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Biomedical subjects

M E Nesbit

Publications and source records attributed to M E Nesbit.

At least 73 records · Page 4Linked to original sources

The accelerated phase of Chediak-Higashi syndrome. An expression of the virus-associated hemophagocytic syndrome?

The clinical and pathologic findings of four patients with Chediak-Higashi syndrome in the accelerated phase were studied in order to clarify the nature of this enigmatic process. Fever, lymphadenopathy, hepatosplenomegaly, and cytopenias were present in every patient. All cases demonstrated extensive parenchymal infiltrates in many organs composed of benign-appearing histiocytes manifesting hemophagocytosis accompanied by lymphocytes and plasma cells. Studies in one patient suggested a viral etiology with the findings of a low blood lymphocyte OKT4 to OKT8 ratio, acquired loss of lymphocyte response to mitogens, the presence of Epstein-Barr virus genome in the mononuclear cells of lymph node, blood, and bone marrow, and possible clinical responses to acyclovir. It is concluded that the accelerated phase of Chediak-Higashi syndrome may be the clinicopathologic expression of the virus-associated hemophagocytic syndrome.

Antibodies, Viral↗

Height of children successfully treated for acute lymphoblastic leukemia: a report from the Late Effects Study Committee of Childrens Cancer Study Group.

One hundred eighty-seven patients diagnosed with acute lymphoblastic leukemia (ALL) between 1972 and 1975 were evaluated for height 6.4-8.8 years (median 7.2 years) from the time of diagnosis of their disease. All patients had been treated on protocols CCG-101/143 conducted by the Childrens Cancer Study Group. After induction of remission, patients received either cranial radiation plus six doses of intrathecal methotrexate, craniospinal radiation, or craniospinal radiation plus abdominal radiation, followed by systemic maintenance chemotherapy for either 3 or 5 years. No significant difference was observed in the distribution of height percentiles at the time of diagnosis compared to expected population standards. After treatment an excess (P less than 0.001) was observed in the proportion of patients in the lower percentiles in conjunction with a decrease in the proportion of patients in the higher percentiles. After adjustment for height at diagnosis, the only factor found to have significant impact on attained height percentile was exposure to craniospinal plus abdominal radiation (P less than 0.001) where there was a fivefold excess in the proportion of patients below the fifth percentile for height. Craniospinal radiation only was not associated with a greater reduction in attained height percentile when compared to cranial radiation only.

Adolescent↗

Comparison of stage IV and IV-S neuroblastoma in the first year of life.

Clinical staging and factors related to survival were evaluated in 44 stage IV-S and 44 stage IV patients with neuroblastoma, ages 0 to 12 months, seen at Children's Cancer Study Group (CCSG) institutions from 1972 to 1979. In 73 patients with complete surgical staging, the life-table projected survival at 3 years was 91% for stage IV-S and 44% for stage IV. The only deaths in stage IV-S disease occurred in three patients less than 2 months old at diagnosis. In stage IV-S, 3 to 12 months old at diagnosis, the disease-free survival was 97%. Chemotherapy or radiation therapy did not appear to improve the survival rate in stage IV-S. These studies further document a significant clinical and biologic difference between patients with stage IV and stage IV-S neuroblastoma and suggest that they require different therapeutic management.

Actuarial Analysis↗

Role of the surgeon in the treatment of children's cancer.

The management of children's tumors has changed significantly in the past several years. New techniques and combined surgical, chemotherapeutic, and radiation approaches are responsible for improved survival in most instances. Cooperation of the surgeon with the specialists in separate disciplines is imperative to continued advancements in neoplastic disease of childhood.

Adolescent↗

Physician's role in home care for children with cancer.

We interviewed 29 physicians who had participated to varying degrees in a home care program for children dying of cancer. All physicians stated that they would use home care again and saw such care as an integral part of their practice. Most physicians felt that home care provides psychological advantages to the family; they viewed assessment of the family's needs by the home care nurses an important factor of home care treatment. Some physicians expressed concern of the nurse's assuming too much responsibility or failing to report significant changes observed in the patient, particularly observations of the side effects of medication. Divergent philosophies of physicians and nurses regarding side effects were seen by some physicians as a potential source of confusion and anxiety for families. The legal ramifications of home care were discussed by only a few physicians; the consensus of opinion among the physicians was that close contact among physician, family, and nurse would assure the absence of legal problems.

Attitude of Health Personnel↗

Correlation of chromosome abnormalities with patient characteristics, histologic subtype, and induction success in children with acute nonlymphocytic leukemia.

Cytogenetic analyses of bone marrow cells were performed in 195 children with acute nonlymphocytic leukemia (ANLL) at diagnosis, as part of Childrens Cancer Study Group Study No. 251. Ninety-six patients (49%) exhibited clonal abnormalities, including trisomy 8 in 18 patients, t(8;21) in 11, t(15;17) in seven, loss of a sex chromosome in seven, monosomy 7 in seven, and the Philadelphia chromosome in four. Clonal abnormalities were found significantly more often in younger patients. Furthermore, recurring cytogenetic abnormalities tended to correlate with specific ages. For example, t(8;21) was associated significantly with children over four years of age, while -7 associated with overall loss of genetic material from the long arm of chromosome 7 (7q) and 11q- were associated significantly with younger children. Recurring chromosome abnormalities also correlated with specific ANLL histologic subtypes, such as t(8;21) with acute myelogenous leukemia and t(15;17) with acute promyelocytic leukemia. Presence or absence of cytogenetic abnormalities was compared with the ability of patients to achieve remission. Individuals exhibiting clonal abnormalities in bone marrow cells had an equally likely chance of achieving remission (74%) as those individuals with normal karyotypes (75%). Nonrandom chromosome abnormalities associated with a high induction success rate included +8 with a 94% induction success rate (P = .13) and t(8;21) with a 91% success rate (P = .46). Patients exhibiting the -7 abnormality associated with overall loss of 7q had a significantly less successful induction outcome, with only 28% achieving remission (P = .02); three of seven patients with t(15;17) died during induction therapy.

Acute Disease↗

Intraocular retinoblastoma group V: an analysis of prognostic factors.

A retrospective analysis of the University of Minnesota (Minneapolis) experience with retinoblastoma is presented. Seventy-five patients were diagnosed with retinoblastoma between 1958 and 1983, of which 53 (71%) had at least one Reese-Ellsworth group V eye. Nineteen group V patients and one group II patient developed extraocular disease recurrence. The cumulative actuarial rate of recurrence at 12 years was 36% for patients with group V disease. The median time from diagnosis to recurrence for unilateral patients was seven months and for bilateral patients 28 months (P = .001). Patients developing extraocular disease had a 10-year actuarial survival rate postrecurrence of 34%. The four long-term survivors of extraocular recurrences had had isolated orbital or local soft tissue recurrences only. Features of group V patients associated with extraocular recurrences were identified by univariate life table analyses. Clinical poor-risk factors included the nongenetic form of the disease (P = .03) and male sex (P = .02). Pathologic poor risk factors included rubeosis (P = .01), undifferentiated histology (P = .03), large tumor size (P = .05), and intraocular extension to the anterior segment (P = .02), retinal pigment epithelium (P = .03), choroid (P less than .001), and optic nerve beyond the lamina cribrosa (P = .02). Treatment-associated poor-risk factors included an optic nerve length of less than 5 mm removed at enucleation (P = .003). Multivariate life table analyses demonstrated the following parameters to be independent poor-prognostic factors: optic nerve length of less than 5 mm removed at enucleation (P = .001), optic nerve involvement (P = .004), and large tumor size (P = .01). These results will help to identify patients with retinoblastoma who are at greatest risk for extraocular recurrence.

Actuarial Analysis↗

Ewing's sarcoma in bones of the hands and feet: a clinicopathologic study and review of the literature.

Review of current data from the Intergroup Ewing's Sarcoma Study (IESS) shows that Ewing's sarcoma (ES) is rare in bones of the hands and feet. Only 12 of 377 evaluable patients in the first two IESS studies had a primary tumor in these small, distal bones. The age distribution was typical for that seen in patients with ES at other sites. Males were affected twice as often as females, and tumors in the bones of the feet were much more common than those in the hands. All signs and symptoms were local in distribution. As in other sites, the dominant histologic pattern was categorized as diffuse. With the exception of those patients with lesions in the calcaneus, the prognosis for disease-free survival was excellent. A literature review of cases of ES reported in bones of the hands and feet showed generally comparable results.

Adolescent↗

Mediastinal mass in acute lymphoblastic leukemia.

Patients with acute lymphoblastic leukemia who have a mediastinal mass at the time of diagnosis are said to have a poor prognosis. However, many factors which may not be independent contribute to the success of treatment. We compared the disease characteristics and results of therapy in children having large mediastinal masses and lymphoblastic leukemia without mediastinal mass. Patients with a mediastinal mass had less evidence of marrow failure but were burdened with considerably more leukemic cells as measured by peripheral blood white count and extent of lymphadenopathy. Since the presence of mediastinal mass was strongly associated with these and other poor prognostic characteristics, we used multivariate techniques to determine which characteristics were independently associated with an increased risk for relapse. White count, extent of lymphadenopathy, age, and sex were significant predictors of early relapse, but when controlled for these variables the presence of a mass did not predict prognosis.

Adolescent↗

Serial thyroid function measurements in children with Hodgkin disease.

Thyroid function was measured serially in 28 children with Hodgkin disease diagnosed from 1971 to 1978. The patients' ages ranged from 4 to 16 years at diagnosis, and treatment consisted of chemotherapy only (four patients), radiation alone (15), or radiation plus chemotherapy (nine). None of the four children given chemotherapy only developed thyroid hypofunction, in contrast to 21 (88%) of the 24 children given high doses of radiation (P less than 0.001). Thyroid function in three patients with compensated hypothyroidism and in one child with primary hypothyroidism reverted to normal without thyroid replacement. One child given chemotherapy only and one child given radiation only became transiently hyperthyroid. These results indicate that patients given combined modality therapy for Hodgkin disease are at high risk for thyroid abnormalities. The results of long-term follow-up of thyroid function demonstrate, however, that all such thyroid abnormalities may not necessarily be permanent.

Adolescent↗

Down syndrome and acute leukemia in children: a 10-year retrospective survey from Childrens Cancer Study Group.

Review of 5406 children with acute lymphoblastic (ALL) or nonlymphoblastic leukemia (ANLL) registered with Childrens Cancer Study Group (CCSG) since 1972 identified 115 patients (2.1%) with Down syndrome. The proportion of patients with Down syndrome was the same for ALL (2.1%) and ANLL (2.1%). Patients with ALL with and without Down syndrome did not differ significantly with respect to age at diagnosis, sex, race, morphology (FAB classification), cell surface markers, initial white blood cell count, pretreatment hemoglobin value, hepatomegaly, lymphadenopathy, presence of mediastinal mass, CNS disease at diagnosis, or prognostic group as defined by age and initial white blood cell count. Patients with ALL-Down syndrome less frequently had splenomegaly, had lower pretreatment platelet counts, and more often had normal or elevated IgG or IgA levels. In addition, they had a significantly lower rate of remission (81% versus 94%), a higher mortality during induction therapy (14% versus 3%), and a poorer overall survival with 5-year life table rates of 50% versus 65% (P less than 0.001). If an initial remission was achieved, there were no significant differences with respect to remission duration, survival, or disease-free survival. Patients with ANLL-Down syndrome were younger at diagnosis than those without Down syndrome. There was no significant difference in the remission rates between these patients. Analysis of findings in patients with ANLL provided results similar to those obtained for patients with ALL with regard to clinical outcome after achievement of an initial remission.

Adolescent↗

Factors associated with IQ scores in long-term survivors of childhood acute lymphoblastic leukemia.

To identify factors which might be associated with intellectual function following treatment for childhood acute lymphoblastic leukemia, 50 long-term survivors were studied using the Wechsler Intelligence Scale for Children-Revised. All patients were diagnosed between 1972 and 1974 and were treated on a single clinical trial protocol with identical induction and maintenance chemotherapy plus central nervous system prophylaxis that included cranial radiation. The mean full scale IQ score for the group was 95 (SEM 2.0), with mean verbal IQ of 94.4 and mean performance IQ of 96.9. Factors which were found to be closely associated with a lower IQ score included female sex (in both verbal IQ and full-scale IQ), longer duration of chemotherapy (in performance IQ), and younger age at the time of radiation (in both verbal IQ and full-scale IQ). The age at the time of radiation was found to be significantly correlated with discrepancy between verbal and performance IQ, with younger age being associated with verbal IQ scores higher than performance IQ scores. When analyses were performed within specific subgroups of patients defined by sex and age at the time of radiation, dose of cranial radiation, concomitant intrathecal methotrexate therapy, and duration of therapy were all found to be correlated with a lower level of intellectual function. These preliminary findings provide direction for future studies to help identify high-risk patients.

Adolescent↗

Stature and Ewing's sarcoma in childhood.

The heights and weights of 291 children with Ewing's sarcoma were compared to population norms in order to explore the association of stature with this cancer. Overall, males were not significantly different from what was expected in either height or weight. When weights for heights of males under 151 cm--the upper limit of the norms--were reviewed, the distribution favored the larger categories. Further analysis of this group revealed that males under 151 cm were, on the average, 2 cm shorter and 0.2 kg lighter than peers. By contrast, females were smaller than expected. The greatest difference was in females under 151 cm; they were, on the average, 4.1 cm shorter and 2.4 kg lighter than their peers. In this series of similarly treated patients, stature at diagnosis did not appear to affect survival. Contrary to previous report, attained height and weight at diagnosis do not appear to be important risk factors for children with Ewing's sarcoma.

Adolescent↗