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Biomedical subjects

T R Kinney

Publications and source records attributed to T R Kinney.

At least 37 records · Page 2Linked to original sources

Emergency department visits by children with sickle hemoglobinopathies: factors associated with hospital admission.

Children with sickle cell disease frequently present to the emergency department (ED) for evaluation of fever or management of pain. We retrospectively analyzed all ED visits by children with sickle hemoglobinopathies during 1990, excluding those for trauma. Of 146 visits by 56 children, 73 (50%) were classified as "Painful Events," 43 (29%) as "Febrile Events," 20 (14%) as "Painful and Febrile Events," and 10 (7%) as "Other." Hospital admission occurred for 42% of Painful Events versus 70% of Febrile Events (P = 0.008) and 85% of Painful and Febrile Events (P = 0.002). For Painful Events, location of pain was not associated with hospitalization, but pain less than 24 hours in duration before the ED visit (P = 0.002) and administration of intravenous fluids and analgesia (P = 0.001) were associated with admission. For children evaluated for Febrile Events, age less than six years (P = 0.016) and maximum temperature greater than 39 degrees C (P = 0.011) were associated with subsequent hospitalization, but total white blood cell count and absolute neutrophil count were not. For Painful and Febrile Events, pain less than 24 hours (P = 0.029) was associated with hospital admission, but age, maximum temperature, white blood cell count, and absolute neutrophil count were not. Although prospective studies are needed to validate these data, the identification of factors predictive of hospital admission should expedite ED care to sickle cell patients.

Adolescent↗

Hemoglobin C disease in infancy and childhood.

We reviewed the clinical course, physical findings, and hematologic values in 16 pediatric patients with hemoglobin C disease, all but one identified by a newborn hemoglobinopathy screening program. The patients had a few symptoms or physical findings. Height and weight percentiles were normal. Patients typically had a mild hemolytic anemia characterized by microcytosis and target cells.

Adolescent↗

Gallbladder sludge in children with sickle cell disease.

Ultrasonographic records of 75 children with sickle cell disease and hepatobiliary symptoms were reviewed. Seventeen had gallbladder sludge, nine with concurrent stones and eight with sludge alone. Because all the children eventually had gallstones, we recommend that elective cholecystectomy be performed on patients with gallbladder sludge.

Anemia, Sickle Cell↗

Psychological adjustment of children with sickle cell disease: stability and change over a 10-month period.

Rates of poor psychological adjustment of children with sickle cell disease remained relatively constant over initial and follow-up assessment points. However, there was relatively little stability in the classification of the adjustment of individuals, low congruence in specific behavior problem patterns and diagnoses in accordance with the Diagnostic and Statistical Manual of Mental Disorders (3rd ed.; American Psychiatric Association, 1980), and less stability in child adjustment by child report than by mother report. With initial levels of adjustment controlled, children's strategies for coping with pain accounted for a significant increment in child-reported symptoms (19%) and mother-reported internalizing behavior problems (8%) at follow-up beyond the contribution of illness and demographic parameters and follow-up interval. The findings suggest that children's coping strategies are a salient intervention target for enhancing adjustment.

Adaptation, Psychological↗

Stability and change in the psychological adjustment of mothers of children and adolescents with cystic fibrosis and sickle cell disease.

Found moderate stability in the classification of maternal adjustment in two longitudinal studies of mothers of children and adolescents with cystic fibrosis and sickle cell disease. In terms of the transactional stress and coping model, stable poor maternal adjustment was associated with higher levels of appraisal of daily stress and palliative coping and low levels of family supportiveness. With initial levels of maternal adjustment, demographic parameters, and follow-up interval controlled, concurrent levels of daily stress accounted for significant portions of variance in maternal adjustment at follow-up for both illness groups. In addition, illness severity, child psychological adjustment, and family conflict added significant increments to maternal adjustment at follow-up in the cystic fibrosis group. Findings are discussed in terms of a basis for subsequent intervention studies to enhance the adjustment of mothers of children with chronic illness.

Adaptation, Psychological↗

Acute splenic sequestration in the absence of palpable splenomegaly.

PURPOSE: Acute splenic sequestration in children with sickle hemoglobinopathies is a medical emergency characterized by splenomegaly, anemia, and thrombocytopenia. PATIENTS AND METHODS: We describe a young girl with hemoglobin SC disease who experienced an acute splenic sequestration without palpable splenomegaly. CONCLUSIONS: We emphasize the need for a high index of suspicion for splenic sequestration and the utility of ultrasonography in documenting splenomegaly.

Acute Disease↗

Hemoglobin sickle-Lepore: report of two siblings and review of the literature.

Hemoglobin Lepore is composed of two normal alpha-globin chains and two delta-beta fusion globin chains that result from nonhomologous crossing over of genetic material during meiosis. The doubly heterozygous condition of sickle hemoglobin with Lepore hemoglobin (Hb S-Lepore) is rare, having been described previously in only nine patients. We report two siblings with Hb S-Lepore who have similar hematologic characteristics but a marked difference in clinical severity.

Child↗

Role of child and maternal processes in the psychological adjustment of children with sickle cell disease.

In this study, 64% of children aged 7-12 years with sickle cell disease were found to have a parent-reported behavior problem, and 50% met the criteria for a Diagnostic and Statistical Manual of Mental Disorders (3rd ed.) diagnosis based on a structural clinical interview of the child. Internalizing types of behavior problems and diagnoses were the most frequent. Support was provided for a transactional stress and coping model in delineating the processes associated with child adjustment. In particular, maternal anxiety accounted for 16%-33% of the variance in mother-reported internalizing and externalizing behavior problems, respectively, and child pain-coping strategies accounted for 21% of the variance in child-reported adjustment problems.

Adaptation, Psychological↗

Psychological adjustment of mothers of children and adolescents with sickle cell disease: the role of stress, coping methods, and family functioning.

Assessed the psychological adjustment of 78 mothers of children and adolescents (7-17 years of age) with sickle cell disease. Support was provided for a transactional stress and coping model in delineating the processes associated with maternal adjustment. In particular, poor maternal adjustment was associated with use of palliative coping methods and high levels of stress related to daily hassles. Variables of the model accounted for 55% of the variance in maternal psychological distress.

Adaptation, Psychological↗

Sickle cell disease pain in children and adolescents: change in pain frequency and coping strategies over time.

Examined 9-month follow-up data obtained from children and adolescents with sickle cell disease (SCD) and their parents participating in a longitudinal study of pain coping strategies. Of 87 subjects completing the baseline assessment of pain coping strategies, 70 (80%) of their parents completed a structured pain interview assessing their child's health care use and activity reduction during painful episodes over the follow-up period. Regression analyses controlling for age and pain frequency revealed that baseline Coping Attempts were associated with higher levels of school, household, and social activity during painful episodes. Baseline Passive Adherence was associated with more frequent health care contacts during the subsequent 9 months. Increases in Negative Thinking over time were associated with further increases in health care contacts during the follow-up period. Comparing pain coping strategies assessed at baseline to pain coping strategies measured at follow-up revealed that pain coping strategies were relatively stable over time for younger children but changed more for adolescents.

Adaptation, Psychological↗

Diagnosis and management of common bile duct stones in patients with sickle hemoglobinopathies.

Nine of 35 patients with sickle hemoglobinopathies and cholelithiasis were found to have concomitant common bile duct (CBD) stones. We describe the diagnosis and management of these patients with CBD stones. Historical information, physical examination, and routine laboratory tests were unable to identify accurately the patients with CBD stones. Similarly, ultrasonography of the hepatobiliary system was an insensitive method for detecting CBD stones, as only 3 of 8 patients were correctly identified. In contrast, both an intraoperative cholangiogram and endoscopic retrograde cholangiopancreatography were sensitive procedures for detection of CBD stones. We conclude that CBD stones are relatively common in patients with sickle cell disease and cholelithiasis, and clinicians should have a high index of suspicion for their presence.

Adolescent↗

Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies.

Nine young patients with sickle hemoglobinopathies successfully underwent laparoscopic cholecystectomy; no complications resulted from the procedure. The mean postoperative hospital stay was 1.6 days. This technique appears to be a safe and efficacious procedure in children with sickle hemoglobinopathies who require cholecystectomy for cholelithiasis.

Adolescent↗

The technique of laparoscopic cholecystectomy in children.

Twelve children underwent elective laparoscopic cholecystectomy for symptomatic cholelithiasis during a 10-month period in one institution. The operative technique that has been described for adults was modified because of the smaller dimensions of pediatric patients. These modifications are discussed in this report, as are new alternatives for evaluating the common duct. No operative complications or conversions to open cholecystectomy occurred, and no complications after surgery were seen during an average follow-up period of 4.5 months. The benefits of laparoscopic cholecystectomy include decreased pain and ileus after surgery, shortened hospitalization, and improved cosmesis. Laparoscopic cholecystectomy is safe and efficacious in children, and it compares favorably with traditional cholecystectomy in the pediatric age group.

Adolescent↗

Relationship of clinical severity to packed cell rheology in sickle cell anemia.

There is wide variation in the clinical manifestations of sickle cell disease (SCD) from one affected individual to another. Many investigators have sought to discern parameters that would explain this variability. In the present studies we have attempted to correlate the frequency of painful events and the extent of end organ failure in SCD with rheologic properties of packed suspensions of sickle cells, using a magneto-acoustic ball microrheometer developed in our laboratory. Using this device we have measured the steady-state viscosity, and the viscous and elastic moduli of cell suspensions in 16 individuals with hemoglobin SS disease who were untransfused and in their steady state. The rheologic parameters were then correlated with clinical parameters. The clinical parameters measured were emergency department visits, hospitalizations, hemoglobin, reticulocyte count, age, and end organ failure (nephropathy, avascular necrosis of bone, stroke, retinopathy, resting hypoxemia after acute chest syndrome(s), leg ulcer, and priapism with impotence). The P value for the correlation between the steady state viscosity and end organ failure was .001 with a correlation coefficient (R value) of .73. The P value for the correlation between the viscous modulus of viscosity and end organ failure was .00006 with an R value of .83. The P value for the correlation between the elastic modulus of viscosity and end organ failure was .0006 with an R value of .76. However, there was no significant correlation between any component of packed cell rheology and emergency department visits or hospitalizations for pain.

Adult↗

Pain in sickle cell disease. Rates and risk factors.

BACKGROUND AND METHODS: Acute episodes of pain are the principal symptom of sickle cell disease, but little is known about the epidemiologic features of these episodes or risk factors for them, nor is it known whether patients with high rates of such episodes die prematurely. We prospectively studied the natural history of sickle cell disease in 3578 patients ranging from newborns to persons up to 66 years old who were followed at clinical centers across the United States. RESULTS: There were 12,290 episodes of pain in 18,356 patient-years. The average rate was 0.8 episode per patient-year in sickle cell anemia, 1.0 episode per patient-year in sickle beta 0-thalassemia, and 0.4 episode per patient-year in hemoglobin SC disease and sickle beta(+)-thalassemia. The rate varied widely within each of these four groups--e.g., 39 percent of patients with sickle cell anemia had no episodes of pain, and 1 percent had more than six episodes per year. The 5.2 percent of patients with 3 to 10 episodes per year had 32.9 percent of all episodes. Among patients with sickle cell anemia who were more than 20 years old, those with high rates of pain episodes tended to die earlier than those with low rates. High rates were associated with a high hematocrit and low fetal hemoglobin levels. alpha-Thalassemia had no effect on pain apart from its association with an increased hematocrit. CONCLUSIONS: The "pain rate" (episodes per year) is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20. Even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival. This outcome is particularly encouraging to investigators studying hydroxyurea and other treatments designed to increase the fetal hemoglobin level.

Adolescent↗

Transient erythroblastopenia in the first year of life.

The distinction between transient erythroblastopenia of childhood (TEC) and congenital red cell aplasia (Blackfan-Diamond anemia) is usually possible at presentation using clinical and laboratory data. When hypoplastic anemia occurs in the first year of life, however, the distinction is more difficult. We describe 7 infants with hypoplastic anemia who illustrate this diagnostic dilemma.

Anemia, Aplastic↗

Sickle cell disease in children and adolescents: the relation of child and parent pain coping strategies to adjustment.

Examined pain coping strategies in 72 children and adolescents with sickle cell disease and their parents using the Coping Strategies Questionnaire, and found that pain coping strategies could be reliably assessed. Children high on Negative Thinking and Passive Adherence were less active, required more health care services, and were more psychologically distressed during painful episodes. Children high on Coping Attempts were more active and required less frequent health care services. Parents high on Coping Attempts and low on Passive Adherence had children who were more active.

Adaptation, Psychological↗