Safety and efficacy of growth-friendly instrumentation for early-onset scoliosis in patients with spinal muscular atrophy type 1 in the disease-modifying treatment era.

Purpose: To evaluate the safety of growth-friendly instrumentation for early-onset scoliosis (EOS) in patients with spinal muscular atrophy (SMA) type 1 who received disease-modifying treatment (DMT) and analyze short-term efficacy.

Methods: Retrospective search was conducted between 2017 and 2023. Patients with genetically confirmed SMA type 1 who were surgically treated for spinal deformity and receiving DMTs (nusinersen, risdiplam, or onasemnogene abeparvovec) were included.

Association of Diabetic Ketoacidosis in Childhood New-Onset Type 1 Diabetes With Day of Presentation in Germany.

Objective: Whether the day of the week on which the child presents affects timely diagnosis and risk of diabetic ketoacidosis (DKA) in children with new-onset type 1 diabetes (T1D) is not known.

Research Design And Methods: We used data of 30,717 children with new-onset T1D during the last 10 years from the German Prospective Diabetes Registry. We determined the odds ratios of T1D diagnosis and DKA on a weekday, public holiday, and school vacation.

Medium-term Results After Femoral Head Resection and Subtrochanteric Valgus Osteotomy in Children and Adolescents With Neuromuscular Disorders.

Background: Various salvage surgical procedures for painful hip dislocation in adolescent patients with cerebral palsy exist. To date, no significant differences among these surgical techniques have been described. In our institution the McHale procedure is the standard of care for painful chronically dislocated hips with or without deformity of the femoral head in patients with cerebral palsy.

Radiolucent zones of biodegradable magnesium-based screws in children and adolescents-a radiographic analysis.

Introduction: Albeit the implantation of magnesium-based biodegradable implants can avoid a second surgery for implant removal, the postoperative occurrence of radiolucent zones around these implants based on corrosion processes has not been previously investigated in children and adolescents. We sought to characterize the distinct temporal and spatial dynamics for magnesium-based implants based on standard clinical routine radiographs.

Materials And Methods: We retrospectively analyzed 29 patients, treated with magnesium-based compression screws (MAGNEZIX CS 2.

Treatment of Unspecific Back Pain in Children and Adolescents: Results of an Evidence-Based Interdisciplinary Guideline.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the treatment and prevention of non-specific back pain in children and adolescents. A comprehensive and systematic literature search identified relevant guidelines and studies. Based on the findings of this literature search, recommendations on treatment and prevention were formulated and voted on by experts in a structured consensus-building process.

Etiology, Risk Factors, and Diagnosis of Back Pain in Children and Adolescents: Evidence- and Consensus-Based Interdisciplinary Recommendations.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the diagnosis of back pain and the treatment of non-specific back pain in children and adolescents. The first part comprises etiology, risk factors, and diagnosis. The second part, published in the same issue, includes treatment and prevention.

Anatomic accuracy, physiologic characteristics, and fidelity of very low birth weight infant airway simulators.

Background: Medical simulation training requires realistic simulators with high fidelity. This prospective multi-center study investigated anatomic precision, physiologic characteristics, and fidelity of four commercially available very low birth weight infant simulators.

Methods: We measured airway angles and distances in the simulators Premature AirwayPaul (SIMCharacters), Premature Anne (Laerdal Medical), Premie HAL S2209 (Gaumard), and Preterm Baby (Lifecast Body Simulation) using computer tomography and compared these to human cadavers of premature stillbirths.

Musculoskeletal Soft-Tissue Sarcoma: Quality Assessment of Initial MRI Reports Shows Frequent Deviation from ESSR Guidelines.

Soft-tissue sarcomas (STS) are a rare subtype of soft-tissue mass and are frequently misinterpreted as benign lesions. Magnetic resonance imaging (MRI) is the primary recommended type of diagnostics. To assess the quality of primary radiology reports, we investigated whether recommended MRI report elements were included in compliance with European Society of Musculoskeletal Radiology (ESSR) guidelines.

Safety and performance of biodegradable magnesium-based implants in children and adolescents.

Aims: Biodegradable magnesium-based alloy implants represent a promising option in orthopedic surgery, as the clinical outcomes have been reported to be comparable to those of titanium implants and no surgical interventions are required for removal. To date, little is known about the results of the use of these implants in children and adolescents. Therefore, the aim of the present study was to analyze the safety and performance of these implants in children and adolescents.

Iatrogenic severe hyperglycemia due to parenteral administration of glucose in children - a case series.

Background: Iatrogenic severe hyperglycemia (ISH) caused by glucose-containing i.v. solution is a potentially fatal treatment error.

Two-year outcome data suggest that less invasive surfactant administration (LISA) is safe. Results from the follow-up of the randomized controlled AMV (avoid mechanical ventilation) study.

Less invasive surfactant administration (LISA) is a method to deliver surfactant to spontaneously breathing premature infants via a thin catheter. Here we report the two-year outcome from the AMV (avoid mechanical ventilation) study, the first randomized controlled trial on this mode of surfactant delivery. No statistically significant differences in weight, length or neurodevelopmental outcome (Bayley II scores) were found between the LISA intervention group (n = 95) and the control group (n = 84) that received standard treatment.

Pedicle screw instrumentation with or without pelvic fixation in neuromuscular scoliosis: Outcome and complications in a series of 37 patients with a minimum 2-year follow-up.

Objective: Neuromuscular scoliosis (NMS) is often associated with rapid progressive spinal deformities. Indications, when to extend the instrumentation to the pelvis for pelvic obliquity are not generally accepted at this time. This study reports on the indications, surgical technique and results using pedicle screw instrumentation exclusively with or without pelvic fixation for spine fusion in patients with NMS.

Serial casting in early onset scoliosis: syndromic scoliosis is no contraindication.

Background: Serial casting is a treatment for early onset scoliosis (EOS) in young children to achieve curve correction before bracing or to postpone initial surgical treatment until the patient is older. Good results have been reported for patients with idiopathic early onset scoliosis (IS). However, there are few reports of results in non-idiopathic cases, and the benefits of non-surgical methods in the syndromic-associated early onset scoliosis subgroup are unknown.

Histological, epidemiological and anatomical analysis of 193 bone tumours of the scapula.

Backround: This study aimed to determine the frequency and distribution of bone tumours of the scapula as well as the histological and anatomical characteristics of these rare lesions in a large case series.

Methods: The records of all lesions of the scapula collected from 1975 to 2018 in our bone tumour registry and institute of pathology were evaluated. During these 43 years, 223 cases were identified.

Active perinatal care of preterm infants in the German Neonatal Network.

Objective: To determine if survival rates of preterm infants receiving active perinatal care improve over time.

Design: The German Neonatal Network is a cohort study of preterm infants with birth weight <1500 g. All eligible infants receiving active perinatal care are registered.

Bone tumours of the clavicle: Histopathological, anatomical and epidemiological analysis of 113 cases.

Backround: This retrospective study aimed to determine the frequency of bone tumours of the clavicle and their histopathological, anatomical and epidemiological characteristics in a large case series.

Methods: The records of 327 lesions of the clavicle collected from 1976 to 2018 in our bone tumour registry and institute of pathology were reviewed. Following data were evaluated: age, gender, side, radiological assessment, tumour location within the clavicle, and histopathological findings.

The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover.

Skeletal pathologies are frequently observed in lysosomal storage disorders, yet the relevance of specific lysosomal enzymes in bone remodeling cell types is poorly defined. Two lysosomal enzymes, ie, cathepsin K (Ctsk) and Acp5 (also known as tartrate-resistant acid phosphatase), have long been known as molecular marker proteins of differentiated osteoclasts. However, whereas the cysteine protease Ctsk is directly involved in the degradation of bone matrix proteins, the molecular function of Acp5 in osteoclasts is still unknown.

Anterior distal femoral hemiepiphysiodesis can reduce fixed flexion deformity of the knee: a retrospective study of 83 knees.

Background and purpose - Fixed knee flexion deformity in children is a common problem in various diseases including myelomeningocele and cerebral palsy. Until now, only a few studies focusing on the surgical procedure of anterior distal femoral hemiepiphysiodesis have been published. We analyzed outcome and correction rate in the largest case series to date of patients treated by staples or 8-plates.

Therapeutic Management of a Substantial Pelvic Aneurysmatic Bone Cyst Including the Off-Label Use of Denosumab in a 35-Year-Old Female Patient.

Aneurysmal bone cysts (ABC) are benign bone tumors, which are highly vascularized. The main course of treatment is curettage followed by bone grafting or cement insertion. Still recurrence remains a main problem for patients.

Recombinant Human Bone Morphogenetic Protein-2 (rhBMP-2) for the Treatment of Nonunion of the Femur in Children and Adolescents: A Retrospective Analysis.

. The aim of this study was to examine clinical and radiographic healing after rhBMP-2 application in children and adolescents presenting with nonunion of the femur and to investigate the safety of rhBMP-2 use in these cases. .

Bone morphogenetic protein-2 for the treatment of congenital pseudarthrosis of the tibia or persistent tibial nonunion in children and adolescents: A retrospective study with a minimum 2-year follow-up.

There is a lack of studies reporting on rhBMP-2 application in pediatric orthopaedics, although few reports demonstrated promising results of the use of rhBMP-2 in children, especially for spine fusion and for the treatment of congenital pseudarthrosis of the tibia. The objectives of this study were (1) to examine clinical and radiographic healing after rhBMP-2 application for the treatment of congenital pseudarthrosis of the tibia (CPT) or persistent tibial nonunion in children and adolescents, and (2) to investigate the safety of rhBMP-2 use in these cases. Therefore we reviewed the medical records of ten patients with a mean age of 8.

Impaired bone remodeling and its correction by combination therapy in a mouse model of mucopolysaccharidosis-I.

Mucopolysaccharidosis-I (MPS-I) is a lysosomal storage disease (LSD) caused by inactivating mutations of IDUA, encoding the glycosaminoglycan-degrading enzyme α-l-iduronidase. Although MPS-I is associated with skeletal abnormalities, the impact of IDUA deficiency on bone remodeling is poorly defined. Here we report that Idua-deficient mice progressively develop a high bone mass phenotype with pathological lysosomal storage in cells of the osteoblast lineage.

Preterm prelabor rupture of membranes and outcome of very-low-birth-weight infants in the German Neonatal Network.

Objective: It was the aim of our study to evaluate the independent effect of preterm prelabor rupture of membranes (PPROM) as a cause of preterm delivery on mortality during primary hospital stay and significant morbidities in very-low-birth-weight (VLBW) infants < 32 weeks of gestation.

Design: Observational, epidemiological study design.

Setting: Population-based cohort, German Neonatal Network (GNN).

Isolated syndesmotic injury in acute ankle trauma: comparison of plain film radiography with 3T MRI.

Objectives: To determine cut-off values and the accuracy of plain film measurements for the detection of isolated syndesmotic injury after acute ankle trauma and to investigate MRI findings of concomitant ankle injury.

Methods: Eighty-four consecutive patients with absent fracture in plain film radiographs were prospectively evaluated for isolated syndesmotic injury after acute ankle trauma. The tibiofibular clear space (TFCS), the tibiofibular overlap (TFO), and the medial clear space (MCS) were independently assessed in plain radiographs by two readers.