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Biomedical subjects

F Berthold

Publications and source records attributed to F Berthold.

At least 109 records · Page 6Linked to original sources
PubMed1987

Fatal aplastic anaemia in a child with features of Dubowitz syndrome.

We describe a boy with features of Dubowitz syndrome who developed anaemia, thrombocytopenia and granulocytopenia at 3 years of age. The family refused blood component transfusion and he died 6 months later from severe anaemia and pulmonary bleeding. This is the second case of bone marrow aplasia in 38 reported cases of Dubowitz syndrome. It is proposed that patients with Dubowitz syndrome need long-term follow up, including complete blood counts.

Anemia, Aplastic↗
PubMed1983

Effect of pentoxifylline on sickle cell thalassaemia: haemorheological and clinical results.

The effect of pentoxifylline on the deformability of red cells in sickle cell thalassaemia was investigated. The fluidity of the blood in sickle cell thalassaemia is disturbed and is accompanied by violent pains and irreversible tissue damage caused by capillary occlusions. After treating a 15-years-old female patient with pentoxifylline (2 g orally each day), the fluidity of the blood improved distinctly, and this correlated with a condition free of clinical symptoms. Erythrocyte filtration by Nuclepore filter increased significantly over the 6-month examination period (initial value: V rel = 0.068 +/- 0.008; after 6 months medication: 0.246 +/- 0.030). In addition, in the single-pore erythrocyte rigidometer (SER) a significantly improved passage time of individual erythrocytes could be demonstrated (initial value: 62.43 +/- 15.72 ms; after 4 weeks medication: 28.13 +/- 7.0 ms). The hitherto high number of rheological occlusions in the SER (48 +/- 30.9 from 200 individual passages) almost completely disappeared after treatment (1.7 +/- 1.2).

Adolescent↗
PubMed1983

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↗
PubMed1982

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↗
PubMed1982

Effect of spleen exposure to ultrasound on cellular and antibody-mediated immune reactions in man.

Spleen exposure to ultrasound has been reported to influence antibody response to sheep red blood cell injection in mice (decreased hemagglutination and hemolytic titers and IgM, IgG2a and IgG2b levels and elevated IgG1 levels). In a controlled clinical trial, we investigated the possible immunosuppressive side-effect of splenic exposure (2.0 mW/m2, 3.5 MHz, 5 minutes) to ultrasound on the immune response to Rubella vaccination in 41 anti-Rubella antibody-negative volunteers. The measured parameters (blood cell count, IgA, IgM, IgG including subclasses IgG1-IgG4, isoagglutinins, anti-Rubella hemagglutinin and hemolysin titers, complement C3, skin tests to mumps and tuberculin, T, B and O lymphocytes, esterase-positive and negative T-cell subsets) suggest changes dependent on the time of vaccination, but provide no evidence of an immunosuppressive effect of ultrasound in man.

Adolescent↗
PubMed1981

[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↗
PubMed1981

[Neuroblastoma study NBL79-society of pediatric oncology -- report after 1 year (author's transl)].

Within 1 year 74 children with neuroblastoma were registered, 30 patients with stage I-III (= 41%) and 44 with stage IV-metastatic disease (= 59%). An aggressive chemotherapy regimen employing Adriamycine, Cyclophosphamide, Vincristine, and Dacarbazine yielded 10/24 partial and 9/24 complete remissions after 9 weeks. 5/24 children were treated less than 9 weeks so far. At the end of the chemotherapy protocol (week 33) 6 recurrences were observed; 3 of these children died. 5 patients remained in complete remission, 1 in partial remission. 12/24 of patients were not evaluable because of treatment less than 33 weeks so far. The one year run of the study is too short to evaluate the benefit of Interferon (randomized trial). The toxicity of the regimen is tolerable, including bone marrow depression, vomiting and hyperpyrexia. Breaking off therapy was only necessary in one patient.

Adolescent↗
PubMed1981

[Dedifferentiating intracerebral neuroblastoma resistant to x-ray and drug therapy -- a case report (author's transl)].

A new case of primary intracerebral neuroblastoma is reported. The patient, a 2 year old boy, was subjected to temporary successful craniotomy, postoperative irradiation and cytostatic therapy without any influence on the recurrence of the tumor. In contrast to the possible well-known maturation of neuroblastomas a dedifferentiating process occurred. The boy died 8 1/2 months after diagnosis.

Brain Neoplasms↗
PubMed1981

[Normal and abnormal cell and antibody mediated immunoreactions. Part II: The monocyte-macrophage system as a central part of cell interactions and changes in cell compartments for clinical diagnostics (author's transl)].

The immune response is a result not of one but many cellular activities. Macrophages proved to be the central part of cooperation as elucidated by in vitro lymphocyte-macrophage clusters and by the three dimensional branching lattice-work of reticular cells in vivo. Presenting antigen, inducing helper cells, producing antibodies, and activating suppressor cells is picked up briefly. Summarizing table on changing immune cell quantities in immunoproliferative and immunodeficiency syndromes is given.

Animals↗
PubMed1980

[Normal and abnormal cell and antibody mediated immunoreactions. Part I: structure, ontogeny and functions of lymphocytic system].

The ontogeny of T and B cells can be clearly demonstrated by developing cell surface markers and cell functions. This may be used as diagnostic aid in determining differentiation of lymphoblasts, therapeutic response of immunodeficiency disorders and as one of explanations for immunoincompetence of newborns. Subpopulations of lymphocytes can be quantitatively and qualitatively changed in a variety of disorders. The main markers of lymphocytic subpopulations and their in vitro and possible in vivo functions are described.

Antigen-Antibody Reactions↗
PubMedDate not supplied

Application of a new high sensitivity luminometer for industrial microbiology and molecular biology.

A compact new luminometer (FB12) has been developed based on a 370-630 nm photon counter and measuring chamber that can accommodate a range of sample formats. The FB12 permits measurements as low as 1000 molecules of luciferase in reporter gene assays. Its sensitivity for ATP is limited by reagent background. If ATP assay reagents had no chemical background, 2 fg of ATP could be detected using 3 SD of instrument background as the detection limit. The FB12 has a dynamic range of six decades and operates under its own microprocessor programme or protocol-based PC software that is integrated with Microsoft(R) Excel(R). An injector port above the sample measuring position allows connection of external reagent injectors. Applications are performed using protocols provided with the FB12 or user defined protocols. Examples are presented that illustrate use of the instrument for research and industrial applications.

Industry↗
PubMedDate not supplied

Optimization of phagocyte chemiluminescence measurements using microplates and vials.

In order to cope with large amounts of samples for chemiluminescence (CL), vials were replaced with microplates. Although various types of plates have been commercially available for quite some time and the free-plate mode is advocated by the producer of the counter, little is known about their impact on the outcome of CL measurements. We tested two different 24-well microplates and six different 96-well microplates in two different luminometers, and results were compared with those achieved with vials. Before these comparative tests, we attempted to optimize measurement conditions. CL sensitivity was highest with luminol concentrations of 0.8-3.3 micromol/L, PMA concentrations of 0.06-80 micromol/L, a pH value of 10 and a temperature of 20 degrees C. An indirect correlation was found between fluid volume and yield in counts: the lower the volume, the higher the counts. With regard to sensitivity and cross-talk, the 96-well Isoplatetrade mark was superior to all other plates tested. While all white plates tested gave acceptable results, usage of the black 96-well plates resulted in an extremely low sensitivity. Plates designed for cell culturing gave even lower counts and a cross-talk of up to 31%. All attempts to reduce cross-talk and improve sensitivity, such as aluminium foil or grids, irrespective of the position of the photomultiplier, did not give results comparable to the original 96-well isoplate. Our results suggest that, with the exception of black 96-well microplates and cell culture plates, all other plates tested have a sufficient sensitivity when compared to vials and acceptable cross-talk, the 96-well Isoplatetrade mark being the best. Both types of luminometers used gave reproducible results, Wallac having a somewhat higher sensitivity, Canberra Packard somewhat less cross-talk.

Humans↗
PubMedDate not supplied

[Improved treatment results in children with AML: Results of study AML-BFM 93].

BACKGROUND: In the multicenter trial AML-BFM 93 daunorubicin or idarubicin was randomly applied in all patients during induction in combination with cytarabine and etoposide. After induction all patients were stratified to the standard or high risk group. To improve outcome in high risk patients high dose cytarabine and mitoxantrone (HAM) was introduced. The placing of HAM as either the 2nd or 3rd therapy block was randomized to evaluate the efficacy and toxicity accordingly. PATIENTS AND METHODS: 471 children with de novo AML entered the trial AML-BFM 93 (161 standard risk, 310 high risk). RESULTS: Overall, 387 of 471 (82 %) patients achieved remission, 5-year survival, event free survival (EFS), and disease free survival were 60 % SE 3 %, 51 % SE 2 % and 62 % SE 3 %, respectively. Idarubicin-based induction resulted in a significantly better blast cell reduction in the bone marrow on day 15 (25 of 144=17 % patients with > 5 % blasts compared to 46 of 149=31 % patients after daunorubicin, pchi(2)=0.01). This was, however, mainly seen in high risk patients treated with idarubicin (19 % vs. 38 %, pchi(2)=0.007). Cardiotoxicity, WHO grade 1 - 3 shortening fraction reduction after induction occurred in 6 % patients in both arms. In the total group of patients probabilities of five years event-free survival and disease-free survival were similar for patients treated with daunorubicin or idarubicin. However, in patients presenting with more than 5 % blasts on day 15 there was a trend for a better outcome after treatment with idarubicin (p logrank 0.06). Outcome in high risk patients was superior in study 93 compared to study 87 (remission rate and 5-year pEFS in study AML-BFM 93 vs. study 87: 78 % vs. 68 %, p=0.007, and 44 % vs. 31 %, p logrank=0.01). The placing of HAM as the 2nd or 3rd therapy block was of minor importance. However, patients who received the daunorubicin treatment during induction benefited from early HAM. CONCLUSION: Compared to study AML-BFM 87 treatment results in study AML 93 improved significantly in high risk patients. This can partly be contributed to the better response on day 15 after idarubicin induction but is mainly due to the introduction of HAM.

Acute Disease↗
PubMedDate not supplied

Traditional and emerging molecular markers in neuroblastoma prognosis: the good, the bad and the ugly.

BACKGROUND: Neuroblastomas (NB) are a heterogeneous group of childhood tumours with a wide range of likelihood for tumour progression. As traditional parameters do not ensure completely accurate prognostic grouping, new molecular markers are needed for assessing the individual patient's prognosis more precisely. PATIENTS AND METHODS: 133 NB of all stages were analysed in blind-trial fashion for telomerase activity (TA), expression of surviving, and MYCN status. These data were correlated with other traditional prognostic indicators and disease outcome. RESULTS AND CONCLUSIONS: TA is a powerful independent prognostic marker for all stages and is capable of differentiating between good and poor outcome in putative "favourable" clinical or biological subgroups of NB patients. High surviving expression is associated with an adverse outcome, but is more difficult to interprete than TA because survivin expression needs to be accurately quantified to be of predictive value. We propose an extended progression model for NB including emerging prognostic markers, with emphasis on telomerase activity.

Biomarkers, Tumor↗