Effect of a gonadotrophin-releasing hormone analogue on lung function in lymphangioleiomyomatosis

BACKGROUND: Lymphangioleiomyomatosis (LAM), a multisystem disease occurring primarily in women, is characterized by cystic lung destruction, and kidney and lymphatic tumors, caused by the proliferation of abnormal-appearing cells (ie, LAM cells) with a smooth muscle cell phenotype that express melanoma antigens and are capable of metastasizing. Estrogen receptors are present in LAM cells, and this finding, along with reports of disease progression during pregnancy or following exogenous estrogen administration, suggest the involvement of estrogens in the pathogenesis of LAM. Consequently, antiestrogen therapies have been employed in treatment. The goal of this prospective study was to evaluate the efficacy of triptorelin, a gonadotrophin-releasing hormone analogue, in 11 premenopausal women with LAM. METHODS: Patients were evaluated at baseline and every 3 to 6 months thereafter, for a total of 36 months. Hormonal assays, pulmonary function tests, 6-min walk tests, high-resolution CT scans of the chest, and bone mineral density studies were performed. RESULTS: Gonadal suppression was achieved in all patients. Overall, a significant decline in lung function was observed; two patients underwent lung transplantation 1 year after study enrollment, and another patient was lost to follow-up. Treatment with triptorelin was associated with a decline in bone mineral density. CONCLUSIONS: Triptorelin appears not to prevent a decline in lung function in patients with LAM. Its use, however, may be associated with the loss of bone mineral density.     

Rehabilitation of Fecal Incontinence After Sphincter-Saving Surgery for Rectal Cancer: Encouraging Results

Purpose  Some patients, having undergone sphincter-saving operations for rectal cancer, may suffer from fecal incontinence. This study was designed to evaluate the results of rehabilitative treatment in patients with fecal incontinence after sphincter-saving operations and to identify the negative factors that influence therapeutic success. Methods  Between January 2000 and June 2007, 88 incontinent patients (54 women; age range, 47–73 years; 69 had received a low anterior rectal resection; 19 a straight coloanal anastomosis) were included in the study. After a preliminary clinical evaluation, including the Wexner Incontinence Scale score, anorectal manometry was performed. All 88 patients underwent rehabilitative treatment according to the “multimodal rehabilitative program” for fecal incontinence. At the end of program, all 88 patients were reassessed by means of a clinical evaluation and anorectal manometry; their results were compared with the clinical and manometric data from ten healthy control subjects. Postrehabilitative Wexner Incontinence Scale scores were used for an arbitrary schedule of patients divided into three classes: Class I, good (score ≤3); Class II, fair (score >3 to ≤6); Class III, poor (score >6). Results  After rehabilitation, there was a significant improvement in the overall mean Wexner Incontinence Scale score (P < 0.03) for both surgical operation types (low anterior rectal resection: P < 0.05; coloanal anastomosis: P < 0.02). Only 21 patients (23.8 percent) were symptom-free, and 37 (42 percent) were considered Class III. A significant postrehabilitative direct correlation was found between: 1) Wexner Incontinence Scale score and degree of genital relaxation (rρ _s 0.78; P < 0.001); 2) Wexner Incontinence Scale score and irradiation (rρ _s 0.72; P < 0.01); and 3) Wexner Incontinence Scale score and pelvic (rρ _s 0.65; P < 0.01) or anal surgery (rρ _s 0.68; P < 0.01). No significant differences were found between prerehabilitative and postrehabilitative anal pressures in low anterior rectal resection and coloanal anastomosis patients. Conclusions  After rehabilitation, some patients become symptom-free, many patients show an improvement in the Wexner Incontinence Scale score, and others exhibit the highest grades of fecal incontinence. Genital relaxation, radiotherapy, and previous pelvic, and/or anal surgery are impeding factors to rehabilitative success.     

Recommendations for the practice of travel medicine

Travel Medicine has emerged as a distinct entity over the last two decades in response to a very substantial increase in international travel and is now forging its own identity, remit and objectives for care of the traveller. Crucial to the formation of any speciality is the definition of recommendations for its practice. This is particularly important and needed for travel medicine as it overlaps with and forms part of day-to-day work in a number of different medical specialities. This document defines a set of recommendations for the practice of travel medicine from the Faculty of Travel Medicine of the Royal College of Physicians and Surgeons of Glasgow. Their objective is to help raise standards of practice and achieve greater uniformity in provision of services, better to protect those who travel. As travel medicine moves towards applying for speciality status, these standards will also contribute to that process.     

Myocardial Edema Imaging by Cardiovascular Magnetic Resonance: Current Status and Future Potential

Cardiovascular magnetic resonance (CMR) imaging is widely established, free of radioactive material or ionizing radiation, and the accepted noninvasive gold standard for numerous noninvasive cardiac markers. Using a technique called T2-weighted imaging, CMR can be used to assess myocardial edema as a reliable marker for acute, potentially reversible myocardial injury. Contrast agents are not required as the myocardial free water content affects the magnetic properties of the tissue, thus providing inherent image contrast. In this review, we illustrate the utility of T2-weighted techniques in the assessment of myocardial edema in a range of clinical scenarios. The detection of myocardial edema is clinically relevant in many acute settings and may be further helpful to better understand the pathophysiology of many non-acute clinical diseases. Currently, T2-weighted CMR represents the only imaging modality that can accurately depict and quantify the presence of myocardial edema in a noninvasive fashion. Thus, T2-weighted imaging should be included in a comprehensive CMR imaging protocol, especially if an acute injury is suspected.     

Bone involvement in adult patients affected with Ehlers-Danlos syndrome.

Summary

The Ehlers-Danlos syndrome is characterized by abnormal connective tissue but bone involvement is debated. We found a reduced BMD and bone quality and increased prevalence of asymptomatic vertebral fractures in eugonadal patients with Ehlers-Danlos syndrome. These findings suggest the need of a bone health evaluation in these patients.

Introduction

The Ehlers-Danlos (EDS) syndrome is characterized by abnormalities of the connective tissue leading to ligamentous laxity and skin and tissue fragility. We evaluated the bone metabolism, bone mineral density (BMD) and bone quality (measured by trabecular bone score, TBS), and the prevalence of vertebral fractures (VFx) in a group of eugonadal adult EDS patients.

Methods

Fifty consecutive Caucasian patients, aged 30–50 years (36 females, 14 males) with classical or hypermobility EDS and 50 age-, gender-, and body mass index (BMI)-matched control subjects were enrolled. In all subjects’ calcium-phosphorous metabolism, bone turnover, BMD at the lumbar spine (LS) and femur (femoral neck, FN and total femur, FT) and TBS by dual-energy X-ray absorptiometry, and the VFx presence by spine radiograph were assessed.

Results

Patients showed reduced BMD (Z-scores LS ?0.45?±?1.00, FN ?0.56?±?1.01, FT ?0.58?±?0.92) and TBS (1.299?±?0.111) and increased prevalence of morphometric VFx (32 %) than controls (Z-scores LS 0.09?±?1.22, FN 0.01?±?0.97, FT 0.08?±?0.89; TBS 1.382?±?0.176; VFx 8 %, p <0.05 for all comparisons), while vitamin D levels, calcium-phosphorous metabolism, and bone turnover were comparable. Fractured EDS patients showed lower TBS values than non-fractured ones (1.245?±?0.138 vs 1.325?±?0.086, p?<?0.05), despite comparable BMD. In EDS patients, the VFx presence was significantly associated with TBS even after adjusting for sex, age, BMD, EDS type, and falls frequency.

Conclusions

EDS patients have reduced BMD and bone quality (as measured by TBS) and increased prevalence of VFx.