Biomedical subjects
M G Burattini
Publications and source records attributed to M G Burattini.
Benign lymphoproliferation syndrome, autoimmune neutropenia and thrombocytopenia in partial Di George syndrome: efficacy of rh G-CSF and prednisone.
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[Evaluation of the effectiveness of gastro-protected proteoferrin in the therapy of sideropenic anemia in childhood].
The clinical efficacy and tolerability of gastroprotected ferritin were assessed in children affected by iron deficiency and/or sideropenic anemia. Forty-seven children with iron-deficiency and/or sideropenic anemia were included in the study and were treated with gastroprotected ferritin at a dose of 4-5 mg/kg/day per os for 4 months. Only 33 children correctly completed the entire treatment cycle, achieving a marked improvement of blood parameters (increased Hb, accompanied by higher levels of sideremia and in particular ferritin, with a contemporary decrease in erythrocytic protoporphyrin and transferrinemia) and clinical symptoms, especially pallor, anorexia, debility, somnolence, hyperactivity, disturbed sleep and excessive sweating. Of the remaining 14 children, 9 failed to present for the planned control after the 4 months of therapy, 3 abandoned therapy due to difficulties of assumption and 2 because of intolerance phenomena, such as nausea and diarrhoea. In conclusion, gastroprotected proteoferrin is efficacious and well tolerated in the treatment of iron deficiency in children.
Survival and causes of death in thalassaemia major.
Survival and causes of death were studied in 1087 Italian patients with thalassaemia major who were born on or after Jan 1, 1960. At the age of 15 years, the Kaplan-Meier estimate of survival after the first decade of life was 80.6% for subjects born in 1960-64, 84.2% for those born in 1965-69, and 96.9% for those born in 1970-74. At the age of 20 years, survival from the age of 10 was 59.1% for patients born in 1960-64, and 70.2% for those born in 1965-69; at 25 years, survival from the age of 10 was 40.7% in the 1960-64 cohort. Overall survival from birth for patients born in 1970-74 was 97.4% at 10 years, and 94.4% at 15 years. The most common cause of death was heart disease, followed by infection, liver disease, and malignancy.
[Monosomy 5 (-5), deletion of the long arm of chromosome 6 (6q) and acquisition of a chromosome 21 (+21) in a boy with acute leukemia at high-risk].
The chromosomal abnormality 6q-, associated with acute lymphatic leukemia, is often found both in T cell form and in non T non B cell forms. The absence of chromosome -5, frequently associated with acute non-lymphatic leukemia of the adult, has been rarely found in the acute non-lymphatic leukemia of the child. Trisomy of chromosome 21 is the most associated with acute non-lymphatic leukemia of the adult, has been rarely found in the acute non-lymphatic leukemia of the child. Trisomy of chromosome 21 is the most frequent alteration found in children and adult with acute lymphatic leukemia. In a child (aged 7) affected by acute lymphatic leukemia the karyotype analysis showed simultaneously the presence of the 3 above mentioned abnormalities. It will be important to evaluate later on how the association of monosomy 5 with the deletion of chromosome's 6 long arm and with an acquired chromosome 21, the last two being indexes of a favourable prognosis, can influence the clinical course of the disease.
Survival in thalassemia with conventional treatment.
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[Glycerol erythrocyte lysis test in the 1st month of life in neonates at term and premature infants].
Foetal erythrocytes have an enhanced resistance to osmotic haemolysis that they retain for the first 5 days after birth, particularly in premature newborns. The erythrocyte fragility test was used to study the increased resistance to osmotic haemolysis in 155 healthy newborns (50 NGA born to term, 55 NGA premature and 50 SGA born to term) as well as 31 newborns (20 born to term and 11 premature) with aspecific hyperbilirubinaemia. The drug used was glycerol which is specific in its reaction to alterations in erythrocyte membrane stability and made it possible to assess changes arising in the first month after birth, both naturally and in response to phototherapy. The results show a much higher resistance in neonatal than adult erythrocytes that gradually decrease during the first month after birth without, however, falling to adult levels. No changes in resistance attributable to the phototherapy adopted in newborns with hyperbilirubinaemia were encountered. The glycerol test proved extremely sensitive in the diagnosis of neonatal haemolytic anaemias easy to use so that congenital spherocytosis can be identified earlier than is otherwise possible.
Coagulation contact phase factors and inhibitors in beta-thalassemia major children.
Selected hemostatic parameters of 23 children affected by beta-thalassemia major were studied and compared to an age- and sex-matched group. Plasma prekallikrein level was reduced in all patients, splenectomized or not. In splenectomized patients, platelet count and in vitro platelet aggregability were significantly increased and Protein C was slightly increased. The activated partial thromboplastin time was prolonged and the normotest reduced. Finally, a reduction in the plasma levels of fibrinogen and of vitamin K-dependent proteins, including the antithrombotic Protein C, was observed in nonsplenectomized patients. Our data indicate that the hemostatic system in patients with thalassemia major may be altered. The relationship between these laboratory changes and clinical manifestations remains to be established.
[Screening for thalassemia and/or iron deficiency in a school-age population in the community of Modugno (Bari)].
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