Limb Long Bones Research Articles

Comparative study on effect of 8-prenlynaringenin and narigenin on activity of osteoclasts cultured in vitro

To compare the effects of 8-prenylnaringenin (PNG) and naringenin (NG) on the activity and apoptosis of osteoclasts cultured in vitro, in order to study physiological activity of 8-prenyl perssad. Osteoclasts were separated from long-limb bones of newly born rabbits, cultured in alpha-MEM containing 10% FBS, and then added with PNG and NG with the concentration of 1 x 10(-5) mol x L(-1). They were stained with TRAP and determined for enzymatic activity with TRAP after 4 d, and analyzed by toluidine blue staining after 7 d. The apoptotic osteoclasts were analyzed by Annexin V-FITC staining after 2, 4, 8, 12, 24, 36, and 48 hours, to observe their apoptosis. Their total RNAs were extracted, and analyzed for TRAP and Cathepsin K expressions by Real-time RT-PCR. Compared with the control group, both of the PNG group and the NG group showed much less osteoclasts (TRAP positive cells), lower TRAP activity and TRAP and Cathepin K (CTSK) expression, and smaller number of bone resorption pits and areas. The PNG group show lower indexes than the NG group. Additionally, the PNG group reached the apoptotic peak of osteoclasts at 12 h after drug administration, whereas the NG group reached after 24 h. And the former had more apoptotic cells than the latter. 8-PNG is much more active than NG in inhibiting the resorption of osteoclasts and inducing apoptosis of osteoclasts. Their only difference lies in 8-prenyl perssad, which is proved to be able to enhance the anti-bone resorption activity of 8-prenylnarigenin.

INTRAMEDULLARY INTERLOCKING NAILING WITHOUT IMAGE INTENSIFIER AND ORTHO TABLE

Interlocking nails are the gold standard treatment of fractures of shaft of long bones of lower limbs. It is also frequently performedfor most of the humerus fractures. The procedure is commonly performed using an image intensifier and orthotable. These are expensive andare not readily available in peripheral/field hospitals especially in resource – poor countries. Design: Retrospective study. Setting: FieldHospital Muzaffarabad and Combined Hospital Kharian. Period: Sep 2007 to July 2011. Patients & Methods: 138 consecutive cases offractures of femur, tibia and humerus shafts fixed with I/M I/L Nails in a field hospital. Reduction was achieved by open method in 87 (96.66%)cases of femur, 24 (60%) case of tibia and 5(62.5%) of humerus. Results: There were 34 females and 100 males’ ratio 1:2.94. All the cases wereadults with mean age 38.2 years and range was 16-78 years. Bones fixed were femur (90) 65.21%, tibia (40) 28.21%, and humerus (8) 5.70%.Fracture line was transverse in 104 (75.3%) and communited 10 (7.2%). Fractures were closed 112 (81.2%) and 26 (18.8%) of case. Recentfractures were 122 (88.4%) and old non united 16 (11.6%). Mean follow up period was 06 months - range 3 months to 1 ½ years. Complicationswere failure to achieve distal interlocking 6 cases, infection 3 cases. Union time averaged 3.5 months. Conclusions: It is therefore concludedthat I/M I/L nailing can be done without image and traction table.

Early diagnosis and treatment of combined thoracoabdominal injury

Objective To summarize the experience in early diagnosis and emergency treatment for combined thoracoabdominal injury (CTI). Methods Clinical data of 58 cases of CTI admitted to our hospital from June 2001 to August 2009 were analyzed retrospectively.All the patients were treated by closed drainage of thoracic cavity,of which 12 cases were treated with thoracotomy,31 with laparotomy,seven with thoracic and abdominal incisions and three with combined thoracoabdominal incision.Internal fixation for long limb bones was performed in 27 cases,spinal laminectomy and pedicle screw fixation in five and amputation in one. Results After operation,six cases were found to have adult respiratory distress syndrome (ARDS) and 12 cases were complicated with multiple organ dysfunction syndrome (MODS).Fifty-three cases were cured and five died. Conclusions CTI based multiple injuries are severe and complicated.CT scan in the early stage plays a significant role in the diagnosis of CTI and closed drainage of thoracic cavity is an important assurance of the security of abdominal operation.Early diagnosis,correct choice of operation procedures and reasonable handling order of injured organs can improve the successful rescue rate for multiple injury patients. Key words: Multiple trauma; First aid; Combined thoracoabdominal injury

Multicentric Giant Cell Tumor: Metachronous Central and Peripheral Involvement

Giant cell tumors are rare in the head and neck region. The most frequently involved sites of giant cell lesions in the head and neck are the maxilla and mandible, whereas the sphenoid and temporal bones are rarely involved. This tumor is usually located in the long bones of limbs. Reparative granuloma and brown tumor of hyperparathyroidism must be included in the differential diagnosis. Here we report the clinical and radiologic findings of a multicentric giant cell tumor with skull base involvement in a female patient. This case report demonstrates the similar pathophysiology of peripheral and central giant cell tumors.

Bone Infarcts in a Case of Chronic Myeloid Leukemia: Chronic Phase

Dear Editor, Infarcts are commonly seen in spleen, and rarely in myocardium and brain, in patients of chronic myeloid leukemia (CML). Bone infarcts, manifesting as severe debilitating pain as an initial presentation of CML have not been reported. We present a case of CML in chronic phase whose initial presentation was severe bone pains with inability to walk. Tc-99m MDP bone scan revealed heterogenous areas of bone infarct involving long bones of both upper and lower limbs. She was treated with Imatinib and Ecosprin. She became complete pain-free in 4 months. This case highlights the unusual presentation of CML-CP and complete recovery with standard therapy. A 32-years old woman presented with diffuse bony-pains involving bilateral upper and lower limbs for 3 weeks duration. She was not able to perform her daily routine activities because of severe pain (ECOG performance status 3). Clinical examination revealed mild pallor and splenomegaly (spleen size 8 cm below costal margin). Hemogram revealed hemoglobin 9.4 g/dl, total leukocyte count 136 × 109/l, platelet count 588 × 109/l and differential count-neutrophils 16%, lymphocytes 22%, myelocytes 24%, metamyelocytes 34%, basophils 3% and blasts 1% (Sokal, Intermediate risk 0.83). Her biochemical profile revealed blood urea 36 mg/dl, creatinine 1.1 mg/dl, uric acid 4.2 mg/dl, calcium 8.6 mg/dl and bilirubin 0.8 mg/dl. Her leukocyte alkaline phosphatase (LAP) score was low and RT-PCR for BCR-ABL was positive for p210. She was diagnosed as a case of chronic myeloid leukemia- Chronic phase (CML-CP). Radiographs of limb bones were normal. Tc-99m MDP bone scan revealed heterogenous increased radiotracer uptake in limb bones (involving bilateral humeri, forearm bones, femurs), suggestive of multiple bone infarcts. Rest of the skeleton was normal. She was started on hydroxyurea 500 mg thrice daily, allopurinol 100 mg thrice daily, imatinib mesylate 400 mg once daily and ecosprin 75 mg once daily. She was given analgesics (paracetamol and tramadol) for bone pains. Hydroxyurea and allopurinol were stopped after 2 weeks and imatinib and ecosprin continued. Patient achieved complete hematological response after 2 months of imatinib therapy but continued to have limb pains for 4 months though with decreased intensity. Pain completely subsided 5 months later (ECOG performance status 0). Repeat bone scan performed at 5 months revealed completely normal bone scan. Quantitative PCR for BCR-ABL at 15 months revealed major molecular response. CML accounts for approximately 15 to 20% of leukemias in adults. The clinical findings at diagnosis of CML-CP vary among reported series. Twenty to 50% of patients are asymptomatic, with the disease first being suspected from routine blood tests [1, 2]. Among symptomatic patients, systemic symptoms such as fatigue (34%), weight loss (20%), abdominal fullness (15%) and bleeding episodes due to platelet dysfunction (21%) are common [2]. Abdominal pain and discomfort may include left upper quadrant pain and early satiety due to the enlarged spleen with or without perisplenitis and/or splenic infarction. Tenderness over the lower sternum, due to an expanding bone marrow, is sometimes seen. Acute gouty arthritis may also present due to overproduction of uric acid [3]. Other frequent findings include splenomegaly (present in 48–76%), anemia (45–62%), white blood cell count above 100 × 109/l (52–72%) and platelet count above 600 × 109 (15–34%) [1, 2]. Involvement of extramedullary tissues such as the lymph nodes, skin, and soft tissues is generally limited to patients with blast crisis. Although thrombocytosis is common, thrombosis is not seen [2]. Other rare manifestations include splenic rupture [4] and priapism [5]. The radiological changes studied in CML include diffuse osteoporosis, focal osteolytic and osteoblastic lesions, chloromas and arthritis [6]. Bone pains are one of the common presentations of hematological malignancies [7] and are usually due to marrow infiltration by leukemic cells causing expansion of bone marrow. Bone infarction with severe pain as initial presentation has never been reported in CML-CP, though a case of CML developing bone infarction and progressing to myelofiberosis has been documented [8]. Our patient presented with diffuse debilitating bone pains due to multiple bone infarcts which is an unusual presentation of CML-CP. The patient responded to the usual treatment with complete disappearance of bone lesions and complete clinical recovery.

Diagnóstico pré-natal de displasia tanatofórica: papel do ultrassom fetal

OBJETIVO: Relatar o caso de um paciente com displasia tanatofórica, uma forma autossômica dominante e letal de nanismo, diagnosticado ainda no período pré-natal, e revisar a literatura, discutindo os principais diagnósticos diferenciais e ressaltando a importância do ultrassom fetal na identificação de fetos portadores dessa displasia. DESCRIÇÃO DO CASO: O paciente é o segundo filho de pais jovens sem história familiar de doenças genéticas. O ultrassom fetal realizado com 35 semanas de gestação mostrou polidrâmnio, proeminência frontal e desproporção entre crânio e tronco, com hipoplasia torácica e encurtamento dos ossos longos, compatível com o diagnóstico de displasia tanatofórica. Ao nascimento, a criança era pequena e possuía hipotonia, macrocefalia, fontanelas amplas, hipoplasia de face média, olhos protrusos, hemangioma plano no nariz e pálpebras, nariz em sela, micrognatia, pescoço e tórax curtos e encurtamento importante de braços, antebraços, coxas e pernas. A avaliação radiográfica mostrou crânio com grande diâmetro transverso, tórax com costelas curtas e corpos vertebrais reduzidos, importante encurtamento e deformidade dos ossos longos dos membros superiores e inferiores (os fêmures eram curvos) e hipoplasia da bacia. Esses achados confirmaram o diagnóstico pré-natal de displasia tanatofórica. O paciente evoluiu para o óbito poucos dias após o nascimento devido à insuficiência respiratória. COMENTÁRIOS: A ultrassonografia fetal é um método não invasivo capaz de diagnosticar inúmeras displasias ósseas, incluindo a tanatofórica. A importância do diagnóstico intra-útero reside no fato de que auxilia no diagnóstico diferencial, e permite o aconselhamento genético à família.

Comparative Osteometric Study of Long Bones in Yankasa Sheep and Red Sokoto Goat

A morphometric study was undertaken on the long bones in two predominant breeds of small ruminant in Northern Guinea Savanna zone of Nigeria, to determine their anatomical differences, and thus, provide a means of differentiating the remains of these two species duringzooarchaeological studies and gross anatomy practical session. The pelvic and pectoral limbs from 15 Yankasa sheep and 15 Red Sokoto goats of both sexes were used for this study. The long bones of the pectoral limbs considered were the humerus, radius, ulna and metacarpal III whilethose of the pelvic limbs were the femur, tibia and metatarsal III. Parameters considered were the mean weights, lengths and diameters of the proximal extremity, mid-shaft and distal extremities of these bones. There was a significant difference (p < 0.001) in the length of the longbones between the two species, with that of the Yankasa sheep being significantly longer than the Red Sokoto goat. Also, the differences in weights and diameters of the proximal, middle and distal shafts of the tibia between the two species were statistically different (p < 0.001).Conversely, the differences in the proximal and distal diameters of the femur between the two species were not statistically significant (p > 0.05). Factors that may be responsible for these differences were discussed. It was concluded that the bones of the Yankasa sheep and Red Sokoto goat can be differentiated by the disparity in the length of the long bones or the disparity in the entire morphometry of the tibia bones.

Painful fingertip swelling of the middle finger

Osteoid osteoma (OO) is a benign bone-forming tumour that accounts for about 12% of benign and 3% of all bone neoplasms [1]. It mainly affects patients during childhood or early adulthood, with a male preponderance. Despite its predilection for the long bones of the lower limbs, this tumour can occur in any bone of the skeleton and may be found in the hand and wrist in up to 10% of cases [2]. However, OOs of the distal phalanx are relatively uncommon, with only a few case series reported in the English literature so far [3–5]. The diagnosis of distal phalangeal OO is challenging for several reasons. First, the typical clinical features may not be present [2, 3, 5]. Pain may not be worse at night, or be relieved by aspirin. Moreover, soft-tissue swelling and erythema, as well as nail deformities, may mislead the physician. Second, its imaging characteristics (i.e. a small radiolucent lesion with central calcification, peripheral sclerosis and periosteal reaction [1]) may also be atypical [3–6]. Because of the presence of thickened Sharpey’s fibres, a periosteal reaction is rarely found in the distal phalanx [6]. Besides, endosteal sclerosis may be counterbalanced by local osteopaenia. Furthermore, other bony lesions of the fingertips, such as chronic osteomyelitis, epidermoid cyst, enchondroma or glomus tumour, may mimic an OO [3, 5, 6]. Zampa et al. recently reported the added value of dynamic contrast-enhanced MR imaging for OO in atypical locations, in terms of nidus conspicuity and diagnostic confidence [7]. Third, the histological features may even be unusual [5]. All those reasons could explain the delay, sometimes of several months to years, in the diagnosis and the treatment of phalangeal OOs. The main clinical differential diagnosis of painful fingertip swelling includes whitlow or paronychia, osteomyelitis, glomus tumour, subungual exostosis, arthritis and OO [3, 5, 6]. In our case, conventional radiographs (see Fig. 1 in the Question), ultrasound and previous CT and MR imaging studies (all not shown) barely displayed a focal lesion at the base of the distal phalanx. Follow-up MRI, performed at our institution several months after the onset of symptoms and after failure of initial surgery, demonstrated persisting diffuse inflammatory changes of the distal and middle phalanges as well as in the The case presentation can be found at doi:10.1007/s00256-011-1218-z

Antibiotic treatment for acute haematogenous osteomyelitis of childhood: Moving towards shorter courses and oral administration

Acute haematogenous osteomyelitis (AHOM) of childhood usually affects the long bones of the lower limbs. Although almost any agent may cause AHOM, Staphylococcus aureus is the most common bacterium, followed by Streptococcus pneumoniae and, in some countries, Salmonella spp. and Kingella kingae. Magnetic resonance imaging (MRI) has improved the diagnostic accuracy of traditional radiography and scintigraphy. Except for the pre-treatment diagnostic sample from bone before the institution of antibiotic therapy, no other surgery is usually required. Traditionally, non-neonatal AHOM has been treated with a 1–3-month course of antibiotics, including an intravenous (i.v.) phase for the first weeks, but recent prospective randomised studies challenge this approach. For most uncomplicated cases, a course of 20 days including an i.v. period of 2–4 days suffices, provided large enough doses of a well-absorbed agent (clindamycin or a first-generation cephalosporin, local resistance permitting) are used, administration is four times daily and most symptoms and signs subside within a few days. Serum C-reactive protein (CRP) is a good guide in monitoring the course of illness, and the antimicrobial can usually be discontinued if CRP has decreased to <20mg/L. Newer and costly agents, such as linezolid, should be reserved for cases due to resistant S. aureus strains. AHOM in neonates and immunocompromised patients probably requires a different approach. Because sequelae may develop slowly, follow-up for at least 1 year post hospitalisation is recommended.

Fetus-in-fetu: imaging and pathologic findings

A 3.5-month-old boy was hospitalized because of an abdominal mass found accidentally. On physical examination, a smooth, firm, nontender mass was present in the right upper quadrant. Abdominal ultrasonography revealed a large, hyperechoic, heterogenous mass with clear boundaries, and scarce blood flow. Abdominal CT scan showed a bulky right retroperitoneal mass. Three-dimensional CT imaging demonstrated spine, iliac bone, and long bones of limbs. The mass was excised successfully. After opening the sac it was noted to contain an incompletely developed fetus with grossly visible limbs, clearly discernible male genitalia, hairs, and a poorly formed head. The fetus was connected to the sac via an 8 cm cord-like structure. Microscopic examination of the mass revealed the presence of skin, cartilage, bone, intestine, and cysts with simple cuboidal epithelium. The use of CT scans enhanced the accuracy of pre-operative diagnosis. Identification of the vertebral column and the long bones of limbs are important indications for the diagnosis. Pathologically, fetus in fetu has many characteristics different from teratoma.

Comparative Osteometric Study of Long Bones in Yankasa Sheep and Red Sokoto Goats

A morphometric study was undertaken on the long bones in the two predominant breeds of small ruminant in Northern Guinea Savanna zone of Nigeria, to determine their anatomical differences and thus, provide a means of differentiating the remains of these two species during zooarchaeological studies and gross anatomy practical session. The pelvic and pectoral limbs from thirty animals (15 Yankasa sheep and 15 Red Sokoto goats) of both sexes were used for this study. The long bones of the pectoral limbs considered were the humerus, radius, ulna and metacarpal III while those of the pelvic limbs were the femur, tibia and metatarsal III. The parameters considered were the mean weights, lengths and diameters of the proximal extremity, mid-shaft and distal extremities of these bones. The result of this study showed that there is a very high statistically significant difference (p 0.05). Factors that may be responsible for these differences were discussed. It is concluded that the bones of the Yankasa sheep and Red Sokoto goat can be differentiated by the disparity in the length of these long bones or the disparity in the entire morphometry of the tibia bone.

Determinação da Estatura por Meio da Medida de Ossos Longos dos Membros Inferiores e dos Ossos da Pelve

Aim: since the Brazilian racial miscegenation is completely sui generis, a study was proposed on human height in order to establish a pattern for long bones of the lower limbs, as well as for the bones of the pelvis. Methodology: the sample included 100 skeletons - being 50 masculine and 50 feminine ones - with previously well known heights, belonging to the Bom Jesus Cemetery at Cuiaba (MT). Femur, tibia, fibula and the bones length of the pelvis were measured. The samples were submitted to statistical analysis through calculations of lineal correlation, lineal regression and Pearson’s t-test. Results: it was obtained a new pattern which allows indirect calculation of the pelvis - iliac bone - participation in the composition of the total height of the person. An actual measurement, just too unknown, in both national and international literature, was established. A trigonometric formula was proposed to calculate the degree of participation of the iliac bone in the body total height. Conclusion: they were also obtained regression formulae for national patterns for femur, tibia and fibula that, since appropriately applied, it will be of interest to estimate the total body stature.

Melorheostosis of the hand in a pediatric patient

Melorheostosis is a rare benign sclerosing bone dysplasia. Its etiology is unknown. Both sexes can be affected. Sites most frequently involved are the long bones of the lower limbs and the adjacent soft tissue structures. The disease is unusual in the upper limbs, and few cases have been reported in the hand. We report the case of a 7-year-old girl who had typical features of melorheostosis in the right hand. Diagnosis was made by conventional radiography and bone scintigraphy. Magnetic resonance contributed to the assessment of soft tissue lesions. Medical treatment allowed pain relief and range of motion improvement. Because melorheostosis has a variable tendency towards progression and association with tumors, a close follow-up of these patients is recommended.

Structural behaviour and strain distribution of the long bones of the human lower limbs

Although stiffness and strength of lower limb bones have been investigated in the past, information is not complete. While the femur has been extensively investigated, little information is available about the strain distribution in the tibia, and the fibula has not been tested in vitro. This study aimed at improving the understanding of the biomechanics of lower limb bones by: (i) measuring the stiffness and strain distributions of the different low limb bones; (ii) assessing the effect of viscoelasticity in whole bones within a physiological range of strain-rates; (iii) assessing the difference in the behaviour in relation to opposite directions of bending and torsion. The structural stiffness and strain distribution of paired femurs, tibias and fibulas from two donors were measured. Each region investigated of each bone was instrumented with 8–16 triaxial strain gauges (over 600 grids in total). Each bone was subjected to 6–12 different loading configurations. Tests were replicated at two different loading speeds covering the physiological range of strain-rates. Viscoelasticity did not have any pronounced effect on the structural stiffness and strain distribution, in the physiological range of loading rates explored in this study. The stiffness and strain distribution varied greatly between bone segments, but also between directions of loading. Different stiffness and strain distributions were observed when opposite directions of torque or opposite directions of bending (in the same plane) were applied. To our knowledge, this study represents the most extensive collection of whole-bone biomechanical properties of lower limb bones.

Amino Acid Profile of Protein from Pelvic Limb Long Bones of Broiler Chickens

The objective of the study was to determine the amino acid profile in protein from long bones of the pelvic limb (femur, tibiotarsus) in ROSS 308 hybrid broiler chickens, and to examine whether it is sex-dependent.The study material was obtained from 120 Ross 308 broiler chickens (60 males and 60 females) at the end of the fattening (40 days of age). The average weight of birds was 2 694 ± 32.6 g for cockerels and 2 345 ± 23.9 g for pullets. The study birds did not show any clinical signs of leg diseases. The long bones of the right pelvic limbs (femur and tibiotarsus) were collected after bird slaughter and after removal of soft tissues, they were used for laboratory analyses. The crude protein content and the amino acid (AA) composition were determined and the obtained values were recalculated to 100% dry matter. The chemical score (CS) and the essential amino acid index (EAAI) were calculated for each AA. As a standard, the hen egg ovalbumin was used. The crude protein content in 100% dry matter shows that the content of crude protein was significantly higher (P ⪬ 0.01) in the femur (405.3 ± 17.21 g/kg) compared to the tibiotarsus (393.5 ± 12.68 g/kg). The same trends were found with regard to sex. Both males and females had significantly (P ⪬ 0.01) higher crude protein content in the femur (406.6 ± 19.34 g/kg and 404.1 ± 15.09 g/kg, respectively) than in the tibiotarsus (395.0 ± 12.16 g/kg and 392.1 ± 13.21 g/kg, respectively). Both bones showed the highest contents of Gly, Glu and Pro, and the lowest contents of Met, Tyr and His. The same trends were found with regard to chicken sex. Collagenous protein has lower contents of most AA in comparison to the standard protein - ovalbumin; especially contents of Ser, Val, Met, Ileu and Phe are substantially lower. On the other hand, collagenous protein had higher contents of Gly, Pro, His and Arg. The collagenous protein EAAI for the femur and the tibiotarsus was 59.0% and 57.4%, respectively. The knowledge of amino acid composition in protein from long bones of the pelvic limb in broiler chickens is important because it can help us understand the origin of bone disorders.

Late-onset sporadic ataxia, pontine lesion, and retroperitoneal fibrosis: a case of Erdheim-Chester disease

A 60-year-old man with progressive gait ataxia and mild pyramidal signs showed at MRI a pontine lesion with post-contrast enhancement in the left middle cerebellar peduncle. Diagnosis of Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, was suggested, further supported by a previously diagnosed retroperitoneal fibrosis. X-ray films demonstrated characteristic bilateral and symmetric osteosclerosis of the long bones of the lower limbs, which at radionuclide studies exhibited a marked increase in technetium-99 uptake. A cerebral 18FDG-PET showed a relevant pontine uptake of the tracer. Re-evaluation of a past retroperitoneal biopsy showed an intense CD68+, CD1a-, and S100- infiltrate of histiocytes with foamy cytoplasm, thus confirming the diagnosis. ECD should be regarded as a rare cause of adult-onset sporadic ataxia, especially when pontine lesions and extraneurological manifestations are present.

Melorheostosis: Case Report with 20-Year Follow-Up

The authors report on a typical case of melorheostosis observed in an 18-year-old man, localized in the II digit of the right hand causing tumefaction and irregular pain. At 20-year follow-up, the patient showed a radiographical slight worsening of the lesion. Radiographic examination showed a mild increase in cortical and endosteal hyperostosis with bone shape alteration in the II digit of the right hand, slight cortical and endosteal hyperostosis at the level of right shoulder girdle involving the humerus. The other part of the skeleton was not affected. Clinical examination revealed swelling and tumefaction of the forefinger of the right hand and reduced articular motion both in proximal and distal interphalangeal joint and metacarpal phalangeal joint. The reminder of the clinical examination was normal. Melorheostosis is an uncommon mesenchymal disorder characterized by a chondral hyperostosis of the long bones associated to a sclerosis of the spongious bone. The etiology of this affection is unknown: the most accredited hypothesis is a developmental anomaly with a sequential sclerotomes distribution. Both genders may be affected, with the long tubular bones of the lower limbs more involved. This anomaly is often asymptomatic but maybe accompanied by pain with a smooth symptom progression and periodic exacerbations. Therapy is mainly symptomatic and comprises anti-inflammatory drugs or surgical approach for removal of soft tissue that may preclude daily activities. This case report demonstrates that melorheostosis is a relatively benign disorder with mildly progressive course.

Sonographische Verlaufskontrolle der sekundären Frakturheilung

We report on the contribution of real-time sonography in comparison to plain radiographs to the assessment of fracture healing of 121 patients suffering fractures of the long bones of the lower limbs. Sonographic morphology of periosteal callus metamorphosis shows basically a persistent increase of echogenicity representing the temporal range from fracture haematoma to the "woven bone". Further criteria are the homogeneity of structure and the development of a marginal interface. Nonunion radiologically classified into hypertrophic and atrophic types may also be differentiated by sonographic criteria and may influence proper orthopaedic management. Regarding sonographic methods using an A-mode amplitude signal, the morphologic assessment of callus metamorphosis is more likely to be translated into clinical practice. Although it yields a variety of information, sonography will be unable to replace radiography due to methodical limitations.

Modified Sofield-Millar operation: less invasive surgery of lower limbs in osteogenesis imperfecta

We present the results of intramedullary rodding of long bones of the lower limbs in children with osteogenesis imperfecta using a modified Sofield-Millar operation. Fourteen patients (mean age at primary operation was 5 years 11 months) were treated with a modified Sofield-Millar operation which allows minimal bone exposure, preservation of the periosteum and keeping the number of osteotomies to the minimum. Union was achieved in all cases within 7 weeks. Of the 14 patients (29 bones) treated with nonelongating rods, rod revisions were needed in 13 patients (26 bones). We found no statistically significant difference between the width of the bone immediately postoperatively and at the final follow-up. The walking ability was improved in four patients. Advantages of less invasive surgery in osteogenesis imperfecta are rapid bone union, no bone atrophy or nonunion, better postoperative mobility and small scars.

Ca2+/Calmodulin-dependent kinase II signaling causes skeletal overgrowth and premature chondrocyte maturation

The long bones of vertebrate limbs originate from cartilage templates and are formed by the process of endochondral ossification. This process requires that chondrocytes undergo a progressive maturation from proliferating to postmitotic prehypertrophic to mature, hypertrophic chondrocytes. Coordinated control of proliferation and maturation regulates growth of the skeletal elements. Various signals and pathways have been implicated in orchestrating these processes, but the underlying intracellular molecular mechanisms are often not entirely known. Here we demonstrated in the chick using replication-competent retroviruses that constitutive activation of Calcium/Calmodulin-dependent kinase II (CaMKII) in the developing wing resulted in elongation of skeletal elements associated with premature differentiation of chondrocytes. The premature maturation of chondrocytes was a cell-autonomous effect of constitutive CaMKII signaling associated with down-regulation of cell-cycle regulators and up-regulation of chondrocyte maturation markers. In contrast, the elongation of the skeletal elements resulted from a non-cell autonomous up-regulation of the Indian hedgehog responsive gene encoding Parathyroid-hormone-related peptide. Reduction of endogenous CaMKII activity by overexpressing an inhibitory peptide resulted in shortening of the skeletal elements associated with a delay in chondrocyte maturation. Thus, CaMKII is an essential component of intracellular signaling pathways regulating chondrocyte maturation.