scholarly journals Differences and Similarities in the Mechanisms and Clinical Expression of Bradykinin-Mediated vs. Mast Cell–Mediated Angioedema

Author(s):  
Marcus Maurer ◽  
Markus Magerl

AbstractAngioedema (AE), transient localized swelling due to extravasated fluid, is commonly classified as mast cell mediator-induced, bradykinin-mediated or of unknown cause. AE often occurs more than once, and it is these recurrent forms of AE that are challenging for patients and physicians, and they are the ones we focus on and refer to as AE in this review. Since effective treatment depends on the causative mediator, reliable and early diagnosis is essential. Although their clinical presentations bear similarities, many forms of angioedema exhibit specific patterns of clinical appearance or disease history that may aid in diagnosis. Here, we describe the most common differences and similarities in the mechanisms and clinical features of bradykinin-mediated and mast cell mediator-induced types of angioedema. We first provide an overview of the diseases that manifest with mast cell mediator-induced versus bradykinin-mediated angioedema as well as their respective underlying pathogenesis. We then compare these diseases for key clinical features, including angioedema location, course and duration of swelling, attack frequency, prevalence and relevance of prodromal signs and symptoms, triggers of angioedema attacks, and other signs and symptoms including wheals, age of onset, and duration. Our review and comparison of the clinical profiles of different types of angioedema incorporate our own clinical experience as well as published information. Our aim is to highlight that mast cell mediator-induced and bradykinin-mediated angioedema types share common features but are different in many aspects. Knowledge of the differences in underlying pathomechanisms and clinical profiles between different types of angioedema can help with the diagnostic approach in affected patients and facilitate targeted and effective treatment.

Author(s):  
Philip Cowen ◽  
Paul Harrison ◽  
Tom Burns

Chapter 1 is concerned with the definition of the key symptoms and signs of psychiatric disorders. Having elicited a patient’s symptoms and signs, the psychiatrist needs to decide how far these phenomena fall into a pattern that has been observed in other psychiatric patients. It covers whether the clinical features conform to a recognized syndrome by combining observations about the patient’s present state with information about the history of the condition. The value of identifying a syndrome is that it helps to predict prognosis and to select an effective treatment. It does this by directing the psychiatrist to the relevant body of accumulated knowledge about the causes, treatment, and outcome in similar patients.


2017 ◽  
Vol 2 (15) ◽  
pp. 9-23 ◽  
Author(s):  
Chorong Oh ◽  
Leonard LaPointe

Dementia is a condition caused by and associated with separate physical changes in the brain. The signs and symptoms of dementia are very similar across the diverse types, and it is difficult to diagnose the category by behavioral symptoms alone. Diagnostic criteria have relied on a constellation of signs and symptoms, but it is critical to understand the neuroanatomical differences among the dementias for a more precise diagnosis and subsequent management. With this regard, this review aims to explore the neuroanatomical aspects of dementia to better understand the nature of distinctive subtypes, signs, and symptoms. This is a review of English language literature published from 1996 to the present day of peer-reviewed academic and medical journal articles that report on older people with dementia. This review examines typical neuroanatomical aspects of dementia and reinforces the importance of a thorough understanding of the neuroanatomical characteristics of the different types of dementia and the differential diagnosis of them.


2020 ◽  
Vol 6 (1) ◽  
pp. 1
Author(s):  
Boushab Mohamed Boushab ◽  
Mohamed Salem Ould Ahmedou Salem ◽  
Ali Ould Mohamed Salem Boukhary ◽  
Philippe Parola ◽  
Leonardo Basco

Severe malaria in adults is not well-studied in Sahelian Africa. Clinical features and mortality associated with severe Plasmodium falciparum malaria in adult patients hospitalized in Kiffa, southern Mauritania, were analysed. Patients over 15 years old admitted for severe malaria between August 2016 and December 2019 were included in the present retrospective study. The World Health Organization (WHO) criteria were used to define severe malaria. The presenting clinical characteristics and outcome were compared. Of 4266 patients hospitalized during the study period, 573 (13.4%) had a positive rapid diagnostic test for malaria, and 99 (17.3%; mean age, 37.5 years; range 15–79 years; sex-ratio M/F, 2.1) satisfied the criteria for severe malaria. On admission, the following signs and symptoms were observed in more than one-fourth of the patients: fever (98%), impairment of consciousness (81.8%), multiple convulsions (70.7%), cardiovascular collapse (61.6%), respiratory distress (43.4%), severe anaemia ≤ 80 g/L (36.4%), haemoglobinuria (27.3%), and renal failure (25.3%). Patients were treated with parenteral quinine or artemether. Fourteen (14.1%) patients died. Multiple convulsions, respiratory distress, severe anaemia, haemoglobinuria, acute renal failure, jaundice, and abnormal bleeding occurred more frequently (p < 0.05) in deceased patients. Mortality due to severe falciparum malaria is high among adults in southern Mauritania. An adoption of the WHO-recommended first-line treatment for severe malaria, such as parenteral artesunate, is required to lower the mortality rate associated with severe malaria.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Aulia Rahmi Pawestri ◽  
Niracha Arjkongharn ◽  
Ragkit Suvannaboon ◽  
Aekkachai Tuekprakhon ◽  
Vichien Srimuninnimit ◽  
...  

AbstractAutoimmune retinopathy (AIR) is a rare immune-mediated inflammation of the retina. The autoantibodies against retinal proteins and glycolytic enzymes were reported to be involved in the pathogenesis. This retrospective cohort study assessed the antiretinal autoantibody profiles and their association with clinical outcomes of AIR patients in Thailand. We included 44 patients, 75% were females, with the overall median age of onset of 48 (17–74, IQR 40–55.5) years. Common clinical presentations were nyctalopia (65.9%), blurred vision (52.3%), constricted visual field (43.2%), and nonrecordable electroretinography (65.9%). Underlying malignancy and autoimmune diseases were found in 2 and 12 female patients, respectively. We found 41 autoantibodies, with anti-α-enolase (65.9%) showing the highest prevalence, followed by anti-CAII (43.2%), anti-aldolase (40.9%), and anti-GAPDH (36.4%). Anti-aldolase was associated with male gender (P = 0.012, OR 7.11, 95% CI 1.54–32.91). Anti-CAII showed significant association with age of onset (P = 0.025, 95% CI − 17.28 to − 1.24), while anti-α-enolase (P = 0.002, OR 4.37, 95% CI 1.83–10.37) and anti-GAPDH (P = 0.001, OR 1.87, 95% CI 1.32–2.64) were significantly associated with nonrecordable electroretinography. Association between the antibody profiles and clinical outcomes may be used to direct and adjust the treatment plans and provide insights in the pathogenesis of AIR.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1249.1-1249
Author(s):  
Y. Hayashi ◽  
K. Izumi ◽  
S. Hama ◽  
M. Higashida-Konishi ◽  
M. Ushikubo ◽  
...  

Background:Polymyositis (PM) and dermatomyositis (DM) are autoimmune inflammatory diseases characterized by proximal myositis. Dysphagia has been reported to develop in 35 to 62% of PM/DM patients and known as poor prognosis factor.Objectives:The purpose of this study is to determine the clinical characteristics of PM/DM patients who present with deglutition disorder.Methods:Consecutive patients with PM/DM who visited National Hospital Organization Tokyo Medical Center between April 2010 and January 2021 are included in this study. We compared clinical features between the patients with and without dysphagia. The diagnosis of dysphagia was based on videofluorography swallow study, and dysphagia requiring gastrostomy was defined as severe dysphagia. The clinical characteristics compared in this study were following: age of onset, levels of serum creatine kinase (CK) and lactate dehydrogenase(LDH), sense of dysphagia, manual muscle test (MMT) score, and complication of malignancy or interstitial pneumonia.Results:A total of 73 patients with PM/DM were identified. Among them, 12 patients were diagnosed with dysphagia, and 5 patients developed severe dysphagia. Patients with dysphagia had the following characteristics compared to patients without dysphagia: higher levels of serum LDH (833.7 ± 500.1 U/L vs 471.9 ± 321.0 U/L, p = 0.0088), higher levels of serum CK at initial examination (6070.3 ± 7184.8 IU/L vs 1534.7 ± 2978.8 IU/L, p = 0.0086) and more frequent sense of dysphagia (90.9% vs 10.6%, p< 0.0001), lower MMT score(3.18 ± 1.07 vs 4.31 ± 0.75, p = 0.0017). In addition to those, patients with severe dysphagia presented older age of onset (mean age 69.4 ± 12.0 vs 51.7 ± 14.8, p = 0.014), more frequent complication of malignancy (80.0% vs 14.8%, p= 0.0048) and less frequent complication of interstitial pneumonia (0.0% vs 55.5%, p= 0.023).Conclusion:These results indicate that dysphagia develops frequently in PM/DM patients with higher levels of serum LDH or CK, sense of dysphagia and low MMT score. Among them, patients with elderly onset or malignancy are at risk for sever dysphagia, and should be treated carefully.Disclosure of Interests:None declared.


2016 ◽  
Vol 43 (3) ◽  
pp. 635-661 ◽  
Author(s):  
ANNE DOROTHEE ROESCH ◽  
VASILIKI CHONDROGIANNI

AbstractStudies examining age of onset (AoO) effects in childhood bilingualism have provided mixed results as to whether early sequential bilingual children (eL2) differ from simultaneous bilingual children (2L1) and L2 children on the acquisition of morphosyntax. Differences between the three groups have been attributed to other factors such as length of exposure (LoE), language abilities, and the phenomenon to be acquired. The present study investigates whether four- to five-year-old German-speaking eL2 children differ from 2L1 children on the acquisition of wh-questions, and whether these differences can be explained by AoO, LoE, and/or knowledge of case marking. The 2L1 children outperformed the eL2 children in terms of accuracy; however, both bilingual groups exhibited similar error patterns. This suggests that 2L1 and eL2 bilingual children are sensitive to the same morphosyntactic cues, when comprehending wh-questions. Finally, children's performance on the different types of wh-questions was explained by a combination of knowledge of case marking, LoE, and AoO.


1987 ◽  
Vol 9 (1) ◽  
pp. 13-14
Author(s):  
Frederick Hecht

Medical genetics is currently enjoying a time of exploration and discovery. Huntington disease has long been of interest in adult medicine. The onset of clinical signs and symptoms is usually delayed until midadulthood. It may seem strange in this context to focus on Huntington disease, but advances in molecular genetics have brought Huntington disease into the purview of pediatrics. These advances in molecular genetics make it possible to detect Huntington disease in a preclinical stage at or even before birth. The molecular approach does not replace prior approaches to Huntington disease but is synergistic and provides a model of the new genetics. Huntington disease is synonymous with Huntington chorea. It is named after George Huntington who, like his father and grandfather before him, studied the disease in families on Long Island, NY. Huntington disease is a more common hereditary disorder than phenylketonuria, which occurs in one of about 10,000 newborns in the United States. By contrast, about one in 2,000 persons is at risk for Huntington disease. Although most cases start clinically in midadulthood, usually between 35 and 42 years of age, there is great variability in age of onset. About 3% of cases are diagnosed as juvenile Huntington disease before the age of 15 years. Late onset is well known after 50 years of age.


2018 ◽  
Vol 49 (16) ◽  
pp. 2709-2716 ◽  
Author(s):  
Ronald J. Gurrera

AbstractBackgroundAnti-NMDA receptor (NMDAr) encephalitis is the most common autoimmune encephalitis in adults. It mimics psychiatric disorders so often that most patients are initially referred to a psychiatrist, and many are misdiagnosed. Without prompt and effective treatment, patients are likely to suffer a protracted course with significant residual disability, or death. This study focuses on the frequency and chronology of salient clinical features in adults with anti-NMDAr encephalitis who are likely to be first evaluated by a psychiatrist because their presentation suggests a primary psychiatric disorder.MethodsA systematic search of PubMed and EMBASE databases identified published reports of anti-NMDAr encephalitis associated with prominent behavioral or psychiatric symptoms. After eliminating redundancies, the frequencies and relative timing of clinical features were tabulated. Signs and symptoms were assigned temporal ranks based on the timing of their first appearance relative to the first appearance of other signs and symptoms in each patient; median ranks were used to compare temporal sequencing of both individual features and major symptom domains.ResultsTwo hundred thirty unique cases (185 female) met study inclusion criteria. The most common features were seizures (60.4%), disorientation/confusion (42.6%), orofacial dyskinesias (39.1%), and mutism/staring (37.4%). Seizures, fever, and cognitive dysfunction were often the earliest features to emerge, but psychiatric features predominated and sequencing varied greatly between individuals.ConclusionsClinicians should consider anti-NMDAr encephalitis when new psychiatric symptoms are accompanied by a recent viral prodrome, seizures or unexplained fever, or when the quality of the psychiatric symptoms is unusual (e.g. non-verbal auditory hallucinations).


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