Biomedical subjects

H Gaze

Publications and source records attributed to H Gaze.

At least 19 recordsLinked to original sources

PubMed1991

Antigliadin and antiendomysium antibody determination for coeliac disease.

The value of IgG and IgA gliadin antibodies (AGA) was compared with that of IgA endomysium antibodies (EMA) for the diagnosis of coeliac disease. Three hundred and six of 340 (90%) children with untreated coeliac disease (flat mucosa) had EMA and 338/340 (99.4%) had IgG AGA and/or IgA AGA. Only 1/340 (a 7 year old boy with selective IgA deficiency) had neither AGA nor EMA. Absence of EMA is more frequent in coeliac patients younger than 2 years than in older patients (32/277 compared with 1/62). EMA were present in 4/211 (2%) of comparison subjects (normal mucosa), IgA AGA in 12/211 (6%), and IgG AGA in 74/211 (35%). The specificity of AGA cannot be calculated from these figures as they are biased. The combined determination of AGA and EMA, taking advantage of the high sensitivity of AGA and the high specificity of EMA, gives an excellent prediction of the condition of the mucosa: 247/248 patients (99.6%) with positive EMA and positive IgG AGA and IgA AGA had a flat mucosa, whereas 136/137 patients (99.3%) with neither AGA nor EMA had a normal mucosa. During a gluten free diet EMA and AGA disappear. Their presence or absence is therefore an indicator of dietary compliance. After reintroduction of gluten into the diet 110/134 (82%) of the patients who had a flat mucosa at diagnosis relapsed, but 24/134 still had a normal mucosa after 2-15 years of challenge. All these patients without a morphological relapse were less than 2 years old at diagnosis so we conclude that patients who are young at diagnosis should be challenged. AGA often reappear earlier than EMA. After one month of challenge 93% of patients are AGA and 69% EMA positive. After more than three years of gluten intake the percentage of AGA positive patients decreased to about 50% whereas the percentage of EMA positive sera was then highest (93%). Therefore EMA are more sensitive for the detection of 'silent' relapse after prolonged periods of gluten intake.

Adolescent↗

PubMed1989

IgG, IgA and IgE gliadin antibody determinations as screening test for untreated coeliac disease in children, a multicentre study.

The diagnostic value of gliadin IgG, IgA and IgE antibody (AB) determinations using the fluorescent immunosorbent test was examined in 586 children with malabsorptive disorders and/or failure to thrive. All patients underwent jejunal biopsy and were on a gluten-containing diet. IgG AB were found in all patients (331/331) with untreated coeliac disease (CD) in our study, but IgA AB in only 295/331 (89%). Therefore a screening test based only on IgA AB determinations is not recommended. By contrast, 203 (80%) of 255 children with other malabsorptive disorders had no gliadin AB, 43 (16.5%) had only IgG AB and only 9 (3.5%) had IgG and IgA AB. IgE AB proved to be of no additional value as a diagnostic tool because they were found in a quarter of the children without CD. Statistical evaluation of combined IgG and IgA AB determination showed at least 96% sensitivity and a specificity of 97%. The subjective ("Bayesian") probability that an actual patient with a given AB test result has CD, is considered: a patient very probably has CD in the case of positive IgG and IgA AB, and no CD in the case of a negative AB result. In the case of negative IgA AB but positive IgG AB the physician's judgement ("prior probability") influences the ("posterior") probability of CD for an actual patient. In contrast to IgG AB, IgA AB decline rapidly after the introduction of a gluten-free diet and may be used for diet control after diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1979

[Familial nonendemic hemolytic uremic syndrome: nephrectomy and transplantation (author's transl)].

A case of familial hemolytic uremic syndrome (HUS) in a four"year-old boy is reported. In his family four adult members are affected with the same disease. In the present patient we decided to perform bilateral nephrectomy because of the inexorable evolution of the disease. The intervention was followed by a distinct improvement, both from the clinical and the laboratory point of view. The child has profited of a renal transplantation. Unfortunately, he died three weeks later because of a severe urological complication. The aim of this study was to examine the effect of bilateral nephrectomy and transplantation on the clinical, laboratory and pathologic anatomical findings, and to analyze the literature, in order to find out whether these measures should be applied in the treatment of familial HUS with severe evolution. The observations seem to confirm the hypothesis of a renal pathogenesis of primary non endemic familial HUS. Nephrectomy seems to stop the disease, and the risk of a recurrence of the HUS after transplantation may be small. The occurrence of the disease in five family members in three generations was interpreted in favour of a genetic predisposition with a dominant gene.

Child, Preschool↗

PubMed1978

Value of 1-hour blood-xylose test in diagnosis of childhood coeliac disease.

In a series of 46 children with untreated coeliac disease and in 102 controls with normal mucosa the 1-hour blood-xylose test was, in view of its simplicity, of much value in the diagnosis of childhood coeliac disease. Only one blood-xylose result was falsely normal in the 46 coeliac patients. It is concluded that a normal blood-xylose value does not exclude coeliac disease and should not prevent peroral biopsy in the presence of strong clinical suspicion. On the other hand, patients who have repeatedly abnormal blood-xylose values merit an intestinal biopsy even in the absence of suggestive clinical symptoms.

Adolescent↗

PubMed1978

One-hour blood-xylose in cystic fibrosis.

One-hour blood-xylose concentrations after an oral xylose load were measured in children with cystic fibrosis (CF) and healthy controls. The mean of the 1-hour blood-xylose values was significantly increased in the group with CF. The finding confirms an earlier observation by Rolles et al. (1973). Its significance is not at present understood but it suggests that small intestinal function should be further investigated in CF.

Adolescent↗

PubMed1978

Re-evaluation of the techique of organ culture for studying gluten toxicity in coeliac disease.

In vitro cytotoxicity of four different gluten fractions was tested in organ culture for up to 48 hours using flat intestinal biopsies from children with coeliac disease. The fractions were (1) a peptic-tryptic digest of gliadin containing a moderate amount of alpha-gliadin, (2) a peptic-tryptic digest of gluten (Frazer fraction III) froma strain of wheat with a high content of alpha-gliadin, (3) alpha-gliadin, and (4) alpha-GT-18,000, a tryptic fragment of alpha-gliadin. The latter three fractions were toxic to coeliac patients in vivo. In vitro, however, none of these fractions proved to be cytotoxic. When added to the culture medium they were not capable of inhibiting the regeneration of the surface epithelium as visualised by histology and electron microscopy. The only difference between cultures with and without gluten fractions was that the former produced slightly more mucus when maintained in vitro as observed in the dissecting microscope. Furthermore, for Frazer fraction III the absence of apparent toxicity was confirmed by the behaviour of brush border enzyme activities during culture. Our results are not in accordance with those reported in the literature. We believe that the criteria used at the present time for the assessment of gluten toxicity in vitro should be extended to include the process of enterocyte desquamation.

Celiac Disease↗

PubMed1978

Protein-losing enteropathy due to segmental erosive and ulcerative intestinal disease cured by limited resection of the bowel.

Two children suffering from extensive intestinal protein loss due to subacute and chronic segmental small bowel disease are presented. In the first case a tentative diagnosis of chronic erosive and ulcerative non-granulomatous jejunitis as described in adults was made. The second child suffered from subacute erosive and ulcerative segmental transmural ileitis following mechanical ileus. In both instances resection of the involved segments of small intestine promptly cured the enteric protein loss. It is suggested that excessive protein loss due to subacute or chronic segmental erosive and ulcerative intestinal disease may be cured definitively by surgical resection. An explorative laparotomy should be performed if broad internistic investigations do not provide an explanation for the severe and prolonged enteric protein loss.

Adolescent↗

PubMed1977

Intraepithelial lymphocytes and one-hour blood-xylose in children with untreated coeliac disease.

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Age Factors↗

PubMed1977

The brush border membrane in hereditary sucrase-isomaltase deficiency: abnormal protein pattern and presence of immunoreactive enzyme.

In a child with hereditary sucrase-isomaltase deficiency immunoreactive enzyme was present in the intact duodenal mucosa. Polyacrylamide gel electrophoresis carried out with membrane fragments of an intestinal biopsy showed an abnormal protein band without enzyme activity. The mucosa had a relatively high residual isomaltase activity which was recovered from the gel in a position suggesting higher than normal molecular weight. The results indicated that in this patient the primary structural defect was in the sucrase moiety which was enzymatically inactive. The isomaltase subunits may have aggregated into a large molecular weight complex because of unavailability of their partners. The observation also provided evidence for separate biosynthesis of the two moieties of the sucrase-isomaltase complex.

Cell Membrane↗

PubMed1976

[The blood-xylose test in childhood: correlation between 1 hour blood-xylose levels and numbers of intraepithelial lymphocytes in intestinal mucosa in celiac disease].

In 59 children who underwent diagnostic small-intestinal biopsies the correlation between the intraepithelial lymphocyte infilration of the distal duodenal mucosa and the blood-xylose concentration 1 h after ingestion of 5 g D-xylose was investigated. 16 patients with active coeliac disease had blood-xylose values below 20 mg%, and the correlation between blood-xylose levels and the numbers of intraepithelial lymphocytes in this same group of patients was found to be highly significant. Provided that the methodology is meticulously followed, it is our experience that the one-hour blood-xylose test, in addition to the clinical investigation, facilitates the decision whether or not to perform a small-intestinal biopsy in the diagnosis and follow-up of coeliac disease in childhood. The test however cannot replace the intestinal biopsy which remains the only acceptable criterion for the diagnosis of this disease.

Adolescent↗

PubMed1975

Urinary 5-hydroxyindoleacetic acid in 8-hour collections as an aid in diagnosis of coeliac disease.

8-hour urine excretions of 5-hydroxyindoleacetic acid (5-HIAA) were measured in 18 children with coeliac disease before treatment and the results expressed as microgram 5-HIAA/mg creatinine. Similar measurements were made on urine collections from an age-matched control group of 24 children. Significantly higher values of 5-HIAA excretion were found in children with untreated coeliac disease. Measurement of the 5-HIAA: creatinine ratio in 8-hour urine collections is therefore proposed as an aid in the diagnosis of coeliac disease.

Celiac Disease↗

PubMed1975

Bile acid excretion after pull-through operation for Hirschsprung's disease.

Four children with chronic diarrhoea and perianal excoriation after a pull-through operation for Hirschsprung's disease have been shown to have increased but not markedly raised levels of faecal bile acids. Bile acid analysis of the 'bile-rich' duodenal fluid obtained after pancreozymin stimulation in 3 of the patients indicated a marked reduction in the proportion of deoxycholic acid conjugates. These findings are compatible with colonic malabsorption of secondary bile acids in these patients which is related in some way to the pull-through operation, but which is not likely to be the cause of the diarrhoea and the anal excoriation.

Adolescent↗

PubMed1975

The agglutinating antibody response in the duodenum in infants with enteropathic E. coli gastroenteritis.

The agglutinating antibody responses in duodenal fluid and serum were measured serially in 15 infants with enteropathogenic E. coli gastroenteritis. Peak levels of duodenal agglutinins were recorded eight to 18 days after the onset of symptoms, and the titres fell within the next seven to 14 days. These antibodies were mainly of the IgA class but IgM antibodies were detected early in the response, especially in the youngest infants. Serum antibody responses were detected in eight patients, but they correlated poorly with the titres of intestinal antibodies. No rise in serum antibodies was found in six infants. Further studies are required to determine whether these differences are host-derived or whether they reflect different pathogenic properties of the infecting organisms.

Agglutinins↗

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