[Handicapped children in foster and adoption families].
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Adoption↗
Biomedical subjects
J Kühl
Publications and source records attributed to J Kühl.
[Xanthogranulomatous pyelonephritis].
An 11-year-old boy suffered from malaise, weight loss and pallor. A palpable abdominal tumor on the right side, anemia and increased C-reactive protein were detected. Intravenous urography revealed destruction of the right kidney resembling Wilms tumor. But ultrasound and computered tomography rised skepticism. Analysis of previously documented cases suggests that xanthogranulomatous pyelonephritis must equally be considered in a child with unilaterally enlarged kidney without function, especially when the child shows fever, leukocytosis, bacteriuria, anemia, leukocyturia, calculi of the urinary tract, abdominal pain and/or a palpable abdominal tumor. Ultrasound and computered tomography can lead to the diagnosis, and identify extrarenal infiltration. Nephrectomy results in complete cure and is therefore the treatment of choice.
Child↗
[Histiocytosis X--a retrospective analysis of 40 cases with localized or disseminated disease].
A retrospective analysis of 40 cases with histiocytosis X was undertaken to find out the course of primarily localized disease, and the prognosis of children with initially disseminated disease. Bone lesions recurred in nine of 23 children with localized histiocytosis X. In eleven cases other organ manifestations occurred as well; in four cases without bone relapse. After an observation period of 1-14 3/12 years, nine of 22 children in remission suffer from long-term sequelae like diabetes insipidus, convulsion, extrahypothalamic CNS-disease, orthopedic disability, growth retardation, dystrophia adiposogenitalis , and chronic headache. Four of 17 children with disseminated histiocytosis X died. Our results and others from the literature indicate various risk factors to be prognostically significant. 1) age less than 2 years 2) involvement of spleen and/or lung 3) elevated Lahey-score 4) dysfunction of the hematopoietic system, liver, and/or lung 5) histologic feature resembling malignant type 6) no response to therapy 7) severely affected general health. These factors can be evaluated initially. Considering our own experiences and some risk factors we suggest the definition of four risk groups: 1.) localized histiocytosis X of bone, lymph nodes or skin; 2.) disseminated histiocytosis X with benign histologic type and Lahey-score of one or two; 3.) Lahey-score of 3-8; 4.) disseminated histiocytosis X with dysfunction of certain organ systems and/or malignant histology.
Bone Diseases↗
[Cervical lymph node tuberculosis in childhood -- still a current problem (author's transl)].
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Adolescent↗
[Clinical picture of infantile cortical hyperostosis].
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Clavicle↗
[Contributions on the knowledge of guiac resin. I. The principle of the blue color reaction].
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Chemical Phenomena↗
Modern treatment strategies in medulloblastoma.
Chemotherapy has an important role in the modern treatment of children with medulloblastoma (MB). In patients at high risk, intense chemotherapy should improve the survival rate. In low-risk patients chemotherapy should allow the dose of craniospinal irradiation to be reduced, which in turn should improve the quality of life. In infants under 3 years of age radiotherapy should be delayed, or even replaced by postoperative chemotherapy. Chemotherapy is also necessary to prevent or treat systemic dissemination. The optimal timing of chemotherapy is a focus of contemporary research.
Antineoplastic Combined Chemotherapy Protocols↗
Current diagnostic and therapeutic management of CNS metastasis in childhood primitive neuroectodermal tumors and ependymomas.
Metastatic disease is a major problem in the management of the most frequent childhood brain tumors, in ependymoma and primitive neuroectodermal tumors (PNET). Today, contrast enhanced craniospinal MRI and careful analysis of craniospinal fluid are a prerequisite for correct staging of both tumors and imply therapeutic consequences. So far metastatic spread of medulloblastoma and some ependymomas is prevented by conventional chemotherapy and radiotherapy. However, some forms of diffuse metastatic dissemination in medulloblastoma are resistant to conventional therapeutic regimens and require new experimental strategies.
Brain Neoplasms↗
Chemotherapy as prophylaxis and treatment of meningosis in children less than 3 years of age with medulloblastoma.
The outcome of children less than 3 years of age with medulloblastoma has been poor in comparison to older children. The cure rates were below 30%, and the quality of life for cured children was frequently reduced by a complex syndrome of long-term sequelae including intellectual retardation and growth hormone deficiency. Due to the deleterious side-effects of radiotherapy in very young children chemotherapy has played an important role in this group of patients. Firstly, chemotherapy should improve their survival rate. Secondly, it should allow dose reduction of craniospinal irradiation and a smaller involved field. With the goal of improving quality of life radiotherapy should be delayed or even replaced by postoperative chemotherapy. The EFS of low-risk patients steadily improved and is now as high as at least 50%. Since most patients of this group do not need radiation, treatment-related long-term sequelae are minimal. High-risk patients, by contrast, with metastatic disease or measurable postoperative tumor still have a very disappointing progression-free survival in a range below 30% at 3 to 4 years in all large studies. Therefore prevention and effective therapy of meningosis, as well as a good response to induction chemotherapy, are essential for the outcome. Strategies to increase the efficacy of conventional treatment modalities in high-risk patients are under investigation. Recently, interesting results have been published on high-dose chemotherapy followed by autologous stem cell rescue and intraventricular administration of the alkylating agent mafosfamide.
Antineoplastic Combined Chemotherapy Protocols↗
[Minimal invasive therapy of localized Langerhans-cell histiocytosis of bone].
PURPOSE: Scaglietti introduced a method of steroid injection for the management of unicameral bone cysts in 1974. Subsequently the intralesional infiltration of corticosteriods has also been recommended as a primary therapy for localized Langerhanscell histiocytosis (LCH). We report our experience with the administration of methylprednisolone acetate in children and young adults localized LCH. MATERIAL AND METHODS: [corrected] Nine patients with localized LCH, aged 2 3/12 to 29 years, were treated with a single--in only one case two--intralesional injection of methylprednisolone acetate as a crystalline suspension. The dose was between 40 and 150 mg depending on the size of the radiolucent defect. We treated 4 lesions in the skull, 3 in the femur, 1 in the distal humerus and 1 in the mandibula. In each case the diagnosis was established by biopsy. Follow-up ranged from 2 to 8 8/12 years (4 years 4 months on average). RESULTS: 7 out of 9 patients with localized LCH had excellent results with complete healing of the lesion. In 2 patients there was no response to the initial injection therapy and dissemination of the disease occurred. In 4 patients with an additional soft tissue tumor, after injection therapy of the bone lesion, the soft tissue tumor resolved without further treatment. CONCLUSION: Intralesional infiltration of methylprednisolone acetate as a primary therapy for localized Langerhans cell histiocytosis leads to rapid relief of pain, restoration of bone morphology and reduction of associated soft tissue tumors. Performed with appropriate skill under sterile condition with the reported high percentage of effectiveness and low recurrence rate, this low invasive method is the treatment of choice, resulting in a lower morbidity and lower costs.
Adolescent↗
[Late hormonal complications after brain tumor treatment in childhood and adolescence: literature review and a model of integrated hormone aftercare].
The variety of endocrine late-effects after brain tumour therapy in childhood is well known. Growth is usually impaired first due to a hypothalamo-pituitary growth hormone (GH) deficiency or a radiation-induced neurosecretory dysfunction of GH secretion. Generally, all hypothalamo-pituitary axes can be affected. Disturbances of puberty, gonadal function, thyroid function and adrenal function can manifest after a long posttherapeutic interval. A retrospective analysis of the auxological and endocrinological data assessed within the German brain tumour study HIT 91 showed that endocrinological follow-up of these patients was not satisfactory. Therefore we have developed a concept for the new brain tumour study: auxological and laboratory parameters will be assessed before oncological therapy and in regular intervals (4, 12, 18, 24 months after the end of tumour therapy). 24 months after the end of tumour therapy all hypothalamo-pituitary axes are to be evaluated with pharmacological stimulation tests.
Aftercare↗
Obesity after childhood craniopharyngioma--German multicenter study on pre-operative risk factors and quality of life.
BACKGROUND: Craniopharyngiomas are tumorous embryogenic malformations. As the survival rate after craniopharyngioma is high (92 %), prognosis and quality of life (QoL) in survivors mainly depend on adverse late effects such as obesity. PATIENTS AND METHODS: We analyzed 214 children and adolescents with craniopharyngioma. The records of 185 patients (86 %) were available for retrospective analysis of weight profiles and risk factors for obesity. Quality of life (QoL) was measured in 145 patients by the Fertigkeitenskala Münster/Heidelberg score (FMH) and in 77 patients by PEDQOL questionnaire. RESULTS: Eighty-two of 185 patients (44 %) developed severe obesity (body mass index [BMI] > 3 SD). Obese patients were compared with 79 patients (43 %) who kept normal weight (BMI < 2 SD). No differences between obese and normal weight patients were found in terms of gender distribution, age at diagnosis and follow-up period. However, the BMI SDS at the time of diagnosis was higher (p < 0.0001) in patients who developed obesity than in those who did not. Furthermore, obese patients presented with bigger tumors (p < 0.05) and a higher rate of a hydrocephalus requiring a shunt (p < 0.05) and hypothalamic involvement (p < 0.05). The mothers of patients who developed severe obesity had a higher BMI (p < 0.001) at the time of diagnosis. Obese patients had a higher height-SDS at diagnosis (p < 0.05) and at the time of last follow-up (p < 0.05) when compared with normal weight patients. A prediction model for severe obesity after craniopharyngioma was calculated by logistic regression based on the risk factors: patient's BMI > 2 SD at diagnosis (p < 0.05; odds ratio: 16.4), hypothalamic involvement (p < 0.05; odds ratio: 3.4) and maternal BMI > 25 kg/m(2) (p < 0.05; odds ratio: 4.6). Significant increases in BMI (p < 0.001) occurred during the early post-operative period especially during the first three years after diagnosis. FMH percentiles correlated negatively with BMI SDS (Spearman r: - 0.37; p < 0.001). Children with craniopharyngioma rated their QoL more negative (p < 0.05) in regard to physical abilities, cognitive functioning and social functioning when compared with healthy children of the same age group. Severely obese patients with craniopharyngioma estimated their QoL lower (p < 0.05) for all domains except for autonomy, cognition and familial integration in comparison with non-obese patients. CONCLUSION: Hypothalamic tumor involvement and familial disposition for obesity are risk factors for the development of severe obesity in patients with craniopharyngioma. As weight gain starts early after diagnosis and severe obesity causes a reduction in QoL, early therapeutic efforts should be considered in patients at risk. To confirm our results the prospective multicenter study Kraniopharyngeom 2000 on children and adolescents with craniopharyngioma was initiated (www.kraniopharyngeom.com).
Adolescent↗
[Neuroradiological differential diagnosis in medulloblastomas and ependymomas: results of the HIT'91-study].
BACKGROUND: Imaging criteria of medulloblastomas and ependymomas were defined retrospectively to allow a preoperative differential diagnosis of these malignant tumors of the posterior fossa. PATIENTS AND METHODS: CT and/or MR-studies of 103 patients with medulloblastomas and 19 ependymomas treated in the HIT'91-trial were centrally reviewed. Evaluation concerned the origin, CT-density or MR-signal intensity, enhancement, internal structural homogeneity and results of staging of the tumors. RESULTS: Medulloblastomas and Ependymomas showed different results concerning all imaging criteria. CONCLUSION: On the basis of differences in localisation, enhancement and tumor growth in most cases a preoperative differential diagnosis between medulloblastomas and ependymomas is possible. Increased density on CT allows the differentiation of the malignant tumors from pilocytic astrocytomas of the fourth ventricle.
Adolescent↗
[Improved prognosis of intracranial germ cell tumors by intensified therapy: results of the MAKEI 89 therapy protocol].
Germ cell tumors of the central nervous system are histological identical to the extracranial tumor sites. According to the localisation germ cell tumors of the CNS are different in symptoms, diagnostic approaches, kind and location of metastases and stratification of therapy. Since 1986 patients with intracranial germ cell tumors are registered in the ongoing study for non-testicular germ cell tumors (MAKEI) of the German Society of Pediatric Oncology and Hematology, and are treated in accordance to therapy guidelines for extracranial sites. In MAKEI 89 therapy strategy was revised with a reduction of radiotherapy and an increased cumulative cisplatinum dose from 200 mg/m2 to 400 mg/m2. Patients with germinoma receive after histologic diagnosis radiotherapy consisting of 30 Gy craniospinal irradiation and 15 Gy tumorboost. Malignant non-germinoma receive after diagnosis by tumor marker in CSF and/or serum 2 courses bleomycin 15 mg/m2 day 1-3, Etoposide 150 mg/m2 day 1 + 2 and cisplatinum 20 mg/m2 days 4-8 (BEP), continued by 2 courses Vinblastine 3 mg/m2 day 1 + 2, Ifosfamide 1500 mg/m2 days 1-5 and cisplatinum 20 mg/m2 days 1-5 (VIP), followed by 30 Gy craniospinal irradiation and 20 Gy tumor boost. In teratoma first line therapy is complete resection. In incomplete resected cases adjuvant chemotherapy according to histological grading is administered. Until 31st January, 1993 101 patients (pts) were registered, containing 69 protocol pts. Diagnosis in protocol pts was teratoma in 8 cases, 2 pts died postnatal because of extended disease, 2/8 pts relapsed, but were salvaged by chemotherapy. 40 pts offered germinomas.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Treatment of non-testicular germ cell tumors in children and adolescents with BEP and VIP: initial results of the MAKEI 89 therapy study].
The pilot protocol of the German Society of Pediatric Oncology for treatment of non testicular germ cell tumors was initiated in November 1987. The final protocol was started at 1. 1. 89. Different therapy was administered depending on histology, primary localisation or stage of tumors. Patients with malignant germ cell tumors such as dysgerminomas, embryonal carcinomas, yolk sac tumors or chorio carcinomas received BEP (Bleomycin 15 mg/m2/days 1-3, Etoposide 100 mg/m2/days 4-8, Cisplatinum 20 mg/m2/days 4-8), followed by VIP (Vinblastine 3 mg/m2/days 1 + 2, Ifosfamide 1500 mg/m2/days 1-5 including Mesna uroprotection and Cisplatinum 20 mg/m2/days 1-5). Patients with ovary tumors of stage 1 were treated with 3 courses of BEP, patients with ovary tumors stage II and extragonadal localisation received 3 courses of VIP in addition to 3 courses of BEP. In cases of extended tumors 4 courses of BEP were followed by delayed resection of tumors and 4 courses of VIP. Patients with intracranial germinomas were treated with 30 Gy of craniospinal radiation therapy and additional 15 Gy as a tumor boost. Since some cases of spinal extension were reported a spinal radiation therapy seems to be indispensable. Patients with intracranial embryonal carcinomas, yolk sac tumors or chorio carcinomas tumors were given 2 courses of BEP and VIP followed by 30 Gy of craniospinal radiation therapy and additional 20 Gy as a tumor boost. Patients with immature teratomas of the ovary grade 1-3 and grade 3 of tumors with extragonadal localisation were treated with 3 courses of BEP after resection of tumors. Until 1. 1. 1991 92 patients were reported to the study--27 with intracranial and 65 with extracranial primary localisation of tumors. 43 patients suffered from teratomas (including 20 immature teratomas grade 1-3), 18 from germinomas (seminomas/dysgerminomas) and 31 from malignant non-seminomatous germ cell tumors. After an observation period of 29 months disease-free survival rate was 80% (79/92 patients, Kaplan-Meier Statistics). Outcome of intracranial tumors was death or relapse in 2/9 patients with malignant non-seminomatous germ cell tumors, in 2/14 patients with intracranial germinomas, in 2/4 patients with teratomas. Patients with extracranial localisation of tumors suffered from death or relapse in 1/21 cases with non-seminomatous tumors, in 0/4 cases with dysgerminomas and 5/39 cases with teratomas. During pilot study one infant with a malignant non-seminomatous germ cell tumor died of a pneumopathia. Therefore infants treated according to the final protocol did not receive Bleomycin.(ABSTRACT TRUNCATED AT 400 WORDS)
Adolescent↗
[Transient myeloproliferation and acute myeloid leukemia in infants with Down's syndrome].
Transient neonatal myeloproliferative disorders (TMD's) indistinguishable from acute leukaemia by clinical and morphological criteria have been described in neonates with Down's syndrome. To analyse its clinical significance, 10 infants under 1 year of age presenting with Down's syndrome and the morphological picture of acute myelogenous leukaemia were reviewed. 3 of these children had true AML leading to death after 2, 8 and 11 months. In the other 7 children the diagnosis TMD was suggested as spontaneous or in one case interferon-induced remission occurred within 4 to 25 weeks after diagnosis. The interferon-treated patient died of SIDS at the age of 11 months. Another one of the TMD children developed fatal erythroleukaemia at the age of 2 years. Regarding initial clinical and haematological parameters, TMD was indistinguishable from true congenital leukaemie. In all patients classification according to the FAB criteria was difficult, as mainly undifferentiated or poorly differentiated myeloid blasts were seen, sometimes with erythro- or megakaryocytic features. Because of the difficulties in the differential diagnosis of TMD and true AML it is recommended to delay specific cytostatic therapy in neonates with Down's syndrome, until definite progression of the leukaemic process is observed or cytogenetic analyses suggesting true AML are available.
Antigens, CD↗
[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↗
[Langerhans cell histiocytosis in childhood--results of the DAL-HX 83 study].
Owing to the unclear and mostly unknown etiology of Langerhans' cell Histiocytosis (LCH) and the unsatisfactory results in treating disseminated LCH a prospective multicentric study DAL-HX 83 was commenced, including 45 different clinics of West-Germany, Austria and Netherlands. From June 1st, 1983 to October 31st, 1986, 97 patients (pts) were involved in this study. 35 pts (9 females, 26 males, medium age 6 2/12 years, age range 0/12-14 2/12 years) suffering from localized disease (28x unifocal bone, 6x isolated skin, 1x isolated lymphnode involvement) were treated by surgery and/or radiation or were just kept in observation. 2 children (1 pt with primary localized bone lesion, 1 child with isolated skin rash) developed a new bone lesion after 1/2 year and 1 1/2 years respectively. 62 pts (33 females, 29 males, medium age 2 years, range 0/12-17 1/2 years) with previously untreated disseminated disease were assigned to 3 different risk groups (A, B and C) and were treated according to a standardized induction and risk adapted maintenance protocol. The whole treatment period was limited to 1 year. 19 pts with multifocal bone involvement (group A, medium age 6 1/2 years) were allocated to regimen A, 30 pts with bone and soft tissue involvement or soft tissue involvement alone (group B, medium age 1 8/12 years) to regimen B and 13 pts with dysfunction of the liver, lungs and/or haematopoietic system (group C, medium age 1 year) to regimen C. So far, 1 pt of group A (19 available pts) developed a new bone lesion after 10 months, another pt a suspicious bone involvement 16 months after diagnosis. A 4 months old girl of group B (27 available pts) died 11 months after diagnosis with progressive organ dysfunction, 2 pts are still alive with recurrent multifocal bone lesions and 1 pt achieved stable 2nd clinical remission after a local relapse (mediastinum). 4 pts of group C (11 available pts) died because of progressive disease between 5 days and 3 years after diagnosis, 3 pts are in partial remission after persistent and recurrent disease episodes. All the others are in clinical remission. The medium observation time of the whole group of pts with disseminated LCH is 1 9/12 years (range 0/12-3 5/12 years). The worst prognostic criteria were found to be the presence of organ dysfunction at diagnosis or its development during the course of disease and the age under two years.(ABSTRACT TRUNCATED AT 400 WORDS)
Adolescent↗