足部骨肿瘤及瘤样病变的发病特点分析

目的分析足部骨肿瘤及瘤样病变的发病特点。方法分析1993～2008年收治的92例足部骨肿瘤及瘤样病变患者的临床资料,分析足部各种骨肿瘤及瘤样病变的发病特点。结果 92例足部骨肿瘤及瘤样病变患者占同期收治骨肿瘤患者（6 247例）的1.5%,其中良性骨肿瘤及瘤样病变79例,占同期收治所有良性骨肿瘤及瘤样病变患者（3 319例）的4.2%;原发恶性骨肿瘤11例,占同期收治原发恶性骨肿瘤患者（2 167例）的0.5%;2例转移癌患者,占同期收治骨转移癌患者（761例）的0.03%。肿瘤发病于跟骨37例、距骨21例、趾骨18例、跖骨11例、楔骨2例、舟骨2例、骰骨1例。结论足部骨肿瘤及瘤样病变发病率低,良性骨肿瘤及瘤样病变的发病率远高于恶性骨肿瘤。部位以跟骨发病率最高,其次为距骨、趾骨。     

Prognostic value of vertebral lesions detected by magnetic resonance imaging in patients with stage I multiple myeloma

We assessed the role of spinal magnetic resonance imaging (MRI) and bone densitometry as prognostic factors in patients with asymptomatic stage I multiple myeloma (MM) and negative skeletal survey. 55 consecutive patients underwent spinal MRI and 41 of them underwent bone densitometry by dual-energy X-ray absorptiometry (DEXA). Spinal MRI studies showed evidence of bone marrow involvement in 17/55 patients (31%). A diffuse pattern was present in three patients and a focal pattern in 14 patients, nine of them with only one nodular lesion. During a median follow-up of 25 months, 10 patients had disease progression, 8/17 patients with abnormal MRI and 2/38 patients with normal MRI. Median time to disease progression was not reached in both groups but was significantly different for patients with normal and those with abnormal patterns on MRI (P < 0.0001). Lumbar BMD was only slightly decreased compared with normal people (median lumbar Z score -0.43) and was not of prognostic value. Using a multivariate analysis the only two independent significant prognostic parameters were abnormal MRI (P<0.001, HR 30.4, 95% CI 4.3-213) and bone marrow plasmacytosis >20% (P=0.004, HR 16.4, 95% Cl 2.6-104). Thus, spinal MRI but not bone densitometry, appeared to be justified in patients with stage I asymptomatic MM and negative skeletal survey.     

类风湿关节炎骨吸收指标与关节破坏的相关性

类风湿关节炎是以慢性对称性多关节滑膜炎、骨及软骨破坏为主要特征的自身免疫性疾病,其发展过程中的骨代谢异常可导致不同程度骨量丢失和骨破坏,骨破坏的相关指标可反映骨量丢失的严重程度,并可预测疾病发展和关节破坏程度。本文对类风湿关节炎患者骨吸收相关指标与关节破坏相关性的最新进展进行综述。     

Transient bone marrow aplasia associated with non-A, non-B hepatitis

We describe two patients who developed aplastic anemia early in the course of non-A, non-B hepatitis. Spontaneous rapid recovery of bone marrow function occurred in both patients. Although early bone marrow transplantation has been recommended as the treatment of choice in hepatitis-associated aplastic anemia, these patients illustrate the need for a period of expectant observation before undertaking bone marrow transplantation in such patients.     

Fetal and postnatal bone development: reviewing the role of mechanical stimuli and nutrition

Fetal and postnatal bone development is by tradition viewed as a process of bone mineral accretion or an increase in bone mass. Accordingly, previous approaches to bone development in neonatology and early childhood have emphasized the determinants of peak bone mass and their relationship to osteopenia, osteoporosis and fractures in later life. This suggests that the neonatal period and early childhood is an important period for bone mineral accrual, and that peak bone mass may be correlated with subsequent skeletal health. Nevertheless, describing fetal and postnatal bone development just in terms of changes in mass or density means looking at bones as if they were amorphous heaps of calcium and phosphorus. In reality, of course, bones are complex three-dimensional structures. It is therefore important to create conditions that stimulate bones to become more stable. We suggest that functional bone physiology can be used to explain fetal and postnatal bone development and to devise strategies for improved bone development in both premature infants and neonates.     

Radiological aid to clinical diagnosis of Schnitzler’s syndrome: multimodality imaging approach

Schnitzler’s syndrome is a rare combination of chronic urticaria, fever of unknown origin, disabling bone pain, and monoclonal gammopathy. We report a case with an unusual radiological manifestation as a solitary sclerotic lesion of the right iliac bone. Its main features on conventional radiography, computed tomography, and magnetic resonance imaging are described, and the main radiological differential diagnoses are discussed to help with the characterization of this syndrome, which requires a combination of clinical, laboratory, and radiological data. On the other hand, although our patient had an excellent clinical response to anakinra, the sclerotic lesion remained unchanged on follow-up X-ray examinations.     

Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood

Skeletal involvement is generally, but not universally, characteristic of Langerhans cell histiocytosis (LCH). We investigated whether the presence of bone lesions at diagnosis is a prognostic factor for survival in LCH. Nine hundred and thirty‐eight children with multisystem (MS) LCH, both high (386 RO+) and low (RO?) risk, were evaluated for bone lesions at diagnosis. Risk organ (RO+) involvement was defined as: haematopoietic system (haemoglobin <100 g/l, and/or white blood cell count <4·0 × 10⁹/l and/or platelet count <100 × 10⁹/l), spleen (>2 cm below the costal margin), liver (>3 cm and/or hypoproteinaemia, hypoalbuminaemia, hyperbilirubinaemia, and/or increased aspartate transaminase/alanine transaminase). Given the general view that prognosis in LCH worsens with increasing extent of disease, the surprising finding was that in MS+RO+ LCH the probability of survival with bone involvement 74 ± 3% (n = 230, 56 events) was reduced to 62 ± 4% (n = 156, 55 events) if this was absent (P = 0·007). An even greater difference was seen in the subgroup of patients with both liver and either haematopoiesis or spleen involvement: 61 ± 5% survival (n = 105; 52 events) if patients had bony lesions, versus 47 ± 5% (n = 111; 39 events) if they did not (P = 0·014). This difference was retained in multivariate analysis (P = 0·048). Although as yet unexplained, we conclude that bone involvement at diagnosis is a previously unrecognized favourable prognostic factor in MS+RO+ LCH.     

Acute and early effects of triiodothyronine administration on serum markers of bone and mineral metabolism

There have been few studies on acute changes of bone metabolism in humans by thyroid hormone. This study aimed to examine the effects of triiodothyronine on serum markers of bone and mineral metabolism during a 7-d course of daily 75 μg therapy in 14 normal volunteers by drawing blood on 1, 2, 3, 5, and 7 d of therapy. Serum calcium concentrations did not significantly change during the course of therapy, while serum phosphorus concentrations were significantly (p<0.05) decreased from 3.21±0.43 mg/dL (mean±SD) to 2.85±0.46 mg/dL on the 7th d. Serum PTH concentrations were significantly decreased from 339±116 pg/mL to 316±29 pg/mL. Serum concentrations of alkali-phosphatase and bone-specific alkali-phosphatase were not significantly changed, but serum osteocalcin concentrations were significantly increased from 5.71±1.98 mg/dL to 6.73±2.24 mg/dL. Serum carboxy-terminal propeptide of type I collagen concentrations were significantly decreased from 137.8±33.7 μg/L to 119.2±33.6 μg/L. Serum pyridinoline cross-linked telopeptide domain of type I collagen concentrations, a bone resorption marker, were significantly increased from 3.40±0.77 to 3.87±1.05 μg/L, and such significant increase was obtained from the 3rd day. The results indicate that some of bone and mineral markers change rapidly in response to triiodothyronine-induced acute thyrotoxicosis, but the manner of change is not the same as that of chronic thyrotoxicosis.     

Eosinophilic fibrohistiocytic lesion of bone marrow associated with monoclonal gammopathy and osteolytic lesions

We describe a 63-year-old male patient with severe osteoporosis, multiple lytic bone lesions, and monoclonal gammopathy (IgG lambda). Whereas the tentative diagnosis in this case was multiple myeloma, bone marrow trephine biopsies of the iliac crest and from an osteolytic lesion of the tibia both showed a peculiar infiltrate consisting of numerous elongated mast cells, eosinophils, and some plasma cells and lymphocytes. The bone marrow lesions fit the diagnosis of eosinophilic fibrohistiocytic lesion of bone marrow (EFHBM). The patient had no abnormality that could be related to a known allergic disease, and no relationship to drug hypersensitivity could be established. The features of the bone marrow infiltrate and its association with monoclonal gammopathy may suggest a linkage between EFHBM and the monoclonal gammopathy.     

Pathophysiology of myeloma bone disease

Multiple myeloma is a tumor of terminally differentiated plasma cells that home to and expand in the bone marrow. It is the second most common hematologic malignancy, with approximately 16,000 new cases per year, and accounts for an estimated 11,000 deaths in the USA. It is the most common cancer to metastasize to bone, with up to 90% of patients developing bone lesions. The bone lesions are purely osteolytic in nature, and up to 60% of patients develop a pathologic fracture over the course of their disease. Bone disease is a hallmark of multiple myeloma, and the bone disease differs from other bone metastasis caused by other tumors. Although both myeloma and other osteolytic metastasis induce increased osteoclastic bone resorption, in contrast to other tumors, osteoblast activity in myeloma is either severely decreased or absent. The basis for this severe imbalance between increased osteoclastic bone resorption and decreased bone formation resulting from suppressed osteoblastic activity has been a topic of extensive investigation during the last several years. The clinical consequences of this extensive accelerated and imbalanced bone destruction process include bone pain, pathologic fractures, hypercalcemia and spinal cord compression syndromes, which can be devastating for patients and significantly impact overall quality of life and expected survival. In this chapter, we will discuss the pathophysiology underlying bone disease in myeloma. This results from the uncoupling of bone remodeling and is characterized by markedly increased activity of osteoclasts and profound decreased activity of osteoblasts. In addition, we also review the emerging data on novel targeted therapies aimed at ameliorating myeloma bone disease.     

Small-bowel perforation caused by fish bone

A diagnosis of small-bowel perforation, caused by a sharp or pointed foreign body, is rarely made preoperatively because the clinical symptoms are usually nonspecific and can mimic other surgical conditions, such as appendicitis and diverticulitis. We report the case of a 62-year-old woman who experienced severe pain in the right iliac fossa and fever for about five days before arrival at our hospital. The presumptive diagnosis was acute purulent appendicitis and an emergency appendectomy was planned. Swelling and erythema were noted in a segment of the small bowel in the lower right abdomen. A tiny pointed object was found penetrating the inflamed portion of the bowel, which proved to be a sharp fish bone (gray snapper). The bone was removed, followed by segmental resection of the terminal ileum and ascending colon. The postoperative course was uneventful.     

Avascular necrosis of bone after allogeneic bone marrow transplantation: clinical findings, incidence and risk factors

Summary. In the present study we describe the incidence, clinical course, and management of avascular necrosis of bone following allogeneic bone marrow transplantation, and identify risk factors related to its development. All patients developing avascular necrosis of bone after allogeneic bone marrow transplantation between January 1974 and September 1992 were included in the analysis and were studied using the Hôpital Saint Louis Bone Marrow Transplant Database and hospital records. 27/727 allogeneic transplant recipients developed avascular necrosis leading to an 8·1% incidence at 5 years, by product limit estimate, ranging from 5% to 11·2%. Symptoms developed 119–1747 d (median 398 d) after transplantation. In these 27 patients a total of 52 joints were affected (mean 1·92 per patient, range 1–7). The hip joint was most often affected (69% of patients). All patients had joint pain that led to diagnosis by means of standard radiographs with or without the help of technetium-99 scans and/or magnetic resonance imaging. All but three patients received steroid therapy for acute graft-versus-host disease. Among 10 factors tested, three were shown to be significantly linked to an increased risk for developing avascular necrosis by multivariate analysis: male gender (relative risk (RR) 4·2, P= 0·002), age older than 16 (RR = 3·87, P= 0·004), and acute graft-versus-host disease requiring steroid therapy (RR = 6·30, P= 0·0002). 10 patients (37%) required joint replacement within 19 months (range 2–42) following diagnosis of avascular necrosis. In conclusion, avascular necrosis of bone is a frequent late complication of allogeneic bone marrow transplantion causing significant morbidity and requiring replacement surgery in one-third of affected patients. In this 18-year single-centre survey, older age, male gender and steroid therapy given for acute graft-versus-host disease were shown to independently increase the risk of avascular necrosis of bone.     

Predialysis calcitriol administration: effects on pre- and post-transplant renal osteodystrophy

Twins with parallel loss of kidney function and moderate hyperparathyroid bone disease were participants in a double-blind study where twin A was given placebo and twin B calcitriol. After 8 months, A's bone disease had not improved, while B's bone had normalized. Thereafter, both received calcitriol until kidney transplantation 11 months later, when both had normal bone structure. Two years after transplantation, both twins had hyperparathyroid bone disease, but A had more pronounced changes. This report illustrates our findings in larger series: When started early in the course of renal failure, calcitriol can reverse pre-transplant hyperparathyroid bone disease and also influence post-transplant bone disease.     

The use of fresh-frozen bone in oral surgery: a clinical study of 14 consecutive cases

Background

Although autologous bone is considered the gold standard among the grafting materials used in implant therapy, it does have a number of drawbacks, in particular morbidity at the site of donation and the limited amount of bone available. To overcome these limitations a number of alternative bone materials have been employed in the last few years. In this study we report the results of the use of homologous fresh-frozen bone from a tissue bank in patients undergoing reconstruction of bone defects in the oral cavity.

Material and methods

Between June 2004 and October 2008, 14 consecutive patients underwent bone reconstruction with fresh-frozen bone from a tissue bank. Four to eight months after surgery, implants were placed in the newly formed bone.

Results

No problems were recorded during the post-operative course. In all cases treatment was successful and osteointegrated implants were placed in the newly formed bone after 4–8 months. All implants showed good osteointegration (100% overall success rate, mean follow-up 20 months), allowing loading with a fixed cemented prosthesis.

Conclusions

Our results support the previous findings that homologous fresh-frozen bone can be considered a valid alternative to autologous bone for the reconstruction of bone defects in the oral cavity in patients undergoing implant therapy.     

Enfermedad de Pyle: un modelo humano de homeostasis corticotrabecular diferenciada

Pyle's disease (OMIN number 265900) is a metaphyseal dysplasia of benign course, inherited with an autosomal recessive pattern. Some 30 genuine cases have been described so far. The cause of this process has been known since 2016, when its relationship to mutations in the gene encoding the sFRP protein, a known inhibitor of the Wnt pathway, was discovered. We report the case of a 58-year-old man, diagnosed with Pyle's disease based on his clinical and radiographic characteristics, whose phenotype suggested a differential control of cortical and trabecular bone homeostasis.     

不同投氟方式对诱导性骨形成影响的实验研究

本文研究了不同投氟方式(连续、间断摄氟)对大鼠诱导性异位骨形成的影响。组织学及骨形态计量学研究结果显示:间断投予氟化钠(NaF)具有与持续投予 NaF 相同的刺激骨形成的作用,且诱导性骨质的结构为成熟的板层骨。即未观察到持续投氟组所出现的编织骨和类骨质。作者认为氟对骨细胞和骨质结构的影响主要取决于骨中氟积聚的程度。     

Autologous bone marrow transplantation for intra-abdominal extramedullary plasmacytoma

Summary A patient with an extramedullary plasmacytoma of the omentum and ovaries is described. In spite of the presence of a very high concentration of paraprotein (103.5 g/l IgG k), bone marrow involvement was not demonstrated either at presentation or during the course of the disease. After debulking surgery, chemotherapy (vincristine, prednisone, and cyclophosphamide) was initiated. Although this resulted in considerable improvement, the paraproteinemia persisted. After ten courses of chemotherapy there was recurrence of the tumor with involvement of the lymph nodes in the right axilla. Chemotherapy was changed to a CHOP regimen (cyclophosphamide, adriamycin, vincristine, and prednisone), followed by autologous bone marrow transplantation with the BEAM regimen (BCNU, etoposide, cytarabine, melphalan) as conditioning therapy. The patient was still in complete remission 1 year after transplantation. This case demonstrates that an extramedullary plasmacytoma may become manifest as extensive but localized disease with high levels of paraprotein, and that autologous bone marrow transplantation as a therapeutic modality can lead to prolonged complete remission of the disease.     

Abnormal bone remodelling in patients with myelomatosis and normal biochemical indices of bone resorption.

We studied bone biopsies from 26 patients with myelomatosis with apparently normal skeletal metabolism. Quantitative histomorphometric measurements suggested that skeletal disease was progressive despite normocalcaemia and normal urinary excretion rates of calcium and hydroxyproline. When biopsies were divided according to the involvement of marrow by plasma cells, bone resorption--as judged by the eroded surface--increased significantly the greater plasma cell burden. Osteoclasts were frequent with moderate tumour burdens, but there was no further increase in the number of osteoclasts when plasma cell infiltration increased by more than 50% of bone marrow. Contrary to expectation, the numbers of osteoblasts and bone formation rates were increased with bone biopsies with moderate tumour burden, but were markedly lower when plasma cell infiltration occupied more than 50% of bone marrow, due to a decreased functional capacity of osteoblasts. We conclude that skeletal bone disease in myeloma is commonly progressive despite apparently stable bone disease as judged by biochemical measurements. The major mechanism of bone loss in myelomatosis is increased osteoclastic resorption but decreased bone formation contributes to bone loss with heavy plasma cell burdens. Urinary excretion of calcium and hydroxyproline provide insensitive indices of bone resorption in myelomatosis.     

A benign course of multicentric Castleman's disease with involvement of the spleen and bone marrow

The multicentric variant of Castleman's disease (MCCD) is associated with a rapidly progressive and fatal course. The case described herein manifested unique clinical and histological features. Initial presentation as isolated splenomegaly was subsequently followed by widespread organ involvement, including lymph nodes and bone marrow. In spite of this, the patient had a very benign course of her disease. The case serves to expand even further the already wide clinical spectrum of Castleman's disease.     

A New Anorganic Equine Bone Substitute for Oral Surgery: Structural Characterization and Regenerative Potential

Different xenogeneic inorganic bone substitutes are currently used as bone grafting materials in oral and maxillo-facial surgery. The aim of the present study was to determine the physicochemical properties and the in vivo performance of an anorganic equine bone (AEB) substitute. AEB is manufactured by applying a process involving heating at >300 °C with the aim of removing all the antigens and the organic components. AEB was structurally characterized by scanning electron microscopy (SEM), X-ray diffraction (XRD), X-ray fluorescence (XRF), and Fourier-transformed infrared (FT-IR) spectroscopy and compared to the anorganic bovine bone (ABB). In order to provide a preliminary evaluation of the in vivo performance of AEB, 18 bone defects were prepared and grafted with AEB (nine sites), or ABB (nine sites) used as a control, in nine Yucatan Minipigs. De novo bone formation, residual bone substitute, as well as local inflammatory and tissue effects were histologically evaluated at 30 and 90 days after implantation. The structural characterization showed that the surface morphology, particle size, chemical composition, and crystalline structure of AEB were similar to cancellous human bone. The histological examination of AEB showed a comparable pattern of newly formed bone and residual biomaterial to that of ABB. Overall, the structural data and pre-clinical evidence reported in the present study suggests that AEB can be effectively used as bone grafting material in oral surgery procedures.