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High Altitude Pulmonary Edema

Pulm Edema of Mountaineers


Presentation

  • This case describes HAPE as first manifestation of a sarcoidosis-related pulmonary hypertension with a very unusual and early presentation of the underlying disease in an apparently healthy mountaineer.[ncbi.nlm.nih.gov]
  • The mean ONSD for HAPE patients on presentation was 5.7 /- 0.44 mm and for controls was 4.7 /- 0.56 mm (p 0.003).[ncbi.nlm.nih.gov]
  • CONCLUSIONS: A significant number of patients presenting to the Pheriche medical aid post with high altitude pulmonary edema were given dexamethasone, sildenafil, or acetazolamide in addition to oxygen, nifedipine, and descent.[ncbi.nlm.nih.gov]
  • Children living at elevation presenting with hypoxemia are commonly misdiagnosed.[ncbi.nlm.nih.gov]
  • A 63-year-old patient with Holmes-Adie syndrome presented an altered peripheral chemoreflex and suffered from high altitude pulmonary edema, suggesting an alteration of sensitive afferent fibers from the peripheral chemoreceptors.[ncbi.nlm.nih.gov]
Mediastinal Lymphadenopathy
  • Subsequent medical reevaluation showed a significant progress of mediastinal lymphadenopathy. Due to the histological proof of sarcoidosis, the initial diagnosis of PAH had to be changed to sarcoidosis-related pulmonary hypertension.[ncbi.nlm.nih.gov]
Cough
  • This case describes a patient with no prior history of HAPE and extensive experience hiking above 2,500 meters who developed progressive dyspnea and cough while ascending to 3,200 meters.[ncbi.nlm.nih.gov]
  • A 26-year-old woman presented with dyspnea and dry cough soon after arriving on the Qinghai-Tibet Plateau (3650 m). Chest radiograph showed diffuse patchy infiltrates. The initial diagnosis was high altitude pulmonary edema (HAPE).[ncbi.nlm.nih.gov]
  • During the next 2 d at altitude, the following symptoms occurred: cough with foamy sputum, cyanosis, and loss of consciousness.[ncbi.nlm.nih.gov]
  • A 39-year-old male, previously well, was admitted at a hospital situated at a height of 3500 m with complaints of increasing breathlessness on effort, cough, and fever of short duration, 5 days after high altitude reascent.[ncbi.nlm.nih.gov]
  • The following measurements were noted: dyspnea, cough, chest pain, cyanosis, pulse rate, blood pressure, respiratory rate, crepitations, radiographic abnormalities, electrocardiogram, peripheral pulse oximetry (Spo(2)) at admission, Spo(2) normalization[ncbi.nlm.nih.gov]
Dyspnea
  • High altitude pulmonary edema (HAPE) is a form of high altitude illness characterized by cough, dyspnea upon exertion progressing to dyspnea at rest and eventual death, seen in patients who ascend over 2,500 meters, particularly if that ascent is rapid[ncbi.nlm.nih.gov]
  • A 26-year-old woman presented with dyspnea and dry cough soon after arriving on the Qinghai-Tibet Plateau (3650 m). Chest radiograph showed diffuse patchy infiltrates. The initial diagnosis was high altitude pulmonary edema (HAPE).[ncbi.nlm.nih.gov]
  • The following measurements were noted: dyspnea, cough, chest pain, cyanosis, pulse rate, blood pressure, respiratory rate, crepitations, radiographic abnormalities, electrocardiogram, peripheral pulse oximetry (Spo(2)) at admission, Spo(2) normalization[ncbi.nlm.nih.gov]
  • In the patient this is manifested as a non-productive cough, mild dyspnea on exertion, and difficulty ascending. 3 This progresses to dyspnea at rest; pink, frothy sputum that may include frank blood; and drowsiness.[westjem.com]
  • “Symptoms include fatigue, severe dyspnea at rest, and cough that is initially dry but may progress to produce pink, frothy sputum. Descent to lower altitudes alleviates the symptoms of HAPE.”[drfrafiq.wordpress.com]
Tachypnea
  • […] of: Difficulty in breathing (dyspnea) at rest Cough Weakness or decreased exercise performance Chest tightness or congestion Signs, at least two of: Crackles or wheezing (while breathing) in at least one lung field Central cyanosis (blue skin color) Tachypnea[drfrafiq.wordpress.com]
  • […] presence of the following: Symptoms: at least two of: - dyspnea at rest - cough - weakness or decreased exercise performance - chest tightness or congestion Signs: at least two of: - crackles or wheezing in at least one lung field - central cyanosis - tachypnea[high-altitude-medicine.com]
  • […] following: Symptoms: at least two of: Shortness of breath at rest Cough Weakness or decreased exercise performance Chest tightness or congestionSigns: at least two of: Crackles or wheezing (while breathing) in at least one lung field Central blue skin color Tachypnea[en.wikipedia.org]
  • […] be kept warm as cold stress will worsen their condition. 1,6,18 If supplemental oxygen is available, high flow oxygen at 2 to 4 liters per minute with a goal oxygen saturation of 90% will decrease pulmonary artery pressures and improve the patient’s tachypnea[emdocs.net]
  • […] uncommon, HAPE is a potentially life-threatening non-cardiogenic pulmonary edema that occurs within 2-4 days after rapid ascent above 2,500 meters (8,000 feet) (Figure). 2 Clinically, the patient will be afebrile and have one or more of the following: tachypnea[westjem.com]
Dry Cough
  • A 26-year-old woman presented with dyspnea and dry cough soon after arriving on the Qinghai-Tibet Plateau (3650 m). Chest radiograph showed diffuse patchy infiltrates. The initial diagnosis was high altitude pulmonary edema (HAPE).[ncbi.nlm.nih.gov]
  • Even now, when I inhale sharply, I can still hear the gasping sound (it sounds a bit like a balloon blowing up, after the normal rush of air when you inhale), and my lungs feel irritated and scratchy, like I'm always on the verge of (dry) coughing.[ask.metafilter.com]
  • This condition is characterized by breathlessness, abnormally rapid heartbeat, mild cough, dry cough to blood stained sputum, and raised pulmonary artery pressure.[rakshapolycoats.com]
  • Initially, patients have dyspnea on exertion, decreased exertion tolerance, and dry cough. Later, dyspnea is present at rest. Pink or bloody sputum and respiratory distress are late findings.[merckmanuals.com]
  • Early signs and symptoms include decreased exercise tolerance compared to fellow travellers, dyspnea on exertion, longer than usual recovery time when resting, and a dry cough. As symptoms progress patients may develop cyanosis and crackles on exam.[emdocs.net]
Dyspnea at Rest
  • High altitude pulmonary edema (HAPE) is a form of high altitude illness characterized by cough, dyspnea upon exertion progressing to dyspnea at rest and eventual death, seen in patients who ascend over 2,500 meters, particularly if that ascent is rapid[ncbi.nlm.nih.gov]
  • “Symptoms include fatigue, severe dyspnea at rest, and cough that is initially dry but may progress to produce pink, frothy sputum. Descent to lower altitudes alleviates the symptoms of HAPE.”[drfrafiq.wordpress.com]
  • Reported clinical manifestations include: dyspnea at rest cough with frothy pink sputum production neurological disturbances associated with concomitant brain edema The pathogenesis is considered to be from the altered permeability of the alveolar-capillary[radiopaedia.org]
  • In the patient this is manifested as a non-productive cough, mild dyspnea on exertion, and difficulty ascending. 3 This progresses to dyspnea at rest; pink, frothy sputum that may include frank blood; and drowsiness.[westjem.com]
  • […] at rest - cough - weakness or decreased exercise performance - chest tightness or congestion Signs: at least two of: - crackles or wheezing in at least one lung field - central cyanosis - tachypnea - tachycardia The symposium consensus committee also[high-altitude-medicine.com]
Cyanosis
  • During the next 2 d at altitude, the following symptoms occurred: cough with foamy sputum, cyanosis, and loss of consciousness.[ncbi.nlm.nih.gov]
  • The following measurements were noted: dyspnea, cough, chest pain, cyanosis, pulse rate, blood pressure, respiratory rate, crepitations, radiographic abnormalities, electrocardiogram, peripheral pulse oximetry (Spo(2)) at admission, Spo(2) normalization[ncbi.nlm.nih.gov]
  • Symptoms, at least two of: Difficulty in breathing (dyspnea) at rest Cough Weakness or decreased exercise performance Chest tightness or congestion Signs, at least two of: Crackles or wheezing (while breathing) in at least one lung field Central cyanosis[drfrafiq.wordpress.com]
  • […] altitude, the presence of the following: Symptoms: at least two of: - dyspnea at rest - cough - weakness or decreased exercise performance - chest tightness or congestion Signs: at least two of: - crackles or wheezing in at least one lung field - central cyanosis[high-altitude-medicine.com]
  • Further Reading Cyanosis - What is Cyanosis? Symptoms of cyanosis Diagnosis of cyanosis Treatment of cyanosis[news-medical.net]
Tachycardia
  • Examination at admission revealed a febrile (38 degrees C) patient with tachycardia (104/min), SaO2 was 82% (on supplemental oxygen), chest examination revealed bilateral crackles in all lung fields, and chest radiograph demonstrated bilateral fluffy[ncbi.nlm.nih.gov]
  • His neck veins are flat and cardiac examination is normal except for tachycardia. He has bilateral crackles. What statement best characterizes this patient’s medical condition? a. Echocardiogram will show decreased left ventricular contractility. b.[drfrafiq.wordpress.com]
  • HAPE leads to dyspnea (shortness of breath), cough, tachycardia (fast heart rate) and decreased arterial oxygen levels. HAPE varies in degree from very mild to potentially fatal. Some people may be genetically susceptible to HAPE.[medicinenet.com]
  • […] the following: Symptoms: at least two of: - dyspnea at rest - cough - weakness or decreased exercise performance - chest tightness or congestion Signs: at least two of: - crackles or wheezing in at least one lung field - central cyanosis - tachypnea - tachycardia[high-altitude-medicine.com]
  • : Shortness of breath at rest Cough Weakness or decreased exercise performance Chest tightness or congestionSigns: at least two of: Crackles or wheezing (while breathing) in at least one lung field Central blue skin color Tachypnea (rapid breathing) Tachycardia[en.wikipedia.org]
Chest Pain
  • The following measurements were noted: dyspnea, cough, chest pain, cyanosis, pulse rate, blood pressure, respiratory rate, crepitations, radiographic abnormalities, electrocardiogram, peripheral pulse oximetry (Spo(2)) at admission, Spo(2) normalization[ncbi.nlm.nih.gov]
  • He denied history of fever and chest pain. He was inducted to high-altitude area (height 11,500 ft) 4 months back.[ijamhrjournal.org]
  • Exercise ECG (Table IV) Each subject underwent maximum symptom-limited (by dyspnoea or chest pain) exercise test and completed stage V of the modified Bruce protocol achieving his maximal target heart rate predicted for his age.[jpma.org.pk]
Headache
  • He has a mild headache after flying to Denver; the next day he drives to a cabin at 10,000 ft, and the following day climbs to 13,500 ft with friends.[drfrafiq.wordpress.com]
  • I started hearing a "gasping" sound after every inhale, plus shortness of breath, and rapid heartbeat (no nausea, headache or pain). It calmed down once I descended and drove back to Denver, where I live.[ask.metafilter.com]
  • I never got headaches worse than headaches that I sometimes get while hiking. I have never felt nauseated. So I never thought that I was susceptible to any high altitude illnesses. Fortunately for me, I have many hobbies. One of them is medicine.[weasel.com]
  • Marked cerebral edema manifests as headache and diffuse encephalopathy with confusion, drowsiness, stupor, and coma. Gait ataxia is a reliable early warning sign.[merckmanuals.com]
  • Far more likely is that you go to Quito and get headaches and feel nauseous and short of breath for a few days. Maybe that'll decide you--it's not dangerous, but it's not necessarily fun either. Hope that helps.[lonelyplanet.com]
Confusion
  • Before HAPE was understood it was commonly confused with pneumonia which resulted in inappropriate treatment.[en.wikipedia.org]
  • Asthma might also be confused with HAPE. Fortunately, asthmatics seem to do better at altitude than at sea-level.[basecampmd.com]
  • A severe headache, vomiting and lethargy will progress to unsteadiness, confusion, drowsiness and ultimately coma. HACE can kill in only a few hours.[altitude.org]
  • Unfortunately these side effects are common with AMS and therefore can easily be misdiagnosed as AMS Drowsiness and confusion is also possible – again these side effects can be confused with AMS Diamox comes in 250mg tablets.[climbkilimanjaroguide.com]
  • In addition to AMS symptoms, lethargy becomes profound, with drowsiness, confusion, and ataxia on tandem gait test, similar to alcohol intoxication.[wwwnc.cdc.gov]
Altered Mental Status
  • Individuals may also develop concurrent HACE due to severe hypoxemia; thus it’s important to monitor for ataxia and altered mental status. 6 In autopsy findings of over 20 persons who died of HAPE, 50% had HACE.[emdocs.net]
  • HACE is an extension of AMS characterized by neurologic findings, particularly ataxia, confusion, or altered mental status. HACE may also occur in the presence of HAPE.[wwwnc.cdc.gov]
Poor Coordination
  • In some cases, people will develop concomitant neurological features such as poor coordination, altered consciousness, or cerebral edema (High-altitude cerebral edema).On physical exam, increased breathing rates, increased heart rates, and a low-grade[en.wikipedia.org]

Workup

  • An extensive workup failed to identify a cardiac or a pulmonary cause of pulmonary hypertension. This patient experienced mild primary pulmonary hypertension related to the intake of anorexigens, which was revealed by high-altitude pulmonary edema.[ncbi.nlm.nih.gov]
Kerley Lines
  • A few Kerley lines may also be visible. In mild high-altitude pulmonary edema, consolidation may be subtle or absent with little or no involvement of the lung periphery.[radiopaedia.org]
  • Radiographic findings in HAPE are consistent with noncardiogenic edema, including a generally normal heart and left atrial size and no evidence of pulmonary venous prominence such as Kerley lines.[journals.lww.com]
Glycosuria
  • Investigations revealed leucocytosis, and urinalysis revealed glycosuria and ketonuria. Subsequent arterial blood gas analysis revealed that acidemia and serum glucose levels were raised.[ncbi.nlm.nih.gov]
Ketonuria
  • Investigations revealed leucocytosis, and urinalysis revealed glycosuria and ketonuria. Subsequent arterial blood gas analysis revealed that acidemia and serum glucose levels were raised.[ncbi.nlm.nih.gov]
Hypocapnia
  • Renal response to short-term hypocapnia in man. Kidney Int 1975;8:376-84. [ PUBMED ] 21. Hackett PH, Roach RC. High altitude cerebral edema. High Alt Med Biol 2004;5:136-46. [ PUBMED ] 22. Leaf DE, Goldfarb DS.[lungindia.com]
Transferrin Increased
  • The pulmonary transcapillary escape of radiolabeled transferrin increased slightly from low to high altitude in the HAPE-susceptible subjects but remained within the limits of normal and did not differ significantly from the control subjects.[ncbi.nlm.nih.gov]

Treatment

  • A review of the literature on the disease suggests that a wide range of strategies is employed for treatment purposes.[ncbi.nlm.nih.gov]
  • This review considers these issues in greater detail, examining the current state of research and clinical practice in HAPE treatment, addressing important questions raised by documented treatment practices, such as the utility of beta agonists, acetazolamide[doi.org]
  • Healthy mountaineers taking part in a trial of prophylactic treatment of HAPE were examined at low (490 m) and high altitude (4,559 m).[ncbi.nlm.nih.gov]
  • However, as outlined in the 2014 WMS Practice Guidelines, its use is recommended for the treatment of people with concomitant HAPE and HACE at the treatment doses recommended for HACE alone.[en.wikipedia.org]
  • Use of a portable hyperbaric chamber is an effective temporizing measure, and nifedipine may be used for treatment of HAPE, although only as an adjunct to descent and/or supplemental oxygen if these methods of treatment are not immediately available to[ncbi.nlm.nih.gov]

Prognosis

  • Navratilova was diagnosed and treated for breast cancer this year and her prognosis is excellent.[abcnews.go.com]

Etiology

  • Clinical manifestations of this disorder, its etiology, treatment, and prevention are discussed.[ncbi.nlm.nih.gov]
  • Accumulating evidence suggests that genetic factors play an important role in the etiology of HAPE. However, conclusions from association studies have been hindered by limited sample size due to the rareness of this disease.[ncbi.nlm.nih.gov]
  • The precise etiology of HAPE remains unclear. This study aimed to investigate whether NR3C1 gene polymorphism is associated with the susceptibility to HAPE.[ncbi.nlm.nih.gov]
  • […] an altered permeability of the alveolar-capillary barrier secondary to intense pulmonary vasoconstriction and high capillary pressure, but previous bronchoalveolar lavage (BAL) findings in well-established HAPE are also consistent with inflammatory etiologic[ncbi.nlm.nih.gov]
  • BACKGROUND: High-altitude pulmonary edema (HAPE) is thought of as an independent clinical disorder with a constitutional or genetic component in its etiology.[ncbi.nlm.nih.gov]

Epidemiology

  • The aim of this article was to present the relevant details concerning epidemiology, pathophysiology, clinical symptoms, prevention, and treatment of high altitude pulmonary edema among climbers in the mountain environment.[ncbi.nlm.nih.gov]
  • Epidemiologic observations in Peru. Chest 1978; 74(4): pp. 372-376. Hackett P.H. High-altitude medicine. Auerbach PS, ed. Wilderness Medicine. 6th Ed. Philadelphia, PA: Mosby; 2012. 2-43. Jones B.E., Stokes S., McKenzie S., and Nilles E.J.[emdocs.net]
  • Ward R (1995) Familial aggregation and genetic epidemiology of blood pressure. In:JH Laragh, BM Brenner (eds.). Hypertension: pathology, diagnosis, and management. Raven Press, New York. pp: 67-88.[clinical-and-molecular-endocrinology.imedpub.com]
  • […] of secondary inflammation in HAPE do not exclude the possibility that pulmonary edema will occur at modest altitudes in some individuals by a preceding or concurrent infectious or inflammatory state, as demonstrated in animals 19, 20 and suggested by epidemiologic[doi.org]
  • The epidemiology of lymphangioleiomyomatosis in Japan: a nationwide cross-sectional study of presenting features and prognostic factors. Respirology. 2007;12:523-30. 肺癌・胸部悪性腫瘍 胸腺癌および肺癌の生物学的特性と治療効果の検討。全国多施設共同研究への参加。長野県内多施設共同研究への計画・実施。[shinshu-u.ac.jp]
Sex distribution
Age distribution

Pathophysiology

  • The critical pathophysiology is an excessive rise in pulmonary vascular resistance or hypoxic pulmonary vasoconstriction (HPV) leading to increased microvascular pressures.[ncbi.nlm.nih.gov]
  • METHODS: Availing the literature, an in-silico study was performed to explore the anticipated role of miRNAs in HAPE pathophysiology.[ncbi.nlm.nih.gov]
  • This paper will review the basic pathophysiology, prevention, and treatment of the most serious of the high altitude clinical syndromes, high altitude pulmonary edema.[ncbi.nlm.nih.gov]
  • The pathophysiology of HAPE is reviewed with a focus on the evidence for rapid reversibility of pulmonary vascular injury.[ncbi.nlm.nih.gov]

Prevention

  • Inhaled salmeterol prevents HAPE presumably by increasing alveolar fluid clearance, the phosphodiesterase-5 inhibitor tadalafil works by acting as a pulmonary vasodilator, and dexamethasone seems to prevent HAPE by stabilizing the capillary endothelium[ncbi.nlm.nih.gov]
  • The suggested rate of ascent is the same that applies to the prevention of acute mountain sickness and high-altitude cerebral edema.[en.wikipedia.org]
  • We therefore hypothesized that the prophylactic administration of nifedipine would prevent its recurrence.[ncbi.nlm.nih.gov]
  • This paper will review the basic pathophysiology, prevention, and treatment of the most serious of the high altitude clinical syndromes, high altitude pulmonary edema.[ncbi.nlm.nih.gov]
  • Clinical manifestations of this disorder, its etiology, treatment, and prevention are discussed.[ncbi.nlm.nih.gov]

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