Introduction The development of scoliosis in neuromuscular diseases is frequently seen in early childhood. Unfortunately, management of scoliosis with braces or physical therapy is usually not successful and leaves the child with a severe spinal deformity at maturity. The treatment with telescopic VEPTR devices theoretically offers the possibility to avoid rapid progression and the need for braces. It also avoids the need for early spine fusion with the deleterious effects on pulmonary growth. Material and methods 13 Children, seven female and six male patients, with an average age of 8 years and 8 months had an Eiffel tower VEPTR construct performed due to progression of neuromuscular scoliosis. Underlying diagnosis were myelomeningocele in six and muscular diseases in five patients. One patient had cerebral palsy and another patient had arthrogryposis. The average follow-up was 11 months. Results Average time for surgery was 110 min. No blood transfusions were required. Average hospital stay was 9 days. The Cobb-angel of the main curve decreased from 62
before to 31
after surgery which is a correction of 50%. Pelvic obliquity was corrected in all cases. No complications during surgery were encountered. Immediate complications after surgery included one wound slough in a patient with spastic quadriparesis and one pneumonia in a patient with spinal muscular atrophy and severe restrictive lung disease. Two patients with myelomeningocele had urinary tract infections which resolved after antibiotic treatment. Late complications included two broken ilium hooks in one patient with myelomeningocele and flexible thoracolumbar kyphoscoliosis and two proximal cradle migrations in patients with myelomeningocele. These problems were revised during the lengthening procedures which were scheduled every 6 months. One patient with poor skin coverage suffered from a deep infection which was revised successfully without removing the device. Patient and parent satisfaction was very high in all cases. Discussion VEPTR was devised as a non fusion technique especially for congenital scoliosis. Recently it has also been recommended for neuromuscular deformities. It is our strategy to apply a bilateral rib to pelvis construct (Eiffel tower-construct) to patients with neuromuscular spinal deformities who are not ambulatory. Our preliminary data suggest that this approach offers a very good correction of spinal deformities during growth with low morbidity and reasonable complication rate. It may avoid performing early spine fusions and the development of thoracic insufficiency syndrome.

Introduction Convex hemiepiphyseodesis is still a reasonable option to treat young patients with congenital scoliosis taking away growth from the convexity. This study aims to clarify, if growth of the concavity can be established by the VEPTR method even after convex hemiepiphyseodesis. Material and Methods 3 patients, 2 girls and 1 boy, had VEPTR surgery at an average of 4 years after thoracic convex hemiepiphyseodesis as the index procedure. Follow up was from 6 months to 4 + 6 years. The age at the time of surgery was 8–9 years. Results Main thoracic curves corrected from 51
to 37
, from 55
to 44
, and from 40
to 36
, respectively. Mean secondary lumbar curves improved from 32
to 20
and from 24
to 20
. For 1 patientwho had 3VEPTR devices implanted, 1 rib had to be removed due to an infected skin slough without compromizing the final result. All patients were treated without a brace. Sport activities were encouraged. Discussion Convex hemiepiphyseodesis reduces the risk of progression of congenital scoliosis but may lead to thoracic insufficiency syndrome later due to impaired longitudinal and latitudinal growth of the thorax. In this limited case study we were able to show that VEPTR may improve pulmonary function of congenital curves by induction of concave growth even after convex hemiepiphyseodesis.

Objective: To evaluate the success rate and subsequent restoration of normal acetabular angle (AC°), and associated growth changes secondary to avascular necrosis of the proximal femur in cases of developmental dysplasia of the hip (DDH) treated during the first six months of life Design: Retrospective study from 1998 to 2006 Setting: Department of Pediatric Orthopedics, Altona Children Hospital, Hamburg, Germany Subjects: Twenty six patients with twenty nine dysplastic hips were evaluated regarding grade of hip dysplasia. The success rate of closed reduction, postoperative restoration of normal acetabular coverage (AC angle) and associated complication were noted. Intervention: Closed reduction of DDH with intraoperative arthrogram followed by spica cast Main Outcome Measures: Success rate of closed reduction and restoration of normal acetabular index. Results: Closed reduction could be achieved in twenty two hips (76%). Out of those twenty two hips fifteen (68%) developed normal acetabular index, six (27%) showed persistent acetabular dysplasia and one (5%) had border -line measurement. Closed reduction was not successful in seven hips. Those seven hips were treated by open reduction and capsulorrhaphy. Conclusion: Closed reduction of DDH in the first six months of life was achieved in (76%) of dislocated hips, and did not show any significant growth changes in the proximal femur. The early changes in the ossific nucleus of capital femoral epiphysis (CFE) alone were found to be of very little value in predicting the nature of development of the hip.

Background Tissue injury due to hypoxia and/or free radicals is common in a variety of disease processes. This cross-sectional study aimed to investigate effect of chronic kidney diseases (CKD) and hemodialysis (HD) on hypoxia and oxidative stress biomarkers. Methods Forty pediatric patients with CKD on HD and 20 healthy children were recruited. Plasma hypoxia induced factor-1α (HIF-1α), vascular endothelial growth factor (VEGF) were measured by specific ELISA kits while, total antioxidant capacity (TAC), total peroxide (TPX), pyruvate and lactate by enzymatic/chemical colorimetric methods. Oxidative stress index (OSI) and lactate/pyruvate (L/P) ratio were calculated.

Background: In "-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies mainly in adult demonstrating renal involvement in "-thalassemia. This prospective study was aimed to investigate renal involvement in pediatric patients with transfusion dependant beta-thalassemia major (TD-"TM), using both conventional and early markers of glomerular and tubular dysfunctions, and to correlate findings to oxidative stress and iron chelation therapy. Methods: Sixty-nine TD-"TM patients (aged 1-16 years) and 15 healthy controls (aged 3-14 years) were enrolled in this study. Based on receiving chelation therapy (deferoxamine, DFO), patients were divided into two groups: group [I] with chelation (n = 34) and group [II] without chelation (n = 35). Levels of creatinine (Cr), calcium (Ca), inorganic phosphorus (PO4), uric acid (UA) and albumin were measured by spectrophotometer. Serum (S) levels of cystatin-C (SCysC) and total antioxidant capacity (STAC) and urinary (U) levels of "2-microglobulin (U"2MG) were measured by immunosorbent assay (ELISA). Urinary N-acetyl-beta-D-glucosaminidase (UNAG) activity and malondialdehyde (UMDA) were measured by chemical methods. Estimated glomerular filtration rate (eGFR) was determined from serum creatinine. Results: In patient with and without chelation, glomerular [elevated SCysC, SCr, Ualbumin/Cr and diminished eGFR]; and tubular dysfunctions [elevated SUA, SPO4, UNAG/Cr, U"2MG/Cr] and oxidative stress marker disturbances [diminished STAC and elevated UMDA/Cr] were reported than controls. In patients with chelation, SCysC was significantly higher while, STAC was significantly lower than those without chelation. In all patients, SCysC showed significant positive correlation with SCr and negative correlation with eGFR; STAC showed significant positive correlation with eGFR and negative correlation with SCysC, SCr, UNAG/Cr; UMDA/Cr showed significant positive correlation with Ualbumin/Cr, U"2MG/Cr, UNAG/Cr. Conclusions: Our data confirm high frequency of glomerular and tubular dysfunctions in TD-"TM pediatric patients which could be attributed to oxidative stress and DFO therapy.