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Biomedical subjects

V F Larcher

Publications and source records attributed to V F Larcher.

34 records · Page 2Linked to original sources
PubMed1983

Immune responses in patients with obstructive jaundice of infancy. Anti-LSP and Clq binding in sera.

To investigate possible involvement of immune responses in the pathogenesis of obstructive jaundice in infancy we measured antibody to liver specific lipoprotein (LSP) by radio immunoassay and immune complexes by their ability to bind C1q in sera from 16 patients with extrahepatic biliary atresia and 16 with neonatal hepatitis and 13 age matched controls. Anti-LSP was present in 6 of 16 with preoperative biliary atresia and 6 of 16 with hepatitis. Mean percentage C1q bound was higher in hepatitis (22 SD 15%) than preoperative biliary atresia (11.1 SD 2.3%). Nine of 16 hepatitis patients had elevated C1q binding as compared with 1 of 16 with biliary atresia. The highest value for anti-LSP and C1q binding were found in sera from patients with histologically severe hepatitis and hepatitis associated with specific viral or bacterial causes. Anti-LSP was significantly raised 5 months post-operatively in all of 6 patients with biliary atresia and poor biliary drainage but only 2 of 5 survivors. Elevated C1q binding was detected in 6 of 7 with poor drainage and 1 of 7 survivors at the same stage. Anti-LSP and C1q binding fell in 4 patients with neonatal hepatitis on recovery. These findings suggest that immunological mechanisms, possibly involving antibody to hepatocyte membrane components and immune complexes, may be involved in the pathogenesis of progressive liver disease in biliary atresia.

Antigen-Antibody Complex↗
PubMed1983

A common congenital immunodeficiency predisposing to infection and atopy in infancy.

Twenty six infants with a congenital immunodeficiency, characterised by failure of their sera to opsonise heat killed bakers' yeast for phagocytosis by normal polymorphonuclear leucocytes, were studied during infancy to determine the frequency of infection and development of atopy. They were compared with controls, matched prospectively for birth date, sex, parental smoking, and atopy and in whom feeding patterns were similar. In 18 of 26 infants the serum defect persisted at age one year. The incidence of infection and atopy, was appreciably greater in the study group than in controls. The 8 children in whom the defect was transient had a similar incidence of infection but a higher incidence of atopy than controls. Eight of 26 mothers and four of 9 fathers tested also had the serum defect, suggesting a strong genetic component. We support the hypothesis that immunodeficiency predisposes to infection and atopy, and that transient immunodeficiency predisposes to atopy.

Breast Feeding↗
PubMed1983

The prelaparotomy diagnosis of extrahepatic biliary atresia.

The diagnostic accuracy of laboratory investigations in the prelaparotomy differentiation between extrahepatic biliary atresia (EHBA) and intrahepatic disease (IHD) was assessed in 86 consecutive infants presenting with conjugated hyperbilirubinaemia. Forty five infants had EHBA and 41 IHD. The mean serum bilirubin concentration, gamma-glutamyltranspeptidase (GGT) activity, and the GGT/aspartate transaminase (AST) ratio were appreciably higher in infants with EHBA than in those with IHD. In infants with IHD, however, serum bilirubin concentrations were in the EHBA range in 19 (47%), as were GGT values in 29 (71%), and GGT/AST ratios in 33 (80%). In individual patients neither increasing nor decreasing GGT values were of diagnostic importance. Failure of biliary excretion of 99Tcm-p-Butyl-ida occurred in 29 of 30 (97%) patients with EHBA but also in 22 of 23 (67%) with IHD. In all 5 patients with IHD associated with alpha 1 antitrypsin deficiency these 4 investigations gave results in the EHBA range. Liver biopsy specimen interpretation, correct in 38 of 42 infants with EHBA, gave an overall accuracy of diagnosis of 86%: the results of 3 further biopsies were equivocal. In 33 of 40 infants with IHD bile duct obstruction was excluded; the remaining 7, including 4 with alpha 1 antitrypsin deficiency, showed equivocal changes. Faecal excretion of 131I rose bengal faecal excretion was less than 10% in 36 of 37 patients with EHBA and in 9 of 26 with IHD, giving an overall accuracy of diagnosis of 84%. In patients in whom genetic disorders, such as alpha 1 antitrypsin deficiency had been excluded, interpretation of liver biopsy specimens together with 131I rose bengal faecal excretion remain the most accurate means of identifying those who need surgery for EHBA and of avoiding unnecessary laparotomy in infants with IHD.

Aspartate Aminotransferases↗
PubMed1983

Yeast opsonisation and complement in children with liver disease. Analysis of 69 cases.

Opsonisation of heat-killed bakers yeast and C3 and C4 concentrations were determined in sera from 69 children with liver disease and 39 controls as simple screening procedures for abnormalities in the complement system. Where significant defects in these moieties were encountered, the activity of the following was measured: total haemolytic complement, C4, C5, total alternative pathway, factor B and D activities were measured. Complement activation was detected by the presence of C3d in EDTA plasma samples. Yeast opsonisation, C3 and C4 concentrations and activities of all complement components measured were significantly (P less than 0.001) reduced in all of 10 children with fulminant hepatic failure (FHF) and six with chronic active hepatitis (CAH). There was no consistent relationship between complement deficiency and standard tests of liver function. C3 breakdown products were identified in plasma from five patients with CAH and complement deficiency but not in three patients with complement deficiencies associated with FHF. Complement concentration and activity improved in children recovering from FHF and in CAH treated by immunosuppressants. Yeast opsonisation index was raised significantly without parallel increase in C3 or C4 concentration in eight of nine children with biliary atresia before surgery and 11 of 13 with alpha-1-antitrypsin deficiency regardless of age or the presence of active liver disease. Yeast opsonisation indices became normal after successful surgery in patients with biliary atresia.

Adolescent↗
PubMed1982

Defective yeast opsonisation and functional deficiency of complement in sickle cell disease.

Opsonisation of heat-killed baker's yeast, functional activity of the total alternative pathway of complement, and factor B detected functionally and immunochemically were significantly reduced in 72 children with sickle cell disease compared with 40 age-matched black control children. There was significant correlation between functional activity of the total alternative pathway and functionally measured factor B, but not between factor B measured functionally and immunochemically. The opsonisation defect could be corrected in vitro by normal serum, and factor B-depleted serum, and was qualitatively similar to that seen in patients with primary yeast opsonisation deficiency. Serial studies showed that these serum defects were persistent. Reduction in the activity of components of the alternative pathway of complement and opsonisation was found in 4 patients who had recovered from pneumococcal meningitis and in one who developed osteomyelitis. Defects of yeast opsonisation and complement which are common in patients with sickle cell disease, may partly explain the children's increased susceptibility to infection, and might help to identify individuals especially at risk.

Adolescent↗
PubMed1982

Bacterial and fungal infection in children with fulminant hepatic failure: possible role of opsonisation and complement deficiency.

Serious bacterial infection, including eight episodes of bacteraemia, developed in seven of 15 (47%) children with fulminant hepatic failure. Those with infections had a slightly higher leucocyte response than those who did not. Serum immunoglobulin concentrations were normal or raised in all patients. Opsonisation of heat-killed baker's yeast, functionally measured total haemolytic complement, C4, C5, total alternative pathway activity, factor B and D activity, and C3 concentrations were all significantly (p less than 0.005) reduced at presentation but returned to normal in those who survived. The severity of defects in yeast opsonisation, C4, and factor B activity at presentation were significantly correlated with the subsequent development of infection. In five patients bacteraemia occurred at a time when opsonisation and complement components were defective. Plasma infusions in vivo improved opsonisation in vitro and only one bacterial isolate was obtained within four days of such an infusion. Those patients who developed infection had received significantly (p less than 0.05) fewer plasma infusions than those who did not. Our findings suggest that both alternative and classical pathways of complement are defective in children with severe liver disease and may contribute to the susceptibility of such patients to infections. Plasma infusions might be useful in reducing the incidence of bacterial infection in such conditions.

Acute Disease↗
PubMed1982

Yeast opsonization in newborn infants and its relationship to parental atopy.

Sera from 30 of 303 (9.9%) unselected term newborn infants were deficient in their ability to opsonize heat-killed baker's yeasts, an incidence which is almost double that seen in adults. Genetic influence is important in some since the mothers of 10 infants with defective opsonization showed the same defect, but it was not related to the sex or race of the infant or to the atopic state of the parents. In others the defect could be due to a functional maturation delay of the complement system, but not to inhibitory factors in neonatal serum since correction of opsonization was achieved with subopsonizing amounts of normal sera. Significantly more infants had sera with high opsonizing capacity (greater than 80% yeasts phagocytosed) when compared with adults; perhaps antibody independent immune mechanisms like this are important in the newborn. This study shows that a common specific immunodeficiency which may predispose to severe infection or atopy can be identified at birth.

Adult↗
PubMed1981

Hepatic haemangiomata: diagnosis and management.

Five cases of hepatic haemangioma are described, and a sixth (previously reported) is reviewed. Clinical features, investigation, and management are described to show the great variability of the complications and prognosis. Five children presented in the first 10 weeks of life with hepatomegaly; 4 developed congestive cardiac failure; 3 had cutaneous haemangiomata. One child presented at age 4 years with hepatomegaly and anaemia, and on investigation had features of chronic disseminated intravascular coagulation. Focal decrease or patchiness in hepatic uptake of technetium-99m colloid, and abnormal intrahepatic circulation was shown in all cases. In 3 children liver biopsy was performed to exclude malignant disease. In one patient there was spontaneous regression of the tumour by age 3 years. In 3 cases hepatic artery ligation was necessary to control congestive cardiac failure which had persisted despite treatment with digoxin, diuretics, and oral corticosteroids, a procedure which was without complications after up to 8 years. One infant with intractable portal hypertension, hepatic vein obstruction, and severe cholestasis died with persisting alimentary haemorrhage and intra-abdominal sepsis. One child aged 4 years showed no immediate response to hepatic artery ligation but the size of her tumour got smaller and the clinical features diminished after irradiation. These tumours cause considerable morbidity and have a high reported mortality. If congestive cardiac failure is not rapidly controlled, hepatic artery ligation should be performed.

Child, Preschool↗
PubMed1981

Mechanism of the serum defect in yeast opsonization in children with fulminant hepatic failure (FHF).

Defective opsonization of heat-killed baker's yeast was found in all 14 children with fulminant hepatic failure (FHF) but returned to normal in two who recovered and was normal in six mothers of patients. Yeast opsonization was significantly correlated with factor B activity of FHF serum but not with other components of classical or alternative pathways of complement. Reconstitution and family studies suggested that defective yeast opsonization in FHF is secondary and, although dependent on factor B activity, is qualitatively and quantitatively similar to that of primary yeast opsonization deficiency. These findings suggest that the factors responsible for opsonization of yeasts, or their regulation, are synthesized or controlled by the liver.

Child↗
PubMed1980

Macroamylasaemia and hepatitis in a twelve-year-old boy.

Macroamylasaemia was found in a 12-year-old boy with persistent, severe abdominal pain and mild hepatitis. No abnormality of the pancreatic or bile ducts was demonstrated. Both the symptoms and the biochemical abnormality of macroamylasaemia settled spontaneously over 10 months. Transient, asymptomatic macroamylasaemia was found in the boy's father.

Age Factors↗
PubMed1980

Yeast opsonisation in children with chronic diarrhoeal states.

Four patients with defective yeast opsonisation and protracted diarrhoea are reported. Plasma infusions improved the opsonising function in all 4 and the diarrhoea in 3. This immunological abnormality was assessed in 100 sequential patients with chronic diarrhoea associated with various gastrointestinal disorders; 52 with protracted diarrhoea and failure to thrive of undetermined cause, 26 with 'toddler diarrhoea', 8 with coeliac disease, 5 with chronic inflammatory bowel disease, and 9 with miscellaneous disorders. 23% of the patients with protracted diarrhoea of undetermined cause had defective opsonisation, a greater proportion (P less than 0.05) than that in 'toddler diarrhoea' or the remaining patients, in whom the frequency (4%) was similar to that (5%) in healthy populations. We suggest that yeast opsonisation be tested in children with protracted diarrhoea, as plasma infusions can be an effective form of treatment.

Child, Preschool↗
PubMed1978

Gluten challenge in treated coeliac disease.

Thirty-two children with a past diagnosis of coeliac disease were reinvestigated by means of gluten challenge. They had received a gluten-free diet for a mean period of 6-5 years (range 0-25-11-0) before gluten challenge, and had a mean age of 9-9 years (range 3-0-15-3) at the onset of challenge. Small intestinal biopsies were performed before and after the gluten challenge (at least 10 g natural gluten per day for 3 months in the majority) and, as judged by light microscopy and morphometric techniques, coeliac disease was confirmed in 25 (78%) of the 32 patients; challenge was continued in the remaining 7 for a total period of 2 years when biopsies were repeated in 6 children and all were normal. Serum and RBC folate, serum iron, Hb level, and clinical symptoms were unpredictable variables in identifying children who developed gluten-induced enteropathies. The social and emotional effects of gluten challenge are discussed. The results highlight the importance of gluten challenge and intestinal biopsy in the diagnosis of coeliac disease and strongly suggest that a normal biopsy after a 3-month gluten challenge (as described above) excludes coeliac disease.

Adolescent↗
PubMed1978

Theophylline toxicity in a neonate.

One of a pair of preterm twins treated with theophylline (2 mg/kg per day) for apnoea developed episodes of hyperglycaemia in glycosuria.

Apnea↗
PubMed1977

Protracted diarrhoea in infancy. Analysis of 82 cases with particular reference to diagnosis and management.

Eighty-two cases of protracted diarrhoea in infancy presenting over a 6-year period have been analysed, with particular reference to diagnosis and management. The patients fell into 1 of 2 categories according to whether a specific diagnosis was established or not. A diagnosis (category 1) was established in 59 (72%), the commonest diagnoses being coeliac disease (33-2%), secondary disaccharide intolerance (12-2%), and cows' milk protein intolerance (12-3%). Other diagnoses included primary sucrase-isomaltase deficiency, Shwachman's syndrome, ulcerative colitis, ganglioneuroma, defective opsonization, staphylococcal pneumonia, and Hirschsprung's disease. Despite intensive investigation in diagnosis could not be established in 23 (28%) infants (category 2). Age of onset of symptoms in this group tended to be earlier than in category 1 patients, and 6 (7%) presented with diarrhoea dating from birth. Of particular interest in these 6 patients was the high incidence of associated extraintestinal anomalies, and of sibs who had died after protracted diarrhoea dating from birth. 4 of these 6 infants died, accounting for a mortality of 5% for the whole series. The remaining 17 (21%) patients in category 2 presented at a mean age of 4-9 weeks with a range of 1-18 weeks. All these 17 patients made an excellent response after institution of a chicken-based dietary formula, the details of which are presented. The pathophysiological mechanisms which may be operating in infants with protracted diarrhoea are discussed.

Animals↗