Search PubMed⌕ Search

Biomedical subjects

G Sirchia

Publications and source records attributed to G Sirchia.

At least 163 records · Page 9Linked to original sources
PubMed1989

Relative iron deficiency in hereditary spherocytosis.

Seventy-three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, transferrin saturation, and serum ferritin than normal matched controls and normal zinc protoporphyrin (ZnPP) levels. On the contrary, nonsplenectomized patients presenting with mild to severe anemia had higher red cell ZnPP concentrations than both splenectomized subjects and matched normal controls. ZnPP in nonsplenectomized patients correlated inversely with Hb concentration, mean corpuscular volume (MCV), mean red cell hemoglobin concentration (MCHC), transferrin saturation, and serum iron, and directly with reticulocyte count. At multiple regression analysis only Hb concentration was a significant explanatory variable for high ZnPP. The authors conclude that a number of nonsplenectomized HS patients have relative iron deficiency primarily because of expansion of erythropoiesis caused by anemia.

Adolescent↗
PubMed1989

Evaluation of platelet concentrates prepared from buffy coats and stored in a glucose-free crystalloid medium.

Comparison was made between platelet concentrates prepared from pools of buffy coats removed from standard blood donations and stored in a glucose-free, commercially available crystalloid solution (BC-PCs) and standard platelet concentrates prepared from platelet-rich plasma (PRP-PCs). Platelet yield in BC-PCs and PRP-PCs was 59 and 75 percent of donated platelets, respectively. The number of total white cells in 1 BC-PC unit, prepared from a pool of 7 buffy coats, was 21 x 10(6), i.e., 50 times lower than that of 7 units of PRP-PCs. The in vitro values of adequate platelet quality were maintained for 10 days in BC-PCs stored in 1000-mL polyolefin bags. Prolonged bleeding times were reduced or corrected in three of three thrombocytopenic leukemic patients evaluated before and after transfusion of stored BC-PCs. Pretransfusion and 1- and 24-hour posttransfusion median platelet counts in 57 leukemic recipients during 4 months of routine transfusion of BC-PCs (n = 93) were 14, 35, and 27 x 10(9) per L, while those of PRP-PCs (n = 246) were 13, 37, and 31 x 10(9) per L, respectively. No reactions to BC-PCs were reported, but a 1.3 percent rate of reaction to PRP-PC transfusions was reported. This study indicates that BC-PCs are a good alternative to PRP-PCs for platelet support of thrombocytopenic patients.

Adult↗
PubMed1989

Sensitivity and predictive value of serum ferritin and free erythrocyte protoporphyrin for iron deficiency.

The sensitivity and predictive value of serum ferritin (SF) and free erythrocyte protoporphyrin (FEP) for iron deficiency (ID) was evaluated by studying 272 subjects with uncomplicated ID (174 with anemia and 98 without) in whom diagnosis was confirmed by the response to iron supplementation. Overall, the sensitivity, at 95% specificity, was 82% (79% in women, 94% in men) for SF and 61% (60% in women, 65% in men) for FEP. The sensitivity varied as a function of hemoglobin values, dropping from over 90% for both tests in the case of severe anemia, to approximately 70% for SF and less than 50% for FEP in the absence of anemia. The predictive value decreases more sharply for FEP than for SF with increasing hemoglobin levels. It is concluded that SF is preferable to FEP for the detection of ID, particularly in the absence of anemia. However, owing to the unsatisfactory predictive value at low prevalence, SF should be used as a screening test for ID without anemia only when the prevalence is at least 20%.

Adult↗
PubMed1988

Erythrocyte pyruvate kinase deficiency: 11 new cases.

Eleven new cases of red cell pyruvate kinase (PK) deficiency with congenital haemolytic disease from 10 unrelated Italian families were characterized using the methods recommended by the International Committee for Standardization in Haematology (ICSH). All patients were double heterozygotes for the PK gene. The 10 variants were designated PK 'Lecce,' 'Parma,' 'Verona,' 'Milano,' 'Soresina,' 'Macerata,' 'Sassari,' 'Genova,' 'Mantova' and 'Brescia.' PK 'Sassari' was associated with glucose-6-phosphate dehydrogenase deficiency in two siblings. All mutants displayed multiple biochemical abnormalities except for PK 'Lecce' that only showed decreased red cell PK activity. No relation was found between the severity of anaemia and either the residual PK activity or specific biochemical enzyme abnormalities. Increased serum ferritin levels were detected in most of the patients, suggesting the need for systematically monitoring iron status in this disease.

Adolescent↗
PubMed1987

Single donor plasma in therapeutic plasma exchange for myasthenia gravis.

Thirty-seven myasthenic patients were treated with therapeutic plasma exchange (TPE) on different performance schedules using either random or single donor plasma. The two schedules had the same effects in terms of efficacy but single donor replacement gave significantly less side effects in the short and long term. A two exchange procedure on alternate days associated with immunosuppression seems to be an optimal schedule for the treatment of myasthenic patients. A single donor two-exchange on alternate days procedure appears to be safe, useful and can be repeated in patients who need frequent TPE.

Adolescent↗
PubMed1986

The clinical importance of leukocyte depletion in regular erythrocyte transfusions.

Antilymphocyte, antigranulocyte and antiplatelet alloantibodies, T lymphocyte subsets, expression of HLA-DR antigens on T lymphocytes and NK cell function were determined in 11 homozygous beta-thalassemic children multitransfused ab initio with Erypur-filtered leukocyte-free red cell units (group A) and in 13 similar children multitransfused with standard packed red cell units (group B). No antibodies were found in group A patients, whereas 69% of group B patients were immunized. The two groups did not differ significantly with regard to the other test results. Considered together, thalassemia patients showed a percentage of T4+ cells and a NK cell function that were significantly lower than those found in a reference group of 16 healthy male blood donors. Thalassemics moreover showed a higher than normal percentage of T3+, T4+ and T8+ cells expressing HLA-DR antigens. The results indicate that leukocyte-free red cells should be the treatment of choice for prospective recipients of multiple transfusions, since they are capable of preventing (or delaying) the production of alloantibodies against leukocytes and platelets. From the data of the present study, it does not seem that the transfusion of leukocyte-free red cells is capable of preventing the abnormalities of some immunological tests that occur in some multitransfused patients. Further investigations, however, are needed to draw conclusions on this problem.

Adult↗