Hereditary angioedema with normal C1 inhibitor function: consensus of an international expert panel.

A new form of hereditary angioedema (HAE) with normal C1 inhibitor (C1INH) was first described in 2000. The lack of clear diagnostic criteria, the heterogeneity among affected patients, and the varying names given to this disease have led to substantial confusion among both physicians and patients. This study was designed to bring more clarity to the diagnosis and potential treatment of HAE with normal C1INH.

Apolipoprotein E, especially apolipoprotein E4, increases the oligomerization of amyloid β peptide.

Alzheimer's disease (AD) is the most common progressive neurodegenerative disorder causing dementia. Massive deposition of amyloid β peptide (Aβ) as senile plaques in the brain is the pathological hallmark of AD, but oligomeric, soluble forms of Aβ have been implicated as the synaptotoxic component. The apolipoprotein E ε 4 (apoE ε4) allele is known to be a genetic risk factor for developing AD.

Anacardic acid inhibits the catalytic activity of matrix metalloproteinase-2 and matrix metalloproteinase-9.

Cashew nut shell liquid (CNSL) has been used in traditional medicine for the treatment of a wide variety of pathophysiological conditions. To further define the mechanism of CNSL action, we investigated the effect of cashew nut shell extract (CNSE) on two matrix metalloproteinases, MMP-2/gelatinase A and MMP-9/gelatinase B, which are known to have critical roles in several disease states. We observed that the major constituent of CNSE, anacardic acid, markedly inhibited the gelatinase activity of 3T3-L1 cells.

Case for an RNA-prion world: a hypothesis based on conformational diversity.

Prions and other misfolded proteins can impart their structure and functions to normal molecules. Based upon a thorough structural assessment of RNA, prions and misfolded proteins, especially from the perspective of conformational diversity, we propose a case for co-existence of these in the pre-biotic world. Analyzing the evolution of physical aspects of biochemical structures, we put forward a case for an RNA-prion pre-biotic world, instead of, merely, the "RNA World".

Using a discrete choice experiment to elicit the demand for a nutritious food: willingness-to-pay for orange maize in rural Zambia.

Using a discrete choice experiment, this paper estimates the willingness to pay for biofortified orange maize in rural Zambia. The study design has five treatment arms, which enable an analysis of the impact of nutrition information, comparing the use of simulated radio versus community leaders in transmitting the nutrition message, on willingness to pay, and to account for possible novelty effects in the magnitude of premiums or discounts. The estimation strategy also takes into account lexicographic preferences of a subset of our respondents.

Structure activity relationship of plumbagin in BRCA1 related cancer cells.

It has been shown earlier that plumbagin, a naturally occurring naphthaquinone has specific anticancer activity in BRCA1 blocked ovarian cancer cells. Plumbagin can induce estrogen dependent cell signaling and apoptosis in BRCA1 blocked ovarian cancer cells. Being a reactive oxygen species (ROS) generator and apoptosis inducing agent, plumbagin has immense potential as a promising anticancer agent.

Hereditary angioedema: classification, pathogenesis, and diagnosis.

Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder associated with a deficiency in C1 inhibitor. More than 200 mutations in this gene, located on chromosome 11, have been identified. Although HAE is often inherited, 20-25% of cases are from new spontaneous mutations and they have no family history of swelling.

A protocol for risk stratification of patients with carboplatin-induced hypersensitivity reactions.

Background: Management of patients with carboplatin-induced hypersensitivity reactions (HSR) has been complicated by high false-negative rates of carboplatin skin test (ST) results. These patients might be at risk for future carboplatin-induced HSRs. In this article we identify a strategy to improve risk stratification of patients with a history of carboplatin-induced HSRs by using a protocol that includes repeat skin testing and drug desensitization.

DCE-MRI model selection for investigating disruption of microvascular function in livers with metastatic disease.

Purpose: To evaluate the Akaike information criterion (AIC) model selection technique as a method for detecting differences in microvascular characteristics between tumorous and non-tumor liver tissue.

Materials And Methods: The AIC was applied to six patient datasets with liver metastases to determine, on a per voxel basis, which of two physiologically plausible candidate models gave a more appropriate description of the data. The dual-input single-compartment Materne model, extended to incorporate a novel portal input function estimation method, was chosen to represent liver tissue and the single-input dual-compartment extended Kety model was used for tumor.

Synthesis and antioxygenic activities of seabuckthorn flavone-3-ols and analogs.

A practical synthesis of polyhydroxy- and regiospecifically methylated flavone-3-ols which are components of commercial 'seabuckthorn flavone' has been achieved by modified Algar-Flynn-Oyamada method. Antioxidant activities of seabuckthorn extracts, isolated products and a number of flavone-3-ols have been determined. Structure-activity relationships have been discussed.

A novel biflavonyloxymethane from Pongamia pinnata and its radical quenching activity.

The root bark of Pongamia pinnata Pierre (syn P. glabra Vent.) has afforded a new biflavonyloxymethane, pongabiflavone, along with a known furanoflavone, 3-methoxy-(7, 8, 2", 3") furanoflavone.

Fractal symmetry of protein interior: what have we learned?

The application of fractal dimension-based constructs to probe the protein interior dates back to the development of the concept of fractal dimension itself. Numerous approaches have been tried and tested over a course of (almost) 30 years with the aim of elucidating the various facets of symmetry of self-similarity prevalent in the protein interior. In the last 5 years especially, there has been a startling upsurge of research that innovatively stretches the limits of fractal-based studies to present an array of unexpected results on the biophysical properties of protein interior.

A decade of respiratory syncytial virus epidemiology and prophylaxis: translating evidence into everyday clinical practice.

Respiratory syncytial virus (RSV) is a common infection in infancy, with nearly all children affected by two years of age. Approximately 0.5% to 2.

Predictors of hospital admission for food-related allergic reactions that present to the emergency department.

Background: guidelines do not provide specific hospitalization criteria for patients presenting to the emergency department (ED) with food-related allergic reactions.

Objective: to determine predictors of hospital admission for ED patients with food-related allergic reactions.

Methods: we performed a medical record review at 3 academic centers of patients presenting to the ED for food-related allergic reactions (International Classification of Diseases, Ninth Revision, Clinical Modification codes 693.

Age-related differences in the clinical presentation of food-induced anaphylaxis.

Food-induced anaphylaxis may be more difficult to recognize in younger children. We describe age-related patterns in the clinical presentation of children with anaphylaxis, which may facilitate the early recognition and treatment of this potentially life-threatening condition.

Current treatment of hereditary angioedema: An update on clinical studies.

Hereditary angioedema (HAE) is a rare, potentially life-threatening disease that manifests as recurrent episodes of nonpruritic swelling that may affect the extremities, face, genitalia, gastrointestinal tract, and/or larynx. HAE is the result of a deficiency of functional C1-esterase inhibitor (C1-INH), a key regulator of the complement, coagulation, and kallikrein-kinin cascades. In HAE patients, overactivation of the kallikrein-kinin cascade results in excessive release of bradykinin, the mediator of the pain and swelling that is characteristic of HAE.

A new biflavonyloxymethane from Pongamia pinnata.

The root bark of Pongamia pinnata Pierre Syn Pongamia glabra (Family: Fabaceae) has afforded a new biflavonyloxymethane, karanjabiflavone, along with a known furanoflavone, pongapin. The structure of this new biflavonyloxymethane was elucidated from extensive spectral studies including 2D-NMR experiments. Both of these compounds possess antioxidant activity.

Transcatheter closure of ruptured sinus of Valsalva aneurysm.

Congenital sinus of Valsalva aneurysm is a rare cardiovascular anomaly. Rupture of sinus of Valsalva (RSOV) is a rare occurrence, occurring in 10-20% of cases. Rupture into right atrium is further a rare occurrence.

Repeat epinephrine treatments for food-related allergic reactions that present to the emergency department.

To date, there are sparse data on epinephrine treatment for food-related anaphylaxis in adults. We sought to establish the frequency of more than one epinephrine treatment for adult patients who present with food-related anaphylaxis to the emergency department (ED). We performed a chart review, at two academic centers, of all adults presenting to the ED for food allergy (ICD9CM codes 693.

Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema.

Background: Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist.

Multiple epinephrine doses for stinging insect hypersensitivity reactions treated in the emergency department.

Background: Data are sparse on epinephrine treatment for stinging insect hypersensitivity (SIH) reactions.

Objective: To establish the frequency of receiving more than 1 dose of epinephrine in patients presenting to the emergency department (ED) with SIH reactions.

Methods: We performed a medical record review of all 153 patients with SIH reactions presenting to 3 EDs in Boston, Massachusetts, between January 1, 2001, and December 31, 2006.