Intravenous methylprednisolone in idiopathic childhood nephrotic syndrome

The aim of our study was to determine the clinical course of children with idiopathic childhood nephrotic syndrome (ICNS) who received intravenous methylprednisolone (ivMP) following failure to achieve remission with standard oral prednisolone therapy. This study was designed as a retrospective case record review from 1993 to 2007. Sixteen children received ivMP over the 15-year study period, of whom ten responded, achieving clinical remission. The remaining six children with steroid resistant nephrotic syndrome (SRNS) underwent biopsy [four focal segmental glomerulosclerosis (FSGS), two minimal change disease (MCD)]. Three responders developed late secondary steroid resistance (two FSGS, one MCD). At the latest follow-up (mean 6.7 years), three of the ten ivMP responders and none (0/6) of the children with SRNS had heavy proteinuria and chronic kidney disease (CKD) stage 3-5. The remaining 13 children demonstrated significant steroid dependency but had achieved stable remission following cyclophosphamide and/or ciclosporin therapy. The majority of children with ICNS who do not respond to 4 weeks of daily prednisolone therapy will enter remission following three to five doses of ivMP, thus avoiding a renal biopsy at initial presentation. These children are likely to develop steroid dependency, and the majority will require treatment with alkylating agents and/or ciclosporin to maintain remission. The requirement for ivMP in this setting appears to be associated with a risk of developing CKD in the longer term.     

Proactive interference as a result of persisting neural representations of previously learned motor skills in primary motor cortex

Learning to control movements in different dynamic environments is marked by proactive interference; learning a first skill interferes with the subsequent learning of a second one. The neural basis of this effect is poorly understood. We tested the idea that proactive interference results from persisting neural representations of previously learned skills in the primary motor cortex (M1). We used repetitive transcranial magnetic stimulation (rTMS) of M1 to disrupt retention of a recently learned motor skill. If interference results from the retention of this skill then its disruption should be associated with reduced interference. Subjects reached to targets while interacting with a robotic arm that applied force fields to the limb. Fifteen minutes of 1-Hz rTMS to M1 impaired the retention of a first force field, and more importantly, reduced proactive interference when subjects learned a second one. Our findings suggest that retention and interference are linked at the level of M1.     

Mindfulness-based psychotherapies: a review of conceptual foundations, empirical evidence and practical considerations

OBJECTIVE: This paper, composed by an interest group of clinicians and researchers based in Melbourne, presents some background to the practice of mindfulness-based therapies as relevant to the general professional reader. We address the empirical evidence for these therapies, the principles through which they might operate, some practical questions facing those wishing to commence practice in this area or to refer patients into mindfulness-based therapies, and some considerations relevant to the conduct and interpretation of research into the therapeutic application of mindfulness. METHOD: Databases (e.g. PsycINFO, MEDLINE) were searched for literature on the impact of mindfulness interventions, and the psychological and biological mechanisms that underpin the effects of mindfulness practice. This paper also draws upon the clinical experience of the author group. RESULTS: Mindfulness practice and principles have their origins in many contemplative and philosophical traditions but individuals can effectively adopt the training and practice of mindfulness in the absence of such traditions or vocabulary. A recent surge of interest regarding mindfulness in therapeutic techniques can be attributed to the publication of some well-designed empirical evaluations of mindfulness-based cognitive therapy. Arising from this as well as a broader history of clinical integration of mindfulness and Western psychotherapies, a growing number of clinicians have interest and enthusiasm to learn the techniques of mindfulness and to integrate them into their therapeutic work. This review highlights the importance of accurate professional awareness and understanding of mindfulness and its therapeutic applications. CONCLUSIONS: The theoretical and empirical literatures on therapeutic applications of mindfulness are in states of significant growth and development. This group suggests, based on this review, that the combination of some well-developed conceptual models for the therapeutic action of mindfulness and a developing empirical base, justifies a degree of optimism that mindfulness-based approaches will become helpful strategies to offer in the care of patients with a wide range of mental and physical health problems.     

Experience with covered stents for the management of hemodialysis polytetrafluoroethylene graft seromas

Prosthetic graft seromas is a rare complication that has been traditionally managed with open methods using partial graft replacement and open drainage. We report the first two cases of hemodialysis graft seromas successfully treated with a covered stent. Both patients underwent arteriovenous graft placement from the brachial artery to the axillary vein using a standard wall, tapered 4 to 7 mm polytetrafluoroethylene graft, but developed a seroma at the arterial end of the graft. Unsuccessful attempts were made to treat these seromas with percutaneous and open drainage. In both patients, an 8 mm x 50 mm Wallgraft (Boston Scientific, Natick, Mass) was retrogradely deployed "bareback" at the arterial end of the graft allowing for complete resolution of the graft seromas.     

EPOS - 27th Annual Meeting

Over the last 15 years, great improvements in genetic engineering and genetic manipulation strategies have led to significant advances in the understanding of the genetics governing embryological limb development. This field of science continues to develop, and the complex genetic interactions and signalling pathways are still not fully understood. In this review we will discuss the roles of the principle genes involved in the three-dimensional patterning of the developing limb and will discuss how errors in these signalling cascades correlate to congenital limb deformity in humans. This review is aimed at orthopaedic surgeons wishing to understand the principles of congenital limb deformity related to genetic signalling errors. It is by no means a comprehensive study of the molecular genetics governing the complex interactions involved in each step of limb development. There are however many syndromes involving limb deformity for which the molecular causes are unknown.     

Autologous in vivo adipose tissue engineering in hyaluronan-based gels--a pilot study

BACKGROUND: There is a major clinical need for strategies for adequately reconstructing the soft tissue defects found after deep burns, tumor resection, or trauma. A promising solution is adipose tissue engineering with preadipocytes, stem-cell derived precursors of the adipose tissue, implanted within biomaterials. This pilot study evaluated hyaluronan gels mixed with autologous undifferentiated preadipocytes in a pig model for their potency to generate new fat. MATERIALS AND METHODS: Preadipocytes were isolated from intra-abdominal pig fat by collagenase digestion, plated on fibronectin-coated culture dishes in Dulbecco's modified Eagle medium/Ham's F12 (Biochrom, Berlin, Germany) combined with 10% pig serum, expanded, and mixed with hyaluronan gel. Two types of gels with varying degrees of amidation of the carboxyl groups were tested (HYADD3, HYADD4). Cell-loaded gels and unseeded controls were injected subcutaneously into the ears of three pigs, explanted at 6 wk, and analyzed histologically. RESULTS: Both cell-loaded specimens were detected macroscopically. They demonstrated a slight volume effect with limited stability after 6 wk. Unloaded HYADD3 and HYADD4 controls could not be identified at the time of explantation. Histology of HYADD3 revealed islets of mature adipocytes and vessels embedded in fat tissue surrounded by gel. In contrast, no fat formation was found in HYADD4 gels when implanted in the ear. CONCLUSIONS: Histological findings demonstrate that HYADD3 is a promising gel for generating adipose tissue. Even though HYADD3 might be a potential material for the reconstruction of small tissue defects, the question remains as to whether the adipose tissue within the gel is attributable to preadipocyte maturation or ingrowth from neighboring tissue.     

Concomitant off-pump myocardial revascularization and pheochromocytoma resection

Surgical management of patients with coexisting ischemic heart disease and pheochromocytoma remains challenging. We present one such case in which hybrid myocardial revascularization (angioplasty with stenting and off-pump coronary artery bypass grafting) and resection of pheochromocytoma were undertaken. Unusual features included simultaneous coronary artery surgery and tumor resection and, in particular, coronary artery surgery being performed without cardiopulmonary bypass.     

Histological abnormalities of the calcaneum in congenital talipes equinovarus

Six calcaneal fragments from patients aged 2, 3, 4, and 5 years with relapsed talipes, and two normal feet from a 40-week-old stillborn fetus were studied. All tissue was sectioned in the sagittal or coronal plane and stained using alcian blue and sirius red to distinguish cartilage and bone. Immunocytochemistry was performed to illustrate collagen types I and II. Within the clubfoot calcaneum, there were fewer chondrocytes and a diminished number of cartilage canals. Although a growth plate was present, the zones of differentiated chondrocytes were not apparent and the chondrocytes were smaller and flatter. The alcian blue staining within the spherical physis was paler than normal, suggesting that the amount of extracellular proteoglycans was reduced. Overall, the growth plate region of the talipes calcaneum resembled that of a permanent cartilage, like articular cartilage. Abnormalities were also seen in the ossification center. Cartilage spicules were rare, and developing bone frequently abutted directly onto the growth plate cartilage. The relative absence of a primary spongiosa suggested that the physis was virtually inactive and endochondral bone formation was retarded. These findings are consistent with the hypothesis that an intrinsic primary growth disorder causes the formation of a small hypoplastic bone and, subsequently, a smaller foot. Received: February 21, 2001 / Accepted: June 12, 2001