involved the left (n=7) and right (n=13) limbs, with anatomical distributions including tibiofibular injuries (n=6), isolated tibial injuries (n=6), foot and ankle injuries (n=5), and femoral-tibial injuries (n=3). The size of soft tissue defects ranged from 23 cm×8 cm to 44 cm×12 cm. Reconstruction employed combined anterolateral thigh-ilioinguinal Flow-through flaps in the size of 24 cm×10 cm to 48 cm×14 cm. The recipient sites were sutured in primary closure in 12 cases, and 8 cases had no available vascular anastomosis sites in the recipient sites, and a cross-leg flap form was used to establish a temporary blood supply, and the flaps were cut off after 3-4 weeks. The donor sites in the thigh were directly sutured. During follow-up, the survival of the flaps, appearance, texture, and related complications were observed; the Vancouver scar scale (VSS) score was used to evaluate the scar condition of the flaps, the lower extremity function scale (LEFS) score was used to evaluate the function of the affected lower limb, and the visual analogue scale (VAS) score was used to evaluate the pain condition of the affected side. RESULTS: Postoperatively, the flap complete necrosis occurred in 1 case, marginal necrosis in 1 case, superficial infections in 2 cases, and venous thrombosis in 1 case. The remaining flaps survived completely with primary wound healing at both recipient and donor sites. Limb salvage was achieved in all patients. All patients were followed up with 12-24 months (mean, 18.4 months). All flaps had satisfactory color, texture, and contour. Fractures reached clinical union in all cases. Donor site morbidity included mild contralateral hip flexion/knee extension limitation (n=1), persistent hypoesthesia (n=3), and chronic pain (n=1) at 6 months. At 12 months after operation, the LEFS, VSS, and VAS scores on the affected side were 62.7±4.6, 3.5±1.1, and 1.2±0.6, respectively, which were superior to those at 1 month after operation (38.6±2.8, 8.5±1.4, 4.7±1.1), and the differences were significant (P<0.05). CONCLUSION The anterolateral thigh-ilioinguinal Flow-through flaps for repairing complex lower limb injuries is a good method. The distal blood supply of the affected side recover well, the survival rate of the flap is high, and the function recovery of the affected limb is good.
Humans ; Male ; Middle Aged ; Female ; Adult ; Plastic Surgery Procedures/methods* ; Aged ; Retrospective Studies ; Soft Tissue Injuries/surgery* ; Surgical Flaps/blood supply* ; Lower Extremity/surgery* ; Thigh/surgery* ; Treatment Outcome ; Leg Injuries/surgery*

Objective:To investigate the clinical diagnosis and treatment of granulomatous prostatitis（GP）in patients with Prostate Imaging Reporting and Data System（PI-RADS）scores ≥ 4 on magnetic resonance imaging（MRI）.Methods:The data of 12 GP patients with PI-RADS score ≥ 4 who were admitted to Beijing Chaoyang Hospital，Capital Medical University，from February 2015 to February 2025，were retrospectively analyzed. The patients were aged 51?73 years（mean 66 years）. Presenting complaints included elevated prostate-specific antigen（PSA）levels in 6 cases，prostatic mass in 2 cases，urinary retention in 3 cases，and gross hematuria in 1 case. All 12 patients had concomitant lower urinary tract symptoms. Medical history revealed pulmonary tuberculosis in 2 cases，testicular tuberculosis in 1 case，close contact with tuberculosis in 1 case（spouse diagnosed with pulmonary tuberculosis 5 years earlier），allergic granulomatous vasculitis in 1 case，and intravesical bacillus Calmette-Guérin（BCG）instillation in 1 case. Digital rectal examination（DRE）showed gradeⅠprostatic hyperplasia in 2 cases，grade Ⅱ in 9 cases，and grade Ⅲ in 1 case. Nodules were palpable in 6 patients. The median PSA was 7.20 ng/ml（ranging 1.11?21.90 ng/ml），with 2 cases < 4 ng/ml. Transrectal ultrasound was performed in 10 patients，and prostate volumes were ranging from 29.48 to 109.78 cm3，with 6 cases > 45 cm3. All 12 patients underwent MRI，and all demonstrated PI-RADS scores ≥ 4，typically presenting as low signal intensity on T2-weighted imaging，high signal on diffusion-weighted imaging（DWI），and low apparent diffusion coefficient（ADC）values. Contrast-enhanced MRI in 8 cases revealed heterogeneous enhancement. One patient underwent 68Ga-prostate-specific membrane antigen positron emission tomography-computed tomography（ 68Ga-PSMA PET-CT），which showed band-like increased uptake in the central zone. All 12 patients were preoperatively suspected of prostate cancer，of whom 10 underwent transrectal biopsy and 2 underwent transperineal biopsy. Pathological characteristics and follow-up results were analyzed. Treatment outcomes were analyzed，The International Prostate Symptom Score（IPSS），quality of life（QOL）score，maximum urinary flow rate（Q max），and post-void residual urine（PVR）volume were compared before and 1 month after treatment. Results:Histopathology confirmed GP in all cases，with chronic inflammation in 11 cases and acute inflammation in 1 case. Immunohistochemistry demonstrated positivity for CD68（macrophage marker），high-molecular weight cytokeratin（HCK），and reticulin staining；periodic acid-Schiff（PAS）and acid-fast staining were positive in 2 cases，corresponding to 1 patient with a prior history of pulmonary tuberculosis and 1 with testicular tuberculosis. Two patients underwent thulium laser enucleation of the prostate，while 10 received conservative treatment，including 2 patients with tuberculosis infection who were referred for anti-tuberculosis therapy and 8 patients treated with oral tamsulosin 0.2 mg once daily. Follow-up was completed in 10 patients：9 were followed for 1 ? 3 months（mean 2.4 months），and 1 patient was followed for 9 years before being diagnosed with prostate cancer. Two additional patients，whose pathological findings suggested a possible diagnosis of tuberculous granulomatous prostatitis，were advised to undergo anti-tuberculosis treatment at another hospital and were subsequently lost to follow-up. Among the 2 patients who underwent thulium laser enucleation，IPSS decreased from 26 and 25 to 6 and 5 respectively，QOL scores decreased from 6 and 5 to 1 and 0 respectively，Q max increased from 4.5 and 4.3 ml/s to 23.0 and 21.9 ml/s respectively. In the 8 patients treated conservatively，IPSS decreased from 18.45±7.17 to 14.45±5.03，and QOL score decreased from 5.09±1.04 to 4.09±0.70 at 1 month after treatment，showing significant improvement（ P < 0.05）. Additionally，one patient initially diagnosed with GP and managed conservatively remained stable for 9 years，but subsequently developed urinary retention and underwent thulium laser enucleation，with postoperative pathology confirming prostate cancer. Conclusions:Clinical manifestations of GP are nonspecific，and the condition can easily be mistaken for prostate cancer due to elevated PSA levels and PI-RADS scores ≥4. Some patients may present with acute urinary retention，but definitive diagnosis still relies on prostate biopsy and immunohistochemistry. Treatment should be individualized according to the underlying etiology，with medication as the mainstay，while transurethral surgical intervention may be considered in cases with obstruction. Although GP is a benign lesion，its potential association with prostate cancer warrants vigilance and emphasizes the importance of long-term follow-up.

Nonunion of osteoporotic vertebral fractures (OVF), predominantly affecting the elderly, can lead to intractable pain, vertebral collapse, progressive kyphotic deformity, and neurological impairment, significantly compromising patients′ quality of life. There exists considerable debate on diagnosis and management of OVF, encompassing key issues such as clinical diagnosis and staging criteria for nonunion, surgical indications and procedure selection, and postoperative rehabilitation planning. Currently, there lacks standardized clinical guideline and expert consensus on the diagnosis and management of OVF nonunion in China. To address this gap, Minimally Invasive Surgery Group of Chinese Orthopedic Association, Osteoporosis Committee of Chinese Association of Orthopedic Surgeons, Prevention and Rehabilitation Committee for Osteoporosis of Chinese Association of Rehabilitation Medicine and Minimally Invasive Orthopedic Surgery Branch of China Association for Geriatric Care jointly organized domestic experts in spinal surgery, endocrinology, and rehabilitation to formulate the Clinical guideline for the diagnosis and treatment for nonunion of osteoporotic vertebral fractures ( version 2025), based on existing literature and clinical experience and adhering to principles of scientific rigor and practicality. The guideline provided 13 evidence-based recommendations encompassing diagnosis and treatment of OVF nonunion, aiming to standardize its clinical management.

Objective To assess the efficacy of clear lens extraction combined with extended depth of focus(EDOF)intraocular lens(IOL)implantation in enhancing visual quality and concurrently opening peripheral angle structures in patients diagnosed with primary angle closure suspect(PACS)and presbyopia.Methods A total of 80 patients with PACS,presbyopia,and transparent lenses were enrolled and divided into two groups based on the type of implanted lens:a control group(n=46)and an experimental group(n=34).The control group underwent clear lens extraction followed by monofocal intraocular lens implantation,while the experimental group underwent clear lens extraction followed by extended depth of focus(EDOF)intraocular lens implantation.Preoperative and postoperative evaluations at 3 months included visual acuity,intraocular pressure,anterior segment structure,defocus curve,and Catquest 9SF-CN questionnaire scores.Adverse events were recorded to assess safety.Results Regarding the angular structure,after 3 months of treatment,results indicated significant increases in central ante-rior chamber depth,angle opening distance at 750 μ m,angle recess area at 750 μ m,trabecular-iris space area at 750 μm,and trabecular-iris angle at 750 μm in both groups compared to preoperative values(all P<0.01).However,no significant differences were observed between the two groups(all P>0.05).For the defocusing curve,visual acuity in the experimental group improved from+1.0 D to-4.0 D after 3 months of treatment(all P<0.05),while the control group showed improvements at+0.5 D,-0.5 D,-1.0 D,-2.0 D,-3.0 D,-3.5 D,and-4.0 D(all P<0.05).Postoperatively at 3 months,the experimental group exhibited superior visual acuity to the control group at-1.0 D to-4.0 D(all P<0.05).According to the Catquest 9SF-CN scale,scores in both groups significantly im-proved after 3 months of treatment(P<0.01),with the experimental group scoring higher than the control group(P<0.01).During the study,transient intraocular pressure elevation occurred in 7 patients(3 in the control group and 4 in the experimental group),but no serious adverse reactions were reported in either group.Conclusions In patients with pseudoexfoliation glaucoma combined with presbyopia,significant improvements in angle structures were observed following clear lens extraction.The implantation of an EDOF IOL notably enhanced postoperative visual quality compared to monofocal IOLs.

Objective To assess the efficacy of clear lens extraction combined with extended depth of focus(EDOF)intraocular lens(IOL)implantation in enhancing visual quality and concurrently opening peripheral angle structures in patients diagnosed with primary angle closure suspect(PACS)and presbyopia.Methods A total of 80 patients with PACS,presbyopia,and transparent lenses were enrolled and divided into two groups based on the type of implanted lens:a control group(n=46)and an experimental group(n=34).The control group underwent clear lens extraction followed by monofocal intraocular lens implantation,while the experimental group underwent clear lens extraction followed by extended depth of focus(EDOF)intraocular lens implantation.Preoperative and postoperative evaluations at 3 months included visual acuity,intraocular pressure,anterior segment structure,defocus curve,and Catquest 9SF-CN questionnaire scores.Adverse events were recorded to assess safety.Results Regarding the angular structure,after 3 months of treatment,results indicated significant increases in central ante-rior chamber depth,angle opening distance at 750 μ m,angle recess area at 750 μ m,trabecular-iris space area at 750 μm,and trabecular-iris angle at 750 μm in both groups compared to preoperative values(all P<0.01).However,no significant differences were observed between the two groups(all P>0.05).For the defocusing curve,visual acuity in the experimental group improved from+1.0 D to-4.0 D after 3 months of treatment(all P<0.05),while the control group showed improvements at+0.5 D,-0.5 D,-1.0 D,-2.0 D,-3.0 D,-3.5 D,and-4.0 D(all P<0.05).Postoperatively at 3 months,the experimental group exhibited superior visual acuity to the control group at-1.0 D to-4.0 D(all P<0.05).According to the Catquest 9SF-CN scale,scores in both groups significantly im-proved after 3 months of treatment(P<0.01),with the experimental group scoring higher than the control group(P<0.01).During the study,transient intraocular pressure elevation occurred in 7 patients(3 in the control group and 4 in the experimental group),but no serious adverse reactions were reported in either group.Conclusions In patients with pseudoexfoliation glaucoma combined with presbyopia,significant improvements in angle structures were observed following clear lens extraction.The implantation of an EDOF IOL notably enhanced postoperative visual quality compared to monofocal IOLs.

Nonunion of osteoporotic vertebral fractures (OVF), predominantly affecting the elderly, can lead to intractable pain, vertebral collapse, progressive kyphotic deformity, and neurological impairment, significantly compromising patients′ quality of life. There exists considerable debate on diagnosis and management of OVF, encompassing key issues such as clinical diagnosis and staging criteria for nonunion, surgical indications and procedure selection, and postoperative rehabilitation planning. Currently, there lacks standardized clinical guideline and expert consensus on the diagnosis and management of OVF nonunion in China. To address this gap, Minimally Invasive Surgery Group of Chinese Orthopedic Association, Osteoporosis Committee of Chinese Association of Orthopedic Surgeons, Prevention and Rehabilitation Committee for Osteoporosis of Chinese Association of Rehabilitation Medicine and Minimally Invasive Orthopedic Surgery Branch of China Association for Geriatric Care jointly organized domestic experts in spinal surgery, endocrinology, and rehabilitation to formulate the Clinical guideline for the diagnosis and treatment for nonunion of osteoporotic vertebral fractures ( version 2025), based on existing literature and clinical experience and adhering to principles of scientific rigor and practicality. The guideline provided 13 evidence-based recommendations encompassing diagnosis and treatment of OVF nonunion, aiming to standardize its clinical management.

This article reports a rare case of thrombotic microangiopathy (TMA) with renal involvement complicated by allogeneic hematopoietic stem cell transplantation (allo-HSCT). The patient appeared increased serum creatinine 20 d after allo-HSCT, and gradually appeared hypertension, oliguria and edema. Despite discontinuing suspected medications, serum creatinine level did not decrease. Treatment with basiliximab and mycophenolate mofetil was initiated to prevent rejection, leading to gradual normalization of urine output and serum creatinine level. However, after stopping mycophenolate mofetil, the patient experienced recurrent increased blood pressure and decreased pulse oximetry, responding well to prednisone but recurring upon cessation, with gradually increased serum creatinine level. Renal pathology indicated that chronic TMA after allo-HSCT caused renal injury, primarily affecting the glomeruli. The renal function achieved long-term stability through low-dose prednisone and symptomatic treatment. By reviewing relevant literature, we discussed the clinical manifestations, laboratory tests, pathological features and treatment strategies of TMA with renal involvement complicated by allo-HSCT.