Primary Ewing sarcoma of the coronoid process of mandible.

Ewing sarcoma (ES) is a rare, primary malignancy of the bone that occurs mainly in childhood and early adolescence. ES usually occurs in long bones of the axial skeleton. Although uncommon in the jaws, ES at this site is most likely to occur in the posterior mandible. The outcome for patients with localised disease has improved over the decades, due to better combination chemotherapies and better methods of local control. We present the clinicopathologic features and management of a case of ES that developed in the left coronoid process of the mandible of a 31-year-old male. Chemotherapy and, later, a segmental mandibulectomy were used to achieve local control. A fibula-free flap repair was performed with good aesthetic results. This case elucidates the importance of the interdisciplinary approach required for the evaluation and treatment of this aggressive neoplasm.

Spinal metastasis of breast cancer presenting after 25 years: An extremely rare presentation.

Breast cancer is the most frequently diagnosed cancer in females of the developed world and is gradually becoming the leading cause in the developing world as well. The innate biology of breast cancer is marked by varied presentations, characteristics, response, recurrence and metastatic phenomenon. Even an early stage breast cancer has the potential to recur and/or metastasize after extremely long duration and this possibility should be borne in the clinician's mind.

Keller's arthroplasty in adults with hallux valgus and hallux rigidus.

BACKGROUND: The role of Keller's resection arthroplasty in the management of adult hallux valgus with hallux rigidus is debatable. There are no studies addressing this particular problem. METHODS: This study is a retrospective review of 32 patients (49 feet), conducted by an independent assessor. Subjective and objective criteria were used to assess the results of surgery. There were 30 women and 2 men with an average age at surgery of 62.5years. RESULTS: The results of surgery in terms of relief of pain, cosmesis and use of regular footwear were satisfactory. Excellent and good subjective results were obtained in 39% and 37% of cases respectively. Radiological analysis revealed decrease in the intermetatarsal and first metatarsophalangeal angle in a significant number of cases. Final results assessed by Vallier's modification of Bonney and MacNab criteria, revealed excellent to good results in 87% of feet. A significant number of complications were noted but there was no association between the occurrence of complications and the final result or the subjective functional grade. There was no association between the amount of resection of proximal phalanx and occurrence of metatarsalgia or the final outcome. CONCLUSION: The results of this study suggest that Keller's arthroplasty has a role in patients with adult hallux valgus associated with degenerative changes in the first metatarsophalangeal joint.

The impact of VKORC1-1639 G>A polymorphism on the maintenance dose of oral anticoagulants for thromboembolic prophylaxis in North India: A pilot study.

BACKGROUND: The dose requirements for oral anticoagulants in thromboembolic events are influenced by promoter polymorphism in the VKORC1 gene. However, limited data are available on the influence of the polymorphism in various Indian populations. The present study aimed at determining the relationship between the VKORC1-1639 G>A genotypes and maintenance doses of oral anticoagulants for therapeutically stable INR values in patients taking Acitrom after valve replacement surgery. MATERIALS AND METHODS: Fifty patients from the northern Indian region were genotyped for VKORC1-1639 G>A by polymerase chain reaction and restriction fragment length polymorphism. Means of the weight-normalized daily Acitrom dose were calculated for every patient. RESULTS AND DISCUSSION: The VKORC1 1639G>A minor allele frequency in the study population (n = 50) was found to be 22%. The patients with a wild type genotype required the maximum drug dose as suggested for full functionality of the enzyme. Heterozygous patients were found to have an intermediate drug dose and the patients with a variant homozygous genotype had the minimum maintenance drug dose requirement. These findings are in concurrence with the effect of the promoter polymorphism on vitamin K epoxide reductase activity.1639G>A minor allele frequency in the study population (n = 50) was found to be 22%. The patients with a wild type genotype required the maximum drug dose as suggested for full functionality of the enzyme. Heterozygous patients were found to have an intermediate drug dose and the patients with a variant homozygous genotype had the minimum maintenance drug dose requirement. These findings are in concurrence with the effect of the promoter polymorphism on vitamin K epoxide reductase activity. CONCLUSION: The VKORC1-1639 G>A status can be indicative of establishing the therapeutic dose of oral anticoagulants in Indian patients.

An ARF/CtBP2 complex regulates BH3-only gene expression and p53-independent apoptosis.

The alternative reading frame (ARF) tumor suppressor exerts both p53-dependent and p53-independent functions. The corepressor C-terminal binding protein (CtBP) interacts with ARF, resulting in proteasome-mediated degradation of CtBP. ARF can induce apoptosis in p53-null colon cancer cells, in a manner dependent on ARF interaction with CtBP. Bik was uniquely identified in an apoptotic gene array as coordinately upregulated in colon cancer cells after either CtBP2 knockdown or ARF overexpression. Validating the array findings, ARF induced Bik mRNA and protein expression, and this activity required an intact CtBP binding domain. Apoptosis induced by CtBP deficiency was substantially impaired when Bik expression was simultaneously silenced. An analysis of the Bik promoter revealed binding sites for the CtBP-interacting basic Kruppel-like factor (BKLF). A Bik promoter luciferase reporter was repressed by BKLF and CtBP2, and ARF reversed CtBP-associated repression. Chromatin immunoprecipitation analyses showed that CtBP was recruited to the Bik promoter largely by BKLF. Expression profiling of BH3-only gene expression in ARF-expressing or CtBP-deficient cells revealed that Bik was uniquely regulated by ARF/CtBP in colon cancer cells, whereas additional BH3-only proteins (Bim, Bmf) showed CtBP-dependent repression in osteosarcoma cells. ARF antagonism of CtBP repression of Bik and other BH3-only genes may have a critical role in ARF-induced p53-independent apoptosis and tumor suppression.

Keratotic vascular papules over the feet: a case of Waldenström's macroglobulinaemia-associated cutaneous macroglobulinosis.

Waldenström's macroglobulinaemia (WM) is a plasma-cell dyscrasia characterized by the monoclonal proliferation of lymphoplasmacytes. A 48-year-old man presented with a 4-year history of multiple painful, hyperkeratotic deep-seated papules over the pressure areas of both soles. He had a 1-year history of Raynaud's phenomenon, intermittent epistaxis, recurrent vomiting, tingling and numbness, and visual disturbances. Histological examination of a skin biopsy found amyloid-like deposits in the upper and mid dermis involving dermal blood vessels, but apart from periodic-acid-Schiff, various stains gave negative results for amyloid. Direct immunofluorescence was positive for IgM antibody. Hence, a diagnosis of WM with cutaneous macroglobulinosis was made. Immunoelectrophoresis found monoclonal IgM kappa antibody, and bone-marrow examination revealed a lymphoplasmacytoid malignancy. The patient's systemic systems were attributed to hyperviscosity syndrome associated with WM and the cutaneous papules were identified as deposits of excess IgM antibodies. The patient received five cycles of chemotherapy, resulting in nearly complete resolution of the skin lesions and systemic symptoms.

Essential thrombocythemia in a young man treated for myocardial infarction.

Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm which can cause thrombohaemorrhagic complications usually involving microvasculature. Medium-sized arterial thrombosis has been reported, but coronary occlusion usually occurs with additional risk factors, for example, smoking, hyperlipidaemia and so on. We present a case of acute myocardial infarction (AMI) in a young man (29 years) with ET but without any coronary artery associated risk factors. He was successfully treated for his AMI and ET with cytoreductive treatment and has recovered well. Due to automated platelet counting, ET is being increasingly identified; early detection can prevent long-term complications, and patients can have normal life span.

Prometheus' spirit: quality survival in advanced hepatocellular carcinoma after gemcitabine and cisplatin-based chemotherapy.

In advanced virus-induced hepatocellular carcinoma (HCC) associated with cirrhosis, the average survival is four months. We report a 56-year-old man with a large-volume advanced HCC, in whom gemcitabine and cisplatin-based chemotherapy resulted in near-complete regression, and quality survival of 24 months.

Survival rates in dysvascular lower limb amputees.

BACKGROUND: To assess the 5-year and 10-year survival rates of major (above ankle disarticulation level) dysvascular lower limb amputees attending a sub-regional Disablement Services Centre (DSC) specialising in amputee rehabilitation. Also to investigate the association between survival rates, cause of dysvascularity, level of amputation, smoking status and occupational status. SETTING: The study was undertaken in sub-regional DSC for amputee rehabilitation covering a base population of about 3.5million people. Over 80% of lower limb amputations were done for dysvascularity (peripheral vascular disease, diabetic or combination). All these patients were followed up in the DSC for their prosthetic/amputee rehabilitation. Modular case records of 201 consecutive patients from 1994 to 1995 who had diagnosis of dysvascularity as the cause of major lower limb amputations, were scrutinised regarding their 10-year survival; demographic details, level of lower limb amputations, Above Knee (AK=Transfemoral), Below Knee (BK=Transtibial), smoking status, occupational status, healing of the stump at first assessment, cause of amputation and association of these factors with survival rates. RESULTS: Of 201 individuals with either AK or BK amputations, 60% (121) had AK amputations and 67% (134) were males, the mean age was 69years of age. Sixty-seven percent (97) had history of smoking, either current 43% (62) or prior 24% (35) smoking, and 59% (68) were skilled or non-skilled manual workers. Fifty-one percent (99) had diagnosis of peripheral vascular disease, whilst 34% (65) had combination of peripheral vascular disease and diabetes, diabetes on its own in 4% (7). In 12% (23) other causes were noted such as embolism, acute ischaemia, venous ulcers, etc. Regarding stumps healing at first assessment, healing was noted in 54% (109) whilst stump was unhealed in 46% (92). The median survival was 48months. Using Cox proportional hazards regression to identify association with survival, the hazard ratio (HR) was significant regarding level of amputation: HR 2.34; 95% confidence interval (CI) (1.58, 3.47), P<0.001 (a recent BK amputation increases the risk by 2.3 compared to a recent AK amputation in diabetic cohort and also in the peripheral vascular disease/diabetes cohort). Hazard ratio was less than 1.0 in bilateral amputees: HR 0.35, 95% CI (0.21, 0.60), P<0.001 (bilateral amputation decreases risk by 0.35). CONCLUSION: Our study indicates that the median survival remains at 4years, which is similar to the previously published evidence in the Finish study of 1998 and the earlier study from Scotland in 1992. Unlike the previous data, our study indicates that patients with BK amputations have a higher hazard ratio than the AK amputees, and an association with diabetes has poorer prognosis regarding survival.

Modified calcaneal index: a new screening tool for osteoporosis based on plain radiographs of the calcaneum.

PURPOSES: To assess osteoporosis using plain radiography of the calcaneum by studying the performance characteristics of the modified calcaneal index through inter- and intra-observer agreement. To study the correlation of the modified calcaneal index to quantitative ultrasound of the calcaneus and bone mineral density (BMD) of the femoral neck and distal radius. METHODS: Lateral calcaneal radiographs of 252 women who participated in a clinical trial for osteoporosis were reviewed. The BMD of the hip and distal radius was measured and the calcanea were assessed using ultrasound. The calcaneal radiographs were graded by 3 clinicians according to a previously described 5-grade calcaneal index. A modified 3-grade calcaneal index was then developed. RESULTS: The highest scores of intra- and inter-observer reliability of the modified calcaneal index were 0.45 and 0.40, respectively, which were higher than those of the 5-grade calcaneal index. The correlation of the modified calcaneal index with other measures was significant (hip BMD, r=0.31; distal radius BMD, r=0.28; calcaneal speed of sound, r=0.20; broadband ultrasound attenuation, r=0.36) [p<0.005]. There were significant differences in hip BMD, distal radial BMD, calcaneal speed of sound, and broadband ultrasound attenuation between the 3 grades of the modified calcaneal index (Kruskal-Wallis 1-way ANOVA; p<0.0001). CONCLUSION: The modified calcaneal index can be used to measure bone structure and skeletal strength and is a suitable screening tool for osteoporosis in places where advanced approaches to bone-status assessment are not available.

The osseofasciocutaneous flap: a new method to transfer fibula along with a sufficient amount of skin.

We describe a new technique of bone transfer, using a deep fascial blood supply to transfer bone together with a large area of skin. The viability of this flap and further confirmation by isotope scanning have established that the bone transfer is vascularised. This provides a simple solution to the complex problem of leg trauma with massive skin and bone loss.

Polychlorinated biphenyls in compost amended soil of a landfill site.

The soil receives enormous quantities of pesticides and PCBs as a waste product each year due to their wide application. The degradation and eventual fate of these chemicals in the soil largely determine ultimate environmental impact. A survey of an abandoned landfill site has been conducted in a fast developing industrial city Nagpur (India) to assess the degree of environmental contamination with PCBs. The purpose was to determine if any of the mixture of PCBs, Aroclor 1254 and Aroclor 1260 are retained in the compost amended soil of the site despite atmospheric losses possible due the peak summer (100-117 degrees F) in the city. The accuracy and precision for Aroclor 1254 and Aroclor 1260 determinations in the soil samples have been calculated as 76.5 and +/- 0.92 and 83.3 and +/- 1.13 respectively. The samples analysis showed the presence of Aroclor 1254 and Aroclor 1254 and Aroclor 1260 up to the levels of 0.55 and 0.80 mg/g respectively. The maximum total PCBs on dry weight basis are found as 1.2 mg/g.

Immersive virtual reality used as a platform for perioperative training for surgical residents.

Perioperative preparations such as operating room setup, patient and equipment positioning, and operating port placement are essential to operative success in minimally invasive surgery. We developed an immersive virtual reality-based training system (REMIS) to provide residents (and other health professionals) with training and evaluation in these perioperative skills. Our program uses the qualities of immersive VR that are available today for inclusion in an ongoing training curriculum for surgical residents. The current application consists of a primary platform for patient positioning for a laparoscopic cholecystectomy. Having completed this module we can create many different simulated problems for other procedures. As a part of the simulation, we have devised a computer-driven real-time data collection system to help us in evaluating trainees and providing feedback during the simulation. The REMIS program trains and evaluates surgical residents and obviates the need to use expensive operating room and surgeon time. It also allows residents to train based on their schedule and does not put patients at increased risk. The method is standardized, allows for repetition if needed, evaluates individual performance, provides the possible complications of incorrect choices, provides training in 3-D environment, and has the capability of being used for various scenarios and professions.

Abdominoperineal electrical injury involving urogenital organs.

Although the incidence of electrical burns is not very high this incidence is rising in India. The residual effects of electrical burns are sometimes very severe and long lasting, if not managed properly. This case report is of a patient who sustained severe electrical burns over his left arm, lower abdomen and perineal area. The injuries were so severe that life-saving disarticulation of the limb together with excision of the involved pubic bone and genitalia and suprapubic urinary diversion was done. During follow up an unusual finding was noticed. There was a peritubal leak with no drainage from the catheter and on removal of the catheter, a manual examination of the urinary bladder revealed a stone which was removed at the same time. Following removal of the stone two ureteric openings with the trigone were seen and it was concluded that the granulating tissue was actually the posterior wall of the bladder. This was then mobilized and reposited into the abdomen. The deficient lower abdomen was reconstructed with a tensor fascia lata flap. A penile reconstruction for the patient is under consideration.

Large transverse fasciocutaneous leg flap: whole leg flap.

Large defects of leg and sole often need massive tissue transfer. As an alternative to microvascular transfer, we have developed a fasciocutaneous flap in which almost the whole of the skin of the opposite leg is transferred based just on the septocutaneous perforators of the posterior tibial artery. The flap has been used as a cross-leg fasciocutaneous flap with potential for use as a free flap by taking a segment of the posterior tibial artery. Our experience with 11 cases is presented. Two patients suffered marginal necrosis while donor site problems were seen in another two patients. We have found this flap to be safe, technically easy and with minimal donor-site morbidity.

Analysis of immunoglobulin deficiency cases: a five year study.

A total of 734 serum specimens from various clinical disorders along with 100 control samples from healthy subjects were processed for estimation of serum IgG, IgA and IgM employing single radial immunodiffusion procedure. Immunoglobulin deficiency, either selective or combined was noted in 31 males and 24 females in all age groups. Of the 55 cases encountered it was secondary immunoglobulin deficiency which was seen on a larger scale and encountered in patients with Multiple myeloma (16 out of 32) followed by Leprosy (14 out of 250), Lymphoma (5 out of 43), Malaria (4 out of 137), Burns (4 out of 52), Rheumatoid arthritis (2 out of 69) and non lymphoreticular malignancies (1 out of 41) in decreasing order of frequency. Primary immunoglobulin deficiency was observed in nine cases comprising of six belonging to Idiopathic late onset immunoglobulin deficiency, two of dysgammaglobulineamia and a solitary case of Ataxia telangiectasia. Panimmunoglobulin deficiency was observed in six cases, 11 had a dual deficiency while 38 showed deficiency of an isolated class with selective IgA deficiency in 20 cases. Furthermore, one patient each had total absence of IgG or IgA while IgM was not detectable in seven patients. A high suspicion index along with a regular rapport between the clinician and the laboratory personnel is necessary in the diagnostic set up of immunoglobulin deficiency states.

Immunochemical studies in multiple myeloma.

The study deals with a total of 72 patients with Plasma cell dyscrasias (PCD) selected on the basis of atypical plasmacytosis in the bone marrow aspirate and radiological evidence of osteolytic lesions. Males(48) outnumbered the females (24). Pathological fracture and paraplegia was the commonest presenting symptom encountered in 38 patients. Electrophoresis of serum for 'M' band and Immunoelectrophoretic analysis of the serum revealed IgG myeloma in 40 patients followed by, IgA myeloma(13), Light chain disease (12) and other variants in remaining seven cases. The urinary Bence Jones proteins were detected in a total of 34 cases and was frequently encountered with IgA myeloma (7 out of 13) compared with IgG myeloma (13 out of 40) when analysed in Disc electrophoresis. Kappa light chain was observed in 21 cases and lambda counterpart in nine cases without any clinical significance. One case of solitary myeloma terminated in characteristic multicentric multiple myelomatosis within a span of six months in the sequential follow up study. We recommend the triangular approach to diagnosis of paraproteinemia with a special emphasis on immunoelectrophoresis for typing multiple myeloma and allied disorders along with disc electrophoresis for the demonstration of urinary Bence Jones protein in the routine set up.