Enterobacter cloacae in an Italian Neonatal Intensive Care Unit: pattern of drug resistance compared with an international database (SENTRY Antimicrobial Surveillance Program).
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Letter↗
Biomedical subjects
C Bottura
Publications and source records attributed to C Bottura.
Hemichrome formation during hemoglobin Zurich denaturation.
Electron paramagnetic resonance (EPR) spectrum of hemoglobin Zurich, after oxidation, storage and heating, showed several absorption derivatives in the high field region (g approximately equal to 2) which are indicative of hemichrome formation. Characteristic visible spectra of hemichromes were observed for oxidized Hb Zurich and for its spontaneous precipitate. The proportional increase of EPR signals at g approximately equal to 2 and decrease at g = 6.37, the constant ratio of absorbance at 540 nm to 280 nm during heating, and the similarity of this ratio for spontaneously precipitated HbA and for Hb Zurich indicate that heme is not lost during the first steps of Hb Zurich denaturation.
Electron Spin Resonance Spectroscopy↗
The association of Hb Stanleyville II with alpha thalassemia and Hb S.
Two unrelated families are described which include two heterozygotes for Hb Stanleyville II, two compound heterozygotes for Hb Stanleyville II and Hb S, and one compound homozygote for the two abnormal hemoglobins. The homozygote did not produce normal alpha chains, the abnormal chains accounted for 32-35% of the total alpha chain of four heterozygotes, and the alpha/non-alpha synthetic ratios were in the range 0.59-0.77. These data demonstrate the presence of an alpha-thalassemia gene linked to the alpha-Stanleyville II mutation.
Adult↗
Splenic function in haemophilia.
Studies of splenic function were carried out on 17 haemophilic patients over 9 years of age, and 20 control patients. The clearance of autologous heat-damaged 99mTc-labelled erythrocytes from circulation and into the spleen was measured: the spleen area by scintillation scanning, and the enumeration of pitted erythrocytes by direct interference microscopy. Splenic enlargement was observed in 10 patients (59%). On the basis of the clearance half-time, splenic function was normal in 3/13 (23%) and hyperactive in 9/13 (69%) patients. One heavily transfused patient had a hypoactive spleen with long clearance half-time, slow splenic uptake of radioactivity and high pit counts. These results demonstrate that the spleen of haemophiliacs is usually enlarged and functionally abnormal.
Adolescent↗
Unbalanced globin synthesis in bone marrow of beta-thalassemia heterozygotes.
Globin-chain synthesis was studied in the peripheral blood and bone marrow of eight beta-thalassemia heterozygotes. Whole cell globin was prepared immediately after the labeling experiment. Chain separation of the bone marrow globin of each case was carried out with both the original material and after filtration on Sephadex G 100. The beta/alpha ratios obtained were (mean +/- SD): 0.46 +/- 0.05 in the peripheral blood, 0.61 +/- 0.06 in the unfiltered bone marrow globin, and 0.52 +/- 0.05 in the bone marrow globin after gel filtration. The results show that beta-thalassemia heterozygotes have a similar beta-chain deficiency in reticulocytes and bone marrow cells, provided whole cell globin is used, which avoids the removal of free alpha-chains.
Bone Marrow↗
Balanced globin synthesis by Hb K Woolwich heterozygotes.
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Child, Preschool↗
Red cell pits appear preferentially in old cells after splenectomy.
The percentage of erythrocytes with pits in the peripheral blood after splenectomy was followed in 4 patients. The pit counts started to increase soon after surgery and were above 20% on the 20th postsplenectomy day. The results demonstrate that the majority of pits developed in circulating erythrocytes. Two other patients with autoimmune hemolytic anemia who had failed to respond to splenectomy presented an increase of pit counts from 5.5-13% to 29.8-47.4%, accompanying an increase of erythrocyte life-span during corticosteroid treatment. The data indicate that pits are formed preferentially in older erythrocytes.
Anemia, Hemolytic, Autoimmune↗
Discrepancy between pit counting and spleen function tests in nutritional anemias and hemoglobinopathy C.
An increased percentage of erythrocytes with crater-like indentations (pits) when viewed under interference contrast microscopy is accepted as indicative of splenic hypofunction. The percentage of pitted erythrocytes was above the upper limit of the normal range (0.0-3.2%) in 6 patients with megaloblastic anemia (4.6-22.6%), 8 patients with iron deficient anemia (5.6-21.0%) and 6 patients with HbC diseases (12.5-45.0%). However, the spleen function was normal or hyperactive when evaluated by the rate of removal from the circulation of heat-damaged 99mTc-labeled autologous erythrocytes. Thus, in these diseases, there is a discrepancy between pit counting in the peripheral blood and other spleen tests.
Adolescent↗
Aspects of splenic hypofunction in old age.
The weight of the human spleen decreases in old age. The percentage of erythrocytes with pits observed by interference contrast microscopy was significantly higher in 39 elderly subjects (median 4.2%, range 1.2%-14.6%) than in 34 younger controls (median 1.4%, range 0.6%-6.2%). Further evidence of splenic hypofunction was obtained from 7 elderly people with increased pit counts: the removal from circulation and accumulation into the spleen of heat-damaged autologous erythrocytes was abnormally slow. However, no difference in the spleen area of the same subjects measured by scintillation scanning was observed in comparison with normals. Thus, the weight reduction of spleen in old age is accompanied by a decrease in splenic function.
Adolescent↗
T-lymphocyte subpopulations in the peripheral blood and bone marrow of patients with aplastic anemia.
A decrease in the absolute number of total lymphocytes, OKT3+ and OKT4+ lymphocytes, and a normal number of OKT8+ lymphocytes were found in the peripheral blood of patients with aplastic anemia. The OKT4:OKT8 ratio was decreased in patients due to a reduction in the percentage of OKT4+ cells and 3 out of 18 patients had a ratio less than 1. The values of the OKT4:OKT8 ratio were not associated either with the severity of the disease or with treatment with androgens. There was no correlation between the OKT4:OKT8 ratio and the number of transfusions received by patients. On the other hand, studies performed with bone marrow lymphocytes showed that the OKT4:OKT8 ratio for both patients and controls was lower than that of the peripheral blood. Since the ratio of OKT4:OKT8 cells in aplastic and control bone marrow was similar no direct pathogenic role can be assigned to the marrow for the imbalance detected in the peripheral blood.
Adolescent↗
Antibody-dependent cellular cytotoxicity in some lymphoreticular diseases.
Antibody-dependent cellular cytotoxicity mediated by K cells against chicken erythrocytes was measured in 113 patients with malignant lymphoreticular disorders and compared with 230 controls. The results were expressed as the specific cytotoxicity of a fixed number of cells and also by cytotoxic capacity, which measures the number of cytolytic units in 1 ml of blood. The values for cytotoxic capacity were normal in the group of untreated patients with non-Hodgkin's lymphomas, multiple myeloma or chronic lymphocytic leukemia and in most of the patients with Hodgkin's disease or acute lymphoblastic leukemia. However, decreased specific cytotoxicity was observed in these same lymphoid leukemia patients, which may be due to dilution of effector cells. The effect of chemotherapy in reducing K-cell activity is more evident in patients with multiple myeloma, followed by patients with Hodgkin's disease, and finally by patients with non-Hodgkin's lymphomas. No case of K-cell neoplastic disease was observed in this series.
Antibody-Dependent Cell Cytotoxicity↗
Treatment of sickle cell diseases with aspirin.
The effects of long-term aspirin for the treatment of sickle cell disease were compared with placebo in a double-blind trial completed by 29 patients. Each patient was submitted to a 5-month period of treatment with aspirin (median dose 31 mg/kg/day) and an equivalent period with placebo. No clinical or laboratory differences were observed between the two phases, including the frequency of painful crises and infectious episodes, hemoglobin concentration, PCV, reticulocytes, Hb F, bilirubin, irreversibly sickled cells, filterability of red cell, sickling in vitro and hypoxia-induced potassium loss.
Adolescent↗
T-cell subsets in patients with aplastic anemia.
Abnormalities of lymphocyte subpopulations have been described in patients with aplastic anemia. In the present report we extend these studies by measuring T cell subsets identified by the presence of Fc receptors for IgM (T mu-lymphocyte) and IgG (T gamma-lymphocyte) in 22 patients and in 48 normal controls. The absolute number of T mu and T gamma lymphocytes was normal in the majority of cases. The percentages of T mu cells was increased in 4 cases and decreased in 2; T gamma cells were increased in 6 patients. The levels of serum immunoglobulins did not correlate with the T mu/T gamma ratio. The pathogenesis of aplastic anemia is discussed in terms of these immunological abnormalities.
Adult↗
Dyserythropoiesis in iron deficiency and in beta-thalassemia.
Nuclear and cytoplasmic abnormalities were quantitated in bone marrow erythroblasts from 15 patients with iron deficient anemia, 5 beta-thalassemia homozygotes, 5 beta-thalassemia heterozygotes, 6 S/beta-thalassemia double heterozygotes and 9 controls. The frequency of dyserythropoietic changes in iron deficiency was 11.90 +/- 5.02% (mean +/- SD) which is significantly higher than 3.36 +/- 1.16% obtained for the control group. The degree of dyserythropoiesis was negatively correlated with hemoglobin level (rS = 0.757). The frequency of dyserythropoietic changes obtained for the beta-thalassemia heterozygotes (5.23 +/- 1.45%) and for S/beta-thalassemia (7.13 +/- 2.00%) was elevated compared with the controls (P less than 0.05 and P less than 0.01, respectively). The highest frequency of dyserythropoiesis (19.88 +/- 7.40%) occurred among beta-thalassemia homozygotes. In all cases studied the abnormalities were observed mainly in the late erythroblasts. In addition, a peculiar cytoplasmic inclusion was observed in Leishman-stained bone marrow or peripheral blood erythroblasts from beta-thalassemia homozygotes, which is probably the result of precipitation of excess alpha-chain. This abnormality of thalassemia erythroblasts in Leishman-stained smears had not been previously reported.
Anemia, Hypochromic↗
beta+-Thalassemia intermedia with low HbF.
Two cases of homozygous beta+-thalassemia intermedia have been detected in a Brazilian family of Portuguese and German extraction. The patients are 39 and 43 years old, showed a normal somatic and sexual development and had been transfused only occasionally. Red blood cell morphology was similar to that of thalassemia major, but they had unusually low levels of HbF (5.0% and 6.8%). Globin chain synthesis measured in reticulocytes was in the same range as other beta-thalassemia homozygotes. One or both genes in this family must be a particularly mild beta-thalassemia allele, despite the fact that the heterozygote members of the family presented clinical, hematological, and biochemical features indistinguishable from the typical heterozygotes for the beta-thalassemia trait with high HbA2.
Adult↗
Splenic function in sickle-cell diseases.
Studies of splenic function were carried out on patients with sickle-cell diseases by the measurement of the clearance of autologous heat-damaged 99mTc-labelled erythrocytes from circulation and into the spleen, the spleen area by a scintillation scanning, the enumeration of pitted erythrocytes by direct-interference microscopy, and the percentage of irreversibly sickled cells (ISC) and of cells with Howell-Jolly bodies. All measurements were performed in seven HbS homozygotes, 10 patients with sickle cell beta(0)-thalassaemia (S/beta(0)-thalassaemia), three patients with sickle-cell disease (SC), four AS heterozygotes and 17 controls. Three different patterns of splenic function were observed among the 20 patients with symptomatic sickle-cell diseases: six patients had enlarged hyperactive spleens, four had enlarged hypoactive spleens, and in 10 patients no splenic activity was detected. The percentage of ISC was higher in sickle-cell anaemia than in S/beta (0)-thalassaemia and very low in SC patients. These results would suggest that the spleen goes through similar successive functional stages in the sickle-cell diseases, namely enlargement in the early years of life, which is followed by hypoactivity and finally atrophy. This evolution seems to be faster in sickle-cell anaemia than in S/beta(0)-thalassaemia and SC disease.
Adolescent↗
K-cell activity in aplastic anaemia.
K-cell activity was measured by specific cytotoxicity and cytotoxic capacity in 29 patients with aplastic anaemia. 9 patients had a reduction of the specific cytotoxicity and 12 had a decreased cytotoxic capacity. The decreased cytotoxic capacity correlates with the severity of aplastic anaemia. Some of the possible causes of the reduction of this activity were investigated.
Adolescent↗
Hereditary hemoglobin disorders in a Brazilian population.
A survey of hereditary hemoglobin disorders in a mixed Brazilian population of the northeast of the State of S. Paulo revealed a 5.3% incidence of abnormal phenotypes among 400 schoolchildren, 4.5% among 602 mothers and 2.8% among 606 newborns. The most common findings were AS (1.9%), AC (0.8%) and beta-thalassemia (0.8%) heterozygotes, which amount to 3.5% of the sample. In a second selected population of 1,023 patients of the Hematologic Clinic of the University Hospital and their relatives, 471 cases of hemoglobinopathies were detected. The most frequent anomalies were heterozygous beta-thalassemia (35.2%) and Hb S (32.5%), followed by sickle-cell anemia (13.0%), homozygous beta-thalassemia (4.0%) and sickle-cell/beta-thalassemia (4.0%). Other defects detected were delta-beta-thalassemia, Hb C, Hb Hasharon and Hb A2'. One family with alpha-thalassemia has been identified that included a girl with Hb H disease. The significance of these findings is discussed with regard to the racial origin of the population of this region.
Brazil↗