G Horneff

Publications (101)230.78 Total impact

• Article: [Biologics register JuMBO : Long-term safety of biologic therapy of juvenile idiopathic arthritis.]

  K Minden, J Klotsche, M Niewerth, G Horneff, A Zink
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  ABSTRACT: In recent years the treatment of juvenile idiopathic arthritis (JIA) has changed dramatically. Nowadays one out of three children with polyarticular JIA is treated with a biologic drug; however, knowledge about the long-term safety of biologics is still limited. Information on drug safety is collected in the JIA biologic register (BiKeR) and the follow-up register juvenile arthritis methotrexate/biologics long-term observation (JuMBO). The latter currently includes information on more than 700 young adults most of whom were treated with etanercept and prospectively followed for more than 5 years. Preliminary data on the long-term safety of etanercept for JIA are therefore available. Over an observation period of 1,800 etanercept exposure-years, events of particular interest, such as malignancies, serious infections and new onset immune-mediated diseases have been recorded which occurred at rates of 0.1, 1.1 and 0.9/100 patient-years, respectively. Overall, new safety risks were not detected during long-term etanercept exposure. Moreover, JuMBO has also provided information on the long-term outcome of JIA and initial evidence suggests that JIA outcome, especially in functional aspects has improved in the biologic era. Data from BiKeR and JuMBO contribute to the risk-benefit assessment of biologic drugs which have been implemented in the routine treatment of JIA.
  Zeitschrift für Rheumatologie 03/2013; · 0.46 Impact Factor
• Article: German evidence and consensus based guidelines 2010 for the treatment of juvenile idiopathic arthritis (JIA)

  G Dueckers, N Guellac, M Arbogast, G Dannecker, I Foeldvari, M Frosch, G Ganser, A Heiligenhaus, G Horneff, A Illhardt, I Kopp, R Krauspe, B Markus, H Michels, M Schneider, W Singendonk, H Sitter, M Spamer, N Wagner, T Niehues
  Pediatric Rheumatology 05/2012; 9:1-1. · 1.44 Impact Factor
• Article: Therapie mit TNFα-Inhibitoren

  G. Horneff, T. Hospach, G. Dannecker, D. Föll, J.P. Haas, H. Girschick, H.I. Huppertz, R. Keitzer, H.J. Laws, K. Minden, R. Trauzeddel, H. Michels, Pharmakotherapiekommission der GKJR
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  ABSTRACT: Inhibitoren von Tumornekrosefaktor α (TNFα) und andere Biologika haben die Therapieoptionen bei juveniler idiopathischer Arthritis erheblich erweitert. Die Wirksamkeit von Etanercept und Adalimumab wurde in randomisierten kontrollierten klinischen Studien nachgewiesen. Die Langzeitsicherheit bleibt Gegenstand laufender Untersuchungen. Die U.S. Food and Drug Administration berichtete im August 2009 über insgesamt 48 bösartige Tumoren bei mit Infliximab, Etanercept oder Adalimumab behandelten Kindern und Jugendlichen, was Fragen bezüglich eines erhöhten Malignomrisikos aufwirft. Lymphome machten mit 50% den größten Anteil der 48Malignome aus. Die Indikation für die Anwendung von TNF-Inhibitoren ist unter Berücksichtigung von Risikofaktoren sorgfältig zu prüfen. Dies gilt insbesondere für nicht zugelassene Substanzen, nicht zugelassene Indikationen und für Kombinationsbehandlungen mit TNF-Inhibitoren und Immunsuppressiva. Andererseits soll aufgrund der aktuellen Datenlage bei keinem Patienten, der die entsprechenden Voraussetzungen erfüllt, eine notwendige Therapie beendet oder nicht begonnen werden. Eine adäquate Aufklärung, Überwachung und Langzeitdokumentation der Behandlung im Register der Gesellschaft für Kinder- und Jugendrheumatologie werden dringlich empfohlen. Tumor necrosis factor α (TNF) inhibitors and other biologics have substantially extended the therapeutic options for juvenile idiopathic arthritis (JIA). The efficacy of etanercept and adalimumab has been proven in randomized, placebo-controlled clinical trials, while their long-term safety is being monitored in ongoing studies. In August 2009, the U.S. Food and Drug Administration reported on 48 malignancies in children and adolescents treated with infliximab, etanercept or adalimumab, raising questions about an increased cancer risk. Lymphomas formed the largest group (24 of 48) of the different cancer types. The indication for use of TNF inhibitors needs to be made very carefully and take risk factors into consideration. This is particularly necessary in the case of non-approved indications, non-approved substances and for the combination of TNF inhibitors with conventional immunosuppressive agents. On the other hand, this therapy should not be withheld from any JIA patient fulfilling the necessary criteria. Adequate patient and family information, thorough long-term follow-up as well as strict treatment documentation in the registry of the German Society of Pediatric and Adolescent Rheumatology (GKJR) are strongly recommended. SchlüsselwörterTumornekrosefaktorinhibitor-Malignomrisiko-Kinder/Jugendliche-Juvenile idiopathische Arthritis-Chronisch-entzündliche Darmerkrankungen KeywordsTumor necrosis factor (TNF) inhibitors-Malignancy-Children/adolescents-Juvenile idiopathic arthritis-Inflammatory bowel disease
  Monatsschrift Kinderheilkunde 05/2012; 158(4):372-377. · 0.27 Impact Factor
• Article: Incontinentia pigmenti Bloch-Sulzberger Fallbericht

  H. Schmeling, J. Wohlrab, K. Mathony, G. Gaber, U. Lieser, S. Burdach, G. Horneff
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  ABSTRACT: Hintergrund. Das Bloch-Sulzberger-Syndrom (Incontinentia pigmenti) ist eine X-chromosomal-dominant vererbte Erkrankung, die in variabler Expressivität verschiedene Organsysteme, die Haut, das Zentralnervensystem, die Augen, die Zähne und das Skelett betrifft. Die Diagnose kann aufgrund typischer Hautmanifestationen in den ersten Lebenswochen gestellt werden. Fallbericht. Die Erstvorstellung des derzeit 2-jährigen Mädchens erfolgte am 2. Lebenstag mit Krampfanfällen im Rahmen einer schweren Enzephalopathie mit nachfolgenden ausgeprägten Marklager- und Rindennekrosen. Das Kind entwickelte eine schwere globale Entwicklungsstörung mit spastischer Tetraplegie und Mikrozephalus. Typische Hauterscheinungen mit initial papulopustulösen Effloreszenzen gefolgt von streifiger und retikulärer Hyperpigmentierung traten erst im Rahmen einer septischen Infektion im Alter von 2 Jahren auf. Die Histologie einer Hautbiopsie sicherte die Diagnose. Resümee. Ohne initialen Hautbefall ist diese seltene Diagnose schwierig, gelingt aber, wenn die typischen Hautmanifestationen im Verlauf hinzutreten. Infektionserkrankungen führen möglicherweise erst zur Ausprägung der Hauterscheinungen. Bei unauffälliger Familienanamnese, Fehlen von Aborten und 2 lebenden gesunden Brüdern kann eine Neumutation angenommen werden. Background. Bloch-Sulzberger syndrome (incontinentia pigmenti) is an x-linked dominant disease, affecting the skin, the central nervous system, the eyes, the teeth and the skeleton with variable expression. Diagnosis is suspected in the presence of typical sequence of skin symptom change during the first weeks of life. Case report. Here, we report on a now two year old girl who first presented with epileptic seizures, severe encephalopathy with distinct necrosis of cerebral medulla and cortex at the age of two days. Thereafter the child developed mental retardation, spastic tetraparesis and microcephaly. There were no distinct skin eruptions. A typical generalised dermatosis appearing with papular and pustular lesions resulting into reticular hyperpigmentation became evident at the age of two years when she suffered from septic lymphadenitis. Diagnosis of incontinentia pigmenti was considered and confirmed by the histologic examination of skin biopsy. Conclusion. This case demonstrates, that if skin changes do not occur during the infant period, diagnosis may be delayed. Since the family history revealed no further affected individuals and the mother had no history of abortions with two living healthy sons, sporadic mutation may have occurred in this child.
  Monatsschrift Kinderheilkunde 04/2012; 149(1):41-44. · 0.27 Impact Factor
• Article: Aktualisierte Stellungnahme der GKJR zur Meldung der FDA über Fälle von Malignomen bei Anti-TNF-behandelten Patienten vom 04.08.2009

  G. Horneff, T. Hospach, G. Dannecker, D. Föll, J.P. Haas, H.J. Girschick, H.I. Huppertz, R. Keitzer, H.J. Laws, H. Michels, K. Minden, R. Trauzeddel
  [show abstract] [hide abstract]
  ABSTRACT: TNF-Inhibitoren und andere Biologika haben die Therapieoptionen bei der juvenilen idiopathischen Arthritis (JIA) erheblich erweitert. Die Wirksamkeit von Etanercept und Adalimumab wurde in randomisierten, kontrollierten, klinischen Studien nachgewiesen und führte zur Zulassung. Die Langzeitsicherheit bleibt Gegenstand von laufenden Untersuchungen. Berichte über Leukämien und Tumoren bei mit Etanercept, Infliximab und Adalimumab behandelten Kindern und Jugendlichen lassen Fragen über ein erhöhtes Malignomrisiko aufkommen, wobei Lymphome mit 50% den größten Anteil der 48 von der FDA berichteten Fälle ausmachten. In Konsequenz ist die Indikation für die Anwendung von TNF-Inhibitoren unter Berücksichtigung von Risikofaktoren, z.B. erhöhter familiärer Malignomhäufigkeit oder Vorbehandlung mit karzinogenen Substanzen wie Cyclophosphamid, sorgfältig zu prüfen. Dies gilt insbesondere für nicht zugelassene Substanzen, nicht zugelassene Indikationen und für Kombinationstherapien von TNF-Inhibitoren mit Immunsuppressiva. Andererseits soll aufgrund der aktuellen Datenlage aber bei keinem Patienten eine notwendige Therapie beendet oder nicht begonnen werden. Eine adäquate Aufklärung, Überwachung und Dokumentation der Behandlung im Register der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR) wird dringend empfohlen. TNF inhibitors and other biologicals have greatly expanded the therapeutic options for juvenile idiopathic arthritis (JIA). While the efficacy of etanercept and adalimumab has been proven in randomized controlled clinical trials, their long-term safety remains the subject of ongoing investigations. Reports of leukaemia and tumours in children and adolescents treated with etanercept, infliximab and adalimumab have raised questions about an increased risk for malignancies, with lymphoma accounting for the largest group at 50% of all 48 malignancies reported by the FDA. Consequently, TNF inhibitors should be indicated under careful consideration of individual risk factors, such as increased family occurrence of malignancies, or pre-treatment with carcinogenic substances such as cyclophosphamide. This is particularly true for non-approved substances, and non-approved indications, and for combination therapy of TNF inhibitors with immunosuppressive drugs. On the other hand, however, treatment should not be stopped or started in any patient in whom treatment is necessary due to the current knowledge. Adequate patient information, surveillance and documentation of treatment in the registry of the GKJR is strongly recommended. SchlüsselwörterTumor-Nekrose-Faktor- (TNF-) Inhibitoren-Malignome-Kinder-Jugendliche-Juvenile idiopathische Arthritis-Chronisch-entzündliche Darmerkrankungen KeywordsTumor necrosis factor (TNF) blockers-Malignancy-Children-Adolescents-Juvenile idiopathic arthritis-Inflammatory bowel disease
  Zeitschrift für Rheumatologie 04/2012; 69(6):561-567. · 0.46 Impact Factor
• Article: Evidence-based, interdisciplinary guidelines for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis.

  A Heiligenhaus, H Michels, C Schumacher, I Kopp, U Neudorf, T Niehues, H Baus, M Becker, B Bertram, G Dannecker, [......], M Gaubitz, G Gerdes, G Horneff, A Illhardt, F Mackensen, K Minden, U Pleyer, M Schneider, N Wagner, M Zierhut
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  ABSTRACT: Uveitis in juvenile idiopathic arthritis (JIA) is frequently associated with the development of complications and visual loss. Topical corticosteroids are the first-choice therapy, and immunosuppression is commonly used. However, treatment has not been standardized. Representatives from the German Ophthalmological Society, Society for Childhood and Adolescent Rheumatology, and the German Society for Rheumatology reached consensus on a standardized treatment strategy according to disease severity in the individual patient. The recommendations were based on a systematic literature analysis in MEDLINE and consensus expert meetings. Evidence and recommendations were graded, and an algorithm for anti-inflammatory treatment and final statements confirmed in a Delphi method. An interdisciplinary, evidence-based treatment guideline for JIA uveitis is presented.
  Rheumatology International 11/2011; 32(5):1121-33. · 1.88 Impact Factor
• Article: [Evidence and consensus based treatment guidelines 2010 for juvenile idiopathic arthritis by the German Society of Paediatric Rheumatology].

  G Dueckers, N Guellac, M Arbogast, G Dannecker, I Foeldvari, M Frosch, G Ganser, A Heiligenhaus, G Horneff, A Illhardt, R Krauspe, B Markus, H Michels, M Schneider, W Singendonk, H Sitter, M Spamer, N Wagner, T Niehues
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  ABSTRACT: Treatment of Juvenile Idiopathic Arthritis (JIA) has improved quality of life in children and adolescents with JIA. Standardisation of care offers the chance to improve the quality of care of those patients. New studies have been published after completion of our last treatment guideline (2007). An updated consensus process is mandatory. A systematic literature analysis in PUBMED (key words: juvenile idiopathic (rheumatoid) arthritis, therapy; limits: humans, published in the last 3 years, all child 0-18 years, clinical trial) revealed 17 relevant studies. Studies relating to diagnosis of JIA, Uveitis, vaccination, transition were excluded. Representatives nominated by scientific societies and organisations were invited to consensus conferences which were hosted by a professional moderator. The following societies were invited: Berufsverband der Kinder- und Jugendärzte (BVKJ), Deutsche Gesellschaft für Kinder- und Jugendmedizin (DGKJ), Deutsche Gesellschaft für Rheumatologie (DGRh), Deutsche Ophthalmologische Gesellschaft (DOG), Deutsche Rheuma-Liga Bundesverband, Verein zur Förderung und Unterstützung rheumatologisch erkrankter Kinder und deren Eltern, Vereinigung für Kinderorthopädie, Zentraler Verband der Physiotherapeuten und Krankengymnasten (ZVK). Consensus conferences were each attended by more than 95% of the nominated representatives. Consensus statements were confirmed by nominal group technique and Delphi method. Updated consensus statements regarding drug therapy, symptomatic and surgical management of JIA were compiled and judged strictly by the criteria of Evidence-Based Medicine (EBM).
  Klinische Pädiatrie 11/2011; 223(6):386-94. · 1.77 Impact Factor
• Article: [Education and training in pediatric and adolescent rheumatology - survey results : Gerd Horneff on behalf of the Committee for Undergraduate Training of the German Society for Rheumatology (DGRh)].

  G Horneff
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  ABSTRACT: In child and adolescent medicine pediatric rheumatology does not carry great weight. This fact is contrasted by the frequent occurrence of symptoms concerning the musculoskeletal system in childhood and adolescence. The obviously low interest in pediatric rheumatology probably has its roots in the education of undergraduates just as the often delayed diagnosis and inadequate therapy can possibly be credited to the content of specialist's training. The improvement of the educational situation is both concern and task of the German Society for Rheumatology (DGRh) and the German Society for Pediatric and Adolescent Rheumatology (GKJR).
  Zeitschrift für Rheumatologie 06/2011; 70(6):530-2. · 0.46 Impact Factor
• Article: Results of the German ESPED-recording of new patients with juvenile dermatomyositis (JDM).

  F Dressler, M Frosch, K Mönkemöller, A Thon, E Weissbarth-Riedel, G Horneff
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  ABSTRACT: In a nation-wide registration project 38 incident cases of juvenile dermatomyositis were collected in Germany over a 2-year-period. Diagnostic methods as well as the primary treatment for these patients were recorded. Detailed information was available for 25 of these patients. Diagnostic as well as therapeutic decisions varied widely. Steroids were used in almost all of the 25 patients either as oral or as parenteral pulse therapy, additional immunosuppressive drugs were used in 52%. We plan to establish national consensus recommendations for diagnostic and therapeutic standards in JDM. Due to the rarity of JDM clinical trials will have to be performed on an international basis.
  Klinische Pädiatrie 04/2011; 223(5):280-2. · 1.77 Impact Factor
• Article: [Juvenile arthritides].

  G Horneff
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  ABSTRACT: Arthritis in children represents a diagnostic and therapeutic challenge. The diagnostic spectrum is broad and a very precise indication for diagnostic and therapeutic procedures, especially in small children, is important. In addition to acute arthritides - viral arthritis, reactive arthritis, Lyme arthritis and septic arthritis - secondary chronic arthritis related to an underlying disease as well as juvenile idiopathic arthritis (JIA), the most common chronic inflammatory systemic disease in children, need to be considered. This overview is a guide to the diagnosis of arthritis in childhood and to evidence-based therapy of JIA in particular. This consists of a combination of nonsteroidal anti-inflammatory drugs, systemic and intraarticular corticosteroids, traditional DMARDs such as sulfasalazine, methotrexate and leflunomide, the TNF inhibitors etanercept, adalimumab and, with restrictions, infliximab, other biopharmaceuticals such as anakinra, canakinumab and rilonacept, and tocilizumab and finally, abatacept.
  Zeitschrift für Rheumatologie 10/2010; 69(8):719-35; quiz 736-7. · 0.46 Impact Factor
• Article: [Malignancy and tumor necrosis factor inhibitors in juvenile idiopathic arthritis].

  G Horneff
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  ABSTRACT: Reports on malignancies observed in children exposed to TNF-inhibitors have raised questions about a potentially increased risk for lymphoma in particular. To date, the number of children exposed to biologicals is small. In addition, knowledge about the background incidence of malignancies in children with JIA and the influence of co-medication is limited. Between 2001 and 2009 five cases of malignancy were documented in the German JIA Etanercept in Children Registry covering 1200 patients, including one case each of non-Hodgkin's lymphoma, Hodgkin's lymphoma, thyroid cancer, yolk sac cancer, and cervical dysplasia. All five patients had been treated with a number of other drugs including cytotoxic drugs (methotrexate, leflunomide, azathioprine, cyclosporine A) before institution of etanercept therapy. All patients were treated with etanercept, while two patients were also treated with adalimumab or infliximab. Malignancy appeared after an etanercept treatment period of between 3 weeks and more than 6 years. At the time of diagnosis, three patients were still on etanercept, five on methotrexate and one on infliximab. In three patients malignancy first occurred in adulthood. All patients recovered. This case series of JIA and malignancy shows that prior to starting treatment with TNF-inhibitors careful consideration needs to be given to the possible benefits and risks. Patients need to be observed long-term and observation should to be continued in adulthood. Although a temporal association has been described to date, a causal role of TNF inhibitors cannot be excluded and parents and/or patients should be appropriately informed about this risk.
  Zeitschrift für Rheumatologie 08/2010; 69(6):516-26. · 0.46 Impact Factor
• Article: [Updated statement by the German Society for Pediatric and Adolescent Rheumatology (GKJR) on the FDA's report regarding malignancies in anti-TNF-treated patients from Aug. 4, 2009].

  G Horneff, T Hospach, G Dannecker, D Föll, J P Haas, H J Girschick, H I Huppertz, R Keitzer, H J Laws, H Michels, K Minden, R Trauzeddel
  [show abstract] [hide abstract]
  ABSTRACT: TNF inhibitors and other biologicals have greatly expanded the therapeutic options for juvenile idiopathic arthritis (JIA). While the efficacy of etanercept and adalimumab has been proven in randomized controlled clinical trials, their long-term safety remains the subject of ongoing investigations. Reports of leukaemia and tumours in children and adolescents treated with etanercept, infliximab and adalimumab have raised questions about an increased risk for malignancies, with lymphoma accounting for the largest group at 50% of all 48 malignancies reported by the FDA.Consequently, TNF inhibitors should be indicated under careful consideration of individual risk factors, such as increased family occurrence of malignancies, or pre-treatment with carcinogenic substances such as cyclophosphamide. This is particularly true for non-approved substances, and non-approved indications, and for combination therapy of TNF inhibitors with immunosuppressive drugs. On the other hand, however, treatment should not be stopped or started in any patient in whom treatment is necessary due to the current knowledge. Adequate patient information, surveillance and documentation of treatment in the registry of the GKJR is strongly recommended.
  Zeitschrift für Rheumatologie 02/2010; 69(6):561-7. · 0.46 Impact Factor
• Article: [Comment of the Society of Pediatric and Adolescent Rheumatology on the US Food and Drug Administration (FDA) announcement regarding cases of malignancy in anti-TNF-treated patients].

  T Hospach, J P Haas, H I Huppertz, R Keitzer, H Michels, R Trauzeddl, D Föll, G Dannecker, G Horneff
  Zeitschrift für Rheumatologie 03/2009; 68(2):162-4. · 0.46 Impact Factor
• Article: Stellungnahme der Gesellschaft für Kinder- und Jugendrheumatologie zur Meldung der US-Food and Drug Administration (FDA) über Fälle von Malignomen bei Anti-TNF-behandelten Patienten

  T. Hospach, J.P. Haas, H.I. Huppertz, R. Keitzer, H. Michels, R. Trauzeddl, D. Föll, G. Dannecker, G. Horneff
  Zeitschrift für Rheumatologie 02/2009; 68(2):162-164. · 0.46 Impact Factor
• Article: Safety and efficacy of combination of etanercept and methotrexate compared to treatment with etanercept only in patients with juvenile idiopathic arthritis (JIA): preliminary data from the German JIA Registry.

  G Horneff, F De Bock, I Foeldvari, H J Girschick, H Michels, D Moebius, H Schmeling
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  ABSTRACT: Etanercept monotherapy has been studied and approved for treatment of polyarticular juvenile idiopathic arthritis (JIA). The following study evaluates the safety and efficacy of combination therapy of etanercept and methotrexate compared to etanercept monotherapy in JIA. We perfomed an open, non-randomised study on patients who had previously failed to respond to at least one disease-modifying antirheumatic drug (DMARD). A total of 722 patients with JIA in whom at least 1 item of follow-up data was recorded were identified; of these, 118 patients treated with further slow acting drugs were excluded. In all, 504 patients were treated with a combination of etanercept and methotrexate. A total of 100 patients treated with etanercept only were in the control group. Efficacy was calculated using the American College of Rheumatology paediatric scores for 30, 50 and 70% improvement (PedACR30/50/70). Adverse events (AEs) and serious adverse events (SAEs) were reported. After 12 months 55 patients in the monotherapy group and 376 patients in the etanercept and methotrexate group were available for comparison. For the intention to treat analysis, 65 patients discontinuing treatment prematurely were included. All activity parameters decreased significantly in both treatment groups. After 12 months 81%/74%/62% of patients of the etanercept and methotrexate group and 70%/63%/45% of patients of the etanercept monotherapy group achieved PedACR30/50/70 scores, respectively (p<0.05 for PedACR30, p<0.01 for PedACR70). The likelihood of achieving a PedACR70 increased with combination therapy with an odds ratio of 2.1 (95% CI 1.2 to 3.5). In total, 25 infectious and 23 non-infectious SAEs including 3 malignancies occurred in the etanercept and methotrexate group, and 1 infectious and 3 non-infectious SAEs occurred in the single etanercept group. The patients' disease activity improved during etanercept monotherapy and etanercept and methotrexate combination therapy. Tolerability in both treatment groups was comparable.
  Annals of the rheumatic diseases 05/2008; 68(4):519-25. · 8.11 Impact Factor
• Article: Lethal double infection with Acremonium strictum and Aspergillus fumigatus during induction chemotherapy in a child with ALL.

  J L Foell, M Fischer, M Seibold, M Borneff-Lipp, A Wawer, G Horneff, S Burdach
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  ABSTRACT: Fungal infections are a major cause of morbidity and mortality in patients during chemotherapeutic treatments and malignant hematologic disease. We present a case of a double fungal infection with disseminated Acremonium strictum (A. strictum) and pulmonary Aspergillus fumigatus (A. fumigatus) and its rapid clinical course. A 17-year-old boy with prolonged neutropenia developed a disseminated fungal infection during induction chemotherapy of his acute lymphoblastic leukemia. The infection was rapidly lethal despite neutrophil recovery and early antifungal combination therapy with amphotericin B and caspofungin. Since there are only a few reports about invasive Acremonium infections, we present this case with regard to differences in the clinic pathologic features of Aspergillosis and other opportunistic fungal infections due to Fusarium or Acremonium species.
  Pediatric Blood & Cancer 12/2007; 49(6):858-61. · 1.89 Impact Factor
• Chapter: Nichtrheumatische Ursachen von Arthralgien und Arthritiden

  K. Buckup, G. Horneff, W. Mannhardt-Laakmann, F. Zepp, M. Weiß, H. J. Girschick, S. Bielack, H. Olschewski
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  ABSTRACT: Eine Vielzahl angeborener, entzündlicher und verletzungsbedingter Erkrankungen können das wachsende Skelettsystem befallen. Zur Diagnose der orthopädischen Erkrankungen gehören die sorgfältige Anamnese und eine systematische klinische Untersuchung (Buckup 2005). Die Untersuchung muss in den typischen menschlichen Positionen des Stehens, Gehens und Liegens vorgenommen werden. Hierdurch bekommt man einen Eindruck, wie sich der Patient bewegt (⊡ Abb. 12.1) und wie er sich aufrichtet. Einige Erkrankungen wie z. B. Fehlhaltungen der Wirbelsäule, Fußfehlstellungen, Beinachsenfehler und Lähmungen können so schnell aufgedeckt werden.
  10/2007: pages 435-507;
• Article: [Inhibitors of tumour necrosis factor-alpha for the treatment of arthritis and uveitis in childhood].

  A Heiligenhaus, G Horneff, K Greiner, F Mackensen, M Zierhut, I Foeldvari, H Michels
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  ABSTRACT: Chronic uveitis in childhood is a common complication of juvenile idiopathic arthritis (JIA) that frequently leads to loss of vision. Besides from corticosteroids and immunosuppressive drugs, Tumour necrosis factor-alpha (TNF-alpha) inhibitors are used frequently. The literature published before September 2006 was evaluated for the usefulness of TNF-alpha inhibitors (etanercept, infliximab, adalimumab) for the treatment of JIA-associated uveitis. TNF-alpha inhibitors are effective drugs for the treatment of chronic uveitis in childhood. The response rate of uveitis in childhood to etanercept was approximately 50%. However, disease recurrence, first manifestations of uveitis and new complications occurred during the treatment. Infliximab and adalimumab appear to be more effective for the treatment of uveitis in childhood than etanercept. The therapy with TNF-alpha inhibitors is expensive and increases the long-term risk for secondary diseases, such as tuberculosis and probably malignant lymphoma. Their use should be restricted to uveitis patients not responding to corticosteroids and at least one of established immunosuppressive drugs.
  Klinische Monatsblätter für Augenheilkunde 07/2007; 224(6):526-31. · 0.51 Impact Factor
• Article: [Other rheumatic diseases with uveitis besides juvenile idiopathic arthritis].

  H Michels, K Greiner, C Heinz, G Horneff, G Ganser
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  ABSTRACT: In childhood and adolescence, uveitis is part of the clinical spectrum of many inflammatory-rheumatic diseases. Besides juvenile idiopathic arthritis juvenile, ankylosing spondylitis, infection-associated arthritides, infantile sarcoidosis, systemic vasculitides, inflammatory bowel diseases, hereditary autoinflammatory syndromes and the TINU syndrome have to be excluded. These inflammatory diseases can be differentiated clinically in connection with immunogenetic and molecular genetic investigations. Early diagnosis of uveitis as well as the underlying diseases is mandatory for an early treatment and therefore for a good prognosis.
  Klinische Monatsblätter für Augenheilkunde 07/2007; 224(6):473-6. · 0.51 Impact Factor
• Article: Akromegalie und generalisierte Lipodystrophie

  F. De Bock, U. Kiwit, P. Hilbert, G. Horneff
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  ABSTRACT: Das kongenitale Berardinelli-Seip-Lipodystrophie-Syndrom (BSCL) ist ein seltenes, autosomal-rezessiv vererbtes Krankheitsbild, das mit genereller Lipoatrophie, Akromegalie, Hepatomegalie, Hypertriglyzeridmie und Insulinresistenz einhergeht. Wir berichten ber den Fall einer 11-monatigen Tochter konsanguiner lybischer Eltern, die wegen ihres seit der Geburt aufflligen ueren vorgestellt wurde. Zustzlich bestanden ein akzeleriertes Skelettalter, eine Myopathie ohne Muskelschwche, jedoch keine mentale Retardierung oder Kardiomyopathie. Diese Konstellation ist typisch fr die Mutation des bscl1-Gens, welche durch eine molekulargenetische Untersuchung besttigt werden konnte. Das Mdchen wird regelmig klinisch und labortechnisch untersucht und befolgt eine ballaststoffreiche Dit mit hohem Anteil ungesttigter langkettiger Fettsuren. Das BSCL stellt eine genetische Extremform des metabolischen Syndroms dar und knnte daher neue Erkenntnisse zum Verstndis von Diabetes mellitus Typ 2 geben.The rare autosomal-recessive Berardinelli-Seip congenital lipodystrophy syndrome (BSCL) is characterized by general lipodystrophy, acromegalic appearance, hypertriglyceridemia, insulin resistance and hepatomegaly. Here, we present the case of an 11month old female infant of consanguineous parents from Libya, who was brought to us for diagnosis because of her unusual appearance since birth. Additionally to the above mentioned symptoms, we found an accelerated bone age, myopathy without muscle weakness, but no signs of mental retardation or cardiomyopathy. This constellation of symptoms is typical for the BSCL1 mutation phenotype, which was confirmed by molecular genetics. The girl will undergo frequent clinical and laboratory controls and follow a fibre- and medium chain fatty acid rich diet. This case demonstrates a genetic extreme ofmetabolic syndrome X and thus could provide new knowledge about diabetes mellitus type 2 and its prophylaxis.
  Monatsschrift Kinderheilkunde 04/2007; 155(5):446-450. · 0.27 Impact Factor