PubMed HealthSearch

Biomedical subjects

E Signer

Publications and source records attributed to E Signer.

At least 55 records · Page 3Linked to original sources

A reliable screening test for childhood celiac disease: fluorescent immunosorbent test for gliadin antibodies. A prospective multicenter study.

The diagnostic value of gliadin antibody determination using the fluorescent immunosorbent test was examined in a prospective multicenter study comprising 251 children with malabsorptive disorders. Antibodies to gliadin were found in all 72 patients (100%) with active celiac disease (29 children with celiac disease proved by challenge, 43 with probable celiac disease). All children up to the age of 7 years had antibodies in high titers. By contrast, 96 (84%) of 114 children with other malabsorptive disorders and a normal mucosa or with partial villous atrophy had no gliadin antibodies, 14 (12%) had a low titer, and only four (3.5%) showed moderate to high titers. Four children with gastrointestinal tract symptoms of cow milk intolerance and a flat mucosa also showed no antibodies. In 24 of 29 children (83%) with cystic fibrosis and six of seven children with Crohn disease (biopsies not performed in either group), no antibodies could be detected. The others had low or elevated titers. In 25 children with acute gastroenteritis (not biopsied) antibodies were not found at hospital admission nor six weeks later after reintroduction of gluten. The determination of antibodies to gliadin with the fluorescent immunosorbent test is a reliable screening test for childhood celiac disease. In our series there were no false negative results in children with untreated celiac disease. A positive gliadin antibody titer is not proof of celiac disease. In each child the diagnosis must be confirmed by small intestinal biopsy even if the gliadin antibody titer is high. The detection of high titers of cow milk antibodies in 27% of patients with celiac disease is of no value.

Adolescent

Bone marrow graft versus ALG in patients with aplastic anaemia.

Eighty-six successive patients with severe aplastic anaemia were admitted to this hospital between January 1976 and October 1982. They were treated and evaluated in a prospective study according to one protocol. 26 patients with an HLA identical sibling underwent bone marrow transplantation. Overall survival calculated according to Kaplan and Meier in this group is 47%, 60 patients without an HLA identical sibling were given antilymphocyte globulin with or without an infusion of HLA-haploidentical marrow. All these 60 patients received low dose androgens after the procedure. In this study the marrow infusion did not significantly improve the results and overall survival was 74% compared to 70% in patients receiving only antilymphocyte globulin and androgens. In a current pilot study combining ALG and high dose prednisone we were able to further increase remission rates. Between HLA-identical siblings we improved the results of marrow transplants significantly by the use of cyclosporin-A instead of methotrexate for prophylaxis against GvHD.

Adolescent

[Marrow transplantation in leukemia-recent trends].

Due to progress in conventional chemotherapy 50-60% of children with acute leukemia can be cured today. Despite these good results, however, 40-50% eventually will relapse and die. Other therapeutic modalities are therefore needed. Transplantation of bone marrow from an HLA-identical sibling offers this possibility. Initiated a decade ago as last therapy for end stage disease patients marrow transplantation is a well tolerated therapy today with well defined risks. In adults, marrow transplantation is clearly superior to conventional chemotherapy if performed early. The incidence of severe Graft-versus-Host-Disease, the major complication of marrow transplantation is much less after early transplantation and Cyclosporin-A, a new immunosuppressive agent further reduces severity of Graft-versus-Host-Disease. Since all complications are less in younger patients marrow transplantation is the therapy of choice for a child with leukemia having an HLA-identical sibling donor.

Acute Disease

[New development in clinical bone marrow transplantation in leukemia].

39 clinical bone marrow transplants (BMT) for leukemia are described. In a historical control series of 18 patients in whom BMT was performed after all chemotherapeutic resources had been exhausted, there is only 1 long-term survivor (5.5%), 8 patients died from GvH reaction, 6 from interstitial pneumonia and 3 from recurrent leukemia. Since 1979 an attempt has been made to transplant patients under optimal conditions (1st complete remission) and cyclosporin-A (CyA) has been used for prophylaxis of GvH reaction instead of MTX. 11 patients were transplanted according to our original proposal (AML and ALL in first remission, CML in chronic phase): 10 have survived without evidence of leukemia (91%), 1 AML died in relapse. 10 patients were grafted in second or later remissions or early relapse: 5 have leukemia-free survival (50%), 1 is living with a relapse. In this group 3 deaths were due to recurrent leukemia and 1 to CMV-infection. In our experience BMT under optimal circumstances does not involve a risk of early mortality and the chances of recurrent leukemia are reduced. Severe or chronic GvH reaction is not seen under CyA. BMT is the treatment of choice for patients with histocompatible sibling donors.

Bone Marrow Transplantation

[Treatment of severe aplastic anemia].

58 patients with severe aplastic anemia (SAA) were treated and evaluated in a prospective study either by bone marrow transplantation (BMT) or by antilymphocyte globulin (ALG). 19 patients were treated with BMT; 9 are still alive 6 months to 5 years after BMT (47%). 39 patients were treated with ALG; 28 are alive 5 months to 5 years after ALG (72%). 24 of these 28 are self-sustaining and in remission. The results show that treatment with ALG is probably superior to treatment with BMT, and also demonstrate that most patients with SAA have a pool of hematopoietic stem cells able to repopulate the marrow after this type of treatment.

Adolescent

Antibodies to gliadin as a screening test for coeliac disease. A prospective study.

The diagnostic value of gliadin antibody determination using the fluorescent immunosorbent test was examined in a prospective study of 57 children with gastrointestinal disease. Antibodies to gliadin were found in all 20 patients with active coeliac disease, whereas 7 of these children (37%) had a normal xylose absorption test despite a flat small gut mucosa. Only 4 (14%) of 28 children with other gastrointestinal conditions had antibodies to gliadin, invariably in low titre. After at least 2 years on a gluten-free diet none of 9 children with coeliac disease in remission had demonstrable gliadin antibodies. The gliadin antibodies disappear slowly, within 6 to 24 months, after withdrawal of gliadin from the diet. 0.8% (5/606) of a healthy control group of children, adolescents and adults (not biopsied) had gliadin antibodies in low titre. Increased mean cow's milk antibody titres were demonstrable in 8 (40%) of 20 patients with active coeliac disease as well as in 9 (32%) of 28 patients with other gastrointestinal lesions. Our studies show that determination of circulating gliadin antibodies is a worthwhile screening test in suspected cases of coeliac disease. In patients so selected there is a definite indication for small intestinal biopsy to confirm the diagnosis.

Adolescent

Bleeding gastric ulcer in a newborn infant diagnosed by transumbilical aortography.

Transumbilical aortography has been utilized for the first time to diagnose the cause of massive upper gastrointestinal hemorrhage in a newborn infant. We consider it a safe and accurate method in cases that meet the following criteria: massive hemorrhage uncontrollable by blood transfusions and supportive medical therapy, and hemorrhage active at the time of study as indicated by fresh blood draining from nasogastric tube.

Aortography

Immunofluorescent antibodies against gliadin: a screening test for coeliac disease.

A sensitive and simple immunofluorescence test for gliadin antibodies is described, which may be suitable as a screening method for coeliac disease especially in cases with no clear-cut indication for performing a biopsy. In serum samples of 19 from 23 patients (83%) with coeliac disease proved by biopsy antibodies against gliadin could be detected. In a control group of 71 children in whom the clinical diagnosis of another malabsorptive disorder was made gliadin-IF-antibodies were found in only 3 children (4%). With better timing of blood sampling (first sample during a period with gluten containing food and in case of a negative result a second sample after 4-8 days of gluten withdrawal) the sensitivity of the screening test will be improved. The described gliadin-IF-test permits the classification of the antibodies into the different immunoglobulin classes. In each child with a positive result IgG antibodies against gliadin were detected. In 9 respectively 4 of the 19 patients with IgG antibodies IgA- respectively IgM-antibodies against gliadin were also found. Antibodies against at least one cow milk protein were identified in nearly all (91%) coeliac patients and in half (49%) of the patients with other malabsorptive disorders.

Antibodies

Gastric emptying in newborns and young infants. Measurement of the rate of emptying using indium-113m-microcolloid.

The rate of gastric emptying was measured in newborns and young infants by a new radio-isotopic method. 28 control babies and 6 infants with projectile vomiting were given a 50 ml standard milk feeding containing 15 muCi of Indium-113m-microcolloid. The radioactivity in the stomach was counted at regular intervals with a gamma-camera. The gastric emptying followed an exponential pattern with a "half-life" of 87 plus or minus 29 minutes in 24 out of 28 control babies. In 6 patients with projectile vomiting gastric emptying was impaired severely. Three of them with hypertrophic pyloric stenosis showed complete stasis of gastric contents. Gastric emptying returned to normal 8-16 days after pyloromyotomy. It is suggested that the radioisotopic technique is helpful in evaluating the severity of pyloric stenosis and that it is of value in studies of the action of pharmacological substances on gastric emptying.

Half-Life