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Biomedical subjects

L N Sutton

Publications and source records attributed to L N Sutton.

At least 109 records · Page 6Linked to original sources
PubMed1989

Gadolinium-DTPA-enhanced magnetic resonance imaging in childhood brain tumors.

Gadolinium DTPA (Gd-DTPA) is a paramagnetic blood-brain barrier contrast agent for MRI that has been used primarily in adults. During May through October 1987, 17 children between the ages of 3 and 18 years with brain tumors underwent MRI examinations, before and after Gd-DTPA (11 gliomas, 4 medulloblastomas, 1 craniopharyngioma, and 1 child with neurofibromatosis and no pathologic diagnosis). We compared T1 and T2 Gd-DTPA-enhanced MRI with concurrent unenhanced MRI and enhanced CT, and then correlated this with the clinical and pathologic findings. Gd-DTPA enhanced tumors in all 7 patients with newly diagnosed tumors and enhanced tumors in 7 of 10 patients without clinical evidence of progressive disease at the time of the study. In the 7 new patients, Gd-DTPA defined tumor margins in all, and demonstrated internal tumor architecture (vessels, necrosis, and cysts) in 5. Areas believed to represent surgical scars showed varying degrees of enhancement. Leptomeningeal tumor spread, including spinal, not seen on pre-Gd-DTPA MRI or on contrast CT, was evident in 2 patients. Gd-DTPA enhancement obscured hemorrhage within the tumor (methemoglobin) in 2 patients. There were no significant side effects. These results suggest that Gd-DTPA-enhanced MRI (1) is safe in children, (2) demonstrates the extent and character of tumors better than unenhanced MRI and enhanced CT, and (3) may allow for noninvasive imaging of leptomeningeal disease, including the spine, not previously demonstrated by any other noninvasive neuroimaging technique.

Adolescent↗
PubMed1989

A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results.

As survival rates have risen for children with malignant primary brain tumors, so has the concern that many survivors have significant permanent cognitive deficits. Cranial irradiation (CRT) has been implicated as the major cause for cognitive dysfunction. To clarify the etiology, incidence, and severity of intellectual compromise in children with brain tumors after CRT, a prospective study was undertaken comparing the neuropsychological outcome in 18 consecutive children with malignant brain tumors treated with CRT to outcome in 14 children harboring brain tumors in similar sites in the nervous system who had not received CRT. Children with cortical or subcortical brain tumors were not eligible for study. Neuropsychological testing was performed after surgery prior to radiotherapy, after radiotherapy, and at 1- and 2-year intervals thereafter. Children who had received CRT had a mean full-scale intelligence quotient (FSIQ) of 105 at diagnosis which fell to 91 by Year 2. Similar declines were noted in their performance intelligence quotient (IQ) and verbal IQ. After CRT, patients demonstrated a statistically significant decline from baseline in FSIQ (p less than 0.02) and verbal IQ (p less than 0.04). Children who had not received CRT did not demonstrate a fall in any cognitive parameter over time. The decline between baseline testing and testing performed at Year 2 in patients who had CRT was inversely correlated with age (p less than 0.02), as younger children demonstrated the greatest loss of intelligence. Children less than 7 years of age at diagnosis had a mean decline in FSIQ of 25 points 2 years posttreatment. No other clinical parameter correlated with the overall IQ or decline in IQ. After CRT, children demonstrated a wide range of dysfunction including deficits in fine motor, visual-motor, and visual-spatial skills and memory difficulties. After CRT, children with brain tumors also demonstrated a fall in a wide range of achievement scores and an increased need, over time, for special help in school. The 2-year results of this study suggest that children with brain tumors treated with CRT are cognitively impaired and that these deficits worsen over time. The younger the child is at the time of treatment, the greater is the likelihood and severity of damage. These children, although not retarded, have a multitude of neurocognitive deficits which detrimentally affects school performance. New treatment strategies are needed for children with malignant brain tumors.

Adolescent↗
PubMed1989

Cerebral blood flow and glucose metabolism in experimental brain edema.

The relationship between cerebral blood flow (CBF) and cerebral metabolic rate of glucose (CMRgl) in the white matter was studied in a plasma infusion model of vasogenic edema in cats. Local CBF, as determined by iodoantipyrine testing, was found to be significantly decreased in edematous white matter (mean +/- standard error of the mean: 17.3 +/- 1.5 ml/100 gm/min) when compared with CBF in the contralateral control white matter (24.8 +/- 1.8 ml/100 gm/min). When the values for edematous brain were corrected for dilution, however, the local CBF averaged 25.3 +/- 1.7 ml/100 gm/min, which was similar to the control value. Local CMRgl was found to be significantly increased in plasma-infused white matter (16.3 +/- 2.2 mumol/100 gm/min) compared with that in control white matter (10.7 +/- 1.3 mumol/100 gm/min). This difference remained, despite correction for dilution and recalculation of local CMRgl values based on altered kinetic constants found in edematous brain. A similar increase in local CMRgl was noted with saline-infusion edema. It is concluded that the increased tissue water level does not alter CBF, but does induce an increase in anaerobic metabolism.

Animals↗
PubMed1989

Isolation and characterization of human fetal macrophages from placenta.

Human fetal macrophages expressing class II major histocompatibility complex (MHC) antigens have been isolated from the stroma of the chorionic plate of term placentas, using enzymatic digestion procedures, and enriched by Percoll density centrifugation. These cells are adherent, phagocytic and express Fc receptors for IgG. By rosetting with bovine erythrocytes coated with IgG, they can be enriched to 77-95% purity. Placental macrophages isolated in this way stimulate the proliferation of lymphocytes from unrelated donors in mixed-cell cultures, and act as accessory cells in oxidative mitogenesis. In a family study, placental macrophages stimulated proliferation of maternal and paternal lymphocytes but there was no evidence for either priming to, or suppression by, the fetal cells when the responses of lymphocytes from the mother and her HLA identical twin were compared. The possibility that these cells can protect the fetus from infection and/or stimulate the production of maternal anti-fetal HLA-antibodies is discussed.

Cell Adhesion↗
PubMed1988

Late recurrence of primitive neuroectodermal tumor/medulloblastoma.

The period of risk for recurrence of primitive neuroectodermal tumor/medulloblastoma (PNET/MB) is not clearly defined. With current treatment since more than 50% of children with PNET/MB can be expected to survive for at least 5 years after diagnosis, determining the evidence of "late" recurrence is of increasing concern. Collins has stated that patients with embryonal tumors who survive, disease free, for a period of time equal to the age at diagnosis plus 9 months can be declared cured. This, so-called Collins' law has been applied to patients with PNET/MB. To determine the incidence of "late" recurrence, factors which impact on recurrence and applicability of Collins' law, the authors studied all patients diagnosed with PNET/MB at the Children's Hospital of Philadelphia, Hospital of the University of Pennsylvania, Philadelphia, and Geisinger Medical Center, Danville, Pennsylvania, between 1970 and 1984. For the 44 patients in this study, the disease-free survival at 5, 10, and 12 years was 54%, 41% and 30%, respectively. For children surviving 5 years, the actuarial survival at 10 years was 75% and at 12 years, 51%. Age, sex, dose of radiotherapy, chemotherapy, or extent of surgery were not predictive of late relapse. Recurrence in three of seven patients (43%) occurred outside the "period of risk" as predicted by Collins. It appears that the "period of risk" for recurrent central nervous system tumors after PNET/MB is as yet undefined and probably indefinite.

Actuarial Analysis↗
PubMed1988

Results of the treatment of children with recurrent gliomas with lomustine and vincristine.

Gliomas comprise over 50% of all childhood brain tumors. Treatment of recurrent childhood gliomas has been disappointing and the effectiveness of therapy has been difficult to judge because of the variable natural history of the disease. Information gathered recently has suggested that treatment with [1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea)] (CCNU) and vincristine (VCR) after radiotherapy is effective in prolonging survival in children with newly diagnosed anaplastic gliomas. The authors have used these same drugs--CCNU (100 mg/m2) and VCR (1.5 mg/m2 up to a maximum dose of 2 mg)--in 6-week cycles for a maximum of eight cycles in children with recurrent gliomas. To date, 15 patients have been treated; five patients had malignant gliomas and ten low-grade gliomas. Three children showed improvement, five had stable disease, and seven had progressive disease. Of the five patients with malignant gliomas, four progressed within two cycles of treatment and one had stable disease for 7 months on treatment and then relapsed. Seven of ten children with low-grade gliomas benefitted from treatment and six remain in continuous remission a median of 16 months after initiation of therapy. Three of these children are off all therapy 21, 30, and 30 months after treatment, respectively. Therapy was well tolerated and toxicity consisted primarily of reversible bone marrow suppression. The authors conclude that CCNU and VCR chemotherapy is effective in children with recurrent low-grade gliomas and can result in relatively long-term disease stabilization. In limited experience of the authors, it is not of benefit in children with recurrent anaplastic lesions.

Adolescent↗
PubMed1988

Treatment of chiasmatic/hypothalamic gliomas of childhood with chemotherapy: an update.

Chiasmatic/hypothalamic gliomas (CHG) of childhood may cause progressive neurological and visual deterioration. Radiotherapy results in at least transient stabilization of tumor growth in most patients but may also have adverse long-term effects, especially in young children. Since 1977, children with progressive CHG under 5 years of age at diagnosis have been treated with combination chemotherapy (actinomycin D and vincristine) without radiotherapy. Twenty-four patients, a median of 1.6 years of age at diagnosis, have been treated and followed for a median of 4.3 years (range, 0.3-10 years). All patients are alive. Nine have developed radiographic or clinical progression, occurring a median of 3 years (range, 2-6.5 years) after initiation of treatment. Fifteen of 24 (62.5%) have remained free of progressive disease and have received no other therapy. Tumor shrinkage was documented in 9 of 24 patients but did not clearly relate to long-term outcome. Full-scale intelligence quotient (IQ) obtained a median of 3.5 years after diagnosis in patients who received only chemotherapy was a mean of 103 (range 84-133). We conclude that chemotherapy can significantly delay the need for radiotherapy in children with CHG and such a delay may be beneficial regarding long-term outcome.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1988

Efficacy of adjuvant chemotherapy for patients with poor-risk medulloblastoma: a preliminary report.

Recent studies have demonstrated that preoperative and postoperative factors can broadly stratify patients with medulloblastoma/primitive neuroectodermal tumors (MB/PNET) into risk groups. For children with factors that suggest poor outcome after treatment with surgery and radiotherapy, the addition of chemotherapy can improve survival. Since 1983, 26 children with poor-risk posterior fossa MB/PNET have been treated at our institution with craniospinal radiation therapy and adjuvant chemotherapy. Chemotherapy consisted of vincristine during radiotherapy and eight 6-week cycles of vincristine, cis-platinum, and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU). Twenty-five of 26 patients (96%) who have been entered on this protocol remain alive and free of disease at a median of 24 months from diagnosis (range 6 to 50 months). Twenty patients have completed all therapy and are at a median of 32 months from initial diagnosis with no evidence of disease. These patients were compared to a group of children with similar prognostic features treated at our institution between 1975 and 1983. Actuarial disease-free survival was statistically significantly better for protocol patients than for historical control subjects (p less than 0.002). This difference was most marked in patients who had received radiation therapy alone (p less than 0.0003). Actuarial 2-year disease-free survival was 96% for patients on protocol as compared to 59% for historical control patients who had been treated with radiotherapy alone. The chemotherapy given in this protocol was well tolerated. The results of this study, although preliminary, suggest that adjuvant chemotherapy is at least transiently effective in improving the rate of disease-free survival for children with poor-risk MB/PNET.

Adolescent↗
PubMed1988

Leptomeningeal dissemination of low-grade gliomas in childhood.

Although leptomeningeal spread (LMS) of primary CNS tumors in children has been well documented in the literature, it has rarely been reported in children with low-grade gliomas. Between 1975 and 1985, 6 of 162 children (3.7%) with low-grade gliomas treated at Children's Hospital of Philadelphia had LMS. LMS was present at diagnosis of the original tumor in one patient, was the first sign of relapse in one patient, occurred simultaneously with local relapse in two patients, and after local relapse in two patients. Pathology of the original tumor was low-grade astrocytoma in five and low-grade oligodendroglioma in one. Primary tumor site was cervical cord in three, chiasm in one, frontal lobe in one, and cerebellum in one. All of the children with LMS had undergone surgical treatment at the time of diagnosis of the primary tumor; four had total resections at some point in their course. Three of the six patients died; three are still alive after treatment with radiation therapy and/or chemotherapy. The longest survival to date has been 3 1/2 years after diagnosis of LMS. We compared clinical characteristics of these six patients with 131 children with low-grade tumors without dissemination treated at our institution during the same time period. LMS, although relatively infrequent, does occur in children with low-grade gliomas, especially spinal cord tumors. LMS may occur at any time during illness and diagnosis may be difficult unless LMS is suspected. Treatment, at times, results in clinical improvement and considerable disease control.

Adolescent↗
PubMed1988

Leptomeningeal spread of intramedullary spinal cord tumors. Report of three cases.

Three patients with intramedullary spinal cord tumors and secondary leptomeningeal spread of their tumors are presented. Two patients had astrocytomas and one had a ganglioglioma. Two tumors were located in the cervical spinal cord and one within the thoracic spinal cord. Review of the past and recent literature shows leptomeningeal dissemination of spinal cord tumors to be relatively rare, but it should be suspected and investigated in any patient whose condition deteriorates following removal of a spinal cord neoplasm.

Adolescent↗
PubMed1988

Intracranial visual pathway gliomas in children with neurofibromatosis.

The association between neurofibromatosis and visual pathway gliomas is well documented. The introduction of computed tomography and magnetic resonance imaging has heralded a new era in the understanding of visual pathway gliomas. Both of these noninvasive neuroinvestigative techniques have demonstrated extensive abnormalities throughout the visual pathway in children with visual pathway gliomas, especially in those with neurofibromatosis. The clinical significance of these abnormal areas of brain, especially in asymptomatic patients, is unknown. In an attempt to clarify the incidence, natural history, and clinical course of patients with neurofibromatosis and visual pathway lesions, we reviewed our experience with 24 patients managed consecutively at Children's Hospital of Philadelphia over the past 12 years. The patients in this series were compared to 29 children with visual pathway gliomas without neurofibromatosis who were evaluated at our institution over the same period of time. Visual pathway gliomas in children with neurofibromatosis differ from those in children without neurofibromatosis. In general, lesions tended to be more extensive in patients with neurofibromatosis and the clinical course of these patients is more variable. Twelve of the 24 patients with neurofibromatosis in our series had symptoms of progressive disease at the time of diagnosis and underwent treatment with variable results. Twelve children with neurofibromatosis and visual pathway lesions had static lesions at the time of diagnosis and, to date, 3 have developed progressive disease. From our review we can make some recommendations concerning the management of children with neurofibromatosis and visual pathway gliomas, but many questions remain unanswered. Sequential follow-up of a large cohort of both asymptomatic and symptomatic children with neurofibromatosis and visual pathway lesions is needed to more definitively outline the best management approach for these patients.

Adolescent↗
PubMed1987

Pineocytomas of childhood. A reappraisal of natural history and response to therapy.

Pineocytomas are pineal parenchymal tumors composed of differentiated cells histologically similar to those of the mature pineal gland. Little is known about the incidence, pattern of growth, or response to treatment of pineocytomas. Between 1975 and 1985, six children with pineocytomas were treated at our institution, and pineocytomas constituted 11% of all newly diagnosed pineal region tumors. The clinical, radiographic, and pathologic features of these six patients with pineocytomas are presented. Initial treatment for these children included craniospinal plus supplemental local radiotherapy (three), local radiotherapy alone (one), or radiation therapy plus adjuvant chemotherapy (two). Four of six patients had tumor recurrence a median of two years after diagnosis. Three patients had leptomeningeal dissemination, one at the time of diagnosis and two following therapy. Our findings suggest that: biopsy is necessary to distinguish pineocytomas from other pineal region tumors; radiation therapy alone is inadequate; and these tumors are aggressive in the pediatric population, with a high propensity for leptomeningeal dissemination.

Adolescent↗
PubMed1987

Outcome of children with primary intramedullary spinal cord tumors.

The influence of clinical and treatment factors on the outcome of children with primary intramedullary spinal cord tumors (PST) was evaluated by reviewing the records of 26 children diagnosed during the 15-year period 1970-1984. Five-year survival was 39%, but 5-year event-free survival (EFS) was only 14%. Eighteen-month EFS was 53% (9/17) among children with low-grade astrocytoma. 100% (2/2) with ependymoma, and 0 of 7 with anaplastic astrocytoma or ganglioglioma. There was no significant difference in the 18-month EFS by location of tumor, duration of symptoms, or extent of surgical removal. Five of 9 children with locally recurrent PST had a second operation, and 4 were alive a median of 56 months later. PST disseminated to the leptomeninges or the III ventricle in 5 children: 2 at diagnosis, 2 as the first sign of disease relapse, and 1 after local recurrence. Given the poor outcome of our children, different methods of treatment for children with tumors in this location should be evaluated.

Adolescent↗
PubMed1987

Management of Chiari II complications in infants with myelomeningocele.

Vocal cord paralysis with inspiratory stridor during infancy is a well-recognized complication of the Arnold-Chiari malformation associated with myelomeningocele. Management of these symptoms, however, remains controversial. The outcome in 19 infants with symptoms managed by us during 1978-1984 was therefore reviewed. Ten infants had stridor alone at presentation (clinical grade 1), four infants also had apnea (grade 2), and five infants also had associated cyanotic spells and dysphagia (grade 3). Of 14 ventricular shunt revisions performed, seven were associated with resolution: in five of eight infants with grade 1, two of four with grade 2, and none of two with grade 3 symptoms. Of the 10 posterior fossa decompressions performed, two were associated with clinical resolution: in one of four infants with grade 1, one of two with grade 2, and none of four with grade 3 symptoms. Mortality over 6 months following onset of symptoms was absent among infants with grade 1, one with grade 2, and three with grade 3 symptoms. We propose that differences in outcome among the three groups of infants reflect differences in pathologic processes within the brain stem. We speculate that infants with grade 2 or 3 symptoms have more extensive brain stem damage, such as hemorrhage, infarction, and necrosis. Use of our classification system may be helpful in further studies on intervention modalities in these infants.

Arnold-Chiari Malformation↗
PubMed1987

Current management of low-grade astrocytomas of childhood.

Astrocytomas are the most frequently encountered brain neoplasms in the pediatric age group. Current management strategies consist of surgery, radiation therapy, chemotherapy and immunotherapy, although the optimal treatment is yet to be defined. It is hoped that new treatment modalities, and reassessment of current regimens will improve outcome, better in terms of survival and quality of life.

Astrocytoma↗
PubMed1987

Quality of life in children with primitive neuroectodermal tumors (medulloblastoma) of the posterior fossa.

We reviewed our experience in 43 consecutive patients with primitive neuroectodermal tumors (medulloblastoma), PNET (MB), treated between 1975 and 1984, to characterize their quality of life and identify factors which impacted on long-term function. Twenty-four of forty-three (56%) of children are alive and free of disease, a median of 4.5 years after diagnosis. The quality of life was analyzed for the 24 long-term survivors. 79% (19 of 24) were functioning well in everyday activities. The median full-scale intelligence quotient (FSIQ), obtained a median of 3.5 years after diagnosis for those tested (n = 17) was 97, with all but 3 (12%) of the patients functioning in the normal range. Specific learning, memory and fine-motor disabilities were found in over one half of patients. Factors associated with poorer performance and lower FSIQ included preoperative obtundation, the need for a permanent shunt, younger age at diagnosis, and a complicated postoperative course. It is concluded that (1) the majority of long-term survivors have 'normal' intellectual function, but may have specific intellectual and academic disabilities, and (2) preoperative and postoperative factors strongly impact on the quality of life of survivors.

Adolescent↗