Studies of bone mineral density in children affected by dietary intolerances

The Pediatric Department of the University of Ferrara has developed a special expertise in the field of hemoglobinopathies and has also an interest in gastrointestinal diseases. It has a long standing collaboration with the Department of Genetics of the University of Verona. The main fields of interest are thalassemia, gluten intolerance, and osteoporosis in its various aspects. Using our previous research experience as a platform, we plan to study Vitamin D metabolism, bone mineral density, the FGF23 and Klotho axis in patients with thalassemia, in patients with adult type lactose intolerance, in patients treated with antiepileptic drugs and in those who suffer from gluten intolerance. Finally, we intend to cooperate with another group (PP9) of the Trans2Care project in order to clarify the role of tissue antitransglutaminases in seronegative patients with symptoms of gluten intolerance

Thalassemia and the heartquake

On May 2012 the city of Ferrara and the surrounding region were hit by several earthquakes. We had the chance to observe the behavior of one thalassemic heart during the shocks, because of a 24-h electrocardiogram recording had been put in place a few hours before the shocks

Subcutaneous bolus injection of deferoxamine in adult patients affected by onco-hematologic diseases and iron overload

Background and Objective. Chelation therapy is often necessary for patients who undergo chronic transfusion therapy for myelodysplastic syndromes. In these patients, deferoxamine, the most widely used chelating agent, has been reported to be effective in reducing the iron burden and the transfusion requirement. Unfortunately, compliance with the drug, that is usually administered by slow subcutaneous infusion via a battery operated pump, is often poor, especially in elderly patients

Cross-sectional study of coeliac autoimmunity in a population of Vietnamese children

Objective: The prevalence of coeliac disease (CD) inVietnam is unknown. To fill this void, we assessed the prevalence of serological markers of CD autoimmunity in a population of children in Hanoi. Setting: The outpatient blood drawing laboratory of the largest paediatric hospital in North Vietnam was used for the study, which was part of an international project of collaboration between Italy and Vietnam. Participants: Children having blood drawn for any reason were included. Exclusion criteria were age younger than 2 years, acquired or congenital immune deficiency and inadequate sample. A total of 1961 children (96%) were enrolled (838 females, 1123 males, median age 5.3 years). Outcomes: Primary outcome was the prevalence of positive autoimmunity to both IgA antitransglutaminase antibodies (anti-tTG) assessed with an ELISA test and antiendomysial antibodies (EMA). Secondary outcome was the prevalence of CD predisposing human leucocyte antigens (HLA) (HLA DQ2/8) in the positive children and in a random group of samples negative for IgA anti-tTG. Results: The IgA anti-tTG test was positive in 21/1961 (1%; 95% CI 0.61% to 1.53%); however, EMA antibodies were negative in all. HLA DQ2/8 was present in 7/21 (33%; 95% CI 14.5% to 56.9%) of the anti-tTG-positive children and in 72/275 (26%; 95% CI 21% to 32%) of those who were negative. Conclusions: Coeliac autoimmunity is rare in Vietnam, although prevalence of HLA DQ2/8 is similar to that of other countries. We hypothesise that the scarce exposure to gluten could be responsible for these findings

The life of patients with thalassemia major

Andrea wakes up late, because the night before he went to a party with his girlfriend. He disconnects the needle and puts his desferal pump in the drawer. Then he rushes to work: he’s a computer designer and his job is quite safe, because it was granted under the law protecting thalassemia patients and other people with medical problems. Nevertheless he does not want to irritate his boss. Besides, in a few days, he will be absent, and work will pile up, as he needs to go to the hospital for his regular blood transfusion

Pica as a manifestation of iron deficiency

Introduction: Pica is the compulsive eating of non-nutritive substances. It is often associated with iron deficiency but its pathophysiology is unknown. Areas covered: We searched the literature using the keywords listed below. Our aim was to describe the phenomenon in its various aspects, to touch briefly on the historical and cultural background, and to examine in more detail the studies that tried to analyze the relative roles of iron deficiency and pica. Expert commentary: Pica is an intriguing symptom known for centuries. Pregnant women and preadolescents are at the highest risk of pica. Iron absorption is reduced in the presence of non-nutritive substances. Iron therapy usually cures the pica behavior. There are different forms of pica, one caused directly by iron deficiency probably due to the lack of iron in some areas of the brain and one more culturally driven and including mostly geophagy

Ischemic Stroke in Infants and Children: Practical Management in Emergency

Stroke is a rare disease in children, with an estimated incidence 13/100000 and a significant impact on morbidity and mortality. Clinical presentation and risk factors, present in almost half of pediatric patients, are not the same as in adults. The diagnosis of stroke in children is often delayed because signs and symptoms can be subtle and nonspecific. History and clinical examination should exclude underlying diseases or predisposing factors. Neuroimaging is crucial in defining diagnosis. Other tests might be necessary, according to the clinical picture. We present here the most recent practical directions on how to diagnose and manage arterial stroke in children, according to different international guidelines on the subject

Malignancies and thalassemia: a review of the literature

Thalassemia is a genetic hematologic disease, characterized by a defect in hemoglobin chain synthesis. Because of safe transfusions and effective chelation therapy, survival of affected patients has significantly improved in the last few decades. However new complications are appearing. Among them are hepatocellular carcinoma and other forms of cancer, particularly hematologic malignancies. The present review focuses on the frequency of cancer in thalassemia patients and on possible predisposing factors