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Biomedical subjects

E R Morgan

Publications and source records attributed to E R Morgan.

At least 19 recordsLinked to original sources

Serum erythropoietin levels in children with leukemia.

OBJECTIVE: Our aim was to test the hypothesis that, in leukemic children, serum erythropoietin (EPO) levels vary inversely with hemoglobin. DESIGN: Twenty-four children (15 males, nine females) with an age range of 1-16 years (mean, 7.7 years) diagnosed with acute leukemia (22 acute lymphocytic, two acute myeloid) were studied over 4 months. Serum EPO and hemoglobin were measured simultaneously at multiple time points in the course of their disease, and a multiple regression analysis was performed to describe the EPO-hemoglobin relationship. RESULTS: In a model adjusted for individual subject, there was a significant correlation between hemoglobin and logEPO in these leukemic children (r = -0.55, P < .01, n = 100). When measurements at hemoglobins less than 10.0 were analyzed the correlation increased significantly (r = -0.88, P < .01, n = 21). However, approximately 20% of the observations fell into one of two groups: an inappropriately low EPO for hemoglobin or an inappropriately elevated EPO for hemoglobin. The clinical characteristics of the children at each of these determinations were not different in any manner from the determinations which fell within the 95% confidence intervals for predicted mean EPO value: each of the outlying points came from a patient who at other times had an appropriate EPO for hemoglobin. CONCLUSIONS: There existed a significant inverse relationship between hemoglobin and EPO, suggesting that the feedback mechanism for EPO is intact. Reasons for inappropriately high or low EPO, for level of hemoglobin, are not clear and may be reflective of other aspects of bone marrow or EPO metabolism.

Acute Disease

Prior fungal infection is not a contraindication to bone marrow transplant in patients with acute leukemia.

PURPOSE: Our aim was to assess the feasibility of bone marrow transplantation (BMT) in patients with acute leukemia who have had prior documented invasive fungal infection within 5 months pretransplant treated aggressively with systemic amphoteric in B and, when applicable, surgical resection of the infected tissue. MATERIALS AND METHODS: We reviewed the charts of patients with acute leukemia at our institution who underwent BMT between August 1992 and April 1994 after being treated for a severe fungal infection. We evaluated criteria for diagnosis of fungal infection, timing of infection in relation to BMT, and antifungal treatment modalities. We determined peritransplant complications, evidence for recurrence of fungal infection during BMT, morbidity related to antifungal drug therapy, and overall outcome in each patient. RESULTS: Fungal infection developed in eight patients. Sites of involvement included lung, liver, spleen, and skin. All patients were treated with systemic amphotericin B. Some also underwent surgical resection of infected tissue following clinical control of infection. All patients underwent BMT. Seven of eight patients engrafted and survived BMT. One patient died of recurrent pulmonary mucormycosis. Three patients are alive and free of leukemia and fungal disease. Four patients died of noninfectious causes and had no evidence of fungal disease at the time of death. CONCLUSIONS: Aggressive therapy of prior fungal infection followed by ongoing anti-fungal prophylaxis in acute leukemia patients may allow BMT without reactivation of the fungus. Reports of larger series of such patients as well as studies of the efficacy of chemoprophylaxis of fungal infections are needed.

Adolescent

Haploidentical related umbilical cord blood stem cell transplant in a child with acute non-lymphocytic leukemia.

Umbilical cord blood stem cells (UCBSC) were used to reconstitute hematopoiesis following myeloablative therapy in a 13-month-old infant with acute nonlymphocytic leukemia (ANLL):FAB-M5 who had failed to sustain a chemotherapeutic remission. The patient's mother was 18 weeks pregnant with her second child at the time of diagnosis. Amniocentesis revealed that the fetus was HLA-haploidentical with the patient at the paternally inherited allele. The umbilical cord blood was harvested and processed by Ficoll centrifugation with 100% recovery of 5 x 10(7) mononuclear cells/kg and then cryopreserved. Two weeks after collection the cells were thawed and then infused into the patient following conditioning with total body irradiation, cyclophosphamide, and etoposide. Graft-versus-host-disease (GVHD) prophylaxis consisted of cyclosporine and methotrexate. The patient experienced clinical grade I GVHD consisting of skin involvement only that resolved within 2 weeks following the addition of corticosteroids. Engraftment was achieved with an absolute neutrophil count (ANC) above 0.5 x 10(9)/l on day 16, a platelet count above 50 x 10(9)/l on day 56, platelet transfusion independence on day 32 and red blood cell transfusion independence after day 44. Three months following transplantation restriction fragment length polymorphism (RFLP) revealed no discernible difference between the donor and the recipient. The patient remains in remission without evidence of GVHD 23 months post-transplant.

Antineoplastic Combined Chemotherapy Protocols

Teaching communication skills. An essential part of residency training.

OBJECTIVE: To design a structured curriculum concerning issues of communication with patients and families for use during training of pediatric residents. BACKGROUND: The stimulus for this initiative arose from residents perceived need for such a program and the realization that a structured approach to communication techniques did not currently exist in our residency and, in fact, in many undergraduate and graduate medical education curricula. METHODS: Our program was designed to address complex and difficult areas in physician-patient interaction, including how to deliver "bad news," deal with hostile parents, and speak to children about serious illness; the psychosocial aspects of death and dying were also covered in the program. Various teaching techniques were used. We attempted to assess residents' response and alteration in behavior consequent to the program. RESULTS: The program was successfully incorporated into the training of our residents and was carried out by using existent personnel; minimal expense was incurred. The residents thought the course was valuable and effective, although no statistically significant change in the communication skills of residents could be demonstrated. CONCLUSIONS: The area of physician-patient communication can be taught in a structured fashion during residency. Programs should be devised to meet the changing needs of training during residency and should incorporate the unique strengths of individual institutions.

Adult

Pancreatic blastomatous tumor in a child responding to therapy used for hepatoblastoma: case report and review of the literature.

A case is reported of a 7-year old girl diagnosed with initially inoperable metastatic embryonic pancreatic tumor, which showed a significant clinical response to chemotherapeutic agents commonly used to treat hepatoblastoma. This regimen was selected because certain histologic features of the tumor demonstrated characteristics seen in hepatic tissue. After two course of chemotherapy (cis-platinum and adriamycin), there was a significant reduction of the primary mass, and it was completely resected, although the tumor subsequently recurred in the metastatic, unoperated site. The embryological relationship between the tumor in this patient and hepatoblastoma, as well as the literature concerning treatment in pediatric pancreatoblastic tumors are reviewed. Complete eradication of tumor appears to be necessary for cure.

Antibiotics, Antineoplastic

Pyomyositis during induction chemotherapy for acute lymphocytic leukemia.

PURPOSE: Pyomyositis is a rare disease in temperate climate regions and frequently has a subacute presentation. Because of this, the entity is often misdiagnosed. PATIENTS AND METHODS: Two boys with acute lymphocytic leukemia (ALL) who presented with muscle pain, shortly after receiving induction chemotherapy, were evaluated. RESULTS: Presenting physical examination and laboratory findings were unremarkable except for extremity pain and tenderness. These symptoms were initially attributed to a neurotoxic side effect of vincristine. As the children's symptoms progressed, muscle abscess formation was finally delineated by gallium and computed tomography scans, and the diagnosis of pyomyositis was made. In both cases, the invading organism was Staphylococcus aureus. Both children responded well to incision and drainage of the abscesses and antibiotic therapy. CONCLUSION: Four cases of pyomyositis occurring in ALL patients shortly after induction chemotherapy have now been described. We feel that when children from this population present with muscle pain, pyomyositis should be part of the differential diagnosis. With early medical and surgical intervention, morbidity and mortality can be avoided.

Abscess

Prenatal detection of neuroblastoma by fetal ultrasonography.

PURPOSE: We report three cases of neuroblastoma diagnosed by prenatal ultrasound examination and examine the biologic features of tumors diagnosed prenatally. PATIENTS AND METHODS: Neuroblastoma is the most common tumor detected in the newborn period. Thus, some of these tumors develop prenatally and should be detectable by maternal ultrasound. Here we report three cases in which a neuroblastoma was suspected on prenatal ultrasonography. In addition, we review selected features of 17 additional cases reported in the literature. RESULTS AND CONCLUSIONS: These data indicate that, although the majority of patients have favorable clinical and biological features and do well, some patients do not, and the DNA index may be the most important predictor of outcome.

Female

Renal functional reserve in long-term survivors of unilateral Wilms tumor.

We hypothesized that long-term survivors of unilateral Wilms tumor would have a decreased renal functional reserve secondary to the consequences of hyperfiltration in the nephrons of the remaining kidney. Therefore we evaluated the renal functional reserve in 12 long-term survivors of Wilms tumor after unilateral nephrectomy (mean +/- SE: 15 +/- 1.1 years; range 9 to 23 years). We measured the creatinine clearance before and after an acute, oral protein load to determine the renal functional reserve. Study subjects and control subjects were matched for age, gender, and body surface area. The basal creatinine clearances were similar (Wilms group 132 +/- 13 vs control group 142 +/- 11 ml/min/1.73 m2; p = not significant (NS]. There was no significant difference in the renal functional reserve between long-term survivors of Wilms tumor and matched control subjects (Wilms group 17 +/- 11 vs control group 25 +/- 11 ml/min/1.73 m2; p = NS). The change in creatinine clearance was not secondary to volume expansion because the fractional excretion of sodium was unchanged with protein loading (Wilms group before loading 0.92 +/- 0.12 vs after loading 0.99 +/- 0.13 (p = NS); control group before loading 0.91 +/- 0.12 vs after loading 1.0 +/- 0.14 (p = NS)). We conclude that up to 15 years after nephrectomy for unilateral Wilms tumor in childhood, there is no evidence of hyperfiltration injury.

Adolescent

Single copies of the N-myc oncogene in neuroblastomas from children presenting with the syndrome of opsoclonus-myoclonus.

Patients with neuroblastoma who present with the syndrome of opsoclonus and myoclonus enjoy a remarkably good prognosis independent of their stage of disease or their age at diagnosis. The presence of N-myc amplification also has been found to be an independent prognostic factor in neuroblastoma. Patients with multicopy N-myc tumors have rapid tumor progression whereas those with single-copy tumors have a significantly better progression-free survival. The authors examined four primary, untreated neuroblastomas for the N-myc copy number from patients who presented with opsoclonus and myoclonus. All four tumors had single copies of N-myc, and all four patients are alive with no evidence of recurrent disease with 6+ to 54+ months' follow-up. This appears to be the only report of N-myc analysis in this group of children. It would be interesting to analyze more neuroblastomas from patients who present with opsoclonus and myoclonus to determine how many of these patients have single N-myc copy tumors.

Child, Preschool

The spectrum of metabolic bone disease in lymphoblastic leukemia.

Eight patients with childhood acute lymphoblastic leukemia (ALL) and hypercalcemia, osteopenia, or vertebral compression fractures seen at our institution during the last 12 years were evaluated for biochemical evidence of bone disease. Five patients were hypercalcemic, three had abnormal phosphorous levels, and four had elevated alkaline phosphatase values. Parathyroid hormone (PTH) was measured by a polyvalent radioimmunoassay in five patients and these levels were abnormally high in three patients. Four of these five patients also had PTH measured by a midregion-specific radioimmunoassay. One patient had a high PTH value. Two patients had low levels and one patient had a normal PTH level. Although these studies suggest diverse biochemical mechanisms may be contributing to the bone changes and hypercalcemia seen in childhood ALL, ectopic PTH production as well as ectopically produced fragments of PTH may have a role in mediating bone resorption and hypercalcemia.

Adolescent

Enhancement of antigen expression on acute lymphoblastic leukemia (ALL) cells by cell incubation.

Leukemic cells from blood and/or marrow specimens of 64 acute lymphoblastic leukemia (ALL) patients were examined for the expression of the Ia-like antigen (Ia) or ALL cell-associated antigen (ALLA) employing indirect immunofluorescent techniques before and after overnight incubation (18 hours) at room temperature in Roswell Park Memorial Institute medium 1640. In 20 cases, the post-incubation cells showed an augmented intensity of the fluorescent stain and an increased (greater than 15%) percentage of the ALLA and/or Ia-bearing cells. Evaluation of cell viability after incubation and of non-specific staining in control preparations using normal rabbit serum in lieu of specific rabbit antiserum to ALLA or Ia excluded selective cell death and increased non-specific stain as causes of the observed increase in fluorescent cell staining. The enhanced antigen expression was observed in those samples in which antigens were detectable on only a small fraction of the leukemic cells. The results demonstrate the necessity of re-examination of leukemic cells for the presence of ALLA and Ia following overnight incubation especially in antigen negative cases.

Antigens, Neoplasm

Varicella in immunocompromised children. Incidence of abdominal pain and organ involvement.

The medical records of 31 immunocompromised patients who experienced varicella infections from 1975 to 1982 were reviewed. Fifteen of these patients had visceral involvement. In these 15 patients, two clinical patterns of progression were noted: (1) Eleven patients with life-threatening involvement experienced hepatitis (n = 11), pneumonitis (n = 11), abdominal pain (n = 11), encephalopathy (n = 10), coagulopathy (n = 10), inappropriate antidiuretic hormone (ADH) syndrome (n = 10), back pain or myalgia (n = 5), and myocarditis (n = 1). Seven of these patients survived, all without sequelae. (2) Four patients with a milder course experienced subclinical hepatitis (n = 4), mild pneumonitis (n = 4), postinfectious encephalitis (n = 1), and septic arthritis associated with disseminated intravascular coagulopathy (n = 1). All four of these patients recovered completely. In patients with severe involvement, intense abdominal pain was frequently the first sign of dissemination. Abdominal pain and inappropriate ADH syndrome were unexplained and have not been previously described in progressive varicella. A predictable pattern of organ involvement enabled starting therapy early and resulted in the survival of 11 of 15 patients.

Abdomen

Wilms' tumor.

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Child

Myelofibrosis in neuroblastoma.

Myelofibrosis, a rare childhood disorder, has been reported as an associated complication of certain hematologic malignancies or as an isolated idiopathic process. In this report, we describe a patient with metastatic neuroblastoma whose initial presentation included the findings of myelofibrosis and leukoerythroblastosis. The myelofibrosis regressed with chemotherapy and did not recur when the patient's tumor reappeared.

Abdominal Neoplasms

Endodermal sinus tumor: a clinical and pathological correlation.

Endodermal sinus tumors occur in the gonads and in a variety of extragonadal locations, particularly as the malignant component of sacral teratomas. Light and electron microscopic studies as well as the presence of alpha fetoprotein support the concept that these lesions arise from germ cells that migrate from the extraembryonic yolk sac. These tumors in all locations are highly malignant and metastasize to lymph nodes, lung, liver and bone. Peritoneal implants are seen from tumors in the ovary, testis, and following ventriculoperitoneal shunts for yolk sac tumors of the pineal gland. There is no satisfactory therapy for the endodermal sinus tumor, regardless of location, when there are distant metastases at the time of diagnosis; however, we have documented complete regression of metastases in one child following therapy with vinblastine, bleomycin, and cis-platinum. Nine of 13 boys with endodermal sinus tumors of the testis survived in this series of patients. All survivors were treated with orchiectomy and lymph node excision. Surgical excision combined with chemotherapy and radiation led to survivors in children with large extragonadal pelvic and retroperitoneal tumors. We recommend vigorous multimodal therapy with surgery and chemotherapy. Radiation is given to the primary lesion when it is too extensive for initial resection or to eradicate residual disease following chemotherapy. Alpha fetoprotein is a valuable tumor marker.

Adolescent

Ovarian granulocytic sarcoma as the primary manifestation of acute infantile myelomonocytic leukemia.

The first known instance of granulocytic sarcoma of the ovary as the presenting manifestation of acute nonlymphocytic leukemia in infancy is reported. The clinical and laboratory findings for a 3-month-old infant who had anemia and a lower abdominal mass subsequently found to represent bilateral ovarian granulocytic sarcomas are discussed. Results of light and electron microscopic examination of bone marrow biopsy specimen were consistent with a diagnosis of acute myelomonocytic leukemia. Hematopoietic (and lymphoproliferative) disorders must be included in the differential diagnosis of ovarian masses in children.

Bone Marrow

Nail hyperpigmentation secondary to therapy with doxorubicin.

Hyperpigmentation of the fingernails can occur during therapy with doxorubicin (Adriamycin). Two patterns are common: diffuse, and transversely banded. The pigment is believed to be melanin, and both patterns disappeared with discontinuation of therapy and subsequent nail growth.

Adolescent