PubMed Health⌕ Search

Biomedical subjects

S Weitzman

Publications and source records attributed to S Weitzman.

At least 73 records · Page 4Linked to original sources

Acute tumour lysis syndrome with no evidence of tumour load.

Acute tumour lysis syndrome (ATLS) is a well-recognised complication of the treatment of a variety of malignant disorders in which a large mass of disease is obvious. ATLS may, however, occur even in the absence of bulk disease. We present two cases which, together with a review of previously reported cases, suggest that a cause of this rare phenomenon is primary renal lymphoma which subsequently develops into the leukaemic phase. This is supported by the observation that some bone marrow aspirates which are normal at the time of ATLS have shortly afterwards demonstrated lymphoblasts. Renal biopsy may not exclude primary lymphoma of the kidney. In excluding lymphoproliferative disease in the differential diagnosis of acute hyperuricaemia, the importance not only of bone marrow examination but of exhaustive investigation of the kidneys is stressed.

Acute Disease↗

Pediatric malignant pheochromocytoma.

Pediatric malignant pheochromocytomas are very rare tumors, and no institution has more than one or two of these problem cases. The authors report on two children with such tumors, over a 9-year period, from two hospitals. In 1984, D.B. (14 years of age) presented with symptoms and signs of extradural metastasis from a right adrenal primary; he also had lung and bone metastases. After spinal decompression almost 4 years. He has remained well and is in remission 6 years later. In 1987, G.R. (13 years of age) presented with a larger right adrenal malignant pheochromocytoma invading surrounding structures; he also had liver metastasis. Preoperative chemotherapy did not shrink the tumor much; it was grossly resected, and there were many postoperative problems. In 1990, bone metastases developed, for which radiotherapy and chemotherapy were used. Three years later, the metastases have not disappeared; he remains on chemotherapy, and his liver function is borderline. From our small experience as well as a literature review, it appears that surgical excision remains the treatment of choice for the pediatric malignant phoechromocytoma. Unresectable tumors may be rendered resectable by intensive chemotherapy (similar to that used for neuroblastoma); adjuvant chemotherapy should be used for residual disease after surgery and for metastatic disease.

Adolescent↗

Relation of carotid artery wall thickness to diabetes mellitus, fasting glucose and insulin, body size, and physical activity. Atherosclerosis Risk in Communities (ARIC) Study Investigators.

BACKGROUND AND PURPOSE: We tested the hypothesis that body mass, waist-to-hip circumference ratio, physical inactivity, diabetes, hyperglycemia, and fasting insulin are each positively associated with asymptomatic carotid artery wall thickness. METHODS: Average intimal-medial carotid wall thickness (an indicator of atherosclerosis) was measured noninvasively by B-mode ultrasonography in cross-sectional samples of 45- to 64-year-old adults, both blacks and whites, free of symptomatic cardiovascular disease, in four US communities. RESULTS: Sample mean carotid wall thickness was approximately 0.7 mm in women (n = 7956) and 0.8 mm in men (n = 6474). Body mass, waist-to-hip ratio, work physical activity, diabetes, and fasting insulin were associated (P < .05) with carotid wall thickness in the hypothesized direction. Adjusted for age, race, smoking, body mass index, artery depth, and Atherosclerosis Risk in Communities field center, mean wall thickness was greater by 0.02 mm in women and 0.03 mm in men for a 0.07-unit (one SD) larger waist-to-hip ratio. Adjusted mean wall thickness was about 0.07 mm thicker in participants with diabetes mellitus and 0.02 mm thicker in participants with hyperglycemia (fasting glucose 6.4 to 7.7 mmol/L) than in subjects with fasting glucose < 6.4 mmol/L. Adjusted mean wall thickness increased by about 0.02 mm with an increase of 100 mmol/L in fasting serum insulin. CONCLUSIONS: Abdominal adiposity, physical inactivity, and abnormal glucose metabolism are associated positively with carotid intimal-medial wall thickness, suggesting these factors contribute to atherogenesis.

Aging↗

Variant translocations of chromosome 22 in Ewing's sarcoma.

Relatively few variant translocations have been reported in primary Ewing's sarcomas (ES). We report two new variant translocations, both of which involve chromosomal rearrangements of 22q12. Cytogenetic studies of tumor cells from a 12-year-old girl revealed a variant translocation, t(7;22)(p22;q12), the second example reported of a simple variant of the 22q12 reciprocal translocation in this type of sarcoma. The identity of this rearrangement was confirmed by in situ hybridization. In addition, a complex translocation was identified in a dysmorphic 15-year-old girl, t(4;11;22)(q21;q24;q12). No previous cases of variant translocations in ES have involved band 7p22 or 4q21, and there are no previous reports of an association between congenital abnormalities and unusual karyotype abnormalities in ES. Both variant translocations conserve the junction on the der (22), providing additional cytogenetic evidence that the sequences on chromosome 22 are critical.

Child↗

Comparison of carboplatin pharmacokinetics between an anephric child and two children with normal renal function.

Carboplatin, a promising second generation platinum compound, is an effective antitumor drug and appears to be less nephrotoxic than cisplatin. We report comparative pharmacokinetics of carboplatin in an anephric child and two children with normal renal function. All three children were infused with carboplatin over 4 hours, the anephric child receiving 100 mg/m2 (25% of the dose received by the other two children). This dose was well tolerated and 18 days later the anephric patient received a second course of carboplatin at 50% of the regular dose received by the control patients. Following this dose the girl experienced severe pancytopenia and recovered with the use of GM-CSF, blood, and platelet transfusion. Blood samples were obtained at timed intervals for 24 hours in the control subjects and for 40 hours in the anephric patient. Plasma and plasma ultrafiltrates were analyzed for total and free platinum. The results show that elimination half-life of total platinum was 13 hours in the children with normal renal function and 42 hours in the anephric child following the 100 mg/m2 dose. The elimination T1/2 of free platinum was 8 hours in a child with normal GFR and 32 hours in the anephric child. In the anephric child the clearance of ultrafiltrable platinum was 10% of the normal total body clearance. We suggest that, in children with renal insufficiency, the dose of platinum derivatives should be carefully chosen, proportionally to the degree of renal impairment.

Blood Proteins↗

Malignant benign neonatal sacrococcygeal teratoma.

Thirty-six patients with benign neonatal sacrococcygeal teratoma (SCT) were treated in our medical center from 1972 to 1990. Mean gestational age was 38.6 +/- 3.3 weeks with a mean birth weight of 3,484.0 +/- 938.5 g. Twenty-nine patients (89%) were females. The majority of the tumors (75%) contained cystic components and 96% were Altman classification I and II. The initial surgical removal of the SCT (including the coccyx) was carried out during the first 7 days of life. Six patients (22%) developed recurrence of the tumor. Three were benign and reappeared locally after 12 +/- 3 months and were reexcised. The mean serum alpha-fetoprotein level in this group was 13 +/- 1 g/L. The malignant recurrence (all originally reported as being mature benign SCT) appeared at 20.3 +/- 1.5 months and had markedly elevated serum alpha-fetoprotein levels (7,320 +/- 4,630 micrograms/L). All the patients in this group had multimodal therapy including complete excision of the recurrent tumor. We conclude that SCT, although histologically benign, has an alarming potential to recur either as a benign or malignant tumor during the first 2 years of life. Close follow-up for at least 3 years (frequent examination, serum alpha-fetoprotein, and diagnostic imaging) is recommended for all patients who had undergone excision of SCT in the newborn period.

Combined Modality Therapy↗

The comprehensive assessment of health status in survivors of childhood cancer: application to high-risk acute lymphoblastic leukaemia.

The health status of 69 survivors of high-risk acute lymphoblastic leukaemia (ALL) is assessed using a multi-attribute classification system. Seven attributes are included: sensation, mobility, emotion, cognition, self-care, pain and fertility. Three to five levels of functioning are defined for each attribute. Comprehensive health states are described as a specific combination of seven attribute levels. The system captures combinations of sequelae. The system provides a compact but comprehensive tool for long term follow up of survivors of childhood cancer. The results underscore the cognitive and emotional burdens of morbidity affecting survivors of high-risk ALL.

Cognition Disorders↗

Primary leptomeningeal melanoma: an unusually aggressive tumor in childhood.

Primary malignant melanoma of the leptomeninges of the central nervous system is a rare and aggressive tumor in children. We report our experience from 1964 to 1990 with this tumor in eight children. The mean age at diagnosis was 4.9 years (range, 1.3 to 13 yr). Five children presented with signs and symptoms of raised intracranial pressure from hydrocephalus secondary to tumoral obliteration of the basal cisterns, but the time from the initial symptomatology to diagnosis was frequently delayed. Three patients in this series had hairy nevi in association with their leptomeningeal melanoma. Cerebrospinal fluid (CSF) analysis typically showed raised opening pressures, decreased glucose, and increased protein concentrations. Malignant melanoma cells were found in the CSF in three patients. Confirmatory radiographic examinations included air encephalography, myelography, and computed tomographic and magnetic resonance scanning. Four patients were treated with lumboperitoneal shunts, and one patient was treated with a ventriculoperitoneal shunt for hydrocephalus. Two patients underwent craniotomies and subtotal excisions of their tumors. In seven patients, a definitive diagnosis of leptomeningeal melanoma was made by pathological examination of tissues sent at surgery or at post mortem. In one case, the diagnosis was established by a detailed cytological analysis of the CSF. Four children died of fulminant disease and tumor spread before treatment could be instituted. The four children who received treatment had a combination of radiation therapy and chemotherapy. One child received intrathecal methotrexate. The two children with the longest survivals (2 and 3 yr, respectively) received cisplatinum and dimethyltriazenoimidazole carboxamide in addition to craniospinal irradiation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Birthweight curves in southern Israel populations.

With the aim of establishing a standard range of birthweights at different gestational ages for Jewish and Bedouin populations, we analyzed 5.5 years of computerized delivery files. Strictly confirmed gestational age was required in this study. This is necessary for proper diagnosis of growth abnormalities, such as growth retardation and macrosomia, for research purposes and for assessment of perinatal morbidity and mortality at a given gestational age. Percentile distributions of birthweights of 46,529 infants are presented. The mean birthweight of male infants was 3,238 g (SD 529 g) and of female infants was 3,117 g (SD 504 g). From week 36 to week 41 of gestation, infants of Jewish mothers were significantly heavier than infants born to Bedouin women, particularly in primiparous women. In both Jewish and Bedouin births, male infants were significantly heavier than female infants after the 36th week of gestation, by 130 g in the former and 127 g in the latter. Although the set of birthweight percentile curves of Jews and Bedouins was similar to the Colorado birthweight curve, some differences were found. At 41 and 42 weeks of pregnancy our infants were heavier and exceeded the standard, whereas at weeks 35 to 38 the standard exceeded the median weight of the Bedouin infants. The differences between our population curves and the commonly used Colorado standard curve emphasize the need for locally based and periodically updated infant birthweight data.

Birth Weight↗

Preoperative care of infants with nephroblastoma. The International Society of Pediatric Oncology 6 experience.

The International Society of Pediatric Oncology (SIOP) recommends preoperative treatment in the management of eligible patients with Wilms' tumor. Until 1980, children younger than 12 months of age (infants) at diagnosis had been excluded from the SIOP trials. SIOP 6, conducted from 1980 to 1987, was the first SIOP study to include infants older than 6 months of age. This retrospective analysis of 145 infants registered to SIOP 6 demonstrates that in infants older than 6 months and having favorable histology (FH), a two-drug preoperative chemotherapy (CT) regimen of 4 weeks significantly ameliorated stage distribution as determined at delayed surgery but did not affect a good outcome. However, the CT dose utilized in SIOP 6 resulted in an unacceptable toxicity in this age group, and SIOP 9, the new SIOP study of Wilms' tumor, recommends a reduced dose of CT in infants. Preoperative CT is not recommended in infants younger than 6 months of age. Specifically, the high incidence (29%) of mesoblastic nephroma in this age group does not justify such an approach. Histopathologic diagnosis should be obtained in these patients before any treatment.

Age Factors↗

Acquired von Willebrand disease in Wilms' tumor patients.

PURPOSE: A prospective study was performed to determine the incidence of acquired von Willebrand disease (vWD) in children with newly diagnosed Wilms' tumor. PATIENTS AND METHODS: Fifty consecutive children with newly diagnosed Wilms' tumor were evaluated. Detailed family and bleeding histories were obtained in all cases. Laboratory evaluation included measurement of the circulating platelet count, bleeding time (BT), factor VIII (FVIII) and von Willebrand factor (vWF) levels, and ristocetin cofactor (RCoF) activity. A vWF multimer analysis was obtained in all cases in which vWD was suspected. RESULTS: Four of 50 (8%) consecutive children with a diagnosis of Wilms' tumor were found to have acquired vWD. Laboratory findings indicated type III vWD in two patients and type I vWD in the other two. CONCLUSIONS: The incidence of acquired vWD in association with Wilms' tumor merits further study through a large prospective trial. Such a trial should include careful family and clinical bleeding histories plus measurement of a platelet count, BT, coagulant FVIII and vWF levels, RCoF activity, and vWF multimer analysis. The response to 1-desamino-8-D-arginine vasopressin (DDAVP) should be tested in all patients with Wilms' tumor and acquired vWD, including patients with a type III profile, before an invasive procedure is performed. Successful use of DDAVP may avoid exposure of affected patients to blood products.

Blood Coagulation↗

A comprehensive multiattribute system for classifying the health status of survivors of childhood cancer.

PURPOSE: A multiattribute health status classification system was devised to describe comprehensively the health status of survivors of childhood cancer. METHODS: The system consists of seven attributes: sensation, mobility, emotion, cognition, self-care, pain, and fertility. Three to five levels of functioning are defined for each attribute. Any specific combination of seven attribute levels constitutes a health state. In the first survey, the system was used to classify the health status of 20 children currently undergoing therapy for high-risk acute lymphoblastic leukemia (ALL), Wilms' tumor, or neuroblastoma, and eight who had completed treatment. A second survey consisted of 13 children with brain tumors on active treatment. RESULTS: In general, independent ratings by clinicians were in agreement, and consensus was readily achieved in 1 to 2 minutes per patient. Children on therapy experienced a higher burden of morbidity than those off treatment. Brain tumor patients experienced more morbidity than patients in the first survey. CONCLUSION: The multiattribute system provides a compact but comprehensive tool for long-term follow-up of survivors of childhood cancer. It captures both multiple sequelae and varying levels of severity. By using a mathematical utility function, a single summary score of health-related quality of life may be assigned to each health state. Additional studies to establish reproducibility, validity, responsiveness, and generalizability are indicated.

Activities of Daily Living↗

Diabetic ketoacidosis. A rare complication of gestational diabetes.

OBJECTIVE: To describe a case of severe DKA in an otherwise healthy pregnant woman. RESEARCH DESIGN AND METHODS: We describe 2.5 yr of close follow-up of a Bedouin woman who was hospitalized for DKA while pregnant with her 11th child. Plasma glucose returned to normal levels immediately after delivery of a dead conceptus. Four months later, while normoglycemic, the patient became pregnant again. During the subsequent pregnancy, GDM was diagnosed at week 20 of gestation. Tight plasma glucose control was achieved with an insulin regimen, and the patient delivered a healthy girl at term. Plasma glucose again returned to normal and remained so to date, 18 mo postpartum. An OGTT and a euglycemic hyperinsulinemic clamp were performed between pregnancies; another OGTT was performed at week 14 of the last pregnancy. Plasma glucose, insulin, and C-peptide were measured in blood samples during these procedures. RESULTS: We established beyond doubt that the patient developed GDM and returned to essentially normal glucose tolerance after her last (12th) delivery. During the 11th pregnancy, gestational diabetes was complicated by severe DKA. CONCLUSIONS: GDM is a common abnormality of glucose metabolism during pregnancy, which affects fetal development and leads to peripartum complications. Our report stresses that under certain circumstances, gestational diabetes can be complicated by DKA and become life-threatening to the mother and fetus.

Adult↗

Ewing's sarcoma: primary involvement of the zygoma undergoing resection and immediate reconstruction.

Because the occurrence of primary Ewing's sarcoma in the facial bones is unusual, it may pose diagnostic and therapeutic problems. Lack of clinical suspicion along with atypical radiographic features may lead to a delayed diagnosis. Furthermore, because of the limited number of cases, precise treatment guidelines are lacking. In our patient, whom we believe to be the first reported with primary Ewing's sarcoma originating in the zygoma, the tumor was successfully managed surgically. A combination of craniofacial and microsurgical principles made surgical resection and immediate reconstruction possible in an area not generally thought to be amenable to surgery; moreover, we thus avoided the potential deleterious effects of radiation in the facial region in a growing child.

Child↗

Neuroblastoma stage IV-S: a heterogeneous disease.

Eighteen patients were diagnosed and treated for Stage IV-S neuroblastoma at The Hospital for Sick Children, Toronto between January 1971 and December 1988. All patients were 6 months of age or younger at diagnosis. Nine patients (50%) have remained disease free with a mean follow-up of 9.3 years. Of the seven patients under 6 weeks of age at presentation, four presented in the early neonatal period and died, three due to mechanical complications related to progressive disease, and one due to late recurrence. The remaining three patients under 6 weeks of age, two of whom had skin involvement at diagnosis, are alive and disease free. Six of the 11 patients over 6 weeks of age at presentation survived, combined modality therapy (CMT) being more effective than single modality treatment. N-myc was studied from tumor tissue at diagnosis in four patients and was amplified in three (25x, 25x, 100x), all of whom had late disease progression and died. The patient with a single gene copy has no evidence of disease 24 months following diagnosis. Our study confirms the heterogeneity described in this clinically defined group of patients. Because of it, management of Stage IV-S neuroblastoma cannot be uniform and until further development of a subclassification, or a reclassification based on molecular biologic markers is developed, pediatric oncologists will regularly be confronted with a decision whether or not to treat a newly presenting patient that fits into the clinical classification IV-S.

Adrenal Gland Neoplasms↗

Preoperative chemotherapy for children with Wilms' tumor.

Five years ago, the use of preoperative chemotherapy for Wilms' tumor was adopted at this institution. Thirty children ranging in age from 5 months to 9 years had histologically confirmed Wilms' tumor (needle biopsy, n = 26; open biopsy, n = 4). Stage was determined by chest and abdominal computed tomography (CT) scan. Bilateral tumors were present in 6 children. All children received actinomycin D and vincristine from 3 weeks to 6 months before surgery. Seven children with bilateral tumors or stage IV disease also received adriamycin. CT-measured tumor masses shrunk in most cases. Subsequently, nephrectomy was performed in 23 patients and partial nephrectomy in 4, 2 of whom had bilateral disease. In 2 patients, residual bilateral well-differentiated epithelial tumor was not surgically resected. One child died before surgery. Reevaluation at delayed total or partial nephrectomy resulted in a downstaging of disease in 12 (41%). Further chemotherapy and radiation was based on the surgical stage. Postoperative chemotherapy (4 months to 2 years) was administered to all patients. The chest and/or abdomen were radiated in 12. Four patients (13%) died, 1 of radiation pneumonitis and 3 of the disease progression (2 with unfavorable histology, 1 of whom had bilateral disease). Two of 4 with unfavorable histology and 4 of 6 with stage IV disease have survived. It is concluded that this preoperative chemotherapy protocol is as effective as the National Wilms' Tumor Study (NWTS) protocol. The treated tumor is smaller, less friable, and easier to remove. Furthermore, because of downstaging, less radiation is necessary for cure.

Biopsy, Needle↗

Reduction in tumor burden allowing partial nephrectomy following preoperative chemotherapy in biopsy proved Wilms tumor.

During the last 6 years a treatment protocol of radiographic staging along with percutaneous biopsy to establish a histological diagnosis has been used in 37 patients with Wilms tumor. Combination chemotherapy was given for 4 to 6 weeks before definitive surgical resection. In 9 patients tumor shrinkage was sufficient to permit preservation of a portion of the affected kidney(s). In stage V disease partial nephrectomy was accomplished in 5 patients. In 4 additional patients with unilateral disease downstaging also allowed partial nephrectomy. The radiological and histological changes that allowed this limited surgery are analyzed and compared.

Antineoplastic Combined Chemotherapy Protocols↗