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Biomedical subjects

B G Leventhal

Publications and source records attributed to B G Leventhal.

At least 37 records · Page 2Linked to original sources
PubMed1990

Nuclear morphometry as a predictor of response to therapy in Wilms tumor: a preliminary report.

Present therapy in Wilms tumor is based upon prognosis. Current trends suggest less intensive chemotherapy and/or radiation therapy for children with favorable histological disease who are considered at low risk for recurrence and increasing the amount of therapy for those with unfavorable histological tumors who are at high risk for recurrence. Currently, pathological stage and histological appearance are used to predict prognosis. We tested a new approach. Histological sections of surgical specimens were obtained from 9 children treated for unilateral Wilms tumor. All patients had favorable histological disease, and received postoperative chemotherapy and/or radiotherapy. These specimens were evaluated by nuclear morphometry with the Hopkins Morphometry System to determine if nuclear shape descriptors could distinguish children who responded to therapy from those whose tumor recurred. Nuclear roundness, ellipticity, convexity and bending energy accurately separated the groups. Nuclear morphometry may provide useful information that will aid in predicting which children with Wilms tumor will respond to therapy and warrants further investigations.

Cell Nucleus↗
PubMed1990

Neurofibromatosis 1: recognition and management of associated neuroblastoma.

Neurofibroma and neuroblastoma both arise from the neural crest, and there has long been speculation regarding a pathogenetic relationship between them. Clinical characteristics do not necessarily distinguish these tumors, therefore the diagnosis of neuroblastoma should be considered in all children with neurofibromatosis 1 (NF-1) who have a rapidly growing or inaccessible mass. A careful physical examination, imaging studies, and urinary catecholamine measurement are indicated. In a child with NF-1 and malignancy, direct tissue examination may be necessary to differentiate malignant from nonmalignant tumor and guide therapy. Furthermore, with the significantly increased risk of certain types of childhood cancer in these patients, we recommend evaluation for this common heritable condition in all patients with malignancy.

Catecholamines↗
PubMed1990

Invasive fungal disease in pediatric acute leukemia patients with fever and neutropenia during induction chemotherapy: a multivariate analysis of risk factors.

We evaluated the courses of 115 consecutive cases of pediatric acute leukemia treated with induction chemotherapy. Seventy-two patients developed fever associated with neutropenia; 15 developed systemic fungal infections. We reviewed multiple demographic and treatment characteristics of these patients in an attempt to identify potential risk factors for the development of invasive fungal disease (IFD). Risk factors identified in a univariate analysis included duration of neutropenia after first fever (P less than .0001), diagnosis of acute nonlymphocytic leukemia (ANLL) (P = .003), onset of fever and neutropenia within 5 days of starting induction chemotherapy (P = .009), and multiple (greater than one) surveillance culture sites positive for fungal organisms (P = .02). In a multiple logistic regression analysis, duration of neutropenia (P less than .001) remained a significant risk factor. The study group of patients had a significantly higher risk of fungal infections than a matched group of leukemia patients developing fever with neutropenia due to postremission consolidation chemotherapy (P = .003). In the first 48 patients, 14 (29%) developed IFD. In the subsequent patients (n = 24), intravenous miconazole (5 mg/kg every 8 hours) was begun at the time of the first fever. One of the 24 patients (4%) given miconazole developed IFD. The use of miconazole was a negative risk factor for the development of IFD in univariate (P = .01) and multivariate (P = .05) analysis. We conclude that pediatric leukemia patients who develop fever associated with neutropenia during induction chemotherapy are at high risk for developing IFD. The role of intravenous miconazole at the time of the first fever in this group deserves further study.

Adolescent↗
PubMed1989

The optic nerve as the site of initial relapse in childhood acute lymphoblastic leukemia.

Three children with acute lymphoblastic leukemia (ALL) developed isolated optic nerve relapse as the initial site of disease recurrence. They were part of an early cohort of 39 children with non-B-cell, non-T-cell ALL without central nervous system (CNS) involvement, treated regardless of initial leukocyte count with intrathecal chemotherapy for CNS prophylaxis. Although the optic nerve is a known site of relapse in patients with systemic and meningeal ALL, it has not been reported to occur in otherwise relapse-free patients. Early diagnosis and treatment prevented blindness and allowed for long-term survival (57+, 49+, and 97+ months, respectively) and possibly cure. Since these patients were treated in a new manner and exhibited a new pattern of relapse, their clinical courses were reviewed. Features considered worrisome, but not diagnostic of CNS leukemia may be of greater import when intrathecal medications are utilized as primary CNS prophylaxis. An expanded definition of CNS leukemia may be necessary.

Child↗
PubMed1989

Detection and incidence of neutralizing antibodies to interferon-alpha-n1.

Sera obtained from 612 patients enrolled in 40 different clinical trials with an interferon-alpha-n1 (IFN-alpha-n1) preparation have been tested for neutralizing activity against this IFN in a cytopathic effect inhibition assay. Positive samples were assayed in triplicate. Of 391 patients treated for human papillomavirus infections, 13 developed neutralizing antibodies (3.3%); two suffering from condylomata acuminata and 11 from juvenile laryngeal papillomatosis (JLP). None has positive titers prior to receiving IFN-alpha-n1 and none appeared to lose the benefits of IFN treatment. Serial specimens from four JLP patients demonstrated that titers rose during IFN treatment and declined postherapy. None of the 221 cancer patients from whom serum samples were available had positive titers. The overall incidence of IFN neutralizing sera from these IFN-alpha-n1 recipients was 2.1%.

Adolescent↗
PubMed1989

Recognizing and managing the late effects of cancer treatment.

Treatment for cancer too often adversely affects the very patients it is meant to help, especially children. Long-term damage can be found in the skeleton and soft tissues, in cardiopulmonary and renal function, and endocrine and neurologic function. The authors survey these late effects, including generalized growth suppression, dental and maxillofacial deficits, and pulmonary disorders, and suggest ways to prevent or alleviate them.

Adolescent↗
PubMed1989

Philadelphia-chromosome positive essential thrombocythemia. Two cases in children.

Two cases of children with essential thrombocythemia (ET) with the presence of a Philadelphia chromosome (Ph1) are presented and discussed. Diagnosis was based on their clinical presentation and marked primary thrombocytosis. The site of the Ph1 translocation, as detected by a 1.2-kb bcr genomic probe, differed in the two patients. These cases, along with other reported cases of Ph1-positive ET in the literature, suggest that the presence of the Ph1 cannot be used to rule out a diagnosis of ET. Additionally, the differing translocation sites in these cases suggests that the exact translocation site may not be significant in determining which cell lineage will predominate in a Ph1-positive myeloproliferative disorder.

Adolescent↗
PubMed1988

Randomized surgical adjuvant trial of interferon alfa-n1 in recurrent papillomatosis.

Sixty-six patients with recurrent respiratory papillomatosis of juvenile onset were treated for six months with interferon alfa-n1 (Wellferon) in a randomized crossover trial. Half received interferon alfa-n1 intramuscularly at a dosage of 5 megaunits per square meter daily for 28 days and then thrice weekly for five months, followed by six months of observation. The other half were observed for six months and then treated. Operations were performed every two months to assess disease extent by a scale developed for this purpose. The score for the patients during the first observation period was stable. There was a statistically significant lowering of score in patients receiving interferon alfa-n1 during both periods of drug administration. Eight of 57 patients with assessable airway disease achieved complete remission, as did one additional patient with disease limited to the nasopharynx. No patients achieved complete remission during six months of observation alone. This difference was statistically significant. Patients without tracheostomy were significantly more likely to achieve remission than those with a tracheostomy. The patients who were observed after discontinuation of the drug therapy showed a significant rise in score within four months. Symptoms of toxicity included transient fever, fatigue, nausea, and headache. Elevations in serum aspartate aminotransferase levels occurred in 64% of the patients. There was an inverse correlation between age and the ability to tolerate the medication. The dose studied may be close to the maximum tolerated dose. It appears that interferon alfa-n1 as an adjuvant to routine surgical management is effective in slowing the growth of respiratory papillomas.

Clinical Trials as Topic↗
PubMed1987

Anaplastic sarcoma arising in a mature metachronous bilateral Wilms' tumor after irradiation and chemotherapy. Spontaneous versus induced malignant change.

A 1-year-old male infant developed a classic Wilms' tumor of the left kidney. Treatment consisted of a left nephrectomy, chemotherapy, and irradiation to the left flank and associated abdomen. Two years later, a mass in the right kidney was discovered; open renal biopsy demonstrated a mature Wilms' tumor consisting entirely of rhabdomyomatous elements in the biopsy specimen. The patient was given a second course of chemotherapy and 2000 rad to the right flank. Over the next 8 years, the mass continued to grow without evidence of metastatic spread. Renal function deteriorated secondary to compression of the surrounding normal renal parenchyma by the enlarging tumor; creatinine clearance from the solitary kidney decreased from 120 ml/min to 40 ml/min during the 12 months prior to removal of the lesion. Via a nephron-sparing procedure, the 3400 g tumor measuring 19 cm X 16 cm X 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms' tumor with a malignant anaplastic sarcoma arising in the central portion. Postoperatively, the patient received a third course of chemotherapy with no irradiation to the tumor bed. Currently, he is disease-free with normal renal function more than 20 years after diagnosis of the metachronous bilateral Wilms' tumor. This is the first reported case of an anaplastic sarcoma arising within a Wilms' tumor; this individual also is the longest surviving patient with metachronous Wilms' tumor. The various possibilities regarding the development of the anaplastic sarcoma within the Wilms' tumor of the right kidney are discussed, including the possible role of chemotherapy and irradiation in the development of a second malignancy.

Antineoplastic Agents↗
PubMed1986

Neurofibromatosis and associated neuroectodermal tumors: a congenital neurocristopathy.

The synchronous occurrence of neurofibromatosis and neuroblastoma has been labeled in the recent literature as a chance event. We report 2 cases of newborn infants with congenital neurofibromatosis and a similar midline pattern of multiple Schwann cell and neuroblastic tumors; other types of ectomesenchymal tumor differentiation are documented, along with supportive ultrastructural and immunohistochemical studies. The tumors may take an aggressive, fatal course despite maximal multimodality antitumor therapy. These 2 cases are reported, with additional literature review, to document a clinically recognizable neurocristopathy that links neuroblastic tumors and neurofibromatosis.

Adrenal Gland Neoplasms↗
PubMed1985

Familial osteosarcoma associated with 13;14 chromosomal rearrangement.

Two prepubertal sisters of American Indian origin developed osteosarcoma at 8 and 12 years of age. This familial occurrence, tumor onset prior to puberty, unusual tumor location in one who also had short stature, and ethnic background all suggest an inborn predisposition to bone cancer rather than a chance occurrence. Rearrangements involving chromosomes #13 and #14 were found in both the surviving proband and mother. Comparison of the arm ratio and prometaphase G-banding patterns of the rearranged chromosomes suggests either deletion of band 14q11.2 or pericentric inversion (with breakpoints at 13q12 and 14q11.2) in the proband's rearranged chromosome, but not in her mother's. Her mother, who had no malignancy, had a typical Robertsonian translocation [t(13;14)(p11;q11)]. Three previously reported children with chromosomal abnormalities developed osteosarcoma at unusually young ages, younger even than in reported sibships with osteosarcoma. The most frequently detected cytogenetic abnormalities in sarcoma tumor cells involve chromosomes #13 and #14. In addition, some cases of bilateral retinoblastoma and familial unilateral retinoblastoma, which are known to be at increased risk for osteosarcoma, are associated with tiny deletions on chromosome #13. Thus, there may be a causal relationship between constitutional loss or rearrangement of genetic material at these breakpoints on chromosomes #13 or #14 and development of osteosarcoma in this family that is similar to that seen in patients with small constitutional chromosomal deletions who develop Wilms' tumor and retinoblastoma.

Adolescent↗
PubMed1985

Malignant transformation in non-irradiated recurrent respiratory papillomatosis.

The clinical, radiographic and post mortem findings occurring in a 6-year-old female with a four-year history of recurrent respiratory papillomatosis (RRP) are described. At autopsy, there were two separate foci of malignant transformation (malignant degeneration) in the bronchioloalveolar papillomata. This patient is the youngest in whom such changes have been described. Malignant transformation is a rare occurrence and is usually seen in older patients with longstanding papillomatosis, therapeutic irradiation, or a history of smoking. Pulmonary spread represents the majority of cases with "spontaneous" malignant transformation.

Age Factors↗