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Pulmonary Hypertension

PH

Pulmonary hypertension (PH) is a rise of blood pressure and resistance in the pulmonary vasculature.

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Presentation

The diagnosis of the disorder is often delayed due to nonspecific symptoms and signs, frequently reflecting the underlying etiology. Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain [2] [3] [4]. Uncommon symptoms include cough, hemoptysis and hoarseness.

Fatigue
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • However, the dyspnea and fatigue subsequently got worsened. Tracing back his family history, together with the electromyography, nerve conduction studies, and the result of muscle biopsy, mitochondrial disease was confirmed.[ncbi.nlm.nih.gov]
  • Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema.[orpha.net]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 31, 2019[patientslikeme.com]
Weakness
  • Also, because your heart is working harder than normal, your right ventricle becomes strained and weak. Your heart may become so weak that it can't pump enough blood to your lungs. This causes heart failure .[web.archive.org]
  • Symptoms Difficulty in breathing is commonest symptom followed by weakness and fainting. Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice.[symptoma.com]
  • Other symptoms include: Ankle and leg swelling Bluish color of the lips or skin ( cyanosis ) Chest pain or pressure, usually in the front of the chest Dizziness or fainting spells Fatigue Increased abdomen size Weakness People with pulmonary hypertension[nlm.nih.gov]
  • High blood pressure in your lung vessels causes your right ventricle and right atrium (heart chambers) to become enlarged and weak and to not pump as well.[wexnermedical.osu.edu]
Raynaud Phenomenon
  • […] doi: 10.1097/BOR.0b013e32834ba6a7 Raynaud phenomenon, scleroderma , overlap syndromes and other fi brosing syndromes: Edited by John Varga Abstract Author Information Authors Article Metrics Metrics The review provides an update of the epidemiology, pathogenesis[journals.lww.com]
  • More rarely, clubbing of digits and Raynaud phenomenon may be observed. Hemoptysis has also been reported. Etiology IPAH is caused by vascular remodeling of small pulmonary arteries due to unknown causes.[orpha.net]
  • phenomenon (mostly in females) have been observed.[orpha.net]
  • More rarely, abdominal distension, clubbing of digits and Raynaud phenomenon (predominantly in females) are observed. Hemoptysis has also been reported.[orpha.net]
  • Pulmonary Hypertension Heart Breathlessness Chest pain Dizziness Fainting Loss of energy Swelling of the arms, legs, ankles or abdomen Dry cough Raynaud’s phenomenon Heart and lung disorders Family history HIV infection Connective tissue disorders Congenital[nm.org]
Gaucher Disease
  • disease, thyroid disorders Others: Tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental pulmonary hypertension Definition Pulmonary hypertension is a rise of blood pressure and resistance in the lung vessels.[symptoma.com]
  • Metabolic disorders: glycogen storage disease, Gaucher's disease, thyroid disorders. Others: tumour obstruction, fibrosing mediastinitis, chronic kidney disease, segmental pulmonary hypertension. Epidemiology [ 2 ] Idiopathic PAH is rare.[patient.info]
  • disease Thyroid disorders Other disorders: Fibrosing mediastinitis Tumor, causing obstruction Chronic kidney disease Segmental pulmonary hypertension Adapted from the Fifth World Symposium on PAH, Nice, 2013; Simonneau G, Gatzoulis MA, AdatiaI, et al[merckmanuals.com]
Anorexia
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • The most common symptoms and their frequency, reported in a national prospective study, are as follows: Dyspnea (60% of patients) Weakness (19%) Recurrent syncope (13%) Additional symptoms include fatigue, lethargy, anorexia, chest pain, and right upper[emedicine.medscape.com]
  • These may include the following: Dyspnea upon exertion Fatigue Lethargy Syncope with exertion Chest pain Anorexia Right upper quadrant pain Less common symptoms include the following: Cough Hemoptysis Hoarseness (due to compression of the recurrent laryngeal[emedicine.medscape.com]
  • Many of the medications are associated with side effects of anorexia or nausea, so careful attention to nutritional status is needed to maintain overall health. Infusion pump management.[web.archive.org]
Dyspnea
  • KEYWORDS: exertional dyspnea; pulmonary hypertension; pulmonary veno-occlusive disease; sudden death[ncbi.nlm.nih.gov]
  • Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography.[ncbi.nlm.nih.gov]
  • The greatest risk is a diagnostic delay due to focus on discarding a pulmonary etiology of dyspnea. Physical examination and early echocardiographic assessment are the keys to avoid overlooking this uncommon pathology. 2017, Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]
  • This patient visited our hospital at 37 weeks of gestation after experiencing dyspnea and chest pain. Clinical evaluation revealed severe fixed pulmonary hypertension.[ncbi.nlm.nih.gov]
  • METHODS AND RESULTS: A 57-year-old man was admitted to our hospital due to presyncope and dyspnea during exercise with a history of CKD-HD.[ncbi.nlm.nih.gov]
Cough
  • Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice. Diagnosis X ray-chest and laboratory studies are the first investigations followed by echocardiography.[symptoma.com]
  • The Ohio State Multidisciplinary Cough Clinic helps people with chronic coughs lasting at least six weeks.[lungcenter.osu.edu]
  • Other symptoms, such as coughing (rarely, coughing up blood) and wheezing, are usually caused by the underlying lung disorder.[merckmanuals.com]
  • Pulmonary Hypertension Heart Breathlessness Chest pain Dizziness Fainting Loss of energy Swelling of the arms, legs, ankles or abdomen Dry cough Raynaud’s phenomenon Heart and lung disorders Family history HIV infection Connective tissue disorders Congenital[nm.org]
  • He had a nonproductive cough the day before he was admitted. The day of admission he had decreased PO intake, lethargy, increased WOB. Patient had no primary care but had his 2 month vaccines from the health department.[ultrasoundoftheweek.com]
Hemoptysis
  • CASE PRESENTATION: Here, we present the case of a 47-year-old man who presented with a 10-year history of progressive hemoptysis and a 2-year history of shortness of breath, in whom a diagnosis of FM was made.[ncbi.nlm.nih.gov]
  • Hemoptysis has also been reported. Etiology IPAH is caused by vascular remodeling of small pulmonary arteries due to unknown causes.[orpha.net]
  • Hemoptysis has also been reported. HPAH patients have a severe clinical with less response to acute vasodilator challenge, lower cardiac index, and higher pulmonary vascular resistance.[orpha.net]
  • Hemoptysis has also been reported.[orpha.net]
  • Uncommon symptoms include cough, hemoptysis and hoarseness. The diagnosis of pulmonary hypertension should be suspected in patients with progressive exertional dyspnea, angina or syncope on exertion and a coexisting possible cause of the condition.[symptoma.com]
Hoarseness
  • Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice. Diagnosis X ray-chest and laboratory studies are the first investigations followed by echocardiography.[symptoma.com]
  • Rarely, people with pulmonary hypertension become hoarse.[merckmanuals.com]
  • Cough, hemoptysis, and hoarseness are less common symptoms. Women are more likely to be symptomatic than men. Physical Examination Physical findings in persons with PAH can be quite variable.[emedicine.medscape.com]
  • Affected individuals may also have a cough, sometimes with blood (hemoptysis), an enlarged heart and liver, low blood pressure (hypotension), and hoarseness due to compression of a nerve in the chest by an enlarged pulmonary artery.[rarediseases.org]
Exertional Dyspnea
  • KEYWORDS: exertional dyspnea; pulmonary hypertension; pulmonary veno-occlusive disease; sudden death[ncbi.nlm.nih.gov]
  • The diagnosis of pulmonary hypertension should be suspected in patients with progressive exertional dyspnea, angina or syncope on exertion and a coexisting possible cause of the condition.[symptoma.com]
  • Symptoms are fatigue, exertional dyspnea, and, occasionally, chest discomfort and syncope. Diagnosis is made by finding elevated pulmonary artery pressure (estimated by echocardiography and confirmed by right heart catheterization).[merckmanuals.com]
  • The most common symptoms of pulmonary hypertension include: Shortness of breath, particularly with exertion (dyspnea) Chest pain or angina Fatigue Dizziness Fainting spells (syncope) Racing pulse or irregular heart beat Swelling in the ankles or legs[utswmed.org]
Chest Pain
  • Signs and symptoms of pulmonary hypertension (PH) may include: Shortness of breath during routine activity, such as climbing two flights of stairs Tiredness Chest pain A racing heartbeat Pain on the upper right side of the abdomen Decreased appetite As[web.archive.org]
  • This patient visited our hospital at 37 weeks of gestation after experiencing dyspnea and chest pain. Clinical evaluation revealed severe fixed pulmonary hypertension.[ncbi.nlm.nih.gov]
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • Initial symptoms include dyspnea, fatigue, syncope, chest pain, palpitations and pedal edema.[orpha.net]
  • Other symptoms include: Ankle and leg swelling Bluish color of the lips or skin ( cyanosis ) Chest pain or pressure, usually in the front of the chest Dizziness or fainting spells Fatigue Increased abdomen size Weakness People with pulmonary hypertension[nlm.nih.gov]
Exertional Syncope
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • Exertional syncope . Sudden cardiac death .[patient.info]
  • Exertional syncope. Sudden cardiac death.[patient.info]
  • Lifestyle Modifications Although there are few data on which to base recommendations about exercise, heavy physical activity or isotonic exercises can evoke exertional syncope.[doi.org]
Graham Steell Murmur
  • Pulmonic regurgitation (Graham Steell murmur) may be apparent. A murmur of tricuspid regurgitation can be present, and a right ventricular lift (heave) may be noted.[emedicine.medscape.com]
  • Steell murmur) may be apparent A murmur of tricuspid regurgitation can be present, and a right ventricular lift (heave) may be noted Jugular venous pulsations may be elevated in the presence of volume overload, right ventricular failure, or both; large[emedicine.medscape.com]
Dizziness
  • Patients experience symptoms such as shortness of breath, dizziness, and fatigue, and the severity of symptoms usually correlates with the progression of the disease.[pfizer.com]
  • Fatigue, dizziness, and fainting spells also can be symptoms. Swelling in the ankles, abdomen or legs, bluish lips and skin, and chest pain may occur as strain on the heart increases.[my.clevelandclinic.org]
  • Other symptoms are fatigue, dizziness, swelling in the ankles or legs (edema), bluish lips and skin (cyanosis), chest pain, racing pulse and palpitations.[who.int]
Lethargy
  • Common presenting features are exertional dyspnea, lethargy, fatigue, exertional syncope and chest pain, peripheral edema, anorexia and right upper quadrant pain. Uncommon symptoms include cough, hemoptysis and hoarseness.[symptoma.com]
  • The day of admission he had decreased PO intake, lethargy, increased WOB. Patient had no primary care but had his 2 month vaccines from the health department. He was found to have profound respiratory acidosis and hypoxemia. Post navigation[ultrasoundoftheweek.com]
  • Other less common symptoms may include: cough , fatigue , lethargy, and dizziness .[emedicinehealth.com]
  • The most common symptoms and their frequency, reported in a national prospective study, are as follows: Dyspnea (60% of patients) Weakness (19%) Recurrent syncope (13%) Additional symptoms include fatigue, lethargy, anorexia, chest pain, and right upper[emedicine.medscape.com]

Workup

The diagnosis of pulmonary hypertension should be suspected in patients with progressive exertional dyspnea, angina or syncope on exertion and a coexisting possible cause of the condition. Electrocardiography (ECG), laboratory studies (complete blood count (CBC), liver function tests, HIV serology, prothrombin time (PT), and activated partial thromboplastin time (aPTT)), arterial blood gas measurements and X-rays of the chest should be obtained. Echocardiography with Doppler flow studies is the most useful noninvasive method and can be employed to estimate the pulmonary artery pressure and ventricular function [5] [6]. Depending on the etiology, further evaluation may include pulmonary function testing, high-resolution computed tomography of the chest, polysomnography, ventilation-perfusion lung scanning and right cardiac catheterization. The diagnosis of most forms of pulmonary hypertension requires right heart catheterization and is confirmed when the mean pulmonary artery pressure is greater than 25 mmHg at rest [1].

Pulmonary Arterial Pressure Increased
  • The current Nice Classification system of PH now lists the following genetic defects that are known to be associated with PAH [8] : BMPR2 ALK1 ENG SMAD9 CAV1 KCNK3 Early in IPAH (and probably in APAH), as the pulmonary artery pressure increases because[emedicine.medscape.com]
Right Axis Deviation
  • Surface electrocardiogram showed right atrial and ventricular overload with right axis deviation. Chest imaging noted enlarged central pulmonary vascularity with bilateral plethoric lung fields.[ncbi.nlm.nih.gov]
  • These signs include the following: Right-axis deviation R/S ratio greater than 1 in lead V1 Right atrial enlargement as indicated by an increased P-wave amplitude in lead II Right bundle branch block PPH is diagnosed if no underlying etiology is found[emedicine.com]
  • Common ECG findings include right axis deviation, R S in V 1 , S 1 Q 3 T 3 (suggesting right ventricular hypertrophy), and peaked P waves (suggesting right atrial dilation).[merckmanuals.com]
  • Common ECG findings include right axis deviation, R S in V1, S1Q3T3 (suggesting right ventricular hypertrophy), and peaked P waves (suggesting right atrial dilation).[merckmanuals.com]
  • axis deviation, right ventricular hypertrophy, and characteristic ST depression and T-wave inversions in the anterior leads.[emedicine.medscape.com]
T Wave Inversion
  • On multivariable logistic regression analysis, S wave depth in lead V5 (odds ratio [OR] 1.25, 95% confidence interval [CI] 1.10-1.47) and depth of T wave inversion in lead V4 (OR 1.21, 95% CI 1.03-1.46) were independent predictors of MPAP 25 mmHg, and[ncbi.nlm.nih.gov]
  • inversions in the anterior leads.[emedicine.medscape.com]
P Pulmonale
  • […] respiratory disease) Examination prominent ‘a’ wave palpable P2 right heart failure – increased JVP, (large V waves), parasternal heave, TR murmur, hepatomegaly, peripherial oedema other signs of CHF hypoxaemia INVESTIGATIONS Bedside ECG – RVH, RAD, p-pulmonale[lifeinthefastlane.com]
Prominent A-Wave
  • ‘a’ wave palpable P2 right heart failure – increased JVP, (large V waves), parasternal heave, TR murmur, hepatomegaly, peripherial oedema other signs of CHF hypoxaemia INVESTIGATIONS Bedside ECG – RVH, RAD, p-pulmonale, tall R waves in V1, right ventricular[lifeinthefastlane.com]
  • A prominent A wave may be observed in the jugular venous pulse. A right-sided fourth heart sound (S 4 ) with a left parasternal heave may be auscultated. Right ventricular failure leads to systemic venous hypertension and cor pulmonale.[emedicine.medscape.com]

Treatment

  • Research is ongoing for better PH treatments. These treatments offer hope for the future.[web.archive.org]
  • Before treatment, there were no significant differences in LVEF, LVFS, NT–proBNP, hsCRP, 6-MWT, LVD, LAD and LVMI between the treatment group and control group.[ncbi.nlm.nih.gov]
  • Comprehensive evaluations are needed to determine the severity of PH and associated diseases and to initiate treatment focused on FC.[ncbi.nlm.nih.gov]
  • Unfortunately, there is no treatment of proven benefit for this condition. Medications used for treatment of pulmonary arterial hypertension are of great interest in this respect.[ncbi.nlm.nih.gov]

Prognosis

  • PAH associated with systemic sclerosis carries a worse prognosis than idiopathic PAH. However, PAH associated with congenital heart disease has a better prognosis than idiopathic PAH.[patient.info]
  • KEYWORDS: Biomarkers; Prognosis; Pulmonary hypertension; Risk stratification[ncbi.nlm.nih.gov]
  • During the course of the disease it can be rarely complicated with pulmonary hypertension (PH) which confers a dismal prognosis.[ncbi.nlm.nih.gov]
  • Prognosis Prognosis varies between different forms, but is generally poor; the mean survival rate after diagnosis is The documents contained in this web site are presented for information purposes only.[orpha.net]

Etiology

  • It has a very low prevalence and its etiology is heterogeneous. Complex congenital cardiac malformations are also rare.[ncbi.nlm.nih.gov]
  • Clinical description Clinical manifestations of IPAH are similar to other forms of PAH regardless of its etiology. IPAH develop in adults and in rare cases in children; women are twice as likely as men to be affected.[orpha.net]
  • The diagnosis of the disorder is often delayed due to nonspecific symptoms and signs, frequently reflecting the underlying etiology.[symptoma.com]
  • Pulmonary hypertension (PH) is a disease with multiple etiologies and is categorized into five broad groups.[ncbi.nlm.nih.gov]
  • The greatest risk is a diagnostic delay due to focus on discarding a pulmonary etiology of dyspnea. Physical examination and early echocardiographic assessment are the keys to avoid overlooking this uncommon pathology. 2017, Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]

Epidemiology

  • This review gives an overview of the pathophysiology and epidemiology of PH associated with left heart disease, and discusses the challenges associated with its management and treatment.[ncbi.nlm.nih.gov]
  • The review provides an update of the epidemiology, pathogenesis, risk factors, screening and treatment of pulmonary arterial hypertension in systemic sclerosis.[ncbi.nlm.nih.gov]
  • Purpose of review: The review provides an update of the epidemiology, pathogenesis, risk factors, screening and treatment of pulmonary arterial hypertension in systemic sclerosis.[web.archive.org]
  • Abstract Purpose of review The review provides an update of the epidemiology, pathogenesis, risk factors, screening and treatment of pulmonary arterial hypertension in systemic sclerosis.[doi.org]
  • The present review is focused on hypertension, pulmonary hypertension, ischemic heart disease, and CHF to newly summarize the studies available to date from the viewpoints of epidemiology, pathogenesis, and treatment.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • The activation of molecular pathways is the pathophysiological underpinning of the biomarkers assessed in peripheral venous blood.[ncbi.nlm.nih.gov]
  • Here, we briefly discuss the pathophysiology of pulmonary hypertension and endothelial dysfunction, along with current treatments.[doi.org]
  • While the pathophysiology underlying the development of pulmonary hypertension (PH) in patients with HHT is not fully understood, it is believed to occur by one of two mechanisms: increases in pulmonary vascular resistance or cardiac output.[ncbi.nlm.nih.gov]
  • Although frequently a target of therapy, its pathophysiology remains poorly understood and its treatment remains undefined.[ncbi.nlm.nih.gov]
  • The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options.[ncbi.nlm.nih.gov]

Prevention

  • Abstract Sleep-disordered breathing (SDB) is a risk factor for cardiovascular disease including acute coronary syndrome and acute myocardial infarction, and treating SDB prevents their development and recurrence and improves a patient's prognosis.[ncbi.nlm.nih.gov]
  • ., dizziness, chest pain, ankle swelling, feeling your heart race or pound [palpitations]). 2,3 Prevention and Treatment While not all pulmonary hypertension can be prevented, efforts to prevent high blood pressure, coronary heart disease, chronic liver[cdc.gov]
  • Given the need for improved diagnostic and therapeutic options for PH, the development or repurposing of metabolic tracers and medications could provide an effective avenue for preventing or even reversing disease progression.[ncbi.nlm.nih.gov]
  • These medicines help prevent blood clots from forming or getting larger. Digoxin. This medicine helps the heart beat stronger and pump more blood.[web.archive.org]

Summary

Pulmonary hypertension is characterized by a mean pulmonary arterial pressure exceeding 25 mm Hg at rest or 30 mm Hg during exercise [1]. The World Health Organization (WHO) classified pulmonary hypertension into five main groups based upon etiology and disease mechanisms:

Pulmonary arterial hypertension (PAH)

Pulmonary hypertension owing to left heart disease

Pulmonary hypertension owing to lung diseases and/or hypoxia

Chronic thromboembolic pulmonary hypertension (CTEPH)

Pulmonary hypertension with unclear multifactorial mechanisms

Patient Information

Definition

Pulmonary hypertension is a rise of blood pressure and resistance in the lung vessels.

Cause

Pulmonary hypertension may be without any cause or with some associated factors like cirrhosis of the liver, appetite suppressants, HIV infection, alpha adrenergic stimulants like amphetamine and cocaine. It may also be due to some underlying diseases like left side heart diseases such as valvular abnormalities, myocardial diseases, and compression of pulmonary vein or respiratory conditions including COPD, interstitial diseases, sleep apnea and high altitude sickness for a long time. It may occur after thromboembolism episode or recurrent thromboembolism.

Symptoms

Difficulty in breathing is commonest symptom followed by weakness and fainting. Other symptoms include fatigue lethargy, loss of appetite, chest pain, cough, blood in cough, hoarseness of voice.

Diagnosis

X ray-chest and laboratory studies are the first investigations followed by echocardiography. Further investigations depend on the underlying cause. To confirm the diagnosis, right-sided cardiac catheterization may be done.

Treatment

Cases are treated with oxygen, pharmacological therapy and if required surgical intervention. In secondary cases management of primary condition is important. Pharmacological agents include calcium channel blockers, endothelin-receptor antagonists, Phosphodiesterase type 5 inhibitors, soluble guanylate cyclase (sGC) stimulators, prostacyclins, digoxin, and anticoagulants.

References

Article

  1. Simonneau G, GalièN, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 Suppl S):5S.
  2. Runo JR, Loyd JE. Primary pulmonary hypertension. Lancet 2003; 361:1533.
  3. Peacock AJ. Primary pulmonary hypertension. Thorax 1999; 54:1107.
  4. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016 Jan 1;37(1):67-119.
  5. Jacobs W, Konings TC, Heymans MW, et al. Noninvasive identification of left-sided heart failure in a population suspected of pulmonary arterial hypertension. Eur Respir J 2015; 46:422.
  6. Berger M, Haimowitz A, Van Tosh A, et al. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 1985; 6:359.

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Last updated: 2018-06-22 01:09