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An 11-year-old boy with chronic granulomatous disease caused by cytochrome b deficiency developed right upper lung lobe aspergillosis. Intracerebral lesions developed on maximum doses of flucytosine and amphotericin B. Treatment with 16 mg/kg oral itraconazole was followed by a dramatic clinical improvement and almost complete disappearance of the intracerebral lesions. Plasma itraconazole levels were between 40 and 3440 ng/ml depending on concomitant medication. Toxicity was restricted to transient elevation of alkaline phosphatase and gamma glutamyl transferase. We conclude that further trials with itraconazole are justified in high risk patients in whom conventional therapy has failed.

Sera from obligate heterozygotes for deficiency of the C8 beta subunit of the eighth component of human complement (C8) were analyzed for the molecular composition of C8. The C8 alpha-gamma and C8 beta subunits were separated by SDS-PAGE, visualized by immunoblotting, and the resulting bands were quantitated by laser densitometry. The laser densitometric absorption data were set to 100 arbitrary units (AU) for both subunits of pooled normal human sera. The AU values of individual normal sera ranged from 45 to 150 AU for C8 alpha-gamma (median 99 AU) and from 45 to 140 AU for C8 beta (median 101), whereas the C8 alpha-gamma/C8 beta-ratio varied from 0.7 to 1.4. Sera from C8 beta-deficient heterozygotes differed, as expected, from the normal sera for the markedly reduced levels of C8 beta (20 to 90 AU, median 55 AU) and for the higher C8 alpha-gamma/C8 beta-ratio (1.3 to 3.5). High voltage agarose gel electrophoresis was used to separate free and C8 beta-bound C8 alpha-gamma. The migration of free and C8 beta-bound C8 alpha-gamma subunit was checked by hemolytic overlay gels and by second dimension SDS-PAGE and immunoblotting. Immunochemical evaluation of C8 alpha-gamma using this system revealed about 5-14% free C8 alpha-gamma in sera with normal C8 and higher levels, from 33-71%, in the C8 beta D heterozygous sera. Functional analysis confirmed the substantial increase of free C8 alpha-gamma in the heterozygous group. We conclude that the C8 in C8 beta D heterozygous sera is characterized by increased amounts of free C8 alpha-gamma due to reduced concentrations of the C8 beta subunit. This finding may help to identify individuals heterozygous for C8 beta deficiency.

The dilemmas of providing i n t e n s i v e c a r e f o r extremely low b i r t hweight i n f a n t s stem from high m o r t a l i t y and morbidity r a t e s , and t h e high c o s t of s u r v i v a l , both i n human and economic terms. Where resources a r e l i m i t e d , p r e d i c t i o n of outcome e a r l y i n t h e course may h e l p i n determining p r i o r i t i e s . I n t h i s study we used m u l t i p l e l o g i st i c r e g r e s s i o n a n a l y s i s t o determine t h e v a r i a b l e s i n d i c a t i n g s e v e r i t y of i l l n e s s i n t h e f i r s t 24 hours of l i f e which made an independent c o n t r i b u t i o n t o t h e p r e d i c t i o n of s u r v i v a l t o 28 days. The p a t i e n t m a t e r i a l consisted of 106 outborn i n f a n t s of birthweight < 801 g admitted between 1980 and 1984. A t 8 hours of age, birthweight (p= 0.041) and FiO (p=0.002) were independent v a r i a b l e s p r e d i c t i n g survival. At 16 an3 24 hours of age, body temperature (p=0.002 and 0.004). pH ( p 0 . 0 0 2 and 0.047), and presence of spontaneous b r e a t h s (p=0.046 and 0.007) independently p r e d i c t e d s u r v i v a l . A s t a t i s t i c a l model based on t h e method of maximum l i k e l i h o o d was computed on t h e b a s i s of t h e s e v a r i a b l e s . Accuracies of p r e d i c t i o n of s u r v i v a l and of death were i n t h e range 77-83 per c e n t a t each of t h e above ages. A l l 7 i n f a n t s whose p r e d i c t e d chances of s u r v i v a l were 0.5 a t 1 6 hours of age had handicapping sequelae by 3 months of age, o r d i e d between 28 days and 3 months of age. Only 3 of 49 i n f a n t s with chances of surv i v a l 5 0.5 had t h e s e outcomes. I t i s concluded t h a t 1. Use of meas u r e s of s e v e r i t y of i l l n e s s can g i v e p r e d i c t i o n s of s u r v i v a l and of death with about 80% accuracy. 2. Survival "against t h e odds" i s a s s o c i a t e d with severe sequelae of prematurity. Antibodies t o Casein (Ca), Lactalbumin (LA), Lactoglobulin (LG) and Bovine Serum Albumin (BSA) were determined by d i r e c t ELISA a t 4 weeks and a t 24 weeks of age i n premature i n f a n t s . Controls were age matched term i n f a n t s . A t 4 weeks, antibody concentrations were s i g n i f i c a n t l y lower i n preterm i n f a n t s . Although a t 24 weeks t h e response t o a l l 4 antigens was s t i l l lower i n preterm i n f a n t s t h e d i f f e r e n c e was only s i g n i f i c a n t f o r a n t i B5A ( a < 5 %, 'ELISA Units 2cr c 0 . 1 % 3a < 1 5 4a < 5 % Antibody formation i n premature i n f a n t s was found t o be deficient a g a i n s t a l l 4 antigens a t 4 weeks and a g a i n s t BSA even a t 6 months of age. ESPR-Abstracts for 39 MOLECULAR PROPERTIES OF C3 CONVERTING FACTOR (C3 COF)V Wahn, C Rieger, W Muller, U Rother (introduced by H G Lenard). Univ. C h i l d r e n ' s Hosp., Dusseldorf , FRG, Univ. Children's Hosp., Marburg, FRG, Univ. Children's Hosp., Hannover, FRG, I n s t . f o r lnununology and ...

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