Cyclical Cushing's syndrome: an update.

Purpose Of Review: This article reviews the features of cyclical hypercortisolism. This syndrome was once considered to be very rare but is now being increasingly recognized.

Recent Findings: Either true cycles or the variant of episodic and fluctuating levels of hypercortisolism can lead to considerable clinical dilemmas, which are discussed.

Fatal lung fibrosis associated with immunodeficiency and gonadal dysgenesis in 46XX sisters--a new syndrome.

Inherited immune deficiencies are a heterogeneous group of diseases that can be either isolated, with the immune defect being the exclusive manifestation, or associated with other abnormalities. We report on two sisters, born to consanguineous parents of Sri-Lankan descent, who presented in infancy with immunodeficiency, gonadal dysgenesis, and fatal lung fibrosis. Immune studies demonstrated combined humoral and cellular abnormalities including reduced immunoglobulin production, an absence of lymphoid tissue, markedly reduced T-lymphocyte numbers and function and reduced newly thymus-derived T-cells.

Supportive-expressive group therapy for primary breast cancer patients: a randomized prospective multicenter trial.

Objective: The aim is to evaluate the effectiveness of a manualized 12-week supportive-expressive group therapy program among primary breast cancer patients treated in community settings, to determine whether highly distressed patients were most likely to benefit and whether therapist's training or experience was related to outcome.

Method: Three hundred and fifty-three women within one year of diagnosis with primary breast cancer were randomly assigned to receive supportive-expressive group therapy or to an education control condition. Participants were recruited from two academic centers and nine oncology practices, which were members of NCI's Community Clinical Oncology Program (CCOP) and were followed over 2 years.

Cyclooxygenase-2 expression correlates with phaeochromocytoma malignancy: evidence for a Bcl-2-dependent mechanism.

Aims: Phaeochromocytomas are rare but potentially life-threatening neuroendocrine tumours of the adrenal medulla or sympathetic nervous system ganglia. There are no histological features which reliably differentiate benign from malignant phaeochromocytomas. The aim of the study was to evaluate cyclooxygenase (COX)-2 and Bcl-2 as tissue-based biomarkers of phaeochromocytoma prognosis.

Low serum immunoglobulin G2 levels in infancy can be transient.

Objective: The immunoglobulin G2 subclasses contain predominantly antipolysaccharide antibodies. It was therefore believed intuitively that low immunoglobulin G2 levels could predispose individuals to infections with encapsulated bacteria. Although many reports initially supported this notion, more recent studies challenged it.

Extremes of L-ficolin concentration in children with recurrent infections are associated with single nucleotide polymorphisms in the FCN2 gene.

L-ficolin (also called ficolin-2, P35 or hucolin) is a soluble pattern recognition molecule of suspected importance in anti-microbial immunity. It activates the lectin pathway of complement and acts as an opsonin. l-ficolin, encoded by the FCN2 gene, recognizes microbial polysaccharides and glycoconjugates rich in GlcNAc or GalNAc.

Distribution of the receptor for advanced glycation end products in the human male reproductive tract: prevalence in men with diabetes mellitus.

Background: Diabetics have a significantly higher percentage of sperm with nuclear DNA (nDNA) fragmentation and increased levels of advanced glycation end products (AGEs), in their testis, epididymis and sperm. As the receptor for AGEs (RAGE) is important to oxidative stress and cell dysfunction, we hypothesise, that it may be involved in sperm nDNA damage.

Methods: Immunohistochemistry was performed to determine the presence of RAGE in the human testis and epididymis.

Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase.

We describe a patient with severe combined immunodeficiency because of aberrations in adenosine deaminase (ADA) who despite adequate replacement with polyethylene glycol-linked ADA (PEG-ADA) for 13 years developed Burkitt's lymphoma. Although treatment corrected the metabolic abnormalities caused by ADA deficiency, it failed to fully restore cellular immunity.

Spared ability to recognise fear from static and moving whole-body cues following bilateral amygdala damage.

Bilateral amygdala lesions impair the ability to identify certain emotions, especially fear, from facial expressions, and neuroimaging studies have demonstrated differential amygdala activation as a function of the emotional expression of faces, even under conditions of subliminal presentation, and again especially for fear. Yet the amygdala's role in processing emotion from other classes of stimuli remains poorly understood. On the basis of its known connectivity as well as prior studies in humans and animals, we hypothesised that the amygdala would be important also for the recognition of fear from body expressions.

Strengthening adoption practice, listening to adoptive families.

In-depth interviews with 500 adoptive families who received postadoption services through Virginia's Adoptive Family Preservation (AFP) program paint a richly detailed picture of the challenges adoptive families face and what they need to sustain adoption for many years after finalization. Findings document the need for support in a variety of forms, including respite, counseling, and information. Numerous implications for strengthening adoption practice through effective training and technical assistance are discussed.

Insulin dependant diabetes mellitus: implications for male reproductive function.

Background: Diabetes mellitus (DM) is increasing in men of reproductive age. Despite this, the prevalence of diabetes in men attending fertility clinics is largely unknown. Furthermore, studies examining the effects of DM on sperm fertility potential have been limited to conventional semen analysis.

Clinical pharmacology education and training at the National Institutes of Health.

In 1965, the National Institute of General Medical Sciences (NIGMS) established the intramural Pharmacology Research Associate Training (PRAT) program with the primary goals of providing postdoctoral training in pharmacology for individuals with or without previous pharmacology graduate training, and allowing individuals with doctoral degrees in pharmacology to obtain advanced training in other areas of science at the National Institutes of Health (NIH). The program utilized research preceptors drawn from laboratories that were conducting pharmacology-related research at the NIH campus. Although primary emphasis was placed on training laboratory scientists, a number of PRAT fellows obtained training that enabled them to pursue successful careers in clinical pharmacology.

Spatial and temporal operation of the Scotia Sea ecosystem: a review of large-scale links in a krill centred food web.

The Scotia Sea ecosystem is a major component of the circumpolar Southern Ocean system, where productivity and predator demand for prey are high. The eastward-flowing Antarctic Circumpolar Current (ACC) and waters from the Weddell-Scotia Confluence dominate the physics of the Scotia Sea, leading to a strong advective flow, intense eddy activity and mixing. There is also strong seasonality, manifest by the changing irradiance and sea ice cover, which leads to shorter summers in the south.

Beliefs about the causes of osteoarthritis among primary care patients.

Objective: Beliefs about the causes of osteoarthritis (OA) have been examined infrequently. The purpose of this study was to provide insight into patients' beliefs about the causes of OA in a primary care population.

Methods: This study involved 31 individuals with OA who were purposively sampled to reflect age, sex, and self-efficacy scores from the intervention arm of a randomized controlled trial examining the effectiveness of an arthritis self-management program in primary care.

Use of modified oligonucleotides to compensate for sequence polymorphisms in the real-time detection of norovirus.

Noroviruses are major causative agents of sporadic and outbreak-associated nonbacterial gastroenteritis worldwide. Real-time RT-PCR has rapidly become the principle means for norovirus detection due to its sensitivity and specificity; however sequence variations in the Norovirus genome can cause problems for real-time chemistries. Using a combination of modified bases and minor groove binder-conjugated Eclipse hybridization probes, a one step real-time RT-PCR assay was designed and optimized to detect norovirus genogroups I and II.

Bone marrow transplantation for cartilage-hair-hypoplasia.

The association of cartilage hair hypoplasia (CHH) with severe combined immunodeficiency (SCID) has been known for more than three decades. Bone marrow transplantation (BMT) remains the only effective treatment that might cure SCID. Surprisingly little has been reported on the experience with BMT in CHH.

Self management of arthritis in primary care: randomised controlled trial.

Objective: To evaluate clinical effectiveness of a self management programme for arthritis in patients in primary care with osteoarthritis.

Design: Randomised controlled trial.

Setting: 74 general practices in the United Kingdom.

NMR in the SPINE Structural Proteomics project.

This paper describes the developments, role and contributions of the NMR spectroscopy groups in the Structural Proteomics In Europe (SPINE) consortium. Focusing on the development of high-throughput (HTP) pipelines for NMR structure determinations of proteins, all aspects from sample preparation, data acquisition, data processing, data analysis to structure determination have been improved with respect to sensitivity, automation, speed, robustness and validation. Specific highlights are protonless (13)C-direct detection methods and inferential structure determinations (ISD).