[Obstructive emphysema of infant during pulmonary tuberculosis]. / Enfisema a valvola del lattante in corso di tubercolosi polmonare.

A rare complication of pulmonary tuberculosis of infant is the obstructive emphysema following to the compression by enlarged lymphadenopathy. The authors report a case of obstructive emphysema observed in a 12 month old infant affected by pulmonary tuberculosis, and debate its more important pathogenethic, clinical and differential diagnosis aspects. At last they comment upon favourable clinical course observed as results of an exclusive medical treatment (antitubercular antibiotic therapy shortly combined with moderate corticotherapy) without making use of surgical, but aggressive, treatment.

[Clinical and pathogenetic observation in a case of pulmonary hemosiderosis]. / Considerazioni clinico-patogenetiche su di un caso di emosiderosi polmonare.

Pulmonary hemosiderosis is a rare disease of unknown aetiology, whose clinical, pathogenetic and prognostic aspects are still open to discussion. The authors report a case of pulmonary hemosiderosis in a 2 years and 3 months old girl. Peculiar features of this case include antibody deficiency (which may contribute to the pathogenesis of the disease) and severe and protracted melena. Fatal outcome, despite several therapeutic trials, confirms the poor prognosis of pulmonary hemosiderosis.

[Autoimmune hemolytic anemia with cold antibodies and hemoglobinuria secondary to EBV infection]. / Anemia emolitica autoimmune da anticorpi freddi con emoglobinuria secondaria ad infezione da EBV.

The infection caused by EBV can be followed by immunological complications. One of these is autoimmune hemolytic anemia that up to today has been observed during infective mononucleosis only a few times. The authors describe a patient with this rare complication of the EBV infection and discuss its main clinical, pathogenetic and laboratory aspects, with particular attention to the presence of hemoglobinuria and to the absence of anti-i-antibodies. The disease's evolution was spontaneously favorable. For this reason and for the possible risks secondary to transfusions and to other therapeutical interventions, the authors believe that in autoimmune hemolytic anemia a vigil wait is more opportune before beginning the therapy.

[Antiendomysium antibodies and antigliadin antibodies in diagnosis and follow-up of celiac disease]. / Anticorpi antiendomisio ed anticorpi antigliadina nella diagnosi e nel follow-up della malattia celiaca.

Anti-endomysium antibodies (AEA) and Antigliadin antibodies (AGA) have been compared in a group of coeliac patients (17 with untreated coeliac disease, 56 after gluten free diet and 14 after gluten challenge), in 69 patients with various intestinal pathological conditions, and in 40 healthy children, in order to appreciate sensitivity and specificity of these tests in diagnosis of coeliac disease. AEA and AGA IgA were detected in 100% and AGA IgG were found in 94.1% of coeliacs with untreated disease; after gluten free diet AGA IgG were found in 27.7% of cases, while AGA IgA and AEA were absent in all coeliacs but one with non correct compliance to diet. After gluten challenge followed by villous atrophy, 78.5% of patients were positive for AGA IgA, 85.71% for AGA IgG and 100% for AEA. Anti-endomysium antibodies were absent both in healthy controls both in control disease patients, while sometimes antigliadin antibodies were present in patients with various intestinal pathology (AGA IgA in 1.44% and AGA IgG in 7.2% of cases). In our experience therefore AEA, because their sensitivity and high specificity, appear to disclose new prospects in diagnosis of coeliac disease.

[Autoimmune hemolytic anemia in the course of Kawasaki disease. A case presentation]. / Anemia emolitica autoimmune in corso di malattia di Kawasaki. Presentazione di un caso.

Kawasaki disease can be characterized by rare complications or by unusual associations. One of these is autoimmune hemolytic anaemia that has been observed seldom up to now. The Authors report a case with this rare association, compare it with cases observed before, and put forth the hypothesis that autoimmune anaemia during Kawasaki disease can be linked to numerous immunological alterations present in mucocutaneous lymph node syndrome. They moreover advise to abstain in these cases from corticosteroid therapy, both to avoid complications, and because, as happened in their patient, this kind of autoimmune hemolytic anaemia can regress spontaneously.

[Clinical and pathogenetic observations on children with chronic mononucleosis]. / Rilievi clinico-patogenetici su bambini con mononucleosi cronica.

Epstein-Barr virus is seldom the causative agent of a prolonged atypical illness, known as chronic mononucleosis syndrome, characterized by a persistent pattern of clinical manifestations and by a defective immune response to specific viral antigens. This paper refers about 6 children for whom clinical and serological findings suggest the chronic Epstein-Barr virus infection. The authors believe that this chronic state might be explained by the unusual antibody pattern to EBV virus, with the persistent presence of anti-EA and the absence of anti-EBNA titers, expression of a reduced EBV-specific cytotoxic T cell activity.

[Antiendomysium antibodies in the diagnosis and follow-up of celiac disease]. / Utilità degli anticorpi antiendomisio nella diagnosi e nel follow up della malattia celiaca.

The authors have studied the proceeding of anti endomysium antibodies (AEA-IgA) in a group of coeliac patients, in order to investigate new tests, not intrusive and easy repeatable, to employ in diagnosis and in follow up of coeliac disease. The research was carried out by an indirect immunofluorescent test on 65 sera of patients with age range 1-11 years; of those patients 14 show the coeliac disease at beginning, 16 were on Gluten Free Diet (GFD) from 3-5 months, 28 were on GFD from 1 year and 7 were on challenge with gluten from 2-4 months. We investigated AEA-IgA in sera of 71 gastroenterological and not-gastroenterological patients of analogous age to coeliacs how control subjects. All coeliac patients (14) with disease at beginning were positive for AEA-IgA. For the patients on GFD we observed an evident correlation between the term of diet and presence of AEA-IgA: 12 of 16 patients on GFD from 3-5 months were positive, but after 1 year of GFD all patients were negative; after 2-4 months from reintroduction of gluten in diet with the challenge we observed that all examined patients were positive for AEA-IgA. In 21 coeliac patients on free diet (14 with the disease at beginning and 7 after challenge) any reduction of percentage positivity was noted with the growth. The investigation of AEA-IgA in all examined control subjects was always negative. Our data suggest that the AEA-IgA investigation, for highly sensitivity and specificity, for easy repeatability and absence of intrusivity, is a diagnostic test that can be usefully combined to others (intestinal biopsy, dosage of anti gliadin antibodies) in diagnosis and in follow up of coeliac disease.

Immunoglobulin heavy chain allotypes in a sample of Sicilian patients with celiac disease.

The authors have studied the immunoglobulin heavy chain allotypes in 14 Sicilian patients with celiac disease (CD) and in 75 healthy controls. No association has been found between CD and Gm or Km phenotypes. When the role of Gm phenotypes in immune response against gliadin was analyzed, fb-positive CD patients on gluten-free diet for 1 year showed an increased antigliadin IgG response. Comparing the values of fb-positive subjects with those of negative ones, a significant difference was observed. These data, demonstrating that the presence or absence of a phenotype is able to define the breadth of immune response against alpha-gliadin antigen, are indicative of the role played by Ig allotypes in CD.

Immunoglobulin heavy-chain allotypes play a role in the clinical history of celiac disease.

We studied the role of the immunoglobulin heavy-chain allotypes in the evolution of celiac disease. Particularly the Gm(fb) phenotype was investigated since this phenotype is able to determine the breadth of immune response against alpha-gliadin antigen. The results show that weight percentiles and intestinal absorption as assessed by xylose test are influenced by Gm(fb) phenotype suggesting that Gm(fb) genes contribute to the determination of clinical aspects and evolution of celiac disease.

[Celiac disease and dietary habits in the 1st year of life]. / Malattia celiaca e abitudini alimentari nel primo anno di vita.

The Authors have performed a study on the dietary habits of 74 celiac children and have analysed retrospectively the type of nursing (breast-feeding or bottle-feeding), the time and the age of gluten introduction. Then they related these data to the age of onset of symptoms. Children breast-fed for 3 months or more showed a marked delay in the onset of the disease and a longer latency time from gluten introduction when compared with bottle-fed children; on the contrary early gluten introduction was not positively correlated to risk of celiac disease. This observations induce to think that breast-feeding may be a protective factor towards coeliac disease, while the precocious gluten introduction is not an important factor of risk correlated to this disease.

[HLA and sprue: a study of Sicilian families with celiac disease]. / HLA e celiachia: studio su alcune famiglie siciliane con malattia celiaca.

The authors have studied the HLA genotypes in 17 Sicilian coeliac children, in their parents and in their healthy brothers. A positive association has been found between coeliac disease (C.D.) and HLA antigens; this was strongest firstly for DQW2 and than for DR7 and DR3. Those antigens however do not result specific for C.D., because they were present in healty-control population too and because one coeliac patient was DQW2, DR7 and DR3 negative. A distortion of vertical transmission of HLA haplotypes has been observed in the studied patients, and this occurred for DR3/DQW2 that was transmitted mainly by paternal way. Moreover, in the healty brothers of our coeliac subjects a significant reduction of HLA antigens DQW2, DR7 and DR3 has not been found. Those observation and the finding that in our families the responsible gene for C.D. seems to have a low penetrance, should induce to search others genetic markers for coeliac disease.

[Jarcho-Levin syndrome or spondylo-costal dysostosis. Apropos of a case]. / La sindrome di Jarcho-Levin o disostosi spondilo-costale. A proposito di un'osservazione.

The Authors report a case of spondylo-costal dysostosis observed in a child 2 years and 1 month old. They recall its clinical and radiological findings and discuss genetic problems and differential diagnosis of this rare disease.

Immune status towards Epstein-Barr virus in a group of Sicilian children.

The prevalence of antibodies to Epstein-Barr virus-determined antigens was studied in 17 children with acute infectious mononucleosis (IM) and in 263 children hospitalized for diseases unrelated to EBV infection. Antibodies against Epstein-Barr viral capsid antigens (VCA) were observed in 173 patients of the control group (66%), but 58 of them (33,5%) had not yet developed antibodies against Epstein-Barr virus-associated nuclear antigen (EBNA). IgM-specific antibodies were not found in any of the children of the control group but were present in all of the 17 patients with IM. The rates of positivity for IgA anti-VCA and IgG anti-early antigen (EA) were similar in all age groups. Anti-viral capsid antigen IgG seropositivity increased to 83% by the age of six years, the mean geometric titre being highest between the ages of 2 and 4 years. Our results suggest that in Sicilian children the primary infection occurs prevalently early in life, in parallel with the occurrence of IM.

[The pseudoachondroplastic form of spondyloepiphyseal dysplasia: apropos of 2 cases]. / Forma pseudoacondroplasica della displasia spondiloepifisaria: a proposito di due osservazioni.

The Authors report two cases of pseudoachondroplasic dysplasia observed in a child aged 2 years and 5 months and in her father 31 years old. They recall the rarity of this disease, its characteristic and radiological findings and dwell upon differential diagnosis between this rare form of condrodysplasia and achondroplasia.

[Clinical remarks on a case of hypocalcemic tetany secondary to celiac disease initially diagnosed as chronic hypoparathyroidism]. / Considerazioni cliniche su di un caso di tetania ipocalcemica secondaria a malattia celiaca, inizialmente diagnosticata come ipoparatiroidismo cronico.

The case of a patient with tetanic hypocalcemia secondary to celiac disease initially diagnosed as chronic hypoparathyroidism is reported. The rarity and clinical peculiarities of the case are underlined and its pathogenesis discussed with particular reference to the difficulties it presents in terms of differential diagnosis.

[Visceral leishmaniasis in infants without bioptic demonstration of the parasite. Presentation of 3 cases]. / Leishmaniosi viscerale del lattante senza dimostrazione bioptica del parassita. Presentazione di tre casi.

The Authors describe three infants with visceral leishmaniasis in whom bioptic research (in marrow, spleen and liver) has not demonstrated presence of leishmania. They remark this unusual aspect of visceral leishmaniasis in infant and debate the epidemiological, diagnostic, and therapeutic aspects of these cases.