Systemic lupus erythematosus presenting with a choreo-athetosic syndrome associated with antiphospholipid antibodies

INTRODUCTION: Chorea is a rare manifestation of systemic lupus erythematosus (1-4 percent), commonly affecting young woman. Chorea is revealing lupic disease in 50 percent, in the other cases it occurs early in the course of the disease. OBSERVATION: A 33-year-old woman was hospitalized for choreo-athetosic movements prevailing on the left leg and arm accompanied by behavioral and general state deterioration. The biological assessment consolidated the diagnosis of lupic disease associated antiphospholipides antibody (aPL). Cerebral magnetic resonance imaging (MRI) was normal except for cortical and subcortical atrophy. The patient was treated by corticosteroids (1mg/kg/day) and then was lost to follow-up. CONCLUSION: We review data in the literature on the pathophysiological mechanisms of lupic chorea focusing particularly on role of aPL.     

Malaria transmission by Anopheles dirus in Attapeu Province, Lao PDR

A study was carried out in four malaria-endemic villages in Attapeu Province, in the southern region of Lao PDR. All-night human landing collections were carried out in May, August, and October 2002, to determine malaria vectors. At the same time, mass blood surveys were also carried out in the same villages. Anopheles dirus was the predominant species in three of the study villages. Sporozoites were found only in An. drius from Phou Hom. However, in Beng Phoukham, An. dirus was positive for oocysts. The distribution of malaria cases was highest in Phou Hom and this correlated well with the vectorial capacity of An. dirus. The risk for infection from An. dirus was also high, at 0.99.     

Pancreas and islet transplantation

The aim of pancreas and islet transplantation is to establish the same status of glucose control that is provided by endogenous secretion of insulin from a healthy native pancreas in order to improve the quality of life and ameliorate secondary diabetic complications in patients with type I insulin-dependent diabetes mellitus (IDDM). Islet transplantation is, theoretically, an ideal solution for patients with IDDM since it is not a major procedure, can be performed radiologically and can be repeated several times without any major discomfort to the patient, but despite experimental and clinical efforts over the past 25 years, long term and consistent insulin independence has not yet been achieved. Pancreas transplantation is indicated for patients with IDDM following also additional selection criteria. In a suitable candidate, the evaluation is also needed to determine the type of pancreas transplantation, based mainly on the degree of nephropathy. Details of the recipient operation together with the anti-reject procedures and actual global results are described analytically. Similar considerations are dedicated to the islet transplantation procedure.     

Platelet-derived growth factor B, but not fibroblast growth factor 2, plasmid DNA improves survival of ischemic myocutaneous flaps

HYPOTHESIS: Tissue flaps are commonly used for surgical reconstruction, especially to cover difficult wounds and in breast reconstruction following mastectomy. Complications due to inadequate flap perfusion are a source of morbidity and, in the lower extremity, can result in amputation. SETTING: Laboratory. INTERVENTIONS: We evaluated the ability of platelet-derived growth factor (PDGF) B and fibroblast growth factor 2 plasmid DNA, formulated in a type I collagen matrix, to promote tissue survival in a rat transverse rectus abdominis muscle flap model based on the inferior deep epigastric vascular supply. In the absence of any therapeutic agent, only about 24% of flap tissue survives in this model. The DNA/matrix formulations were delivered subcutaneously into the skin paddles 7 days before flap elevation, and tissues were harvested 7 days later. RESULTS: Our studies reveal dramatic increases in overall vascularity after treatment with PDGF-B and fibroblast growth factor 2 plasmid DNA; however, only PDGF-B increased flap survival (130% increase at 228 micro g/cm(2) of plasmid DNA vs controls; P<.01). Transdermal spectral imaging demonstrated an increase in patent vessels supporting blood flow in flaps treated with PDGF-B plasmid DNA vs the fibroblast growth factor 2 transgene. CONCLUSION: Matrix-enabled gene therapy may provide an effective nonsurgical approach for promoting flap survival and is well suited for surgical applications in which transient therapeutic transgene expression is desired.     

Ethnicity and survival on dialysis in west London

BACKGROUND: Indo-Asian and Afro-Caribbean patients have higher rates of renal failure and requirement for renal replacement therapy than the general population in the UK. Despite this, information regarding survival on dialysis is limited. METHODS: The incident hemodialysis population of a large west London renal service was reviewed from 1996 to 2001 (N = 465). RESULTS: The cohort's ethnic background was Indo-Asian (30.8%), Caucasian (49%), Afro-Caribbean (18.3%), and other (1.9%). Indo-Asians and Afro-Caribbeans were younger than Caucasian patients, with a higher rate of diabetes mellitus. Survival on hemodialysis for Indo-Asians was 97.5% and 81.6% at 1 and 3 years, respectively, compared with 92.7% and 75.2% for Caucasians, and 97.5% and 85.3% for Afro-Caribbeans (P = nonsignificant). Dialysis adequacy was observed to be associated with survival. Patients with mean single pool Kt/V of over 1.4 had survival of 90.6% and 74.8% at 2 and 5 years, respectively, compared with 74.0% and 42.9% for those with Kt/V less than 1.4 (P < 0.001). There were significantly more patients in the Indo-Asian cohort with a mean Kt/V of 1.4 or over (87.4%) compared with Caucasians (57.6%) and Afro-Caribbeans (52.4%), and the benefit of higher Kt/V was seen in all ethnic groups. In a multivariate analysis of factors including Kt/V over 1.4, age, diabetic status, gender, and ethnicity, Indo-Asian or Afro-Caribbean ethnicity did not confer a survival disadvantage. The strongest predictors of survival were age and dialysis adequacy. CONCLUSION: Indo-Asian and Afro-Caribbean hemodialysis patients have survival comparable to Caucasians despite a higher burden of diabetes.     

Ethical issues in non-heart-beating donation

A shortage of organ donors and the large number of patients desperately waiting for kidney transplant have led to the search for new sources of transplantable organs. The waiting list has grown at an alarming rate resulting in increased waiting times and deaths. The introduction of non heart beating (NHB) donation programmes generates a lot of ethical issues. How should death of a patient be defined in the case of NHB donation? Is there a strict separation of responsibilities of the medical teams in the different phases of the procedure (patient treatment and actual donation)? How should consent be obtained? Is sufficient respect and care given to the patient and his family? How is the viability of the organs assessed and how should the organs be allocated? We believe that it is very important to debate these issues and to try to outline an ethical framework for NHB donation that can enjoy the widest possible community support.     

The genetic contribution to carpal tunnel syndrome in women: a twin study

OBJECTIVE: To assess the relative genetic and environmental contribution to carpal tunnel syndrome (CTS) using a classic twin study of monozygotic (MZ) and dizygotic (DZ) twins.METHODS:The study group comprised unselected female twin pairs, between 20 and 80 years of age, from the St Thomas' UK Adult Twin Registry. Individuals completed a questionnaire that included details on potential risk factors for CTS. The diagnosis of CTS was made using a standardized hand pain diagram and validated criteria. The genetic contribution to CTS was assessed using variance component and regression methods, the heritability was adjusted for environmental confounders. The role of individual risk factors was assessed by a nested case-control study.RESULTS: An overall prevalence of 14.2% for CTS was found in a population of 4,488 females, comprising 867 MZ and 970 DZ twin pairs, and 814 singletons. The concordance for CTS was significantly higher in MZ compared with DZ twins (case-wise concordance values of 0.35 and 0.24 respectively, with a significantly increased MZ:DZ ratio of 1.48; P = 0.03). Modeling produced a heritability estimate of 0.46 (95% CI 0.34-0.58) that was essentially unchanged after adjustment for environmental risk factors including age, body mass index, physical activities, and hormonal/reproductive factors. No major influence of any individual risk factor was seen in the case-control analysis of 520 cases and 3,154 controls, apart from a modest association with menopausal status with an increased risk of 1.53 and 1.43 in the peri and postmenopausal groups. There was no overall effect of age or body mass index.CONCLUSION: This is the first study to explore the genetic component of CTS. Our data show that up to half of the liability to CTS in women is genetically determined, and this appears to be the single strongest risk factor, with only minor contributions from known environmental factors. Further studies should focus on genetic mechanisms that may lead to tests for susceptibility and detection of those at risk of developing CTS.     

Optimal reference mammography: a comparison of mammograms obtained 1 and 2 years before the present examination

OBJECTIVE: We assessed and compared the benefit of using images acquired 1 year or 2 years previously during mammography interpretations. MATERIALS AND METHODS: Eleven radiologists and one resident reviewed 128 cases three times: once without prior mammograms for comparison, once with mammograms from the most recent (1 year) examination, and once with mammograms acquired 2 years previously. They were asked to determine whether the patient should be recalled for additional procedures. Performances under the three conditions were compared. RESULTS: Radiologists were significantly more accurate (p < 0.001) when comparison mammograms (obtained 1 or 2 years previously) were available. Although sensitivity was not significantly affected between the availability of mammograms from 1 or 2 years earlier (p > 0.10), the specificity was. Specificity using mammograms from the latest examination (obtained 1 year previously) as a reference was significantly better (p = 0.03) than specificity using mammograms obtained 2 years previously. CONCLUSION: Comparison mammograms are important for accurate diagnosis-in particular, for increasing specificity. The latest prior examination seems to be the optimal one for this purpose.     

Lower extremity above-knee prosthesis-associated erectile dysfunction

Blunt pelvic and perineal trauma has been previously reported to result in site-specific veno-occlusive dysfunction and/or site-specific cavernosal artery insufficiency. We herein describe a case of erectile dysfunction in a young previously potent amputee. We postulate that the erectile dysfunction is associated with a newly described form of blunt trauma, that is, site-specific compression from a perineal weight-bearing lower extremity above-knee prosthetic device. It is hypothesized that when the force exerted by the above-knee prosthesis is directed medially towards the ischiopubic ramus, the penile crura and common penile arterial blood supply become susceptible to crush-like injury, since they are in fixed anatomic locations in the perineum sandwiched between the compressive force and the bone. Clinical evaluation of the erectile dysfunction in this patient revealed site-specific corporal veno-occlusive dysfunction and site-specific common penile arterial occlusive pathology in the precise region of the contact of the above-knee prosthesis with the perineum. Further research is needed in above-knee prosthesis design to prevent erectile dysfunction.