Plasma zinc levels inversely correlate with vascular cell adhesion molecule-1 concentration in children with sickle cell disease.

Zinc deficiency has been implicated in impaired cell-mediated immunity of children with sickle cell disease (SCD). However, its influence on the expression of vascular cell-adhesion molecule-1 (VCAM-1) on endothelial cells, a protein involved in vasoocclusion, has not been previously investigated. We therefore measured (soluble) sVCAM-1 and zinc in 76 SCD children and 96 non-SCD children, mean age 7.

Differential effects of hydroxyurea and zileuton on interleukin-13 secretion by activated murine spleen cells: implication on the expression of vascular cell adhesion molecule-1 and vasoocclusion in sickle cell anemia.

Background: Interleukin-13 (IL-13), a TH2 cytokine, upregulates the expression of vascular cell adhesion molecule-1 on endothelial cells, a factor involved in vasoocclusion in sickle cell disease (SCD). Hydroxyurea improves clinical status of SCD patients in part by induction of fetal hemoglobin. Its effect on IL-13 secretion has not been investigated.

Gamma/delta T-cell lymphoma as a recurrent complication after transplantation.

We present a case of gamma-delta (gammadelta) T-cell lymphoma as a recurrent event in a pediatric liver transplant recipient. Liver transplantation was performed during infancy in an 18-month-old black girl because of cryptogenic cirrhosis. The patient received immunosuppression with cyclosporine and prednisone.

Effects of iron deficiency on the secretion of interleukin-10 by mitogen-activated and non-activated murine spleen cells.

Interleukin (IL)-10 plays crucial regulatory roles in immune responses by inhibiting the secretion of several cytokines (IL-2, IL-12, interferon-gamma (IFN-gamma)) and lymphocyte proliferation. Iron deficiency, a public health problem for children, alters these immune responses. To determine whether these changes are related to altered IL-10 secretion, we measured IL-10 in 24 and 48 h supernatant of spleen cell cultures from iron deficient (ID), control (C), pairfed (PF), and ID mice fed the control diet (iron repletion) for 3 (R3) and 14 (R14) days (d, n = 12/group).

Blast crisis of chronic myeloid leukemia: diagnosis prompted by T(8;9).

T(8;9) is a relatively new translocation that has been reported in a few patients with chronic myeloid leukemia (CML) but never in acute myeloid leukemia (AML). We report here a patient who presented with AML with t(8; 9). He lacked the Philadelphia chromosome but tested positive for the gene by the polymerase chain reaction method.

Association between increased levels of TNF-alpha, decreased levels of prealbumin and retinol-binding protein, and disease outcome.

We determined whether there is an association between tumor necrosis factor-alpha (TNF-alpha), undernutrition [prealbumin (PA) <160 mg/L, retinol binding protein (RBP) <30 mg/L], disease stage, outcome (death or survival), and race in children with leukemia. TNF-alpha, PA, and RBP were measured in 52 patients (0.8 to 17 years old): 18 African Americans, 34 whites; 27 newly diagnosed (ND), and 25 in clinical remission (CR).

Lack of difference in iron status assessed by soluble transferrin receptor between children with cerebral malaria and those with non-cerebral malaria.

We conducted this study to determine whether children with cerebral malaria are less likely to have tissue iron deficiency than those with non-cerebral malaria. Iron status was assessed by soluble transferrin receptor (sTfR), serum ferritin, and haemoglobin in 44 Zaïrian children: 15 with cerebral malaria, 14 with non-cerebral malaria, and 15 without malaria (age range 0.5-16 years).

Increased circulating levels of soluble HLA class I heterodimers in patients with sickle cell disease.

This study examined the presence of a persistent state of low-grade inflammation in sickle cell anemia patients by measuring circulating sHLA-I heterodimers and C-reactive protein during the steady state and after recent crises. Thirty-nine pediatric sickle hemoglobinopathy patients were studied during the steady state and 11 patients were evaluated within 1 month of a painful crisis. A disease severity score was generated for each patient, and soluble HLA-I (sHLA-I) and C-reactive protein levels were determined.

Tumor necrosis factor alpha in children with sickle cell disease in stable condition.

Tumor necrosis factor alpha (TNF-alpha) is known to induce wasting in humans and animals. This study was undertaken to determine TNF-alpha concentrations in children with sickle cell disease (SCD) and whether high TNF-alpha levels are more likely to be present in children with growth deficits, infection, or pain crisis. Tumor necrosis factor alpha was measured using enzyme immunoassay in 143 blood samples obtained from 101 children.

Hemoglobin A2 levels in healthy persons, sickle cell disease, sickle cell trait, and beta-thalassemia by capillary isoelectric focusing.

Simultaneously measuring major and minor hemoglobin (Hb) variants by capillary isoelective focusing, we obtained HbA2 intervals in healthy volunteers (n = 412) (reference value) and patients with HbS or beta-thalassemia. We classified normal HbA2 reference intervals into three age groups: 5 months or younger (1.2% +/- 1.

Immune thrombocytopenia and hemolytic anemia associated with Hodgkin disease.

Purpose: We discuss an unusual clinical presentation of Hodgkin disease with immune thrombocytopenia and autoimmune hemolytic anemia.

Patients And Methods: A 4-year-old boy presented to us with a large anterior mediastinal mass, thrombocytopenia, and Coombs' positive hemolytic anemia refractory to transfusion therapy. Biopsy of the anterior mediastinal mass was possible only after administration of intravenous immunoglobulin to raise the platelet count.

Serum transferrin receptor concentrations in women with mild malnutrition.

We determined the influence of undernutrition on blood soluble transferrin receptor (sTfR) concentrations, an indicator of iron deficiency, in 99 Zairean women (aged 16-45 y) without inflammation. They were recruited during a survey on iron deficiency in rural Bas-Zaire. sTfR was measured by enzyme immunoassay, and indicators of nutritional status [albumin, transthyretin (or prealbumin), and retinol binding protein] were measured by radial immunodiffusion.

Soluble transferrin receptor as an index of iron status in Zaïrian children with malaria.

This study was designed to evaluate soluble transferrin receptor (sTfR) as an index of iron status in 0.5-16-year-old Zaïrian children: 17 with symptomatic malaria, 8 with asymptomatic malaria, and 15 controls. sTfR was also measured in 20 plasma samples obtained from iron sufficient laboratory employees.

Assessment of iron status of Zairean women of childbearing age by serum transferrin receptor.

Soluble transferrin receptor (sTfR), previously shown to be a sensitive indicator of tissue iron deficiency, was used to assess iron status of 104 Zairean women (69 lactating, 19 pregnant, and 16 nonpregnant, nonlactating women). Thirteen iron-sufficient female laboratory employees were also studied. Mean sTfR concentrations were higher in pregnant women (9.

Reference levels of acute phase reactant proteins in healthy Zairean women in the reproductive age group.

Acute phase proteins (APP) are plasma proteins secreted by the liver following inflammation. APP reference levels are unknown for healthy female Zaireans. The purpose of this study was to determine: (a) the reference levels of alpha 1-acid glycoprotein (AGP), C-reactive protein (CRP), ceruloplasmin (Cp), and haptoglobin (Hp) in healthy Zairean adult women, and (b) whether the levels are different between lactating (Group I), pregnant (Group II) and non-pregnant/non-lactating women (Group III).

Recombinant tissue plasminogen activator (rt-PA) for veno-occlusive liver disease in pediatric autologous bone marrow transplant patients.

The pathogenesis of veno-occlusive disease (VOD) of the liver appears to be secondary to endothelial damage of terminal hepatic venules, which leads to activation of the coagulation cascade, fibrin deposition, and eventual fibrous obliteration of the hepatic venules. Patients with VOD usually present with jaundice, hepatomegaly, weight gain, and ascites. This complication is usually associated with a high mortality rate.

Usefulness of serum ferritin levels in the assessment of iron status in non-pregnant Zairean women of childbearing age.

We studied the usefulness of serum ferritin for the assessment of iron deficiency (ID) or ID anaemia (IDA) of 186 lactating and 27 non-lactating Zairean women (15-45 years old). Haemoglobin (Hb), serum iron (SI), total iron binding capacity (TIBC), and transferrin saturation (TS) were also measured. Participants were recruited in rural Bas-Zaire State in the summers of 1986 and 1989.

The efficacy of ticarcillin-clavulanate and gentamicin as empiric treatment for febrile neutropenic pediatric patients with cancer.

The therapeutic efficacy of ticarcillin-clavulanate and gentamicin was assessed in 56 pediatric cancer patients with 95 febrile neutropenic episodes. The mean temperature of these children on admission was 102 degrees F (range 101 degrees to 106 degrees F), with a median absolute neutrophil count of 60. The median hospital stay was 7 days.

An epidemiological study of hemoglobin levels and prevalence of anemia in young children from Bas-Zaire.

A population of 183 Zairean infants and young children (0.5 to 48 months old) who lived in rural Bas-Zaire has been examined in regard to hemoglobin (Hb), hematocrit (Hct) and mean corpuscular hemoglobin concentration (MCHC). The mean Hb was 9.

Current treatment and prognosis of Wilms' tumor.

Thanks to the nationally and internationally organized efforts refinements in the therapy of Wilms' tumor have allowed restructuring and reduction of therapeutic strategies. A decrease in tumor relapse and long term sequelae are the immediate goals which will no doubt be the result of tailoring chemotherapy and radiation therapy exposure together with improved surgical techniques and outstanding supportive care.

Retinoblastoma: a review.

Retinoblastoma is the most common primary ocular malignancy in childhood, usually occurring below the age of five. Recent advances in molecular biology have enabled understanding of retinoblastoma tumorigenesis as well as detecting carriers of the mutant retinoblastoma allele. It is possible now to predict prenatally whether a child carries the retinoblastoma genome.

Bone marrow transplantation: a children's hospital of New Orleans experience.

Bone marrow transplantation has become an important therapeutic strategy in the treatment of pediatric malignancies and certain congenital disorders. We initiated a bone marrow transplant program at Children's Hospital in New Orleans at the start of January 1989. Both allogeneic and autologous bone marrow transplantations have been performed.

The effect of recombinant GM-CSF and G-CSF on the bone marrow cells of children with acute lymphoblastic leukemia.

Colony stimulating factors (CSFs) are glycoprotein hormones that regulate growth and differentiation of hematopoietic progenitor cells. Their use to stimulate granulocyte precursors during periods of neutropenia in patients with acute myeloid leukemia (AML) is limited by their concomitant stimulation of the proliferation of myeloblasts. The effects of these agents on leukemic lymphoblasts is not entirely known.