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. Carlos Monge Medrano described the first case of “Sindrome eritrémico de altura” in Alberto Hurtado described excessive blood unsaturation in 1964.

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

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

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

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.

Hematocrit at different altitudes

Where is the trigger of CMS located ? COMMON DENOMINATOR: CHRONIC HYPOXIA 1. In the Respiratory Center ? 2. In the Lungs? 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 ? 3. In the Carotid Bodies?

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

The most evident signs of CMS EPO Ca0 2 SaO 2 PAP Pulmonary Hypertension Right Heart Hypertrophy Increased Polycythemia These 3 clinical signs are above normal values for each altitude and are reversible at sea level CMS is due to different kinds of disease + chronic hypoxia CMS is due to different kinds of disease + chronic hypoxia

Disease + Chronic Hypoxia EPO 0202 SaO 2 PAP Pulmonary Hypertension Right Heart Hypertrophy Increased Polycythemia 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.

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

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

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

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

OXYGEN TRANSPORT MECHANISMS Q V Hb HR SV RRTV.. STROKE VOL. HEART RATE RESP. RATETIDAL VOL.

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