Parenting and child distress during cancer procedures: a multidimensional assessment.

We examined the relationship between children's distress during invasive cancer procedures and parent anxiety, parent disciplinary attitudes, and parent behavior during the medical procedure. Sixty-six children with cancer and their parents were evaluated during a routine bone marrow aspiration. Significantly higher levels of distress were obtained for young (under age 8) versus older children. Patterns of relationships with parent variables also varied by age. Anxious parents of young children reported relying on less effective discipline strategies. They also were less reassuring prior to to medical procedure. Age differences in the correlations between child distress and parenting are discussed in terms of developmental differences in children's dependence on caregivers for emotional regulation and control. Implications for clinical distress reduction programs are also discussed.

Soft-tissue sarcomas other than rhabdomyosarcoma in children.

Forty-seven children with nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) were treated by the Hematology-Oncology Service at Texas Children's Hospital, Houston, Texas, between 1958 and 1990. The male:female ratio was 1:1, and the median age was 11 years (3 weeks-16 years). A preexisting condition was found in 9/47 (19%) patients including neurofibromatosis (3), Down's syndrome (1), spina bifida (1), congenital facial asymmetry (1), giant pigmented nevus (1), juvenile onset diabetes mellitus (1), and acquired immune deficiency syndrome (1). The site of primary tumor was head and neck (3), trunk (33), and extremities (11). Twenty-four patients (51%) have survived free of disease with a median follow-up of 5 years (4 months-22 years). No patient whose disease recurred achieved a second remission. Of the 19 patients with group I disease, 16 (84%) survived free of disease. Wide excision of the primary tumor, with no microscopic residual disease, was associated with the greatest chance of disease-free survival.

The treatment of Wilms' tumor patients with pulmonary metastases detected only with computed tomography: a report from the National Wilms' Tumor Study.

To evaluate the prognosis of patients with Wilms' tumor who have pulmonary densities identified on a computed tomographic (CT) scan of the chest, but have a negative plain chest radiograph, we reviewed the treatments and outcome of 32 patients randomized or followed on National Wilms' Tumor Study (NWTS)-3. The 4-year event-free and overall survival percentages of 18 of these patients who had a favorable histology tumor and were treated as stage IV tumors with three or four drugs plus whole-lung irradiation were 88.1% and 94.0%, respectively. The 4-year event-free and overall survival percentages for nine favorable histology patients treated less aggressively based on the extent of locoregional disease with two or three drugs and without whole-lung irradiation were 88.9% and 88.0%, respectively. There were no statistically significant differences in the 4-year event-free or overall survival percentages between the two groups. The current data do not demonstrate improved survival for favorable histology patients treated with whole-lung irradiation for pulmonary metastases identified only on chest CT scan. However, due to the small number of patients included, no statistically valid conclusions regarding the roles of Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH) and/or whole-lung irradiation in the treatment of these patients can be drawn from the present analysis. Additional patients need to be systematically studied to determine if these preliminary observations can be confirmed.

Essential thrombocythemia in a child: management with anagrelide.

Essential thrombocythemia (ET) is a rare disorder in children. An 11-year-old white boy was first seen in January 1986 with symptoms of abdominal pain. His platelet count was 1.5 million/mm3. Other hematological values and coagulation studies, including bleeding time, were normal. There was laboratory evidence of mild platelet dysfunction. Using the criteria of the Polycythemia Vera Study Group, a diagnosis of ET was made. He developed frequent headaches. Aspirin was prescribed for the next 2 years at varying doses and frequency. During the period, platelet counts ranged between 1 and 3 million/mm3. In view of progressive headaches and evidence of increasing platelet dysfunction, further treatment was indicated. The use of a new agent, anagrelide, reported effective in adults with ET, resulted in amelioration of symptoms and improvement in quantitative and qualitative platelet control with no significant untoward effects.

Toxicity of high-dose cytosine arabinoside in the treatment of advanced childhood tumors resistant to conventional therapy. A Pediatric Oncology Group study.

Experience with high-dose cytosine arabinoside (HDAC) in pediatric solid tumors is limited. Sixteen children with solid tumors resistant to conventional therapies were registered in a pilot Pediatric Oncology Group (POG) study that required the administration of HDAC at 3 g/m2 every 12 hours for four doses. There were four cases of rhabdomyosarcoma, two cases of fibrosarcoma, four cases of neuroblastoma, and one case each of germ cell tumor, Wilm's tumor, retinoblastoma, hepatocellular carcinoma, Ewing's sarcoma, and Burkitt's lymphoma. All eligible patients had advanced diseases and had previously received extensive chemotherapy. Thirteen patients received one course of HDAC and three patients received two courses of HDAC. Due to prior treatments, patients had less than normal marrow reserves. Short-term toxicity included nausea, vomiting, suppression of hemopoiesis, drug fever, and increased blood urea nitrogen (BUN), creatinine, and liver enzymes. All evaluable patients recovered from their toxicities. There were no drug-related deaths. None of the patients had neurologic problems, including the only patient with prior irradiation to the skull. With the above schedule, HDAC appears to have manageable toxicity.

Value of the postmortem examination in a pediatric population with leukemia.

Because of concerns about the declining autopsy rate, an attempt was made to evaluate the contributions from the postmortem examination in a pediatric population with leukemia. Accordingly, 161 autopsies performed between 1970 and 1985 were reviewed and the diagnoses compared with those listed in the clinical records of the same patients. New diagnoses were grouped into diagnoses of diseases thought to have contributed to the death of the patient, those of diseases thought to have contributed to the morbidity of the patient, those of presumed toxic reactions to drug therapy, and diagnoses of academic interest. The newly diagnosed diseases thought to have contributed to the patient's death either had been totally unsuspected by the clinician or had been suspected but incorrectly diagnosed. The most common revelation was the identification of mycotic infections that were thought by the clinician to have been bacterial in origin. The clinical diagnosis of these kinds of infections progressively improved during the study period. Because the changes in diagnoses and therapy, particularly the increasing use of antimycotic therapy, could be directly attributed to autopsy findings and, more recently, because of the expanding use of more toxic multiagent chemotherapy, we believe that the postmortem examination remains an important procedure, even in clinical situations where much is known about the patient.

Typhlitis. An 18-year experience and postmortem review.

A review of pediatric autopsy results at the Texas Children's Hospital, Baylor College of Medicine from 1970 through 1987 was conducted. Thirty-three cases of typhlitis were identified in patients with acute leukemia and two cases each in patients with lymphoblastic lymphoma and aplastic anemia. Patients ranged in age from 10 months to 17 years. Fifty-seven percent were male and 43% were female. All were myelosuppressed. A postmortem incidence rate of 24% was determined for patients with acute leukemia. Common symptoms included abdominal pain and distention in 78% of patients and acute lower gastrointestinal bleeding in 35%. Abdominal radiographs varied in spectrum from a nonspecific bowel gas pattern to frank right colonic pneumatosis intestinalis. Thirty-three patients received chemotherapy within 30 days before onset of abdominal symptoms. All patients were febrile (greater than 38.5 degrees C), and 33 received broad-spectrum antibiotics. Three patients received amphotericin B. Premortem, 84% of organisms cultured from blood were bacterial whereas 16% were fungal. Fungal pathogens accounted for 53% of new microorganisms seen at autopsy. Postmortem examination showed typhlitis in the following anatomic distributions: (1) confined to the cecum; (2) involving the cecum and ileum; (3) involving the cecum, ileum, and ascending colon; or (4) involving the cecum, with sporadic ulcers throughout the intestine. This review includes clinical and postmortem features of typhlitis and current strategies for diagnosis and management.

Bone marrow transplantation in childhood leukemia using reverse isolation techniques.

Between 1979 and 1986, 29 pediatric patients underwent bone marrow transplantation at Texas Children's Hospital using routine reverse isolation. Laminar air flow rooms, prophylactic antibiotics, and gut sterilization were not utilized. The diagnoses included acute lymphocytic leukemia (ALL) (16 patients), acute nonlymphocytic leukemia (ANLL) (10 patients), and chronic myelogenous leukemia (CML) (three patients). All patients had fever during hospitalization. There were 11 episodes of bacteremia in seven patients giving a bacteremia rate of 37.9%. Moderate-to-severe (grade II-IV) acute graft-versus-host disease (GVHD) was seen in eight patients (27.6%). The incidence of infection and GVHD during the first 100 days post-transplantation is comparable to published reports from centers utilizing rigid isolation and sterilization of the gut. It is suggested that bone marrow transplantation may be done using standard reverse isolation techniques without increasing the morbidity or mortality of the procedure.

Successful outcome of severe aplastic anemia following Epstein-Barr virus infection.

Severe aplastic anemia has been reported in children following Epstein-Barr virus (EBV) infection. The pathophysiology of disease is not known, but an immunologic mechanism is suspected because of the rapid response to steroids. A patient with severe aplastic anemia following EBV infection experienced bone marrow recovery after treatment with high-dose prednisone. After reviewing the literature it is concluded that steroid therapy is indicated in this select group of patients.

Human leukocyte alpha interferon-induced transient neurotoxicity in children.

Neuropsychiatric complications with recombinant alpha interferon (rIFN-alpha) have been described in adults. Little documentation of similar toxicity in pediatric phase I and phase II trials exists. Two children, treated with rIFN-alpha, experienced confusion, somnolence and syncope, associated with transient electroencephalographic abnormalities. Symptoms abated and electroencephalographic findings improved with termination of therapy. Readministration at lower dosages has not been associated with recurrence of neurotoxicity.

Head and neck malignancies in children: an age-incidence study.

One hundred forty-seven (147) children under 18 years have been treated for head and neck malignancies at Texas Children's Hospital from 1970 to 1985 and form the data base for this study. In order of frequency, the most common tumors were lymphoma, Langerhans' histiocytosis (LH), rhabdomyosarcoma, neuroblastoma, and other soft-tissue sarcoma. Children under 2 were most likely to have LH; those from 3 to 5, LH or lymphoma; and those over 5, lymphoma.

Malignant rhabdoid tumor of the extremity.

Two cases of malignant rhabdoid tumor (MRT) involving the sciatic nerve are described. Despite the close affiliation of the tumor with neural tissue, staining characteristics in these patients do not suggest a neural origin of MRT. Survival in patients with renal and extrarenal MRT has been poor. Our patients were treated with an aggressive chemotherapy program using cisplatin, Adriamycin (doxorubicin), vincristine, cyclophosphamide, actinomycin D, and DTIC. One child died of progressive disease; the other is well 35 months after diagnosis.

Effect of trimethoprim/sulfamethoxazole prophylaxis on outcome of childhood lymphocytic leukemia. A Pediatric Oncology Group Study.

The Pediatric Oncology Group (POG) undertook a prospective randomized trial using a single chemotherapy regimen with or without trimethoprim/sulfamethoxazole (TS). In a previous acute lymphocytic leukemia (ALL) study of initial therapy, investigators were free to use TS prophylaxis or not. Analysis of those data seemed to favor TS for duration of continuous complete remission. In the study reported here, of 126 randomized patients with ALL, 63 received TS. There was no effect of TS on disease-free survival after 3 years follow-up. Overall severe toxicity did not differ. However, granulocytopenia was somewhat more severe in the TS group. Hepatic toxicity, measured by enzyme elevation approached significance in the TS group versus controls. Some institutions declined randomization and treated with or without TS as a routine. Outcome and toxicities did not differ from randomized patients. There was no statistically significant effect on severe, life-threatening or fatal infection between the randomized TS versus control groups. Children not receiving TS developed varicella more often, a disease for which one would not expect TS to show a preventative effect. Pneumocystis pneumonias were not reported. The authors conclude that TS prophylaxis did not increase the continuous complete remission rate in children with ALL or decrease the incidence of infection. Toxicity is somewhat higher on TS.

Bilateral Wilms' tumor.

Recent studies indicate a favorable outcome with bilateral Wilms' tumor. From 1971 to 1985, ten children between the ages of 6 months and 5 years were treated for this disease. During the early part of the series, five patients had nephrectomy on one side and partial nephrectomy on the other side, and one patient had bilateral partial nephrectomies at the same operation. More recently four patients initially had a biopsy of both tumors and lymph node sampling followed by chemotherapy. At the second-look procedure two patients had multiple biopsies because there was no obvious tumor. Histologically there was no tumor on the third-look procedure in these two patients. Nine patients had a favorable histology of the Wilms' tumor. One patient had a favorable histology on one side and an unfavorable type of histology on the other side. Eight patients are surviving between 6 months and 13 years. Two died of extensive disease within 16 months of diagnosis. One patient had an unfavorable histology. The good results following partial nephrectomies have led us to attempt to conserve additional tissue, as has been done in the last four patients. Our early results suggest biopsy of the tumor followed by chemotherapy, then a second look, and if necessary, third-look procedures may result in preservation of functioning renal tissue.

Extraneural metastases from medulloblastoma: long-term survival after sequentially scheduled chemotherapy and radiotherapy.

Extraneural relapse of medulloblastoma is an uncommon event which has typically been associated with a fatal outcome. We present a case of a 2 1/2-year-old male who developed extensive bone marrow and bone metastases 19 months after diagnosis and treatment of a medulloblastoma. This patient was successfully treated with a sequentially scheduled combination of vincristine, cyclophosphamide, and doxorubicin and remained free of disease for more than 5 years from the time of relapse.

The role of the family physician in the care of the child with cancer.

Cancer in the child is no longer a death sentence. Although the treatment has become complex, the survival rate has increased impressively. Modern treatment involves a multidisciplinary approach, which should include the primary physician as a key member of the highly skilled treatment team.

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.