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Biomedical subjects

C S August

Publications and source records attributed to C S August.

At least 19 recordsLinked to original sources
PubMed1995

Fractionated total-body irradiation preceding high-dose cytosine arabinoside as a preparative regimen for bone marrow transplantation in children with acute leukemia.

Twenty children with acute leukemia between 3 and 19 years of age underwent allogeneic bone marrow transplantation from HLA-matched sibling donors after conditioning with total-body irradiation (1,200 cGy in six fractions of 200 cGy twice daily for 3 days) and high dose cytosine arabinoside (3 g/m2 given every 12 hours for 12 doses). Three patients died with acute toxicity. Six patients developed grade II acute graft versus host disease. With a median follow-up of 68 months (range 26-96 months), thirteen children (65%) are alive and in remission with Karnofsky scores of 90-100%. A patient with AML in resistant relapse went into remission but relapsed and died 5 months post-transplantation. Three other patients relapsed, 8, 12, and 16 months post BMT. Our results suggest that this conditioning regimen is associated with high but manageable acute toxicity and may be highly effective in controlling leukemia resistant to conventional chemotherapy.

Adolescent↗
PubMed1995

Impact of donor and recipient characteristics on the development of acute and chronic graft-versus-host disease following pediatric bone marrow transplantation.

Current knowledge of risk factors for graft-versus-host disease (GVHD) in pediatric bone marrow transplantation is derived from studies focusing primarily on adults. We reviewed 100 pediatric HLA-matched allogeneic marrow transplants to identify donor and recipient factors (age, sex, age mismatch, sex mismatch) associated with increased incidence of acute or chronic GVHD. The incidence of acute (32%) and chronic (29%) GVHD were very low. In univariate analyses, recipient age (P = 0.003), donor age (P = 0.002), donor sex (P = 0.089) and age mismatch (P = 0.018) are related to acute GVHD. Prior acute GVHD (P = 0.0001), recipient age (P = 0.057), donor age (P = 0.016), donor sex (P = 0.01) and sex mismatch (P = 0.024) are associated with chronic GVHD. In multivariate analyses, older donor age (P = 0.003) and female donor sex (P = 0.046) independently predict acute GVHD. Only acute GVHD (P = 0.0001) was independently related to chronic GVHD. When acute GVHD was excluded, older donor age (P = 0.032) and sex mismatch (P = 0.005) predict chronic GVHD. Thus, the incidence of acute and chronic GVHD were very low in our pediatric population, especially in the youngest patients. Older donor age and female donor sex are associated with a higher incidence of acute GVHD. Prior acute GVHD, older donor age and sex mismatch are associated with a higher incidence of chronic GVHD.

Acute Disease↗
PubMed1992

Chronic graft-versus-host disease and pulmonary function.

Pulmonary complications are a major cause of morbidity and mortality in bone marrow transplant recipients. Earlier series, consisting mainly of adults, have shown evidence of obstructive changes of pulmonary functions in association with chronic graft-versus-host disease (CGVHD). We longitudinally evaluated spirometry in 46 patients who received bone marrow transplants as children or as young adults to determine whether they had similar abnormalities. Group mean FEV1/FVC, and percent predicted FVC, FEV1, and FEF25-75 values did not demonstrate obstructive changes in association with CGVHD in this patient population. Our findings suggest that younger patients with CGVHD, as a group, may fare better than older bone marrow transplant recipients with CGVHD. However, due to small sample sizes, it cannot be conclusively stated that the pulmonary function parameters analyzed do not differ in the two patient groups.

Adolescent↗
PubMed1992

Bone marrow transplantation for Niemann-Pick type IA disease.

Bone marrow transplantation has been undertaken with encouraging results as therapy for a wide variety of lysosomal storage diseases. We report a case of Niemann-Pick disease Type IA in which, despite the presence of only mild hypotonia with depressed reflexes, the clinical course of the disease appeared to be only slightly modified by this procedure, which was performed at the earliest practical opportunity. The patient was diagnosed early when asymptomatic, because of a family history of an affected sibling who died at 14 months. He received a bone marrow transplant from an HLA-identical, MLC non-reactive sibling donor, whose leukocyte sphingomyelinase activity was in the homozygote normal range. There was adequate engraftment as evidenced by persistently normal leukocyte sphingomyelinase activities, and there was no evidence of graft-versus-host disease. Visceral storage and neurological impairment were less rapidly progressive than in his untreated sibling but he eventually died at 30 months. Autopsy confirmed that this was essentially due to the effects of the underlying Niemann-Pick disease. We conclude that despite some success in other neurovisceral lysosomal storage disorders, bone marrow transplantation is not likely to be an adequate treatment for Niemann-Pick disease Type IA.

Bone Marrow Transplantation↗
PubMed1992

Sites of relapse in patients with neuroblastoma following bone marrow transplantation in relation to preparatory "debulking" treatments.

Forty-one patients with high-risk neuroblastoma were treated between September 1977 and December 1987 at the Children's Hospital of Philadelphia with supralethal chemotherapy and total-body irradiation rescued by bone marrow transplantation. Twenty-six patients were treated following relapse and 15 were newly diagnosed. At the time of evaluation, January 1991, 11 of 41 patients (26.8%) remained in complete remission. Actuarial survival rates of patients transplanted following relapse were 0.35 and 0.31 at 2 and 5 years, respectively, and actuarial disease-free survival rates were 0.38 at 12 months and 0.27 at 24 months. The 2- and 5-year actuarial survival values for the patients with newly diagnosed disease were 0.53 and 0.25, respectively, and the 12- and 24-month disease-free survival rates were 0.47 and 0.27, respectively. There was no significant difference in survival between these groups. Twenty-nine of the 41 patients reviewed were available for analysis of the effect of local treatment. Thirteen had a combination of surgery and radiation (RT), 2 had surgery alone, 9 had RT alone, and in 5 patients no local treatment was given. The local relapse rate was 17%; it was 15% following surgery plus RT and 22% following RT alone. The failure rate combining local and distant relapse is 62% for surgery plus RT and 44% for RT alone. Although a local relapse rate of 17% is imperfect, it is a relatively small contribution to the overall relapse of 62%.

Adrenal Gland Neoplasms↗
PubMed1992

Growth in children after bone marrow transplantation for advanced neuroblastoma compared with growth after transplantation for leukemia or aplastic anemia.

The linear growth of 26 children with progressive and advanced neuroblastoma treated with high-dose chemotherapy, total body irradiation, and bone marrow transplantation between 1978 and 1988 at the Children's Hospital of Philadelphia was compared with the growth of 33 children who had transplants for leukemia and of 12 who had transplants for aplastic anemia. The mean growth velocity, expressed as a standard deviation score, for the children who underwent bone marrow transplantation for neuroblastoma was -2.83. This was significantly (p less than 0.005) less than the standard deviation scores for children with transplants for acute lymphoblastic leukemia, acute nonlymphocytic leukemia, and aplastic anemia, which were -0.98, -0.07, and -1.05, respectively. A 6-year follow-up study of 32 long-term survivors of cancer revealed that the 11 patients with neuroblastoma continued to grow poorly, whereas a comparison group of 21 survivors of bone marrow transplantation for leukemia had essentially normal growth 2 years after the procedure. Major therapeutic differences between the two groups included the doses of local radiotherapy and the type and number of cytotoxic agents used. In comparison with the relatively mild growth-inhibiting effects of preparative regimens for leukemia and aplastic anemia, the very intensive preparative regimens used in patients with neuroblastoma have significant negative effects on growth.

Anemia, Aplastic↗
PubMed1992

Interstitial pneumonitis, pulmonary fibrosis, and chronic graft-versus-host disease.

Pneumonopathies in association with graft-versus-host disease (GVHD) are known, but the evolution of biopsy-proven interstitial pneumonitis (IP) to pulmonary fibrosis as a major pulmonary manifestation in an individual patient with chronic GVHD has not been previously reported. We present a patient with chronic GVHD who developed IP and then pulmonary fibrosis. We suggest that IP with evolution to pulmonary fibrosis was a major pulmonary manifestation of chronic GVHD in this patient.

Adolescent↗
PubMed1992

Exercise assessment of cardiac function in children and young adults before and after bone marrow transplantation.

Cardiac toxicity is a potential complication of bone marrow transplantation because recipients frequently receive cardiotoxic chemotherapy and/or irradiation before transplantation. Most studies indicate that transient cardiac toxicity occurs within weeks of transplantation, but few studies have evaluated either cardiac status before or late after transplantation. Cardiac performance was assessed via cycle ergometry in 20 children and young adults before transplantation and 31 other children and young adults after transplantation. Mean survival time in the group post-transplantation was 3.9 years with a range of 11 months to 12.1 years. Left ventricular size and shortening fraction at rest were assessed via echocardiography. Data were compared to those of 70 healthy subjects from our laboratory. Patients before and after transplantation had normal oxygen consumptions and cardiac indices at rest. During exercise, however, patients treated for cancer both before and after bone marrow transplantation had reduced exercise times, reduced maximal oxygen consumptions, and reduced ventilatory anaerobic thresholds. Cardiac reserve, as judged by the response of the cardiac output during exercise, was reduced severely. There were no significant differences between the groups tested before and after transplantation. Patients who had been treated for aplastic anemia, who had received less intensive therapy before transplantation, performed significantly better than did patients treated for cancer. Despite these findings, only four patients had abnormalities by echocardiography. In conclusion, before transplantation patients with oncologic diagnoses had serious limitations in exercise performance, most likely as a result of the effects of the cardiotoxic therapy given as part of their conventional cancer therapy. Long-term survivors of bone marrow transplantation also had similar abnormalities.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Hurler syndrome with special reference to histologic abnormalities of the growth plate.

Hurler syndrome is a mucopolysaccharide disorder resulting from an heritable deficiency in alpha-L-iduronidase, an enzyme required in the catabolism of heparan sulfate and dermatan sulfate glycosaminoglycan (GAGs). The resultant intracellular accumulation of GAG leads to disruption of the intracellular and extracellular environment and dysfunction of multiple organ systems. Among the most noted manifestations of this disease is disproportionate short trunk dwarfism, which develops during the first years of life. Histochemical and electron-microscopic observations on a 30-month-old child with Hurler syndrome showed marked irregularities in chondrocyte orientation within the growth plate, along with disruption of the normal columnar architecture. Vacuolization with enlargement of the cellular border was the characteristic ultrastructural finding. An heritable abnormality in the enzymatic degradation of structural glycosaminoglycans leads to profound disruption of the normal mechanisms of growth and development.

Cartilage↗
PubMed1990

Bone marrow transplantation for childhood Ki-1 lymphoma.

Two children with Ki-1 antigen-positive, non-Hodgkin's lymphoma received high-dose chemotherapy, fractionated total body irradiation (TBI), and allogeneic bone marrow transplantation. Both patients had relapsed multiple times on conventional chemotherapy and radiation therapy. Following transplantation, there was successful engraftment with disappearance of clinical signs and symptoms of their disease. As of June 1, 1989 they are in continuous unmaintained complete remission, 56 and 40 months, respectively, after bone marrow transplantation.

Adolescent↗
PubMed1990

Adenovirus-related hemophagocytic syndrome after bone marrow transplantation.

Four weeks following autologous bone marrow transplantation for Wilms' tumor, a patient developed fever, hepatomegaly, coagulation disorders and pancytopenia. Bone marrow studies showed progressively increased hemophagocytosis of normal hematopoietic progenitors by histiocytes resulting in aplasia. Adenovirus type 11 was consistently isolated from urine and stool cultures, and one of the marrow aspirates. At autopsy, adenovirus was isolated from the lungs, liver, heart, intestine and spleen. These findings are consistent with the previously described virus-associated hemophagocytic syndrome, which have not been associated with bone marrow transplantation. This case suggests that this diagnosis should be considered in any bone marrow transplant patient who has evidence of secondary graft failure.

Adenovirus Infections, Human↗
PubMed1988

Viral infections in pediatric bone marrow transplant patients.

The records of 96 pediatric patients with aplastic anemia or a malignancy who underwent bone marrow transplantation between 1979 and 1986 at The Children's Hospital of Philadelphia were reviewed for laboratory evidence of viral infections. The most common viral diseases identified were herpes simplex virus (HSV), cytomegalovirus and adenoviruses, which were found in 19 (20%), 17 (18) and 17 (18) patients, respectively. HSV was more common in patients with than without graft vs. host disease (GVHD) (9 of 30; 30% vs. 10 of 66; 15%), but the difference did not reach statistical significance. Late or prolonged isolation of HSV occurred in patients with chronic GVHD. Cytomegalovirus was significantly more common in patients with than without GVHD (10 of 30; 33% vs. 7 of 66; 11%). The presence of pretransplant antibody to cytomegalovirus or HSV was a good predictor of subsequent infection. Adenoviruses were isolated from all 3 patients with Burkitt's lymphoma. Adenovirus type 12, a serotype uncommon in man and known to be highly tumorigenic in young hamsters, was recovered from 4 patients. Adenoviruses were not notably more common in patients with GVHD (6 of 30; 20% vs. 11 of 66; 17%). Other viral infections demonstrated included 5 parainfluenza, 4 enteroviruses, 3 human immunodeficiency virus, 1 respiratory syncytial virus, 1 influenza B and 1 rhinovirus.

Adenovirus Infections, Human↗
PubMed1988

Massive intravascular hemolysis associated with intravenous immunoglobulin in bone marrow transplant recipients.

We report two group B Rho(D)-positive bone marrow transplant recipients who had sudden onset of massive intravascular hemolysis following the use of intravenous immunoglobulin (i.v. Ig). The first case developed chills and hypotension followed by hemoglobinuria with the first infusion of i.v. Ig. More severe symptoms occurred when infusion was reattempted. The second case developed hemoglobinuria without other symptomatology. In both patients, direct and indirect antiglobulin tests became positive following the use of i.v. Ig. Sera and eluates demonstrated IgG anti-B. Samples of i.v. Ig received by both patients contained both anti-B and anti-A and were incompatible with patients' red blood cells. These serologic findings indicate acute hemolysis secondary to the presence of IgG anti-B in i.v. Ig preparations.

ABO Blood-Group System↗