Phlebotomy improves Pulmonary Gas Exchange in Chronic Mountain Polycythemia

Cruz, Julio C.; Díaz, Carlos Jerjes-Sánchez; Marticorena, Emilio; Hilario, Vilma

doi:10.1159/000194097

Cited by 36 publications

(17 citation statements)

References 22 publications

(43 reference statements)

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“…Previous human studies have also suggested that an excessive increase in blood viscosity is involved in impaired gas exchange and reduced exercise performance. This has been observed in cases of polycythemia vera (27), high-altitude polycythemia, and chronic mountain sickness (4,52,53). Of note, upon either phlebotomy or hemodilution that reduced the hematocrit from excessive to normal (e.g., sea level) values, individuals experienced a stimulation of ventilation and an improved ventilation-perfusion pattern.…”

Section: Discussionmentioning

confidence: 90%

Excessive erythrocytosis in adult mice overexpressing erythropoietin leads to hepatic, renal, neuronal, and muscular degeneration

Heinicke

Baum²,

Ogunshola

et al. 2006

American Journal of Physiology-Regulatory, Integrative and Comp

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To investigate the consequences of inborn excessive erythrocytosis, we made use of our transgenic mouse line (tg6) that constitutively overexpresses erythropoietin (Epo) in a hypoxia-independent manner, thereby reaching hematocrit levels of up to 0.89. We detected expression of human Epo in the brain and, to a lesser extent, in the lung but not in the heart, kidney, or liver of tg6 mice. Although no acute cardiovascular complications are observed, tg6 animals have a reduced lifespan. Decreased swim performance was observed in 5-mo-old tg6 mice. At about 7 mo, several tg6 animals developed spastic contractions of the hindlimbs followed by paralysis. Morphological analysis by light and electron microscopy showed degenerative processes in liver and kidney characterized by increased vascular permeability, chronic progressive inflammation, hemosiderin deposition, and general vasodilatation. Moreover, most of the animals showed severe nerve fiber degeneration of the sciatic nerve, decreased number of neuromuscular junctions, and degeneration of skeletal muscle fibers. Most probably, the developing demyelinating neuropathy resulted in muscular degeneration demonstrated in the extensor digitorum longus muscle. Taken together, chronically increased Epo levels inducing excessive erythrocytosis leads to multiple organ degeneration and reduced life expectancy. This model allows investigation of the impact of excessive erythrocytosis in individuals suffering from polycythemia vera, chronic mountain sickness, or in subjects tempted to abuse Epo by means of gene doping.

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Section: Discussionmentioning

confidence: 90%

Excessive erythrocytosis in adult mice overexpressing erythropoietin leads to hepatic, renal, neuronal, and muscular degeneration

Heinicke

Baum²,

Ogunshola

et al. 2006

American Journal of Physiology-Regulatory, Integrative and Comp

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show abstract

“…Treatment for CMS includes relocation to a lower altitude, periodic phlebotomy [84], isovolemic hemodilution [85,86] or supplemental oxygen [87]. The long-term use of respiratory stimulants, such as medroxyprogesterone [88] or acetazolamide [89-91], also improves oxygenation and polycythemia in CMS patients.…”

Section: Respiratory Disorders Associated With Chronic Exposure To Himentioning

confidence: 99%

Lung disease at high altitude

Stream¹,

Luks²,

Grissom³

2009

Expert Review of Respiratory Medicine

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Large numbers of people travel to high altitudes, entering an environment of hypobaric hypoxia. Exposure to low oxygen tension leads to a series of important physiologic responses that allow individuals to tolerate these hypoxic conditions. However, in some cases hypoxia triggers maladaptive responses that lead to various forms of acute and chronic high altitude illness, such as high-altitude pulmonary edema or chronic mountain sickness. Because the respiratory system plays a critical role in these adaptive and maladaptive responses, patients with underlying lung disease may be at increased risk for complications in this environment and warrant careful evaluation before any planned sojourn to higher altitudes. In this review, we describe respiratory disorders that occur with both acute and chronic exposures to high altitudes. These disorders may occur in any individual who ascends to high altitude, regardless of his/her baseline pulmonary status. We then consider the safety of high-altitude travel in patients with various forms of underlying lung disease. The available data regarding how these patients fare in hypoxic conditions are reviewed, and recommendations are provided for management prior to and during the planned sojourn.

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“…MONGE and WHITTEMBURY [56] described a syndrome marked by the triad of polycythaemia, hypoxaemia and impaired mental function in which affected individuals complain of headache, fatigue, impaired concentration, irritability and impaired exercise tolerance, and physical examination demonstrates clubbing, congested mucosal surfaces and cyanosis. Treatment involves relocation to lower elevations or, in cases where relocation is not feasible, periodic phlebotomy [57], isovolaemic haemodilution [58,59] and long-term use of respiratory stimulants such as acetazolamide [60] or medroxyprogesterone [61]. Right heart failure was not described in the report by MONGE and WHITTEMBURY [56], but has subsequently been described in advanced stages of the disease in populations outside the Andes [62].…”

Section: High-altitude Illnessmentioning

confidence: 99%

Travel to high altitude with pre-existing lung disease

Luks

Swenson²

2007

Eur Respir J

107

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The pathophysiology of high-altitude illnesses has been well studied in normal individuals, but little is known about the risks of high-altitude travel in patients with pre-existing lung disease. Although it would seem self-evident that any patient with lung disease might not do well at high altitude, the type and severity of disease will determine the likelihood of difficulty in a high-altitude environment. The present review examines whether these individuals are at risk of developing one of the main forms of acute or chronic high-altitude illness and whether the underlying lung disease itself will get worse at high elevations. Several groups of pulmonary disorders are considered, including obstructive, restrictive, vascular, control of ventilation, pleural and neuromuscular diseases. Attempts will be made to classify the risks faced by each of these groups at high altitude and to provide recommendations regarding evaluation prior to high-altitude travel, advice for or against taking such excursions, and effective prophylactic measures.

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Phlebotomy improves Pulmonary Gas Exchange in Chronic Mountain Polycythemia

Cited by 36 publications

References 22 publications

Excessive erythrocytosis in adult mice overexpressing erythropoietin leads to hepatic, renal, neuronal, and muscular degeneration

Excessive erythrocytosis in adult mice overexpressing erythropoietin leads to hepatic, renal, neuronal, and muscular degeneration

Lung disease at high altitude

Travel to high altitude with pre-existing lung disease

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