Document Detail


Respiratory aspects of Shwachman's syndrome in adults.
MedLine Citation:
PMID:  2731608     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Shwachman's Syndrome is a rare disorder which causes considerable morbidity in childhood and can be confused with cystic fibrosis. Six patients (4 males; mean age 27 yrs) are described in order to illustrate the clinical picture of the syndrome in adults. Recognized features persisting in later life include pancreatic insufficiency and bone marrow dysfunction (all patients), short stature (5/6), advanced dental caries (4/6), and impaired glucose tolerance (2/6). Chest radiograph was normal in all patients whilst lung function tests showed mild restriction in three and obstruction in two. All patients were cyclically neutropenic (nadir count 2.1 x 10(9).l-1). Four of the six patients were thrombocytopenic and three had persistent immunoglobulin deficiencies. Neutrophil chemotaxis was abnormal in four patients and the nitro blue tetrazolium test was abnormal in a fifth. Two patients suffered from recurrent respiratory infections, one of whom died from bone marrow aplasia. Although Shwachman's Syndrome may be less troublesome in adults than in children, many of the abnormalities persist into adult life and may continue to cause diagnostic difficulty.
Authors:
J Wiggins; D M Geddes
Related Documents :
50628 - Solitary myeloma. a study of 10 black patients during an 8-year period.
18843778 - Circulating osteoprotegerin and soluble rank ligand in systemic sclerosis.
9042988 - Long-term effect of gluten restriction on bone mineral density of patients with coeliac...
2365718 - Orthopaedic manifestations of blastomycosis.
834968 - Double contrast x-ray examination of stomach.
7557098 - Hypoaminotransferasemia in patients undergoing long-term hemodialysis: clinical and bio...
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The European respiratory journal     Volume:  2     ISSN:  0903-1936     ISO Abbreviation:  Eur. Respir. J.     Publication Date:  1989 Mar 
Date Detail:
Created Date:  1989-07-18     Completed Date:  1989-07-18     Revised Date:  2013-05-23    
Medline Journal Info:
Nlm Unique ID:  8803460     Medline TA:  Eur Respir J     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  285-8     Citation Subset:  IM    
Affiliation:
Brompton Hospital, London.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adult
Body Height
Bone Marrow Diseases / physiopathology*
Female
Humans
Male
Pancreatic Diseases / physiopathology*
Respiration*
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Insufficient oxygen concentration obtained at domiciliary controls of eighteen concentrators.
Next Document:  Is expressed emotion an index of a transactional process? II. Patient's coping style.