Levels of impairment of sensori-motor functions in children with early brain damage

Sixty-three ambulatory, educable children with early brain damage were tested neurologically, with the WISC, and with supplementary sonsory and perceptual tasks derived from studies of cases of late brain injury. Performance on these latter tasks was also assessed in normal children over a wide range of ages.Early damage seemed to spare elementary sensory function while motor impairment was conspicuous, particularly impairment of the oculomotor system, and the latter correlated with dificient performance on spatial tasks. Performance on a variety of verbal and non-verbal tasks correlated with lateralization of symptoms in the brain-damaged group; a reciprocal pattern emerged depending upon whether the right or left side of their body was predominantly involved.The results suggest that the adult pattern of hemispheric specialization antedates birth and that damage which does not encroach directly on the language zones leaves that pattern intact if somewhat attenuated, particularly in the case of left hemisphere functions. There were in the group of 63 only 12 with right-sided (left hemisphere) neurological signs and only 3 of these were dysphasic. This inequality of lateralizing signs could reflect a sampling bias (childred with right-sided signs may be more language impaired and less “educable”) or some greater invulnerability of the left hemisphere to early damage.     

Memory for remote events in anterograde amnesia: recognition of public figures from newsphotographs.

Patients with anterograde amnesia, including the case H.M. and a group of alcoholic Korsakov cases, were tested for their prompted and unprompted recognition of public figures who had become famous at various points in time from the 1920's to the 1960's. The comparisons between prompted and unprompted recognition for figures from before and after the onsets of the patients' amnesic syndromes were discussed in relation to competing theories of anterograde amnesia. The results were interpreted as not favoring the strong versions of either a faulty-storage or a faulty-retrieval theory.     

Why Do People Die of Anaphylaxis?—A Clinical Review

Anaphylaxis is a source of anxiety for patients and healthcare providers. It is a medical emergency that presents with a broad array of symptoms and signs, many of which can be deceptively similar to other diseases such as myocardial infarction, asthma, or panic attacks. In addition to these diagnostic challenges, anaphylaxis presents management difficulties due to rapid onset and progression, lack of appropriate self-treatment education and implementation by patients, severity of the allergic response, exacerbating medications or concurrent disease, and unpredictability. The most common causes of anaphylaxis are food allergies, stinging insects and immunotherapy (allergy shots) but idiopathic anaphylaxis, latex allergy and drug hypersensitive all contribute to the epidemiology. Reactions to IVP and other dyes are coined anaphylactoid reactions but have identical pathophysiology and treatment, once the mast cell has been degranulated. As many antigens can be the trigger for fatal anaphylaxis, it is useful to examine the features of each etiology individually, highlighting factors common to all fatal anaphylaxis and some specific to certain etiologies. Generally what distinguishes a fatal from non fatal reaction is often just the rapidity to apply correct therapy. Prevention is clearly the key and should identify high-risk patients in an attempt to minimize the likely of a severe reaction. Although fatal anaphylaxis is rare, it is likely underreported.     

Mastocytosis

Variants of mastocytosis can present with puzzling cutaneous and systemic symptoms and signs that can result in an erroneous diagnosis of idiopathic urticaria or idiopathic anayphylaxis. The molecular basis of mastocytosis is now better understood, with updated classification based on distinct growth factor and oncogene abnormalities. Elicitation of a full history and careful attention to the skin examination will usually provide the clinician enough information to deduce that the condition is not simply chronic idiopathic urticaria. This article was presented at the American College of Asthma, Allergy, and Immunology Annual Meeting in Anaheim, CA, November 6, 2005.     

Eosinophilic gastroenteritis and citrus-induced urticaria

The immunological basis of eosinophilic gastroenteritis (EGE) is an interesting contrast between the enigma of urticaria and the increasing usage of molecular technology in clinical allergy. Little is known about the natural history of EGE. It has been known to spontaneously remit, but the typical course, especially in adults, is one of chronic and intermittent disease. Given the often chronic nature of this disease, it is important to use relatively benign treatments initially and limit the use of systemic corticosteroids. Also, given the fact that eosinophilic infiltration of the gastrointestinal tract may also be a manifestation of other potentially dangerous disease processes, such as malignancy or hypereosinophilic syndrome, which may be initially diagnosed as EGE, routine surveillance of the cardiopulmonary and gastrointestinal systems is important. We present a patient who demonstrates the variability of presentation and treatment response in this multifaceted disease. The fact that he has apparently entered remission also makes his an uncommon presentation of EGE.     

Amino acid variations in hepatitis C virus p7 and sensitivity to antiviral combination therapy with amantadine in chronic hepatitis C

BACKGROUND: Formation of transmembrane ion channels by hepatitis C virus (HCV) p7 and abrogation of channel function by amantadine was demonstrated in vitro. The relevance of HCV p7 amino acid (aa) variations for response to antiviral therapy with amantadine is unknown. METHODS: HCV p7 was sequenced in 86 individuals who were infected with HCV genotype 1. Thirty-six of 86 patients received amantadine within an interferon-alpha (IFN-alpha)-based antiviral therapy. Helical wheel modelling for HCV p7 was performed. RESULTS: No significant correlation of overall aa variations within HCV p7 was observed with response to IFN-alpha-based therapy with amantadine in HCV genotype 1alpha/b infected patients. When analysis was restricted to non-conservative aa variations, a higher number of aa substitutions within complete HCV p7 and transmembrane helix 2 was associated with non-response in HCV-1b-infected patients receiving therapy with amantadine (P=0.015 and P=0.037, respectively), without amantadine (P=0.106 and P=0.118, respectively), and in the total cohort of HCV-1b-infected patients (P=0.00007 and P=0.011, respectively). Furthermore, substitution L20F was observed more often in non-responders than responders with HCV-1b infection and therapy with amantadine (P=0.099). By in silico modelling, aa 20 was located toward the p7 channel lumen. Substitution L20F may impair amantadine action by altering the shape of the ion channel pore. CONCLUSION: Substitution L20F within HCV p7 may be associated with non-response to combination therapy specifically with amantadine in HCV-1b-infected patients. Non-responders with HCV-1b infection showed higher numbers of non-conservative aa variations within HCV p7 than responders, irrespective of the application of amantadine.     

Cloning and characterization of profilin (Pru du 4), a cross-reactive almond (Prunus dulcis) allergen

BACKGROUND: The identity of allergenic almond proteins is incomplete. OBJECTIVE: Our objective was to characterize patient IgE reactivity to a recombinant and corresponding native almond allergen. METHODS: An almond cDNA library was screened with sera from patients with allergy for IgE binding proteins. Two reactive clones were sequenced, and 1 was expressed. The expressed recombinant allergen and its native counterpart (purified from unprocessed almond flour) were assayed by 1-dimensional and 2-dimensional gel electrophoresis, dot blot, and ELISA, and screened for cross-reactivity with grass profilin. RESULTS: The 2 selected clones encoded profilin (designated Pru du 4) sequences that differed by 2 silent mutations. By dot-blot analyses, 6 of 18 patient sera (33%) reacted with the recombinant Pru du 4 protein, and 8 of 18 (44%) reacted with the native form. ELISA results were similar. Almond and ryegrass profilins were mutually inhibitable. Two-dimensional immunoblotting revealed the presence of more than 1 native almond profilin isoform. The strength of reactivity of some patients' serum IgE differed markedly between assays and between native and recombinant profilins. CONCLUSION: Almond nut profilin is an IgE-binding food protein that is cross-reactive with grass pollen profilin and is susceptible to denaturation, resulting in variable reactivity between assay types and between patients. CLINICAL IMPLICATIONS: Serum IgE of nearly half of the tested patients with almond allergy reacts with almond nut profilin. Because most patients also had pollinosis, the well-known cross-reactivity between pollen and food profilins could account for this pattern of reactivity.     

Nonallergic Drug Hypersensitivity Reactions

Background: Nonallergic drug hypersensitivities, also referred to as pseudoallergic or anaphylactoid reactions, have clinical manifestations that are often indistinguishable from allergic reactions. Methods: We performed a PubMed search using the terms 'drug allergy, drug hypersensitivity, pseudoallergies, anaphylaxis and nonallergic drug reactions' and reviewed 511 publications dated between 1970 and 2012. A total of 160 papers that were relevant to the most common nonallergic drug hypersensitivity reactions were selected for discussion. Results: Nonallergic drug hypersensitivities do not involve either IgE-mediated (type 1) or delayed (type 4) hypersensitivity. Nonallergic hypersensitivities are commonly referred to as pseudoallergic or idiosyncratic reactions. The common nonallergic drug hypersensitivities are secondary to chemotherapeutic drugs, radiocontrast agents, vancomycin, nonsteroidal anti-inflammatory agents, local anesthetic reactions and opiates. Protocols for skin testing of radiocontrast, nonsteroidal anti-inflammatory agents, local anesthetics and chemotherapeutic agents have been developed, though most have not been validated or standardized. Other diagnostic tests include in vitro-specific IgE tests, and the current 'gold' standard is usually an oral challenge or bronchoprovocation test. In the case of aspirin, even though it is not believed to be IgE-mediated, a 'desensitization' protocol has been developed and utilized successfully, although the mechanism of this desensitization is unclear. Conclusions: Diagnostic methods exist to distinguish allergic from nonallergic drug hypersensitivity reactions. The best option in nonallergic drug hypersensitivity is avoidance. If that is not possible, premedication protocols have been developed, although the success of premedication varies amongst drugs and patients.     

Asthma and Pregnancy

Asthma is probably the most common serious medical disorder that may complicate pregnancy. A third of pregnant women with asthma will experience worsening of their symptoms, a third will see improvement of their symptoms and a third will see no change. The primary goal is to maintain optimal control of asthma for maternal health and well-being as well as fetal maturation. Vital patient education should cover the use of controller medication, avoidance of asthma triggers and early treatment of asthma exacerbations. Proper asthma management should ideally be started in the preconception period. Since smoking is probably the most modifiable risk factor of asthma, pregnant woman should avoid active and passive smoking. Acute asthma exacerbation during the first trimester is associated with an increased risk of congenital malformations. Poorly controlled asthma is associated with low birth weight, preeclampsia, and preterm birth. Medications used for asthma control in the non-pregnant population are generally the same in pregnancy with a few exceptions. Inhaled corticosteroids (ICS) are the preferred controller therapy. Budesonide is the preferred ICS. Long-acting B-agonists (LABA) are the preferred add-on therapy to medium to high dose ICS. Major triggers for asthma exacerbations during pregnancy are viral infections and ICS nonadherence.