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Duchenne muscular dystrophy is a progressive disease usually associated with loss of ambulation and progressive scoliosis. Immobilisation and glucocorticoid treatment are predisposing factors for reduced bone mineral density (BMD). Analysis of quantitative computed tomography revealed low BMD in thoracic and lumbar vertebrae in comparison to age- and sex-matched healthy controls.Evaluation of vertebral bone mineral density (BMD) in Duchenne Muscular Dystrophy (DMD) adolescents with untreated advanced scoliosis and comparison with the BMD values of healthy age-matched controls, based on quantitative computer tomography.Thirty-seven DMD adolescents (age 15.6 ± 2.5 years) with spinal deformity were evaluated clinically and radiologically prior to definite spinal fusion and compared to 31 male and age-matched healthy individuals (age 15.7 ± 2.3 years). Data related to previous medical treatment, physiotherapy and ambulatory status was also analysed. Scoliotic curves were measured on plain sitting radiographs of the spine. The BMD Z-scores of the thoracic and lumbar vertebrae were calculated with QCTpro® (Mindways Software Inc., USA), based on data sets of preoperative, phantom pre-calibrated spinal computed tomography scans.A statistically significant lower BMD could be found in DMD adolescents, when compared to healthy controls, showing an average value for the lumbar spine of 80.5 ± 30.5 mg/cmDMD adolescents with scoliosis have strongly reduced BMD Z-scores, especially in the lumbar spine in comparison to healthy controls. These findings support the implementation of a standardised screening and treatment protocol. Level of evidence/clinical relevance: therapeutic level III.

Purpose Magnetically controlled implant systems have been established to treat severe progressive spinal deformity in children. The purpose of this study was to evaluate (1) the ratio between achieved and expected distraction length, (2) the complication rate and its risk factors as well as (3) the correlation of the distraction length and the length of the spine. Methods A total of 40 patients with an average follow-up of 34 (14 to 57) months were prospectively included in the study. Children underwent lengthening procedures every three months. The ratio between the distraction lengths was determined by comparing the measured distraction length of the rod on radiographs with the distraction length displayed on the external remote controller for the magnetically controlled growing rod (MCGR). Age, weight, height, and complications were repeatedly recorded. Results The analysis of 746 procedures showed the actual distraction to be 94.4% of the expected one. No difference between implants on the concave and convex spinal side was observed. The overall complication rate was 4.6% mainly because of failure of the implant or lack of implant extension, which was directly related to an increased BMI. There was also a strong correlation between achieved implant distraction length and gain in spinal length. Conclusions Our study demonstrates a high ratio (0.94) between achieved and expected distraction length of magnetically controlled spinal rods. The complication rate was low (4.6%) and correlated to a high BMI. The correlation between the achieved implant distraction length and spinal length indicates the efficiency of the MCGR therapy. Level of evidence Therapeutic Level IV.

Magnetically controlled growing rods (MCGR) are commonly implanted for the treatment of early-onset scoliosis. While most authors report favorable short-term results, little is known about long-term deformity correction. This prospective cohort study assesses spinal deformity control in a homogeneous spinal muscular atrophy (SMA) patient group treated with MCGR implants, a standardized lengthening protocol and a minimum follow-up of four years. 17 SMA patients with progressive scoliosis were treated with MCGR implanted parallel to the spine with rib-to-pelvis fixation. Radiologic measurements were performed before and after MCGR implantation and during external lengthening procedures. These included measurements of the scoliotic curve, kyphosis, lordosis, pelvic obliquity and the spinal length. Additional clinical data of the complications were also analyzed. 17 children (mean age 7.4 years) were surgically treated and underwent a total of 376 lengthenings. Complication rates were 3.5% in respect to all interventions or 41% of the patients had complications during 3.5% of the lengthening sessions. The initial implantation significantly reduced the main scoliotic curve by 59%, with the correction remaining constant throughout the follow-up. Pelvic obliquity was also significantly and permanently corrected by 72%, whereas kyphosis and lordosis were not influenced. The spinal length could be significantly increased mostly during the first year of treatment. Bilateral implantation of MCGRs for correction of spinal deformity in children with SMA showed no decrease of the lengthening potential during a four-year follow-up. Therefore, the previously described ‘law of diminishing returns’ could not be applied to this patient population. Level of Evidence/Clinical relevance: Therapeutic Level IV.

Background Context In several studies, vertical expandable prosthetic titanium rib (VEPTR) implants have shown good scoliosis control in children with the longest reported follow-up of 3.6 years. For growing rods, recent studies suggest a decreased efficiency of correction starting just after that time. To our knowledge, no long-term results of children with VEPTR treatment are available. Purpose This study aimed to evaluate spinal deformity in scoliotic children and to investigate correction potential of VEPTR implants at several time points of treatment, particularly after long-term follow-up. Study Design/Setting We performed a retrospective case series of 32 children with spinal deformity and VEPTR treatment with analysis of clinical and radiological data pre- and post-VEPTR implantation and every 2 years during the follow-up period. Patient Sample Thirty-two patients with spinal deformity and VEPTR treatment comprised the patient sample. Outcome Measures Patients had a primary VEPTR implantation due to spinal deformity and thoracic insufficiency syndrome and repeated lengthening procedures every 6 months. Clinical data were assessed and radiological parameters were analyzed. The main thoracic scoliotic curve and associated curves as well as kyphosis, lordosis, pelvic obliquity, and spinal length were measured in all radiographs until the end of VEPTR treatment or the last available examination. Methods Development of the different parameters during follow-up was evaluated and statistical analysis was performed with Statistica version 13.0. No funding was obtained for this study. The authors have no conflicts of interest to declare. Results Directly after VEPTR implantation, thoracic and lumbar curves corrected significantly, were stable at 2.8-year follow-up, and increased at 5.5-year follow-up, whereas cervical scoliosis was not affected by the treatment. The sagittal profile was initially improved both in kyphosis and lordosis. However, at 5.5-year follow-up, hyperkyphosis had deteriorated beyond the initial deformity. Pelvic obliquity was significantly restored especially in neuromuscular patients, and increasing spinal length was achieved within the 5.5-year follow-up. Conclusion In children with spinal deformity, implantation of the VEPTR device sufficiently corrected the deformity in all planes. During long-term follow-up, scoliosis increased slightly and was rather well controlled, whereas the implant system was not able to prevent deterioration of hyperkyphosis. Pelvic obliquity was well balanced and spinal lengthening was achieved during long-term follow-up.

BACKGROUND Almost all children with spinal muscular atrophy (SMA) develop a scoliosis during childhood and adolescence. In the last decades, growth-friendly spinal implants have been established as an interim solution for these patients until definite spinal fusion can be performed. The effect of those implants on the final outcome has yet to be described. OBJECTIVE To assess the effect of prior growth-friendly spinal surgical treatment on the outcome after spinal fusion in SMA children in comparison to untreated SMA patients through the prospective study. METHODS A total of 28 SMA patients with (n = 14) and without (n = 14) prior surgical treatment with growth-friendly implants were included. Average surgical treatment prior to definite spinal fusion was 4.9 yr. Scoliotic curve angle, pelvic obliquity, spinal length, kyphosis, and lordosis were evaluated for children with prior treatment and before and after dorsal spondylodesis for all children. RESULTS The curve angle before definite spinal fusion averaged at 104° for SMA patients without prior treatment and 71° for patients with prior treatment. Spondylodesis reduced the scoliotic curve to 50° and 33°, respectively, which equals a correction of 52% vs 54%. Pelvic obliquity could be improved by spinal fusion in all patients with better results in the pretreated group. Results for spinal length, kyphosis, and lordosis were similar in both groups. CONCLUSION These data show the positive effect of prior growth-friendly surgical treatment on radiographic results of spinal fusion in children with SMA. Both scoliotic curve angles and pelvic obliquity showed significantly better values when patients had growth-friendly implants before definite spinal fusion.

Cohen syndrome (CS) is a rare autosomal recessive disorder associated with mutations in the vacuolar protein sorting 13 homolog B (VPS13B; formerly COH1) gene. The core clinical phenotype comprises a characteristic facial gestalt, marked developmental delay, and myopia. Additional, nonobligatory features include obesity, microcephaly, short stature, muscular hypotonia, scoliosis, narrow hands and feet, progressive retinopathy, as well as neutropenia. Here we report a novel homozygous nonsense mutation in the VPS13B gene and previously undescribed clinical features in a 19-year-old woman with developmental delay, intellectual disability, and a particular facial appearance. The patient showed several features consistent with CS. In addition, the parents observed congenital alacrima and anhidrosis persisting until onset of puberty. The diagnosis was not established based on the clinical phenotype. We performed whole-genome sequencing and identified a novel homozygous nonsense mutation c.62T>G (NM_152564.4), p.(Leu21*) in the VPS13B gene. Our findings extended the previously reported phenotype of CS. We conclude that transient, prepubertal alacrima and anhidrosis are part of the phenotypic spectrum of CS associated with a novel homozygous nonsense mutation in the VPS13B gene. peerReviewed

In pediatric flat foot a differentiation has to be made between the flexible and the rigid form. The diagnosis is based on the history, clinical examination as well as pedobarography, gait analysis and imaging techniques. It is important to rule out neuropediatric conditions such as muscular dystrophies, Ehlers-Danlos- or Marfan syndrome. In children six years of age and younger a flexible flat foot is nearly always physiological (97% of all 19 months old children). Up to the age of ten years the medial column of the foot is developing. Only a minority of children (4% in ten year olds) has a persistent or progressive deformity. Beyond to age of ten there is a danger of deformity decompensation as well as an increased rigidity. Only a minority of children develops some pain ( 2%). A clear risk factor for persistent pediatric flat foot is obesity (62% of six year old children with flat foot are obese). Pathogenetic factors include muscular, bony or soft tissue conditions. However, there specific rule is still unclear. Prevention consists in a thorough parent information about the normal development as well as encouragement of regular sportive activities. Soft and large enough shoes should be carried as a protection. Barfoot walking has to be encouraged on uneven grounds. If physiotherapy is needed different methods can be applied. Orthosis treatment should include a proprioceptive approach. Surgical interventions in children are rare. If surgical treatment is planned a detailed algorhythm should be used before utilizing one of the many different surgical methods.Der kindliche Knick-Senk-Fuß ist eine 3-dimensionale Fußfehlstellung mit vermehrter Valgusstellung der Ferse, Abflachung der medialen Längswölbung und Abduktion des Vorfußes. Man unterscheidet die flexible von der kontrakten (rigiden) Form. Die Diagnostik gliedert sich in Anamnese, klinische Untersuchung, apparative Diagnostik wie Pedobarografie und die Ganganalyse sowie bildgebende Verfahren. Wichtig ist die Einbeziehung in ein Therapieschema von eventuell vorliegenden pädiatrischen oder neuropädiatrischen Erkrankungen (z. B. Muskeldystrophien, Ehlers-Danlos- oder Marfan-Syndrom u. Ä.). Es zeigt sich, dass bei Kindern ≤ 6 Jahre der flexible Knick-Senk-Fuß fast ausschließlich physiologisch vorkommt (bei 97% aller 19 Monate alten Kinder). Bis zum Alter von 10 Jahren bildet sich die mediale Fußlängswölbung zunehmend aus. Bei einem kleinen Anteil (10-jährige Kinder 4%) persistiert der Knick-Senk-Fuß oder wird progredient. Oberhalb von 10 Jahren besteht die Gefahr der Dekompensation mit zunehmender Rigidität. Nur die Minderheit der Kinder entwickelt eine Schmerzsymptomatik ( 2%). Ein Risikofaktor zur Persistenz des Knick-Senk-Fußes stellt das Übergewicht im Kindesalter dar (62% der 6-Jährigen mit Knick-Senk-Fuß sind übergewichtig). Pathogenetische Faktoren können muskulär, knöchern und bindegewebig sind, wobei hier der Einfluss der unterschiedlichen Faktoren kontrovers diskutiert wird. Die Hauptprävention besteht in einer umfassenden Aufklärung über eine normale kindliche motorische Entwicklung. Daraus resultiert die Eigenförderung der physiologischen Entwicklung durch tägliche Bewegung, das Anstreben eines normalen Gewichtes, das Tragen von weichen Schuhen mit ausreichender Länge und Weite zum Schutz und das Barfußlaufen auf wechselnden Untergründen. Im Falle einer nötigen Intervention kann Physiotherapie inklusive manueller Techniken nötig werden. Eine Einlagen- oder Orthesenversorgung sollte bevorzugt mit einer sensomotorischen Bettung erfolgen. Operative Eingriffe sind selten. Im Falle einer geplanten operativen Intervention muss ein differenziertes Indikationsschema angewandt werden. Verschiedenste operative knöcherne und weichteilige Methoden stehen zur Verfügung.

Spine-based fixation of magnetically controlled lengthening devices has been successfully performed for children with early-onset scoliosis. However, spinal manipulation may lead to ossifications, stiffness, and autofusion as previously described. To avoid these problems, a surgical technique combining bilateral externally controlled magnetic device implantation with a rib cradle and pelvic hook fixation was introduced by us in 2011. By using a bilateral single-rib or double-rib cradle fixation and a pelvic hook, the magnetic device is percutaneously inserted. The spine corrects indirectly without further manipulation. In small rib diameter or severe osteoporosis, double-rib cradles are used. Our introduced technique enables modification of the rib vertebral angle, which may be beneficial in children with spinal muscular atrophy and rib-cage deformity. This nonrandomized prospective study describes 18 children with neuromuscular scoliosis treated first by this method. All patients followed the same protocol, with expansion procedures being performed 5 months after surgery and every 3 months thereafter. Clinical, radiologic, and complication data were analyzed, showing a significant reduction in scoliosis and pelvic obliquity and an increase in spinal length, which could be maintained over a follow-up period of 1.2 years. The overall complication rate was lower than previously described, with 8%, 2 of them requiring surgery.

Study design Case Series. Objective To describe the post-VEPTR (vertical expandable prosthetic titanium rib) treatment changes in early-onset spinal deformity (EOSD), which may influence the final correction spondylodesis. Summary of background data The VEPTR device, originally developed for the treatment of congenital rib cage malformation, is nowadays more widely used in the treatment of EOSD. At present, only a few reports describe the possible complications that may occur with repeated lengthening procedures of the VEPTR, thereby making the final spondylodesis more complicated and less satisfactory. Methods X-rays of 5 children treated for EOSD with 2 unilateral VEPTR (each rib to rib and rib to lumbar lamina) were analyzed for curve patterns and Cobb angles before, during, and at the end of VEPTR treatment, and after the final spondylodesis. Intraoperative observations during the spondylodesis, which influenced the possibilities of the curve correction, were documented. Results All patients showed a marked decompensation of the frontal balance and a high degree of rigidity of the main curve and the compensatory curves after treatment with the VEPTR device. Because of this spontaneous autofusion of spinal segments, migration of the rib cradles and/or the laminar hook, and a change in the curve patterns, the final fusion had to be longer in all patients than the primary deformity would have intended. Conclusions If an EOSD is treated with VEPTR, the curve progression and, in particular, the development of a high thoracic hyperkyphosis or rotation of the main curve should be critically observed. Autofusion of ribs and vertebral bodies may make the final correction spondylodesis even more challenging and risky for the patient and the end result less satisfactory.

Erectile dysfunction (ED) is often associated with increased cardiovascular risk. There is increasing evidence suggesting that dysfunction of the vascular endothelium with reduced bioavailability of nitric oxide (NO) may be the pathogenetic link between ED and cardiovascular disease. The crucial importance of the NO-guanylatecyclase-cGMP-phosphodiesterase pathway for penile erection is mirrored by the efficacy of phosphodiesterase-5 (PDE5) inhibitors in the treatment of ED. In contrast to other currently available PDE5 inhibitors with a half-life time of about 4 h Tadalafil has a half-life time of about 17.5 h resulting in erectile responsiveness for up to 36 h after 1 single dose. Most clinical experience has been reported with on-demand use of PDE-5 inhibitors, but meanwhile several studies were able to demonstrate that Tadalafil given daily in low (2.5 and 5 mg) doses is both highly effective and well-tolerated. In three randomized, double-blind, placebo-controlled multi-center trials, various validated measures of erectile function indicated that once daily Tadalafil at doses of 2.5, 5, and 10 mg was significantly superior to placebo.In another mono-center trial, once daily Tadalafil has shown significant efficacy even after failure of on-demand treatment. In a controlled cross-over study of on-demand versus daily Tadalafil treatment, 72% of the patients preferred once daily administration, mainly because of superior and longer efficacy allowing a more spontaneous sexual life. Interestingly in a pilot study of on-demand versus chronic administration of Tadalafil for 4 weeks, only regular dosing improved several markers of endothelial function.

Torsional deformity in the lower extremities is well known in children and adolescents with hemiplegic cerebral palsy, however, little or nothing is known about possible differences in torsional behavior of the upper extremities. Alterations of torsion in the lower extremities influence the gait, alterations in the rotation behavior of the upper extremities, for example after fractures, lead to limited movement of the arms or to an altered movement radius. The aim of this study was to investigate whether there is a difference in humeral torsion between affected and non-affected arms in children and adolescents with hemiplegic cerebral palsy and which factors play a decisive role.A total of 33 children and adolescents with hemiplegic cerebral palsy (mean age 9.7 years, range 3-16 years) answered a questionnaire and were examined clinically, photographically and with a new ultrasound method. Severity of cerebral palsy was classified into three levels. Ultrasound measurements of humeral torsion were compared with the results from a normal collective (n=149).In approximately half (n=16, 48.5%) of the 33 children investigated, humeral torsion was directed more internally, in 17 (51.5%) children humeral torsion was directed more externally compared to the healthy side. The difference in humeral torsion between the affected and non-affected sides was significant (p0.05) in both groups. Differences in humeral torsion between the affected and non-affected sides in children with hemiplegic cerebral palsy were significantly increased (p0.00001) compared to differences in humeral torsion between left and right arms in healthy children. A correlation with the level of severity of hemiplegic cerebral palsy was observed but there was no correlation with age. Children and adolescents with external torsional deformity had a smaller range of rotation, a larger muscle force deficit of the affected upper extremity and did less sport using the affected arm than children and adolescents with internal torsional deformity.Humeral torsion deformity in children and adolescents with hemiplegic cerebral palsy was proven to exist in addition to the already well known difference in torsion of the lower extremities. Analogous to increased activity of external or internal rotator muscles codetermined by the palsy, increased external or internal humeral torsion occurs. External torsion deformity was associated with a smaller range of motion and seemed to have a greater importance for routine daily activities (e.g. muscle force, sports ability) than internal torsion deformity.

Inter- and intraobserver reliability and learning curve using Graf's ultrasonographic hip examination were assessed.189 participants of the basic, advanced and final courses on hip ultrasound using the Graf method (DEGUM) were asked to answer a questionnaire and to measure 34 normal and pathological sonographic hip examinations. Measurement differences and the learning curve during the different courses were evaluated.There was a significant (p = 0.001) improvement of the average angle between the basic (6.74 degrees [+/- 1.46 degrees ]) and the advanced course (6.22 degrees [+/- 1.17 degrees ]). However, there were no better results in the final course and no additional improvement. Participants had higher variations when measuring the beta-angle than the alpha-angle. A significant improvement was seen if the participants performed a large number of hip ultrasounds between the courses. Higher measurement differences were seen in pathological hip ultrasounds and in ultrasonographic examinations of poorer quality.During the educational courses on hip ultrasound using the Graf method, a high intra- and interobserver variability was seen. These findings were most prominent in measurement of the beta-angle, in pathological hip ultrasounds and in ultrasonographic hip examinations of poorer quality. The best results were achieved by participants who had performed a large number of examinations between courses. For the improvement of ultrasonographic measurements, a large number of self-performed examinations and a training in potential mistakes seem to be of great importance.

Children with congenital thoracic scoliosis associated with fused ribs and unilateral unsegmented bars adjacent to convex hemivertebrae will inevitably develop thoracic insufficiency syndrome and curve progression with hemithorax compression without treatment. It is assumed that the concave side of such curves and their unilateral unsegmented bars do not grow. In the past early spinal fusion was performed with consecutive short thoracic spines and loss of lung volume. Little attention has been paid to lung function. These patients often suffered from lung failure and early death due to a small thorax.A new surgical technique is based on an indirect deformity correction and enlargement of the thorax due to a longitudinal implant, the vertical expandable prosthetic titanium rib (VEPTR). The spine is not fused, thus promoting growth of the spine, the thorax and the lungs. Elongation of the implant is done every six months. Since 2002 this method has been performed on fifteen children in Basel as the first European center.Patients (mean age 6 years; 11 months to 12 years) were suffering from thoracic insufficiency syndrome due to unilateral unsegmented bars with fused ribs (n = 4), absent ribs (n = 2), bilaterally fused ribs (n = 2), hemivertebrae (n = 3) or neuromuscular scoliosis (n = 6). Doing fifteen primarily implantations and thirteen elongations there were three complications (two hook dislocations, one skin breakage). All patients improved cosmetically, functionally and radiologically which was shown on X-rays as a reduction of the Cobb angle from an average of 76 degrees (40-110 degrees ) to 55 degrees (30-67 degrees ).Expansion thoracoplasty and VEPTR implantation is a new treatment concept for children with thoracic insufficiency syndrome due to spinal deformities, which is based on distraction and expansion of the thorax thus allowing growth of the spine, the thorax and probably lungs. Presently it seems to be superior to any other method for the treatment of small children with progressive scoliosis and thoracic insufficiency syndrome.

Expansion thoracoplasty and vertical expandable prosthetic titanium rib (VEPTR; Synthes Spine Co., West Chester, Pennsylvania, USA) implantation is a new method for the treatment of thoracic insufficiency syndrome and congenital spinal deformity in children. The longitudinal rib implant expands the thorax and indirectly corrects spinal deformity, thus allowing spinal, thoracic and probably lung growth. VEPTR has been used since 1989 in San Antonio, USA, and was introduced to Europe in 2002. This paper describes the preliminary experience with the European patients. Fifteen children with progressive scoliosis had a VEPTR implantation at a mean age of 6 years (11 months to 12 years). Nine children had thoracic insufficiency syndrome due to unilateral unsegmented bars (n = 4), absent ribs (n = 1), hemivertebrae (n = 2) or bilateral fused ribs (n = 2). Six children had severe thoracolumbar scoliosis and pelvic obliquity due to neuromuscular scoliosis. After VEPTR implantation, families and patients reported improvement of the thoracic insufficiency syndrome and better sitting abilities in the neuromuscular patients, as well as radical cosmetic improvement. There were three complications (skin breakage, lumbar hook displacement, rib fracture) after performing fifteen primary VEPTR implantations and 13 expansion surgeries in eight patients. Our experience suggests that expansion thoracoplasty and VEPTR implantation is a safe and efficient method for the treatment of thoracic insufficiency syndrome in young children with severe scoliosis.

We compared the electromyographic (EMG) signals of lower extremity muscle groups in 10 children with hemiplegic cerebral palsy (CP) while walking barefoot and in a hinged ankle-foot orthosis (HAFO). All children had excessive plantarflexion and initial toe-contact on the affected side when walking barefoot, a typical gait pattern for hemiplegic patients. The patients walked with a physiological heel-toe gait pattern when wearing the HAFO. The peak activity of the tibialis anterior muscle was reduced by 36.1% at initial contact and loading response phase and by 57.3% just after toe-off when using a HAFO. The decrease in activity was thought to result from the change in gait pattern from a toe-gait to a heel-toe gait as well as the use of a HAFO. The HAFO also slightly decreased muscle activity in the proximal leg muscles mainly during swing phase, improved stride length, decreased cadence, improved walking speed, increased peak hip flexion, improved kinematics in loading response phase at the knee, and reduced the excessive ankle plantarflexion.

We reviewed 15 patients, nine girls and six boys, with chronic anterior dislocation of the radial head which was treated by ulnar osteotomy, external fixation and open reconstruction of the elbow joint but without repair of the annular ligament. Their mean age was 9.5 years (5 to 15) and the mean interval between the injury and reconstruction was 22 months (2 months to 7 years). All radial heads remained reduced at a mean follow-up of 20 months (6 months to 5 years). Normal ranges of movement for flexion, extension, pronation and supination were unchanged in 96.1% (49/51) and worse in 3.9% (2/51). Limited ranges of movement were improved in 77.8% (7/9), unchanged in 11% (1/9) and further decreased in 11% (1/9).There were two superficial pin-track infections and two cases of delayed union but with no serious complications. Reconstruction of the radiocapitellar joint is easier using external fixation since accurate correction of the ulna can be determined empirically and active functional exercises started immediately. Only patients with a radial head of normal shape were selected for treatment by this method.

Aflatoxins are a family of fungal toxins that are carcinogenic to man and cause immunosuppression, cancer and growth reduction in animals. We conducted a cross-sectional study among 480 children (age 9 months to 5 years) across 4 agro-ecological zones (SS, NGS, SGS and CS) in Benin and Togo to identify the effect of aflatoxin exposure on child growth and assess the pattern of exposure. Prior reports on this study [Gong, Y.Y.,Cardwell, K., Hounsa, A., Egal, S., Turner, Hall, A.J., Wild, C.P., 2002. Dietary aflatoxin exposure and impaired growth in young children from Benin and Togo: cross sectional study. British Medical Journal 325, 20-21, Gong, Y.Y., Egal, S., Hounsa, A., Turner, P.C., Hall, A.J., Cardwell, K., Wild, C.P., 2003. Determinants of aflatoxin exposure in young children from Benin and Togo, West Africa: the critical role of weaning and weaning foods. International Journal of Epidemiology, 32, 556-562] showed that aflatoxin exposure among these children is widespread (99%) and that growth faltering is associated with high blood aflatoxin-albumin adducts (AF-alb adducts), a measure of recent past exposure. The present report demonstrates that consumption of maize is an important source of aflatoxin exposure for the survey population. Higher AF-alb adducts were correlated with higher A. flavus (CFU) infestation of maize (p=0.006), higher aflatoxin contamination (ppb) of maize (p

The purpose of the study was to evaluate the prevalence of foot pain and foot deformities in adolescents. It was aimed to obtain information on the association between foot pain and foot deformities.A total of 2 368 adolescents (age 14.5 +/- 0.7 years) were evaluated. The frequency of foot pain was probed by using a self-reporting questionnaire. The foot deformities were evaluated during clinical examinations by school doctors.The prevalence of foot pain was 14.0 % and the prevalence of foot deformities was 13.7 %. The prevalence of pain was significantly higher in adolescents with foot deformity (17.8 %) than in persons without deformity (13.4 %), p0.05. The prevalence of a flexible flat foot was 6.2 % and the prevalence of a rigid flat foot was 0.5 %. Other deformities registered were splay foot (2.3 %) and flexible splay-flat foot (2.0). The prevalence of hallux-valgus deformity was 3.5 %. A total of 3.5 % patients were suffering from a plantar hyperkeratosis. This was significantly correlated to a high pain prevalence (the hyperkeratosis was significantly associated with a high prevalence of pain). Significant factors which were significantly associated with foot pain were foot deformity (1.4 fold) and hyperkeratosis (75 fold). Foot pain was 1.4 fold higher in children with foot deformity and 75 fold higher in feet with hyperkeratosis.The prevalence of foot pain and foot deformity in adolescent is high. Mild deformities (flexible flat foot and splay foot) are physiological variations without any association to foot pain. Pathological conditions that are associated with foot pain are the rigid flat foot, the hallux valgus and the cavus deformity. Plantar hyperkeratosis is an indicator of foot pathology.

Children with congenital thoracic scoliosis associated with fused ribs and unilateral unsegmented bars adjacent to convex hemivertebrae will invariably have curve progression without treatment. It is assumed that the unilateral unsegmented bars do not grow and therefore early spinal fusion has been performed in the past with consecutive short thoracic spines and loss of lung volume. A new surgical technique is based on an indirect deformity correction and enlargement of the thorax via a longitudinal implant, the vertical expandable prosthetic titanium rib (VEPTR). The length of the thoracic spine after VEPTR implantation was analyzed in 21 children with an average follow-up of 4.2 years. Eighteen children did not have any prior surgery while three had already undergone spinal fusion. Using a 3-D analysis of computed tomography, a significant growth of the concave (7.9 mm/7.1% increase per year) and convex (8.3 mm/6.4%) side of the thoracic spine was found with no significant difference between sides. Unilateral unsegmented bars showed significant growth as well. Contrary to common knowledge, we were able to demonstrate the growth of the concave side of the thoracic spine and of the unilateral unsegmented bars after expansion thoracoplasty and VEPTR implantation.

Ultrasound investigation of the hip according to Graf is performed, whenever possible, as a routine screening test for hip dysplasia and dislocation in neonates. However, in spite of screening, hip dysplasia and/or dislocation is identified in a number of children after the third month of life only. The present study presents an analysis of reasons and causes. Between August 1999 and July 2001 children aged between six months and five years were documented, in whom the diagnosis of hip dysplasia or dislocation was made, despite normal ultrasound findings at primary investigation and who required non operative or operative treatment by a specialist surgeon working in Switzerland in paediatric orthopaedics. The study included 26 children (17 girls / 9 boys). Hip dysplasia and/or dislocation was diagnosed between the age of 6 days and 41 months. Twelve children had to be excluded from the sample (n = 26), four were infants who had basic neurological disorders or multiple anomalies and there were eight children from abroad whose original documentation could not be obtained. Analysis of the remaining 14 children showed that 43% were misdiagnosed (n = 6) and a treatment error occurred in 36% (n = 5) of cases. One child presented with a teratogenic hip dislocation. In two other infants with normal primary ultrasound findings, hip dysplasia was identified radiographically once the child started to walk. Delayed diagnosis can be the result of technical errors or misinterpretation. The six patients meeting the criteria of misinterpretation can be expressed as a rate of 0.04% of all births. However, even if the disorder is correctly diagnosed, its identification and treatment may be inadequate or fail to produce the desired results. This was the case for five of the children, that is, for a rate of 0.03% of births.

Background: Children with congenital thoracic scoliosis associated with fused ribs with a unilateral unsegmented bar adjacent to convex hemivertebrae will invariably have curve progression without treatment. Surgery has been thought to have a negligible growth-inhibition effect on the thoracic spine in such patients because it has been assumed that the concave side of the curve and the unilateral unsegmented bar do not grow, but we are unaware of any conclusive studies regarding this assumption. Methods: The changes in the length of the concave and convex sides, anterior and posterior vertebral edges, posterior arch, and unilateral unsegmented bars of the thoracic spine were measured in the twenty-one children with congenital scoliosis and fused ribs after expansion thoracoplasty had been carried out with use of a vertical, expandable titanium prosthetic rib. Three of these children had undergone posterior spinal fusion previously. Measurements were made with use of a three-dimensional software program that analyzed baseline and follow-up computed tomography scans. The technique was validated through measurement of the thorax of a small female adult cadaver. Results: The patients without spine fusion had an average age of 3.3 years at the time of the baseline computed tomography scan, and the average duration of follow-up was 4.2 years. On the average, these patients showed significant growth (p < 0.0001) of the concave side of the thoracic spine (an increase in length of 7.9 mm/yr, or 7.1%/yr) and the convex side (8.3 mm/yr, or 6.4%/yr) compared with the baseline lengths. There was no significant difference in the increases in length (p = 0.38) between the concave and convex sides. Eleven patients with an unsegmented bar had an average 7.3% increase in the length of the bar (p < 0.0001). In the three children with prior spinal fusion, the increase in length averaged only 4.6 mm/yr (3%/yr) on the concave side of the thoracic spine and 3.7 mm/yr (2.2%/yr) on the convex side; both increases were significant (p < 0.0001). Conclusions: Longitudinal growth of the thoracic spine in a normal child has been estimated to be 0.6 cm/yr between the ages of five and nine years. After expansion thoracoplasty, growth of the thoracic spine was approximately 8 mm/yr in our series of children with congenital scoliosis and fused ribs. After expansion thoracoplasty, both the concave and the convex side of the thoracic spine and unilateral unsegmented bars appeared to grow in these patients. When a thorax is already foreshortened by congenital scoliosis, control of spine deformity with expansion thoracoplasty allows growth of the thoracic spine, and it is likely that the longer thorax provides additional volume for growth of the underlying lungs with probable clinical benefit. Level of Evidence: Therapeutic study, Level IV (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence.

A subcutaneous, T-phenotypic anaplastic large cell lymphoma (CD30/Ki1-positive, EBV positive) was diagnosed in a HIV-infected bisexual man. Without chemotherapy the patient had a sustained long-term remission of this tumor (more than three years) after the initiation of highly active antiretroviral therapy. By PCR analysis of T-cell receptor beta gene rearrangements the tumor was found to be oligoclonal. Improvement of cellular immune function by antiretroviral therapy is the only recognizable factor which may have led to tumor remission. This hypothesis is supported by parallels to EBV associated polyclonal lymphoproliferation in allogeneic transplantat recipients where regression of lymphoma can be induced by reducing immunosuppressive therapy.

The effectiveness of antimicrobial prophylaxis was evaluated on the basis of data collected in a study on quality management carried out in 28 East German hospitals, involving 4477 laparoscopic and conventional cholecystectomies (197 of which with revision of the common bile duct). In 3128 patients a laparoscopic procedure (with consecutive conversion to an open cholecystectomy in 236 cases) and in 1349 patients a primarily conventional open cholecystectomy had been performed (a total of 2217 cases with and 2260 cases without antibiotic cover). The results obtained were significantly better in the group receiving prophylaxis than in patients not under antimicrobial cover. This applied to septic wound healing disorders, general and specific postoperative complications, postoperative chest infections, re-operations and postoperative lethality. On the basis of these results, it is strongly recommended that, in the future, neither laparoscopic nor open conventional cholecystectomy should be carried out without proper perioperative antimicrobial prophylaxis-this all the more so since such measures also result in a shorter hospital stay and thus reduced costs.

Seven cases of large cell lymphoma (LCL) developing simultaneously or secondarily to lymphocyte-predominant Hodgkin's disease (nodular paragranuloma [NP]) were investigated for the presence of Epstein-Barr virus genomic material and the chromosomal translocation t(14;18) involving the major breakpoint region of the bcl-2 gene using the polymerase chain reaction on paraffin-embedded material. The translocation t(14;18) and Epstein-Barr virus could not be demonstrated in any case. The expression of the bcl-2 oncogene product was investigated using immunohistochemistry. Only in one case were the lymphocytic and histiocytic cells positively stained with the bcl-2 antibody, whereas the associated LCL demonstrated a completely negative immunoreaction. In another case the LCL had a positive immunoreaction with this antibody and the corresponding lymphocytic and histiocytic cells were completely negative. We conclude that Epstein-Barr virus infection is rare or absent in NP and in the LCLs associated with NP. The chromosomal translocation t(14;18) does not seem to be a factor in the transformation of NP into LCL.

Diurnal fluctuations in vision are common after radial keratotomy (RK), associated with a myopic change in refraction and keratometric power mostly in the morning. After photorefractive keratectomy (PRK), only a small percentage of patients report fluctuations of vision. We tested ten such patients who had undergone excimer laser PRK with regard to refraction, keratometry, and visual acuity 5 to 20 months after surgery. Patients were examined five times during one day from 8:00 a.m. to 8:00 p.m. Ten volunteers serving as a control group were examined following the same protocol. None of the patients showed an increase in minus spherical power of the manifest refraction between morning and evening. The change in manifest refraction from morning to evening was +0.3 D +/- 0.3 D, which was not significantly different from the control group. The change in refraction did not correlate with keratometric power, follow-up time of age of the patients.

We have investigated seven cases of large cell lymphomas (LCL) developing simultaneously or metachronously to nodular lymphocyte-predominant Hodgkin's disease (nodular paragranuloma, NP) for the presence of EBV and the chromosomal translocation t(14;18) by use of the polymerase chain reaction (PCR). The expression of the bcl-2 oncogene product in these cases and in five cases of progressive transformation of germinal centres as a potential precursor of NP was detected immunohistochemically with the monoclonal antibodies bcl-2-100 and bcl-2-124. All cases investigated were negative for EBV genomic material. The chromosomal translocation t(14;18) was also absent. Expression of the bcl-2 oncogene could be detected only in one case of nodular paragranuloma and in an unrelated case of LCL. Hence, LCL developing out of NP differ from other germinal center derived high-grade lymphomas.

Prolactin secretion is affected by various diseases as well as by many drugs in humans and animals. While marked hyperprolactinaemia suggests the presence of a pituitary tumor, moderate changes may also occur in various endocrine or non-endocrine disorders. Drugs can interfere with prolactin regulation via complex mechanisms at the hypothalamus or at the pituitary site, but possible changes in prolactin metabolism are poorly understood as yet. This survey of the literature up to June 1986 covers the influence of various groups of drugs and agents on the plasma prolactin level under various conditions. It contains information that will facilitate evaluation of whether hyper- or hypoprolactinaemia may result from therapeutic intervention or must be related to an underlying disease. It is obvious that more subtle changes can be revealed by the use of dynamic tests either to stimulate or to suppress prolactin secretion.

A primary resection and reanastomosis of the colon is often the procedure of choice, even in the presence of a significant stenotic element, provided there are no mayor disturbances of water-electrolyte homeostasis. In a series of 100 consecutive colonic resections (23 complicated by perforation, 9 by ileus, and 2 by bleeding), 97 patients were treated by primary resection (92 without a protective colostomy). No untoward effects were noted specifically due to this technique. The overall mortality rate was 4%, a favorable result when compared with the cumulative risk of a multiple stage procedure.

A case of discrete, localised Morbus Ménétrier is reported, with pitting of a giant mucosal fold at the antrum, where the patients' main symptom was recurrent gastric haemorrhage. The presentation posed considerable diagnostic difficulties that were only clarified through a partial gastric resection and subsequent histological examination.

Aetiology of colonic perforation is reviewed and discussed. 8 cases of "spontaneous" ileo-colic perforations observed between 1975 and 1977 are presented. Two of these patients had recently undergone appendectomy, and 4 others showed a simultaneous distal carcinoma of the large bowel. Histo-pathological evaluation did not reveal the cause of intestinal perforation. Operative treatment and results are given.

A prospective study concerning 100 consecutive cases of colonic resections and anastomosis with single layer sutures is reported where radiological controls 10-12 days postoperatively had been carried out. An anastomotic insufficency was recognized clinically in 15%; in 6% a small leakage was only detectable radiologically. 50% of all anastomotic deficencies were seen in cases with preexisting perforation peritonitis or ileus, especially if an emergency resection had been necessary due to septic complications. 3 out of the 7 deaths were connected with dehiscence in the suture line.

Small intestine perforations caused by systemic disorders are, except with M. Crohn, extremely rare. Therefore we report two cases of "spontaneous" small intestine perforations: one with hypersensitivity angiitis and one with Moya-Moya-disease, the latter not yet described in the literature.

100 consecutive colonic resections for carcinoma are reported. The average age was 67.5 +/- 10.8 years. In 93% of all cases primary resection without colostomy was carried out. The overall operative mortality rate was 1% and the mean hospitalization period 19.8 +/- 7.3 days.

Two series of 50 consecutive elective colonic resections with bowel preparation either with Neomycin (group A) or Neomycin combined with Metronidazole (group B) are presented. 17 bacterial inflammatory complications in group A versus 10 in group B were noted. It is concluded that the addition of an antianaerobic antibiotic agent in elective colonic surgery might be of beneficial effect.