Surgical treatment of neuromuscular scoliosis: current techniques.

41 consecutive patients surgically treated at Our Department by posterior only instrumented fusion from January 1995 to January 2009 were reviewed. There were 20 females and 21 males with a mean age of 15.8 years (range, 10 to 38). Diagnosis was: cerebral palsy (13 cases), Duchenne muscular dystrophy (7), spinal amyotrophy (7), myelomeningocele (5), poliomyelitis (3), Friedreich's ataxia (2), Escobar syndrome (2), Steinert's disease (1), Charcot Marie Tooth disease (1). Main scoliosis Cobb angle averaged 94.05° (range, 34° to 165°), the curve was thoracic in 19 cases, thoracolumbar or lumbar in 22 cases. Kyphosis (T5-T12) averaged 42.86° (range, 7° to 90°), lordosis was 33.57°. The fusion was extended to the lumbar tract in 23 patients, to the sacrum in the other 18. Our results showed that, in patients with neuromuscular scoliosis, posterior instrumented fusion is a safe and effective procedure and is the treatment of choice for patients with limited respiratory function, as in Duchenne muscular dystrophy and spinal muscular atrophy. The surgery should be performed as early as possible, and the extension of the fusion to the sacrum should be avoided in patients with residual walking ability.

Surgical treatment for scoliosis associated with rare disease.

This is a retrospective study of 11 patients, 7 females and 4 males, treated at Our Department for an early onset scoliosis (EOS) associated with rare syndromes with growing spinal implants (Growing Rod or VEPTR-like) from 2006 to 2011. Mean follow-up was 24 months (range, 12 to 36). The mean age at surgery was 7. Patients were affected by Escobar's syndrome (1), scoliosis associated to congenital heart disease (1), Arnold Chiari type 1 (1), syringomyelia (1), NF 1 (2), Prader-Willi syndrome (1), trisomy 8 (1), arthrogryposis (2) and spondylo-rib dysplasia (1). Each patient was studied from the genetic point of view, and were performed: brain-spine MRI, pulmonary function tests, Cardio-US and abdominal US, neuropsychiatric and neurological evaluation, C0-C2 CT scan. After first implant and lengthening procedures (11), the correction of the thoracic curve averaged 50%. Unfortunately, a little loss of correction of the lumbar curve occurred during the follow up. There were 8 post-operative complications, that required revision surgery in 2 cases. Our results confirmed the effectiveness and safety of growing spinal implants in the treatment of early-onset scoliosis in rare syndromes.

Complications incidence in the treatment of early onset scoliosis with growing spinal implants.

Early onset scoliosis (EOS) surgery based on growing spinal implants can lead to several complications. Aim of the study was to identify strategies to prevent those complications. A retrospective review was conducted to identify all pediatric patients affected by EOS surgically treated with growing rod or Vertical Expandable Prosthetic Titanium Rib (VEPTR) at our division between 2006 and 2011. Nineteen consecutive patients (8 males, 11 females; mean age 6.8 years) were included. The scoliosis was: idiopathic in 7 cases, congenital in 5, associated with congenital heart disease in 2, with syringomyelia and Arnold Chiari syndrome in 1, with neurofibromatosis type 1 (NF1) in 1, with Prader Willi syndrome in 1, with trisomy 8 in 1, with arthrogryposis in 1. Instrumentation used was: growing rod in 9 patients (dual rod construct in 8 cases, single rod in 1), VEPTR in 10 (always rib to spine construct). At a mean follow-up of 28 months (range, 12 to 55) 12 mechanical complications occurred in 8 of 19 patients treated (42.1%). Among cases treated with growing rod (9) 6 complications occurred in 4 patients (44.4%): revision was performed in 4 cases due to proximal anchors migration, in 2 cases due to a rod breakage. Among cases treated with VEPTR (10) 6 complications occurred in 4 patients (40%): revision was performed in 4 cases due to rib fracture with anchors migration, in 1 case due to vertebral anchor migration and in 1 case due to proximal and distal anchor migration. So, in our series mechanical complications rate was 42.1%. Our strategy to prevent these complications is to use hooks as proximal anchors, to avoid single rod construct and to use a brace as external support until final surgery is performed. If it's possible, is better to substitute VEPTR with a dual Growing Rod implant when patient's age and anatomy permits this.

[Promoting the quality of health surveillance of workers exposed to wood dust, with particular care to NPSC, in the territory of the Health Agency of Florence]. / Promozione della qualità della sorveglianza sanitaria dei lavoratori esposti a polvere di legno, con particolare riferimento ai TuNS, nel territorio di competenza della Azienda Sanitoria di Firenze.

Wood dust can cause occupational-related naso-sinusal cancer, characterized by a latency period of about 40 years. The Tuscany Cancer Registry estimates that cases of NPSC are from 20-25 per year into the Region (33% related to wood dust). These neoplasms are surgically treatable at early-stage and, for this reason, a rapid endoscopic diagnosis is considered to be reasonably useful for prognostic issues. We used a questionnaire to investigate nasal symptoms and NOSQ and SOLAR questionnaires to highlight respiratory/skins diseases, and a spirometry for each worker. Subjects with a working-age of more than 15 years, and those that were positive to the questionnaire and/or to the medical history were were referred to a specialist in otolaryngology. The prevalence of endoscopic positive findings--detected especially in subjects with a working age of more than 15 years--confirms the significance of the problem.

The prevention of neural complications in the surgical treatment of scoliosis: the role of the neurophysiological intraoperative monitoring.

Iatrogenic spinal cord injury is the most feared complication of scoliosis surgery. The importance of combined somatosensory evoked potentials (SEP) and motor evoked potentials (MEP) monitoring during spine surgery is well known. The current authors retrospectively evaluated the results of neurophysiological intraoperative monitoring (IOM) in a large population of patients who underwent surgical treatment for spinal deformity. Intraoperative monitoring of SEPs and transcranial electrical stimulation MEPs (TES-MEP) was performed in 172 successive patients who underwent surgical treatment of idiopathic (128 pts), congenital (15 pts) or syndromic (29 pts) scoliosis. The first 106 patients (Group 1) underwent only SEP monitoring, while the other 66 patients (Group 2) underwent combined SEP and TES-MEP monitoring, when the technique was introduced in the current authors' institution. Halogenate anaesthesia (Sevoflurane, MAC 0.6-1.2) was performed in Group 1 cases, total intravenous anaesthesia (Propofol infusion, 6-10 mg/kg/h) in Group 2 patients. A neurophysiological "alert" was defined as a reduction in amplitude (unilateral or bilateral) of at least 50% for SEPs and of 65% for TES-MEPs compared with baseline. In Group 1, two patients (1.9%) developed postoperative neurologic deficits following surgical correction of spinal deformity, consisting of permanent paraparesis in one case and transient paraparesis secondary to spinal cord ischaemia in the other. Twelve patients presented intraoperative significant changes of neurophysiological parameters that improved following corrective actions by surgeons and anaesthesiologists, and did not show any postoperative neurologic deficits. In ten cases the alert was apparently unrelated to surgical manoeuvres or to pharmacological interventions and no postoperative neurologic deficits were noted. Considering the patients of Group 2, two patients (3.0%) presented transient postoperative neurologic deficits preceded by significant intraoperative changes in SEPs and TES-MEPs. In five cases a transient reduction in the amplitudes of SEPs (1 patient) and/or TES-MEPs (5 patients) was recorded intraoperatively with no postoperative neurologic deficits. In conclusion, in the current series of 172 patients the overall prevalence of postoperative neurologic deficit was 2.3% (4 patients). When combined SEP and TES-MEP monitoring was performed, the sensitivity and specificity of IOM for sensory-motor impairment was 100 and 98%, respectively. Combined SEP and TES-MEP monitoring must be regarded as the neurophysiological standard for intraoperative detection of emerging spinal cord injury during corrective spinal deformity surgery. Early detection affords the surgical team an opportunity to perform rapid intervention to prevent injury progression or possibly to reverse impending neurologic sequelae.

Thoracoplasty in the surgical treatment of adolescent idiopathic scoliosis.

A consecutive series of 40 adolescents surgically treated between 1998-2001, by posterior spinal fusion and thoracoplasty were compared with a similar group of 40 adolescents treated in the same period by posterior only segmental fusion. Clinical and radiographic analysis was performed, including the SRS-30 questionnaire and Pulmonary Function Tests (PFT). Minimum five years follow-up was requested. No statistical differences were found between the two groups in PFT's both pre-operatively and at latest follow up. Our findings suggest that thoracoplasty did not adversely affected long-term PFT's in AIS patients treated by posterior spinal fusion alone.

An innovative diagnostic procedure of vertebral deformities without exposure to X-rays.

The objective of the study was to compare standard manual X-ray measurements of vertebral deformities and values obtained from the Ortelius 800. 52 Patients (41 females and 11 males: mean age 20.35 years) with adolescent vertebral deformities, was studied. The patients were evaluated with standard radiographic views and the Ortelius 800. The parameters considered for the comparison were the angles of scoliosis and kyphosis and the values of global axial deformity, shoulder asymmetry and pelvic tilt. We also evaluated the modification of pelvic/shoulder angle after surgery (this parameter allows to evaluate vertebral rotation and can be derived only from the Ortelius 800). This study allowed us to conclude that there is a perfect agreement between measurements with the Ortelius 800 and those resulting from standard x-rays. The system has also proved to have the capability of quantifying changes in vertebral rotation.

[Nutritional and Functional assessment of peritoneal dialysis patients in the clinical practice: Report from MITO-DP Group]. / Valutazione Nutrizionale e Funzionale dei pazienti in dialisi peritoneale nella pratica clinica: l'esperienza del Gruppo Medico-Infermieristico Toscano di Dialisi Peritoneale (M.I.TO.-DP).

Nutritional abnormalities and physical inactivity are risk factors of increased morbidity and mortality in patients with ESRD. Identify and define malnutrition, in particular protein-energy depletion (PEW), is an important task in the management of renal patients. The aim of this multicenter observational study was to implement the assessment of nutritional status and functional capacity in patients on peritoneal dialysis, including tests and validated methods which are relatively easy to apply in daily clinical practice. The study includes all the 133 prevalent patients (80 m, 53 f, age 65 14 years), in peritoneal dialysis treatment (vintage 26 19 months) in 9 centers in Tuscany. We performed anthropometry, bioimpedance (BIA), clinical biochemistry, evaluation of habitual physical activity (RAPA tests) and performance (Sit-To-Stand test), appetite-evaluation questionnaire, and indices including the Malnutrition Inflammation Score (MIS), Geriatric Nutrition Risk Index (GNRI), Charlson comorbidity index, Barthel and Karnowsky index. The latter showed a condition of dependence in 7.2% and 19.7% of cases, respectively. Poor appetite was recorded in 48.2%. The majority of patients fell within the overweight / obesity range (51%) with waist circumference values associated with increased cardiovascular risk in 51% of males and 60% of females. At the BIA analysis, a BCMI <8 kg/m2 was detected in 39% of patients; an estimated protein intake <1.0 g / kg/d was found in 59% of cases; 34% of patients had serum albumin <3.5 g / dl; control of acidosis was good (bicarbonate 25.4 3.8 mM) but hyperphosphatemia was present in 64.6% of patients. A condition of sedentary or light physical activity was reported by 65.1% of patients, vigorous activity only by 11.9%. The 86.5% of patients able to perform the Sit-to-stand test reported a lower than the reference values for age and sex. A diagnosis of PEW was possible in 8% of our series, while a MIS score> 11, indicative of PEW, took place in 12.7% of cases. The values of the MIS correlated directly with age and the degree of comorbidity and inversely with the sit-to-stand test, RAPA tests and appetite level. The data in this study show that single tests indicative of malnutrition disorders are frequent to be found in our series of peritoneal dialysis patients. However, a diagnosis of PEW is quite infrequent. A large percentage of patients are overweight with increased abdominal adiposity, and reduced cell mass and protein intake below recommended levels; the level of habitual physical activity is low, and the level of physical capability is scarce. Therefore it is conceivable a nutritional counseling intervention to increase the intake of proteins, limiting the phosphorus and (when indicated) energy intake and to stimulating spontaneous physical activity or arranging assisted programs for functional rehabilitation. Close monitoring of the nutritional status and implementation of programs of adapted physical activity should have a prominent role in the clinical management of patients on peritoneal dialysis.

Reduction of beta-endorphin levels in the amniotic fluid of heroin addicts.

Plasma levels of beta-endorphin and beta-lipotropin in 18 heroin addicts were found to be similar to those in pregnant control subjects at all stages of gestation. In contrast, amniotic fluid levels of beta-endorphin were significantly lower in heroin abusers than in controls in both the first and second trimesters. Amniotic fluid levels of beta-lipotropin were reduced only in the second trimester. At term, when the patients were almost drug free, the amniotic fluid levels of both peptides were in the control range. These data support the existence of a specific control mechanism for amniotic fluid levels of beta-endorphin and beta-lipotropin, independent of the maternal pituitary, and show that exogenous opiates interfere with it negatively.

Identification and characterization of functional angiotensin II receptors in human neuroblastoma cells.

The presence of specific AII receptors in 6 different human neuroblastoma cell lines was investigated using binding, cAMP and [Ca2+]i studies. We found high affinity (0.1 nM), low capacity ((1-2).10(3) sites/cell) binding sites for [125I](Sar-1,Ile-8)AII in one half of the cell lines studied. In the positive cell lines mathematical modeling of multiple competition curves among AII and analogs strongly indicated the presence of a homogenous class of sites, i.e., AT1 receptors. The presence of AT1 receptors was further substantiated by AII-induced inhibition of VIP-stimulated cAMP levels and by AII-evoked [Ca2+]i transient. The density of AT1 receptors in neuroblastoma cells was not affected by treatment with pertussis toxin and retinoic acid but was significantly increased by subacute treatment with VIP. In neuroblastoma cells, AII does not stimulate DNA synthesis, suggesting other roles rather than mitogenesis. Neuroblastoma cells represents an interesting model to investigate the function of AII in neural crest derived tissues.

Hypothalamus-pituitary-adrenal axis of heroin addicts.

The hypothalamus-pituitary-adrenal axis of heroin addicts was investigated by evaluating plasma cortisol levels throughout the day in 37 heroin abusers (HA), 17 of whom showed detectable morphine levels, indicating heroin administration in the previous hours and in 12 controls. All HA showed lower cortisol levels in basal condition (100.7 +/- 61.7 ng/ml, M +/- S.D.) compared to the control group (159.7 +/- 40.6, P less than 0.05). Moreover all HA (65.1 +/- 28.9%), and in particular those taking heroin during the study (39.1 +/- 41.4%), show a reduced cortisol decrease in the evening, which was significantly lower than in controls (85.3 +/- 10.1%; P less than 0.01). As far as the acute effects of opiates are concerned morphine (0.1 mg/kg) significantly reduced plasma cortisol levels within 60 min in a group of 8 healthy subjects. The same pattern was displayed by only 3/8 HA whose morphine levels were higher than 1 ng/ml, i.e. in those reaching the highest plasma morphine concentrations. These data indicate that chronic opiate abuse leads to a hypoadrenalism which could be the result of morphine-induced changes at the hypothalamic level.

Atrial natriuretic peptide and hemodynamic changes after concentrated ascitic fluid reinfusion in cirrhotic patients.

In order to assess the role, if any, of atrial natriuretic peptide (ANP) and hemodynamic changes in diuretic response to concentrated ascitic fluid reinfusion (CAFR) in cirrhotic patients, we studied 10 patients with liver cirrhosis before and after aspiration, concentration and intravenous reinfusion of their ascitic fluid. Basal ANP levels were higher than in normal subjects, but not related to the degree of water and sodium excretion. CAFR induced a variable degree of ANP changes and diuretic response, and these two parameters were strictly correlated to each other. No major changes of cardiac output and systemic vascular resistances were observed after CAFR. In conclusion, the apparent resistance to ANP diuretic action observed in basal condition in cirrhotic patients seems to revert after expanding blood volume by CAFR.

Antagonists for the human oxytocin receptor: an in vitro study.

The oxytocin antagonist [Mpa1, D-Tyr(Et)2, Thr4, Orn8]-oxytocin has been successfully used for treating premature labour. The interactions of this antagonist with neurohypophysialhormone receptors in the human myometrium were investigated. Competition curves among [3H]oxytocin, [3H]arginine vasopressin, [3H][1-(beta-mercapto-beta,beta-cyclopentamethylenepropionic acid)2-(O-methyl)-tyrosine, 8-arginine] vasopressin, the corresponding unlabelled peptides and a series of oxytocin antagonists including [Mpa1,D-Tyr(Et)2,Thr4,Orn8]-oxytocin were constructed from results taken from the myometrium of pregnant women and rabbits, and were analysed simultaneously using the computer program LIGAND. The biological activity of [Mpa1,D-Tyr(Et)2,Thr4,Orn8]-oxytocin in the human uterus was investigated by studying its effect on oxytocin-induced intracellular Ca2+ mobilization in human myometrial cells in culture that were expressing high concentrations of oxytocin receptors. The results indicate that [Mpa1,D-Tyr(Et)2,Thr4,Orn8]-oxytocin and related antagonists are selective for the oxytocin receptor in the myometrium of pregnant rabbits but not of pregnant women. In women, they bind with high affinity to the V1 vasopressin receptor. In myometrial cells [Mpa1,D-Tyr(Et)2,Thr4,Orn8]- oxytocin inhibits the oxytocin-induced increase in intracellular Ca2+ concentration in a dose-dependent fashion, with an IC50 value of 5 nmol l-1. The uterine relaxant effect of this antagonist might result not only from the block of the oxytocin receptor, but also from interaction with the V1 vasopressin receptor.