Deficiency of laryngeal collagen type II in an infant with respiratory problems
University of
Vienna, Department of Neonatology, Waehringer Guertel 18, A 1090 Vienna, Austria
Correspondence to: Dr Lubec.
Accepted 3 November 1997
A dysmorphic infant is described who presented with laryngeal
collapse leading to intubation and respiratory problems that were
assigned clinically to the Sussman syndrome. The baby had repeated
episodes of respiratory distress necessitating assisted ventilation. At
6 months old, uvulopharyngopalatotomy was done to enlarge the
supraglottic airway without any benefit. Surgical reduction of the
tongue and cricoid splitting did not ameliorate the respiratory
distress; repeated extubation attempts failed with the baby developing
stridor, respiratory distress, and episodes of cardiac arrest. At 10 months old he developed seizures and computed tomography showed diffuse
cerebral atrophy consistent with hypoxic-ischaemic damage. He died at
17 months old. Western blots using antibodies against collagen
1 (II) showed an absence of collagen type II in
laryngeal tissue, which may explain the laryngeal collapse and impaired
respiratory functions.
© 1998 by Archives of Disease in Childhood
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