ADULT RESPIRATORY DISTRESS SYNDROME (ARDS) IN A CASE
OF ATAXIA TELANGIECTASIA (AT) (CASE REPORT)
Maalawi MY.,
Mohammed A.; Mahfouz A.E and Abdel Aziz S.,
Al Mowasat
Hospital, Dammam, KSA
Objective:
To highlight to possibility of occurrence of ARDS in cases of AT. To
differentiate between ARDS and bronchiolitis obliterans which is a
characteristic finding in AT and to start adequate management by using high
PEEP and recent pharmacological strategies.
Methods: The
case was diagnosed by thorough history, family pedigree and clinical
manifestations. Immunoglobin levels, alpha feto protein assay and
karyotyping were done. Computed Tomography (CT) of the brain and chest,
CT-guided lung biopsy confirmed the diagnosis.
Results: The
characteristic clinical manifestations will be presented by figures.
Significant laboratory results were lymphopenia, a low level of IgA:19
mg/dl and a very high level of Alpha fetoprotein: 194 lu/ml.
CT scan of
the brain showed cerebellar atrophy. CT scan of the chest revealed mediastinal
lymph node enlargement. Diffuse alveolar opacities and pulmonary
consolidation, CT guided lung biopsy and its histopathologic examination
revealed the features of resolving diffuse alveolar damage suggestive of
resolving ARDS.
All
radiological and histological pictures will be presented by figures.
Conclusion:
Pulmonary disease in AT is common in the form of and pulmonary
infections by mycoplasma pneumoniae, cytomegalovirus and pseudomonas
aeroginosa. Our case showed ARDS which was diagnosed and proved by lung
biopsy, early diagnosis lead to adequate management by special ventilatory
support and recent pharmacologic agents.