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Down Syndrome Abstract
of the Month: Oct 2001

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Pulmonary edema in 6 children with Down syndrome during travel to moderate altitudes

Durmowicz AG
Pediatrics 108(2):443-447, 2001

Division of Pediatric Pulmonology, Univ. of Utah Health Science Center and Primary Children's Medical Center, Salt Lake City, Utah.

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Objective: Children with Down syndrome (DS) are living longer and are increasingly participating in recreational activities. When a child with DS was diagnosed with high-altitude pulmonary edema (HAPE), this study was undertaken to determine whether and under what circumstances children with DS develop HAPE. Design: A retrospective review of the medical records of Children's Hospital, Denver, Colorado was performed for children with a discharge diagnosis of HAPE. Diagnostic criteria for HAPE included the presence of crackles or frothy sputum production on examination, hypoxemia, chest radiograph findings consistent with pulmonary edema, and rapid clinical improvement after descent or oxygen therapy. Results: A total of 52 patients with HAPE were found of whom 6 also had DS. The age range of the children with DS was 2 to 14 years. HAPE developed at altitudes ranging from 1738 to 3252 m. Four children developed HAPE within 24 hours of arrival to altitude. Three children had chronic pulmonary hypertension, and 4 had either an existing cardiac defect with left-to-right shunt or previously had a defect with left-to-right shunt that had been repaired. One child had Eisenmenger syndrome with chronic right-to-left shunting of blood. Five children had preexisting illnesses before travel to altitude. Conclusion: Children with DS often have medical problems such as chronic pulmonary hypertension, frequent infections, and pulmonary vascular overperfusion and injury from existing or previous cardiac defects. These problems all may be viewed as risk factors for HAPE and thus result in the rapid development of HAPE at low altitudes. Care should be taken when traveling to even moderate altitudes with children with DS.

My comments:

First, a quick translation of the altitudes for those who are metric-disabled: "1738 to 3253 m" is equivalent to "5702 to 10,673 feet."

Unfortunately, this study only looked retrospectively at children who had developed high-altitude pulmonary edema (HAPE). What we now need is a study of children with DS who visit high-altitude areas and see how many develop HAPE to get an idea of risk. So the most we can say now is that developing HAPE is a possibility, but we don't know how much of a possibility.

Coincidentally, the Fall 2001 conference for the National Down Syndrome Congress was scheduled for Denver before being cancelled due to the events of Sept 11. Before the cancellation, the NDSC had addressed this study by stating that at a previous NDSC convention in Denver, there were no reports of children with DS attending the convention developing HAPE.

Therefore, while there is no current admonition for children or adults with DS to avoid high-altitude areas, parents and caregivers should be aware of the possibility and be on the lookout for the associated symptoms: cough, rapid respiratory rate, cyanosis and lethargy.

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