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Monge´s Disease

Monge´s Disease. Prof. Dr. Gustavo Zubieta-Castillo High Altitude Pathology Institute (IPPA) La Paz, Bolivia. History- fundamental landmarks. F. Viault first reported an increase of hematocrit in 1889 in the Peruvian Cordillera.

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Monge´s Disease

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  1. Monge´s Disease Prof. Dr. Gustavo Zubieta-Castillo High Altitude Pathology Institute (IPPA) La Paz, Bolivia

  2. History- fundamental landmarks • F. Viault first reported an increase of hematocrit in 1889 in the Peruvian Cordillera. • Carlos Monge Medrano described the first case of “Sindrome eritrémico de altura” in 1925. • Alberto Hurtado described excessive blood unsaturation in 1964.

  3. Introductory comments about CMS in the 3rd World Congress on Mountain Medicine and Physiology. Matsumoto, Japan 1998 “Unfortunately, the concepts and description of the disorder have varied so much from one investigator to another, that comparison of experiences is difficult and sometimes impossible.” Toshio Kobayashi, Shigeru Matsuyama, Carlos Monge, Hideki Ohno, John T. Reeves. Progress in Mountain Medicine and High Altitude Physiology

  4. Monge´s Disease • Also know as: • Chronic Mountain Sickness (CMS) • Increased polycythemia • Erythrocytosis • Erythremia

  5. Countries where CMS has been found KYRGHYSTAN NEPAL CHINA U.S.A. PAKISTAN INDIA PERU BOLIVIA CHILE

  6. CMS and altitude differences • In only one city like La Paz, there are big differences in the degree of increased polycythemia between residents at 3600 m and 4100 m.

  7. Hematocrit at different altitudes

  8. Where is the trigger of CMS located ? 1. In the Respiratory Center ? 2. In the Lungs? 3. In the Carotid Bodies? 4. In the Heart ? 5. In the Kidney? 6. In the Gonads ? 7. In the Bone Marrow ? (In hemoglobin anomalies ?) 8. In Aging ? 9. In a combination of 2 or more factors ? COMMON DENOMINATOR: CHRONIC HYPOXIA

  9. The trigger of CMS is located in different systems and organs (according to different authors) in a feedback mechanism with the respiratory center

  10. The most evident signs of CMS SaO2 PAP Pulmonary Hypertension 1 1 Right Heart Hypertrophy 2 2 EPO Increased Polycythemia 3 Ca02 These 3 clinical signs are above normal values for each altitude and are reversible at sea level 3 CMS is due to different kinds of disease + chronic hypoxia

  11. Disease + Chronic Hypoxia SaO2 PAP Pulmonary Hypertension 1 1 Right Heart Hypertrophy 2 2 EPO Increased Polycythemia 3 02 3 Above normal values for altitude and reversible at sea level The sole presence of these three clinical signs in a patient under chronic hypoxia should be known as Monge‘s Disease or CMS, regardless of the pathogenesis. Other signs and symptoms can be present or absent in each particular case. This would permit a clear identification of the disease.

  12. Hematocrit in relation to Age in CMS, La Paz, 3510 m

  13. Hematocrit in relation to FVC in CMS, La Paz 3510 m

  14. CMS in the real life scenario • This is a patient that comes to consultation because: • of a cyanotic appearance • or a casual laboratory test gave a high hematocrit level • was phlebotomized or • was treated with phenylhydrazine a toxic drug, or • the physician in charge was alarmed

  15. CMS in the real life scenario Most patients with CMS are carrying out their lives normally. They have no symptoms. They are mostly concerned about their cyanotic looks. or somebody told them that they have CMS and scared them. they off and on feel temporarily dyspneic: Triple Hypoxia Syndrome

  16. OXYGEN TRANSPORT MECHANISMS . RESPIRATION V PNEUMO-DYNAMIC PUMP RR TV RESP. RATE TIDAL VOL. Hb . SV HR HEMO-DYNAMIC PUMP STROKE VOL. Q HEART RATE CIRCULATION

  17. Our favorite photo from the 3rd World Congress in Japan:

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