Fish mortality by impingement on the cooling-water intake screens of Britain's largest direct-cooled power station.

An estimated 5.66 x 10(7) fish (summed quarterly 95% confidence intervals: 3.01 x 10(7)-1.07 x 10(8)) weighing 258.4 t (143.2-467.9 t) were killed on the cooling-water intake screens of the 2400 MW Longannet Power Station (Forth estuary) in January 1999--December 2000. Abundance and number of species (40) collected were close to predictions for a power station of this size and latitude. Potential losses of equivalent adult whiting (Merlangius merlangus), cod (Gadus morhua), and plaice (Pleuronectes platessa) through deaths of juveniles were estimated at 353.1 t (208.0-603.2 t) worth approximately euro 429,266 (euro 246,592-752,765) in 1999--2000. Fish catch-per-trawl in the estuary was generally not noticeably greater during a year of low water withdrawal (coal miners' strike of 1984--1985) when compared to other years from 1982 to 2001, except for gobies (Pomatoschistus spp.). A fish-return system is being tested at Longannet to reduce mortality.

Langerhans cell histiocytosis after therapy for a malignant germ cell tumor of the central nervous system.

Langerhans cell histiocytosis (LCH) is a clonal neoplastic disorder that results in a spectrum of clinical manifestations. Known to be associated with a variety of malignant diseases, LCH may precede, coincide with, or develop after the diagnosis of cancer. A child with a malignant germ cell tumor of the brain who subsequently experienced LCH is reported. The 8-year-old boy was treated for an immature teratoma of the posterior fossa with gross total resection and craniospinal irradiation preceding bleomycin, etoposide, and vinblastine chemotherapy for four cycles. Seven months after completion of therapy, he experienced multifocal bone disease with LCH.

Late echocardiographic findings following childhood chemotherapy with normal serial cardiac monitoring.

PURPOSE: Late development of myocardial dysfunction years following successful treatment of childhood malignancy with anthracyclines is well documented. There have been few studies of late cardiac performance in children in whom serial monitoring during treatment suggested normal cardiac performance, and those studies that do exist rely on the results of extensive evaluation. It was our purpose to determine whether findings consistent with known late cardiac changes could be discovered in these patients by echocardiographic monitoring similar to that routinely performed during treatment. PATIENTS AND METHODS: A total 28 consecutive asymptomatic patients who had completed anthracycline therapy at least 3 years previously, had been free of malignant disease since the completion of therapy, and who had had normal serial echocardiographic studies during and at completion of treatment were restudied by echocardiography. Of these 28, 12 had undergone mediastinal radiation as part of their acute treatment. RESULTS: Four patients (14%) of the study group were found to have abnormally low values for left ventricular shortening and ejection fractions. All four had also received mediastinal radiation. The remaining 24 patients, while having values for shortening fraction within the normal range, had, as a group, experienced a significant decrease in echocardiographic left ventricular shortening since completion of treatment. In these patients, left ventricular wall thickness had not increased commensurate with growth in body size and left ventricular cavity dimension. CONCLUSIONS: The known incidence of late asymptomatic cardiac dysfunction is confirmed despite the presence of persistently normal echocardiographic monitoring studies during and at completion of anthracycline treatment. Additionally, as a population, these patients show impaired myocardial growth over time, placing them at risk for future myocardial failure. Normal echocardiographic monitoring studies during antineoplastic treatment in children may not necessarily predict that patients will be free of the development of late cardiac dysfunction. Routine serial echocardiographic monitoring can, however, be helpful in the long-term management of these patients.

Gaucher's disease complicated by bleeding esophageal varices and colonic infiltration by Gaucher cells.

We report a 10-yr-old child with Gaucher's disease who developed upper gastrointestinal bleeding from esophageal varices, as well as hemorrhage from a colonic polyp infiltrated with Gaucher cells. Both the varices and polyp were treated endoscopically, and the outcome was successful. Although gastrointestinal hemorrhage due to portal hypertension is considered a rare complication of Gaucher's disease, colonic infiltration with Gaucher cells has not been recognized previously.

Prognostic significance of terminal transferase activity in childhood acute lymphoblastic leukemia: a prospective analysis of 164 patients.

Whether the level of terminal deoxynucleotidyl transferase (TdT) activity in mononuclear cells from bone marrow and peripheral blood has prognostic significance has been analyzed prospectively in 164 children with T and non-T, non-B marked acute lymphoblastic leukemia (ALL). TdT was measured at diagnosis to assess its value as a predictor of duration of remission and length of survival. It was measured repeatedly during remission to assess whether it could predict relapse. Ninety-seven percent of the children achieved a complete remission of their disease, and 40% relapsed during the study. The level of TdT activity in blasts at diagnosis varied 1000-fold from patient to patient. There was no statistically significant relationship between TdT activity in cells at diagnosis and the achievement of complete remission, the duration of remission, or length of survival. TdT activity was significantly increased in the bone marrow of 65% of patients at the time of marrow morphological relapse, but was rarely increased in marrow from patients with isolated testicular or central nervous system relapse. Wide fluctuations in TdT activity were characteristically seen in mononuclear cells from the marrow and peripheral blood of patients with ALL at all stages of their disease. An isolated high value of TdT activity in the bone marrow or peripheral blood cannot be taken as evidence of impending relapse. Quantitative measurements of TdT activity alone on mononuclear cells from bone marrow and peripheral blood are helpful in differential diagnosis, but cannot guide therapy of children with ALL.

Acquired coagulation inhibitor delaying fibrinopeptide release.

A 14-yr-old girl Down syndrome developed a unique type of circulating inhibitor causing a mild bleeding tendency and interfering strongly with coagulation tests, including reptilase time, and with the reaction of purified fibrinogen and thrombin. The concentration of all coagulation factors was normal. The inhibitor had no direct effect on thrombin activity or on the aggregation of fibrin monomer in plasma. Chromatography on DEAE-cellulose and neutralization by immune sera revealed that the inhibitor was an immunoglobulin of IgG class with both kappa and lambda determinants. Isolated inhibitor delayed the release of fibrinopeptide A from a normal fibrinogen reacting with thrombin and retarded the onset of visible clotting, but had no effect on the the final degree of clottability. The clinical and laboratory features of this patient resemble those of patients with congenital dysfibrinogenemia associated with abnormal fibrinopeptide release.

Adenosine deaminase, terminal deoxynucleotidyl transferase (TdT), and cell surface markers in childhood acute leukemia.

The purpose of this report is to compare measurements of enzymatic activities and cell surface markers as methods of distinguishing subtypes of lymphoid leukemias of childhood. Twenty-six children ages 2-15 yr were studied. Terminal deoxynucleotidyl transferase (TdT) activity was high in blasts from all 20 children with either null or T cell acute lymphoblastic leukemia. The activity of adenosine deaminase per cell was higher (P less than 0.005) and that of TdT lower (p less than 0.05) in T than in null cell lymphoblasts, although there was some overlap in values. Blasts from 3 children with acute lymphoid leukemia were positive for surface-associated immunoglobulins. The neoplastic lymphoid cells from these children differed from T and null cell leukemic lymphoblasts by having very low levels of TdT and adenosine deaminase activity. Measurements of adenosine deaminase and TdT may complement measurements of cell surface markers and distinguish biochemical subtypes of acute lymphoid leukemia.

Monocyte functional capacity in chronic neutropenia.

The bactericidal activity of monocytes from a child with chronic benign granulocytopenia who has had virtual absence of neutrophils yet minimal infections since birth was examined against Escherichia coli and Staphylococcus aureus and compared with that of monocyte and neutrophils from 20 control subjects. Studies on monocyte function in this patient with no neutrophils revealed normal monocyte kill of both organisms when compared with control monocytes. Monocyte and neutrophil killing of both organisms was similar in control subjects at bacteria to phagocyte ratios of 1:1. When ratios of 3:1 were employed, however, control neutrophils were more effective than control and patient monocytes in reducing the number of viable organisms. These findings support the neutrophil as the more effective blood phagocyte but stress the importance of monocyte functional capacity in patients compromised by granulocytopenia or neutrophil functional defects.

Terminal deoxynucleotidyltransferase distribution in neoplastic and hematopoietic cells.

In the present study, terminal deoxynucleotidyltransferase was examined in the peripheral blood and (or) bone marrow of 115 children with a variety of neoplastic, hematologic, and other unrelated disorders. Terminal deoxynucleotidyltransferase activity was present at 4.08+/-0.74 U/108 cells in 23 morphologicall normal bone marrow samples from childhood controls. Terminal transferase was present at greater than 23 U/108 nucleated cells and at greater than31 U/108 blasts in the bone marrow of all children with acute lymphoblastic leukemia studied at initial diagnosis and at disease relapse. Terminal deoxynucleotidyltransferase was detectable at low levels, less than 7.5 U/108 cells, in all remission marrow smaples. Bone marrow terminal transferase activity was markedly elevated in all untreated acute lymphoblastic leukemia patients, whereas low levels which were difficult to interpret were present in the peripheral blood samples of two patients at diagnosis and six patients at relapse who had low absolute lymphoblast counts. Because of greater variation in the lymphoblast content of peripheral blood, bone marrow assays are more reliable in detecting disease activity. Marrow terminal deoxynucleotidyltransferase values obtained during the active phase of acute lymphoblastic leukemia were significantly greater than those found in other types of leukemia, bone marrow malignancies, and hematologic disorders. Terminal transferase determinations in blast cells of two patients with leukemic conversion of non-Hodgkin's lymphoma and in tumor cells from one patient with Burkitt's lymphoma were within the control range. These dat further define the usefulness of terminal deoxynucleotidyltrnasferase assay in the differentiation and classication of hematologic malignancies.

Serial observations on terminal deoxynucleotidyl transferase activity and lymphoblast surface markers in acute lymphoblastic leukemia.

Terminal deoxynucleotidyl transferase activity and cell surface markers were measured in peripheral lymphoid cells from 27 children with acute lymphoblastic leukemia in various phases of their disease. Lymphoblasts from untreated patients had smooth surface ultrastructure but heterogeneous surface receptors. Greater than 60% of lymphoblasts from 4 to 7 untreated patients formed rosettes with sheep red blood cells. Transferase activity was variable, ranging from 8 to 210 units/10(8) blasts, but it was consistently elevated at diagnosis and in relapse. Transferase levels did not correlate with the presence of lymphoblast surface receptors. During induction therapy transferase activity decreased rapidly, but it remained elevated in peripheral lymphoid cells even when blasts were not detectable in peripheral blood smears. Patients in remission had normal surface receptors and undetectable or minimally elevated levels of transferase. Terminal transferase activity may be a sensitive biochemical marker for a primitive cell population and may be important in the evaluation of therapeutic effectiveness in acute lymphoblastic leukemia.

Scanning electron microscopic observations of the human respiratory tract.

As viewed by scanning electron microscopy, the luminal surface of the human trachea at 12 weeks' gestation shows a predominance of microvillous-covered, nonciliated cells, in contrast to the heavily ciliated tracheobronchial surface seen at 34 weeks' gestation. Hyaline membrane disease produces a confluent lining material in the lung periphery that obscures the bronchiolar and alveolar surface architecture. Large saucer-shaped alveoli, numerous alveolar pores, and an abundance of in situ alveolar macrophages are observed in chronic bronchitis and in emphysematous lungs; The scanning electron microscope offers an additional tool for the study of developmental and pathological processes in the human respiratory tract.