A 4-year-old boy with a short history of jaundice and hepatomegaly was studied by endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). A large obstructing choledochal tumour was found at the level of cystic duct insertion and at surgery, a botryoid sarcoma was removed. Subsequent cytotoxic therapy was complicated by persistent dilatation of the extrahepatic bile ducts which necessitated eventual excision. There was no evidence of persistent tumour.
's College H o s p i t a l 3-akivary.cdffeine c l e a r a n c e (SCC) can be considered a measure of h e p a t i c m i c r o s o m a l enzyme a c t i v i t y . ~t may t h u s p r o v i d e a q u a n t i t a t i v e a s s e s s m e n t of h e p a t i c f u n c t i o n . T o a s s e s s t h i s we have s t u d i e d 20 c h i l d r e n w i t h a v a r i e t y of l i v e r d i s o r d e r s ; 8 hadp i r r h o s i s w i t h s e r u m a l b u m i n (ALB) < 3 5 g l l . C a f f e i n e (3mgIkg) w a s a d m i n i s t e r e d o r a l l y f o l l o w i n g an o v e r n i g h t f a s t . S a l i v a r y \ c a f f e i n e c o n c e n t r a t i o n was m e a s u r e d by HPLC a t 0,1,4,6,12,24 hr. C a f f e i n e s a l i v a h a l f l i f e ( t ) was c a l c u l a t e d from SCC. t + i n n o r m a l c o n t r o l s r a n g e d from 4.2 t o 5.4 h r . t t i n l i v e r d i s e a s e from 4.4 t o 56 hr ; a s i g n i f i c a n t c o r r e l a t i o n was obtained between t and ALB (r=-0.65). T h e r e were a l s o s i g n i f i c a n t c o r r e l a t i o n s f wlth prothrombin time (PT) and t o t a l serum b i l i r u b i n ( r = 0.65 and 0.84 r e s p e c t i v e l y ) ; t h e r e was no r e l a t i o n s h i p between t t and any o t h e r l i v e r f u n c t i o n t e s t . However, t h e mean PT and t + were s i g n i f i c a n t l y higher in the 8 c i r r h o t i c c h i l d r e n when compared t o the o t h e r c h i l d r e n . I n two c h i l d r e n , one w i t h b i l i a r y c i r r h o s i s and t h e o t h e r with a l p h a -1 -a n t i t r y p s i n , SCC improved d r a m a t i c a l l y following o r t h o p t i c l i v e r t r a n s p l a n t a t i o n , tt before / a f t e r ; 4 1 t o 5.5 h r ,9.6 t o 5.4 h r , r e s p e c t i v e l y . I n o n e c h i l d w i t h W i l s o n ' s d i s e a s e t changed f r o m 56 t o 7.0 hr f o l l o w i n g t r e a t m e n t . We conclude t k a t the SCC may permit q u a n t i f i c a t i o n of l i v e r f u n c t i o n i n a fashion not p o s s i b l e w i t h s t a n d a r s e s s t h e frequency of s i g n i f i c a n t c o n g e n i t a l anomalies b u t s i d e t h e h e p a t o b i l i a r y system & t h e i r e f f e c t on management., we have r e t r o s p e c t i v e l y analysed t h e c a s e r e c o r d s of 237 c a s e s seenbetween June '70 & J u n e '85. Major anomalies occurred i n 47 (20%) I27 having more thim one, 16 had one o r more c a r d i a c anomalies (Pulmonary s t e n o s i s 7 , VSD 5 , ASD 3 , PDA3, o t h e r s 7 ) r e q u i r i n g spec i f i c i n v e s t i g a t i o n which sometimes delayed b i l i a r y s u r g e r y . 0 p e r a tkve management was complicated i n 3 w i t h i n t e s t i n a l a t r e s i a r e -F e q u i r i n g p r i o r s u r g e r y & a f u r t h e r 16 had i n t e s t i n a l m n l r o t a t i o n . h7 (7%) had p o l y s p l e n i a & 2 a s p l e n i a . I n 1 0 of t h e s e t h e p o r t a l t e i n was pre-duodenal. 9 had abdominal s i t u s i n v e r s u s making d i sg e c t i o n & i d e n t i f i c a t i o n o f s ...
Research, Washington DC, USA. Shigella infections are characterized by invasion of epithelial surfaces. A 140 megadalton plasmid was shown to be responsible for the expression of virulence. This plasmid encodes several outer membrane proteins, which are absent in the plasmid free avirulent organisms. As invasion does not occur in immune subjects, it seemed of interest to study the immune response to the plasmid coded proteins (PCP), and to compare it with the response to the somatic antigen, lipopolysaccharide (LPS).Five monkeys were infected by a S. flexneri 2a strain. Blood was drawn at 0,7,14,30 days post infection. Rectal biopsies were taken at the same time, and cultured for 24 hrs. Blood and culture media immunoglobulins were measured and characterized by ELISA and immunoblotting techniques.PCP were found to be of major importance in the immune response IgG serum antibody to PCP reached a higher titer than that to LP$ and persisted longer. A similar IgA and IgG response was found in the intestine. Four PCP protei~ls were recognized by most animals, as determined by immunoblotting. This new information suggests that the PCP serve a major role in the host response to infection, and deserve further studies to clarify their role in the pathogenesis of shigellosis. Mucosal DNA levels paralleled these changes. A subsequent study demnstrated rapid reversal of impairment of mcosal hyperplasia following reinstitution of EFA containing diets. Hepatic triene:tetraene ratios confirmed EFA deficiency in the palmitic acid group. EFA deficiency impaired mcosal adaptation following resection. This effect was reversible, and may explain stimlatory effects of long chain triglycerides on mcosal hyperplasia. DIFFERING IMMUNE MECHANISMS TRIGGERING AUTOIMMUNTTY IN PRIMARY SCLEROSING CEOLANGITIS (PSC) AND AUTOIMMUNB CHRONIC ACTIVE HEPATITIS (aCAH) OF CHILDHOODG.Mieli-Vergani,A.Lobo-Yeo,AP Mowat, D.Vergani.Depts. Child Health 5 Immunology, King's College Hospital London. PSC histological features may resemble those of aCAH and increased autoantibody and IgC levels are found in both conditions. PSC, however, is less responsive to innunesuppressants. To investigate whether this derives from differences in regulatory and/or effector immune mechanisms we have studied 7 children with PSC, 14 with aCAH and 26 healthy children as controls. Lymphocyte cytotoxicity values to autologous hepatocytes were similarly elevated in 5 children with PSC (meankSD, 50i9b) and in 7 with kCAH (53k168) studied. In contrast, T suppressor number and Con-A induced function were normal in patients with PSC (23.5+3.1% and 50.8t5.3%), but siDificantly decreased in children with aCAH (15.8+4.7% and 7.6i6.53, when compared to controls (24.2?5.4%, pt0.02 and 51.7k4.0, pt0.01 Rank Test). In 7 PSC patients percentages of activated T cells expressing HLA-DR (4.4i1.22) or IL2r (0.2k0.3%) were similar to controls (2.92k0.396 and 0.14i 0.206) while significantly increased numbers of both were found in 14 aCAH patients (6.3i2.7, pt0.05 and 18.0k3.2 ptO.OO1). Our dat...
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