. Cardiovasch I n s t i t u t e , Univ. of C a l i f o r n i a , San Francisco, Ca. We have developed an i n t e r c o s t a l r e t r a c t o m e t e r f o r t h e nonnvasive e s t i m a t i o n of i n t r a p l e u r a l p r e s s u r e . I t c o n s i s t s of a cored l u c i t e d i s c covered by a t h i n a c e t a t e membrane. The scord l i n e s and t h e membrane w i t h t h e d i s c form a small space which s f i l l e d w i t h l i q u i d . The membrane i s a t t a c h e d t o t h e s k i n and ny r e t r a c t i v e f o r c e a p p l i e d t o t h e device by t h e i n t e r c o s t a lpace is t r a n s l a t e d i n t o p r e s s u r e changes i n t h e l i q u i d , detected y a 1' 50A Statham s t r a i n guage.Twelve s t u d i e s were performed i n 3 i n f a n t s with h y a l i n e memrane d i s e a s e , averaging 1180 g weight, and 30 wks G.A. Comparion w i t h estimated i n t r a p l e u r a l p r e s s u r e was made using an esohageal b a l l o o n placed i n t h e mid-thorax.The r e t r a c t o m e t e r was a l i b r a t e d by measuring t h e occluded airway p r e s s u r e , which ollowed changes i n esophageal p r e s s u r e t o w i t h i n ? 1 cm H20 over range of 30 cm H20. This c a l i b r a t i o n method a l s o allows f o r he d e t e c t i o n of a r t i f a c t s due t o i n a p p r o p r i a t e p o s i t i o n i n g of he device. The r e g r e s s i o n l i n e of r e t r a c t o m e t e r output vs esohageal p r e s s u r e had an average s l o p e of 0.4 and p r e d i c t e d t h e sophageal p r e s s u r e t o w i t h i n ? 1.7 cm Hz0 (95% confidence l i m i t ) I n using t h e device, c a r e must be taken t o e l i m i n a t e a r t i f a c t s ue t o movement of t h e instrument and f a u l t y attachment t o t h e h e s t w a l l . The response of t h e device i s a t t e n u a t e d by subcutneous t i s s u e , roughly i n proportion t o t h e t h i c k n e s s of t h e i s s u e . (Supported i n p a r t by Contract HR 6-2093) VALUATION OF ANTIBIOTIC THERAPY I N PATIENTS WITH CF.; Braverman, S h i r l e y ; Laraya-Cuasay , Helen; Yasmin, Nasira Temple Univ. o f P e d i a t r i c s , S t . C h r i s t o p h e r ' s Hosp i t a l f o r Children, P h i l a d e l p h i a . Pa. 19133 The e f f e c t of secondary b e c t e r i a l i n f e c t i o n s of t h e bronchopulmonary system on p a t i e n t s with CF has been f i r m l y e s t a b l i s h e d and i t has been p o s s i b l e t o p a r t i a l l y c o n t r o l t h e s e i n f e c t i o n s b aggressive b r o n c h i a l drainage and e n e r g e t i c a n t i b i o t i c t h e r a p y . The t h e r a p e u t i c e f f e c t of t h e s e m o d a l i t i e s of treatment has not been evaluated q u a n t i t a t i v e l y .This study attempts t o measure t h e e f f i c a c y o f amikacin with c a r b e n i c i l l i n i n 15 p a t i e n t s (median age 14 y r s ) with pseudomona i n f e c t i o n . Subjects were scored on t h e s e v e r i t y of d i s e a s e acco r d i n g t o parameters i n c l i n i c a l ...

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A 2 1/2 y e a r old male child(D.E.) with multiple congenital anomalies, severe psychomotor retardation, generalizedseizures, chroni c herpetic gingivostomatitis, overwhelming pneumococcal meningitis and sepsis was found t o have a partial combined immunodeficiency.His female sibling(J.E.) who followed a similar course and who died a t 4 1/2 y e a r s of age was found t o have sudanophilic leukodystrophy.During the course of his hospitalization, D. E. developed intractable diarrhea and was noted to have a decreased lymphoproliferative r esponse to PHA, decreased T-cell rosette formation, a persistent lymphopenia and normal erythrocyte adenine deaminase (ADA) activity. Immunoglobulin therapy and parenteral hyperalimentation failed t o alter his clinical course. Following transplantation of a 16-week fetal thymus enclosed i n a millipore chamber there was prompt clearing of the herpetic lesions and cessation of the diarrhea within a few days after transplantation and a n appearance of lymphocyte responsiveness to PHA one month Iater. Nine months after transplantation the patient remains infection-free and continues t o display an intact cellular immunity. This r e p o r t describes a previously unrecognized presentation of combined immune deficiency and sugg e s t s a new approach t o therapy. SERUM I g E LEVELS IN PARENTS AND CHILDREN. J o h n W.G e r r a r d , S a n d r a H o r n e , P e g g y V i c k e r s , J o h n W. Mac-K e n z i e , N a t h a n G o l u b o f f , J o h n Z . G a r s o n , C a r l o s S. M a n i n g a s . C o l . o f M e d i c i n e , U n i v . o f S a s k a t c h e w a n , D e p t . o f P e d i a t r i c s , U n i v e r s i t y H o s p . , S a s k a t o o n , S a s k . , C a n a d a .S e r u m I g E l e v e l s h a v e b e e n s t u d i e d i n 8 0 f a m i lies. L e v e l s less t h a n 1 5 0 u/ml w e r e c o n s i d e r e d l o w , l e v e l s o f 1 5 0 u/ml o r above w e r e c o n s i d e r e d h i g h . F i f t y -o n e m a t i n g s w e r e b e t w e e n p a r e n t s w i t h l o w l e v e l s o f I g E ; 1 9 % o f t h e i r o f f s p r i n g h a d h i g h l e ve l s . T w e n t y -t w o m a t i n g s w e r e b e t w e e n p a r e n t s o n e o f whom h a d a l o w l e v e l o f I g E a n d o n e o f whom h a d a h i g h l e v e l ; a p p r o x i m a t e l y h a l f ( 4 3 % ) o f t h e i r c h i l d r e n h a d h i g h l e v e l s . S e v e n o f t h e m a t i n g s w e r e b e t w e e n p a r e n t s w i t h h i g h l e v e l s , a p p r o x i m a t e l y t h r e e -q u a r t e r s ( 7 7 % ) o f t h e i r c h i l d r e n h a d h i g h l e ve l s . T h e r e s u l t s are c o n s i s t e n t w i t h l o w I g E l e v e l s b e i n g d e t e r m i n e d b y t h e p r e s e n c e o f t w o d o m i n a n t g e n e s , t h e a b s e n c e o f o n e o r t h e o t h e r p e r m i t t i n g h i g h l e v e l s t o occur. T h e u l t i m a t e l e v e l s o f I g E are p r o b a b l y a l s o i n f l u e n c e d b y a g e a n d e n v i r o nm e n t a l f a c t o r s s u c h as e x p o s u r e t ...