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Biomedical subjects

C S August

Publications and source records attributed to C S August.

At least 37 records · Page 2Linked to original sources
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↗
PubMed1986

Osteopetrosis: MR characteristics at 1.5 T.

Four patients with proved osteopetrosis (three with the infantile malignant form and one with the benign form) were examined with magnetic resonance imaging at 1.5 T. All patients were studied in the coronal and sagittal planes using both short and long repetition time/echo time sequences. The infantile malignant form was characterized by a complete lack of signal from the marrow alternating with a signal intensity equivalent to that of the intervertebral disks, resulting in a "stepladder" appearance. In the benign form or after successful marrow transplantation in the infantile malignant form, intermediate or high signal intensity in the vertebrae was noted, suggesting the presence of some marrow elements.

Child, Preschool↗
PubMed1986

Serial evaluation of lymphocyte function in bone marrow-grafted patients.

Deficiencies in both cellular and humoral immunity follow human bone marrow transplantation, predisposing recipients to life-threatening infections. Peripheral blood mononuclear cells (cells of donor marrow origin) from nine patients were collected serially at 3-month intervals during the first year post transplant and evaluated for proliferation and lymphokine (gamma interferon and interleukin-2) production in vitro. Cultures of patient cells or those of normal adult volunteers were stimulated by phytohemagglutinin (PHA) in vitro, and lymphocyte blastogenesis was assayed by tritiated thymidine uptake on day 2. PHA blastogenesis for peripheral blood mononuclear cell cultures from patients post bone marrow transplant achieved normal levels 3-6 months post transplant. Supernatants produced by cells from marrow recipients (less than 12 months post transplant) had lower-than-normal IFN-gamma activity and decreased IL-2 activity. Two patients with acute graft-vs-host disease (GVHD) had persistently depressed PHA blastogenesis, IFN-gamma production, and IL-2 production at 12 months post transplant.

Bone Marrow Transplantation↗
PubMed1984

Absorption of oral aminoglycosides following bone marrow transplantation.

Four patients with severe gastrointestinal reactions receiving oral "nonabsorbable" antibiotics for gut sterilization following bone marrow transplantation absorbed clinically significant amounts of aminoglycoside (gentamicin and/or tobramycin). Serum concentrations of 2.2, 2.6, 5.8, and 12.0 micrograms/ml were measured. Two of these patients had acute graft versus host reactions and two had severe mucositis following cytoreduction with intensive chemotherapy and irradiation. Nephrotoxicity occurred in the latter patients. One patient was studied in detail. Her hospital course and investigative results are presented. Four additional patients with mild gastrointestinal reactions following cytoreduction did not absorb gentamicin when their toxicity was maximal. Serum aminoglycoside determinations are necessary in patients receiving oral aminoglycosides for gut sterilization following bone marrow transplantation if moderate to severe gastrointestinal reactions occur.

Adolescent↗
PubMed1984

Neurological complications of bone marrow transplantation in childhood.

Bone marrow transplantation, used in the treatment of cancer, aplastic anemia, and metabolic diseases, involves the use of potentially neurotoxic agents to suppress immunity and eradicate malignancy. Fifty-seven patients with a median age of 11 years (age range, 6 months to 24 years) underwent bone marrow transplantation at the Children's Hospital of Philadelphia. Fifty-nine percent developed neurological abnormalities. Twenty-six patients (46%) had central nervous system (CNS) dysfunction, including infection (8), cerebrovascular accident (5), CNS leukemia (7), metabolic encephalopathy (5), and paraparesis with CNS toxoplasmosis (1). Neuropsychological dysfunction was present in 4 of 5 long-term survivors who were tested. Fourteen of 19 patients (74%) on whom postmortem examination was performed were found to have CNS abnormalities, including cerebral atrophy (10), focal cerebral injury (6), leukemia (5), and infection (3). Fourteen patients (24%) had peripheral nervous system dysfunction. CNS dysfunction was more common in patients with lymphoreticular malignancies. Cerebrovascular accidents (in patients with lymphoreticular malignancies) and infections (in our general population and in patients with lymphoreticular malignancies) occurred more often in our patients than in patients with similar illnesses who did not undergo bone marrow transplantation. The combination of prior treatment and preparative therapy for bone marrow transplantation predisposes patients to neurological and neuropsychological sequelae.

Adolescent↗
PubMed1984

Pulmonary function in patients undergoing bone marrow transplantation.

Pulmonary function was studied prospectively in 25 children with leukemia and aplastic anemia undergoing bone marrow transplantation (BMT). Whereas 11 patients have died, only one did so primarily due to interstitial pneumonia. Fourteen patients (56%) survived a median of at least 36 months. Seventeen patients received pulmonary function tests (PFTs). Four patients transplanted for leukemia in relapse following preparation with a very intensive regimen (cyclophosphamide, 200 mg/kg, total body irradiation, 1,000 rad, BCNU, cytosine arabinoside) developed restrictive lung changes. Patients undergoing BMT for aplastic anemia and leukemia in remission prepared with more commonly used and less intensive regimens maintained normal pulmonary function. As new regimens are devised, PFTs should be utilized to characterize the pulmonary toxicity of these regimens as well.

Adolescent↗
PubMed1984

Bone marrow transplantation in chronic granulomatous disease.

A 5-month-old Amish infant boy with chronic granulomatous disease underwent bone marrow transplantation from his 5-year-old, histocompatible brother after a preconditioning regimen of busulfan 2 mg/kg/day for 4 days, followed by cyclophosphamide 50 mg/kg/day for 4 days. At the time of bone marrow transplantation, he was free of infection, and remained so throughout the course of the transplant. He was engrafted promptly, with complete reversal of the neutrophil function defect and no sign of graft-versus-host disease. This was followed by loss of the erythroid graft and deterioration in neutrophil function over a period of 9 months. Sixteen months after transplantation, he is free of infection and growing normally, with essentially no evidence for neutrophil engraftment.

Bone Marrow Transplantation↗
PubMed1984

Bone marrow transplantation. Problems and prospects.

Over the last 15 years, human bone marrow transplantation has evolved from experimental therapy of last resort for patients with end-stage leukemia and marrow aplasia. The increasing success and use of marrow transplants has stemmed from important advances made in (1) the understanding and definition of the major histocompatibility complex in man, (2) the development of safe yet efficacious immunosuppressive and myeloablative preconditioning regimens, and (3) the development of techniques of intensive supportive care for these patients during their intra- and post-transplant course.

Adolescent↗
PubMed1984

Treatment of advanced neuroblastoma with supralethal chemotherapy, radiation, and allogeneic or autologous marrow reconstitution.

Ten children with recurrent metastatic (stage IV) neuroblastoma received local radiation therapy, supralethal chemotherapy, and total-body irradiation. Rescue with infusions of either allogeneic (four patients) or autologous (six patients) bone marrow followed. The drugs given to the first two patients were individualized combinations based on previous tumor responses. Both patients died with recurrent tumor three and nine months posttransplant. The eight remaining patients were treated more uniformly with local irradiation, VM-26, doxorubicin, melphalan (L-phenylalanine mustard), and 1,000-rad total-body irradiation in three fractions. Two of these patients had cardiac dysfunction and received no doxorubicin. Three children died in the immediate posttransplant period with disseminated fungal infections. A fourth relapsed and died nine months posttransplant. As of December 1, 1983, two children who received allogeneic marrow grafts have survived in complete remission for 54 and 36 months, and two children who received autologous marrow grafts have survived in complete remission for 35 and 22 months. These results suggest that relapsed metastatic neuroblastoma can be controlled by supralethal combinations of chemotherapy and irradiation coupled with bone-marrow rescue.

Adolescent↗