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Biomedical subjects

J Wilimas

Publications and source records attributed to J Wilimas.

At least 37 records · Page 2Linked to original sources
PubMed1988

Teratoid Wilms' tumor: the St Jude experience.

The term "teratoid" Wilms' tumor has been used recently to describe an unusual type of tumor in which, although classic nephroblastoma tissue is present, there is a significant diversity of cell types and tissues present. Examination of tissue samples from 290 patients treated at St Jude Children's Research Hospital from 1964 to 1987 disclosed that three children had teratoid Wilms' tumor. All three children had renal tumors and two of them presented with bilateral pyeloureteral obstruction, uremia, and hypertension. When compared with classic nephroblastoma, two of the children with teratoid elements responded poorly to chemotherapy and irradiation, although no metastatic disease was identified. One child died with sepsis and renal failure; the other two are surviving disease-free for 7 1/2 years and 26+ months since diagnosis. Because of the tendency for bilateral involvement, ureteral obstruction, and uremia, and their relative resistance to chemotherapy and irradiation, surgery is the principal form of therapy for patients with these tumors.

Child, Preschool↗
PubMed1986

Central nervous system toxicity following the treatment of pediatric patients with ifosfamide/mesna.

Ifosfamide/mesna treatment of 50 patients with pediatric malignant solid tumors was associated with the development of neurotoxic signs and symptoms in 11 of these individuals who received 29 courses of treatment. Neurologic toxicity included changes in mental status, cerebellar function, cranial nerve, and cerebellar and motor system function, including seizures. All symptoms, signs, and EEG abnormalities were transient. Some of the affected individuals failed to develop acute neurotoxic signs of symptoms when retreated with ifosfamide. A grading system for scoring these neurologic abnormalities is presented for comparison of acute neurotoxic effects of other agents. Recommendations are made regarding early termination or delay of ifosfamide/mesna treatments in the presence of significant neurotoxicity.

Adolescent↗
PubMed1985

Lymphocyte phenotype and function in chronically transfused children with sickle cell disease.

Immunologic studies were performed on mononuclear cells from ten chronically transfused children with sickle cell disease, and the results were compared with those from five other groups: 21 sickle cell patients who were not receiving regular transfusions, 6 chronically transfused children with other forms of refractory anemia, 10 previously splenectomized children, 5 boys with hemophilia A, and 27 normal adult controls. The helper:suppressor T cell ratios (T4:T8) in all groups except hemophiliacs were normal, were unrelated to the number of units transfused, and were not suggestive of findings reported for patients with the acquired immune deficiency syndrome (AIDS) or groups at risk for the syndrome. Percentages of T3, T4, and T8 cells were low in sickle cell and splenectomized patients, but not in chronically transfused patients with other anemias. Serum IgG was frequently elevated, and IgG synthesis in vitro was increased relative to IgM synthesis in sickle cell patients. Coculture experiments indicated that such findings may stem from a selective increase in IgG synthesis by B cells. Thus, transfused sickle cell patients have a particular pattern of immunologic abnormalities that is distinct from that seen in AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Developmental pattern of splenic dysfunction in sickle cell disorders.

Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs greater than or equal to 3.5%. Cross-sectional analysis of pocked RBC data from 2,086 patients showed differences in the developmental pattern of splenic dysfunction between several disorders. In hemoglobin SS disease (sickle cell anemia) and hemoglobin S beta(0) thalassemia, splenic dysfunction (greater than or equal to 3.5% pocked RBCs) often occurred in the first 6 to 12 months of life. In hemoglobin S beta(+) thalassemia, splenic dysfunction occurred less frequently and later. Splenic dysfunction in hemoglobin SC disease (sickle cell-hemoglobin C) was intermediate. The level of pocked RBCs was inversely associated with fetal hemoglobin (P less than .007) and directly associated with age (P less than or equal to .001). These patterns of splenic dysfunction reflect the known severity of hemolysis and intravascular sickling and are consistent with the epidemiology of severe bacterial meningitis and sepsis in these diseases. Serial measurement of pocked RBCs permits determination of the onset of splenic dysfunction and the time of increased susceptibility to severe bacterial infections.

Age Factors↗
PubMed1984

Distribution and properties of type I and type II binding proteins in the cyclic adenosine 3':5'-monophosphate-dependent protein kinase system in Wilms' tumor.

We compared the relative amounts and properties of cyclic adenosine 3':5'-monophosphate (cAMP)-binding proteins in surgical specimens of Wilms' tumor and normal kidney. Cytosolic fractions of both tissues contained type I and type II isozymes of cAMP-dependent protein kinase (adenosine triphosphate: protein phosphotransferase, EC 2.7.1.37). Among tumor samples, the mean ratio of type I to type II cAMP-binding activity was 2.76 +/- 0.52 (S.D.) contrasted with 1.36 +/- 0.23 for normal kidney (p less than 0.001). The total soluble cAMP-binding activities in normal and malignant tissues differed only slightly. Photoaffinity labeling of cytosol from either tissue, using cyclic adenosine 3':5'-[8-azido-32P]monophosphate, disclosed three cAMP-binding proteins (Mr 47,000, 51,000, and 55,000) that were identified as regulatory subunits of the holoenzyme. Three lower-molecular-weight proteins with unknown function were considered to be proteolytic products of the larger proteins. The Mr 47,000 protein, a monomeric regulatory subunit of type I kinase, was clearly the dominant protein in tumor specimens, but it was much less abundant in normal kidney. The temperature sensitivities of the cAMP-binding proteins and their dissociation constants for cyclic adenosine 3':5'-[8-azido-32P]monophosphate incorporation did not differ appreciably between tumor and normal tissues. Wilms' tumor appears to have a full complement of regulatory subunits of cAMP-dependent protein kinase that are capable of normal cellular function.

Carcinoma, Hepatocellular↗
PubMed1984

Cyclic ultrastructural abnormalities in human cyclic neutropenia.

The ultrastructure of neutrophils from two brothers with cyclic neutropenia was studied at various time intervals of the neutrophil cycle, which varied from 15 to 21 days. During the nonneutropenic portion (middle third) of the cycle, when the blood neutrophil counts approached normal, the majority of blood and marrow segmented neutrophils appeared morphologically normal, whereas marrow promyelocytes demonstrated increased autophagy with decreased condensation of primary granules. During the early neutropenic portion of the cycle (first third), increased autophagy and granule abnormalities persisted in promyelocytes, myelocytes, and the few segmented neutrophils present. During the late neutropenic portion (last third) of the cycle, which preceded neutrophil recovery, most promyelocytes appeared morphologically normal, whereas the few segmented neutrophils present frequently contained increased autophagic and/or heterophagic vacuoles and decreased cytoplasmic granules. Abundant neutrophil debris was present in marrow macrophages consistent with dysgranulopoiesis and intramedullary destruction of abnormal myeloid cells. Similar changes were not observed in monocytes, eosinophils, or basophils. These results demonstrate a cyclic insult which has its initial morphologic impact on the promyelocyte of patients with cyclic neutropenia.

Adolescent↗
PubMed1983

Hydrocele formation in patients with Hodgkin's disease.

Hydroceles developed in 12 (7.7%) of 155 consecutive male patients who had Hodgkin's disease treated between 1962 and 1981. Except for one patient who had a hydrocele at diagnosis, all developed this complication during remission, 8 months to 7.5 years after inverted-Y or abdominopelvic 60Co irradiation (3000-3800 rad). Hydroceles were bilateral in five patients and on the right side in seven. No tumor was found in the hydrocele sacs of the ten patients who underwent hydrocelectomy. Lymphatic obstruction was evidenced by lymphatic dilation in the hernia sac in six patients. The authors believe postirradiation fibrosis of the lymphatic pathway was the main cause of hydrocele formation.

Adolescent↗
PubMed1983

Immunoregulatory abnormalities in Evans syndrome.

Immune function in six patients with Evans syndrome (Coombs-positive hemolytic anemia and immune thrombocytopenia) was compared to that in seven with chronic ITP. The two groups differed in measurements of T-cell subsets and immunoglobulin production. Evans syndrome patients had decreased T4 (T-helper) (P = 0.025), increased T8 (T-suppressor) (P = 0.008), and a decreased ratio of T4:T8 cells (P = 0.0009) when compared to controls. Results in chronic ITP patients were similar to those in controls. Serum IgG, IgM, and IgA levels and in vitro synthesis of IgG and/or IgM were decreased in most Evans syndrome patients. Diminished in vivo and in vitro immunoglobulin synthesis in Evans syndrome is consistent with the decreased T4:T8 ratio in these patients. The altered T4:T8 ratio may represent an unsuccessful response to an autoimmune process in which the trigger is unknown.

Adolescent↗
PubMed1982

Hereditary elliptocytosis: morphologic abnormalities during acute hepatitis.

A 10-year-old black girl with hereditary elliptocytosis had a transient increase in hemolysis and unusual red cell morphologic changes during an episode of acute hepatitis. Changes in membrane lipids of these elliptocytes with defective membrane skeletal protein and abnormal distribution of cholesterol may be responsible for this phenomenon.

Acute Disease↗
PubMed1982

Clonogenic assay for Wilms' tumor: improved technique for obtaining single-cell suspensions and evidence for tumor cell specificity.

Wilms' tumors from seven patients were dissociated by mechanical and enzymatic means; this technique resulted in single-cell suspensions for five specimens and a few aggregates for two. By dye exclusion, cell viability ranged from 56 to 100% (median, 92%). All seven preparations produced more than five colonies/2 x 10(5) cells plated. Forty-three colonies grown from cells of a glucose-6-phosphate dehydrogenase heterozygote were of the same glucose-6-phosphate dehydrogenase isoenzyme type as the original tumor, indicating that the assay is specific for tumor cells. We attribute the high rate of colony formation to an improved method of cell preparation (combined mechanical and enzymatic dissociation of tumors) which may be applicable to other primary human tumors assayed in the soft agar system.

Cell Separation↗
PubMed1981

Surgical management of priapism in children with sickle cell anemia.

Priapism has been reported to occur in 2 to 5 per cent of patients with sickle cell disease. Recently, creation of a percutaneous fistula between the glans penis and the corpora cavernosa has been applied widely in the management of patients with priapism, except in those cases of sickle cell anemia. Reported failures of such shunting procedures in the sickle cell patient has perhaps unduly denied surgical benefits to that population. We have managed successfully 5 such children with sickle cell anemia and priapism without complications by creation of a fistula between the glans penis and the corpora cavernosa. Our treatment plan is presented, which includes early shunting should initial nonoperative treatment fail.

Anemia, Sickle Cell↗
PubMed1980

Long-term results of treatment of children and adolescents with Hodgkin's disease.

Fifty-four children and adolescents with Hodgkin's disease Stages I--IV were treated with chemotherapy plus radiotherapy from 1967 to 1972. Thirty-eight patients (70%) remain in continuous complete remission. Nine patients have died, four of progressive disease, three of pneumonitis, one with probable pneumococcal sepsis, and one of acute myelocytic leukemia. Significant retardation of height and crown-rump length occurred, particularly in boys who received at least mantle or abdominal radiotherapy when younger than age 16 years. Five women have amenorrhea and no patient has clinical evidence of hypothyroidism. The major long-term effects of therapy in this group of patients has been growth retardation. Future studies to minimize long-term effects of therapy are necessary but must be carefully designed so that present cure rates are not jeopardized.

Adolescent↗
PubMed1980

Evans syndrome in childhood.

The combination of idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia is rare in childhood. Among 164 instances of ITP and 15 instances of AHA, 11 patients were found to have this combination. Three were found to have systemic lupus erythematosus, one had aplastic anemia, and seven had Evans syndrome. Neutropenia, at times associated with bacterial infections, occurred in four of the latter patients. Unlike most cases of ITP or AHA in childhood, the clinical course of Evans syndrome is usually chronic and relapsing. Treatment including corticosteroids, splenectomy, and immunosuppressive agents has been generally unsatisfactory. In view of the frequent presence of antibodies directed at red blood cells, platelets, neutrophils, and lymphocytes, immunopancytopenia may be a better term for this condition.

Adrenal Cortex Hormones↗
PubMed1980

Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents.

Since 1974 we have entered 12 children with sickle cell disease and strokes on a transfusion protocol to maintain hemoglobin S less than 20%. Serial arteriography, EEGs, brain or CT scans, and neuropsychologic testing were also obtained. Transfusion has been stopped in ten patients after one to two years. Seven of these ten patients have had second strokes five weeks to 11 months after cessation of transfusion (median three months). Arteriography was normal at the time of the initial stroke in two patients; one of these had a second stroke. Arteriograms did not improve during transfusion therapy. EEGs and brain and CT scans were occasionally useful at the time of the initial stroke but were of little value in following these patients. Neuropsychologic testing indicated severe impairment of sensory-motor and cognitive processes at the time of the initial stroke and was useful in following improvement or deterioration and in designing remedial education programs. We conclude that short-term transfusion therapy will not prevent second strokes once transfusion is stopped and that arteriography is of limited value in these patients.

Anemia, Sickle Cell↗
PubMed1979

Hypertrophy and hyperplasia of islets of Langerhans associated with androgen therapy.

Hypertrophy and hyperplasia of the islets of Langerhans were observed in three patients treated with androgen-anabolic steroids for aplastic anemia. These patients formed a unique subset of patients with aplastic anemia in that they all had Fanconi's anemia, all demonstrated glucose intolerance following institution of androgen therapy, all had received androgen for at least 42 months prior to death, and all had coexistent benign liver cell tumors. Androgen-induced glucose intolerance may play a major role in the pathogenesis of both the pancreatic and hepatic abnormalities.

Androgens↗
PubMed1978

Value of serum copper levels and erythrocyte sedimentation rates as indicators of disease activity in children with Hodgkin's disease.

Initial and serial serum copper levels (SCL) and erythrocyte sedimentation rates (ESR) of 29 children with Hodgkin's disease were reviewed to determine the relationship of these features to disease activity. Only six of 10 patients who relapsed had SCL greater than adult upper normal levels. Although correction for age increased this to 7, it also increased the number of patients with false positive results to 9 of 19. Although 9 of the 10 patients at relapse had an increase in SCL over the preceding value, almost one-fifth of patients in remission had increases in consecutive SCL greater than the average increase of patients who relapsed. We conclude that SCL, even when age corrected and consecutively followed, are not useful as indicators of disease activity in children with Hodgkin's disease. Although the ESR increased to greater than 20 mm/hour in 9 of 10 patients who relapsed, this determination could not be considered a useful early indicator of disease recurrence since it was so frequently elevated in patients who were free of disease (62 of 109 determinations). The extremely nonspecific nature of SCL and ESR in childhood renders these tests unreliable, particularly in the individual child.

Adolescent↗