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2.1
Ayerza Syndrome
Ayerza Disease

Ayerza syndrome is named after Abel Ayerza and encompasses the clinical features of pulmonary hypertension. It is a progressive disease with mixed etiology, characterized by increased resistance in the pulmonary blood vessels.

Presentation

Ayerza syndrome (AS) describes the signs of pulmonary (arterial) hypertension [1]. It is mostly diagnosed in adults and has a higher prevalence in females than males. Defined by a mean pulmonary artery pressure of more than or equal to 25 mmHg and a capillary wedge pressure of over 15 mmHg [2], this medical entity can arise from a number of pathologies that may be cardiac or pulmonary in origin, or related to other systemic diseases, drugs, and toxins. Conditions that precipitate AS include chronic obstructive pulmonary disease (COPD), interstitial lung disease and fibrosis, left heart failure, autoimmune diseases and human immunodeficiency virus (HIV) infection. A significant proportion of cases are idiopathic, with no apparent cause, of which 70% have a mutation in the bone morphogenetic protein receptor type 2 (BMPR2) gene [3] [4] and few have in other genes, for example, activin receptor-like kinase type 1 (ACVRL1). Up to 40% of those with no family history of pulmonary hypertension (PH) also carry the gene mutation [5]. PH is progressive and may lead to death, especially if left untreated. Patients typically experience an insidious onset of symptoms that initially occur during exercise, and worsen as the ailment progresses. Consequently, this causes a delay in presentation and diagnosis. Early signs include dyspnea, syncope, dizziness, and cough [6]. Late manifestations are usually due to the underlying or resultant cardiac dysfunction and venous congestion. Among those reported are angina, hyperactive precordium, paroxysmal nocturnal dyspnea, hemoptysis, raised jugular venous pressure, abnormal heart sounds, and edema. Less common complaints include signs of liver failure such as ascites, and digital clubbing, which appear in an advanced stage.

Entire Body System

  • Fatigue

    855 MILITARY SYMPOSIUM FATIGUE IN AIRCRAFT PILOTS* Ross A. [nejm.org]

    NEWS.com.au | Top Stories In particular, fatigue does not improve, possibly due to co-existing medical problems, such as obstructive sleep apnea or obesity hypoventilation, according to Dr, Greening. [wordnik.com]

    724.8 Fallot's 745.2 falx (see also Hemorrhage, brain) 431 familial eczema-thrombocytopenia 279.12 Fanconi's (anemia) (congenital pancytopenia) 284.09 Fanconi (-de Toni) (-Debré) (cystinosis) 270.0 Farber (-Uzman) (disseminated lipogranulomatosis) 272.8 fatigue [theodora.com]

  • Plethora

    Diagnosis and investigation of AS involve a plethora of invasive and non-invasive techniques. These are done in order to gauge the presence of an underlying pathology as well as to plan treatment and map prognosis based on the severity of symptoms. [symptoma.com]

Respiratoric

  • Dyspnea

    Ayerza's disease Ayer·za's disease ə-'yər-zəz- n a complex of symptoms marked esp. by cyanosis, dyspnea, polycythemia, and sclerosis of the pulmonary artery Ayerza ä-'yer-sä Abel (1861-1918) Argentinean physician. [medicine.academic.ru]

    Among those reported are angina, hyperactive precordium, paroxysmal nocturnal dyspnea, hemoptysis, raised jugular venous pressure, abnormal heart sounds, and edema. [symptoma.com]

    Abstract In 1919 Aldred Scott Warthin 1 published a clinical report and autopsy protocol entitled "A Case of Ayerza's Disease: Chronic Cyanosis, Dyspnea, and Erythremia, Associated with Syphilitic Arteriosclerosis of the Pulmonary Artery." [annals.org]

    P., a stevedore, white, aged 48, was first seen in October, 1924, complaining of a cough productive of a small amount of mucus, a feeling of constriction across the upper part of the chest and dyspnea, which had developed over a period of five years. [jamanetwork.com]

    […] than 200 scientific publications, Abel Ayerza was the first Argentinian name to enter world medicine by dint of “Ayerza’s disease” Ayerza’s disease[edit] A syndrome of signs and symptoms which include sclerosis of the pulmonary artery, polycythemia, dyspnea [en.wikipedia.org]

  • Clubbed Finger

    Dyspnoea was severe at rest and there was central cyanosis, clubbed fingers and tachypnea. In the respiratory examination, wet crackles and wheezing were abundant in both lungs. [err.ersjournals.com]

Cardiovascular

  • Cyanosis

    In 1901 he studied a case in which cyanosis and sclerosis of the pulmonary vessels appeared during autopsy. He reported his findings only in a lecture. A student, Francisco C. [medicine.academic.ru]

    A·yer·za syn·drome ( ah-yār'shah ), sclerosis of the pulmonary arteries in chronic cor pulmonale; associated with severe cyanosis, it is a condition resembling polycythemia vera but resulting from primary pulmonary arteriosclerosis or primary pulmonary [medical-dictionary.thefreedictionary.com]

    In order to differentiate it from other diseases Dr Ayerza called it “cardíaco negro” (black cardiac) due to the extreme degree of cyanosis observed in these patients. [err.ersjournals.com]

Liver, Gall & Pancreas

  • Hepatomegaly

    In the cardiovascular examination there was jugular venous distension, hepatomegaly, hepatojugular reflux, ascites and oedema of lower limbs. [err.ersjournals.com]

    […] artery 093.89 heart 093.89 block 093.89 decompensation 093.89 disease 093.89 failure 093.89 valve (see also Syphilis, endocarditis) 093.20 hemianesthesia 094.89 hemianopsia 095.8 hemiparesis 094.89 hemiplegia 094.89 hepatic artery 093.89 hepatitis 095.3 hepatomegaly [theodora.com]

Musculoskeletal

  • Metatarsalgia

    733.3 Morgagni (-Stewart-Morel) (hyperostosis frontalis interna) 733.3 Morgagni-Adams-Stokes (syncope with heart block) 426.9 Morquio (-Brailsford) (-Ullrich) (mucopolysaccharidosis IV) 277.5 Morris (testicular feminization) 259.5 Morton's (foot) (metatarsalgia [theodora.com]

Skin

  • Flushing

    I hurriedly flushed them all down the toilet, before I heard the voice of the person who gave me this curse, the only love of my life. "Hey, you ok Gray?" ...................................................................... [wattpad.com]

    Moreover, if we consider pharmacoeconomic implications, patients that take tadalafil would pay two-thirds of the cost of sildenafil therapy. [77] However, there are some adverse effects of this drug such as headache, diarrhea, nausea, back pain, dyspepsia, flushing [wikivisually.com]

    […] hemorrhagic pancreatitis) 577.0 Fitz-Hugh and Curtis 098.86 due to Chlamydia trachomatis 099.56 Neisseria gonorrhoeae (gonococcal peritonitis) 098.86 Flajani (-Basedow) (exophthalmic goiter) 242.0 floppy infant 781.99 iris 364.81 valve (mitral) 424.0 flush [theodora.com]

Neurologic

  • Somnolence

    […] brain) 013.2 ulcer, bladder 596.8 Somatization reaction, somatic reaction (see also Disorder, psychosomatic) 306.9 disorder 300.81 Somatoform disorder 300.82 atypical 300.82 severe 300.81 undifferentiated 300.82 Somnambulism 307.46 hysterical 300.13 Somnolence [theodora.com]

Workup

Diagnosis and investigation of AS involve a plethora of invasive and non-invasive techniques. These are done in order to gauge the presence of an underlying pathology as well as to plan treatment and map prognosis based on the severity of symptoms [7]. The diagnostic test for AS is right heart catheterization, as it allows direct measurement of the pulmonary arterial pressure (which will be above 25 mmHg) and thus confirms the condition. Pulmonary capillary wedge pressure may be within the normal range.

Laboratory examination includes full blood count, assessment of liver, thyroid and renal function, immunological assays, arterial blood gas analysis (ABGA), and human immunodeficiency virus (HIV) testing [8]. Cardiac biomarkers such as brain natriuretic peptide (BNP) may be raised, indicating heart abnormalities [9].
Imaging may involve:

  • Electrocardiogram (ECG): Shows signs of ventricular hypertrophy.
  • Echocardiogram.
  • Spirometry, ventilation-perfusion (V/Q) scanning, and pulmonary angiography to measure lung function.
  • Administration of vasodilators to provoke a dilation of the pulmonary vessels, such as nitric oxide and adenosine, as well as other tests for staging and treatment planning [10].
  • Chest X-ray: Shows increased lung perfusion as well as enlarged pulmonary vessels.
  • Computerized tomography: Useful in chronic thromboembolic pulmonary arterial hypertension.
  • Magnetic resonance imaging (MRI).

Serum

  • Hypercapnia

    Blood tests reveal a hyperglobulia and a polyglobulia, an anoxemia and often a hypercapnia (see). On an ECG — signs of the expressed hypertrophy and an overload of the right departments of heart. [bigmed.info]

Microbiology

  • Streptococcus Pneumoniae

    […] callinarum) (choleraesuis) (enteritidis) (suipestifer) 003.1 Serratia 038.44 Shigella (see also Dysentery, bacillary) 004.9 specified organism NEC 038.8 staphylococcal 038.10 aureus 038.11 specified organism NEC 038.19 streptococcal (anaerobic) 038.0 Streptococcus [theodora.com]

Treatment

Avoid potential pitfalls in diagnosis and treatment by referring to "Side Effects and Complications" sections in each chapter. [books.google.com]

These are done in order to gauge the presence of an underlying pathology as well as to plan treatment and map prognosis based on the severity of symptoms. [symptoma.com]

Type your tag names separated by a space and hit enter Abstract The lecture deals with detailed information about the etiology, pathogenesis and clinical manifestations of primary (idiopathic) pulmonaiy hypertension, intensive treatment and anesthesia [unboundmedicine.com]

Treatment: Ayerza syndrome: Although treatment of this disease consists primarily of that necessary for the underlying disease, several medications and oxygen are used in different clinical settings. [medigoo.com]

Prognosis

Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Pulmonary hypertension from other Diseases Epidemiology & Demographics Risk Factors Screening Natural History, Complications and Prognosis [wikidoc.org]

These are done in order to gauge the presence of an underlying pathology as well as to plan treatment and map prognosis based on the severity of symptoms. [symptoma.com]

Prognosis Prognosis depends on the etiology of the lesion. References 1. Boisvert CJ, MD, Tran NA, Al Othman B, MD (2016). Anisocoria. American Academy of Ophthalmology, EyeWiki. Retrieved March 3, 2019, from: https://eyewiki.aao.org/Anisocoria 2. [eyewiki.aao.org]

The prognosis worsens when atrial flutter is diagnosed in older children, and the probability of concomitant associated heart disease increases. [scielo.org.mx]

Genetics can contribute to the prognosis of Brugada syndrome: a pilot model for risk stratification. Eur J Human Genetics 2013;21:911–7. [ecrjournal.com]

Etiology

Type your tag names separated by a space and hit enter Abstract The lecture deals with detailed information about the etiology, pathogenesis and clinical manifestations of primary (idiopathic) pulmonaiy hypertension, intensive treatment and anesthesia [unboundmedicine.com]

Etiology The etiology of Parkinson sign is tied to compression or inflammation of the posterior cavernous sinus. [eyewiki.aao.org]

Clinical Laboratory Techniques, Cough/diagnosis, Cough/etiology, Diagnosis, Differential, Fever/diagnosis, Fever/etiology, Humans, Male, Pandemics, Radiography, Thoracic/methods 11. [pesquisa.bvsalud.org]

A structured diagnostic approach enables you to determine underlying etiology, then guides you to appropriate pharmacologic and non-pharmacologic therapeutic options, based upon their potential risks and benefits. [books.google.com]

Reversible pulmonary hypertension in POEMS syndrome--another etiology of triggered pulmonary vasculopathy? Paciocco G, Bossone E, Erba H, Rubenfire M. Paciocco G, et al. Can J Cardiol. 2000 Aug;16(8):1007-12. Can J Cardiol. 2000. [pubmed.ncbi.nlm.nih.gov]

Epidemiology

Тема - темы Antipyretics, Brugada Syndrome, COVID-19 Vaccines, COVID-19, Defibrillators, Implantable, Adult, Antipyretics/adverse effects, Brugada Syndrome/diagnosis, Brugada Syndrome/epidemiology, Brugada Syndrome/therapy, COVID-19/epidemiology, COVID [pesquisa.bvsalud.org]

[…] navigation Jump to search For patient information click here Pulmonary Hypertension Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Pulmonary hypertension from other Diseases Epidemiology [wikidoc.org]

| N Engl J Med 1988; 318:549-557 ALTHOUGH earlier studies1 2 3 suggested that dietary fish had some properties that could potentially prevent coronary artery disease, it was not until the epidemiologic studies of Bang et al.4 and Dyerberg et al.5 in [nejm.org]

Pregnancy is sometimes described as contraindicated in these women. [87] [88] [89] Epidemiology [ edit ] The epidemiology of IPAH is about 125–150 deaths per year in the U.S., and worldwide the incidence is similar to the U.S. at 4 cases per million. [wikivisually.com]

Pathophysiology

Focusing on clinical decision making, this new reference provides current, comprehensive coverage of the pathophysiology, diagnosis, and treatment of pulmonary hypertension and vasculitis, as well as other related health issues. [books.google.com]

Jump to navigation Jump to search For patient information click here Pulmonary Hypertension Microchapters Home Patient Information Overview Historical Perspective Classification Pathophysiology Causes Differentiating Pulmonary hypertension from other [wikidoc.org]

Aerza-Arrilaga syndrome: pathogenesis and pathophysiology. Bulletin of Emergency Medicine. 2016; 9(1):107-11. (In Russ). Tsareva NA. Updated classification and diagnosis of pulmonary hypertension. Consilium Medicum. 2017;19(3):66-71. (In Russ). [journals.eco-vector.com]

Year: 2019 Chronic thromboembolic pulmonary hypertension associated with Klippel Trenaunay syndrome: A retrospective series of 5 patients Source: Annual Congress 2011 - Clinical features of pulmonary thromboembolism Year: 2011 Pulmonary hypertension: pathophysiology [ers-education.org]

[…] img=PMC3223717_kjo-25-459-g005&req=4 Pathophysiology Parkinson sign results when both the sympathetic chain and CN VI are disrupted simultaneously. These paths intersect in the cavernous sinus, which gives this sign great localizing value. [eyewiki.aao.org]

Prevention

Secondary Prevention Cost-Effectiveness of Therapy Future or Investigational Therapies Case Studies Case #1 Pulmonary hypertension On the Web Most recent articles Most cited articles Review articles CME Programs Powerpoint slides Google Images American [wikidoc.org]

Статья в английский | MEDLINE | ID: covidwho-1968146 Тема - темы Brugada Syndrome, COVID-19 Vaccines, COVID-19, Brugada Syndrome/diagnosis, Brugada Syndrome/epidemiology, Brugada Syndrome/therapy, COVID-19/prevention & control, COVID-19 Vaccines/adverse [pesquisa.bvsalud.org]

The Genetics Home Reference of the National Institutes of Health indicates that BMPR2 mutation may prevent cell death or increase the rate of cell division. This causes an overgrowth of cells in the small bronchial arteries. [brighthub.com]

How do atrial pacing algorithms prevent atrial arrhythmias? Europace 2004;6:351–362. 28. Israel CW, Hohnloser SH. Pacing to prevent atrial fibrillation. J Cardiovasc Electrophysiol 2003;14:S20–S26. 29. [cardiologieladomiciliu.ro]

The BMPR2 mutation may prevent cell death or increase the rate of cell division. This causes an overgrowth of cells in the small bronchial arteries. This narrows the small arteries in the lungs, forcing the pulmonary artery to work harder. [medigoo.com]

References

  1. Mazzei JA, Mazzei ME. A tribute: Abel Ayerza and pulmonary hypertension. Eur Respir Rev. 2011;20(122):220–221.
  2. Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126(1 Suppl):35S–62S.
  3. Deng Z, Morse JH, Slager SL, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet. 2000;67(3):737–744.
  4. Cogan JD, Pauciulo MW, Batchman AP, et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174(5):590–598.
  5. Machado RD, Aldred MA, James V, et al. Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006;27(2):121–132.
  6. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336(2):111–117.
  7. Barst RJ, McGoon M, Torbicki A, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):40S–47S.
  8. Raymond R, Hinderliter A, Willis P, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol. 2002;39(7):1214–1219.
  9. Torbicki A, Kurzyna M, Kuca P, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. 2003;108(7):844–848.
  10. Enright PL, McBurnie MA, Bittner V, et al. The 6-minute walk test: A quick measure of functional status in elderly adults. Chest. 2003;123(2):387-398.
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