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The Chicago Classification is the most used classification for primary esophageal motility disorders (PEMD). This classification was recently updated to the 4.0 version. This opinion piece focuses on the possible implications for the treatment of PMED determined by the new classification. Chicago Classification 4.0 included two new concepts for the diagnosis of achalasia: (1) type III achalasia diagnosis demands 100% absent peristalsis defined as either failed peristalsis or spasm; (2) “inconclusive diagnosis of achalasia” was added as a possibility. Both may decrease unnecessary treatment. Esophagogastric junction outflow obstruction, distal esophageal spasm, and hypercontractile esophagus were only considered clinically significant when correlated to supportive testing and relevant clinical symptoms and in the absence of gastroesophageal reflux disease. This may decrease the surge of treatment, especially peroral endoscopic myotomy, based solely on manometric diagnosis.

Acute back pain (LA) is a considerable problem in any care setting. The Emergency Department (ED) intended to provide immediate, timely and effective care. Our objective was to estimate the prevalence and describe the usual care process.To estimate prevalence, a random sample was included of 5,333 consultations admitted in ED at hospital XX between January and December 2016. LA cases were defined according to the diagnosis at arrival. A retrospective cohort with all LA cases was used to describe complementary studies and therapeutic behaviors established in ED or within the subsequent 30 days outpatient follow-up.LA represented 4.37% (95% CI: 3.83-4.95%) of consultations. A total of 1,096 cases of LA were included: mean age of 53.26 ± 19.85 years, 55.93% (613) female sex and few comorbidities. The median attention time was 3.28 hours and 6.20% was admitted. 70% (778) received treatment in CEA, being the most used administration routes: intravenous (61.86%), and intramuscular (20.62%). The analgesics used were: 55.38% corticosteroids, 55.02% NSAIDs, 34.85% opioids. The complementary studies requested during ED or until 30 days after discharge, were: 52.19% laboratory, 47.17% radiography, 28.38% RM, 10.77% tomography, 9.12% interventionism.This study provides epidemiological data of LA in Argentina and demonstrates the need to promote the rational use of resources.La lumbalgia aguda (LA) es un problema en diferentes ámbitos de atención. La Central de Emergencias de Adultos (CEA) intenta brindar atención inmediata, especializada y eficaz. Nuestro objetivo fue estimar la prevalencia y describir el proceso de atención habitual.Para la estimación de prevalencia, se incluyó una muestra aleatoria de 5,333 consultas admitidas en CEA de un solo hospital entre Enero y Diciembre de 2016. Se definió como numerador a los casos de LA según diagnóstico de triaje al ingreso. Una cohorte retrospectiva con todos los casos de LA se utilizó para describir estudios complementarios y conductas terapéuticas instaurados en CEA, con seguimiento ambulatorio posterior.La lumbalgia aguda representó 4.37% (IC 95%: 3.83-4.95%) de las consultas. Se incluyeron 1,096 casos de LA: edad media de 53.26 ± 19.85 años, 55.93% (613) sexo femenino y escasas comorbilidades. La mediana de tiempo de atención fue de 3.28 horas y se internó 6.20%, 70% (778) recibió tratamiento en CEA, las vías de administración más utilizadas fueron: endovenosa (61.86%) e intramuscular (20.62%). Los analgésicos utilizados fueron: 55.38% corticoides, 55.02% AINE, 34.85% opiáceos. Los estudios complementarios solicitados en CEA o hasta los 30 días postegreso fueron: 52.19% laboratorio, 47.17% radiografía/s, 28.38% resonancia/s, 10.77% tomografía/s, 9.12% intervencionismo/s.Este estudio aporta datos epidemiológicos sobre LA y evidencia la necesidad de fomentar el uso racional de los recursos.

Background: The complex pathophysiology of Alzheimer’s disease (AD) hampers the development of effective treatments. Attempts to prevent neurodegeneration in AD have failed so far, highlighting the need for further clarification of the underlying cellular and molecular mechanisms. Neuroinflammation seems to play a crucial role in disease progression, although its specific contribution to AD pathogenesis remains elusive. We have previously shown that the modulation of the endocannabinoid system (ECS) renders beneficial effects in a context of amyloidosis, which triggers neuroinflammation. In the 5xFAD model, the genetic inactivation of the enzyme that degrades anandamide (AEA), the fatty acid amide hydrolase (FAAH), was associated with a significant amelioration of the memory deficit. Methods: In this work, we use electrophysiology, flow cytometry and molecular analysis to evaluate the cellular and molecular mechanisms underlying the improvement associated to the increased endocannabinoid tone in the 5xFAD mouse model. Results: We demonstrate that the chronic enhancement of the endocannabinoid tone rescues hippocampal synaptic plasticity in the 5xFAD mouse model. At the CA3–CA1 synapse, both basal synaptic transmission and long-term potentiation (LTP) of synaptic transmission are normalized upon FAAH genetic inactivation, in a CB1 receptor (CB1R)- and TRPV1 receptor-independent manner. Dendritic spine density in CA1 pyramidal neurons, which is notably decreased in 6-month-old 5xFAD animals, is also restored. Importantly, we reveal that the expression of microglial factors linked to phagocytic activity, such as TREM2 and CTSD, and other factors related to amyloid beta clearance and involved in neuron–glia crosstalk, such as complement component C3 and complement receptor C3AR, are specifically upregulated in 5xFAD/FAAH animals. Conclusion: In summary, our findings support the therapeutic potential of modulating, rather than suppressing, neuroinflammation in Alzheimer’s disease. In our model, the long-term enhancement of the endocannabinoid tone triggered augmented microglial activation and amyloid beta phagocytosis, and a consequent reversal in the neuronal phenotype associated to the disease., Ministerio de Ciencia e Innovacion (SAF 2016-75959-R and PID2019-108992RB-I00) and NIH grant DA033760. I.B-C. is a recipient of a postdoctoral fellowship from Comunidad Autonoma de Madrid (PEJD-2017-POST/BMD-4478).

Gastroesophageal reflux disease (GERD) is the most common disease of the upper gastrointestinal tract in the Western world. GERD pathophysiology is multifactorial. Different mechanisms may contribute to GERD including an increase in the transdiaphragmatic pressure gradient (TPG). The pathophysiology of GERD linked to TPG is not entirely understood. This review shows that TPG is an important contributor to GERD even when an intact esophagogastric barrier is present in the setting of obesity and pulmonary diseases. RESUMO A doença do refluxo gastroesofágico (DRGE) é a enfermidade mais comum do trato digestivo alto no mundo ocidental. A fisiopatologia da DRGE é multifatorial. Diferentes mecanismos podem contribuir para um aumento do gradiente pressórico transdiafragmático (GPT). A fisiopatologia da DRGE associada ao GPT não é totalmente compreendida. Esta revisão enfoca que o GPT é um importante contribuinte para DRGE mesmo na presença de uma barreira gastroesofágica intacta como na obesidade e doenças pulmonares crônicas.

Pathophysiology of gastroesophageal reflux disease (GERD) is multifactorial. An increased transdiaphragmatic pressure gradient (TPG) may be a main element for GERD in the obese patient. This study aims to evaluate the role of TPG in the physiopathology of GERD in obese individuals. We studied 47 unselected consecutive candidates for bariatric operations (body mass index (BMI) > 35). All patients underwent high-resolution manometry and esophageal pH monitoring. Individuals were grouped as GERD + or GERD − based on DeMeester score. Abdominal pressure (AP) and thoracic pressure (TP), transdiaphragmatic pressure gradient (AP-TP), and lower esophageal sphincter (LES) retention pressure (LES basal pressure-TPG) were determined. Manometric variables were compared with a group of 20 lean healthy individuals (BMI

Gastroesophageal reflux disease (GERD) is associated with different pulmonary diseases, including chronic obstructive pulmonary disease (COPD). Whether GERD is contributory to COPD severity remains unclear. This study aims to evaluate the contribution of GERD to the clinical manifestation of COPD based on ventilatory parameters and yearly clinical exacerbations. We studied 48 patients (56% females, age 66 years) with COPD. All patients underwent high-resolution manometry and esophageal pH monitoring. The patients were separated into two groups according to the presence of GERD. GERD was present in 21 (44%) patients. GERD + and GERD - groups did not differ in regard to gender, age, and body mass index. Pulmonary parameters were not different in the absence or presence of GERD. The number of yearly exacerbations was higher in patients GERD+. The severity of GERD (as measured by DeMeester score) correlated with the number of exacerbations. Our results show the following: 1) GERD does not influence pulmonary parameters and 2) GERD is associated with a higher number of annual clinical exacerbations. We believe GERD must be objectively tested in patients with COPD because the prevalence of GERD in these patients is underestimated when only symptoms are considered. GERD treatment might decrease the frequency of episodes of exacerbation.

The aim of this study was to describe 2 process indicators related to taking blood cultures (BC) in an Adult Emergency Department of a tertiary university hospital in Buenos Aires,and to describe the changes after a series of educational activities for health professionals was implemented during May 2016 as regards the appropriate indication of BC and the proper collection technique.A retrospective cohort study was designed to assess its effectiveness, which consecutively included all patients admitted during 2015-2016. The BC request rate was used as a process indicator, and the percentage of contaminated BCs and the true positives rate were used as quality indicators. Both were measured monthly and prospectively during the period of study.The annual adjusted rate of BC requests was 4.9% (95% CI 4.8-5) in 2015 and 2.9% (95% CI 2.8-2.9) in 2016. The rate of false positive (contaminated) BCs was 4.5% in 2015 and 4.3% after the educational intervention. The true positive BCs were 8.3% in 2015 and 12% post-intervention.These findings prove how important and effective the educational interventions are.

Background There is limited European literature on nursing and sustainability; nursing students are poorly prepared on the connections between resources, climate change, sustainability, and health, so they must acquire knowledge and develop skills and competencies in this field. The use of digital technologies and teaching via E-learning has grown, and has been widely adopted as a learning method for nursing. Objectives The aim of the current study was to test and evaluate digital educational materials on environmental sustainability and health, in the context of university nursing education in different European countries. Design An observational cross-sectional design. Settings University of Plymouth, University of Jaen, and University of Esslingen for Nursing Degree Studies. Participants 299 nursing students: 161 students from University of Jaen; 106 from Plymouth; and 32 from Esslingen. 22 professional evaluators with different profiles were recruited: Teachers, Clinical professionals, Delphi Experts, and Technical Experts. Methods We conducted a piloting and validation process. The materials were designed and adapted to the NurSusTOOLKIT Sustainability Literacy and Competency framework. Evaluation was developed by professionals and students. We used the Spanish Standard for the assessment of Digital Educational Material Quality at University level questionnaire. All students provided informed consent prior to taking part in the learning and evaluation. Results The overall evaluations of materials by students and professionals were 7.98 ± 1.28 and 8.50 ± 1.17, respectively. The Ability to generate learning was scored higher among students (mean difference: 0.84; 0.22–1.47; p = 0.008). In the overall assessment by students, statistically significant differences were found between the three universities (Welch: 11.69, p Conclusions Students, professionals, and technical experts considered the materials to be very good quality, especially regarding the quality of contents, format, and design. For students, these materials can generate reflection and learning regarding environmental and health issues during nursing training.

Rationale There is growing recognition that in addition to universally recognised domains and indicators of wellbeing (such as population health and life expectancy), additional frameworks are required to fully explain and measure Indigenous wellbeing. In particular, Indigenous Australian wellbeing is largely determined by colonisation, historical trauma, grief, loss, and ongoing social marginalisation. Dominant mainstream indicators of wellbeing based on the biomedical model may therefore be inadequate and not entirely relevant in the Indigenous context. It is possible that “standard” wellbeing instruments fail to adequately assess indicators of health and wellbeing within societies that have a more holistic view of health. Objective The aim of this critical review was to identify, document, and evaluate the use of social and emotional wellbeing measures within the Australian Indigenous community. Method The instruments were systematically described regarding their intrinsic properties (e.g., generic v. disease-specific, domains assessed, extent of cross-cultural adaptation and psychometric characteristics) and their purpose of utilisation in studies (e.g., study setting, intervention, clinical purpose or survey). We included 33 studies, in which 22 distinct instruments were used. Results Three major categories of social and emotional wellb eing instruments were identified: unmodified standard instruments (10), cross-culturally adapted standard instruments (6), and Indigenous developed measures (6). Recommendations are made for researchers and practitioners who assess social and emotional wellbeing in Indigenous Australians, which may also be applicable to other minority groups where a more holistic framework of wellbeing is applied. Conclusion It is advised that standard instruments only be used if they have been subject to a formal cross-cultural adaptation process, and Indigenous developed measures continue to be developed, refined, and validated within a diverse range of research and clinical settings. Refereed/Peer-reviewed

Chronic obstructive pulmonary disease (COPD) patients have a high incidence of gastroesophageal reflux disease (GERD) whose pathophysiology seems to be linked to an increased trans-diaphragmatic pressure gradient and not to a defective esophagogastric barrier. Inhaled beta agonist bronchodilators are a common therapy used by patients with COPD. This drug knowingly not only leads to a decrease in the lower esophageal sphincter (LES) resting pressure, favoring GERD, but also may improve ventilatory parameters, therefore preventing GERD.This study aims to evaluate the effect of inhaled beta agonist bronchodilators on the trans-diaphragmatic pressure gradient and the esophagogastric barrier.We studied 21 patients (mean age 67 years, 57 % males) with COPD and GERD. All patients underwent high-resolution manometry and esophageal pH monitoring. Abdominal and thoracic pressure, trans-diaphragmatic pressure gradient (abdominal-thoracic pressure), and the LES retention pressure (LES basal pressure-transdiaphragmatic gradient) were measured before and 5 min after inhaling beta agonist bronchodilators.The administration of inhaled beta agonist bronchodilators leads to the following: (a) a simultaneous increase in abdominal and thoracic pressure not affecting the trans-diaphragmatic pressure gradient and (b) a decrease in the LES resting pressure with a reduction of the LES retention pressure.In conclusion, inhaled beta agonist bronchodilators not only increase the thoracic pressure but also lead to an increased abdominal pressure favoring GERD by affecting the esophagogastric barrier.

Patients with AIDS (acquired immunodeficiency syndrome) may have rheumatic complaints such as arthritis and arthralgia, dry eyes, increased salivary glands, lower back pain, enthesitis etc. Autoantibodies like ANA (antinuclear antibody) and RF (rheumatoid factor) may also be present.To study the prevalence of rheumatic complaints in AIDS patients and correlate them with the presence of ANA and RF.We studied 69 patients with AIDS (28.9% women and 71.0% men) with a mean age of 40.8 ± 8.9 years, median disease duration of 60 months, for rheumatic complaints, ANA, ENA-6 (anti-Ro, anti-La, anti-Sm, anti-RNP, anti-Scl70 and anti-Jo1) and RF. We collected demographic data, CD4+ and CD8+ cell count and values of viral load.Arthralgia was present in 39.1%, sicca symptoms in 21.7%, inflammatory lumbar pain in 13.4%, enthesopathy in 6.6%, parotid enlargement in 1.4%, RF in 10.1% and ANA in 8.6%. All patients were negative for ENA-6. ANA was more common in older patients (p = 0.03) and in those with higher viral load (p = 0.006). No association was found with the presence of RF.The most common manifestation in this context was arthralgia. ANA presence was associated with age of the patients and viral load.

We present our experience in the management and treatment of chronic exertional compartment syndrome (CECS) of the legs in 23 adolescents. Twenty-one patients were affected in the anteroexternal compartment, one in the deep posterior compartment, and one in both. Seventeen of the patients were in-line competitive skaters; the rest practiced soccer or athletics. Mean age at the time of diagnosis was 16 (range, 14-18) years, and the age at the onset of symptoms was 15 (range, 13-17) years. After a careful differential diagnosis, the diagnosis was established on the basis of clinical signs and symptoms and confirmed by intracompartmental pressure (ICP) measurements. Based on our results, the following criteria are considered to be adequate: baseline ICP (at rest)10 mm Hg,20 mm Hg 1 minute after stopping exercise,20 mm Hg 5 minutes later, and15 minutes to achieve ICP normalization. All patients but one accepted surgical treatment (complete subcutaneous fasciotomy). No major complication was observed and only one minor complication occurred. All patients returned to the practice of their sports without any symptomatology 6 weeks after the surgery (mean follow-up, 4.8 years). To date there have been no relapses.

We have investigated the spatial relationship between sites containing newly synthesized RNA and domains containing proteins involved in transcription, such as RNA polymerase II and the transcription factors TFIIH, Oct1, BRG1, E2F-1 and glucocorticoid receptors, using dual immunofluorescence labelling followed by confocal microscopy on cultured cells. As expected, a high degree of colocalisation between the RNA polymerase II and sites containing newly synthesised RNA was observed. Like the newly synthesised RNA and the RNA polymerase II, we found that all the transcription factors that we studied are distributed more or less homogeneously throughout the nucleoplasm, occupying numerous small domains. In addition to these small domains, TFIIH was found concentrated in coiled bodies and Oct1 in a single large domain of about 1.5 microm in 30% of the cells in an asynchronous HeLa cell culture. Remarkably, we found little or no relationship between the spatial distribution of the glucocorticoid receptor, Oct1 and E2F-1 on the one hand and RNA polymerase II and transcription sites on the other hand. In contrast, a significant but incomplete overlap was observed between the spatial distributions of transcription sites and BRG1 and TFIIH. These results indicate that many of the transcription factor-rich nuclear domains are not actively involved in transcription. They may represent incomplete transcription initiation complexes, inhibitory complexes, or storage sites.

Background The increasing phenomenon of online pharmacies has potential for serious public health problems. This study aimed to evaluate the possibility of accessing a prescription drug in the absence of a prescription for an Italian purchaser. Methods Fluoxetine pills were ordered from several online pharmacies. The study included website analysis, and the quality of the received product including packaging, chemical and microbiological analyses. Results Orders could be placed correctly on 61 of the 98 selected websites, and a sales transaction was concluded successfully on 17 websites. Thirteen drug samples were eventually received. In one case it was necessary to fill in a questionnaire before ordering the drugs. All websites displayed aggressive marketing strategies. There was wide variation in terms of domain registration, company base (when declared) and manufacturer's location (mostly India). All pills were delivered in sealed blister packs showing the lot number and manufacturer's details. A leaflet was enclosed in one case only. In three cases we received more pills than ordered, and in one case Viagra pills as a free gift. Pharmacopoeia microbiological requirements were satisfied. Chemical analysis revealed that the active principle was always present, although many samples did not meet the Pharmacopoeia “other impurities” or “total impurities” criteria. Heavy metals and solvents regulated by the Pharmacopoeia did not exceed the set limits; some of the non-regulated ones were also assessed, in some cases with a positive result (e.g. styrene). Conclusion About 20% of purchase attempts resulted in delivery of the drugs, even in the absence of a medical prescription. Traceability was poor and drug quality was generally worse compared to conventional pharmacy-purchased products. Based on all these broad-spectrum results, user safety appears not to be globally guaranteed.

The idea of establishing an examination that accredits the training of the specialists in orthopaedic surgery at the end of their educational period as residents is subject to controversy. With the aim of encouraging the development of this examination, the present members of the National Commission of the Specialty of Orthopaedics (CNE) have reviewed the results obtained in previous examination.The results of the voluntary final exam for Orthopaedics residents, and of the surveys of participant opinions for the years 2006 to 2011 are presented.The total number of participants was 231, growing from 19 in 2005, to 71 in 2011. The overall mean score in the period reviewed (2006-2011) was 6.72 out of 10 points. In these 6 years, 9 participants failed (4.25%). The mean score for the test was 7.57. The overall mean score of the oral exam was 6.57. The worst results were obtained in the general knowledge section of the oral examination. Nobody has ever failed the section on reconstruction of the lower extremity. The upper extremity section in the oral examination achieves the best average results.The examination has established its place in the structure of orthopaedic resident training in our country, even without making it official by the Administration. We must positively acknowledge those candidates who chose to take the examination and be evaluated by their peers in order to prove their qualification and distinction. This recognition will be the best encouragement for future generations.

Background: Genetic Hypophosphatemic Rickets (HR) is a group of diseases characterized by renal phosphate wasting with inappropriately low or normal 1,25-dihydroxyvitamin D3 (1,25(OH)2D) serum levels. The most common form of HR is X-linked dominant HR (XLHR) which is caused by inactivating mutations in the PHEX gene. The purpose of this study was to perform genetic diagnosis in a cohort of patients with clinical diagnosis of HR, to perform genotype-phenotype correlations of those patients and to compare our data with other HR cohort studies. Methods: Forty three affected individuals from 36 non related families were analyzed. For the genetic analysis, the PHEX gene was sequenced in all of the patients and in 13 cases the study was complemented by mRNA sequencing and Multiple Ligation Probe Assay. For the genotype-phenotype correlation study, the clinical and biochemical phenotype of the patients was compared with the type of mutation, which was grouped into clearly deleterious or likely causative, using the Mann-Whitney and Fisher’s exact test. Results: Mutations in the PHEX gene were identified in all the patients thus confirming an XLHR. Thirty four different mutations were found distributed throughout the gene with higher density at the 3’ end. The majority of the mutations were novel (69.4%), most of them resulted in a truncated PHEX protein (83.3%) and were family specific (88.9%). Tubular reabsorption of phosphate (TRP) and 1,25(OH)2D serum levels were significantly lower in patients carrying clearly deleterious mutations than in patients carrying likely causative ones (61.39 ± 19.76 vs. 80.14 ± 8.80%, p = 0.028 and 40.93 ± 30.73 vs. 78.46 ± 36.27 pg/ml, p = 0.013). Conclusions: PHEX gene mutations were found in all the HR cases analyzed, which was in contrast with other cohort studies. Patients with clearly deleterious PHEX mutations had lower TRP and 1,25(OH)2D levels suggesting that the PHEX type of mutation might predict the XLHR phenotype severity., MM was supported by Xunta de Galicia (INCITE Lucas Labrada) and LL by a grant from Xunta de Galicia (PGIDIT06PXIC9101136PN).

Posterior sternoclavicular dislocation is a relatively uncommon lesion, but must always be considered in the event of trauma to the scapulothoracic area in which initial radiology does not show signs of fracture. Its diagnosis and treatment must be carried out promptly because of the possible serious complications that may occur through the clavicle compressing nearby structures. The authors report two cases, which were diagnosed by CT-scan. In the first case, treatment consisted in orthopedic reduction, while in the second case open reduction and fixation with Kirschner wires was required. The result was satisfactory in both cases, and the patients remain asymptomatic three and five years after trauma.

Usually, there is a physiological fall in nocturnal blood pressure among all individuals, both hypertensive and normotensive individuals. The lack of nocturnal blood pressure (BP) fall may be associated with the risk of developing cardiovascular complications in hypertensive patients. Cardiovascular and hormonal factors associated with the lack of nocturnal blood pressure fall was studied in individuals aged over 55, those most exposed to this kind of complications.A total of 108 individuals aged over 55 and with a wide range of BP (59 normotensive and 49 light-moderate hypertensive with no previous treatment) were studied. Two groups were established: dipper and non-dipper, with a fall over 10% in nocturnal SBP and DBP or not, respectively. Patients included in the study underwent serum hormonal measurements (renin, aldosterone, endoteline-1, atrial natriuretic peptide, free epinephrine and norepinephrine), continuous blood pressure monitoring for 24 hours (CBPM) and echocardiography with measurement of left ventricular mass (LVM), cardiac output, and peripheral vascular resistances (PVR) (determined in function of mean blood pressure and cardiac output).Fifty-one individuals were dipper and 57 were non-dipper. Significantly higher cholesterol (p0.05) and free norepinephrine (p0.001) levels among dipper compared with non-dipper individuals were observed. Non-dipper individuals had PVR significantly higher than dipper individuals (p0.05). Values of diurnal BP, other hormonal measurements, and CBPM did not differ significantly between the two groups.Non-dipper individuals aged over 55 have lower circulating free norepinephrine values and higher peripheral vascular resistances than dipper individuals, irrespective of diurnal blood pressure values. The left ventricular mass does not differ significantly between the two groups.

The authors describe a rare case of Lisfranc's dislocation that occurred after a 13-year-old boy kicked the ground while playing soccer. He presented with complete pure dorsal tarsometatarsal dislocation of the first metatarsal. He was treated succesfully by closed reduction and percutaneous K-wire fixation. No complication was found and he returned to his preinjury lifestyle at 3 months. No sequelae are found 3.5 years later.

A case of monostotic fibrous dysplasia involving both alae of the sacrum is reported. Only 2 cases of monostotic sacral involvement were published previously. The lesion was detected in a 42-year-old man suffering from lumbosacral pain after minimal trauma. Radiographic studies revealed cystic images on both alae of the sacrum, with internal condensations and some liquid contents. A trephine biopsy did not produce enough diagnostic data, and a posterior surgical approach was elected. Curettage and refilling with allograft were performed. The pathologic anatomy study diagnosed a fibrous dysplasia. Two years after surgery, the patient was asymptomatic.

We carried out a case-control study with 90 patients with Legg-Calvé-Perthes disease (LCPD) and 183 normal children, as controls, selected at random to determine whether the condition of passive smoking is related to the disease. Seventy-one of 90 of the LCPD group (78.9%) were passive smokers. Only 79 of 183 (43.2%) in the control group were passive smokers (p = 0.00000). We did not find any statistical relationship between passive smoking and evolution of the condition (p = 0.42883), Catterall extension (p = 0.60544), final Stulberg result (p = 0.53201), or presence of sequelae (p = 0.53256). We also could not find any statistical difference between ages (p = 0.18). The odds ratio was 5.3203 (95% confidence interval 2.92-9.69). The association between LCPD and passive smoking, after controlling for age and gender, became significant (p = 0.0000). Thus the risk of LCPD in passive smoking children is more than five times higher than in children who are not exposed to smoke. It seems that passive smoking is a factor directly or indirectly associated with LCPD.

We show the effectiveness of treatment with continuous intrathecal baclofen infusion in a case of hereditary generalized dystonia refractory to anticholinergics, tetrabenazine, pimozide, L-dopa, benzodiazepines and thalamotomy.A 26 years old female patient, when she was 11 years old began with torsion dystonia in her left feet, that progressively worsened to involve her entire body. She had painful spasms. She had four brothers, three of them with dystonia and one healthy. Her uncle grandfather had similar symptoms. Complementary explorations to reject secondary origin was negatives. She was treated with high and progressive dosages of anticholinergics, pimozide, tetrabenazine, benzodiazepines, L-dopa and thalamotomy without improvement. Underwent intrathecal baclofen test dosing, we used 25, 50, 100 micrograms/day, the last one with improvement during 10 hours. A pump was inserted with an initial dose of 220 micrograms/day. After pump insertion, baclofen dosage was gradually increased to 450 micrograms/day. She had a great improvement in right part of her body and less in her left body. Painful spasms had disappeared.We propose continuous baclofen intrathecal infusion pump for patients with severe torsion dystonia that not response to ordinal treatment.

Transverse stress fracture of the patella in a child is an exceptional injury in childhood. Only one case has been previously reported. The present study reports a new case of a 12-year-old boy with anterior knee pain misdiagnosed for 5 months as Sinding-Larsen-Johansson disease who developed sudden anterior right knee pain and pop while running in a soccer match. Surgery was performed. The evolution was favorable without any immobilization required and the patient achieved full recovery.

Two new cases of triplane fracture of the distal tibia are reported in the proximal phalanx of the thumb and the distal radius, respectively, of a 12-year-old girl and a 13-year-old boy. Neither fracture showed any displacement, achieving healing at 4 weeks of external immobilization. Triplane fractures can occur across growth plates other than the distal tibia. Because of the rapid physiologic physeal arrest, the potential for growth deformity is null. In cases without displacement, these fractures should be treated conservatively by external immobilization, as one would treat a one-plane fracture.

Purpose A high index of suspicion is necessary to make the diagnosis of stress fractures in athletes. This is a case report of a soccer player who sustained a transverse stress fracture of the patella. Case summary The soccer player experienced mild discomfort to the patella for 1 month. He then developed severe pain after a twisting motion while playing soccer. Radiological examination showed an undisplaced transverse fracture of the patella. After immobilization in a plaster cast and rehabilitation, he returned to normal activity in 3 months. Discussion Only six cases have been reported in the literature to date. Relevance This case illustrates the need to consider a stress fracture when pain in the patella persists.

Two main principles of nuclear organization have been outlined on the basis of contributions by many research groups in recent years. The first principle is that interphase chromosomes occupy discrete territories in the nucleus, with no intermingling of the DNA from different chromosomes. Within a chromosome territory the DNA is organized in chromatin fibers at several levels of folding, that meander through the territory. Transcription and replication take place at the surface of these higher order chromatin fibers, probably on locally unfolded DNA templates. The second principle is that different types of nuclear domains are associated with several specific gene loci. This holds for clusters of interchromatin granules, coiled bodies, RNA 3'-cleavage factor-containing nuclear bodies (cleavage bodies) and probably PML-containing nuclear bodies. These domains may play an important role in the spatial arrangement of genes in the interphase nucleus. Despite these new insights, our knowledge of the function of many nuclear compartments and the molecular interactions responsible for the dynamic organization of a compartmentalized nucleus is still in its infancy.

Plantar dislocation of the metatarsophalangeal joint of the great toe is a rare injury. We report one example which occurred in a lactating lady following minor trauma, and which was successfully treated by closed manipulation. There was no evidence of major generalised ligamentous laxity.

This overview describes the spatial distribution of several enzymatic machineries and functions in the interphase nucleus. Three general observations can be made. First, many components of the different nuclear machineries are distributed in the nucleus in a characteristic way for each component. They are often found concentrated in specific domains. Second, nuclear machineries for the synthesis and processing of RNA and DNA are associated with an insoluble nuclear structure, called nuclear matrix. Evidently, handling of DNA and RNA is done by immobilized enzyme systems. Finally, the nucleus seems to be divided in two major compartments. One is occupied by compact chromosomes, the other compartment is the space between the chromosomes. In the latter, transcription takes place at the surface of chromosomal domains and it houses the splicing machinery. The relevance of nuclear organization for efficient gene expression is discussed.

We report 3 cases of dorsal dislocation of the first metatarsophalangeal joint treated by closed reduction. Associated dislocation of the sesamoid complex may make reduction difficult. We suggest a modification of Jahss's classification and have confirmed our findings in amputation specimens.

This article describes repair of osteochondritis dissecans of the knee by arthroscopic Herbert screw fixation and reports the healing response and clinical results. Eleven patients were subjected to surgery using arthroscopy and all were affected by symptomatic osteochondritis dissecans of the medial femoral condyle. Average follow-up time was 16.3 months (13-29). One to four Herbert screws were used, either as the only fixation method or in conjunction with absorbable pins [poly (p-dioxanone)], depending on size and fragmentation of the injury. The patients were non-weight bearing for 10 weeks. The radiographic evidence of healing was correlated with the clinical outcome. The results were excellent or good in 81.8% of the patients. Arthroscopic Herbert screw fixation is an effective and safe method of repair for osteochondritis dissecans of the knee.

Case report of a plumber's fatal work accident. Investigations on the causes of death made at post mortem showed that the worker had absorbed a large quantity of freon 22 (chlorodifluoromethane) which is known to be a narcotic agent and capable of inducing cardiac arrhythmia. It is believed freon inhalation was the cause of loss of consciousness with consequent death from drowning in the water issuing from the pipes. It is concluded that preventive measures need to be reinforced by adequate information to the workforce on the risks connected to this type of gas.

We retrospectively analyzed thirty-seven cases of infantile pulmonary tuberculosis that had been subjected to bronchoscopy. Although this exploration produced evidence of bronchial wall irritation, the macroscopic findings, as well as the histological ones, were frequently insufficient to make the diagnosis of tuberculosis. The bacteriological study of bronchial exudates of these patients was more efficient since we encountered BAAR in 34.2% of the samples.

The authors present the case of a closed posteriomedial enucleation of the talus, which is the first ever reported in the literature. The patient's course is described and available publications are fully reviewed. The authors believe that in spite of the high rate of necrosis observed in talus enucleations, it is valid to attempt treatment designed to prevent necrosis, reserving arthrodesis for those cases where necrosis has already occurred or where walking is impaired.

After suffering traumatic volar dislocation of the first metacarpophalangeal joint, a 27-year-old patient was treated using closed reduction of the thumb. The patient regained full function and stability of the joint after 5 months. A review of the literature indicates that open reduction is the most common treatment for volar dislocation, but our case report suggests that conservative treatment using closed reduction can be highly effective.

A case of intermittent priapism with neurogenic claudication caused by spinal stenosis is reported. The symptoms disappeared completely after decompressive laminectomy.

Summary A 12-year-old boy had a history of pain in the right knee for 2 months, mainly pain in extension. He could not recall any trauma to the knee. The radiograph suggested osteochondritis dissecans in the anterior spine. An arthroscopy was performed and a prominence in the tibial insertion of the anterior cruciate ligament in the anterointernal area was observed, and the fragment resected. The diagnosis of osteochondritis dissecans was confirmed by pathological study. As far as we are aware, this is the first case describing this new location.

A case of dorsal dislocation of the first metatarso-phalangeal joint is reported because it is a very unusual injury, and the literature is reviewed.

We report the case of a girl, aged 2 years, who was able to dislocate her right hip voluntarily and in whom there was no other abnormality. She was given psychological treatment and the condition resolved after 2 years. Eight similar cases of habitual dislocation of the hip are reported in the world literature.

We have followed for more than ten years three women suffering from a S.N. which appeared in their fourth decade. In these patients no malignancy or other known toxic and infectious aetiologies could be found. According to the authors the clinical symptomatology as far as the C.S.F. and the neurophysiological findings justify the separation of S.N. from G.B.S., being the first disorder characterized by an elective damage of the neurones of the ganglia of the posterior roots. Therefore the eponym of "ganglio-neuronopathy" seems appropriate.