scholarly journals Management of Patients with Hereditary Angioedema During the COVID-19 Pandemic

2021 ◽  
Vol 19 (3) ◽  
pp. 166-173
Author(s):  
Eray YILDIZ ◽  
Şevket ARSLAN ◽  
Fatih ÇÖLKESEN ◽  
Filiz Sadi AYKAN ◽  
Recep EVCEN ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
Clinical Symptoms ◽  
Type I ◽  
Type Ii ◽  
Kinin System ◽  
Beneficial Effects ◽  
Phone Calls ◽  
History Of ◽  
Kallikrein Kinin System

Objective: The aim of this study was to determine the clinical course and treatment outcomes of patients with hereditary angioedema (HAE) after infection with coronavirus disease 2019 (COVID-19). Materials and Methods: Thirty-nine patients with HAE were included in this study. These patients were regularly followed up over phone calls since the first COVID-19 case was seen in our country. Patients were asked to visit the hospital if there was a history of contact with a confirmed COVID-19 patient or if the patient developed clinical symptoms of COVID-19.Results: There were 21 (54%) patients with type I HAE, and 18 (46%) with type II HAE. All patients received treatment for angioedema attacks (C1-inhibitor [C1-INH], icatibant), and seven (20%) received long-term prophylaxis (danazol). Treatment for attacks was continued for all patients during the pandemic. Patients taking danazol were switched to long-term prophylaxis using the C1-INH concentrate. Eleven (28%) patients with HAE developed COVID-19 during this study. Only one patient had severe COVID-19. Six patients (54.5%) were diagnosed with type II HAE, and five (45.5%) were diagnosed with type I HAE. The most common COVID-19 symptoms were fever (7/11; 64%) and myalgia (6/11; 55%). Mild angioedema attacks were experienced by 36% (4/11) of the HAE patients diagnosed with COVID-19. Icatibant was used in all patients.Conclusion: Agents used for HAE block the kallikrein-kinin system and may be useful in the treatment of COVID-19. Considering their beneficial effects on COVID-19, it is recommended that HAE patients should continue the use of agents blocking the kallikrein-kinin system. Keywords: COVID-19, hereditary angioedema, kallikrein-kinin system, bradykinin, C1-INH

Hereditary angioedema: Pathophysiology (HAE type I, HAE type II, and HAE nC1-INH)

2020 ◽  
Vol 41 (6) ◽  
pp. S14-S17 ◽  
Author(s):  
H. James Wedner
Keyword(s):  
Serine Protease ◽  
Hereditary Angioedema ◽  
Normal Activity ◽  
Genetic Alterations ◽  
Type I ◽  
Factor Xii ◽  
Kinin System ◽  
Major Pathway ◽  
Major Mechanism ◽  
Kallikrein Kinin System

The pathophysiology of hereditary angioedema (HAE) in virtually all cases is the result of the uncontrolled production of the vasoactive peptide bradykinin. C1 inhibitor (C1-INH) is a serine protease inhibitor, which, under normal circumstances, is the regulator of critical enzymes that are active in the cascades that result in bradykinin generation. In the classic forms of HAE, C1-INH is not produced in sufficient quantities (<40% of normal) or the function is <40% of normal activity. The major pathway for the production of bradykinin is the “contact system,” also known as the kallikrein-kinin system. This system begins with the activation of factor XII (FXII) to FXIIa, by a variety of physiologic and pathologic stimuli. FXIIa is a serine protease that binds to surfaces and cleaves prekallikrein to the active serine protease kallikrein. Kallikrein then cleaves high-molecular-weight kininogen to release the nonapeptide bradykinin. Bradykinin binds to the bradykinin β2 receptor, which increases vascular permeability and allows the flow of fluids into the extracellular space and results in angioedema. The two major enzymes generated in this cascade FXIIa and kallikrein are inhibited by C1-INH, which is the major regulator of this cascade. Failure to adequately control the production of bradykinin is thus the major mechanism for HAE. Several other types of HAE in which C1-INH is not decreased (HAE nlC1-INH) have been described. The alterations in FXII and plasminogen (also a serine protease inhibited by C1-INH) like with classic HAE are the result of dysregulation of bradykinin generation. Only genetic alterations in angiopoietin-1 may not be related to bradykinin generation, rather related to the control of the effect of bradykinin on the vascular endothelium.

scholarly journals To Compare Efficacy of Nishkatakadi Kwath with Metformin in Madhumeha With Special Refrence to Diabetes Mellitus ii

2020 ◽  
Vol 3 (1) ◽  
pp. 01-16
Author(s):  
Bhairav B. Tawshikar ◽  
Shrikant G. Deshmukh ◽  
Yashashree B. Kulkarni
Keyword(s):  
Diabetes Mellitus ◽  
Clinical Symptoms ◽  
The Body ◽  
Type I ◽  
Type Ii ◽  
Diabetes Mellitus Type Ii ◽  
Ancient Times ◽  
Almost All ◽  
And Control

Madhumeha is a disease known since ancient times to the mankind, its upsurge is quiet alarming. On the basis of its symptomatology Madhumeha can be correlated to the features of Diabetes mellitus. Diabetes mellitus is a metabolic disorder of carbohydrate, fat, & protein characterized by hyperglycemia with or without glycosuria. It is associated with long-term potentially catastrophic effects on almost all systems of the body. Ayurveda can provide better management for Madhumeha without hazardous side effects. In Ayurveda, Madhumeha has been described as one among one of the 20 types of Prameha and is a sub-type of Vatika Prameha. In the former type the patients are usually asthenic can be correlated with Type I DM and in the latter type patients are obese and can be equated with Type II DM. Nishakatakadi Kwath has been mentioned in Sahasrayoga under Kashaya Prakaran for Prameha Chikitsa, Nishakatakadi Kwath is being used widely for the treatment of Madhumeha in Kerala. That is why Nishakatakadi Kwath has been selected for the present study. In this study to study various clinical symptoms of madhumeha with special reference to Diabetes Mellitus II. To compare efficacy of Nishkatakadi Kwath with Metformin inthe management of Madhumeha, To observe any possible adverse effect of Nishkatakadi kwath if reported during the study. In the present study Trial and Control groups comprising 30 patients each of Madhumeha (Diabetes Mellitus type II) were treated with Nishkatakadi kwath with tab metformin and Tab Metformin alone respectively. This study shows that both the drugs NISHKATAKADI KWATH with metformin are more effective than METFORMIN in Madhumeha to reduce symptoms and patients score.

scholarly journals Successful use of lanadelumab in an elderly patient with type II hereditary angioedema

2021 ◽  
Author(s):  
Tasha S. Hellu ◽  
Samuel L. Weiss ◽  
Derek M. Smith
Keyword(s):  
Hereditary Angioedema ◽  
Genetic Disorder ◽  
Poor Quality ◽  
Type I ◽  
Type Ii ◽  
C1 Esterase Inhibitor ◽  
Submucosal Edema ◽  
Esterase Inhibitor

Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurring episodes of subcutaneous and/or submucosal edema without urticaria due to an excess of bradykinin (1, 2). HAE is classified into 2 main types (1). Type I HAE is caused by deficiency of C1 esterase inhibitor, accounting for 85% of cases (1). Type II HAE occurs in only 15% of cases and is marked by normal to elevated levels of C1 esterase inhibitor but with a reduction in activity (1). An angioedema attack can range in severity depending on the location and degree of edema (2). Furthermore, patients with HAE are often diagnosed with anxiety and depression secondary to their poor quality of life (3). Thus, long-term prophylaxis of attacks can be crucial to reduce the physical and psychological implications. For long-term prophylaxis, lanadelumab, a subcutaneously delivered monoclonal antibody inhibitor of plasma kallikrein, has been proven to decrease the frequency of HAE attacks without significant side effects (4). However, data is limited, specifically regarding patients with type II HAE and patients >/= 65 years (4).

scholarly journals Treatment of Hereditary Angioedema

2021 ◽  
Vol 31 (1) ◽  
pp. 1-16
Author(s):  
T Caballero
Keyword(s):  
Hereditary Angioedema ◽  
New Drugs ◽  
Current Therapy ◽  
Factor Xii ◽  
Short Term ◽  
Kinin System ◽  
C1 Esterase Inhibitor ◽  
Kallikrein Kinin System ◽  
Esterase Inhibitor

Hereditary angioedema due to C1-esterase inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease. In the last decade, new drugs and new indications for old drugs have played a role in the management of C1-INH-HAE. This review examines current therapy for C1-INH-HAE and provides a brief summary of drugs that are under development. Increased knowledge of the pathophysiology of C1-INH-HAE has been crucial for advances in the field, with inhibition of the kallikrein-kinin system (plasma kallikrein, activated factor XII) as a key area in the discovery of new drugs, some of which are already marketed for treatment of C1-INH-HAE. Pharmacological treatment is based on 3 pillars: treatment of acute angioedema attacks (on-demand treatment), short-term (preprocedure) prophylaxis, and long-term prophylaxis. The 4 drugs that are currently available for the treatment of acute angioedema attacks (purified plasma-derived human C1 esterase inhibitor concentrate, icatibant acetate, ecallantide, recombinant human C1 esterase inhibitor) are all authorized for self-administration, except ecallantide. Purified plasma-derived human C1 esterase inhibitor concentrate is the treatment of choice for short-term prophylaxis. Tranexamic acid, danazol, intravenous and subcutaneous nanofiltered purified plasma-derived human C1 esterase inhibitor concentrate, and lanadelumab can be used for long-term prophylaxis. New drugs are being investigated, mainly as long-term prophylaxis, and are aimed at blocking the kallikrein-kinin system by means of antiprekallikrein, antikallikrein, and anti–activated FXII action.

scholarly journals Minimal Invasive Surgery for Gastroesophageal Reflux Disease and Hiatus Hernia—Our Experience: A Case Series with Review of Literature

2021 ◽  
Author(s):  
Rafique Umer Harvitkar ◽  
Abhijit Joshi
Keyword(s):  
Retrospective Study ◽  
Gastroesophageal Reflux Disease ◽  
Gastroesophageal Reflux ◽  
Operating Time ◽  
Type I ◽  
Type Ii ◽  
The Mean ◽  
Gerd Symptoms

Abstract Introduction Laparoscopic fundoplication (LF) has almost completely replaced the open procedure performed for gastroesophageal reflux disease (GERD) and hiatus hernia (HH). Several studies have suggested that long-term results with surgery for GERD are better than a medical line of management. In this retrospective study, we outline our experience with LF over 10 years. Also, we analyze the factors that would help us in better patient selection, thereby positively affecting the outcomes of surgery. Patients and Methods In this retrospective study, we identified 27 patients (14 females and 13 males) operated upon by a single surgeon from 2010 to 2020 at our institution. Out of these, 25 patients (12 females and 13 males) had GERD with type I HH and 2 (both females) had type II HH without GERD. The age range was 24 to 75 years. All patients had undergone oesophago-gastro-duodenoscopy (OGD scopy). A total of 25 patients had various degrees of esophagitis. Two patients had no esophagitis. These patients were analyzed for age, sex, symptoms, preoperative evaluation, exact procedure performed (Nissen’s vs. Toupet’s vs. cruroplasty + gastropexy), morbidity/mortality, and functional outcomes. They were also reviewed to examine the length of stay, length of procedure, complications, and recurrent symptoms on follow-up. Symptoms were assessed objectively with a score for six classical GERD symptoms preoperatively and on follow-up at 1-, 4- and 6-weeks postsurgery. Further evaluation was performed after 6 months and then annually for 2 years. Results 14 females (53%) and 13 males (48%) with a diagnosis of GERD (with type I HH) and type II HH were operated upon. The mean age was 46 years (24–75 years) and the mean body mass index (BMI) was 27 (18–32). The range of duration of the preoperative symptoms was 6 months to 2 years. The average operating time dropped from 130 minutes for the first 12 cases to 90 minutes for the last 15 cases. The mean hospital stay was 3 days (range: 2–4 days). In the immediate postoperative period, 72% (n = 18) of the patients reported improvement in the GERD symptoms, while 2 (8%) patients described heartburn (grade I, mild, daily) and 1 (4%) patient described bloating (grade I, daily). A total of 5 patients (20%) reported mild dysphagia to solids in the first 2 postoperative weeks. These symptoms settled down after 2 to 5 weeks of postoperative proton-pump inhibitor (PPI) therapy and by adjusting consistency of oral feeds. There was no conversion to open, and we observed no perioperative mortality. There were no patients who underwent redo surgeries in the series. Conclusion LF is a safe and highly effective procedure for a patient with symptoms of GERD, and it gives long-term relief from the symptoms. Stringent selection criteria are necessary to optimize the results of surgery. Experience is associated with a significant reduction of operating time.

The Natural History of Cerebral Dural Arteriovenous Fistulae

Neurosurgery ◽  
2012 ◽  
Vol 71 (3) ◽  
pp. 594-603 ◽  
Author(s):  
Bradley A. Gross ◽  
Rose Du
Keyword(s):  
Natural History ◽  
Significant Risk ◽  
Venous Drainage ◽  
Type I ◽  
Type Ii ◽  
Arteriovenous Fistulae ◽  
Type Iii ◽  
History Of ◽  
Venous Ectasia ◽  
Cortical Venous Drainage

Abstract BACKGROUND: Hemorrhage from cerebral dural arteriovenous fistulae (dAVF) is a considerable source of neurological morbidity and even mortality. OBJECTIVE: To evaluate the natural history of cerebral dAVF. METHODS: We reviewed our own cohort of 70 dAVF and incorporated results from the literature, synthesizing pooled hemorrhage rates and evaluating risk factors for 395 dAVF in 6 studies. RESULTS: No hemorrhages occurred during 409 lesion-years of follow-up of Borden type I dAVF; however, cortical venous drainage developed in 1.4%. Like type I dAVF, type II dAVF demonstrated a female predilection and were most commonly transverse-sigmoid or cavernous. Eighteen percent of type II dAVF presented with hemorrhage (95% confidence interval [CI]: 8%-36%), and the annual hemorrhage rate was 6% (95% CI: 0.1%-19%). Borden type III dAVF demonstrated a male predilection and were most commonly tentorial or petrosal. Thirty-four percent presented with hemorrhage (95% CI: 0.4%-49%), with an annual hemorrhage rate of 10% (95% CI: 4%-20%), increasing to 21% for those with venous ectasia (95% CI: 4%-66%). The hemorrhage rate decreased to 2% for asymptomatic or minimally symptomatic type II or III dAVF (95% CI: 0.2%-8%), and increased to 10% for those presenting with nonhemorrhagic neurological deficits (95% CI: 0.9%-41%) and to 46% for those presenting with hemorrhage (95% CI: 11%-130%). CONCLUSION: Venous ectasia is a significant risk factor for hemorrhage among dAVF with cortical venous drainage. In addition, those with hemorrhagic presentation, even compared with nonhemorrhagic neurological deficit presentation, as well as Borden type III dAVF compared with type II dAVF demonstrated a trend toward greater hemorrhage rates.

A New Classification for Pathologies of Spinal Meninges, Part 1: Dural Cysts, Dissections, and Ectasias

Neurosurgery ◽  
2017 ◽  
Vol 81 (1) ◽  
pp. 29-44 ◽  
Author(s):  
Jörg Klekamp
Keyword(s):  
Structural Changes ◽  
Long Term Results ◽  
Type I ◽  
Type Ii ◽  
New Classification ◽  
Type Iii ◽  
Dural Sac ◽  
Spinal Cord Herniation ◽  
Dural Defects

Abstract BACKGROUND: The clinical significance of pathologies of the spinal dura is often unclear and their management controversial. OBJECTIVE: To classify spinal dural pathologies analogous to vascular aneurysms, present their symptoms and surgical results. METHODS: Among 1519 patients with spinal space-occupying lesions, 66 patients demonstrated dural pathologies. Neuroradiological and surgical features were reviewed and clinical data analyzed. RESULTS: Saccular dural diverticula (type I, n = 28) caused by defects of both dural layers, dissections between dural layers (type II, n = 29) due to defects of the inner layer, and dural ectasias (type III, n = 9) related to structural changes of the dura were distinguished. For all types, symptoms consisted of local pain followed by signs of radiculopathy or myelopathy, while one patient with dural ectasia presented a low-pressure syndrome and 10 patients with dural dissections additional spinal cord herniation. Type I and type II pathologies required occlusion of their dural defects via extradural (type I) or intradural (type II) approaches. For type III pathologies of the dural sac no surgery was recommended. Favorable results were obtained in all 14 patients with type I and 13 of 15 patients with type II pathologies undergoing surgery. CONCLUSION: The majority of dural pathologies involving root sleeves remain asymptomatic, while those of the dural sac commonly lead to pain and neurological symptoms. Type I and type II pathologies were treated with good long-term results occluding their dural defects, while ectasias of the dural sac (type III) were managed conservatively.

scholarly journals Verbal threat learning does not spare loved ones

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cristina Morato ◽  
Pedro Guerra ◽  
Florian Bublatzky
Keyword(s):  
Fear Learning ◽  
Pain Experience ◽  
Trauma Research ◽  
Learning History ◽  
Beneficial Effects ◽  
Electric Shocks ◽  
History Of ◽  
Threat Cues ◽  
Verbal Threat

AbstractSignificant others provide individuals with a sense of safety and security. However, the mechanisms that underlie attachment-induced safety are hardly understood. Recent research has shown beneficial effects when viewing pictures of the romantic partner, leading to reduced pain experience and defensive responding. Building upon this, we examined the inhibitory capacity of loved face pictures on fear learning in an instructed threat paradigm. Pictures of loved familiar or unknown individuals served as signals for either threat of electric shocks or safety, while a broad set of psychophysiological measures was recorded. We assumed that a long-term learning history of beneficial relations interferes with social threat learning. Nevertheless, results yielded a typical pattern of physiological defense activation towards threat cues, regardless of whether threat was signaled by an unknown or a loved face. These findings call into question the notion that pictures of loved individuals are shielded against becoming threat cues, with implications for attachment and trauma research.

scholarly journals Snow Avalanches and Acoustic Emissions

1983 ◽  
Vol 4 ◽  
pp. 271-276 ◽  
Author(s):  
R. A. Sommerfeld ◽  
H. Gubler
Keyword(s):  
Acoustic Emissions ◽  
Limited Range ◽  
Slope Instability ◽  
Type I ◽  
Type Ii ◽  
Noise Levels ◽  
Active Layers ◽  
Differential Movement ◽  
Emission Activity

Analyses of several years of data show that acoustic emission activity is greater from unstable snowpacks than from stable snowpacks. Two types of signals have been identified: type I spikes and type II long-term elevation of the noise level. It is thought that the type I signals originate from macroscopic cracks. The type II signals may originate from differential movement on shearing surfaces, but this is less certain. Increased noise levels of both types correlate well with slope instability, when the slope stability is known. In some climates the limited range of signal detection might be a significant problem. A foam-mounted geophone set into the snow near active layers appears to be the best sensor available at present.

scholarly journals Outcomes of long term treatments of type I hereditary angioedema in a Turkish family

2017 ◽  
Vol 92 (5) ◽  
pp. 655-660 ◽  
Author(s):  
Gulsen Akoglu ◽  
Belgin Kesim ◽  
Gokhan Yildiz ◽  
Ahmet Metin
Keyword(s):  
Hereditary Angioedema ◽  
Type I ◽  
Turkish Family
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