Requirements for improving health and well‐being of children with Prader‐Willi syndrome and their families

Prader‐Willi syndrome (PWS) is a rare genetic condition with multi‐system involvement. The literature was reviewed to describe neurodevelopment and the behavioural phenotype, endocrine and metabolic disorders and respiratory and sleep functioning. Implications for child and family quality of life were explored. Challenging behaviours contribute to poorer well‐being and quality of life for both the child and caregiver. Recent evidence indicates healthy outcomes of weight and height can be achieved with growth hormone therapy and dietary restriction and should be the current target for all individuals with PWS. Gaps in the literature included therapies to manage challenging behaviours, as well as understanding the effects of growth hormone on respiratory and sleep function. New knowledge regarding the transition of children and families from schooling and paediatric health services to employment, accommodation and adult health services is also needed. Developing a national population‐based registry could address these knowledge gaps and inform advocacy for support services that improve the well‐being of individuals with PWS and their families.     

Adaptive technique for high-definition MR imaging of moving structures

An adaptive technique for measuring and correcting the effects of patient motion during magnetic resonance image acquisition was developed and tested. A set of algorithms that can reverse the effects of object displacements and phase shifts was used. These algorithms essentially transfer the frame of reference of the image reconstruction from the static frame of the imager couch to the moving "visceral frame." An accurate record of tissue motion during image acquisition is required. To achieve this, the authors used specially encoded "navigator" echoes that are interleaved with the imaging sequence. Postprocessing of the navigator echo data provides a highly detailed record of the displacements and phase shifts that occur during imaging. Phantom studies demonstrated that the technique can directly correct image degradation caused by motion. In contrast to conventional artifact reduction techniques, such as ordered phase encoding and gradient moment nulling, this new method has a unique capacity to reduce motion unsharpness. Preliminary in vivo studies have demonstrated that the technique can markedly improve images degraded by voluntary motion and shows promise for addressing the problem of respiratory motion in thoracoabdominal imaging.     

Retrieval of a displaced detachable coil and intracranial stent with an L5 Merci Retriever during endovascular embolization of an intracranial aneurysm.

Coil displacement during endovascular embolization of an intracranial aneurysm can result in significant thromboembolic complications. To avoid this problem, microsnares and other techniques have been developed to retrieve a lost foreign body in the cerebral circulation. The Merci Retriever (Concentric Medical, Inc., Mountain View, CA) is an FDA-approved device for clot removal in the intracranial vessels. Modifications to its original design have enhanced this device not only for embolectomy but also for intravascular foreign body retrieval. To our review, we report the first use of an L5 Merci Retriever to evacuate a displaced detachable endovascular coil and intracranial stent during aneurysm treatment.     

Scalp psoriasis: European consensus on grading and treatment algorithm

The scalp is a common site of involvement of psoriasis and, for many patients, is a challenging aspect of their disease. This can be attributed not only to the scaling, itching and cosmetic embarrassment that go with scalp psoriasis, but also to the fact that the scalp skin is relatively inaccessible, making topical therapies difficult to apply. The proximity of sensitive facial skin can also limit the use of potentially irritating topical treatments. Nevertheless, the specific challenges of scalp psoriasis are often neglected by treatment guidelines. This paper summarizes the discussions that took place at an international conference of experts convened in Geneva, Switzerland, in March 2008. The objective of the meeting was to review the available treatments for scalp psoriasis in terms of efficacy, safety, convenience, and the implications for patient compliance with treatment. In addition, definitions of mild, moderate and severe scalp psoriasis were agreed. This paper presents a treatment algorithm that includes recommendations for patients in all three categories. It considers the role of potent topical corticosteroids, vitamin D3 derivatives, salicylic acid preparations, and photo- and radiotherapy, as well as systemic therapies, including newer biological agents, for patients with widespread psoriasis with scalp involvement. Data from clinical trials indicate that a potent topical corticosteroid in a short-contact formulation is the most appropriate treatment for most patients with scalp psoriasis.     

Embolization of an iodine-125 radioactive seed from the prostate gland into the right ventricle: An unusual pattern of seed migration

Transperineal permanent brachytherapy using iodine-125 or palladium-103 seeds is a standard treatment modality for localized prostate cancer. Migration of seeds to the lungs is a common phenomenon, whereas migration of seeds to the right ventricle is a rare event. We report a case of iodine-125 seed migration to the right ventricle as demonstrated by chest CT scan and add to the very few published reports on this finding. These rare patients did not suffer adverse effects from such event.     

A randomized trial of leukocyte-depleted platelet transfusion to modify alloimmunization in patients with leukemia

In an effort to determine whether the use of leukocyte (WBC) depleted platelets could modify the development of alloimmunization, 98 adult patients with acute nonlymphocytic leukemia receiving initial induction therapy were randomized to receive standard pooled platelet concentrates (PC) or WBC-depleted PC. WBC depletion was produced by an additional centrifugation of pooled PC, with removal of 81% of WBC and an associated platelet loss of 27%. Lymphocytotoxic antibody (LCTAb) levels were monitored as a serologic marker of alloimmunization. Overall, 5 of 25 evaluable patients receiving WBC-depleted PC developed LCTAb, compared to 13/31 receiving standard PC (p = 0.071). There was no significant difference in alloimmunization rate in the subgroup of patients who had no previous exposure to histocompatibility antigens by pregnancy or prior transfusions (4/15 alloimmunized receiving WBC depleted versus 4/12 receiving standard PC). There was no difference in the number of patients in each group who required HLA-matched platelets during induction therapy. In view of the significant loss of platelets with WBC depletion, the expense and difficulty of providing WBC-poor RBC, the absence of impact on the need for HLA-matched platelets during induction, and the small potential benefit from this approach, WBC- depleted platelets should not be utilized to prevent alloimmunization in patients with leukemia.     

Absolute flow measurements using pulsed Doppler US. Work in progress

Quantitative flow measurements were assessed in both laboratory and canine models using pulsed Doppler ultrasonography (US). A hydrodynamic model consisting of a Harvard pulsatile pump, a water bath, tubing, and a variable resistance reservoir was used to obtain absolute volumetric flow measurements. Parameters including angle of incidence, size of tubing, stroke volume, stroke rate, sample volume, and transducer frequency were changed independently. The effect of varying these parameters on the determination of absolute flow was analyzed. Absolute flow measurements using duplex US were performed in the canine aorta and femoral artery with reference to the electromagnetic flow probe. These data are presented, along with methods to reduce error in flow measurements that can be directly applied to quantitative estimates of blood flow in humans.