Bone Cements ; Femur ; pathology ; Humans ; Osteolysis, Essential ; therapy ; Syndrome

Gorham-Stout disease (GSD) is a sporadic chronic disease characterized by progressive bone dissolution, absorption, and disappearance along with lymphatic vessel infiltration in bone-marrow cavities. Although the osteolytic mechanism of GSD has been widely studied, the cause of lymphatic hyperplasia in GSD is rarely investigated. In this study, by comparing the RNA expression profile of osteoclasts (OCs) with that of OC precursors (OCPs) by RNA sequencing, we identified a new factor, semaphorin 3A (Sema3A), which is an osteoprotective factor involved in the lymphatic expansion of GSD. Compared to OCPs, OCs enhanced the growth, migration, and tube formation of lymphatic endothelial cells (LECs), in which the expression of Sema3A is low compared to that in OCPs. In the presence of recombinant Sema3A, the growth, migration, and tube formation of LECs were inhibited, further confirming the inhibitory effect of Sema3A on LECs in vitro. Using an LEC-induced GSD mouse model, the effect of Sema3A was examined by injecting lentivirus-expressing Sema3A into the tibiae in vivo. We found that the overexpression of Sema3A in tibiae suppressed the expansion of LECs and alleviated bone loss, whereas the injection of lentivirus expressing Sema3A short hairpin RNA (shRNA) into the tibiae caused GSD-like phenotypes. Histological staining further demonstrated that OCs decreased and osteocalcin increased after Sema3A lentiviral treatment, compared with the control. Based on the above results, we propose that reduced Sema3A in OCs is one of the mechanisms contributing to the pathogeneses of GSD and that expressing Sema3A represents a new approach for the treatment of GSD.
Animals ; Mice ; Endothelial Cells/metabolism* ; Lymphatic Vessels ; Osteoclasts/pathology* ; Osteolysis, Essential/pathology* ; Semaphorin-3A/metabolism*

Peri-implantitis is one of the most important biological complications in the field of oral implantology. Identifying the causative factors of peri-implant inflammation and osteolysis is crucial for the disease's prevention and treatment. The underlying risk factors and detailed pathogenesis of peri-implantitis remain to be elucidated. Titanium-based implants as the most widely used implant inevitably release titanium particles into the surrounding tissue. Notably, the concentration of titanium particles increases significantly at peri-implantitis sites, suggesting titanium particles as a potential risk factor for the condition. Previous studies have indicated that titanium particles can induce peripheral osteolysis and foster the development of aseptic osteoarthritis in orthopedic joint replacement. However, it remains unconfirmed whether this phenomenon also triggers inflammation and bone resorption in peri-implant tissues. This review summarizes the distribution of titanium particles around the implant, the potential roles in peri-implantitis and the prevalent prevention strategies, which expects to provide new directions for the study of the pathogenesis and treatment of peri-implantitis.
Humans ; Peri-Implantitis/pathology* ; Titanium/pharmacology* ; Dental Implants/adverse effects* ; Osteolysis/pathology* ; Inflammation/chemically induced*

Objective: To investigate the clinicopathological features, clinical manifestations and different diagnosis of patients with complicated lymphatic anomaly. Methods: The clinical and pathologic data of four patients with complicated lymphatic anomaly diagnosed and treated in Peking Union Medical College Hospital from January 2000 to December 2021 were collected and analyzed. Results: One Gorham-Stout disease case and three generalized lymphatic anomaly cases were included in this cohort. Patients' ages ranged from 7 to 32 years. There were three males and one female. The positions of biopsy included three bone biopsy and one bronchus biopsy. Microscopically, all cases showed diffuse enlarged lymphatic channels. At the same time, osteogenesis was obvious in Gorham-Stout disease case. Radiologically, cortical loss was seen in Gorham-Stout disease, and lytic bone confined to the medullary cavity presented in generalized lymphatic anomaly. The three generalized lymphatic anomaly cases also had coagulopathy, and two had effusion. Conclusions: The histologic feature of complicated lymphatic anomaly was diffuse lymphatic malformation, and the diagnosis depends on clinical and pathologic information. The treatment and prognosis of these diseases are different, and therefore it is necessary to understand their clinical and pathologic features and make the correct diagnosis.
Male ; Humans ; Female ; Child ; Adolescent ; Young Adult ; Adult ; Osteolysis, Essential/pathology* ; Lymphatic Abnormalities/surgery* ; Bone and Bones/pathology* ; Diagnosis, Differential ; Prognosis

Arteriovenous fistulas of the extracranial vertebral artery are rare. The authors report a case of a spontaneous arteriovenous fistula of the extracranial vertebral artery presenting as an osteolytic lesion at the body of the axis. The patient presented with headache and posterior neck pain. The fistula was obliterated by an endovascular trapping.
Adult ; Arteriovenous Fistula/*pathology/surgery ; Axis/*pathology ; Case Report ; Follow-Up Studies ; Human ; Male ; Osteolysis/pathology ; Support, Non-U.S. Gov't ; Vertebral Artery/*pathology/surgery

Polyfibromatosis syndrome is a rare disease entity that is characterized by various clinical features such as palmar, plantar, and penile fibromatoses, keloid formations of the skin, and erosive arthropathy. Its precise pathophysiology or etiology remains unclear. In addition to distinctive diverse skin manifestations, patients with polyfibromatosis have been previously reported to show erosive arthropathy with significant limitation of movement at affected joints. However, the presence of erosive polyarthropathy in polyfibromatosis has not emphasized in previous cases. Here, we report a case of polyfibromatosis syndrome combined with painless massive structural destruction of hand and foot joints, and review the characteristics of erosive arthropathy in previous cases.
Adult ; Arthrography ; Diagnosis, Differential ; Fibroma/*diagnosis/pathology/radiography ; Foot Joints/pathology/radiography ; Hand Joints/pathology/*radiography ; Humans ; Magnetic Resonance Imaging ; Male ; Metacarpophalangeal Joint/pathology/radiography ; Osteolysis/*diagnosis/etiology

INTRODUCTION: Although most stable total knee arthroplasties remain so for many years, recently asep-tic loosening of prosthesis due to osteolysis which are induced by polyethylene wear is one of important causes in TKA failure. The authours experienced two cases of TKA revision due to aseptic loosening, and analysed wear particles in association with aseptic loosening. MATERIALS AND METHODS: From Oct 1988 to July 1999, two cases among 400 TKA underwent revision TKA due to aseptic loosening. The periprosthetic interface membrane tissues were collected for analysis of wear particle. After enzymatic digestion, EDS and FT-IR employed for chemical and physical charac-teristics and SEM employed for size and morphology of particle. RESULTS: Radiolucent line were seen around tibial component and aseptic loosening were observed also. At revision operations, hypertrophy of synovium and interface membrane between prosthesis & host bone were confirmed. Pathology showed synovial hypertrophy and giant cell which impressed chronic inflammation due to foreign body. The isolated particles were mainly polyethylene debris. And the size of the particle was variable. Some were identified grossly, several mm, others were several micrometer. Also, submi-cron particles were seen. CONCLUSIONS: If aseptic loosening was developed in TKA, polyethylene wear particle should be consid-ered as a cause of loosening. The wear particles were variable in sized and more bigger than that in previ-ous reports of THA.
Arthroplasty* ; Digestion ; Foreign Bodies ; Giant Cells ; Hypertrophy ; Inflammation ; Knee* ; Membranes ; Osteolysis ; Pathology ; Polyethylene ; Prostheses and Implants ; Synovial Membrane

A 25-yr-old woman presented with a right pleural effusion. Destruction of 9th through 12th ribs, adjacent vertebral bodies, and transverse processes was noted on plain radiograph and a large low-attenuated, irregular shaped mass lesion with peripheral rim enhancement, destroying vertebral body and transverse process, was revealed on the computed tomographic scan. Magnetic resonance imaging showed high signal on T1- weighted image and iso- and low signal on T2-weighted image for the mass lesion replacing the vertebral bony cortex and marrow space. An open rib biopsy revealed the histopathological changes of Gorham's disease (essential osteolysis), even though only bloody fluid filling the empty space and rib and vertebral transverse process destruction were grossly observed on operation. Even though there was no definite response to radiotherapy and pleurodesis, the patient showed stable condition up to 20 months after diagnosis.
Adult ; Chylothorax/complications/*pathology/radiotherapy ; Female ; Humans ; Magnetic Resonance Imaging/methods ; Osteolysis, Essential/complications/*pathology/*radiotherapy ; Pleurodesis ; Time Factors ; Tomography, X-Ray Computed ; Treatment Outcome

We report a case of mediastinal lymphangioma associated with Gorham's disease in a 38-year-old man who had suffered recurrent clavicular fractures during a seven-year period. Mediastinal widening associated with osteolysis of the clavicles and the sternal manubrium was revealed by chest radiography, while computed tomography demonstrated a cystic anterior mediastinal mass infiltrating mediastinal fat and associated with osseous destruction of the clavicles and manubrium. Chylothorax recurred during the course of the disease.
Adult ; Case Report ; Chylothorax/*complications ; Human ; Lymphangioma/*complications/radiography ; Male ; Mediastinal Neoplasms/*complications/radiography ; Osteolysis, Essential/*complications/pathology/radiography ; Thoracic Vertebrae/*pathology ; Tomography, X-Ray Computed

This study aims to investigate the therapeutic effect of icariin(ICA) on thioacetamide(TAA)-induced femoral osteolysis in rats. RAW264.7 cells were treated with TAA and ICA. Cell counting kit-8(CCK-8) assay was used to detect cell proliferation, and tartrate-resistant acid phosphatase(TRAP) staining to examine the formation of osteoclasts. The expression of TRAP, cathepsin K, c-FOS, and NFATc1 in RAW264.7 cells was determined by Western blot and immunofluorescence method. Thirty-two SD rats were randomized into the control group, TAA group(intraperitoneal injection of TAA at 300 mg·kg~(-1)), ICA group(gavage of ICA at 600 mg·kg~(-1)) and TAA + ICA group(intraperitoneal injection of TAA at 300 mg·kg~(-1) and gavage of ICA at 600 mg·kg~(-1)). Administration was performed every other day for 6 weeks. Body weight and length of femur were recorded at execution. Pathological injury and osteoclast differentiation of femur were observed based on hematoxylin-eosin(HE) staining and TRAP staining, and the changes of bone metabolism-related indexes alkaline phosphatase(ALP), calcium(Ca), phosphorus(P), magnesium(Mg), and cross-linked N-telopeptide of type Ⅰ collagen(NTX-Ⅰ) in serum were detected. Three-point bending test and micro-CT were applied to evaluate the quality of femur, and Western blot to detect the levels of osteoclast-related proteins TRAP, cathepsin K, RANK, RANKL, p38, p-p38, ERK, p-ERK, JNK, p-JNK, c-Fos, and NFATc1. The results showed ICA could inhibit TAA-induced production of TRAP-positive cells, the expression of osteoclast-related proteins, and nuclear translocation of NFATc1. ICA alleviated the weight loss, reduction of femur length, and growth inhibition induced by TAA in SD rats. ICA ameliorated the decline of femur elastic modulus caused by TAA and significantly restored trabecular bone mineral density(BMD), trabecular pattern factor(Tb.Pf), trabecular number(Tb.N), trabecular thickness(Tb.Th), and structure model index(SMI), thus improving bone structure. Western blot results showed ICA suppressed femoral osteoclast differentiation induced by TAA through RANKL-p38/ERK-NFATc1 signaling pathway. ICA inhibits osteoclast differentiation and prevents TAA-induced osteolysis by down-regulating RANKL-p38/ERK-NFAT signaling pathway.
Rats ; Animals ; Osteoclasts ; Cathepsin K/pharmacology* ; Thioacetamide/pharmacology* ; Bone Resorption/pathology* ; Osteolysis/pathology* ; Cell Differentiation ; Rats, Sprague-Dawley ; NFATC Transcription Factors/metabolism*