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Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2), by providing guidance on common and important clinical issues, such as: 1) How should HAE be diagnosed? 2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? 3) What are the goals of treatment? 4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast feeding women? 5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients.

The plaques associated with Alzheimer's disease are formed as a result of the aggregation of Aβ peptides, which vary in length from 38 to 43 amino acids. The 1-40 peptide is the most abundant, while the 1-42 peptide appears to be the most destructive to neurons and/or glial cells in a variety of assays. We have demonstrated that aggregated Aβ, a state prior to plaque formation, will activate the plasma bradykinin-forming pathway when tested in vitro. Aggregation is zinc-dependent, optimal at 25–50 µM, and the rate of aggregation is paralleled by the rate of activation of the bradykinin-forming pathway as assessed by plasma kallikrein formation. The aggregation of Aβ 1-38, 1-40, and 1-42 is optimal after incubation for 3 days, 3 h, and under 1 min, respectively. The cascade is initiated by the autoactivation of factor XII upon binding to aggregated Aβ; then, prekallikrein is converted to kallikrein, which cleaves high-molecular-weight kininogen (HK) to release bradykinin. Studies by a variety of other researchers have demonstrated the presence of each "activation-step" in either the plasma or spinal fluid of patients with Alzheimer's disease, including activated factor XII, kallikrein, and bradykinin itself. There is also evidence that activation is more prominent as dementia worsens. We now have medications that can block each step of the bradykinin-forming pathway as currently employed for the therapy of hereditary angioedema. Given the current state of therapy for Alzheimer's disease, which includes monoclonal antibodies that retard the rate of progression by 30% at most and have significant side effects, it seems imperative to explore prophylaxis using one of the long-acting agents that target plasma kallikrein or factor XIIa. There is a long-acting bradykinin antagonist in development, and techniques to target kallikrein mRNA to lower levels or knock out the prekallikrein gene are being developed. [ABSTRACT FROM AUTHOR]

This article provides an update on the diagnosis, pathogenesis, and treatment of chronic spontaneous urticaria (CSU). CSU is a form of chronic urticaria characterized by the presence of urticarial lesions most days of the week and no identifiable cause. The article presents an algorithm for the diagnosis of CSU and discusses its association with autoimmunity. The recommended treatments for CSU include high-dose antihistamines, omalizumab, and cyclosporine. Other potential treatments are also being studied. The article emphasizes the importance of properly conducted clinical trials and highlights medications that are not recommended due to low success rates or inadequate study design. [Extracted from the article]

Recognizing barriers to academic success and full student development, some of which are medical in nature, is a primary task of school psychologists. Expanding biomedical information compels school-based psychologists to collaborate with medical professionals when their input can clarify diagnostic issues and expand treatment choices. This article specifies five indications, or red flags, that typically warrant a medical consultation: (a) paroxysmal changes in consciousness, (b) acute changes in functioning, (c) progressive loss of previously mastered developmental milestones or skills, (d) behavioral or attention problems with possible organic bases, and (e) the presence of physical stigmata or dysmorphic features. The article reviews the rationale for seeking medical consultations and suggests with whom school psychologists might consult. In addition, several questions are posed whose answers could inform practice.

Special services usage and related assessment procedures were investigated for 50 students with epilepsy. Fifty-six percent of students with epilepsy received special education services, with mental retardation designation and self-contained placement common, especially among individuals with epilepsy plus a coexisting neurological diagnosis. Sixteen percent of participants had an "other health impairment" designation, and several of these were deemed eligible in at least one other special education category. The majority of students who had been evaluated were administered IQ, adaptive behavior, speech/language, and sensorimotor components, whereas few were assessed for executive or memory functions. Little information about antiepileptic drugs, their effects, or the actual manifestation of students' seizures appeared in special services school documents. Additional empirical information regarding services for children with chronic health conditions, such as epilepsy, is necessary to improve school psychologists' practice. (Contains 6 tables.)

Chronic urticaria is a skin disorder characterized by transient pruritic weals that recur from day to day for 6 weeks or more. It has a great impact on patients' quality of life. In spite of this prevalence and morbidity, we are only beginning to understand its physiopathology and we do not have a curative treatment. Moreover, a patient with chronic urticaria may undergo extensive laboratory evaluations seeking a cause only to be frustrated when none is found. In recent years there have been significant advances in our understanding of some of the molecular mechanisms responsible for hive formation. The presence and probable role of IgG autoantibodies directed against epitopes expressed on the alpha-chain of the IgE receptor and to lesser extent, to IgE in a subset of patients is generally acknowledged. These autoantibodies activate complement to release C5a, which augments histamine release, and IL4 and leukotriene C4 are released as well. A perivascular cellular infiltrate results without predominance of either Th1 or Th2 lymphocyte subpopulations. Basophils of all chronic urticaria patients (autoimmune or idiopathic) are hyperresponsive to serum, regardless of source, but poorly responsive to anti IgE. In this review we will summarize the recent contributions to this field and try to provide insights to possible future directions for research on this disease.