Sweet's syndrome involoving the musculoskeletal system during treatment of promyelocytic leukemia with all-trans retinoic acid

Induction therapy of promyelocytic leukemia with all-trans retinoic acid is a standard therapy despite significant side-effects. The most important, the "retinoic acid syndrome", consists of a hyperinflammatory reaction with capillary leakage (edema, pleural, and pericardial effusion), infiltration of myeloid cells into internal organs and systemic signs of inflammation. We describe here two cases of another hyperinflammatory reaction during all-trans retinoic acid therapy, the Sweet's syndrome, consisting of infiltrates of the skin and internal organs by neutrophilic granulocytes. Fever, painful erythematous cutaneous plaques, prominent musculoskeletal involvement (myositis, fasciitis), a sterile pulmonary infiltration and intercurrent proteinuria characterized the clinical course of all-trans retinoic acid-associated Sweet's syndrome. Treatment with glucocorticoids led to resolution of the syndrome within 48 h. Three other cases of all-trans retinoic acid-associated Sweet's syndrome without involvement of internal organs, prominent on our cases, were published previously. Recognition of ATRA-associated Sweet's syndrome is of practical importance.

Diffusion-weighted imaging in musculoskeletal radiology-clinical applications and future directions

Diffusion-weighted imaging (DWI) is an established diagnostic tool with regards to the central nervous system (CNS) and research into its application in the musculoskeletal system has been growing. It has been shown that DWI has utility in differentiating vertebral compression fractures from malignant ones, assessing partial and complete tears of the anterior cruciate ligament (ACL), monitoring tumor response to therapy, and characterization of soft-tissue and bone tumors. DWI is however less useful in differentiating malignant vs. infectious processes. As of yet, no definitive qualitative or quantitative properties have been established due to reasons ranging from variability in acquisition protocols to overlapping imaging characteristics. Even with these limitations, DWI can still provide clinically useful information, increasing diagnostic accuracy and improving patient management when magnetic resonance imaging (MRI) findings are inconclusive. The purpose of this article is to summarize recent research into DWI applications in the musculoskeletal system.

Microscopic polyangiitis: Clinical presentation

Microscopic polyangiitis (MPA) is a member of the family of ANCA-associated vasculitides. Its characteristic histology shows a necrotizing small vessel vasculitis with little or absent immune deposits (pauci-immune vasculitis). In Western countries MPA shows a lower prevalence than Wegener's disease, it affects more men than women and commences at the age of > or = 50 years. The two organs most typically involved and often defining prognosis are the kidneys and the lungs. MPA may concomitantly or sequentially involve other organs such as the nervous system, the skin, the musculoskeletal system, but also the heart, the eye and the intestines. Treatment decisions should be based on severity and pattern of organ involvement and respect the five factor score (FFS). Life- or organ- threatening disease is treated with glucocorticoids and (pulse) cyclophosphamide. Plasmapheresis and i.v.immunoglobulins have been shown to be beneficial as additional measure in severe cases. If renal function is preserved, Methotrexate may be considered to induce remission, and if the FFS equals 0, remission may be induced with glucocorticoid monotherapy. Maintenance therapy is recommended with Azathioprin, mycophenolate mofetil may be used as a second line drug. Biologic agents such as monoclonal antibodies to tumor necrosis factor a and B cell depleting rituximab have been shown to bear remission-inducing quality.

Because someone has recommended it - Patients’ reasons for choosing acupuncture or qigong

Question: Recent research has indicated a considerable contribution of placebo effects to the outcome of acupuncture treatments: especially patients’ expectations seem to have an influence [1] and [2]. In this context it is important to better understand the patient–practitioner relationship. The aim of this study was to investigate why patients in Switzerland choose acupuncture or qigong, and what they expect from the treatment and the practitioners [3]. Methods: A qualitative survey with open questions was performed among 38 patients newly enrolled for treatment in 7 different practices for Traditional Chinese Medicine ((TCM); 6 practices for acupuncture, 1 for qigong). Questions aimed to identify reasons for choosing TCM, knowledge about its range of indications, and patients’ expectations towards method and therapist. Answers were categorised and analysed by frequency. Results: The most common reasons for choosing TCM were recommendation by acquaintances, the idea of trying a new treatment and the perception of TCM as being a gentle method. The majority of respondents had poor knowledge about the range of conditions to be treated with TCM: pain of the musculoskeletal system, headaches and chronic problems were considered as main indications. Surprisingly, gynaecological or gastro-intestinal diseases were not mentioned by the respondents. Practitioners were expected to have professional competence, provide information, empathy and understanding. Conclusions: The most striking result of this survey was the fact that patients knew very little about TCM and its indications. Thus, more precise information about TCM and other complementary methods should be offered to the general public, which would help patients to decide whether to consider TCM for the treatment of their disease. And, on the other hand, for the therapists it is important to better understand and respond to patients’ expectations in order to achieve better treatment results. The results of this qualitative survey were briefly discussed with the participating practitioners, who found them remarkable and support further quantitative studies. We plan to further investigate this topic. References 1. J. Pariente, P. White, R.S.J. Frackowiak, G. Lewith. Neuroimage, 25 (2005), pp. 1161–1167 2. M. Karst, D. Schneidewind, D. Scheinichen. Forsch Komplementmed, 17 (2010), pp. 21–27 3. S.D. Klein. Dt Ztschr f Akup, 52 (2009), pp. 18–23

Botulinum Toxin Type A for the Treatment of Equinus Deformity in to Patients With Mucopolysaccharidosis Type II

Mucopolysaccharidoses are lysosomal storage disorders that are caused by a deficiency in the enzymes that degrade glycosaminoglycans. The accumulation of glycosaminoglycans affects multiple systems, resulting in coarse facial features, short stature, organomegaly, and variable neurological changes from normal intelligence to severe mental retardation and spasticity. Effects on the musculoskeletal system include dysostosis multiplex, joint stiffness, and muscle shortening. This article reports 2 patients with mucopolysaccharidosis type II (Hunter syndrome) who showed progressive equinus deformity of the feet. Both patients were treated with intramuscular botulinum toxin type A injections in the gastrocnemius and the soleus muscles, followed by serial casting. In both patients, passive range of motion, muscle tone, and gait performance were significantly improved. Botulinum toxin type A injections followed by serial casting are a therapeutic option for contractures in patients with mucopolysaccharidosis. However, the long-term effects and the effect of application in other muscles remain unknown.

Steady state free precession magnetization transfer imaging

The formerly proposed concept for magnetization transfer imaging (MTI) using balanced steady-state free precession (SSFP) image acquisitions is in this work extended to nonbalanced protocols. This allows SSFP-based MTI of targets with high susceptibility variation (such as the musculoskeletal system), or at ultra-high magnetic fields (where balanced SSFP suffers from considerable off-resonance related image degradations). In the first part, SSFP-based MTI in human brain is analyzed based on magnetization transfer ratio (MTR) histograms. High correlations are observed among all different SSFP MTI protocols and thereby ensure proper conceptual extension to nonbalanced SSFP. The second part demonstrates SSFP-based MTI allowing fast acquisition of high resolution volumetric MTR data from human brain and cartilage at low (1.5T) to ultra-high (7.0T) magnetic fields.

The murine Fgfrl1 receptor is essential for the development of the metanephric kidney

Fgfrl1 is a novel member of the fibroblast growth factor receptor family. Its extracellular domain resembles the four conventional Fgfrs, while its intracellular domain lacks the tyrosine kinase domain necessary for Fgf mediated signal transduction. During embryonic development Fgfrl1 is expressed in the musculoskeletal system, in the lung, the pancreas and the metanephric kidney. Targeted disruption of the Fgfrl1 gene leads to the perinatal death of the mice due to a hypoplastic diaphragm, which is unable to inflate the lungs. Here we show that Fgfrl1-/- embryos also fail to develop the metanephric kidney. While the rest of the urogenital system, including bladder, ureter and sexual organs, develops normally, a dramatic reduction of ureteric branching morphogenesis and a lack of mesenchymal-to-epithelial transition in the nephrogenic mesenchyme result in severe renal dysgenesis. The failure of nephron induction might be explained by the absence of the tubulogenic markers Wnt4, Fgf8, Pax8 and Lim1 at E12.5 of the mutant animals. We also observed a loss of Pax2 positive nephron precursor cells and an increase of apoptosis in the cortical zone of the remnant kidney. Fgfrl1 is therefore essential for mesenchymal differentiation in the early steps of nephrogenesis.

"Because someone has recommended it" - patients' reasons for choosing acupuncture or qigong

Background Recent research has indicated a considerable contribution of placebo effects to the outcome of acupuncture treatments: especially patients’ expectations seem to have an influence [1, 2]. In this context it is important to better understand the patient-practitioner relationship. The aim of this study was to investigate why patients in Switzerland choose acupuncture or qigong, and what they expect from the treatment and the practitioners [3]. Method A qualitative survey with open questions was performed among 38 patients newly enrolled for treatment in 7 different practices for Traditional Chinese Medicine ((TCM); 6 practices for acupuncture, 1 for qigong). Questions aimed to identify reasons for choosing TCM, knowledge about its range of indications, and patients’ expectations towards method and therapist. Answers were categorised and analysed by frequency. Results The most common reasons for choosing TCM were recommendation by acquaintances, the idea of trying a new treatment and the perception of TCM as being a gentle method. The majority of respondents had poor knowledge about the range of conditions to be treated with TCM: pain of the musculoskeletal system, headaches and chronic problems were considered as main indications. Surprisingly, gynaecological or gastro-intestinal diseases were not mentioned by the respondents. Practitioners were expected to have professional competence, provide information, empathy and understanding. Discussion The most striking result of this survey was the fact that patients knew very little about TCM and its indications. Thus, more precise information about TCM and other complementary methods should be offered to the general public, which would help patients to decide whether to consider TCM for the treatment of their disease. And, on the other hand, for the therapists it is important to better understand and respond to patients’ expectations in order to achieve better treatment results. The results of this qualitative survey were briefly discussed with the participating practitioners, who found them remarkable and support further quantitative studies. We plan to further investigate this topic. Literature 1 Pariente J et al., Neuroimage 2005;25:1161-67 2 Karst M et al., Forsch Komplementmed 2010;17:21-7 3 Klein SD., Dt Ztschr f Akup. 2009;52:18-23

Essentials of forensic post-mortem MR imaging in adults

Post-mortem MR (PMMR) imaging is a powerful diagnostic tool with a wide scope in forensic radiology. In the past 20 years, PMMR has been used as both an adjunct and an alternative to autopsy. The role of PMMR in forensic death investigations largely depends on the rules and habits of local jurisdictions, availability of experts, financial resources, and individual case circumstances. PMMR images are affected by post-mortem changes, including position-dependent sedimentation, variable body temperature and decomposition. Investigators must be familiar with the appearance of normal findings on PMMR to distinguish them from disease or injury. Coronal whole-body images provide a comprehensive overview. Notably, short tau inversion–recovery (STIR) images enable investigators to screen for pathological fluid accumulation, to which we refer as “forensic sentinel sign”. If scan time is short, subsequent PMMR imaging may be focussed on regions with a positive forensic sentinel sign. PMMR offers excellent anatomical detail and is especially useful to visualize pathologies of the brain, heart, subcutaneous fat tissue and abdominal organs. PMMR may also be used to document skeletal injury. Cardiovascular imaging is a core area of PMMR imaging and growing evidence indicates that PMMR is able to detect ischaemic injury at an earlier stage than traditional autopsy and routine histology. The aim of this review is to present an overview of normal findings on forensic PMMR, provide general advice on the application of PMMR and summarise the current literature on PMMR imaging of the head and neck, cardiovascular system, abdomen and musculoskeletal system.

MR Spectroscopy and Spectroscopic Imaging for Evaluation of Skeletal Muscle Metabolism: Basics and Applications in Metabolic Diseases

Magnetic resonance spectroscopy (MRS) and spectroscopic imaging (MRSI) provide metabolic information on the musculoskeletal system, thus helping to understand the biochemical and pathophysiological nature of numerous diseases. In particular, MRS has been used to study the energy metabolism of muscular tissue since the very beginning of magnetic resonance examinations in humans when small-bore magnets for studies of the limbs became available. Even more than in other organs, the observation of non-proton-nuclei was important in muscle tissue. Spatial localization was less demanding in these studies, however, high temporal resolution was necessary to follow metabolism during exercise and recovery. The observation of high-energy phosphates during and after the application of workload gives insight into oxidative phosphorylation, a process that takes place in the mitochondria and characterizes impaired mitochondrial function. New applications in insulin-resistant patients followed the development of volume-selective 1H-MRS in whole-body magnets. Nowadays, multinuclear MRS and MRSI of the musculoskeletal system provide several windows to vital biochemical pathways noninvasively. It is shown how MRS and MRSI have been used in numerous diseases to characterize an involvement of the muscular metabolism.

Sleep quality, the neglected outcome variable in clinical studies focusing on locomotor system; a construct validation study

In addition to general health and pain, sleep is highly relevant to judging the well-being of an individual. Of these three important outcome variables, however, sleep is neglected in most outcome studies.Sleep is a very important resource for recovery from daily stresses and strains, and any alteration of sleep will likely affect mental and physical health, especially during disease. Sleep assessment therefore should be standard in all population-based or clinical studies focusing on the locomotor system. Yet current sleep assessment tools are either too long or too specific for general use.