[Diagnostic protocol in the neurourological bladder in patients with myelodysplasia].
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Biomedical subjects
Publications and source records attributed to G Arena.
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The surgical treatment of the CoAo plus Hypoplastic Aortic Arch is debated. The point is whether to treat or not the associated tubular hypoplasia performing an extended Resection and "End to End" Anastomosis (ERETE) or a subclavian flap angioplasty (Waldhausen). According to some report in the literature we called an arch hypoplastic if its diameter in the first or second portion was less than the patient body weight plus 1. Between January 1988 and December 1992 in our Institution 38 patients less than 3 months underwent aortic coarctation repair. In 14 cases we were able to consider the aortic arch as hypoplastic. These patients were aged between 3 days and 45 days (median 15 days), the body weight was between 2.6 and 3.9 kgs (median 3.2 kgs). 6 patients had CoAo as isolated lesion, in 3 cases there was Ventricular Septal Defect (VSD) associated. In 5 patients major intracardiac anomalies were associated (1 DORV, 1 TGA, 2 SV, 1 SV+Arterio-Ventricular discordance). In 7 cases in order to treat the arch an ERETE was performed. The other 7 patients had a Waldhausen procedure. In 5 cases a Pulmonary Artery Banding (PAB) was associated, in 1 case a palliative arterial switch operation. The hospital death was 43% (3 patients) in the ERETE group, and 28% (2 patients) in the Waldhausen group. The echocardiography and or angiography performed during the follow-up period showed a nice growth of the arch in both groups. Although from our present data it's impossible to get definitive conclusions, the ERETE doesn't give better result in terms of hospital death and long term aortic arch growth.(ABSTRACT TRUNCATED AT 250 WORDS)
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Since the beginning of the use of Antigliadin Antibodies (AGA) in the screening of coeliac disease (CD) we have observed an increasing in the total number of cases diagnosed, in particular of the cases with monosymptomatic and atypical forms. Iron deficiency anemia is one of the more frequent findings that we can find in CD, either in association with other typical coeliac signs, or as an isolated expression of the disease. The first aim of our study was to determine the incidence of iron deficiency anemia in our patients affected by CD at the moment of diagnosis. The second aim was to determine the incidence of CD in a group of 96 patients attending our Pediatric Hematology department for iron deficiency anemia of unknown etiology and refractory to iron therapy. 103 patients out of our 212 coeliacs (48.5%) showed hypochromic and microcytic anemia. In the second sample we found 6 (6.2%) patients, positive in AGA and Antiendomysium Antibodies (AEA), that showed a typical coeliac picture at the jejunal biopsy. Our study confirms the high incidence of iron deficiency anemia in patients affected by coeliac disease. However the most important conclusion of our study is that a certain percentage of patients affected by hypochromic anemia of unknown etiology may be affected by coeliac disease. It is only by performing the specific screening tests (AGA and AEA) in the patients affected by iron deficiency anemia of unknown etiology, that we can diagnose this monosymptomatic expression of CD.
Coeliac disease (CD) is a gluten intolerance caused by a combination of genetic and environmental factors such as nutrition and infections. Monozygotic twins appear to have a concordance for CD up to 71%. This paper reports a third case of late onset of CD in monozygotic twin girls. The twins were defined as monozygotic based upon paired clinical and laboratory examinations. Clinical examinations included genotypic, phenotypic and dermatoglyphic analysis, while laboratory examinations included HLA typing and blood groups. Following European Society of Pediatric Gastroenterology and Nutrition criteria, CD was diagnosed in both girls, though 4 years and 8/12 months apart. The twins achieved clinical, laboratory and histological remissions within 1 year, after the institution of a gluten-free diet. Genetic markers are undoubtedly the main precondition for CD development. Environmental factors, however, may play a more significant role in triggering the onset of disease.
The coexistence of Down's syndrome (DS) and coeliac disease (CD) has been occasionally reported and both diseases are often related to autoimmune disorders. The pathogenetic factor that links CD and DS may be an altered immune system and/or the presence of a common genetic factor. Some epidemiological investigations, performed in patients with CD, showed an increased incidence of DS compared to the natural incidence of this abnormality in the general population. We studied the prevalence of CD in 83 individuals with DS compared to a group of 200 patients with other gastroenterologic disorders and a random scholastic sample of 500 non symptomatic children. IgG and IgA antigliadin antibodies (AGA) were determined in all patients. Antiendomysium antibodies (EmA) were investigated in all the patients of the first group, while in the other two groups, 27 and 108 cases respectively, selected by AGA positivity, were investigated for EmA. The percentage of AGA IgA positivity in the first group was 31.3% (26/83), in gastroenterologic controls 10% (20/200), in scholastic sample 2.8% (14/500), that shows a significant statistical difference. On the contrary EmA were positive in quite a similar percentage in the three groups. Duodenal [correction of Jejunal] biopsies, were performed in 11 DS patients and in 9 of the other two groups. EmA were positive only in the case with subtotal atrophy in all the groups: 5/11 in the first, 2/4 in the second, 2/5 in the third. On the contrary AGA IgA were often positive also in patients with non coeliac histologic findings.(ABSTRACT TRUNCATED AT 250 WORDS)
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