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Biomedical subjects

M Rister

Publications and source records attributed to M Rister.

65 records · Page 4Linked to original sources

[Intracranial germ cell tumors: analysis of the therapy study MAKEI 83/86 and changes in protocol for the follow-up study].

As part of the Cooperative Germ Cell Tumors Studies MAKEI 83/86 of the German Society of Pediatric Oncology, 37 patients with intracranial germ cell tumors were registered. Based on histological criteria and tumor markers, 26 were classified as germinomas, 9 as fully malignant non-germinomatous germ cell tumors (2 yolk sac tumors, 1 embryonal carcinoma, 1 choriocarcinoma, 5 mixed type germ cell tumors), and 2 were mature teratomas. Of 26 patients with germinomas, 14 received radiotherapy only, all patients are surviving disease-free, median period of observation: 2 years, 10 patients were treated with both chemotherapy and radiotherapy, 8 of these patients are surviving disease-free. Of the 2 patients who received chemotherapy only, none is surviving. Of 9 patients with fully malignant non-germinomatous germ cell tumors, 4 are surviving following surgery, cisplatinum-based chemotherapy and radiotherapy more than 2 years following diagnosis, 1 of these 4 patients with stable disease. Of 2 patients with mature teratomas, 1 is surviving disease-free. Based on these data, recommendations for diagnostic evaluation and therapy of intracranial germ cell tumors are outlined.

Antineoplastic Combined Chemotherapy Protocols↗

[Myelosarcoma as the primary manifestation of acute myeloid leukemia].

Myelosarcoma ("Granulocytic sarcoma", "Chloroma") is an extramedullary tumor composed of granulocytic precursor cells and related to myelogenous leukemia. If the tumor precedes acute leukemia diagnosis is difficult and requires special diagnostic techniques. This is documented by the presented 7.5 years old girl with primary myelosarcoma. In spite of early and intensive chemotherapy and radiation the sarcoma soon was followed by acute myelogenous leukemia with skin infiltrations. Cytogenetic classifications may in future lead to the development of a differentiated therapy of myelosarcoma.

Antineoplastic Combined Chemotherapy Protocols↗

[Use of the Broviac/Hickman catheter in pediatric oncology].

This retrospective study reports data from 28 children with malignancy aged from 4 months to 15 years to whom 32 Broviac/Hickman indwelling central venous catheters were inserted. Catheter placement ranged from 36 to 381 days with a median of 177 days; thus a cumulative period of more than 12 patients' years could be analyzed. The patients were not continuously hospitalized but spent a median of 44% of their time as catheter-patients at home. The maintenance of the catheter was performed by the parents in an uniformed regimen daily. We registered a total of 22 manageable complications - corresponding to one complication per 202 implantation days. No patient suffered sequelae from the Broviac/Hickman-catheter. Occlusion (12 times) was the most common mechanical complication but patency could be resolved in all cases by installing streptokinase. Four dislocations demanded reimplantation of the catheter. One leak of the external segment was repaired using the commercial repair kit. There were 62 febrile episodes in 22 of 28 patients with simultaneously profound neutropenia in 45% of the febrile episodes. Blood cultures were positive in 11 patients and in 5 of these a catheter-related bacteremia persisted during antibiotic treatment thus requiring catheter explantation. At the end of therapy 40% of the catheters could be removed by manual pull, the rest required surgical explantation. These results demonstrate that with strict maintenance the implantation of a Broviac-Hickman-catheter is associated with an acceptable complication rate even in immunocompromized patients.

Antineoplastic Agents↗

[Myeloablative chemo- and radiotherapy with autologous and allogenic bone marrow reconstitution in children with metastatic neuroblastoma].

22 children with metastatic neuroblastoma received myeloablative chemoradiotherapy followed by bone marrow transplantation (BMT). The duration of preceding chemotherapy was 4-30 months and included treatment of recurrences in 10 children. At BMT 12 patients were in CR, 9 in PR and one had tumor progression. 10/15 of autologous bone marrows were purged using immunomagnetic bead method of Kemshead and 2/15 using 4 hydroperoxycyclophosphamide. Myeloablative therapy consisted of melphalan and total body irradiation (TBI) in 13 patients (three each supplemented by vincristine or adriamycin/etoposide), in one child of melphalan and mIBG and in 3 children of melphalan alone. 3 children received double autograft and 2 cyclophosphamide (and TBI). 10 patients survived 0-32 months from BMT and 5-48 months from diagnosis, respectively. 12 patients died including 7/12 of tumor progression and 5/12 of toxicity (venoocclusive disease, gut toxicity, septicemia, pneumonia). We conclude that at this point BMT after conventional high dose chemotherapy may provide the only real chance of survival for a significant number of children with metastatic neuroblastoma.

Abdominal Neoplasms↗

[Evaluation of the delayed skin reaction in chronically ill children].

The evaluation of delayed cutaneous hypersensitivity by using a standardized test system offers an assessment of cell-mediated immunity. The number and diameter of positive responses induced by seven antigens result in a score. The study included 21 healthy children, 20 children with bacterial infections as well as 20 patients with chronic renal insufficiency. In addition, 16 children with cystic fibrosis were also studied. No differences in the skin reactions was found in children with bacterial infections, showing a score of 13 +/- 6 mm. But children with cystic fibrosis or with renal insufficiency exhibited a severe impaired cell-mediated immunity. To what extent the alteration of cell-mediated immunity affects the course of the various diseases, has to be investigated by further prospective studies.

Adolescent↗

[Myeloperoxidase deficiency as a cause of recurrent infections].

Myeloperoxidase (MPO) deficiency is a common hereditary leukocyte function defect. A two year old girl with MPO-deficiency suffered from recurrent skin infections. No MPO-activity was detectable in leukocytes of her peripheral blood smears, while NBT reduction and chemotactic activity was normal. The quantitative enzyme determination in leukocyte sonicates confirmed the total MPO-deficiency in the girl's leukocytes and a partial MPO-deficiency in the cells of her mother. The patient leukocytes demonstrated also an impaired chemiluminescence.

Chemotaxis, Leukocyte↗

[Changes in the granulocyte membrane in mucoviscidosis].

Important polymorphonuclear leukocyte (PMN) functions are dependent on an intact cytoskeleton, consistent of the microtubulus and microfilament system. This can be assayed from the mobility of fluorescence labelled Concanavalin A (Con A) receptor complexes on the cell surface. Con A shows an uniform surface distribution on cells with an intact microtubulus system, whereas it's disruption causes a Con A cap formation. The alteration of the microtubulus and microfilament systems induces a patchy Con A distribution. Only 44% PMNs of 20 children with cystic fibrosis exhibited with a random Con A fluorescence distribution an intact cytoskeleton compared to 69% control PMNs. But 32% cystic fibrosis PMNs and 23% control PMNs showed a Con A capping phenomena, which is associated with a microtubulus alteration. In addition, 24% cystic fibrosis PMNs demonstrated a patchy fluorescence, representing a microtubulus and microfilament disruption, compared to 8% patched control PMNs. This study supports the idea, that the chronic bacterial infections cause in cystic fibrosis PMNs a cytoskeleton defect, which represents as a leukocyte-function defect an additional infection promoting factor.

Adolescent↗

[Team assessment of pain in children].

Using the Smiley analogue scale we investigated the correlation between the self-assessment of defined pain by children and the estimation of this pain by parents, nurses and physicians. In addition, the correlation was studied between the patient's mood and the pain intensity stated by himself and by others. The results of 111 patients, aged from 2(9)/12 to 17(6)/12 years, exhibited a high correlation between the self-assessments of pain intensity and pain duration by all three observing groups. Between patients and parents the best correlation was observed in the estimation of severe pain. All three observing groups inclined mainly to over- than to underestimate the pain intensity. This behavior pattern was mainly observed in parents. The patient's emotion did not influence the self-assessment of pain intensity and pain duration. But a great correlation was observed between the patient's emotion and the pain assessment by all three observing groups. Using a Smiley analogue scale the pain assessment by children or by others is a helpful tool for an individual pain therapy in the daily clinical routine.

Adolescent↗

[De Barsy-Moens-Dierckx syndrome: unusual course in a neonate].

We report about a premature infant with a De Barsy-Moens-Dierckx-syndrome, which is a rare cutaneo-oculo-cerebral malformation-syndrome. It is defined by the combination of a progeroid aspect, cutis laxa, growth retardation, cornea clouding, mental retardation and athetoid movements. Furthermore, the reported case showed a remarkable thermolability and suffered from generalised seizures resistant to therapy. Despite extensive sonographic examinations the prepartal diagnosis seems to be very difficult.

Abnormalities, Multiple↗

Treatment of essential thrombocythemia in childhood.

Essential thrombocythemia is a rare myeloproliferative disorder in childhood. For symptomatic patients with platelet counts greater than 1000 x 10(9)/L, cytoreductive treatment is recommended. The authors describe a 5-year-old boy with symptomatic essential thrombocythemia who was treated with anagrelide (Agrylin) for 23 months. He responded well but early on developed anemia. Because anagrelide is thought to specifically inhibit thrombopoesis, anemia is assumed to be a rather rare and late adverse effect. Its early occurence in this patient might indicate an increased vulnerability to anagrelide (Agrylin) in young children.

Anemia↗