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Biomedical subjects

D J Fernbach

Publications and source records attributed to D J Fernbach.

At least 37 records · Page 2Linked to original sources
PubMed1985

Prognosis for Wilms' tumor patients with nonmetastatic disease at diagnosis--results of the second National Wilms' Tumor Study.

Multivariate statistical methods were used to study prognosis for 632 patients entered on the second National Wilms' Tumor Study who had nonmetastatic, unilateral disease at diagnosis. Separate analyses were conducted for each of four endpoints: abdominal recurrence, distant metastasis, relapse without regard to site, and death. The two most important predictors for metastasis and general relapse were an unfavorable (anaplastic or sarcomatous) histology and the presence of microscopically confirmed disease in the regional lymph nodes. Operative spillage of tumor increased the rates of abdominal recurrence and death, even after accounting for histology and lymph node effects. The presence of a tumor thrombus in the renal vein or IVC increased the risk of metastasis, and intrarenal vascular invasion was associated with general relapse after accounting for histology, lymph nodes, and spillage. However, these latter associations were weaker, and some uncertainty remains regarding the true prognostic import of such findings due to a high degree of collinearity among variables. By contrast to the results of a similar data analysis for the first National Wilms' Tumor Study, specimen weight had no bearing on outcome, and the effects of age at diagnosis were entirely explained by the association of age with other more critical factors.

Adolescent↗
PubMed1985

Early detection of bacteremia in an outpatient clinic.

A total of 570 patients, aged less than 24 months, who were seen in the emergency room at Texas Children's Hospital for evaluation of acute febrile illness had a WBC count, differential count, and a blood culture obtained. Bacteremia occurred in 7.7% (n = 44) (22 Haemophilus influenzae; 17 Streptococcus pneumoniae; three Escherichia coli; one group B Streptococcus; one Staphylococcus aureus). The sensitivity, specificity, and positive predictive value of morphologic changes (vacuolization and toxic granulation) of polymorphonuclear neutrophils (segmented neutrophils plus band cells), WBC count greater than or equal to 15,000/microL and band cells greater than or equal to 500/microL were examined. Additionally, abnormalities in three of four tests (WBC count greater than or equal to 15,000/microL, band cells greater than or equal to 500/microL, segmented neutrophils greater than or equal to 10,000/microL, and/or total polymorphonuclear neutrophils greater than or equal to 10,500/microL) were evaluated. Morphologic changes of polymorphonuclear neutrophils were noted frequently in patients with culture-proven bacteremia (63% and 51% positive predictive value for vacuolization and toxic granulation, respectively) and were more predictive of bacterial infection than traditional tests. If both vacuolization and toxic granulation were present, the positive predictive value increased to 76%. Examination of the peripheral blood smear may provide important adjunctive information for the presence of bacteremia prior to bacteriologic confirmation.

Ambulatory Care↗
PubMed1984

Childhood cancer and occupational radiation exposure in parents.

To test the hypothesis that a parent's job exposure to radiation affects his or her child's risk of cancer, the authors compared this exposure during the year before the child's birth for parents of children with and without cancer. Parents of children with cancer were no more likely to have worked in occupations, industries, or combined occupations and industries with potential ionizing radiation exposure. Bone cancer and Wilms' tumor occurred more frequently among children of fathers in all industries with moderate potential ionizing radiation exposure. Children with cancer more often had fathers who were aircraft mechanics (odds ratio (OR) = infinity, one-sided 95% lower limit = 1.5; P = 0.04). Although four of these six were military aircraft mechanics, only children whose fathers had military jobs with potential ionizing radiation exposure had an increased cancer risk (OR = 2.73; P = 0.01). Four cancer types occurred more often among children of fathers in specific radiation-related occupations: rhabdomyosarcoma among children whose fathers were petroleum industry foremen; retinoblastoma among children whose fathers were radio and television repairmen; central nervous system cancers and other lymphatic cancers among children of Air Force fathers. Because numbers of case fathers are small and confidence limits are broad, the associations identified by this study need to be confirmed in other studies. Better identification and gradation of occupational exposure to radiation would increase the sensitivity to detect associations.

Adult↗
PubMed1984

Newborn screening for sickling hemoglobinopathies. Houston, 1976 to 1980.

Fifty of 52 infants with cord blood hemoglobin electrophoresis patterns indicative of a possible sickling hemoglobinopathy (SH) were followed up prospectively. A retrospective group of 39 children whose mothers were contacted two to four years after the birth of a child with a positive newborn screening test formed a comparison group. During an average follow-up period of 30 months, children of mothers who were informed early of a positive cord blood test were hospitalized more readily for complications of an SH. During this same period, five (6%) of 88 infants with fetal and sickle hemoglobin (FS) or fetal, sickle, and C hemoglobins (FSC) in their cord blood died, all before 6 months of age. All five deaths occurred in infants whose parents were unaware of their child's disease; four of the five deaths may have been caused by sepsis. These results alone did not prove that screening significantly reduces mortality but did show that newborn screening increases parental awareness of complications and may, therefore, improve the prognosis in sickle cell disease in infancy.

Anemia, Sickle Cell↗
PubMed1984

Elevated myelin basic protein levels in the cerebrospinal fluid of children with acute lymphoblastic leukemia.

Cerebrospinal fluid was examined from 70 children with acute lymphoblastic leukemia for evidence of active myelin breakdown based on the release of myelin basic protein (MBP). Fifty-three asymptomatic children were followed from diagnosis with serial MBP determinations. Eight (15.1%) of 53 children had abnormal elevations of MBP, six of eight before receiving presymptomatic central nervous system therapy. Long-term observations are in progress. For comparison, six children with clinical and radiologic findings of leukoencephalopathy had abnormal MBP determinations, whereas no abnormalities were detected in 11 children with meningeal leukemia.

Adolescent↗
PubMed1984

Degenerative changes in neutrophils: an indicator of bacterial infection.

A review was made of 195 peripheral blood smears of 157 neonates who required sepsis work-up in the first month of life. Degenerative changes of neutrophils including vacuolization and toxic granulation were observed frequently in infants with culture-proven bacterial sepsis. Of 21 peripheral blood smears from 20 neonates subsequently proven to have bacterial infection, 17 had vacuolization present in the neutrophils for a sensitivity of 81%, a specificity of 93%, and a positive predictive accuracy of 59%. Toxic granulation changes in neutrophils showed similar results. This simple test can be performed easily in all hospitals; it does not require special laboratory facilities. The test appears to provide a valuable adjunct in the early detection of neonatal bacterial infection.

Bacterial Infections↗
PubMed1983

Circulating immune complexes in childhood malignancies: a Pediatric Oncology Group study.

Pretreatment serum samples obtained at diagnosis from 89 children with various pediatric malignancies were examined for circulating immune complexes (CIC) using the [125I]Clq binding assay. The study population consisted of 35 children with acute lymphocytic leukemia (ALL), 22 children with acute non-lymphocytic leukemia (ALL), 24 with neuroblastoma (NB), and eight with osteosarcoma (OS). Concomitant quantitation of immunoglobulins was performed in 55 patients, revealing normal values for age. Increased levels of CIC at diagnosis were found in 9%, 22%, 42%, and 50% of children with ALL, AML, NB, and OS, respectively. Except for a higher proportion of CIC-positive patients observed in stage IV NB (nine of 17) compared to stage I-III NB (one of seven), no correlation was observed between initial CIC level and presenting clinical features, response to treatment, prognosis, or presence of infection. Longitudinal sampling of six NB and two OS patients did not reveal a clear relationship between disease activity and quantity of CIC. For the pediatric malignancies studied, these data demonstrate minimal value in quantitating CIC as a means of assessing disease activity or predicting response to treatment and are in contrast to the apparently adverse effect of elevated pretreatment CIC on response to therapy and survival observed in adults with ALL, AML, and OS.

Adolescent↗
PubMed1982

Wilms tumor with aniridia/iris dysplasia and apparently normal chromosomes.

Two patients with Wilms tumor, iris dysplasia (complete aniridia in one and subtle iris defects in the other), normal karyotypes, and no gene loss demonstrable by enzyme marker and direct DNA analyses are presented. The findings indicate that aniridia and less severe iris defects define a risk for Wilms tumor even in the absence of del (11p13), and that there is as yet no consistent biochemical genetic marker for the aniridia-Wilms tumor association.

Adolescent↗
PubMed1981

Childhood acute leukemia: a search for occult extramedullary disease prior to discontinuation of chemotherapy.

Between January 1978 and September 1979, 29 children with acute leukemia in complete continuous remission for three or more years were examined for evidence of occult extramedullary disease immediately prior to discontinuation of chemotherapy. Bilateral open wedge testicular biopsy demonstrated the presence of bilateral leukemic infiltrates in 2/13 boys. Gallium scans prior to biopsy had identified unilateral infiltration in an enlarged testis in one patient but failed to identify microscopic disease in the opposite testis or in the testes of the second patient. Percutaneous kidney and liver biopsies, pelvic ultrasonography, intravenous pyelogram, skeletal survey, cranial computed axial tomography scan, electroencephalography, and ophthalmologic examinations failed to demonstrate evidence of occult disease. Except for testicular biopsy, this study does not support extensive clinical or invasive procedures to identify extramedullary disease prior to discontinuation of chemotherapy.

Adolescent↗
PubMed1981

Myeloproliferative diseases in childhood.

The experience with myeloproliferative diseases (MPD) in children of a large pediatric hematology-oncology service during a 20-year period is reviewed. Twenty-seven patients with myeloproliferative diseases were treated, six with the juvenile type of chronic myelogenous leukemia (CML), 10 with the adult type of CML, three with familial MPD, one with unclassifiable MPD, and seven with acute leukemia in whom myelofibrosis was either a prodromal or terminal event. The literature is reviewed with particular emphasis regarding the relationships between the juvenile type of CML and monocytic leukemia, the adult type of CML and acute nonlymphocytic leukemia, and the relationship of myelofibrosis and myeloid metaplasia to the acute leukemias. New therapeutic approaches are needed in this heterogenous but interrelated group of disorders.

Adolescent↗
PubMed1980

Comparison of prednisolone, vincristine, methotrexate and 6-mercaptopurine vs. 6-mercaptopurine and prednisone maintenance therapy in childhood acute leukemia: a Southwest Oncology Group Study.

The study was designed to compare the relapse rate and toxicity of intermittent prednisone, oncovin (vincristine), methotrexate, and 6-mercaptopurine (POMP) vs. 6-mercaptopurine daily and a prednisone pulse every three months (PIP) for maintenance therapy in acute leukemia. Children with acute lymphoblastic, acute undifferentiated, or acute stem-cell leukemia were stratified on the basis of initial leukocyte count and age, then randomly assigned to POMP or PIP maintenance therapy. All patients received cranial irradiation and intrathecal chemotherapy. Of the 67 patients receiving POMP maintenance, 20(30%) remain in continuous remission. The median length of continuous remission achieved with POMP therapy was 49 weeks. Of 80 patients receiving PIP maintenance, 25(31%) remain in remission. The median length of continuous remission for PIP was 62 weeks. Of the possible prognostic factors evaluated, the only significant factor was the prognostic grouping base in age and initial leukocyte regimen was associated with a higher incidence of toxic reactions, frequently causing therapy interruption. The results for both regimens as studied are inferior to those for 6-mercaptopurine methotrexate maintenance regimens as reported by others.

Age Factors↗
PubMed1979

Childhood multifocal osteosarcoma--diagnosis by 99mtechnetium bone scan: a case report.

A 14-year-old boy with osteosarcoma presented with evidence of a solitary bone lesion by clinical and radiographic examination. A preoperative 99mtechnetium bone scan revealed multiple skeletal osteoblastic abnormalities and upon biopsy of one of these lesions, the diagnosis of multifocal osteosarcoma was established. This unusual case dramatically illustrates the value of 99mtechnetium bone scan in preoperative staging of patients with osteosarcoma.

Adolescent↗