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Biomedical subjects

F Schilling

Publications and source records attributed to F Schilling.

At least 19 recordsLinked to original sources
PubMed1998

10 years' neuroblastoma screening in Europe: preliminary results of a clinical and biological review from the Study Group for Evaluation of Neuroblastoma Screening in Europe (SENSE).

Between January 1986 and May 1996, 870,313 children were tested in European neuroblastoma (NB) screening programmes. Among these children, 82 cases of NB (age range 4-24 months, median 11 months) were detected by screening. 83% of the patients had localised NB and 17% were diagnosed with generalised NB (stage 4, 10%; stage 4s, 7%). Unfavourable biological markers (MYCN amplification, loss of heterozygosity (LOH) 1p36, DNA di/tetraploidy) were observed in 14% of 76 biologically examined cases. The median follow-up time of all the patients was 21.5 months (range 1-101 months). To date, 69 patients are in complete remission (CR) and 2 patients have died due to therapy (stage 4, 1 patient; stage 3, 1 patient with unfavourable markers). Apart from screened patients, 16 other patients with NB were found who had previously had a normal screening test, i.e. 'false negative' patients (age range 10-41 months, median 31.5 months). The median interval between screening and diagnosis was 24.5 months (range 6-35 months). 11 of the 'false negative' patients suffered from generalised NB (stage 4) and 5 had localised NB at diagnosis. Unfavourable biological markers were observed in 7/12 patients. 5 patients have died, 2 achieved partial remission and 9 CR. 9 of the 11 patients with unfavourable biological markers diagnosed due to NB screening are currently in CR. It is very likely that, among the patients without unfavourable biological markers, we detected tumours which may have regressed spontaneously. These children may have undergone 'unnecessary,' but unavoidable, diagnostic procedures and therapy. To reduce the number of 'false negative' patients, a later screening could be helpful and should be evaluated.

Age Distribution↗
PubMed1998

[Chronic recurrent multifocal osteomyelitis (CRMO)].

Chronic recurrent multifocal osteomyelitis (CRMO) is an unusual clinical entity. More than 200 cases are described in the literature and it is presented here with special reference to its radiological aspects. It is an acquired disease of the skeleton which occurs predominantly during childhood and adolescence. About ten per cent of cases begin in early or, rarely, in later adult life. This variant is described here for the first time and is discussed as "adult CRMO". The underlying pathology is a bland, predominantly lympho-plasma cellular osteomyelitis which is self-limiting and leads to bone sclerosis (Garrè). It probably involves an abnormal immune process which follows an infection but remains clinically latent and remains aseptic and sterile. In a quarter of cases there is an association with pustulosis palmo-plantaris and its relationship with psoriatic arthropathy is discussed. The clinical, histopathological and imaging features (radiological and particularly MRT) and the bone changes are described. This provides a spectrum of symptoms; the radiological differential diagnosis and the relationship with hyperostotic spondyloarthroses during adult life are discussed. The relationship between CRMO, the SAPHO syndrome and acquired hyperostosis syndrome are analysed.

Adult↗
PubMed1998

[Spondarthritis hyperostotica pustulo-psoriatica: nosologic study with clinical and radiologic presentation in relation to the SAPHO syndrome].

Spondarthritis hyperostotica pustulo-psoriatica (Spond.hyp.pp-Schilling), corresponding nosologically to pustulotic arthroosteitis, is a dermato-skeletal "double system" disease of adults. It consists of the triad (a) palmo-plantar pustulosis (Ppp) or, alternatively, Königsbeck-Barber-type psoriasis, (b) sternocostoclavicular hyperostosis (SCCH), and (c) truncal-skeletal changes with syndesmophite-like, hyperostotic and/or parasyndesmophite-like ossifications of layers of the anterior vertebral ligament taken together in the sense of a desmophytal hyperostosis. There is also a potential for sclerosing inflammatory arthritis of the sacro-iliac joints and "dry" inflammatory arthritis of peripheral joints. Thus, the pustulo-psoriatic terrain seems to have a decisive influence on osseous pathology. A total of 38 cases from a study during the years 1982 to 1992 is analysed with regard to morphological characteristics. Rare cases with diaphyseal and pelvic hyperostotic lesions subsequent to bland sclerosing osteomyelitis constitute an overlapping region to chronic recurrent multifocal osteomyelitis (CRMO) and illustrate the relationship between hyperostotic spondarthritis and CRMO. The syndromes of "acquired hyperostosis" and "SAPHO", the former more radiologically oriented and the latter more clinically oriented, together with mainly CRMO and hyperostotic spondarthritis and its forms, constitute the "Spond.hyp.pp.". Although hyperostosis is a guidepost for the radiologist and SAPHO symptoms are one for the clinician, the syndrome does not represent a diagnosis by itself and requires further differentiation. In this report the entity "Spond.hyp. pp." is considered and required contributions from rheumatologically and osteologically oriented radiologist.

Acquired Hyperostosis Syndrome↗
PubMed1998

Computer-aided documentation and therapy planning in pediatric oncology.

In the past 20 years considerable progress was made in pediatric oncology concerning the results of therapy in Germany. Nationwide multicentre trials provide protocols for the therapy of the children. The calculation of the chemotherapy according to the protocols is rather complex and the documentation efforts for the pediatricians are enormous. Thus, we developed as a first step an application system for Computer Aided Therapy Planning In Pediatric Oncology (CATIPO), that is in routine use in about 20 pediatric clinics in Germany. In order to support the physician comprehensively with the documentation that is necessary for the trials we currently develop a Documentation System for Pediatric Oncology (DOSPO) that comprises the functionality of chemotherapy planning according to the protocols released by the trial centres. Besides supporting the physician in clinical routine the major objective is to improve the quality of the documented data. To reach this aims DOSPO combines research aspects of decision support and clinical documentation: formal representation of general protocols, calculating of a particular therapy for a patient, data acquisition, communication interfaces for transferring the data to the trial centres. In order to support trial centres an authoring system and a central data dictionary will be developed. This will enable the trial centres to develop new modules for trial-specific data acquisition in the clinics.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1996

Autosomal dominant spastic paraplegia with anticipation maps to a 4-cM interval on chromosome 2p21-p24 in a large German family.

Autosomal dominant familial spastic paraplegias (AD-FSP) are a group of genetically heterogeneous diseases characterised by a progressive spasticity of the lower limbs. Three loci have already been identified by genetic linkage studies on chromosomes 2p, 14q and 15q. Here we present linkage data from a large German family displaying AD-FSP with anticipation which confirms the existence of the FSP2 locus on chromosome 2p. The recombination events observed in our family define the critical region for the FSP2 gene to be within a 4-cM interval, flanked by markers D2S400 and D2S367. Moreover, clinical data from our family show evidence of anticipation, a phenomenon caused by trinucleotide expansion in several other neurodegenerative diseases.

Chromosome Mapping↗
PubMed1996

Comparison of the diagnostic and prognostic value of biological markers in neuroblastoma. Proposal for a common methodology of analysis. SENSE group.

BACKGROUND: The prognosis of pediatric neuroblastoma depends both on clinical presentation and on certain cellular and molecular characteristics. Screening programs have been initiated in infants of less than one year of age, based on the hypothesis that neuroblastoma progresses from early to late clinical stages through a classical multistep process linked to an accumulation of molecular abnormalities. However, recent analyses suggest that most cases discovered by screening are low stage tumors considered as dysembryogenetic residues devoid from major abnormalities and that high-grade tumors with molecular abnormalities are unrelated diseases. AIM OF THE REVIEW: To confirm one or the other hypothesis, and eventually identify biological factors possibly responsible for the initiation and progression of the disease, it is of utmost importance that all investigators agree on biological criteria for analysis when neuroblastoma tissue is available in screened and unscreened populations. This paper reviews the biological tools available for prognosis in neuroblastoma, the priority for analysis of biological markers according to both methodological reliability and feasibility, and the conditions of tissue storage for further analysis of these biological markers. CONCLUSION: The standardized biological evaluation of neuroblastoma will allow to collect sufficient data for multivariate analysis; such analysis is now fundamental if one wants to clearly define the respective impacts of biological abnormalities on neuroblastoma progression.

Biomarkers, Tumor↗
PubMed1996

[Sleep disorders in neurologic diseases].

Sleep disorders in central or peripheral nervous system diseases frequently occur, but they often are neglected in diagnosis and therapy. During the last 20 years, sleep medicine has obtained more and more importance. It is possible to draw conclusions about the topical organization of sleep-wake-regulation by investigating of certain diseases. In the following survey the most important clinical pictures in neurology are described in consideration of an affected sleep. Typical symptoms and polysomnographic findings as well as recommendations for therapy are demonstrated.

Humans↗
PubMed1993

Anomalous plasma concentrations and impaired secretion of growth factors in Fanconi's anemia.

In Fanconi's anemia, which is known to be an autosomal recessive Mendelian trait with four complementary groups. In addition to stunning phenotypic variation at clinical and cellular levels, aplastic pancytopenia is a common feature. Since either an early block of differentiation in stem cells or their insufficient support by stromal functions could be an underlying factor, levels of stem cell factor (SCF) and cytokines have been measured in blood and in supernatants of monocytes after stimulation with granulocyte-macrophage colony stimulating factor (GM-CSF). In two of three FA patients, no GM-CSF was detectable, and simultaneously SCF was decreased to 8% and 15% of normal values. The combination of low SCF and GM-CSF may be implied in the pathogenesis of marrow aplasia, since comparison with W/Sl mice shows that impairment of the SCF/c-kit function alone has different effects. Also, this explains that treatment with GM-CSF in a recent study enhanced only leukogenesis and not all three lineages. In the third patient, both factors were normal, and here a different mechanism may act. In all three FA patients, interleukin 6 (IL-6) production in stimulated monocytes was decreased, which may hamper immune defense of infections in a nonspecific way.

Adolescent↗
PubMed1990

Traditional and new types of spondarthritis with special consideration of spondylodiscitis.

In rheumatology the so-called "seronegative spondarthritis" is a group of diseases characterized by the presence of HLA-B 27. This group includes the typical ankylosing spondylitis as well as atypical spondylopathies such as those occurring in psoriasis, Reiter's disease and chronic inflammatory enteropathies, which attack mainly the spine and secondarily the peripheral joints. In some severe cases, non-infectious, sterile spondylodiscitis was observed. These can lead to instability and fracture, followed by pseudarthrosis of the involved segment of the spine. In contrast to these traditional spondarthritides three new types are marked by the lack of HLA-B 27. 1) "Spondarthritis hyperostotica pustulo-psoriatica" (F. Schilling), a very rare variation of psoriatic spondylopathy, sometimes accompanied by spondylodiscitis. 2) Arthritis and spondarthritis in acne fulminans. 3) Destructive arthropathy and spondylopathy in long-term hemodialysis, occasionally occurring with spondylodiscitis, a very new type of spondarthritis. The amyloid B (beta-2-micro-globulin), discovered only four years ago, plays a dominant role in the pathogenetic chain of this disease. Details of the etiology of these very impressive diseases are presented. Destructive spondylodiscitis will no doubt be a challenge to neurosurgeons.

Acne Vulgaris↗