Biomedical subjects

E O Burgert

Publications and source records attributed to E O Burgert.

At least 19 recordsLinked to original sources

PubMed1993

Episodic central neurogenic hyperventilation in an awake child with systemic histiocytosis.

A 3 year old boy with systemic histiocytosis, diabetes insipidus and a lytic parietal bone lesion experienced episodes of central neurogenic hyperventilation 3 weeks after radiation to the head but was conscious and alert at presentation. At admission, the PaO2 was 133 mmHg, PaCO2 was 8 mmHg and pH 7.65. Magnetic resonance imaging revealed a pontomedullary lesion that resolved during the ensuring year. Central neurogenic hyperventilation has not been described previously as a complication of systemic histiocytosis.

Brain Diseases↗

PubMed1990

Ewing's sarcoma metastatic at diagnosis. Results and comparisons of two intergroup Ewing's sarcoma studies.

Two Pediatric Intergroup Ewing's Sarcoma studies of patients with metastatic disease (IESS-MD) have used multimodal therapy consisting of intensive combination chemotherapy and radiation therapy (XRT) to areas of gross disease detected at the time of diagnosis. In IESS-MD-I, conducted from 1975 to 1977, 53 eligible patients were entered and received the chemotherapeutic agents vincristine, Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH), cyclophosphamide, and dactinomycin with concomitant XRT (VACA + XRT). In IESS-MD-II, conducted from 1980 to 1983, 69 eligible patients were entered and received 5-fluorouracil (5FU) in addition to the chemotherapeutic agents of IESS-MD-I; initial intensive chemotherapy was given and XRT was delayed until week 10 (VACA + 5FU, delayed XRT). The best response rate (complete and partial remissions combined) was 73% in IESS-MD-I and 70% in IESS-MD-II, so there was no statistical evidence of a difference in response rates (P = 0.62). The length of best response also was similar between studies (P = 0.79), with approximately 30% of the patients on both studies remaining in remission at 3 years. The percentage of patients surviving 5 years or more was 30 on the first study and 28 on the second study (P = 0.49). The major sites of relapse after a response were lung and bone, each occurring with nearly equal frequency. The age of the patient was related to both best response rate and survival: patients 10 years of age or younger had substantially higher response and survival rates than patients 11 years of age or older. The favorable prognosis for younger patients might be explained by a more favorable distribution of primary sites at diagnosis; 39% of patients 10 years of age or younger had rib primary sites, compared with only 16% for patients older than 10 years of age (P = 0.05). The frequency of life-threatening toxicity was substantially higher in IESS-MD-I (30%) than in IESS-MD-II (9%), but the frequency of fatal toxicity was similar (6% to 7%). Fatal complications included Adriamycin-induced cardiomyopathy, Pneumocystis carinii pneumonia, unspecified pneumonitis, and sepsis. The most common toxicity and complications were leukopenia and infections.

Adolescent↗

PubMed1990

Hematologic disorders in 13 patients with acquired trisomy 21 and 13 individuals with Down syndrome.

To study the role of trisomy 21 in hematologic malignancies, we investigated the hematologic disorders of 13 patients with acquired trisomy 21 and 13 individuals with Down syndrome (DS). The most common hematologic malignancy among the patients with acquired trisomy 21 involved both granulocytic and monocytic lineages. By comparison, the hematologic disorders among the DS patients were predominantly acute lymphocytic leukemia and acute megakaryocytic leukemia. Although our sample was small, our results suggest that most patients with acquired trisomy 21 have different hematologic disorders than individuals with DS. Perhaps the role of trisomy 21 in the development of hematologic malignancy is different in constitutional trisomy 21 than it is in acquired trisomy 21.

Aged↗

PubMed1990

Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study.

A total of 342 previously untreated eligible children were entered into the first Intergroup Ewing's Sarcoma Study (IESS) between May 1973 and November 1978. In group I institutions, patients were randomized between treatment 1 (radiotherapy to primary lesion plus cyclophosphamide, vincristine, dactinomycin, and Adriamycin [doxorubicin; Adria Laboratories, Columbus, OH] [VAC plus ADR]) or treatment 2 (same as treatment 1 without ADR), and group II institutions randomized patients between treatment 2 or treatment 3 (same as treatment 2 plus bilateral pulmonary radiotherapy [VAC plus BPR]). The percentages of patients relapse-free and surviving (RFS) at 5 years for treatments 1, 2, and 3 were 60%, 24%, and 44%, respectively. There was strong statistical evidence of a significant advantage in RFS for treatment 1 (VAC plus ADR) versus 2 (VAC alone) (P less than .001) and 3 (P less than .05) and also of treatment 3 versus 2 (P less than .001). Similar significant results were observed with respect to overall survival. Patients with disease at pelvic sites have significantly poorer survival at 5 years than those with disease at nonpelvic sites (34% v 57%; P less than .001). Among pelvic cases, there was no evidence of differing survival by treatment (P = .81), but among nonpelvic cases, there was strong evidence of differing survival by treatment (P less than .001). The overall percentage of patients developing metastatic disease was 44%; the percentages by treatments 1, 2, and 3 were 30%, 72%, and 42%, respectively. The overall incidence of local recurrence was 15%, and there was no evidence that local recurrence rate differed by treatment. Patient characteristics related to prognosis, both with respect to RFS and overall survival experience, were primary site (nonpelvic patients were most favorable) and patient age (younger patients were more favorable).

Adolescent↗

PubMed1990

Multimodal therapy for the management of nonpelvic, localized Ewing's sarcoma of bone: intergroup study IESS-II.

Two hundred fourteen eligible patients with previously untreated, localized Ewing's sarcoma of bone were randomized on IESS-II to receive Adriamycin (ADR; doxorubicin; Adria Laboratories, Columbus, OH), cyclophosphamide, vincristine, and dactinomycin by either a high-dose intermittent method (treatment [trt] 1) or a moderate-dose continuous method (trt 2) similar to the four-drug arm of IESS-I. Patient characteristics (sex, primary site, type of surgery) were stratified at the time of registration; these and other patient characteristics (age, time from symptoms to diagnosis, race) were distributed similarly between treatments. Surgical resection was encouraged, but not mandatory. Local radiation therapy was the same as for IESS-I. The median follow-up time is 5.6 years. The overall outcome was significantly better on trt 1 than on trt 2. At 5 years, the estimated percentages of patients who were disease-free, relapse-free, and surviving were 68%, 73%, and 77% for trt 1 and 48%, 56%, and 63% for trt 2 (P = .02, .03, and .05, respectively). The major reason for treatment failure for both treatment groups was the development of metastatic disease. The lung was the most common site of metastases followed by bone sites. The combined incidence of severe or worse toxicity (67%) was comparable between the treatments; however, severe or worse cardiovascular toxicity was significantly greater on trt 1. Tne only treatment-associated deaths (N = 3) were on trt 1 and were cardiac-related.

Adolescent↗

PubMed1989

Acute megakaryocytic leukemia (M7) in children.

We analyzed the clinical and laboratory features of eight children (median age, 20 months; range, 13 months to 11 years) with acute megakaryocytic leukemia (M7) and compared the findings with those reported in the literature. The diagnosis was supported by ultrastructural examination for platelet peroxidase or immunophenotyping for glycoprotein IIb/IIIa or the von Willebrand factor protein. Two patients had Down's syndrome. Initial findings included anemia (in all patients), thrombocytopenia (in six), myelofibrosis (in three), lytic bone lesions (in two), and pronounced leukocytosis (in one). Stem cell culture studies of peripheral blood specimens revealed an aberrant phenotype of the megakaryocytes in one patient and reversal to a normal pattern after successful therapy. Remission was achieved in seven of the eight patients after aggressive chemotherapy, and four patients remained in remission 27 to 57 months after diagnosis. Three of these four patients underwent allogeneic bone marrow transplantation. M7 leukemia is not infrequent in children younger than 3 years of age, especially in those with Down's syndrome. The availability of monoclonal antibodies specific to restricted antigens of the megakaryocytic lineage has made the diagnosis of M7 leukemia both possible and practical.

Anemia↗

PubMed1988

Cardiopulmonary function in long-term survivors of childhood Hodgkin's lymphoma: a pilot study.

Twelve patients who had received mantle radiotherapy for Hodgkin's lymphoma during childhood underwent cardiopulmonary testing 7 years or more after the initial diagnosis and treatment. All but one patient had been asymptomatic. Results of echocardiography, pulmonary function tests, or exercise studies were abnormal in 9 of the 12 patients. Long-term follow-up of cardiopulmonary function will be important to determine the ultimate significance of these abnormalities. These potential complications must be considered in planning prospective therapeutic studies in children with Hodgkin's disease.

Adolescent↗

PubMed1988

Cyclophosphamide-induced hemorrhagic cystitis in Ewing's sarcoma.

Recent improvements in survival of patients with Ewing's sarcoma have been made since the addition of cyclophosphamide-based adjuvant chemotherapy to primary surgery and radiation. A potential limitation to cyclophosphamide use is its urotoxicity, primarily in the form of hemorrhagic cystitis. The incidence of this adverse effect in patients treated for Ewing's sarcoma has not been established. In a Mayo Clinic series of 116 patients with Ewing's sarcoma treated with cyclophosphamide, 17 (15%) developed hemorrhagic cystitis diagnosed on the basis of gross hematuria or cystoscopic findings (or both). Microscopic hematuria also occurred in 53% of patients (56 of 105 examined). The dose and duration of therapy appeared to be unrelated to the development of urotoxicity. Most patients recovered uneventfully with or without discontinuation of cyclophosphamide therapy, but a significant loss of blood occurred in three patients, and one patient required a cystectomy because of bladder fibrosis. Long-term follow-up is mandatory in these patients because of late recurrences of hemorrhagic cystitis or the possibility of bladder carcinoma. New therapies, directed at protecting the bladder from urotoxicity during cyclophosphamide treatment, are available.

Adolescent↗

PubMed1987

Abdominal Burkitt's lymphoma: role of early aggressive surgery.

Current surgical teaching advocates debulking of the abdominal Burkitt's lymphoma to decrease tumor burden and improve survival. The records of 16 children who had Burkitt's lymphoma were reviewed. Eleven of these children presented with abdominal tumors. Five of these 11 patients are long-term survivors and six have died. Three of the tumors were primarily resected; two were in patients who became long-term survivors. One resection was complicated by acute renal failure, leading to the early demise of the patient. Four tumors were debulked; only one of these was in a long-term survivor. Acute renal failure also complicated the hospital course in one of these children. Finally, four patients underwent initial incisional biopsies and plans were made for subsequent resection of any residual tumor 6 weeks later. Two of these children survived and two have died. The results of this pilot study do not confirm that there is a clear advantage to aggressive operative cytoreduction. Although this approach decreases the total tumor burden, it may also impose severe metabolic complications and postpone the administration of chemotherapy. These results do suggest the need for a multi-institutional review of the surgical management of abdominal Burkitt's lymphoma.

Abdominal Neoplasms↗

PubMed1986

Ewing's sarcoma of bone. Experience with 140 patients.

The records of 140 patients with histologically verified Ewing's sarcoma of bone treated between 1969 and 1982 were studied retrospectively. Various factors thought to be relevant to prognosis were analyzed. Three statistically significant factors were found: presence of metastatic disease, elevation of the sedimentation rate, and location of the tumor in the pelvis. In addition, patients who underwent complete surgical excision of the primary lesion had a better survival rate (74% at 5 years) than those who did not (34% at 5 years). It is concluded that patients with surgically accessible lesions should undergo treatment consisting of surgery, chemotherapy, and, in selected cases, radiation.

Actuarial Analysis↗

PubMed1986

Hereditary red cell disorders in Southeast Asian refugees and the effect on the prevalence of thalassemia disorders in the United States.

This investigation established the frequency and prevalence of hemoglobin E, alpha and beta thalassemia, and glucose-6-phosphate dehydrogenase deficiency in a group of Southeast Asian individuals and determined the impact upon the prevalence of thalassemia disorders in the United States. The resettlement program of Southeast Asian refugees has significantly increased the total number of serious thalassemic disorders in the United States. For several generations Southeast Asian children will be the principal thalassemic group.

Asia, Southeastern↗

PubMed1986

Wilms' tumor in a 'lump' kidney associated with sacral agenesis.

Many cases of Wilms' tumor developing in a horseshoe kidney have been previously reported. We present a case of Wilms' tumor developing in a "lump" kidney, associated with sacral agenesis. We discuss the implications for staging and make recommendations for follow-up in these cancer-prone children.

Bone and Bones↗

PubMed1985

Hematologic genetic disorders among Southeast Asian refugees.

Resettlement of Southeast Asian refugees has introduced into the Western Hemisphere many persons of all major ethnic groups from Indochina. They represent several distinctive cultural, genetic, and linguistic groups, and the prevalence of genetic traits among them varies accordingly. We studied 778 Southeast Asian persons resettled in the upper Midwest who belonged to 182 unrelated families from the five major Southeast Asian ethnic groups. High prevalences of hemoglobin E, alpha- and beta-thalassemia disorders, and glucose-6-phosphate dehydrogenase deficiency were found. The prevalences of these four conditions in the refugees are among the highest known in the world. For these groups, iron deficiency is an uncommon cause of microcytosis; instead, the most frequent causes are hemoglobin E and alpha-thalassemia-1. Very serious thalassemic disorders occur with unusually high frequency in the refugees, especially in the Tai-Dam.

Asia, Southeastern↗

PubMed1984

Sequential half-body irradiation (SHBI) and combination chemotherapy as salvage treatment for failed Ewing's sarcoma--a pilot study.

This study was undertaken to evaluate the toxicity of sequential half-body irradiation (SHBI) and combination chemotherapy (5-FU, VM-26 and BCNU) in patients who had failed primary aggressive therapy for their Ewing's sarcoma. A secondary goal was to evaluate the response of these previously treated patients to the combination of systemic radiation and multi-agent chemotherapy. The first patient in the study was treated with SHBI only and died 139 days following retreatment. Four subsequent patients successfully received the first cycle of combination chemotherapy. However, only one completed both upper and lower half-body irradiation while the remaining three patients, because of rapid progression of their disease, completed either the upper or the lower portion of their half-body irradiation (HBI). The time from retreatment to disease progression in these four patients ranged from 45 to 97 days (mean 79 days) and the time from retreatment to death ranged from 72 to 193 days (mean 126 days). The combination chemotherapy was tolerated well by all the patients, and the SHBI was accompanied by mild nausea and some vomiting within the first few hours following treatment. Failure to give the second half of the half-body irradiation and to complete further chemotherapy in three of the patients was a result of the progressive nature of the patients' disease and not to any limitations imposed by poor blood counts. Half-body irradiation provided good pain relief within 24 hours for all of the patients. Systemic radiation contributes to the palliative treatment of patients with failed Ewing's sarcoma, but appears to be relatively ineffective when the tumor burden is high.

Adolescent↗

PubMed1984

Intraoperative irradiation: a new technique in pediatric oncology.

Adverse effects on normal tissue are the principal limiting factor in the use of radiation therapy. Maturing tissues are most susceptible to these side effects, so high-dose applications can be a particularly difficult challenge in children. Intraoperative radiation therapy (IORT) enables the radiation oncologist to displace or shield dose-limiting organs or structures. These first two cases of children treated with IORT suggest that IORT, used alone or in conjunction with external beam irradiation, may be useful in the pediatric age group.

Child↗

PubMed1984

Randomized study of systemic chemotherapy following complete excision of nonosseous sarcomas.

Between June 1975 and April 1981, 61 of the 177 eligible patients whose nonosseous sarcomas of extremity or trunk origin had been completely excised primarily or after local recurrences agreed to participate in a randomized study of adjuvant chemotherapy. Dermatofibrosarcoma, lymphomas, myeloma, Kaposi's sarcoma, and embryonal rhabdomyosarcoma were excluded as were patients with significant second primary cancers and those who received either preoperative or postoperative radiation therapy. After stratification by anatomic status of disease, site of origin, and histologic grade, a random one half of the 61 participants began alternating courses of vincristine/cyclophosphamide/dactinomycin, and vincristine/doxorubicin/dacarbazine at six-week intervals for one year. The control group was evaluated at six-week intervals without adjuvant chemotherapy, but these patients were offered this chemotherapy later if they had progressive disease excised. Although 30% of the 61 patients experienced local recurrence of disease within the first five years after randomization, and only 54% were continuously disease free for five or more years, 82% were surviving at five years (Kaplan-Meier calculations) with a median follow-up of 64.3 months. Partial suppression of distant metastasis by adjuvant chemotherapy was apparent in the overall study, in the extremity tumor category, and in the subgroup of patients who had received limb-sparing surgery; however, no survival advantage for chemotherapy-treated patients was demonstrated. The 30 adjuvant chemotherapy-treated patients received a total of three thoracotomies as compared with 17 salvage thoracotomies for the 31 control patients; however, salvage surgery for local recurrences has been similar in the two groups. Recent improvement in the survival of patients with soft-tissue sarcomas is not necessarily a result of adjuvant chemotherapy or radiation therapy.

Adolescent↗

PubMed1983

Primary Ewing's sarcoma of the ribs. A report from the intergroup Ewing's sarcoma study.

Thirty-six patients with primary Ewing's sarcoma of the ribs have been reviewed. Of these, 21 had clinically localized disease at diagnosis and were entered on protocol IESS 7299, eight had regional and seven metastatic disease at diagnosis and were entered on protocol 7450. The 21 with localized disease were treated with surgical excision or biopsy, followed by local radiotherapy (in all but one patient) and randomization to one of three chemotherapy regimens. Eleven patients (52%) remain disease-free for periods ranging from 18 to 64 months, respectively. Seven of eight patients who underwent complete surgical excision of the primary lesion remain disease-free, compared with four of 12 (excluding one patient who died disease-free) who remain disease-free after partial excision or biopsy. However, analysis of size of tumor at diagnosis reveals that smaller primary tumors have a better prognosis irrespective of extent of surgery. Protocol IESS 7450, consisted of radiotherapy to all areas of known disease and four drug chemotherapy. Four (50%) with regional disease but none with metastatic disease have remained alive and continuously disease-free. It is concluded that an aggressive approach to Ewing's sarcoma of the ribs is justified by the results, as even regional disease may be curable. The apparent prognostic advantage for those patients undergoing surgical excision may be explained by patient selection.

Adolescent↗

PubMed1983

Comparison of intermediate-dose methotrexate with cranial irradiation for the post-induction treatment of acute lymphocytic leukemia in children.

We compared two regimens with respect to their ability to prolong disease-free survival in 506 children and adolescents with acute lymphocytic leukemia. All responders to induction therapy were randomized to treatment with 2400 rad of cranial irradiation plus intrathecal methotrexate or to treatment with intermediate-dose methotrexate plus intrathecal methotrexate, as prophylaxis for involvement of the central nervous system and other "sanctuary" areas. Patients were then treated with a standard maintenance regimen. Complete responders were stratified into either standard-risk or increased-risk groups on the basis of age and white-cell count at presentation. Among patients with standard risk, hematologic relapses occurred in 9 of 117 given methotrexate and 24 of 120 given irradiation (P less than 0.01). The rate of central-nervous-system relapse was higher in the methotrexate group (23 of 117) than in the irradiation group (8 of 120) (P = 0.01). Among patients with increased risk, radiation offered greater protection to the central nervous system than methotrexate (P = 0.03); there was no difference in the rate of hematologic relapse. In both risk strata the frequency of testicular relapse was significantly lower in the methotrexate group (1 patient) than the radiation group (10 patients) (P = 0.01). Methotrexate offered better protection against systemic relapse in standard-risk patients and better protection against testicular relapse overall, but it offered less protection against relapses in the central nervous system than cranial irradiation.

Adolescent↗