I have just seen a 26 y/o patient in the office for recurrent respiratory tract infections. He has a Hx of prune-belly syndrome (poor abdominal muscle development with severe urinary tract abnormalities) and has been on HD after a failed renal Txp.
These patients tend to have a restrictive physiology because of the oligohydramnios and sometimes even have hypoplastic lung. Indeed his TLC is ~66%. His Cxr is pretty unremarkable but he also has an associated obstructive defect: FEV1 is ~36% witha ratio of<70%. Has anybody seen this condition (or similar ones) be associated with asthma/reactive airways disease?
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Never heard of this before, and if it were just the absence of abdominal musculature, one would predict hyperinflation--so hypoplastic lung would be a possibility. Any chest wall deformities?
As to the obstruction, again I think the lack of abdominal musculature could impact the forced expiration, resulting in decreased flows.
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