[Histological picture of liver disease in thalassemia intermedia]. / Quadro istologico dell'epatopatia nella Talassemia intermedia.

Thalassemia Intermedia (TI) is a clinical definition in use for a spectrum of clinical conditions ranging in severity from the asymptomatic carrier status to the transfusion-dependent status. The histological lesions of the liver in patients affected by TI has not seen well characterized yet. The aim of this study was to define the histological picture of liver disease in TI. To this end we studied our pool of 22 Thalassemia Intermedia patients who underwent liver biopsy; none of them had blood transfusion. We took into consideration fibrosis according to Ishak's staging and iron overload according to Sciot's grading. Moreover, we determined the hepatic iron content by atomic absorption spectroscopy. Our results have shown that, in all patients studied, the histological picture was characterized by the increase in liver iron stores. By Perls's stain haemosiderin appeared to be stored mainly in hepatocytes in the majority of cases. Inspite of the large amounts of liver iron in these patients, fibrosis was absent or mild. We also noted the presence of iron-free-foci, in part of the patients. Our data show that the histological and histochemical picture of liver disease in Thalassemia Intermedia is very similar to that found in Thalassemia Major. The finding of iron-free-foci could induce to consider a follow up of these patients, even if asymptomatic.

[Celiac disease and the evolution of its diagnosis. Comparison and experience at a hospital pediatric department (1975-1993). (Second part)]. / La malattia celiaca e l'evoluzione nella sua diagnosi. Confronti ed esperienze di un reparto pediatrico ospedaliero (1975-1993). (Seconda parte).

In a period of over 18 years the prominent medical bibliographic marks with regard to definition, diagnosis and examinations of coeliac disease (CD) have been compared and as far as possible reproduced. The results confirm the remarks derivating from wider statistics. From the beginning of 1975 to the first six months of 1993 in Merate Hospital Pediatric Division, 323 patients were submitted to a first jejunal peroral biopsy in 133 cases (41.2%) CD was diagnosed. Since 34 children (25.6%) concluded the ESPGAN diagnostic iter with 3 consecutive biopsies, the reasons why the other patients didn't finish or respect the programs are here examined. Since 1987 a specific anti-gliadin (IgA and IgG) antibodies titrimetry has been available either in the investigation of suspect symptomatology or like control mark during the assessment or after a sure CD diagnosis. Since october 1992 antiendomysium antibodies (EMA or AEA IgA) have been determined only in selected patients. From the examination of 24 subjects now checked with AGA IgA/IgG and EMA and with a first positive biopsy, it is possible to point out that only one jejunal biopsy (or at the most a second one as a control during the gluten challenge) with the guarantee of haematologic patterns doesn't raise doubts about a CD diagnosis. Analogous considerations mainly refer to the atypical CD "late onset" when a constant lack of AGA and EMA during gluten free diet (GFD) or their changes in a non compliance or in gluten challenge, can exclude a following hystological confirmation. By this experience it follows that a specific antigliadin and antiendomysium antibodies investigation is indispensable to the shortening of diagnostic times, to the reduction of an often unwelcome invasive diagnostic method and to the discovery of the "CD iceberg".

[N.B.T. - PABA screening test for exocrine pancreatic function in healthy children. Preliminary investigation in 60 normal children]. / N.B.T. - PABA-test: screening diagnostico della funzione pancreatica esocrina. Indagine preliminare su 60 soggetti sani.

The theoretical bases of PABA test as a diagnostic screening test of exocrine pancreatic insufficiency, its mode of execution and the findings obtained in 60 healthy subjects ranging in age from 2 to 14 years are reported. Those conditions related to extra-pancreatic disorders or to other factors that may interfere with the test and reduce its reliability are also discussed.

Pulmonary alveolar microlithiasis. A case report with a discussion of differential diagnosis.

The rare disease of pulmonary alveolar microlithiasis is characterized by innumerable microscopic stones, mainly of tribasic calcium phosphate, within the pulmonary alveoli. In a 13-year-old boy an earlier radiological examination showed diffuse lung opacity. The main differential diagnoses taken into consideration were sarcoidosis, idiopathic hemosiderosis, diffuse interstitial fibrosis and alveolar proteinosis. The correct diagnosis was made after a lung biopsy which showed intra-alveolar microliths. The radiological findings are in agreement with the reports in the literature. The specific X-ray features of pulmonary alveolar microlithiasis are calcified opacities, nodules, hilifugal trabeculations and pleural striae. These radiological signs are sufficiently diagnostic to restrict lung biopsy to particularly difficult cases. The disease is resistant to all therapeutic measures.