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Biomedical subjects

F Lampert

Publications and source records attributed to F Lampert.

At least 145 records · Page 8Linked to original sources

Metastatic neuroblastoma in an infant: translocation (1;11), deletion (2) and double minute chromosomes.

An extensive chromosomal analysis was performed in cells of primary tumour and bone marrow metastases from an 8-month-old girl with disseminated neuroblastoma prior to therapy. A modal chromosome number of 46 was found. Consistent abnormalities in the karyotype were seen: in particular, a translocation (1;11), a deletion (2)(p21) and a chromosome 7p+. Furthermore, double minute chromosomes of two distinct sizes were found in 50%-80% of metaphases in the cell populations examined. These extra chromosomal double minutes might be the cytogenetic form of amplified cellular oncogene-DNA.

Adrenal Gland Neoplasms↗

Fatal outcome of acute thrombocytopenic purpura associated with a viral infection in an 11-year-old child.

A case of fatal intracranial hemorrhage is reported in an eleven year old girl with acute idiopathic thrombocytopenic purpura following a viral infection. The patient was randomized to the IgG-arm of the ITP therapy study. Immunoglobulin administration was not followed by a raise of the thrombocyte count. Neither the IgG therapy nor intensive therapeutic measurements were able to prevent the fatal course of cerebral hemorrhage in this case. Pathological and immunological findings indicate that our patient suffered from a fulminant ITP which must be considered as a part of a still active viral disease.

Blood Transfusion↗

Red cell membrane abnormalities in two cases with a special type of a hereditary megaloblastoid hemolytic anemia.

Case reports are presented of two related patients suffering from a hereditary megaloblastoid hemolytic anemia which at the moment cannot be categorized into one of the well-known entities. The main characteristics of the disease consisted of constant jaundice, macrocytic normochromic anemia, marked hemolysis without a substantial decrease in osmotic resistance, increased iron turnover and hepatic hemosiderosis at a relatively young age. One patient had to undergo splenectomy due to hemolytic crises, the other one cholecystectomy due to gallstones. In contrast to their uncharacteristic morphology in smear, red cells displayed highly variable forms ("lumpy", "Y", "U", drumstick forms) when examined in transmission and scanning electron microscopes. These changes corresponded well with reduced filtrability and aggregability of erythrocytes. The apparent relative blood viscosity was unchanged. The protein pattern of ghosts in SDS gel-electrophoresis revealed neither defects nor additional bands. Changes in the lipid composition of the membrane were indirectly deduced from electron spin-resonance studies, which showed an additional signal at g = 2.192. Similarly, the lipid related membrane mobility agent A2C failed to exert the usual stabilizing effect against osmotic stress. The negative surface potential, estimated by free flow electrophoresis, was only altered in the splenectomized patient. It is concluded that the primary abnormal physical properties of the enlarged red cell contribute at least in part to the marked hemolysis. The similar findings in the two related patients and the fact that the disorder was obviously congenital suggest a special subtype of a megaloblastoid hemolytic anemia.

Adolescent↗

[Concordant monocytic leukemia in twin infants].

Acute monocytic leukemia was diagnosed almost simultaneously in 6-month-old male identical twins. Inspite of chemotherapy one twin died of disseminated intravascular coagulation with pulmonary haemorrhage; the other one, however, went into long-term remission. Conception of the twins had taken place inspite of intrauterine device (copper T).

Age Factors↗

Ultrastructural, biochemical, and cell-culture studies of a presumed extraskeletal Ewing's sarcoma with special reference to differential diagnosis from neuroblastoma.

The history of a 6-year-old girl with a tumor originating from thoracic spine and finally becoming resistant to surgery, radio-, and chemotherapy is reported. Tumor-biopsy material was studied by light and electron microscopy, in cell culture, by acetylcholinesterase ultracytochemistry, and by quantitative catecholamine analysis and this led to the rejection of the initial diagnosis of a neuroblastoma. Light microscopy revealed a uniform population of undifferentiated cells incompletely lobulated by broad fibrovascular septa. Using the electron microscope, cells were characterized by large intracellular pools of glycogen, little cytoplasm with an abundance of free ribosomes and a paucity of organelles. A few cells displayed desmosome-like attachment sites. Staining for specific and unspecific acetylcholinesterase was negative with light and electron microscopy, as were the results of catecholamine histofluorescence using the glyoxylic acid method. The latter result was confirmed by the negative outcome of quantitative analyses of dopamine, noradrenaline, and adrenaline with high pressure liquid chromatography nd electrochemical detection in tissue samples. Tumor cells could easily be maintained in culture for up to 4 weeks. None of a variety of treatments that are known to favor expression of neuronal characteristics in neuroblastoma cells (serum withdrawal, nerve growth factor, dbcAMP, dexamethasone) induced morphological differentiation in cultured tumor cells. On the basis of the clinical history, morphology, and of our experiments with tumor cells, the diagnosis of a so-called extraskeletal Ewing's sarcoma is most likely. Our results strengthen the view that a cell biology approach may be valuable in neuroblastoma differential diagnosis.

Acetylcholinesterase↗

[Improved prognosis in Wilms' tumor due to adjuvant combination drug therapy].

78 children with Wilms' tumor stage I--IV diagnosed since 1960 are presented. There were two groups of patients: one group consisting of 35 patients which received adjuvant chemotherapy for 1 year, the other group consisting of 43 patients which received no chemotherapy. Surgical excision and irradiation was identical in both groups. The prognosis was greatly improved by adjuvant chemotherapy: 4-year survival rates increased from 25 to 69%. Late side effects from radiation and chemotherapy were noted: 15/78 patients suffered from scoliosis and all patients treated by chemotherapy had a decreased lymphotoxin activity over years. The following factors appear to be related to prognosis: the extent of disease in patients determined by better techniques, histopathology of the tumor, and the nature of treatment.

Age Factors↗

[Chromosomal studies in metastatic neuroblastoma].

Chromosomes were prepared directly from primary tumor and bone marrow aspirates of an 8 months old infant and a 2 years old boy with metastatic neuroblastoma. A total of 765 tumor metaphases were counted after Giemsa-banding and partially karyotyped. "Double minute" chromosomes were found in the metaphases of both primary tumor and metastases of the infant, but only in the primary tumor of the older child. In both patients, structural imbalances (in addition to other translocations) were seen in the short arm of chromosome no. 1. The modal chromosome number was 46 in the infant's primary tumor and bone marrow metastases, but 84 in the primary tumor and 46 and over 100 in the bone marrow metastasis of the older child. Clonal evolution of metastasizing neuroblastoma cells is discussed.

Adrenal Gland Neoplasms↗

[Leucocyte number, differential count and sedimentation rate in 9 "classic" childhood infections. (author's transl)].

A retrospective analysis of absolute numbers in 802 white blood counts and 396 sedimentation rates of 407 children, admitted between 1973-78, with 9 "classic" infections was done and evaluated for diagnostic usefulness. As diagnostic meaningful it was found: Lymphocytosis in pertussis; lymphocytopenia and slight increased sedimentation rate in measles; nothing particular in mumps; slight increased sedimentation rate in chicken pox; increase in mononuclear cells, particularly atypical lymphocytes and sedimentation rate in infectious mononucleosis; leucocytopenia caused by neutrocytopenia and lymphocytopenia in exanthema subitum (roseola infantum); increased sedimentation rate in scarlet fever; lymphocytopenia and a high sedimentation rate in mycoplasma-pneumonia; leucocytopenia with lymphocytopenia in rubella.

Adolescent↗

[Hypomagnesemic coma during therapy of septicaemia in a patient with acute lymphoblastic leukemia. (author's transl)].

During induction therapy of acute lymphoblastic leukemia a 10 year old boy developed a hyperuremic nephropathy and subsequently a staphylococcal septicemia at the beginning of the 3. week. Specific treatment was started leading to severe hypomagnesemia and generalized seizures with coma for 30 hours, which finally responded to magnesium replacement. The possible additive effect of nephropathy, gentamicin, and furosemide due to urinary loss of magnesium is discussed and should encourage further observations.

Child↗