Management of spinal deformities and tibial pseudarthrosis in children with neurofibromatosis type 1 (NF-1)

The skeletal system is affected in up to 60% of patients with neurofibromatosis type 1. The most commonly observed entities are spinal deformities and tibial dysplasia. Early recognition of radiologic osseous dystrophy signs is of utmost importance because worsening of the deformities without treatment is commonly observed and surgical intervention is often necessary. Due to the relative rarity and the heterogenic presentation of the disease, evidence regarding the best surgical strategy is still lacking.!##!Purpose!#!To report our experience with the treatment of skeletal manifestations in pediatric patients with (neurofibromatosis type 1) NF-1 and to present the results with our treatment protocols.!##!Materials and methods!#!This is a retrospective, single expert center study on children with spinal deformities and tibial dysplasia associated with NF-1 treated between 2006 and 2020 in a tertiary referral institution.!##!Results!#!Spinal deformity: Thirty-three patients (n = 33) were included. Mean age at index surgery was 9.8 years. In 30 patients (91%), the deformity was localized in the thoracic and/or lumbar spine, and in 3 patients (9%), there was isolated involvement of the cervical spine. Eleven patients (33%) received definitive spinal fusion as an index procedure and 22 (67%) were treated by means of 'growth-preserving' spinal surgery. Halo-gravity traction before index surgery was applied in 11 patients (33%). Progression of deformity was stopped in all patients and a mean curve correction of 60% (range 23-98%) was achieved. Mechanical problems with instrumentation requiring revision surgery were observed in 55% of the patients treated by growth-preserving techniques and in none of the patients treated by definitive fusion. One patient (3%) developed a late incomplete paraplegia due to a progressive kyphotic deformity. Tibial dysplasia: The study group comprised of 14 patients. In 5 of them (36%) pathological fractures were present on initial presentation. In the remaining 9 patients (64%), anterior tibial bowing without fracture was observed initially. Four of them (n = 4, 28%) subsequently developed a pathologic fracture despite brace treatment. Surgical treatment was indicated in 89% of the children with pathological fractures. This involved resection of the pseudarthrosis, autologous bone grafting, and intramedullary nailing combined with external fixation in some of the cases. In 50% of the patients, bone morphogenic protein was used 'off-label' in order to promote union. Healing of the pseudarthrosis was achieved in all of the cases and occurred between 5 to 13 months after the index surgical intervention. Four of the patients treated surgically needed more than one surgical intervention in order to achieve union; one patient had a re-fracture. All patients had a good functional result at last follow-up.!##!Conclusion!#!Early surgical intervention is recommended for the treatment dystrophic spinal deformity in children with NF-1. Good and sustainable curve correction without relevant thoracic growth inhibition can be achieved with growth-preserving techniques alone or in combination with short spinal fusion at the apex of the curve. Preoperative halo-gravity traction is a safe and very effective tool for the correction of severe and rigid deformity in order to avoid neurologic injury. Fracture union in tibial dysplasia with satisfactory functional results can be obtained in over 80% of the children by means of surgical resection of the pseudarthrosis, intramedullary nailing, and bone grafting. Wearing a brace until skeletal maturity is achieved is mandatory in order to minimize the risk of re-fracture

Does Juvenile Idiopathic Arthritis Affect the Course of Legg–Calvé–Perthes Disease? A Case-Control Study with a Mean Follow-Up of 8 Years

Background: This study aimed to determine the clinical and radiological course in children who had Legg–Calvé–Perthes disease (LCPD) associated with juvenile idiopathic arthritis (JIA). Methods: In a retrospective chart review between 2007 and 2019, eight consecutive JIA patients diagnosed with concomitant LCPD were identified and compared with a case-control group of 10 children with LCPD only. Results: LCPD was diagnosed at a mean age of 8.1 years (3.0–14.7) in children with JIA as compared to 6.1 years (2.9–10.0) in controls. According to the modified Harris Hip Score (mHHS), four children with JIA and all controls had an excellent result. Regarding the fragmentation severity and the duration of each stage, we found no differences using the lateral pillar and modified Elizabethtown classification. Five hips were classified as Stulberg I/II, two hips as Stulberg III, and one hip as Stulberg V with no evidence of hip dysplasia or severe overcoverage in either group. Conclusions: The radiological outcome of LCPD did not differ between both groups, while the clinical outcome was slightly better in controls. Physicians should be aware that children with LCPD may have JIA too. In suspicious cases, further investigations are recommended, and patients should be referred to pediatric rheumatologists

HIV-Ausbreitung bei i.v.-Drogenkonsumenten (IDU) in Mittel- und Osteuropa: Konsequenzen für Epidemiologie und Prävention in Deutschland

Bornemann R, Krämer A. HIV-Ausbreitung bei i.v.-Drogenkonsumenten (IDU) in Mittel- und Osteuropa: Konsequenzen für Epidemiologie und Prävention in Deutschland. In: Brockmeyer NH, Hoffmann K, Reimann G, Stücker M, Altmeyer P, Brodt R, eds. HIV-Infekt : Epidemiologie, Prävention, Pathogenese, Diagnostik, Therapie, Psycho-Soziologie. Berlin: Springer; 2000: 140-147

Aetiology, comorbidities and cofactors of chronic leg ulcers: retrospective evaluation of 1 000 patients from 10 specialised dermatological wound care centers in Germany

Numerous comorbidities and cofactors have been known to influence wound healing processes. In this multicentre study, clinical data of 1 000 patients with chronic leg ulcers from ten specialised dermatological wound care centers were analysed. The patient cohort comprised 567 females and 433 males with an average age of 69.9 years. The wounds persisted on average for 40.8months and had a mean size of 43.7 cm(2). Venous leg ulcers represented the most common entity accounting for 51.3% of all chronic wounds, followed by mixed-type ulcers in 12.9% and arterial ulcerations in 11.0% of the patients. Vasculitis was diagnosed in 4.5%, trauma in 3.2%, pyoderma gangrenosum in 2.8%, lymphoedema in 1.7%, neoplasia in 1.0% and delayed post-surgical wound healing in 0.6% of the included patients. In total, 70.5% of patients suffered from arterial hypertension, 45.2% were obese, 27.2% had non-insulin dependent diabetes, and 24.4% dyslipidaemia. Altogether 18.4% suffered from metabolic syndrome. Cofactors and comorbidities of patients with chronic leg ulcers have previously been studied but not in detail. Here, we were able to demonstrate the existence of several potentially relevant cofactors, comorbidities of their associations and geographical distributions, which should be routinely examined in patients with chronic leg ulcers and - if possible - treated

Konjunkturprognose, Mai 2020: Covid-19-Pandemie löst tiefe Rezession in der Schweiz aus

Seit dem Winter hat sich das Sars-Cov-2-Virus überall in der Welt verbreitet. Die Regionen sind zwar unterschiedlich betroffen, aber es gibt kaum ein Land ohne Covid-19-Erkrankungen. Wegen des intensiven weltweiten Austauschs von Personen und Waren sowie der Nähe zu Italien, war die Schweiz vergleichsweise früh mit einem exponentiellen Anstieg der Fallzahlen konfrontiert. Um die Zunahme der Ansteckungen, Hospitalisierungen und Todesfälle einzudämmen, ordnete der Bundesrat Mitte März auf Basis des Epidemiengesetzes zum grossen Teil per Notrecht Massnahmen an, welche direkte Kontakte zwischen Menschen auf das Notwendigste reduzieren sollten. Dadurch wurden auch viele wirtschaftliche Aktivitäten eingeschränkt

Konjunkturanalyse: Prognose 2018/2019. Breit abgestützter Aufschwung

Der vorliegende Beitrag dokumentiert die Ergebnisse der Frühjahrsprognose 2018 der KOF Konjunkturforschungsstelle der ETH Zürich vom 28.März 2018. Im ersten Teil werden die jüngsten Wirtschaftsentwicklungen im Ausland und in der Schweiz diskutiert und die wichtigsten Prognoseergebnisse in den verschiedenen Wirtschaftsbereichen dargestellt. Im zweiten Teil folgen die detaillierten Prognosen für die Schweiz, aufgeteilt nach den wichtigsten verwendungsseitigen Komponenten des Bruttoinlandprodukts

Konjunkturanalyse: Prognose 2021 / 2022 Der Aufschwung ist da – früher und stärker als erwartet

Der vorliegende Beitrag dokumentiert die Ergebnisse der Konjunkturprognose Sommer 2021 der KOF Konjunkturforschungsstelle der ETH Zürich vom 22. Juni 2021. Im ersten Kapitel werden die jüngsten Wirtschaftsentwicklungen in der Schweiz diskutiert. Im zweiten Kapitel folgen die wichtigsten Prognoseergebnisse für die verschiedenen Wirtschaftsbereiche, aufgeteilt nach den wichtigsten verwendungsseitigen Komponenten des Bruttoinlandprodukts. Das dritte Kapitel erörtert die wichtigsten Prognoseergebnisse für die Weltwirtschaft. Zuletzt folgt ein ausführlicher Datenanhang.This text summarises the 2021 summer forecast of the KOF Swiss Economic Institute at ETH Zurich, dated 22 June 2021. The first chapter discusses recent economic developments in Switzerland. The second presents the main forecast results across the various sectors of the economy, decomposed into the main demand components of GDP. The third chapter discusses the most important forecast results for the international economy. The final chapter consists of a comprehensive data appendix