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H Z Grotto

Publications and source records attributed to H Z Grotto.

13 recordsLinked to original sources
PubMed1999

Soluble transferrin receptor in sickle cell diseases: correlation with spleen function.

OBJECTIVE: To correlate spleen function with soluble transferrin receptor (sTfR) levels and red cell ferritin (RCF) values in patients with sickle cell diseases. DESIGN: Prospective study. LOCATION: University Hospital, School of Medical Sciences, State University of Campinas; a tertiary hospital. PARTICIPANTS: 60 patients with sickle cell diseases, in a steady state, who had not received blood transfusions for 3 months; 28 normal individuals with no clinical or laboratory signs of anemia. MEASUREMENTS: Determination of serum iron, transferrin iron-binding capacity, serum ferritin, RCF and sTfR. Evaluation of spleen function: erythrocytes with pits were quantified. RESULTS: Patients with sickle cell anemia had sTfR levels significantly higher than in normal individuals or those with HbSC (p=0.0001) and there was an inverse correlation between sTfR and fetal Hb (p=0.0016). RCF values were significantly higher in sickle cell anemia patients than in normal individuals or those with HbSC (p=0.0001), and there was a correlation between RCF and pitted erythrocytes (p=0.0512). CONCLUSION: The association between sTfR and fetal Hb confirms the contribution of fetal Hb to improving the hemolytic state by minimizing the consequent reactive erythrocyte expansion. High sTfR levels are not related to the degree of spleen function deficiency seen in sickle cell disease patients. The deficiency in the exocytosis process of the spleen occurring in sickle cell anemia patients may contribute to their accumulation of RCF.

Adult↗
PubMed1994

Hb Lepore Baltimore (delta 50Ser beta 86Ala) identified by DNA analysis in a Brazilian family.

Genomic DNA from two Brazilian hemoglobin (Hb) Lepore heterozygotes of Italian ancestry have been studied in order to identify the Hb Lepore type and to sequence the breakpoint region. The two genes were sequenced after PCR amplification and had the delta globin sequence up to exon 2 codon 68 while the first specific base for the beta globin gene was at codon 86 of the second exon; between the two ends, they had 51 base pairs in common with the delta and beta globin genes. These data indicate that the mutation was of the Hb Lepore Baltimore type. The Lepore chromosome haplotype was different from that previously described in members of a Spanish family with Hb Lepore Baltimore. These data suggest that independent mutations have given rise to Hb Lepore Baltimore in different regions of the world.

Anemia↗
PubMed1993

Pattern of nucleolar organizer regions in human leukemic cells.

The pattern of nucleolar organizer regions in bone marrow smears of 40 patients with acute myeloid leukemia (AML), 27 with acute lymphoid leukemia (ALL) and 15 control cases were studied. All normal blasts as well as leukemic blasts revealed at least one cluster of AgNORs. In AML the mean number of clusters (2.87 +/- 0.48) and dots (0.23 +/- 0.17) per cell was significantly lower than in normal myeloblasts (3.27 +/- 0.14 resp. 0.34 +/- 0.12). In ALL the number of clusters (2.82 +/- 0.62) was not significantly different from that of AML blasts. While in all control cases the distribution of the number of clusters per cell within a case could be approximated by a modified Poisson equation, this did not hold for 9 ALL and in 13 AML patients. The histogram of those cases showed a lower dispersion. In ALL, but not in AML, the number of clusters per cell correlated with the peripheral leukocyte count. The present results strengthen the concept that clusters and dots should be regarded as separate entities and suggest that their pattern is related to duration of cell cycle as it has been shown in other tissues.

Adolescent↗
PubMed1991

Hyposplenism in AIDS.

Explore the source record for details and available documents.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Nucleolar organizer regions in normal hematopoiesis: relationship to cellular proliferation and maturation.

The pattern of nucleolar organizer regions (NORs) in normal interphase hematopoietic cells was studied employing the Ag-NOR technique in bone marrow smears. A characteristic pattern of clusters and dots was observed for each cell type. The presence of clusters correlated with cells in proliferation, while in non-proliferating cells only dots were seen. In granulopoiesis, as in erythropoiesis, the number of clusters was highest in the most immature cells, decreasing as maturation took place. Conversely, the number of dots was lowest in the most immature cells, increasing with maturation. In the mature endstage cells, only few dots were seen. The results justify separate counting of clusters and dots, which probably represent different physiologic activities. The present study offers basic data for the study of benign and malignant hematologic disorders where alterations of cellular proliferation and/or maturation occur.

Bone Marrow↗
PubMedDate not supplied

Comparison of red cell distribution width and a red cell discriminant function incorporating volume dispersion for distinguishing iron deficiency from beta thalassemia trait in patients with microcytosis.

The red cell distribution width (RDW), and another red cell discriminant function incorporating RDW (MCV2 x RDW/Hgb x 100) were determined in a group of 30 patients with iron deficiency anemia, 30 patients with beta thalassemia trait, and 30 normal subjects. Both RDW and (MCV2 x RDW/Hgb x 100) mean values were significantly higher in iron deficiency anemia than in beta thalassemia trait (p < 0.001). Taking RDW equal or above 21.0 percent among microcytic anemia patients, we identified correctly 90.0 percent of patients with iron deficiency anemia. The sensitivity and specificity of the test were 90.0 percent (IC 95 percent: 0.75-0.98) and 77.0 percent (IC 95 percent: 0.60-0.88), respectively. RDW values below 21.0 percent identified correctly 77.0 percent of beta thalassemia trait with a sensitivity and a specificity of 77.0 percent (IC 95 percent: 0.60-0.88) and 90.0 percent (IC 95 percent: 0.75-0.96), respectively. Taking values of (MCV2 x RDW/Hgb x 100) above and below 80.0 percent as indicative of iron deficiency and beta thalassemia trait, respectively, we identified correctly 97.0 percent of those patients in each group. Both sensitivity and specificity were 97.0 percent (IC 95 percent: 0.84-0.99). These results indicated that the red cell discriminant function incorporating volume dispersion (MCV2 x RDW/Hgb x 100) is a highly sensitive and specific method in the initial screening of patients with microcytic anemia and is better than RDW in differentiating iron deficiency anemia from beta thalassemia trait.

Anemia, Iron-Deficiency↗
PubMedDate not supplied

[Clinical aspects and complete blood counts in children exposed to HIV-1: comparison between infected patients and seroreverters]

OBJECTIVE: To analyze the evolution of clinical and hematological aspects of children exposed to the vertical transmission of HIV-1, comparing infected patients with uninfected ones or seroreverters. METHODS: Prospective, descriptive, longitudinal study. We analyzed 79 children born from HIV-1 infected mothers, under clinical follow up from March, 1996 until November, 1997, at the Immunodeficiency division of the Hospital de Clínicas da Unicamp (State University Hospital of Campinas). RESULTS: Failure to thrive was observed in both groups, but was greater among seroreverters. Among the infected children, 23 mothers did not use AZT during pregnancy, 16 of them (61.5%) had been breastfed, four were classified into clinical category N, seven into A and fifteen into B. Clinical manifestations in patients younger than one year were seen in 18 infected children (69.2%). Anemia was observed in 73.1% of the infected group and in 41.5% of the seroreverters (P<0.008). The comparison between the groups showed that the most common hematologic alterations in the infected children was microcytosis and hypochromia (P<0.05), lymphopenia between 15 and 18 months (P<0.05), monocytosis between 9 and 12 months (P<0.05) and a tendency towards high ferritin levels, with no statistical significance. CONCLUSIONS: Microcytic and hypochromic anemia were observed in both groups: iron deficiency in the uninfected children, and chronic disease anemia in the infected ones. The infected children presented with monocytosis and lymphopenia at an earlier stage.

Journal Article↗
PubMedDate not supplied

Pattern of splenic phagocytic function in Brazilian patients with sickle cell disease.

The splenic function measured by the counts of "pitted" erythrocytes has been assessed in 87 patients with sickle cell disease (59 homozygotes for hemoglobin S (SS), 14 double heterozygotes for Hb S and beta zero thalassemia (S/beta zero thal), 4 S/beta+ thal and 10 SC patients) in Southeast Brazil. Results showed a progressive increase in pit counts according to age. The reduction pattern in the splenic function was similar for the SS, S/beta zero thal and S/beta+ thal patients, and over the age of 12 almost all patients presented counts compatible with severe splenic hypofunction. Patients with SC hemoglobinopathy presented slower development of hyposplenism and lower levels of pit counts even in advanced ages. Except for S/beta+ thal patients, the developmental pattern of hyposplenism was not different from that reported among patients in the United States and Jamaica.

Adolescent↗
PubMedDate not supplied

[Differentiation between heterozygotic beta-thalassemia and iron deficiency anemia].

Several laboratory tests have been proposed for the differentiation of beta thalassemia from iron deficiency, including decision functions based on red blood cells indices generated by electronic cell counters. The accuracy of these screening methods was assessed in 192 patients with microcytosis known to be secondary to beta thalassemia minor and 72 patients with iron deficiency. The functions evaluated were: 1) discriminant function of England an Fraser: MCV--(5xHb)--RBC--8.4; 2) ratio of MCH/RBC; 3) ratio of MCV/RBC; 4) ratio of (MCV)2 x MCH and 5) the erythrocyte count. The discriminant function of England and Fraser showed the highs, percentage of correct distinction between iron deficiency and beta thalassemia minor, although diagnosis errors occurred in 10%. Mentzer ratio MCV/RBC detected all cases of beta thalassemia but was a poor index for iron deficiency detection. The tested discriminant function, with exception of the Mentzer ratio, although not sufficiently accurate for definitive diagnosis, appears to be a useful technique in the initial screening of patients with microcytosis.

Adult↗