Syncope in a 14yo female with ventricular tachycardia; an atypical etiology.

Wide complex tachycardias are rare in the pediatric population and may be due to ventricular tachycardia, aberrant conduction or antidromic tachycardia each with multiple underlying etiologies. We present a 14 yo female in extremis with syncope at rest witnessed by her mother, found in ventricular tachycardia by EMS who challenged with IVF hydration and amiodarone. Consecutive adequate fluid challenges and antiarrhythmics in the emergency department failed requiring synchronized cardioversion for stabilization. Subsequent viral panels, imaging, genetic testing and cardiac biopsy confirmed a diagnosis of arrhythmogenic right (and left) ventricular dysplasia.

Muromonab-CD3 (Orthoclone OKT3), methylprednisolone and cyclosporine for acute graft-versus-host disease prophylaxis in allogeneic bone marrow transplantation.

We report the results of a prospective non-randomized phase II study of Muromonab-CD3 (Orthoclone OKT3), an anti-CD3 monoclonal antibody, with methylprednisolone (MP) and cyclosporine (CSA) for acute GVHD (aGVHD) prophylaxis in 22 hematologic malignancy patients. OKT3 was given at 0.1 mg/kg/day with a maximum dose of 5 mg/day. Initial MP dose was 1000 mg before OKT3, with subsequent doses at 1 mg/kg/day before each OKT3 infusion with a planned taper beginning at day +28. CSA (3 mg/kg/day) was given as a continuous infusion at day -1 and adjusted to maintain serum levels between 250 and 399 ng/ml. Allogeneic BMT donors were HLA-matched siblings (n = 17), single HLA-mismatched-related (n = 1) and HLA-matched unrelated (n = 4). All patients achieved neutrophil engraftment at a median 11 days (range, 8-25 days). By intent-to-treat, the cumulative incidence of grade II-IV aGVHD was 33% (95% CI 13-53%) at a median 26 days post-BMT (range, 14-84 days). Chronic GVHD developed in 11/12 evaluable patients. Eight patients (36%) developed OKT3 first dose reactions; no cases of post-transplant lymphoproliferative disorder were observed. OKT3 depleted peripheral CD3+ cells in vivo as measured by flow cytometry. OKT3+MP+CSA combination is moderately effective aGVHD prophylaxis, however, it is unlikely to be superior to CSA+MTX.

Effect of adding dopexamine to intraoperative volume expansion in patients undergoing major elective abdominal surgery.

BACKGROUND: The contribution of low-dose dopexamine to outcome, when given to increase cardiac output in patients already treated with fluids during major abdominal surgery, is not yet known. METHOD: We carried out a randomized double-blind placebo-controlled trial. All 100 patients studied were given fluid infusions during surgery guided by stroke volume measurements made with an oesophageal Doppler probe. Patients were randomized to receive dopexamine at the rate of 0.25 microg kg(-1) min(-1) or saline 0.9% (control) for the first 24 h after the start of surgery. The primary outcome measure was the incidence of postoperative morbidity. RESULTS: There were no statistically significant differences between groups in the incidence of postoperative complications, the length of hospital stay, the incidence of morbidity and the use of critical care facilities. The patients randomized to receive dopexamine had significantly more pre-existing disease than the control patients. Mortality in both groups was significantly less than predicted by the POSSUM (Physiological and Operative Severity Score for the enUmeration of Mortality and morbidity) risk prediction score. CONCLUSION: We could not demonstrate an advantage to using low-dose dopexamine in high-risk patients during major abdominal surgery.

Tonsillectomy by guillotine is less painful than by dissection.

Most tonsillectomies are carried out by dissection. Only a small minority of otolaryngologists still routinely perform guillotine tonsillectomy. We carried out a prospective study on 86 children undergoing tonsillectomy utilising a standard anaesthetic and analgesic regimen to compare post-operative pain after dissection tonsillectomy and guillotine tonsillectomy using a Popper's hemostatic guillotine. Guillotine tonsillectomy was significantly less painful (P<0.001) than dissection tonsillectomy. The relative risk of experiencing moderately severe to severe pain was 0.36 (95% CI, 0.18-0.72) in the guillotine group. A significant proportion of children experience moderately severe to severe pain despite a comprehensive analgesic regimen confirming that post operative pain remains an important issue after this operation. On the basis of our findings we advocate tonsillectomy by guillotine in children. The less pain that arises within the first 24 h may be particularly important if performing tonsillectomy as a day-case procedure.

Regulation of human natural killer cell migration and proliferation by the exodus subfamily of CC chemokines.

Natural killer (NK) cells play an important role in innate and adaptive immune responses to obligate intracellular pathogens. Nevertheless, the regulation of NK cell trafficking and migration to inflammatory sites is poorly understood. Exodus-1/MIP-3alpha/LARC, Exodus-2/6Ckine/SLC, and Exodus-3/MIP-3beta/ELC/CKbeta-11 are CC chemokines that share a unique aspartate-cysteine-cysteine-leucine motif near their amino terminus and preferentially stimulate the migration of T lymphocytes. The effects of Exodus chemokines on human NK cells were examined. Exodus-1, -2, and -3 did not induce detectable chemotaxis of resting peripheral blood NK cells. In contrast, Exodus-2 and -3 stimulated migration of polyclonal activated peripheral blood NK cells in a dose-dependent fashion. Exodus-2 and -3 also induced dose-dependent chemotaxis of NKL, an IL-2-dependent human NK cell line. Results of modified checkerboard assays indicate that migration of NKL cells in response to Exodus-2 and -3 represents true chemotaxis and not simply chemokinesis. Exodus-1, -2, and -3 did not induce NK cell proliferation in the absence of other stimuli. Nevertheless, Exodus-2 and -3 significantly augmented IL-2-induced proliferation of normal human CD56(dim) NK cells. In contrast, Exodus-1, -2, and -3 did not affect the cytolytic activity of resting or activated peripheral blood NK cells. Expression of message for CCR7, a shared receptor for Exodus-2 and -3, was detected in activated polyclonal NK cells and NKL cells but not resting NK cells. Taken together, these results indicate that Exodus-2 and -3 can participate in the recruitment and proliferation of activated NK cells. Exodus-2 and -3 may regulate interactions between T cells and NK cells that are crucial for the generation of optimal immune responses.

Private patients in NHS hospitals: comparison of two sources of information.

BACKGROUND: The use of National Health Service (NHS) hospitals to treat private patients is debatable on the grounds of equity of access. Hospital Episodes Statistics (HES) annual reports are the only routine source of information on the scale of this activity. The accuracy of the information is doubted. This enquiry tested the completeness of HES data against information obtained directly from private patient unit managers. METHOD: Managers of the 71 pay bed units in NHS hospitals in England were asked to supply from local registers and accounts the numbers of in-patients and day cases admitted in 1995-1996. Their reports were matched with the numbers of first consultant episodes for private in-patients and day cases shown for those hospitals in the HES data file for that year. RESULTS: Of the 71 units 62 responded; 53 of these gave usable data. The 53 included, and 18 excluded from the comparison, matched on median and range of bed numbers. Managers identified 16 per cent more total admissions than did HES, 62,572 against 54,131; 13 per cent more in-patient admissions, 39,776 against 35,319; and 21 per cent more day cases, 22,796 against 18,812. More total admissions were reported by managers of 38 pay bed units than were recorded in HES, fewer by 12, and equal numbers by three. Similar sized discrepancies were noted for in-patient admissions and day cases. Reasons for the under-reporting of private patients in HES included the use of separate patient administration systems for private patients with a failure to feed data to HES, and the omission of some provider units altogether by a minority of trusts from the returns made to the Department of Health. CONCLUSION: Overall, HES underestimates the amount of private patient activity reported directly by NHS hospitals. No method of validating private patient data is currently available. An amendment to an existing statistical return would provide a check on numbers. Central guidance on the inclusion of private patient activity in data transmitted by providers to the HES processing agency should be reinforced.

Ischemic preconditioning attenuates functional, metabolic, and morphologic injury from ischemic acute renal failure in the rat.

Ischemic preconditioning has been shown to ameliorate injury due to subsequent ischemia in several organs. However, relatively little is known about preconditioning and the kidney. To address this, rats were randomized to control (C, N = 14), 2 min of ischemic preconditioning (P2 N = 10), 3 periods of 2 min of ischemia separated by 5 min periods of reflow (P2,3 N = 7), or three 5 min periods of ischemia separated by 5 min of reflow (P5,3 N = 6) prior to 45 min of bilateral renal ischemia followed by 24 hours of reperfusion. We observed a lower serum creatinine after 24 hours of reflow in P2, P2, 3 but not P5, 3 rats compared with C. Histology was examined in the C and P2, 3 groups and demonstrated less severe injury in the P2, 3 group. To gain insight into the mechanism by which preconditioning ameliorated ischemic injury, we performed near IR spectroscopy and 31P NMR spectroscopy. Based on near IR spectroscopy, the P2, 3 group had closer coupling of cytochrome aa3 redox state with that of hemoglobin during reflow. In the 31P NMR studies, the changes in ATP and pHi were similar during ischemia, but the P2, 3 group recovered ATP and pHi faster than C. These data suggest that ischemic preconditioning may ameliorate ischemic renal injury as assessed by functional, metabolic and morphological methods. The mechanism(s) by which this occurs requires additional study.

The MEDIGATE graphical user interface for entry of physical findings: design principles and implementation. Medical Examination Direct Iconic and Graphic Augmented Text Entry System.

The solution to many of the problems of the computer-based recording of the medical record has been elusive, largely due to difficulties in the capture of those data elements that comprise the records of the Present Illness and of the Physical Findings. Reliable input of data has proven to be more complex than originally envisioned by early work in the field. This has led to more research and development into better data collection protocols and easy to use human-computer interfaces as support tools. The Medical Examination Direct Iconic and Graphic Augmented Text Entry System (MEDIGATE System) is a computer enhanced interactive graphic and textual record of the findings from physical examinations designed to provide ease of user input and to support organization and processing of the data characterizing these findings. The primary design objective of the MEDIGATE System is to develop and evaluate different interface designs for recording observations from the physical examination in an attempt to overcome some of the deficiencies in this major component of the individual record of health and illness.

Expectant management as an option for men with stage T1c prostate cancer: a preliminary study.

The outcome of patients selecting observation of clinical stage T1c prostate cancer is unknown. A total of 101 men with clinical stage T1c prostate cancer were evaluated, counseled, and monitored in a standard fashion. Altogether, 27 men who elected observation were older and had greater co-morbidity but similar tumor characteristics as compared with 74 men who elected radical prostatectomy. In all, 9 men demonstrated clinical or biochemical evidence of progression after a mean follow-up of 23 months; 4 men who underwent radical prostatectomy had specimen-confined disease and undetectable post-operative levels of PSA. Observation appears to be a viable option for some men with clinical stage T1c prostate cancer.

Cutaneous pseudosarcomatous polyp: a histological and immunohistochemical study.

Two unusual acquired polypoid skin lesions exhibited prominent histological atypia, but were biologically benign. Both patients were elderly females. The lesions clinically mimicked fibroepithelial polyp or nevus lipomatosus. Both had been present for about 20 years. One lesion was located on the back, the other on the posterior thigh. Each lesion exhibited dilated, hyalinized vessels in the dermis with focal fibrin deposits, myxoid stroma, and a population of bizarre, pleomorphic spindle to stellate cells, some of which were multinucleated. Occasional atypical mitoses were present. One lesion had abundant admixed fat. Immunohistochemical staining was strongly positive only for vimentin. The lesions share features with degenerating angiofibroma and vaginal pseudosarcomatous polyp. As in these lesions, the atypia is most probably reactive and degenerative.

Epidermolytic hyperkeratosis in nevi. A possible marker for atypia.

Epidermolytic hyperkeratosis (EH) has been described as a reaction pattern in a variety of solitary skin lesions. We have noted that EH seems to occur more frequently in association with atypical than with typical acquired nevi. To support or refute this observation, the prevalence of EH in 250 cases of atypical compound nevi and in 250 cases of typical compound nevi was determined. EH was identified in 10 cases of atypical compound nevi and in two cases of typical compound nevi, which is a statistically significant difference with p value < 0.02. Since EH occurred in only 4% of atypical nevi examined, it should not be considered a diagnostic criterion. However, the identification of EH in otherwise histologically typical nevi should prompt the pathologist to make sure the lesion has been adequately sampled.

Comparison of subcapsular and total orchiectomy for treatment of metastatic prostate cancer.

OBJECTIVES: To determine whether subcapsular orchiectomy provides suboptimal treatment of metastatic prostate cancer when used to avoid the psychologic consequences of the empty scrotum that results from total orchiectomy. METHODS: We compared testosterone and prostate-specific antigen levels and survival of 37 patients who underwent total orchiectomy and 37 patients who underwent subcapsular orchiectomy for metastatic prostate cancer. RESULTS: The two groups of 37 patients were similar by clinical parameters. Postoperatively, testosterone levels were 21 +/- 11 ng/dL for subcapsular versus 21 +/- 9 ng/dL for total orchiectomy patients. Tumor response was similar in the two groups when assessed by prostate-specific antigen measured 3 weeks, 6 months, and 1, 2, and 3 years postoperatively. Survival was similar when assessed using Kaplan-Meier analysis (P = 0.76). CONCLUSIONS: Subcapsular orchiectomy is a viable option for treatment of metastatic prostate cancer.

The epidemiology of sports and exercise related injury in the United Kingdom.

A national study of exercise related morbidity (ERM) in England and Wales was carried out using a postal questionnaire sent to 28,857 adults aged 16-45 years. The questionnaire asked about regular participation in sports or other recreational fitness activities involving physical exercise, and for details of any injuries occurring during a 28 d reference period. A return rate of 68% was achieved. Comparisons with other national data sources indicated that the information obtained was reliable. It is estimated that each year there are 29 million incidents resulting in new or recurrent injuries, however minor, of which 9.8 million (95% confidence interval 8.1 to 11.4 million) result in new 'substantive' injuries which are potentially serious, result in treatment, or in participants being unable to take part in their usual activities. Soccer accounted for more than 25% of all ERM, but the risk of a substantive injury in rugby was three times that in soccer. Over one third of ERM occurred in men aged 16-25 years. The most frequently reported injuries were sprains and strains of the lower limbs. Treatment was sought in approximately 25% of ERM incidents and 7% of all new ERM incidents involved attendance at a hospital accident and emergency department. The treatment provider most likely to be consulted was a general practitioner, but physiotherapists and complementary medicine practitioners were also consulted frequently. To maximize the health benefits of exercise, research strategies to reduce the volume and severity of ERM and to identify the most appropriate ways of managing ERM should be set.

Intracorneal nuclear dust aggregates in dermatitis herpetiformis. A clue to diagnosis.

Dermatitis herpetiformis has a characteristic histologic pattern consisting of subepidermal blisters often containing fibrin, infiltrates of neutrophils and nuclear dust at tips of dermal papillae, and papillary dermal edema. These are features of early and evolving lesions. We present two cases of clinically typical dermatitis herpetiformis with previously unreported histologic features that may provide a significant diagnostic clue. In each of these cases there were focal collections of nuclear dust in the cornified layer of the epidermis, a finding that may represent a resolving phase of dermatitis herpetiformis, beyond the usual papillary dermal neutrophilic microabscesses seen in early lesions. Biopsy material was available for immunofluorescent studies in one of the cases presented. In addition to the granular pattern of IgA positivity at the dermal-epidermal junction, which is diagnostic of dermatitis herpetiformis, this biopsy also showed similar IgA positivity in the intracorneal nuclear dust aggregates. In the second case, initial sections showed only intracorneal nuclear dust, but at deeper levels there were more typical diagnostic microabscesses at the tips of dermal papillae.

The left ventricular resectoscope. A new instrument for the comprehensive resection of the left ventricular outflow tract in hypertrophic obstructive cardiomyopathy (HCOM).

After disappointing results with non-surgical measures there is now a move back towards surgical left ventricular outflow tract resection as a treatment of choice for hypertrophic obstructive cardiomyopathy. The traditional operation presents various technical difficulties and occasionally disappointing results. Using a diathermy loop to resect the hypertrophied outflow tract has provided a much more convenient and reliable technique. This paper describes an instrument incorporating all the features of this technique (Left ventricular resectoscope).