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Medical mystery
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Medical mystery

My 10 yr old underwent  8th sinus surgery!  Infection was bilateral in sphenoid, frontal,ethmoid, and maxillary.  Polyps also removed. Contiues to have chronic sinus problems along with "mild asthma".  Two sweat chloride tests  and blood test (-).   Blood work  shows low t-cell functions specifically CD4 and CD8.  Her titers are low.  Pneunonoccal ( responded after recieved booster) now her tetnus and diptheria  low ( waiting to see if booster worked.)  Because IGG,IGA, levels  normal  have been told no immune decifiency. Alleregy tests (-).Several episodes this year fever 104 and wheezing.  The chest films are negative for pneumonia.  Two HRCT chest films without contrast have these findings....Tracheobronchial structures show mild prominence of bronchial diameter with mild central wall thickening. However, the bronchial structures do not meet the minimum critera for bronchiectasis as the are smaller than the adjacent pulmonary arterioles.  2nd HRCT had this finding...Minimal linear opacities at periphery of bilateral lower lobes on supine images, which resolve on prone images, reflects subsegmental atelectasis.  Ligamentum arteriosum is incidentally calcified.  A few non radiographically enlarged bilateral axillary lymph nodes are present.   A biopsy was done last year (during her 7th sinus surgery that was non resposive after 4 months of high dose antibiotics ( Augmenten ES 1875mg)  to rule out Ciliary Dyskenisia.  This biopsy  I was told was negative and now am being told by new ENT that it was not a YES or NO for Ciliary Dyskenisia.  Here is what the report said..  The rhinoprobe scrapings are cellular but contain no cells with preserved cilia. Neutrophila and lymphocytes are present.  The carina biopsy is well oriented but the surfase mucosa consists mainly of METAPLASTIC SQUAMOUS EPITHELIUM.. No intact cilia are present.  Unable to perform ultrastructural examination due to metaplastic change of the carina mucosa.

Can you help me understand any of this?
242588_tn?1224275300
It sounds like cystic fibrosis (CF) has been ruled out as the cause of your daughter’s symptoms based on the results of the 2 sweat chloride tests and the CF DNA blood test.  What is less clear is her immunologic status.  Without reviewing her immune work up in detail, it is difficult to know if an immune deficiency has been adequately addressed.  The other issue is whether she might have a ciliary defect.  The biopsy to address this was inadequate.  Thus, it sounds as if her immune state and the functioning of her cilia are still areas that may need attention.

We recommend that you seek a second opinion, either at National Jewish Medical and Research Center or a comparable institution.
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