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Biomedical subjects

G Janssen

Publications and source records attributed to G Janssen.

105 records · Page 6Linked to original sources

Boswellic acids in the palliative therapy of children with progressive or relapsed brain tumors.

19 children and adolescents with intracranial tumors received a palliative therapy with H 15 at a maximum dose of 126 mg/kg BW/day. All patients had previously been treated with conventional therapy. No side effects were observed during a median 9 months application. The recently reported antiedematous effect of H 15 was documented by MRI in one patient with a peritumoral edema, thus sparing steroid therapy with its typical side effects. Five/19 children reported an improvement of their general health status; this might be a psychological effect of hope for tumor response during palliative care. Three/17 patients with malignant tumors showed a mainly transient improvement of neurological symptoms such as pareses and ataxia. Three further patients showed an increased muscular strength and one cachectic patient achieved a weight gain. These improvements might be attributed to the antiedematous effect of H 15. Because of the palliative situation of these patients, H 15 application was performed without prior rebiopsy for histological evaluation. Overlapping effects with a previous radiotherapy or chemotherapy may have occurred. An antiproliferative effect cannot be stated. To prevent an uncritical use of H 15, further studies with prospective central documentation have to be initiated to evaluate the clinical indications for H 15 in palliative therapy, optimal dosage and duration of application.

Adolescent↗

[Psychosocial support of patients with homozygous beta-thalassaemia].

The prognosis and therewith the quality of life of patients with beta-thalassaemia major is decisively influenced by the compliance with the therapy of the patients and their families, who are massively burdened with this lifelong and much time requiring treatment. To improve the compliance with therapy a group of 10 afflicted adolescents and young adults aged between 15 and 27 years was founded in 1992. The aims are to get to know the reasons for the unsatisfactory compliance with therapy, to promote the exchange of experience how to deal with the disease and its treatment, to give comprehensive medical informations and to improve in this way the own responsibility and the compliance with therapy. At the monthly meetings aspects of interaction between parents and the child with a hereditary disease are discussed. Also the themes of self-image and body image of these adolescents are set who are stigmatized by thalassaemia and grow up in uncertainty about the development of their disease and often suffer from mortal fear. Possibilities and limits of integration of these chronically ill patients within school and profession, and not at last within the clinique are debated. Although two members of this group have died, our patients show more interest in their disease, their therapy and their prospects since the beginning of this psychosocial care. The compliance with therapy has become better in the majority of patients.

Adolescent↗

[Out-patient palliative care of children with cancer and their families].

Pediatric palliative care is still in its beginning. Most children with life-threatening disorders succumb with incomplete symptom control under in-patient conditions. Due to the limited number of patients and large hospital-home distances the realization of out-patient concepts of palliative care especially for children and adolescents with cancer is purposeful and beneficial. In a pilot project such an a aforementioned concept of pediatric palliative care proved successfully working despite insufficient conditions. Guidelines of pediatric palliative care are to be defined at short notice in order to ascertain autonomy and mobility of patients aiming at highest possible quality of life. The recently initiated program of integrating ambulatory and in-patient care within the regulations set forth in Germany's last health care system reform offers the chance of palliative home care of children in their terminal phase of life.

Adolescent↗

Bone marrow oedema and aseptic osteonecrosis in children and adolescents with acute lymphoblastic leukaemia or non-Hodgkin-lymphoma treated with hyperbaric-oxygen-therapy (HBO): an approach to cure? -- BME/AON and hyperbaric oxygen therapy as a treatment modality.

BACKGROUND: There is a striking need for additional therapies of bone marrow oedema (BME) and aseptic osteonecrosis (AON) in paediatric oncology patients. Hyperbaric oxygenation (HBO) therapy used in the treatment of osteoradionecrosis is demonstrated effectiveness. Aim of this retrospective analysis was to investigate whether HBO-therapy might lead to subjective as well as objective effects in the treatment of BME and/or AON in paediatric oncology patients with acute lymphoblastic leukaemia (ALL) or Non-Hodgkin lymphoma (NHL). PATIENTS AND METHODS: Between 11/1988 and 01/2001 27/291 (9.3 %) patients with ALL or NHL were diagnosed with a BME and/or AON in the Clinic for Paediatric Oncology, Haematology, and Immunology at University of Dusseldorf. 19/27 patients were submitted to HBO-therapy. Patients received average 45 HBO-treatments per patient (min. 13, max. 80 treatments). The affected regions were re-evaluated with MRI for radiological extent of lesions every 3 months. Pain in its intensity and localisation was serially recorded during HBO-therapy as key symptom in 11 of 19 patients. RESULTS: 27 patients (15 females, 12 males; mean age at diagnosis of malignancy 8.2 +/- 4.7 (SD) years, range 7 months to 16 years) presented with 138 lesions. 133/138 lesions were localised in the lower extremities. At diagnosis of BME and/or AON, 78/133 lesions were shown in females and 55/133 lesions in male. Girls < 10 years predominantly presented BME (33 BME vs. 6 AON), girls aged > 10 years predominantly offered AON (28 AON vs. 11 BME). BME was more often exhibited in boys < 10 years (34 BME vs. 10 AON) and rarely in boys > 10 years (4 BME vs. 6 AON). 11 patients treated with HBO-therapy were serially evaluated for pain intensity throughout their HBO-therapy courses by visual analogue scale (VAS) assessment. During the first 15 treatment courses the HBO-therapy a clear-cut reduction of pain was observed. The mean pain score before the first HBO-treatment unit was 2.4 +/- 2.7 (X +/- SD), decreased before the fifth to 1.6 +/- 1.7 and prior to the 35 (th) and 40 (th) HBO treatment to 0. Girls < 10 years treated with HBO showed an increase of BME (31 --> 46) and declining AON numbers (6 --> 2). Girls > 10 years with and without HBO-therapy showed decrease of BME lesions (7 --> 4 vs. 4 --> 0), whereas AON increased in the HBO-treated group (28 --> 29) as well as the non-treated group (0 --> 4). Males < 10 years showed an increase in BME lesion numbers despite HBO intervention (24 --> 26). The AON lesion numbers dropped in parallel (6 --> 3). Male patients not treated with HBO showed constant numbers of BME (11-->11) and a decreased numbers of AON (4 --> 2). All differences are statistically not significant. CONCLUSIONS: Children and adolescents diagnosed with ALL or NHL have a risk for accruement of BME and/or AON irrespective of the age, with an almost exclusive involvement of the lower extremities. Lesions of pedal bones and ankle joints predominantly affect children < 10 years. Lesions of knee and hip joints predominantly affect children > 10 years. In children < 10 years of age we demonstrate declining AON numbers and conversion of AON to BME thereby implicating possible beneficial effect of HBO in such patients. HBO failed to show beneficial effect on BME whether by preventing new lesions or by improving existent lesions in children > 10 years.

Adolescent↗

Intensification of chelating-therapy in patients with thalassemia major.

With the introduction of "hypertransfusion" regimens the extent of disease- and therapy-related hemosiderosis has become the survival limiting factor for patients with beta-thalassemia major as iron transferred with transfusions cannot be excreted by physiological means. Subsequent introduction of deferoxamine therapy for iron elimination and prophylaxis of hemosiderosis has improved prognosis and life quality of these patients considerably. We report our experience with seven adolescent patients with beta-thalassemia and ineffective subcutaneous therapy and severe hemosiderosis-related organ complications. For that reason they received i. v. intensified chelate therapy. The patients were given 70 to 120 mg/kg DFO 7 days a week continuously via a Port-a-cath or Hickman central venous line. Under high-dose i. v. DFO therapy, serum ferritin levels significantly decreased in all patients. Target serum ferritin levels of 3 000 ng/ml were reached after 12 to 20 months of treatment. In 3 of the 5 patients that were treated for longer than 43 months serum ferritin levels even dropped below 2 000 ng/ml. Serum ferritin levels also correlated well with SQUID examinations. Therefore, monitoring of serum ferritin may be useful to monitor patient's compliance and control intensified DFO therapy. Continuous administration of the intensified DFO therapy induced normalization of liver function and left ventricular cardiac function in all patients who are still alive. Two patients died due to cardiac decompensation. In five patients 19 episodes of central catheter-related infections were observed (1.5 infections per 1 000 catheter days). No DFO-associated allergic reactions nor irreversible organ dysfunction were observed. Our results indicate that intensified i. v. DFO therapy is an effective and safe method for treatment of severe organ dysfunction in patients with thalassemia major. The most severe problems are catheter-related infections and inconsistent long-term compliance.

Adolescent↗

[Combined hypophyseal function test in children with homozygous beta-thalassemia].

Nowadays, endocrine problems like retarded sexual maturation and growth deficiency, lead to substantial suffering in patients with beta-thalassemia major. The aim of the present study is the differentiation between a possible insufficiency of the pituitary gland and dependent target organs, respectively. 12 patients (age 1.9-18.10 years) were examined by means of a combined pituitary function test. The corticotropic axis turned out to be normal as shown by a regular stimulation of cortisol in all patients tested. The thyrotropic function was unaffected in all children: elevated levels of TSH pointed to primary hypothyroidism. Pubertal values of LH and FSH were found in only 3 out of 7 patients at pubertal age. In 10 out of 12 children, stimulation led to a rise in STH levels above 10 ng/ml. On the whole, there was no evidence for pituitary insufficiency in beta-thalassemia major.

Adolescent↗

[Experiences with the combination carboplatin/VP 16 in the treatment of recurrent tumors in children].

The efficiency and toxicity of Carboplatin, a derivate of cisplatin in clinical use since 1981, was investigated in combination with VP16 in 11 children with recurrent or refractory tumors. 10 patients received therapy as outpatients without hydration. 160 mg/m2/day of Carboplatin were administered days 1 to 3, or days 1 to 5 as one hour infusion, combined with VP16 100 mg/m2/day, days 1 to 3, or days 1 to 5 as one hour infusion. One patient received Carboplatin, 600 mg/m2, as 24 hrs. continuous infusion in combination with VM26, 150 mg/m2/day as one hour infusion, 8 hours after Carboplatin. 9 of the treated children had measurable disease. 3 of 9 patients achieved a tumor response of more than 50% (partial remission). 5 of 9 children showed tumor response of less than 50% (stable disease). 1 out of 9 patients had progressive disease. 8 of 9 patients reported improvement or disappearance of clinical symptoms after the first course. All patients had severe myelosuppression with different times to recover. The WBC nadir was 14 days after Carboplatin/VP16. The nadir platelet counts occurred 21 days after therapy with fast recovery. Non-hematological side effects were minimal.

Adolescent↗

[Hepatitis B infections in cytostatically treated children].

Between Nov. 83 and Oct. 84 15 children under chemotherapy for malignant diseases, 2 relatives and 2 nurses developed hepatitis-B antigen seropositivity. Epidemiological studies gave evidence for non-parenteral spread of the infection. The course of the disease was usually asymptomatic or mild. Amongst the patients who developed hepatitis-B there was a considerable number of children who had presented with a recurrence of their malignant disease. Two characteristic cases of hepatitis B are presented showing the wide range of clinical manifestations of this disease in immunocompromised children with ALL.

Adolescent↗

Cerebellar mutism syndrome.

Since 1980, a growing number of pediatric patients with mutism following posterior fossa surgery have been recognized. This syndrome typically affects children and in rare cases young adults who become mute one or two days after tumor operation but do not show disturbances of consciousness or language comprehension. The disorder persists for 1 to 4 months. The pathogenesis is still unknown. Of 21 children who underwent surgery for large posterior fossa tumors between 1991 and 1995, 6 developed cerebellar mutism. Histologically the tumors were classified as astrocytoma WHO grade I, astrocytoma WHO grade II and ependymoma WHO grade III in one case and medulloblastoma WHO grade IV in three cases. Besides the clinical course, intraoperative findings and CT or MRI data are evaluated and discussed considering possible etiological hypotheses. Our own experience and also literature reviews suggest that the lesion of the cerebellar hemispheres might be the most important one of multiple factors causing cerebellar mutism. Generally the syndrome is transient. The diagnosis should not delay adjuvant therapy in patients with a malignancy.

Adolescent↗

Investigation of the kinetics of degradation of hexopyranosylated cytosine nucleosides using liquid chromatography.

Liquid chromatography was used to follow the degradation of hexopyranosylated cytosine nucleosides in buffers of acid, neutral and alkaline pH and of constant ionic strength. The compounds were found to degrade by hydrolysis to cytosine and/or by deamination to the corresponding uracil nucleosides. Degradation in acid is influenced by the number of sugar hydroxyl groups, presence of sugar double bonds and the type of anomer. Stability of some of the compounds was compared with that of related thymine nucleosides. Temperature studies support a unimolecular mechanism of hydrolysis at pH 1.22.

Buffers↗

Long-term endocrinologic changes in subjects practicing the Transcendental Meditation and TM-Sidhi program.

The Transcendental Meditation (TM) and a more advanced program, the TM-Sidhi program, have been reported to produce a number of acute and long-term metabolic and electrophysiologic changes. To investigate the possibility that the practice of these techniques may be associated with long-term endocrinologic changes, we prospectively evaluated 11 male subjects before and over a 3-year period after starting the TM-Sidhi program. A progressive decrease in serum TSH, growth hormone, and prolactin levels occurred over the 3 years while no consistent change in cortisol, T4, or T3 levels was observed. These results suggest that the long-term practice of the TM and TM-Sidhi program may have effects on neuroendocrine function. Further studies using 24-hr monitoring with frequent blood sampling will, however, be needed to fully assess the significance of the simultaneous decline of the anterior pituitary hormones with maintenance of levels of hormones from peripheral endocrine glands.

Adult↗

Treatment of relapsed Langerhans cell histiocytosis by cyclosporin A combined with etoposide and prednisone.

Optimal treatment for Langerhans cell histiocytosis (LCH) has not yet been established. High-risk patients with systemic LCH may have a fatal course of the disease despite intensive treatment. New approaches using cyclosporin A (CSA) showed promising results. Here, we report on a 4-year-old boy who presented with systemic LCH of skin, liver, bone, bone marrow, and soft tissue infiltrates. The patient was refractory to conventional therapy including VP16, prednisone, 6-mer-captopurine, methotrexate, and vinblastine. Therefore the patient was treated with CSA as continuous therapy (serum levels were kept between 300 and 400 ng/mL) as well as intensification with VP16 and prednisone every 4 weeks. As early as 4 months after starting this treatment, clinical symptoms completely disappeared except for a slightly enlarged liver. During the next 12 months all clinical symptoms except a limited skin involvement vanished although treatment with VP16 and prednisone was stopped and CSA serum levels were kept between 100 and 150 mg/mL. In conclusion, intensive therapy using high-dose CSA combined with VP16 and prednisone might be a therapeutic option for patients with otherwise refractory LCH.

Antineoplastic Agents, Hormonal↗