Atsuhiro Imakiire

A report of two cases of Werner's syndrome and review of the literature.

Two cases of Werners syndrome are reported. The first case is that of a man with grey hair since his 20s, and alopecia since aged about 50 years. At the age of 53 years, Werners syndrome was diagnosed, along with a malignant soft tissue tumour of the hand. The patient underwent ray amputation for the tumour. The subsequent histopathological diagnosis was synovial cell sarcoma, and the patient died of lung metastasis at 15 weeks postsurgery. The second case is that of a woman diagnosed with diabetes mellitus when aged 34 years. At 39 years, a bilateral cataract was diagnosed and at 40 years, diabetic gangrene of the left calcaneal region and calcaneal osteomyelitis necessitated left below-knee amputation. The incidence of Werners syndrome in Japan is extremely high (about 1000 of the around 1300 cases reported worldwide) compared to other countries. Most patients develop malignant tumour or arteriosclerosis, the most important complications of this syndrome. The average life expectancy for patients with Werners syndrome is 46 years. The incidence of epithelial cancer and mesenchymal sarcoma is 10 times that of the general population. The onset of symptoms of Werners syndrome generally precedes any later symptoms of associated conditions, such as malignant tumour. Therefore, early recognition of Werners syndrome is important to assist identification of malignant tumours at an early stage in this patient group.

Lunate excision, capitate osteotomy, and intercarpal arthrodesis for advanced Kienböck disease. Long-term follow-up.

Background: Kienböck disease is caused by aseptic necrosis of the lunate. In the advanced stages of the disease, carpal collapse, joint incongruity, and osteoarthritis develop. We performed lunate excision, capitate osteotomy, and intercarpal arthrodesis (the modified procedure of Graner et al.) on fifteen patients with stage-IIIB or IV Kienböck disease. This report is a review of the findings in these patients. Methods: The subjects ranged in age from twenty-six to fifty-four years (mean, 39.2 years) at the time of surgery. We evaluated the results more than five years postoperatively (range, sixty-two to 145 months postoperatively; mean, 79.3 months postoperatively). Therapeutic results were evaluated according to the scoring system of Evans et al. Results: Pain disappeared after surgery in most patients. Others had a reduction in the intensity of the pain to a mild level. The grip strength on the affected side had recovered to about 80% of that on the unaffected side twelve months after surgery. The long-term results were graded as good in eleven of the patients, as fair in two, and as poor in two. Postoperative radiographs showed that the carpal bone parameters (carpal height index and radioscaphoid angle) had improved. Radiographic osteoarthritic changes occurred in all of the patients; however, except for moderate limitation of the range of motion at the wrist joint, these findings did not affect the level of pain, grip strength, or activities of daily living. Conclusions: Lunate excision followed by capitate osteotomy and intercarpal arthrodesis (the modified procedure of Graner et al.) is a reliable form of treatment for advanced Kienböck disease, with favorable results for at least five years postoperatively.

Chemotherapeutic agents sensitize sarcoma cell lines to tumor necrosis factor-related apoptosis-inducing ligand-induced caspase-8 activation, apoptosis and loss of mitochondrial membrane potential

Chemotherapeutic agents have been used for the treatment of patients with osteosarcoma (OS). However, inherent or acquired resistance to these agents is a serious problem in the management of OS patients. Tumor necrosis factor‐related apoptosis‐inducing ligand (TRAIL) is considered to induce apoptosis in a variety of cancer cells but not normal cells. In the present study, we examined whether chemotherapeutic agents enhance TRAIL‐induced apoptosis in the sarcoma cell lines MG‐63 and SaOS‐2. Pretreatment with sub‐toxic or slightly toxic concentrations of chemotherapeutic agents (cis‐diammine dichloroplatinum, CDDP and doxorubicin, DXR) sensitized both cell lines to TRAIL‐induced apoptosis, as assessed by the propidium iodide or Annexin V‐Cy5 staining method. These cell lines expressed death receptors TRAIL‐receptor 1 (TRAIL‐R1) and TRAIL‐R2, which were unaltered by treatment with CDDP, as assessed by flow cytometry. The decoy receptors TRAIL‐R3 and ‐R4 were barely detected in both cell lines. CDDP down‐regulated c‐FLIP, tending to lower the activation threshold required for TRAIL‐induced caspase‐8 activation. The CDDP‐pretreated cells indeed demonstrated more increased TRAIL‐mediated caspase‐8 activation, loss of mitochondrial membrane potential (ΔΨm), and apoptosis than untreated cells. Consequently, the activated caspase‐8 might lead to either activation of effector caspases such as caspase‐3 or loss in ΔΨm. Both the increased caspase activation and mitochondrial dysfunction induced by combination of CDDP and TRAIL would contribute to enhanced apoptotic cell death. The results of the present study would be valuable for the design of novel treatment modalities for patients with OS.

The radiographic study in the relationship of the glenohumeral joint

Accurate reproduction of anatomic relationship is important in non‐constrained prosthetic arthroplasty. The accurate lateral glenohumeral offset, which indicates a parameter of the lever arm of the deltoid and supraspinatus muscles, is one of the most important elements in achieving the efficient shoulder functions after prosthetic reconstruction. However, to our knowledge, there has been no detailed study on the influence of minute changes in the neck shaft angle, within the normal range, on lateral glenohumeral offset. In this study, we evaluated the relationship between the neck shaft angle and various geometric measurement values in the glenohumeral joint.

Morphological studies on the ageing and osteoarthritis of the articular cartilage in C57 black mice.

Purpose. To study the cause and mechanism of joint degeneration in osteoarthritis, through histopathological and ultrastructural-histochemical experiments on the articular cartilage of the knees of the C57 black mouse. Methods. 192 C57 black mice and a control group of 64 C57BL/6J mice were used in this study. The left and right knee articular capsules of the joints were removed and stained. Each articular cartilage sample was examined and osteoarthritic changes were assessed using a transmission electron microscope. The severity of osteoarthritis in the knee joint cartilage of C57 black mice was histologically assessed using a classification system described by Okabe, based on Maiers system. Results. The incidence and the severity of osteoarthritis gradually increased with age; the incidence increased from 20% at 2 months to 80% at 16 months. Irreversible changes appeared at an advanced stage, and the process of degeneration was quite similar to that in human osteoarthritis. Through transmission electron microscopy, we observed poorly developed Golgi apparatus, markedly increased intracellular microfilaments, decreased proteoglycan granules, and broken collagen networks in all stages of osteoarthritis. By contrast, Golgi apparatus and other organelles were well developed in histologically normal mice of all ages. Proteoglycan granules, which mainly consisted of keratan sulphate, were observed; collagen networks were maintained. Conclusion. Disturbed protein transport and sugar synthesis in chondrocytes, caused by the deficient development of the Golgi apparatus, could result in degenerative changes in articular cartilage. The structure and function of the matrix were maintained mainly because of the continued presence of keratan sulphate.

Microwear phenomena of ultrahigh molecular weight polyethylene cups and debris morphology related to γ radiation dose in simulator study

Ultrahigh molecular weight polyethylene (PE) cups with 0, 2.5, 50, 100, and 150 Mrad radiation treatments were run in a hip simulator for comparison of the microwear phenomena on the cup surfaces with the corresponding debris morphology. In general, the size and frequency of the PE surface fibrils and the size of the retrieved PE debris decreased with increasing radiation dose. The fibril size and shape on the cup surfaces were well correlated with the radiation dose. The fibrillar shape and size were found to be proportional to the square root of the radiation dose. However, the trend for size and shape factors of the wear debris related to the radiation dose was weak at best. Thus, the morphology of the PE fibrils on the cup surfaces was more sensitive to variations in the radiation dose than the actual wear debris. The wear response appeared to be a three-step process, which was dependent on the formation of surface nodules or ripples, the teasing out of surface fibrils, and the toughness of the PE matrix in releasing a wear fibril to form a debris particle. The tougher PE became with increasing radiation dose, the harder it was for the PE fibrils to break out into wear particles.

Cervical Vertigo After Hair Shampoo Treatment at a Hairdressing Salon : A Case Report

STUDY DESIGN A case report of cervical vertigo that occurred after shampoo treatment in a hairdressing salon. Abnormalities were detected on magnetic resonance images and magnetic resonance angiography scans. OBJECTIVES To describe the diagnosis of cervical vertigo caused by neck hyperextension. SUMMARY OF BACKGROUND DATA Neck hyperextension may induce vertigo and dizziness; the pathophysiology remains unclear, however, because subjective findings are usually difficult to document. METHODS The diagnosis, treatment, and outcome of a patient with cervical vertigo that occurred after hair shampoo treatment in a hair dressing salon were assessed. RESULTS Magnetic resonance angiography demonstrated narrowing of the left vertebral artery, whereas magnetic resonance imaging showed cerebellar infarction. Treatment included rest and drugs that activate cerebral circulation and prevent platelet aggregation. Improvement was noted within few days. CONCLUSIONS The authors suggest that the hyperextended neck position during hair shampoo treatment in a beauty parlor may be a risk factor for back lifting or cerebellum vascular insufficiency. Public education should lead to avoidance of this position during hair shampoo treatment at hair dressing salons.

RETRACTED: Cement Spacer Loaded With Antibiotics for Infected Implants of the Hip Joint

It is difficult to treat infected implants of the hip joints. Such treatment involves immeasurable physical and psychological suffering of the patients. We used antibiotic-impregnated cement spacers in 17 cases of infection after total hip arthroplasty and bipolar arthroplasty with good clinical results. We thoroughly removed any foreign material and formed an antibiotic-impregnated cement spacer into a similar shape as that of the implants. A cement spacer enables high-concentration antibiotics to act on infected sites. Also, it can prevent leg length discrepancy and atrophy of bones or muscles. Although cement spacers have been reported to have problems regarding shape and strength, we achieved good results with a cement spacer mold in the present study. No recurring infection has been found at a mean follow-up period of 3 years and 2 months.

Failure of ceramic THR with liner dislocation--a case report.

In September 1998, an all-alumina total hip replacement was performed in a 57-year-old woman using an ABS Cup System THR (Kyocera Ltd. Kyoto, Japan) (ABS HA Shell (CH 46)/ABS Liner (28-46)/Ball Head (28N: -4)/Perfi x Stem #12-M) for left traumatic femoral head necrosis (Figure 1a). The liner had an alumina inlay packed with polyethylene.In May 2002 discomfort appeared in the hip, with crepitation when walking. 3 weeks later, dis-location of the liner was confi rmed on radiographs. The liner had rotated about 90° in the metal shell and the ball head had been displaced in superolat-eral direction (Figure 1b). At the revision opera-tion, we observed pale gray color contamination in the synovia surrounding the neck and pseudocap-sule. The inlay had been dislocated in the liner and the stem neck had been impinged against the rim of the inlay. The ball head was in contact with the metal shell in the superolateral area. We found no distraction of ceramic parts. The liner, metal shell and alumina ball were revised into a polyethylene liner, metal shell and metal ball (Figure 1c).For the revised implant, we confi rmed the lesion and degree of injury of the implants by the naked eye and light microscopy, and observed the wear pattern of the ball head and the inlay. We used scanning electron microscopy (SEM) to examine the microstructure of the bearing surface. Based on macroscopic observation, about two-thirds of the circumference of the marginal part of the liner polyethylene had suffered a loss around the notch, to lock the liner between the shell. Metal contamination was observed on the rim of the alu-mina inlay, which had been dislocated in the liner (Figure 2).The SEM fi ndings in the normal load-bearing area suggested a very low wear rate, because the machine marks had just been smoothed out (grade II) (Shishido et al. 2002). On the other hand, in the rim of the bearing surface of the inlay and in the peripheral area of the ball head, the grain of the surface had been pulled out (grade V), so the wear was considered to be more severe (Figure 3).

Traumatic anterior dislocation of the hip associated with ipsilateral femoral shaft fracture in a child: A case report

Traumatic anterior dislocation of the hip joint in children is rare, and only one case with ipsilateral femoral fracture has been reported in Japan. We report a case of such dislocation and a review of the literature. The patient was a 31-month-old girl who was injured in a car accident while asleep on a tilted front passenger seat. Radiographic examination showed dislocation of the right obturator foramen and transverse fracture of the ipsilateral femoral shaft. The dislocation of the right hip was easily reduced without anaesthesia during radiography. We applied Bryant traction after reduction for 4 weeks, followed by cast application for 3 weeks. Walking with support and full weightbearing were permitted 14 weeks and 16 weeks after the injury, respectively. Radiography at 4.5 years after the injury showed a mildly enlarged right femoral head and femur overgrowth of approximately 8 mm. Magnetic resonance imaging showed no evidence of suspected avascular necrosis of the femoral head. The patient has no subjective or objective symptoms, and is able to engage in all usual activities. The detailed mechanism of the injury is unknown. We assume that the lower leg was dislocated through abduction during flexion, or abducent, external flexion, considering that the child was sleeping at the time of the accident. Since she was hurled to the back seat, it was assumed that strong external force was vertically added to the femur, which caused the abducent force.