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Respiratory Complications of Metatropic Dwarfism
Journal article   Peer reviewed

Respiratory Complications of Metatropic Dwarfism

Jaques Belik, Endla K. Anday, Frederick Kaplan and Elaine Zackai
Clinical pediatrics, v 24(9), pp 504-511
Sep 1985
DOI: https://doi.org/10.1177/000992288502400907
PMID: 4017401
Featured in Collection :   UN Sustainable Development Goals @ Drexel

Abstract

Two infants with clinical and radiologic features of metatropic dwarfism presented in the neonatal period with episodes of cyanosis. Diagnostic studies to determine the etiology of these spells, including electrocardiogram, electroencephalogram, arterial blood gases, and metabolic and sepsis studies, were unremarkable. Chest roentgenograms revealed the characteristic long, narrow thoracic cage with no evidence of parenchymal disease. Cervical spine stability evaluation, pulmonary function studies, and chest impedance monitoring with qualitative air flow thermistor studies and transcutaneous oxygen monitoring were carried out. Both patients demonstrated a significant increase in resistance of the respiratory system following passive maneuvering of the head from a neutral position, suggestive of hypopharyngeal air flow obstruction. Obstructive sleep apnea resulting in cyanosis was documented in both patients. All other studies failed to yield a cause for the episodes of cyanosis. Our investigation failed to alter the clinical course which resulted in respiratory arrest and death by 7 months of age. A table is presented for the differentiation of skeletal dysplasias presenting in the perinatal period.

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4 Record Views
10 citations in Web of Science
11 citations in Scopus

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#3 Good Health and Well-Being

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Web of Science research areas
Pediatrics
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