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Biomedical subjects

T R Kinney

Publications and source records attributed to T R Kinney.

At least 55 records · Page 3Linked to original sources
PubMed1990

Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease.

In 1,814 patients with sickle cell disease who had been transfused, the overall rate of alloimmunization to erythrocyte antigens was 18.6%. The rate of alloimmunization in this group appears to be an explicit function of the number of transfusions received because it increases exponentially with increasing numbers of transfusions. Alloimmunization usually occurred with less than 15 transfusions, although the rate of alloimmunization continued to increase when more transfusions were given. The rate of alloimmunization was less in patients with hemoglobin SC disease and sickle-beta+ thalassemia because these patients had received fewer transfusions. Children less than 10 years old had a slightly lower rate of alloimmunization than patients in other age groups even after correction for the number of transfusions given. Women were more frequently alloimmunized than men; this was largely due to the fact that women received more transfusions than men, but in the age group 16 to 20 years the increase may have been due in part to alloimmunization owing to pregnancy. Forty-five percent of those alloimmunized made antibodies of only one specificity; 17% made four or more antibodies reacting with different antigens. Antibodies to the C and E antigens of the Rh group, the Kell antigen, and the Lewis antigens were most commonly made. These findings may be important in formulating a rational transfusion policy in sickle cell disease.

Adolescent↗
PubMed1990

Long-term management of splenic sequestration in children with sickle cell disease.

We describe our experience with 23 children with sickle cell disease and splenic sequestration crisis, emphasizing our management approaches and the patients' subsequent clinical courses. Our data illustrate that sequestration crisis may occur despite a reduction in hemoglobin S concentration to less than 30% of the total hemoglobin mass. In addition, the risk of recurrent splenic sequestration was similar for patients who received transfusion therapy and for those who were simply observed. We conclude that a short-term transfusion program to prevent recurrent splenic sequestration is of limited benefit.

Anemia, Sickle Cell↗
PubMed1989

Nonblack patients with sickle cell disease have African beta S gene cluster haplotypes.

Of 18 nonblack patients with sickle cell disease, 14 had sickle cell anemia, 2 had hemoglobin SC disease, and 2 had hemoglobin S-beta o-thalassemia. The beta s gene cluster haplotypes that were determined in 7 patients were of African origin and were identified as Central African Republic, Central African Republic minor II, Benin, and Senegal. The haplotype Central African Republic minor II was present on the beta o-thalassemia chromosome in 2 patients. None of 10 patients whose alpha-gene status was determined had alpha-thalassemia-2. These data strongly support the concept that the beta s gene on chromosome 11 of these individuals is of African origin and that the alpha-gene locus on chromosome 16 is of white or native American origin. The clinical severity of the disease in these nonblack patients is appropriate to their haplotype without alpha-thalassemia-2 and is comparable with that of black patients. All persons with congenital hemolytic anemia should be examined for the presence of sickle cell disease regardless of physical appearance or ethnic background.

Adult↗
PubMed1989

Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease.

A study of the natural history of sickle hemoglobinopathies was begun in March 1979. By August 1987, a total of 2824 patients less than 20 years of age were enrolled. There have been 14,670 person-years of follow-up. Seventy-three deaths have occurred. Most of the deaths were in patients with hemoglobin SS. The peak incidence of death was between 1 and 3 years of age, and the major cause in these young patients was infection. Cerebrovascular accidents and traumatic events exceeded infections as a cause of death in patients greater than 10 years of age. There was limited success in identifying risk factors for death. Comparison of this study's overall mortality of 2.6% (0.5 deaths per 100 person-years) with previous reports indicates improvement of survival in US patients less than 20 years of age with sickle hemoglobinopathies. This improvement is most likely due to parental education and counseling about the illness and the early institution of antibiotics in suspected infections.

Adolescent↗
PubMed1988

Childhood hepatic mesenchymoma: successful treatment with surgery and multiple-agent chemotherapy.

Malignant undifferentiated sarcoma (mesenchymoma) of the liver is a rare tumor of childhood, with fewer than 100 cases reported in the literature. The overall prognosis for patients with this malignancy is poor. In this report, two children with hepatic mesenchymoma underwent surgical excision followed by 2 years of adjuvant chemotherapy. Rotating cycles of cisplatinum, vincristine/doxorubicin, vincristine/cyclophosphamide, and vincristine/actinomycin D were given with minimal toxicity. Both patients are alive, with no evidence of disease at least 5 years from diagnosis. Previous reports of treatment are reviewed.

Adolescent↗
PubMed1988

Elective cholecystectomy in children with sickle hemoglobinopathies. Successful outcome using a preoperative transfusion regimen.

Twenty-seven children with major sickle hemoglobinopathies underwent elective cholecystectomy for cholelithiasis. All were managed with a preoperative transfusion regimen to achieve a hemoglobin concentration of 11-14 g/dl with greater than 65% hemoglobin A. Intraoperative cholangiography revealed common bile duct stones in five patients, although only one case was diagnosed by preoperative ultrasonographic examination. Twenty-four children underwent incidental appendectomy by total intussusception. There were no vaso-occlusive events nor any other perioperative morbidity or mortality. Four months after cholecystectomy, one boy had a small bowel obstruction requiring surgical re-exploration. No patients had transfusion-acquired infection, although one boy had erythrocyte allosensitization to Lewis A antigen. This preoperative transfusion regimen and careful perioperative management permits safe elective cholecystectomy in children with sickle cell disease.

Adolescent↗
PubMed1987

Efficacy of intravenous gamma globulin in autoimmune-mediated pediatric blood dyscrasias.

Sixteen pediatric patients diagnosed with a variety of autoimmune-mediated hematocytopenias were treated with one to 50 courses of intravenous gamma globulin (IVIG), pH 4.25, over the course of one to 30 months. Thirteen patients had immune thrombocytopenic purpura (ITP), two had autoimmune neutropenia, and one had autoimmune hemolytic anemia. In one patient, chronic ITP was associated with systemic lupus erythematosis, and in a second patient, acute ITP was the presenting manifestation of infection with human immunodeficiency virus. Initial therapy consisted of 400 mg/kg/dose daily for five days for the first seven patients treated, and 1,000 mg/kg/dose daily for two days for the remaining nine patients. In 15 of 16 patients, there was a response to IVIG therapy. In nine of 16 patients, maintenance IVIG therapy for two to more than 30 months was required. Minimal toxicity was experienced in four of 210 separate infusions. Data are presented to support the use of IVIG in the management of childhood autoimmune disorders.

Adolescent↗
PubMed1987

Treatment of platelet alloimmunization with intravenous immunoglobulin. Two case reports and review of the literature.

Two pediatric patients with severe aplastic anemia, elevated antiplatelet antibody levels, refractoriness to human lymphocyte antigen-matched platelet transfusions, and sustained bleeding problems were treated with intravenous immunoglobulin (IVIG), pH 4.25, for three to over nine months. Improved responses to platelet infusions and improved hemostasis were demonstrated in both patients. A review of the published literature analyzing the role of IVIG in the treatment of platelet alloimmunization is presented.

Anemia, Aplastic↗
PubMed1987

Effect of the beta 73 amino acid on the hydrophobicity, solubility, and the kinetics of polymerization of deoxyhemoglobin S.

The role of Asp-beta 73 on the surface hydrophobicity and solubility of hemoglobin was studied using Hb A, Hb S, Hb C Harlem (alpha 2 beta 2Val-6,Asn-73), and Hb Korle Bu (alpha 2 beta 2Asn-73). The surface hydrophobicity of the oxy form of these hemoglobins increased in the order of Hb A, Hb Korle Bu, Hb S, and Hb C Harlem, coinciding with the change in solubility. The same is not true for deoxyhemoglobins. The solubilities of deoxy-Hb S and deoxy-Hb C Harlem were much lower than that expected from their surface hydrophobicity. Although the hydrophobicity of deoxy-Hb C Harlem is greater than that of deoxy-Hb S, the solubility of deoxy-Hb S is only one-third that of deoxy-Hb C Harlem. This deviation must be caused by the substitution of Asn for Asp at the beta 73 position and its inhibitory effect on hydrogen bonding in Hb S polymers. The kinetics of the polymerization of 1:1 mixtures of the deoxy form of S-C Harlem, A-C Harlem, Korle Bu-S, and Korle Bu-C Harlem were studied in comparison with that of deoxy-Hb S and deoxy-Hb C Harlem alone. All of these binary mixtures polymerized with a distinct delay time prior to polymerization. Based on the results of kinetic studies, the probability factors for nucleation of S-C Harlem, A-S, A-C Harlem, S-Korle Bu, and Korle Bu-C Harlem hybrid hemoglobins were calculated as 0.65, 0.5, 0.5, 0.15, and 0.17, respectively, in comparison with that of Hb S (1.0). The probability factor for Hb C Harlem alone was 0.3. These data suggest that the Asp-beta 73 is directly involved in nucleation during Hb S polymerization and that the beta 73 is always trans to the active Val-beta 6 in the formation of nuclei.

Aspartic Acid↗
PubMed1987

Treatment of lymphohistiocytic erythrophagocytosis with VP-16 and aziridinylbenzoquinone.

Lymphohistiocytic erythrophagocytosis (LE) is a usually fatal disease characterized by fever, organomegaly, hyperlipidemia, central nervous system involvement, and cellular immunodeficiency. Treatment with corticosteroids, cytotoxic chemotherapy, and blood exchange is unsuccessful. We have treated two children with the epipodophyllotoxin VP-16 and with intrathecal chemotherapy. Each patient had an initial complete response, and one remains in remission 36 months after therapy began. Aziridinylbenzoquinone (AZQ) therapy induced a complete response in a patient who relapsed during VP-16 therapy. A combination of VP-16 and intrathecal chemotherapy appears to be the most effective therapy for LE, and further evaluation of the role of AZQ is indicated.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1987

Therapeutic considerations in childhood idiopathic thrombocytopenic purpura.

This monograph reviews the literature regarding therapy for childhood idiopathic thrombocytopenic purpura (ITP). The role and mechanism of action are discussed for corticosteroids, splenectomy, and intravenous gamma globulin. Alternative therapies with potent immunosuppressive agents are also mentioned. Guidelines for the management of children with ITP are presented.

Antineoplastic Agents↗
PubMed1987

Immunopathology of childhood idiopathic thrombocytopenic purpura.

Modern laboratory techniques have begun to elucidate the pathophysiology of chronic childhood ITP. Quantitative assays of PAIgG, complement, immune complexes, and platelet kinetic studies have all provided important information. Chronic ITP of childhood appears to be similar to adult ITP, with production of an antibody directed against platelets and megakaryocytes. Most of the antibody is produced in the spleen, but other parts of the RES can also produce antibody. Complement, immune complexes, and cell-mediated immunity may play a role in the pathogenesis. Sensitized platelets are cleared by the RES, particularly in the spleen. Platelet kinetic studies show that platelet turnover is usually rapid with compensatory increased thrombopoiesis, but there are some patients who have decreased thrombopoiesis. Acute ITP of childhood is a brief illness, characterized by abrupt onset of hemorrhagic symptoms and complete recovery. It often follows a viral illness, suggesting that immune complexes as well as antibodies are important in the pathogenesis. Both the spleen and liver may be important organs of immune clearance.

Acute Disease↗