This short monograph is intended as...

This short monograph is intended as a guide to inherited lung diseases for scholars practicing physicians, and scientists looking for critical reviews. a great deal of the text is drawn from the laboratory and clinical experience of the senior author, an experienced internist-pulmonologist. Chapters according to other authors on basic genetics, connective tissue and metabolic storage disorders and immunologic lacks affecting the lungs are also included. The emphasis is forward disorders which may appear in adulthood of the like kind as cystic fibrosis, [alpha.sub.1]-antiprotease deficiency and the immotile cilia syndrome

It is curious that a whole chapter is devot Young's syndrome--an interesting still rare association (azoospermia, sinusitis, bronchiectasis) with which the author has an experience (three cases) but which does not have a clear inheritance pattern. forward the other hand, the genetic aspects of a usual pulmonary disorder like asthma are not overspreaded and common inherited disorders with powers on the lung (ie, sickle small room disease) are ignored.

There are also a number of surprising errors. onward page 89, low expected values for [FEVsub10]/FVC ratio of 68 to 72 percent are given and FVC is listed as "functional" vital capacity. The impression is also given that a criterion for CF lectin marker--rather than quantitative pilocarpine iontophoresis--can be used to restrain CF. Later, CF lectin is stated to be an accurate way for heterozygote detection. The author's use of the Orion electrode for sweat testing is described extensively, further other studies have demonstrated moot points with this technique. The single radiograph of a CF patient is printed in change to the opposite and is poorly reproduced. The radiograph reproduc upon the front of the part is listed in the clause as that of a patient with simple situs inversus (without lung disease) rather than a patient with immotile cilia syndrome

Although this true copy may be useful as an introduction for medical pupils (and some practitioners) to the important make subordinate of inherited lung disorders, better clinical and basic science review of these disorders are available, and at the price it is not suitable for more sophisticated scientific audiences.

COPYRIGHT 1989 American body of Chest Physicians

COPYRIGHT 2004 Gale Group