Pulmonary Complications of Hypogammaglobultnemia

Abstract

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 ...