Burnout, staff support, and coping in Pediatric Oncology.

Goals Of Work: The goals of the study were the following: (1) to study the rate of burnout of the staff in Pediatric Oncology and compare it with that of a group of staff in other pediatric specialties, (2) to find out if job satisfaction, role clarity, staff support, and ways of coping are related to the burnout of these two groups, and (3) as a secondary aim, to identify other parameters, i.e., profession, experience, having children, etc.

Second and third malignant solid tumor in a girl with ovarian Sertoli-Leydig tumor.

We report a Sertoli-Leydig cell (SLC) tumor of the right ovary in a 10-year-old girl, which was dealt with surgical removal. Three months after resection, she presented with a new episode of acute abdomen because of an abdominal mass, which histologically was compatible with an undifferentiated embryonal rhabdomyosarcoma. Chemotherapy, according to SIOP-??? 89 protocol, was administered additionally to radiotherapy (3,960 cGy).

Infections in a pediatric patient cohort with acute lymphoblastic leukemia during the entire course of treatment.

Goals: To assess the type, frequency, severity, and outcome of all infectious episodes in a pediatric patient cohort with acute lymphoblastic leukemia (ALL) from a single institution during the entire length of leukemia treatment.

Patients And Methods: Eighty-six patients were treated according to a modified ALL Berlin-Frankfurt-Munster protocol. Retrospective analysis of all types of infections according to the treatment phase and the degree of neutropenia is presented.

Classic transient erythroblastopenia of childhood with human parvovirus B19 genome detection in the blood and bone marrow.

The etiology of transient erythroblastopenia of childhood (TEC) remains unknown, although an association with viral infections has been proposed. The authors describe a 3.5-year-old girl with classic TEC concomitantly with human parvovirus B19 (HPV) infection.

Decrease of CD4+ and B-lymphocyte populations is not associated with severe infectious complications in children with acute lymphoblastic leukemia during maintenance.

The suppression of lymphopoiesis and immune competence during the maintenance phase in children with acute lymphoblastic leukemia (ALL) and the occurrence of infectious complications remain an unexplored area. In this study we assessed lymphocyte subpopulation disturbances during maintenance for childhood ALL along with the incidence, type, and severity of infections that occur during that period in the absence of neutropenia. Twenty-eight children (13 boys, 15 girls) with ALL aged 3-14 years (median 7 years) and treated according to the ALL-BFM 90/95 protocol were studied during maintenance for ALL.

Clinical and morphological features of paediatric myelodysplastic syndromes: a review of 34 cases.

Background: The clinical and morphological spectrum of myelodysplastic syndromes (MDS) during childhood has not yet been completely documented. We herein present the clinical features and morphological data from peripheral blood (PB), bone marrow aspirates (BMA) and bone marrow biopsies (BMB) of a series of paediatric MDS patients, with particular emphasis on their specific morphological characteristics and their diverse underlying genetic background.

Patients And Methods: Thirty-four patients with MDS (median age 8.

Osteosarcoma as a second tumor after treatment for primary non-Hodgkin's lymphoma in a child with ataxia-telangiectasia: presentation of a case and review of possible pathogenetic mechanisms.

Patients with ataxia-telangiectasia (A-T) and cancer are exposed to additional toxicity due to their underlying inability to repair chemotherapy-induced DNA damage. The authors report the development of osteosarcoma as a second neoplasia in a child with A-T who was treated, without being irradiated, for non-Hodgkin's lymphoma as a primary malignancy. This is the first report of osteosarcoma associated with A-T.

Nasopharyngeal carcinoma in childhood and adolescence: a single institution's experience with treatment modalities during the last 15 years.

Pediatric nasopharyngeal carcinoma (NPC) represents a locally advanced undifferentiated tumor with widely varying epidemiological features and with a high cure rate when combined modality treatment is provided. Both local and systemic treatment is necessary, and additional treatment with biologic modifiers seems promising. In this study, clinical experience and therapeutic results of 10 children with newly diagnosed NPC, treated in a single pediatric hematology/oncology institution in Athens over a period of 15 years, are analyzed.

Ineffective erythropoiesis underlies the clinical heterogeneity of congenital dyserythropoietic anemia type II (CDA II).

Background: Congenital dyserythropoietic anemia type II (CDA II) is an autosomal recessive disease, and is the most common CDA. The qualitative and quantitative defects of erythropoiesis in CDA II, as estimated by the hematological and biochemical parameters at the time of diagnosis, may not reflect the heterogeneity of the disease course of each patient.

Methods: Three pediatric patients with CDA II are herein presented, having an heterogeneous clinical course.

Correlation of cerebrospinal fluid beta-glucuronidase activity with plasma methotrexate concentrations in leukemic children receiving high-dose methotrexate.

Background: The activity of lysosomal enzymes is increased in body fluids during inflammation, in which cellular malfunction and cellular death occurs. Because chemotherapy also causes cell malfunction and death, for identifying a neurologic effect, we studied the activity of beta-glucuronidase in the cerebrospinal fluid (CSF) of leukemic children during treatment.

Procedure: The beta-glucuronidase activity in CSF was determined in 13 patients with B-precursor acute lymphoblastic leukemia (ALL) treated with the medium risk arm of ALL Berlin-Frankfurt-Munster (BFM) 95 protocol.

Cerebellar granulocytic sarcoma in an infant with CD56+ acute monoblastic leukemia.

Granulocytic sarcoma (GS) is a form of extramedullary leukaemia (EML). The presence of the neural cell adhesion molecule (NCAM) on the surface of the blasts, which is recognized by the CD56 monoclonal antibody, enhances their propensity for tissue penetration. GS within the central nervous system (CNS), in particular within the cerebellum, is extremely uncommon.

Chromosome fragility and predisposition to childhood malignancies.

In order to clarify the possible connection between autosomal folate sensitive Fragile Sites (FS) and genetic susceptibility to haemopoetic disease in children we investigated the frequency and distribution of FS in the Peripheral Blood Lymphocytes (PBL) of 56 children with newly diagnosed and untreated haematologic malignancies and their parents. The incidence was compared with that of 146 normal controls (children and adults). In all patients the Bone Marrow (BM) karyotype was also determined.

Aggregation of childhood leukemia in geographic areas of Greece.

A total of 872 children aged up to 14 years, who were diagnosed with leukemia in Greece during the decade 1980-89, were allocated by place of residence to the 601 administrative districts of the country. Evaluation of spatial clustering was done using the Potthoff-Whittinghill method, which validly assesses heterogeneity of leukemia risk among districts with variable expected numbers of cases. There was highly significant evidence for spatial clustering occurring particularly among children living in urban and, to a lesser extent, semi-urban areas.

Immune response of immunocompromised children with malignancies to a recombinant hepatitis B vaccine.

The aim of this study was to interpret the antibody response to hepatitis B vaccination following an intensified four-dose schedule in 140 cancer patients who presented at our clinic between January 1, 1993 and December 31, 1994. According to therapy status, the patients were divided into two groups: group A consisted of 76 patients undergoing chemotherapy and group B of 64 patients in complete remission and off treatment. The eligibility requirements were negative hepatitis B virus (HBV), HCV, and human immunodeficiency virus serologic markers.

Infant leukaemia after in utero exposure to radiation from Chernobyl.

There has been no documented increase in childhood leukaemia following the Chernobyl accident. However, different forms of childhood leukaemia may not be equally susceptible to radiation carcinogenesis. Infant leukaemia is a distinct form associated with a specific genetic abnormality.

Space-time clustering of childhood leukaemia in Greece: evidence supporting a viral aetiology.

The method introduced by Knox for evaluation of space-time clustering has been applied to 872 cases of childhood (0-14 year old) leukaemia diagnosed in Greece over the 10 year period 1980-89. Greek towns are characterised by substantial population mixing due to internal migration, whereas there is relative isolation in mountainous rural areas. Predetermined space (5 km) and time (1 year) limits were used on the basis of previous reports in order to define the clustering cell.

Secondary malignancies in a child with Hodgkin's disease: T-cell lymphoma and myelodysplastic syndrome evolving into acute nonlymphoblastic leukaemia.

Hodgkin's disease (HD) has been linked to an increase risk of second malignant neoplasms (SMN), especially non-Hodgkin's lymphoma (NHL) and acute nonlymphoblastic leukaemia (ANLL). The mutagenic property of cytotoxic therapy as well as defective immunity have been implicated as playing a major role in the development of SMN in patients previously treated for HD. We report a case of a 14-year-old girl with HD who developed two different second malignancies within a latent period of 28 months following HD diagnosis.

Survival from childhood leukemia depending on socioeconomic status in Athens.

One hundred and twenty children first diagnosed as having acute leukemia between 1988 and 1992 in Athens, Greece, were followed until May 15, 1993. The socioeconomic status of the children's families was assessed by means of paternal occupation, paternal schooling, maternal schooling, ownership of a car, ability to choose a private medical facility and freedom in the choice of the attending physician. The analysis was done by proportional-hazards modelling, controlling for age and gender.

Seventeen years of experience with chronic idiopathic thrombocytopenic purpura in childhood. Is therapy always better?

Between 1975 and 1992 450 children with idiopathic thrombocytopenic purpura (ITP) were diagnosed, and of those 100 (22%) developed the chronic form of the disease. Approximately half the patients with chronic ITP presented with mild to moderate hemorrhagic manifestations at the onset of purpura (30 cases) and/or later during the course of the disease (25 cases). The incidence of intracranial hemorrhage was 1%, and the mortality rate due to overwhelming septicemia after splenectomy was also 1%.

Trends and geographical distribution of childhood leukemia in Greece in relation to the Chernobyl accident.

Parts of Greece have been exposed to fallout radiation from the Chernobyl accident as much as any of the countries boardering with the former Soviet Union, because of the direction of the prevailing winds after the accident. Although fallout radiation did not reach levels expected to be associated with measurable effects, there is widespread concern in Greece that the incidence of childhood leukemia may be rising in the more heavily affected parts of Greece. Patient discharge data from all Greek hospitals treating childhood leukemia were used to calculate the annual incidence of the disease from January 1980 to June 1986 (preaccident period), from July 1986 to June 1988 (immediate postaccident period) and from July 1988 to June 1991 ("relevant" post-accident period, that accommodates the presumed latent period of the disease).

Auxologic data and hormonal profile in long-term survivors of childhood acute lymphoid leukemia.

Purpose: Eighty patients, 50 girls and 30 boys 13.5 +/- 4.09 years of age, on continuous first remission, were evaluated 7.

Diabetes mellitus and exocrine pancreatic insufficiency in a girl with auto-immune haemolysis.

A 7-year-old girl developed diabetes mellitus and exocrine pancreatic insufficiency after 3.5 years of almost continuous treatment with azathioprine and/or prednisone for idiopathic auto-immune haemolytic anaemia. Although both drugs have been reported to preserve endogenous insulin secretion and to interrupt the diabetogenic process they may be responsible for diabetes and exocrine pancreatic insufficiency in our patient.