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Fulltext Severe presentation of non-ossifying fibroma of the femur in osteoglophonic dysplasia

Kumar A, Chong YT, Jamil K, Rusli E

BMJ Case Rep, 2021 Nov 05;14(11).
PMID: 34740908 DOI: 10.1136/bcr-2021-245415

Osteoglophonic dwarfism, also known as osteoglophonic dysplasia (OD), is an uncommon skeletal dysplasia with an autosomal dominant mode of inheritance, which equally affects boys and girls. OD is saliently featured by craniosynostosis, dysmorphic facial features, impacted mandibular teeth, rhizomelic limb shortening and non-ossifying fibromas habitually at the metaphyseal regions, which usually disappear after skeletal maturity. The long bones in OD are portrayed by this distinguishable 'hollowed-out' appearance with metaphyseal cystic defects that have a natural history of spontaneous resolution. We report a case of a rare and unusual presentation of OD in a 23-year-old woman whom has been diagnosed with OD during her early childhood. She presented with a progressively enlarging right thigh swelling associated with pain for the past 1 year. Her right femur plain radiograph revealed diffuse lysis of the whole femur with cortical thinning. MRI revealed multiple bilateral femur benign cystic lesion synonymous with a severe spectrum of OD. She was started on a trial of oral bisphosphonates, which led to a significant improvement in pain.

Matched MeSH terms: Femur/pathology
Marrow Fat-Cortical Bone Relationship in β-Thalassemia: A Study Using MRI

Ismail UN, Azlan CA, Khairullah S, Azman RR, Omar NF, Md Shah MN, et al.

J Magn Reson Imaging, 2024 Dec;60(6):2447-2456.
PMID: 38556790 DOI: 10.1002/jmri.29366

BACKGROUND: Growing evidence suggests that marrow adipocytes play an active role in the regulation of bone metabolism and hematopoiesis. However, research on the relationship between bone and fat in the context of hematological diseases, particularly β-thalassemia, remains limited.
PURPOSE: To investigate the relationship between marrow fat and cortical bone thickness in β-thalassemia and to identify key determinants influencing these variables.
STUDY TYPE: Prospective.
SUBJECTS: Thirty-five subjects in four subject groups of increasing disease severity: 6 healthy control (25.0 ± 5.3 years, 2 male), 4 β-thalassemia minor, 13 intermedia, and 12 major (29.1 ± 6.4 years, 15 male).
FIELD STRENGTH/SEQUENCE: 3.0 T, 3D fast low angle shot sequence and T1-weighted turbo spin echo.
ASSESSMENT: Analyses on proton density fat fraction (PDFF) and R2* values in femur subregions (femoral head, greater trochanter, intertrochanteric, diaphysis, distal) and cortical thickness (CBI) of the subjects' left femur. Clinical data such as age, sex, body mass index (BMI), and disease severity were also included.
STATISTICAL TESTS: One-way analysis of variance (ANOVA), mixed ANOVA, Pearson correlation and multiple regression. P-values <0.05 were considered significant.
RESULTS: Bone marrow PDFF significantly varied between the femur subregions, F(2.89,89.63) = 44.185 and disease severity, F(1,3) = 12.357. A significant interaction between subject groups and femur subregions on bone marrow PDFF was observed, F(8.67,89.63) = 3.723. Notably, a moderate positive correlation was observed between PDFF and CBI (r = 0.33-0.45). Multiple regression models for both PDFF (R2 = 0.476, F(13,151) = 10.547) and CBI (R2 = 0.477, F(13,151) = 10.580) were significant. Significant predictors for PDFF were disease severity (βTMi = 0.36, βTMa = 0.17), CBI (β = 0.24), R2* (β = -0.32), and height (β = -0.29) while for CBI, the significant determinants were sex (β = -0.27), BMI (β = 0.55), disease severity (βTMi = 2.15), and PDFF (β = 0.25).
DATA CONCLUSION: This study revealed a positive correlation between bone marrow fat fraction and cortical bone thickness in β-thalassemia with varying disease severity, potentially indicating a complex interplay between bone health and marrow composition.
EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 3.

Matched MeSH terms: Femur/pathology
A "healthy apatite" for bone repair

Best SM, Patel N, Porter AE, Bonfield W

Med J Malaysia, 2004 May;59 Suppl B:129-30.
PMID: 15468852

Bone is unique in its ability to adapt structure to functional requirements, but as is all too obvious in an ever-ageing population it is susceptible to a number of degenerative diseases. Therefore there is an increasing need for materials for bone replacement. Clearly, the ideal material with which to replace bone, would be bone itself, but the major problem now facing us is that there is an insufficient supply of the natural bone to satisfy the clinical requirements. Hence, there is a need for the development of chemically synthesised bone graft substitutes

Matched MeSH terms: Femur/pathology
Microscopic evaluation of the natural coral (Porites spp.) post-implantation in sheep femur

Fadilah A, Zuki AB, Loqman MY, Zamri-Saad M, Al-Salihi KA, Norimah Y, et al.

Med J Malaysia, 2004 May;59 Suppl B:127-8.
PMID: 15468851

The study was carried out with the aim to evaluate natural coral (Porites spp.) implanted in sheep femur microscopically. Twelve adult, male sheep were used in this study. The defect area was implanted with coral and monitored for up to 12 weeks. The sheep were euthanased at 2,4,8, and 12 weeks post-implantation. Microscopically, natural coral implanted into bone tissue have shown gradual resorption and progressively replaced by new bone. At 12 weeks post-implantation, the implanted site was almost completely surrounded by mature bone. The results showed that natural coral was found to be a biodegradable and osteo-conductive biomaterial, which acted as a scaffold for a direct osteoblastic apposition.

Matched MeSH terms: Femur/pathology*
Filling of the resultant cavity after curettage of benign bone tumours is still controversial

Ismail TB, Mahmoud MM, Ahmed OE, Bola AH, Bahaa ZH

Med J Malaysia, 2023 Mar;78(2):163-170.
PMID: 36988525

INTRODUCTION: Benign bone tumours occur most commonly during the first through third decades of life and often weaken the bones, which may predispose them to pathological fractures. Great diversity and debate in the management of primary bone tumours are based on the tumour extent. There has been an increasing trend toward the intra-operative filling of these lesions. We hypothesised that in some benign bone tumours, filling the resulting cavity after curettage was unnecessary. This study was carried out to determine whether it is necessary to fill the resultant cavity after the curettage of benign bone tumours and to represent various fillers.
MATERIALS AND METHODS: A retrospective study of patients diagnosed as benign bone tumours according to the Enneking classification who underwent simple or extended curettage at Menoufia university-Orthopedic Oncology Division (with or without grafting or filling) during the surgical treatment (Jan 2015 to Feb 2020). A review of the medical records was done. Lesions' size (length, width and depth) was measured on plain radiographs using the image j program. When applicable, degrees of filling of the resultant cavity were classified into four categories, according to Modified Neer's classification. Functional evaluation using the musculoskeletal tumour society (MSTS) score was reviewed.
RESULTS: Overall, 88 patients diagnosed with a primary bone tumour and who received the surgical intervention were included in the study. The mean age of the patients was 22.61+13.497 (3-58) years. There were 48 males and 40 females (54 right and 34 left). The mean follow-up period was 28.09+16.13 months. The most common location was the distal femur in 15 patients, the proximal femur in 10 patients and the proximal tibia in 12 patients. The most common diagnosis was giant cell tumour in 20 patients, followed by UBC in 19 patients, ABC in 15 patients and enchondroma in 13 patients. Twenty-three patients had simple curettage, while 65 patients had extended curettage. Mean MSTS was 28.78±1.68. Fifty-five lesions were classified according to modified Neer's classification.Thirtty-two patients were classified as type 1 with complete healing,22 patient was classified as type 2 with partial healing, and only one was classified as a recurrent lesion. Seven patients (7.9%) developed local recurrences.
CONCLUSION: Filling the resulting cavity after the removal of the pathological tissues is usually necessary but not always required. This is determined by the type of lesion and the size of the resulting cavity following curettage. Individualised surgery is required; additional fixation should be considered.

Matched MeSH terms: Femur/pathology
Gross, radiology and ultrasonographic evaluation of coral post-implantation in sheep femur

Fadilah A, Zuki AB, Loqman MY, Zamri-Saad M, Norimah Y, Asnah H

Med J Malaysia, 2004 May;59 Suppl B:178-9.
PMID: 15468876

The study was carried out to evaluate macroscopically the ability of coral to repair a large size bone defect. A total 12 adult, male sheep were used in the study. The large bone defect (2.5cm x 0.5cm x 0.5cm) was created surgically on the left proximal femur and replaced by a block of coral (Porites sp.). Radiographs were obtained immediately after surgery and at 2, 4, 8 and 12 weeks post-implantation. Ultrasonographic examinations were carried out every 2 weeks after implantation up to 12 weeks using ultrasound machine (TOSHIBA Capasee II) connected with 7MHz frequency transducer. The sheep were euthanased at 2, 4, 8, and 12 weeks post-implantation and the bone examined grossly. Both ultrasonographs and radiographs taken at 8 and 12 weeks showed that the implants had been resorbed and left the space that much reduced in size. There was no sign of implant rejection observed in all animals. The results showed that processed coral has potential to become bone substitute for reconstructive bone surgery.

Matched MeSH terms: Femur/pathology
Microstructure and properties of crystalline bioglass compositions prepared by polymeric route

Doreya MI, Mona EW, Afaf ES, Hanan HB

Med J Malaysia, 2004 May;59 Suppl B:21-2.
PMID: 15468799

The standard bioglass composition GS45 as well as with excess silica GS50 or with the addition of 5% titanium oxide GS45+Ti5, were prepared by the polymeric route. The different glass components were added to the formed polymer. Firing at 700 degrees C gave an amorphous product with microporous texture that readily crystallizes out at 900 degrees C. The prepared materials were highly porous with two modes of pore system micro-pores and macro-pores with a size ranging between 100 microm to 0.006 microm and a porosity reaching 73%. The measured bulk density was between 0.36 to 1.1g/cm3. The fired material preserved the former structure of the polymer precursor. Biocompatibility was verified in vitro and vivo. IR of the specimens previously immersed in SBF revealed the formation of apatite like layer. While the histology sections of implants in rate femurs showed new bone tissue or bone trabeculae after 21 days.

Matched MeSH terms: Femur/pathology
Fulltext Cryptococcal osteomyelitis of the femur: a case report and review of literature

Zainal AI, Wong SL, Pan KL, Wong OL, Tzar MN

Trop Biomed, 2011 Aug;28(2):444-9.
PMID: 22041767 MyJurnal

Fungal osteomyelitis is a rare opportunistic infection. It exhibits some clinical and radiological similarities to several other bone pathologies. A diagnostic delay may result in significant increase in morbidity. We report a case of a 37-year-old man with underlying hypogammaglobulinaemia presented with isolated cryptococcal osteomyelitis of the femur.

Matched MeSH terms: Femur/pathology*
Fulltext Morphological study of the newly designed cementless femoral stem

Baharuddin MY, Salleh ShH, Zulkifly AH, Lee MH, Mohd Noor A

Biomed Res Int, 2014;2014:692328.
PMID: 25025068 DOI: 10.1155/2014/692328

A morphology study was essential to the development of the cementless femoral stem because accurate dimensions for both the periosteal and endosteal canal ensure primary fixation stability for the stem, bone interface, and prevent stress shielding at the calcar region. This paper focused on a three-dimensional femoral model for Asian patients that applied preoperative planning and femoral stem design. We measured various femoral parameters such as the femoral head offset, collodiaphyseal angle, bowing angle, anteversion, and medullary canal diameters from the osteotomy level to 150 mm below the osteotomy level to determine the position of the isthmus. Other indices and ratios for the endosteal canal, metaphyseal, and flares were computed and examined. The results showed that Asian femurs are smaller than Western femurs, except in the metaphyseal region. The canal flare index (CFI) was poorly correlated (r < 0.50) to the metaphyseal canal flare index (MCFI), but correlated well (r = 0.66) with the corticomedullary index (CMI). The diversity of the femoral size, particularly in the metaphyseal region, allows for proper femoral stem design for Asian patients, improves osseointegration, and prolongs the life of the implant.

Matched MeSH terms: Femur/pathology
The effects of Labisia pumila on postmenopausal osteoporotic rat model: Dose and time-dependent micro-CT analysis

Effendy NM, Khamis MF, Soelaiman IN, Shuid AN

J Xray Sci Technol, 2014;22(4):503-18.
PMID: 25080117 DOI: 10.3233/XST-140441

Postmenopausal osteoporosis is best treated and prevented by estrogen replacement therapy (ERT). Although effective, ERT may cause breast cancer, uterine cancer and cardiovascular problems. Labisia pumila var. alata (LP), a herb with phytoestrogenic, antioxidative and anti-inflammatory effects has potential as an ERT alternative.

Matched MeSH terms: Femur/pathology
Cell based therapy for osteoarthritis in a sheep model: gross and histological assessment

Alfaqeh H, Norhamdan MY, Chua KH, Chen HC, Aminuddin BS, Ruszymah BH

Med J Malaysia, 2008 Jul;63 Suppl A:37-8.
PMID: 19024972

This study was to determine if autologous bone marrow mesenchymal stem cells (BMSCs) cultured in chondrogenic medium could repair surgically induced osteoarthritis. Sheep BMSCs were cultured in medium containing 5ng/ml TGFbeta3 + 50ng/ml IGF-1 for three weeks. The cultured cells were then suspended at density of 2x10(6) cell/ml and injected intraarticularly into the osteoarthritic knee joint. After six weeks, the distal head of the femur and the proximal tibial plateau were removed and stained with H&E. The results indicated that knee joints treated with autologous BMSCs cultured in chondrogenic medium showed clear evidence of articular cartilage regeneration in comparison with other groups.

Matched MeSH terms: Femur/pathology
Metallic materials stimulating bone formation

Kokubo T

Med J Malaysia, 2004 May;59 Suppl B:91-2.
PMID: 15468833

Metallic materials implanted into bone defects are generally encapsulated by a fibrous tissue. Some metallic materials such as titanium and tantalum, however, have been revealed to bond to the living bone without forming the fibrous tissue, when they were subjected to NaOH solution and heat treatments. Thus treated metals form bone tissue around them even in muscle, when they take a porous form. This kind of osteoconductive and osteoinductive properties are attributed to sodium titanate or tantalate layer on their surfaces formed by the NaOH and heat treatments. These layers induce the deposition of bonelike apatite on the surface of the metals in the living body. This kind of bioactive metals are useful as bone substitutes even highly loaded portions, such as hip joint, spine and tooth root.

Matched MeSH terms: Femur/pathology
Bone-related complications of transfusion-dependent beta thalassemia among children and adolescents

Mirhosseini NZ, Shahar S, Ghayour-Mobarhan M, Banihashem A, Kamaruddin NA, Hatef MR, et al.

J. Bone Miner. Metab., 2013 Jul;31(4):468-76.
PMID: 23475127 DOI: 10.1007/s00774-013-0433-1

Thalassemia and the blood transfusion complications associated with it predispose children to poor bone health. This study was conducted to determine the prevalence of bone-related abnormalities and identify the bone health predictors within this population. One hundred and forty transfusion-dependent beta thalassemic subjects 8-18 years old in Mashhad, Iran, participated in this cross-sectional study. Anthropometric measures, dietary intake, bone-related biomarkers and bone densitometry, were assessed. The incidence of underweight and short stature was 33.6 and 41.4 %, respectively, which were indicators of malnutrition among thalassemic subjects in this study. Low bone density was detected in the lumbar spine and femoral region in 82 and 52 % of subjects, respectively. Hypocalcemia and hypophosphatemia were seen in 22 and 18.2 %, whilst vitamin D deficiency was present in more than 85 % of thalassemic children and adolescents. The relationships between weight, height and other anthropometric indices, serum calcium and bone markers, intake of macronutrients, zinc and vitamin E with bone mineral density (BMD) and bone mineral content (BMC) in the lumbar spine and femoral area were positively related, indicating that better nutritional status were associated with higher BMD and BMC values. Puberty, gender and serum osteocalcin were negative predictors for BMD and BMC values, whereas age, weight and height were the positive predictors. High incidence of low bone density and deficit in other aspects of bone health among thalassemia patients makes routine bone health assessment necessary for this vulnerable group. Considering influencing factors, dietary counseling and preventive supplementation therapy for this high risk group of children and adolescents may be necessary, although this should be assessed by intervention studies.

Matched MeSH terms: Femur/pathology
Fulltext A novel design, analysis and 3D printing of Ti-6Al-4V alloy bio-inspired porous femoral stem

Mehboob H, Tarlochan F, Mehboob A, Chang SH, Ramesh S, Harun WSW, et al.

J Mater Sci Mater Med, 2020 Aug 20;31(9):78.
PMID: 32816091 DOI: 10.1007/s10856-020-06420-7

The current study is proposing a design envelope for porous Ti-6Al-4V alloy femoral stems to survive under fatigue loads. Numerical computational analysis of these stems with a body-centered-cube (BCC) structure is conducted in ABAQUS. Femoral stems without shell and with various outer dense shell thicknesses (0.5, 1.0, 1.5, and 2 mm) and inner cores (porosities of 90, 77, 63, 47, 30, and 18%) are analyzed. A design space (envelope) is derived by using stem stiffnesses close to that of the femur bone, maximum fatigue stresses of 0.3σys in the porous part, and endurance limits of the dense part of the stems. The Soderberg approach is successfully employed to compute the factor of safety Nf > 1.1. Fully porous stems without dense shells are concluded to fail under fatigue load. It is thus safe to use the porous stems with a shell thickness of 1.5 and 2 mm for all porosities (18-90%), 1 mm shell with 18 and 30% porosities, and 0.5 mm shell with 18% porosity. The reduction in stress shielding was achieved by 28%. Porous stems incorporated BCC structures with dense shells and beads were successfully printed.

Matched MeSH terms: Femur/pathology*
The use of delta-tocotrienol and lovastatin for anti-osteoporotic therapy

Abdul-Majeed S, Mohamed N, Soelaiman IN

Life Sci, 2015 Mar 15;125:42-8.
PMID: 25534439 DOI: 10.1016/j.lfs.2014.12.012

Statins are competitive inhibitors of HMGCoA reductase and are commonly used as antihypercholesterolemic agents. Experimental studies clearly demonstrate the beneficial effects of statins on bone. Tocotrienols have also been shown to have anti-osteoporotic effects on the skeletal system. This study was conducted to observe the effect of a combination of delta-tocotrienol and lovastatin on structural bone histomorphometry and bone biomechanical strength in a postmenopausal rat model at clinically tolerable doses, and to compare it with the effect of delta-tocotrienol or lovastatin.

Matched MeSH terms: Femur/pathology
Fulltext Time and dose-dependent effects of Labisia pumila on the bone strength of postmenopausal osteoporosis rat model

Mohd Effendy N, Abdullah S, Yunoh MF, Shuid AN

BMC Complement Altern Med, 2015 Mar 12;15:58.
PMID: 25887391 DOI: 10.1186/s12906-015-0567-x

BACKGROUND: Post-menopausal osteoporosis has long been treated and prevented by estrogen replacement therapy (ERT). Despite its effectiveness, ERT is associated with serious adverse effects. Labisia pumila var. alata (LP) is a herb with potential as an alternative agent to ERT due to its phytoestrogenic, antioxidative and anti-inflammatory effects on bone. This study aimed to determine the effects of LP supplementation on bone biomechanical strength of postmenopausal osteoporosis rat model.

METHODS: Ninety-six female Sprague-Dawley rats aged 4 to 5 months old were randomly divided into six groups; six rats in the baseline group (BL) and eighteen rats in each group of; Sham- operated (Sham), ovariectomised control (OVXC) and ovariectomised with daily oral gavages of Premarin at 64.5 μg/kg (ERT), LP at 20 mg/kg (LP20) and LP at 100 mg/kg (LP100) respectively. These groups were subdivided into three, six and nine weeks of treatment periods. Rats in BL group were euthanized before the start of the study, while other rats were euthanized after completion of their treatments. Femora were dissected out for biomechanical strength analysis using Instron Universal Model 5848 Micro Tester.

RESULTS: OVXC group showed deterioration in the bone biomechanical strength with time. Both ERT and LP supplemented rats showed improvements in bone strength parameters such as maximum load, displacement, stiffness, stress, and Young Modulus. The most improved bone strength was seen in rats given LP at the dose of 100 mg/kg for nine weeks.

CONCLUSION: LP supplementation at 100 mg/kg was more effective than ERT in reversing ovariectomy-induced bone biomechanical changes.

Matched MeSH terms: Femur/pathology
Effect of Cissus quadrangularis Linn on the development of osteopenia induced by ovariectomy in rats

Potu BK, Nampurath GK, Rao MS, Bhat KM

Clin Ter, 2011;162(4):307-12.
PMID: 21912817

The aim of our study was to see the efficacy of petroleum ether extract of Cissus quadrangularis (CQ) on development of osteopenia in ovariectomy induced Wistar rats.

Matched MeSH terms: Femur/pathology
50% Ethanol extract of Orthosiphon stamineus modulates genotoxicity and clastogenicity induced by mitomycin C

Al-Dualimi DW, Shah Abdul Majid A, Al-Shimary SFF, Al-Saadi AA, Al Zarzour R, Asif M, et al.

Drug Chem Toxicol, 2018 Jan;41(1):82-88.
PMID: 28635332 DOI: 10.1080/01480545.2017.1317785

Herbal products contain a variety of compounds which may be useful in protecting against cellular damage caused by mutagens. Orthosiphon stamineus (O.s) also known as Cat whiskers. The herb has been shown anti-oxidative properties and can modulate key cellular proteins that have cytoprotective effect. The study aimed to evaluate the effects of different doses (250, 500 and 1000 mg kg-1) of 50% ethanol extract of O.s (Et. O.s) on micro-nucleated polychromatic erythrocytes (MNPCE), Polychromatic to normachromatic erythrocytes ratio (PCE/NCE), Mitotic index (MI), and Chromosomal aberration (CA) in Bab/c mice. Moreover, these parameters were used to evaluate the anti-genotoxic and clastogenic potencies of (Et. O.s) against mitomycin c (MMC) that interact with biological molecules and induce genotoxic and clastogenic disorders in non-tumor cells. MMC (4 mg kg-1) was injected intraperitoneally (i.p.) to the mice before and after treatment with three different doses of (Et. O.s). The results indicated that the extract at different doses did not show significant (p ≥ 0.05) differences in (MNPCE), (PCE/NCE) ratios, and (CA) values. The higher doses sowed high (MI) values compared with untreated control group. MMC showed significant increase (p ≤ 0.001) in (MNPCE), (CA) and reduce (PCE/NCE) and (MI) values compared with untreated control group. Treatment with (Et. O.s) at different doses before and after MMC injection showed to modulate MNPCE, PCE/NCE ratios, CA and MI values in mice bone marrow cells suggesting genoprotective potential of this plant extract.

Matched MeSH terms: Femur/pathology

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