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To evaluate malnutrition in chronic liver disease, and its relationship to nutrient deficiencies and hepatic dysfunction, 27 children with end-stage liver disease were studied. Mean protein-energy intakes were 70% of recommended daily intakes. The patients were underweight and stunted with reduced mean triceps and subscapular skinfold thicknesses and midupper arm circumference. Mean total body potassium was only 63 +/- 18% of that expected for age and sex. Deficiency of essential fatty acids (32%), and low concentrations of fat-soluble vitamins (A, 92%; E, 32%), iron (32%), zinc (42%), and selenium (13%) were common. Serum ammonia concentrations were raised in all patients, and increased methionine, tyrosine, and glutamic acid, and reduced glutamine concentrations were noted. There was no correlation between the degree of malnutrition and the degree of liver synthetic function, the degree of cholestasis, or the degree of liver injury. We suggest that potentially correctable factors in addition to liver failure (eg, inadequate absorbed intake) were important determinants of malnutrition in these patients.

Plant aerial surfaces comprise a complex habitat for microorganisms, and many plant-associated bacteria, such as the pathogen Pseudomonas syringae, exhibit density-dependent survival on leaves by utilizing quorum sensing (QS). QS is often mediated by diffusible signals called N-acyl-homoserine lactones (AHLs), and P. syringae utilizes N-3-oxo-hexanoyl-dl-homoserine lactone (3OC6HSL) to control traits influencing epiphytic fitness and virulence. The P. syringae pathovar syringae B728a genome sequence revealed two putative AHL acylases, termed HacA (Psyr_1971) and HacB (Psyr_4858), which are N-terminal nucleophile hydrolases that inactivate AHLs by cleaving their amide bonds. HacA is a secreted AHL acylase that degrades only long-chain (C > or = 8) AHLs, while HacB is not secreted and degrades all tested AHLs. Targeted disruptions of hacA, hacB, and hacA and hacB together do not alter endogenous 3OC6HSL levels under the tested conditions. Surprisingly, targeted disruptions of hacA alone and hacA and hacB together confer complementable phenotypes that are very similar to autoaggregative phenotypes seen in other species. While AHL acylases might enable P. syringae B728a to degrade signals of competing species and block expression of their QS-dependent traits, these enzymes also play fundamental roles in biofilm formation.

which reliable administration of phototherapy cannot This study represents a multicenter survey on the be guaranteed. (HEPATOLOGY 1996;24:311-315.) management of patients with Crigler-Najjar syndrome (CNS) type 1. The aim of the survey was to find guiding principles for physicians in the care of these patients. Fifty-seven patients were included. At the time of incluCrigler-Najjar syndrome (CNS) is characterized by unconsion, 21 patients had received a liver transplant (37%). jugated hyperbilirubinemia since birth.1 CNS type 1 results The average age at transplantation was 9.1 { 6.9 years from a complete deficiency of the enzyme bilirubin UDP-(range, 1-23 years); the age of the patients who had not glucuronosyltransferase (UGT), and CNS type 2 results from been transplanted at the time of inclusion was 6.9 { 6.0 a partial deficiency. Bilirubin UGT is a member of the UGT years (range, 0-23 years). Brain damage had developed family, a group of enzymes encoded by the UGT1 and UGT2 in 15 patients (26%). Five patients died, and 10 are alive genes on human chromosomes 2 and 4. UGTs are located in with some degree of mental or physical handicap. In 2 the endoplasmic reticulum of the hepatocyte. UGT isoforms patients, ages 22 and 23 years, early signs of bilirubin are present in various other organs such as intestine, kidney, encephalopathy could be reversed, in 1 by prompt medi-lungs, and adrenals. 3-5 The after retransplantation, 2 improved neurologically, and first report was in 1952 by Crigler and Najjar.1 Before the 4 remained neurologically impaired. The age of 8 pa-advent of phototherapy, the disease was lethal, with death tients with and 13 without brain damage at or before occurring by the age of 15 months. transplantation was 14.3 { 5.9 and 5.9 { 5.4 years (P In CNS type 1, serum bilirubin values of untreated patients õ .01), respectively. Therapy of CNS type 1 consists of are in excess of 350 mmol/L, and bile contains not more than phototherapy (12 h/d), followed by liver transplantation. traces of bilirubin conjugates. 6,7 Kernicterus is a complication Phototherapy, although initially very effective, is so-that can also develop later in life.1,8,9 CNS type 2 is less secially inconvenient and becomes less efficient in the vere, with serum bilirubin values usually not exceeding 350 older age group, thus also decreasing compliance. Cur-mmol/L. The bile of these patients contains bilirubin monorently, liver transplantation is the only effective ther-and diglucuronides in low concentration.6,7 Their serum biliapy. This survey shows that, in a significant number of rubin responds to phenobarbital treatment with a decrease patients, liver transplantation is performed after some by 30% or more.10 Genetically, both diseases result from muform of brain damage has already occurred. From this, tations in the exons of the UGT1 gene.2 CNS types 1 and one must conclude that liver transplantation should be 2 are autosomal recessive diseases because both alleles are performed at a young age, particularly in situations in aff...

T-phylloplanin proteins secreted to aerial surfaces of tobacco (Nicotiana tabacum) by short procumbent trichomes inhibit spore germination and blue mold disease caused by the oomycete pathogen Peronospora tabacina. Many other plants were found to contain water-washed leaf surface proteins (phylloplanins), but the functions and properties of these are not known. Here we extend earlier evidence for the antifungal activity of T-phylloplanins using a reverse genetics approach. RNA interference of the T-phylloplanin gene in tobacco 'T.I. 1068' resulted in loss of T-phylloplanin mRNA and protein, loss of in vitro spore germination inhibition activity, and leaf infection inhibition activity of leaf water washes from RNA interference plants, and young knockdown plants were susceptible to disease. The glycoprotein character, adaxial-leaf-surface enrichment of, and renewability of T-phylloplanins are also described. We also report that leaf water washes of sunflower (Helianthus annuus) and jimson weed (Datura metel), but not soybean (Glycine max), like that of tobacco, possess ProteinaseK-and boiling-sensitive P. tabacina spore germination and tobacco leaf infection inhibition activities. Results establish that T-phylloplaninins of tobacco are active in P. tabacina inhibition, and indicate that leaf surface proteins of certain non-Nicotiana species that are not susceptible to P. tabacina disease can inhibit germination of spores of this oomycete pathogen and inhibit tobacco leaf infection by this pathogen.

Techniques are described for the isolation, microculture and assessment of viability of Giardia intestinalis. The susceptibility of five recent Brisbane isolates and the Portland 1 stock to metronidazole, tinidazole, furazolidone and quinacrine has been determined. All stocks showed little variation in sensitivity to these drugs. The 3H-thymidine incorporation assay proved to be a much more sensitive method of assessing relative drug activity than either motility or dye exclusion methods. Tinidazole proved to be the most effective drug in vitro followed by furazolidone and metronidazole. Quinacrine was the least effective. The generation time of stocks in vitro varied from 12.5 to 44.2 h and the possible significance of this to failures in treatment is discussed.

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