Overlap Syndrome Research Articles

Atypical Alzheimer's disease: new insights into an overlapping spectrum between the language and visual variants.

Overlap between language and visual variants of atypical Alzheimer's disease (AD) has been reported. However, the extent, frequency of overlap, and its neuroanatomical underpinnings remain unclear. Eighty-two biomarker-confirmed AD patients who presented with either predominant language (n = 34) or visuospatial/perceptual (n = 48) deficits underwent detailed clinical examinations, MRI, and [18F]flortaucipir-PET. Subgroups were defined based on language/visual testing and patterns of volume loss and tau uptake were assessed. 28% of the language group had visual dysfunction (marked in 8%), and 47% of the visual group had language impairment (marked in 26%). Progressive involvement of the parieto-occipital and frontal lobes was noted with greater visual impairment in the language group, and greater left parieto-temporal and frontal involvement with worsening language impairment in the visual group. Only 25% of our cohort showed a pure language or visual presentation, highlighting the high frequency of syndromic overlap in atypical AD and the diagnostic challenge of categorical phenotyping.

The effect of rabeprazole on the secretory and motor function of the gastrointestinal tract

Parietal cell proton pump inhibitors (PPIs) have become firmly established in clinician practice. When discussing the features of their action, as a rule, researchers focus on their antisecretory effect. In the available literature, we found only a single report on the effect of PPIs on the motor-evacuation function of the gastrointestinal tract (GIT). We have found that the PPI rabeprazole not only has an antisecretory effect, but also normalizes the motor-evacuation function of all parts of the gastrointestinal tract. In this regard, it can be used in patients with gastric hypersecretion in functional gastrointestinal disorders (FGID) with impaired motor-evacuation function. Aim. To study the effect of the antisecretory drug rabeprazole on the state of gastrointestinal motility in patients suffering from FGID of the gastrointestinal tract and overlap syndrome (overlap syndrome) with functional dyspepsia (FD) with gastric hypersecretion and irritable bowel syndrome (IBS). Material and methods. We examined 30 patients suffering from FGID of the gastrointestinal tract and cross syndrome - FD with gastric hypersecretion and IBS. To relieve clinical symptoms of FD, the drug rabeprazole 20 mg was prescribed once a day for 14 days. Before and after the start of treatment, patients were examined with VAS and SF-36 tests, intragastric pH-metry and peripheral electrogastroenterocolography (EGECG) were performed. Results. In all 30 patients with FGID of the gastrointestinal tract with FD and overlap syndrome with IBS, gastric hypersecretion was eliminated within 3 hours after taking 20 mg of rabeprazole. 2 weeks after the start of treatment, abdominal pain and heartburn were completely eliminated in 28 (93.3%) patients. In all (100%) patients with FGID of the gastrointestinal tract with gastric hypersecretion and overlap syndrome, after two weeks of treatment, sour belching disappeared, and nausea disappeared in 23 (77%) patients. Rabeprazole therapy in the examined individuals was accompanied by a marked improvement in the quality of life in all assessed SF-36 parameters. 2 weeks after starting rabeprazole, in patients with FGID of the gastrointestinal tract and overlap syndrome of FD and IBS, the frequency of bowel movements and stool quality were normalized, and according to the results of EGECG, the relative myoelectric activity (P(i)/PS) of the gastrointestinal tract and the rhythmicity coefficient (Kritm) were normalized. Conclusion. Rabeprazole quickly neutralizes gastric secretion and helps eliminate symptoms of FD. It also has a beneficial effect on the motor-evacuation function of all parts of the gastrointestinal tract, normalizing the frequency of bowel movements, stool quality and myoelectric activity of all parts of the gastrointestinal tract according to EGECG. Rabeprazole can be used in patients with FGID with FD with gastric hypersecretion and overlap syndrome with IBS.

Clinical Image: Dystrophic Calcinosis in Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome.

Clinical Image: Dystrophic Calcinosis in Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome.

An integrated metabo-lipidomics profile of induced sputum for the identification of novel biomarkers in the differential diagnosis of asthma and COPD

BackgroundDue to their complexity and to the presence of common clinical features, differentiation between asthma and chronic obstructive pulmonary disease (COPD) can be a challenging task, complicated in such cases also by asthma–COPD overlap syndrome. The distinct immune/inflammatory and structural substrates of COPD and asthma are responsible for significant differences in the responses to standard pharmacologic treatments. Therefore, an accurate diagnosis is of central relevance to assure the appropriate therapeutic intervention in order to achieve safe and effective patient care. Induced sputum (IS) accurately mirrors inflammation in the airways, providing a more direct picture of lung cell metabolism in comparison to those specimen that reflect analytes in the systemic circulation.MethodsAn integrated untargeted metabolomics and lipidomics analysis was performed in IS of asthmatic (n = 15) and COPD (n = 22) patients based on Ultra-High-Pressure Liquid Chromatography-Mass Spectrometry (UHPLC-MS) and UHPLC–tandem MS (UHPLC-MS/MS). Partial Least Squares-Discriminant Analysis (PLS-DA) was applied to resulting dataset. The analysis of main enriched metabolic pathways and the association of the preliminary metabolites/lipids pattern identified to clinical parameters of asthma/COPD differentiation were explored. Multivariate ROC analysis was performed in order to determine the discriminatory power and the reliability of the putative biomarkers for diagnosis between COPD and asthma.ResultsPLS-DA indicated a clear separation between COPD and asthmatic patients. Among the 15 selected candidate biomarkers based on Variable Importance in Projection scores, putrescine showed the highest score. A differential IS bio-signature of 22 metabolites and lipids was found, which showed statistically significant variations between asthma and COPD. Of these 22 compounds, 18 were decreased and 4 increased in COPD compared to asthmatic patients. The IS levels of Phosphatidylethanolamine (PE) (34:1), Phosphatidylglycerol (PG) (18:1;18:2) and spermine were significantly higher in asthmatic subjects compared to COPD.ConclusionsThis is the first pilot study to analyse the IS metabolomics/lipidomics signatures relevant in discriminating asthma vs COPD. The role of polyamines, of 6-Hydroxykynurenic acid and of d-rhamnose as well as of other important players related to the alteration of glycerophospholipid, aminoacid/biotin and energy metabolism provided the construction of a diagnostic model that, if validated on a larger prospective cohort, might be used to rapidly and accurately discriminate asthma from COPD.Graphical

Associations of Digital Ulcers in Patients with Systemic Sclerosis: An 8-Year Retrospective Study

Introduction: This study aimed to investigate the associations of digital ulcers (DUs) in patients with systemic sclerosis (SSc). Methods: This retrospective study investigated the demographic characteristics, specific autoantibodies, organ involvement, and laboratory tests in patients with SSc from our hospital. Results: This study enrolled 144 patients with SSc. The DU+ group consisted of 15 (10.4%) patients. Patients with SSc having DUs have longer disease duration, higher fibrinogen, higher fibrin degradation product, and lower cholesterol. None of the patients used cholesterol-lowering drugs before onset of DUs. The study also demonstrated a higher prevalence of anti-dsDNA and anti-histone antibodies in patients with SSc with DUs. Anti-dsDNA antibody is a specific antibody for SLE with a specificity of 96–99%. A total of 86.1% (124/144) of patients suffered from diffuse cutaneous SSc, and 28.5% (41/144) of patients suffered from overlap syndrome. Conclusion: Our study indicated that patients with SSc with fibrinogen of >2.895 g/L (p = 0.043) and cholesterol of <3.340 mmol/L (p = 0.036), which is equal to 129.258 mg/dL, are at high risk of developing DUs.

Pulmonary hypertension in newly diagnosed obstructive sleep apnea-chronic obstructive pulmonary disease overlap syndrome patients attending a tertiary care centre-a cross-sectional analysis.

Overlap syndrome is the association of obstructive sleep apnea (OSA) with chronic obstructive pulmonary disorder and with other chronic respiratory diseases. Patients with overlap syndrome have a worse prognosis compared with chronic obstructive pulmonary disorder or OSA alone. Additionally, patients with combined chronic obstructive pulmonary disorder and OSA are more likely to develop pulmonary hypertension and right heart failure much earlier than those without overlap. To assess the occurrence of pulmonary hypertension (PH) in newly diagnosed OSA-chronic obstructive pulmonary disorder overlap syndrome patients attending a tertiary care centre. This cross-sectional study was conducted at Department of Pulmonary Medicine, Government Medical College, Kozhikode, Kerala, South India, among patients with OSA above 40years of age who were proactively evaluated to pick out those with undiagnosed overlap. A period of 6months after getting ethical clearance from June 2018 was selected as the study period. Among patients with symptoms suggestive of OSA above 40years of age who gave the informed consent were enrolled after screening with Standard Sleep questionnaires (Berlin questionnaire, STOP BANG and Epworth Sleepiness Scale). Enrolled patients underwent routine spirometry and sleep study using the standard Level 1 overnight polysomnography (Level1 OPSG). Patients were classified in to two groups as OSA patients (group I), having an apnea hypopnea index (AHI) > 5/hr alone and the second group as those OSA patients (group II), with an obstructive spirometry pattern who were the overlap group. Arterial blood gas analysis (a sample of radial arterial blood was drawn with the patient awake and supine, and was analyzed for pH, PaCO2 and PaO2) and echocardiography (ECHO) of the two groups were compared as a non-invasive method to assess pulmonary artery hypertension and results were analyzed in a systematic manner. Among the 84 patients enrolled, 16.7% had overlap syndrome and the rest had OSA alone. Statistically significant higher mean weight and BMI for those with overlap syndrome compared to the OSA group were observed. Mean FVC (forced vital capacity), FEV1 (forced expiratory volume 1s), and FEV1/FVC were lower in those with overlap syndrome compared to OSA group. The mean values of ABG parameters revealed higher PaCO2 and lower PaO2 among the group with overlap syndrome which were statistically significant. However, there was no significant difference in resting room air SaO2 value between the two groups. The mean values of sleep duration and efficiency were significantly lower in those with overlap syndrome with a p value < 0.001. The mean value of arousal and REM (rapid eye movement) sleep percentage were significantly higher among those with overlap syndrome (p < 0.001). Mean value of NREM (non-rapid eye movement) sleep percentage was lower among the group with overlap syndrome compared with the OSA group, and this difference was statistically significant. The mean AHI value of the overlap syndrome group was 39.79 ± 7.54 and this was significantly higher than the OSA group (p < 0.004). Among the 14 patients who had ECHO evidence of pulmonary hypertension, 9 (64.3%) belonged to the overlap group which shows that they are a highly vulnerable group for developing pulmonary hypertension (PH) and requires early detection and more rigorous treatment. This study confirms that OSA patients with modest daytime level of hypoxemia and mild-moderate chronic airflow limitation have a high prevalence of PH. Chronic airway obstruction may be asymptomatic in some subjects and this stresses the necessity of pulmonary function test in OSA.

Comorbid functional pathology in gastroenterology. Possibilities and prospects for drug treatment: A review

Functional disorders of the gastrointestinal tract (FD GT) and their intersection are relevant due to their high prevalence in the population, decrease and, in some cases, loss of performance, low quality of life and treatment difficulties. Despite the large number of consensuses and clinical recommendations at the national and international level, the effectiveness of therapy for FD GT and their overlap does not exceed 30-50%. There is a high degree of comorbidity of these diseases, called “overlap syndromes,” due to common pathogenetic mechanisms. This predetermines the need to use drugs with a multi-target therapeutic effect in the treatment of such pathology. Such drugs include trimebutine (Trimedate®), probiotics and psychotropic drugs. Data obtained in recent years on the presence of anti-inflammatory, antimicrobial, regenerative effects in relation to nervous and epithelial tissue and anti-carcinogenic effects in trimebutine make it unique for the treatment of gastrointestinal diseases and their intersection. Currently, there is a search for new promising drugs for the treatment of both individual FD GT and their overlap, as well as improving the regimens of existing treatment methods. The review article is devoted to existing opportunities and new horizons for the treatment of FD GT and their overlap.

Clinical results of orthognatic surgery in the treatment of obstructive sleep apnea syndrome: a systematic review

Introduction: Obstructive sleep apnea syndrome (OSAS) is characterized by intermittent partial or complete obstruction of the airways during sleep and is called OSAS syndrome when associated with day/night symptoms and/or comorbidities, such as systemic arterial hypertension or diabetes mellitus. The prevalence reaches 32% in the general population, varies between 1% and 20% when associated with COPD (overlap syndrome), and is described as above 60% in populations with COPD and obesity. Orthognathic surgery (OC) can correct deformities of the maxillary and mandibular bones in OSAS. Objective: It was to develop the main approaches and clinical results of orthognathic surgery for the treatment of obstructive sleep apnea syndrome through a systematic review. Methods: The PRISMA Platform systematic review rules were followed. The search was carried out from November 2023 to February 2024 in the Scopus, PubMed, Science Direct, Scielo, and Google Scholar databases. The quality of the studies was based on the GRADE instrument and the risk of bias was analyzed according to the Cochrane instrument. Results and Conclusion: 146 articles were found, 58 articles were evaluated and 26 were included in this systematic review. Considering the Cochrane tool for risk of bias, the global assessment resulted in 32 studies with a high risk of bias and 22 studies that did not meet GRADE. Most studies showed homogeneity in their results, with X2 =65.8%&gt;50%. Maxillomandibular advancement surgery is a successful treatment for obstructive sleep apnea, but there are still concerns about cosmetic results due to the major advances involved. Bimaxillary advancement osteotomy significantly increases oropharyngeal volume and contracted superficial areas, which remain stable between 6 months and 1 year postoperatively.

The clinical and hemodynamic characteristics of pulmonary hypertension in patients with OSA-COPD overlap syndrome

BackgroundOur study aimed to assess the clinical and hemodynamic characteristics of pulmonary hypertension (PH) in patients with overlapping obstructive sleep apnea (OSA) and chronic obstructive pulmonary disease (COPD), referred to OSA-COPD overlap syndrome (OS). MethodsWe enrolled a total of 116 patients with OS, COPD, or OSA who underwent right heart catheterization (RHC) due to suspected PH. We conducted a retrospective analysis of the clinical and hemodynamic characteristics of these patients. ResultsAmong the three groups (OS group, n = 26; COPD group, n = 36; OSA group, n = 54), the prevalence of PH was higher in the OS group (n = 17, 65.4%)compared to OSA group (n = 26,48.1%) and COPD group (n = 20,55.6 %). Among three groups with PH, the superior vena cava pressure (CVP) and right ventricular pressure (RAP) were higher in the OS group than in the OSA group (P < 0.05). Patients in the OS and COPD groups had higher pulmonary artery wedge pressure (PAWP) than in the OSA group (14.88 ± 4.79 mmHg, 13.45 ± 3.68 mmHg vs. 11.00 ± 3.51 mmHg, respectively, P < 0.05). OS patients with PH exhibited higher respiratory event index (REI), time spent with SpO2 <90%, oxygen desaturation index (ODI), minimal SpO2 (MinSpO2) and mean SpO2 (MSpO2) compared to OS patients without PH. After adjusting for potential covariates, we found that MinSpO2 (OR 0.937, 95 % CI 0.882–0.994, P = 0.032), MSpO2 (OR 0.805, 95% CI 0.682–0.949, P = 0.010), time spent with SpO2 <90% (OR 1.422, 95% CI 1.137–1.780, P = 0.002), and FEV1 % pred (OR 0.977, 95 % CI 0.962–0.993, P = 0.005) were related to the development of PH. ConclusionsPatients with OS showed higher prevalence of PH, along with higher PAWP, CVP and RAP. Worse nocturnal hypoxemia was found in OS patients with PH.

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: Localized scleroderma, systemic sclerosis and overlap syndromes.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes.

Thirteen-Year Follow-Up of a Patient with Liver Cirrhosis Resulting from the Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis: Severe COVID-19 and Liver Transplantation

Aim: to present the difficulties of long-term management of a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) who suffered from severe COVID-19 infection.Key points. The diagnosis of liver cirrhosis as an outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) was established at the patient’s age of 33 years. At the age of 40, the patient became pregnant for the first time, the pregnancy proceeded well, and a cesarean section was performed at 36 weeks. At the age of 45, the patient suffered a severe new coronavirus infection, followed by decompensation of liver cirrhosis, which required liver transplantation 4 months after COVID-19, followed by a favorable postoperative course.Conclusion. This clinical case demonstrates the successful onset and outcome of pregnancy in a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis). The pronounced activity of the disease after severe new coronavirus infection required liver transplantation with successful outcome.

LUPUS Cerebritis in SLE / SJOGREN'S Overlap Syndrome

LUPUS Cerebritis in SLE / SJOGREN'S Overlap Syndrome

A Cross-Sectional Study Evaluating the Association of Brachial Artery Flow Mediated Vasodilation with Physical Activity Measured by Accelerometry in Patients with the Overlap of Obstructive Sleep Apnea and Chronic Obstructive Pulmonary Disease.

Cardiovascular disease (CVD) is the leading cause of morbidity and mortality in Overlap Syndrome (OS), the co-occurrence of Obstructive Sleep Apnea and Chronic Obstructive Pulmonary Disease. Clustering of patients in subgroups with similar pre-clinical manifestations (ie, endothelial dysfunction) may identify relevant therapeutic phenotype categories for patients with OS who are at high risk of CVD. We therefore conducted a cross-sectional pilot study of endothelial function in 7 patients with OS (Forced Expiratory Volume in 1 second/Forced Vital Capacity < 0.7) on continuous positive airway pressure therapy (n = 7) to assess the relationship between FMD and physical activity. We found a strong association between FMD and step counts (rho = 0.77, p = 0.04); and FMD and moderate physical activity (rho = 0.9, p = 0.005). Further, larger studies are needed to confirm that FMD may identify patients with OS at high risk of CVD who benefit from increased physical activity.

Chronic ozone exposure in mice mimics clinical asthma-COPD overlap syndrome and is attenuated by tiotropium

Chronic ozone exposure in mice mimics clinical asthma-COPD overlap syndrome and is attenuated by tiotropium

The role of neutrophil to lymphocyte ratio in patients with COPD-OSA overlap syndrome.

The aim of this study is to investigate the role of the neutrophil to lymphocyte ratio (NLR) in patients diagnosed with the chronic obstructive pulmonary disease-obstructive sleep apnea (COPD-OSA) overlap syndrome and comorbid pulmonary hypertension (PH). We enrolled a consecutive of stable COPD patients and conducted spirometry measurements, nocturnal polysomnography (PSG), and echocardiography for all participants. Clinical laboratory data were collected. A total of 178 patients with stable COPD were enrolled among whom 33.14% (59/178) were diagnosed with OSA. Of the patients with overlap syndrome, 35.59% (21/59) showed comorbid PH, compared to 34.45% (41/119) in COPD patients without OSA. There was no significant difference in the occurrence of PH between COPD with and without OSA. NLR was significantly higher in patients with overlap syndrome compared to those with either disease alone. The difference in NLR between COPD-OSA patients with and without PH was not significant. Correlation analysis revealed that NLR was associated with age, total sleep time spent with oxygen saturation below 90% (T90), CRP, pulmonary artery systolic pressure (PASP), and minimum peripheral capillary oxygen saturation (SpO2min) in all COPD patients. NLR was identified as an independent factor contributing to OSA in COPD. The median cut-off value for detecting OSA in stable COPD was 2.49. However, NLR was not found to be a predictor for PH in COPD-OSA overlap syndrome. NLR can serve as a predictive marker for comorbid OSA in patients with COPD. NLR is expected to increase its clinical application as a convenient and cost-effective biomarker for COPD-OSA overlap syndrome.

Screening and early warning system for chronic obstructive pulmonary disease with obstructive sleep apnoea based on the medical Internet of Things in three levels of healthcare: protocol for a prospective, multicentre, observational cohort study

IntroductionChronic obstructive pulmonary disease (COPD) and obstructive sleep apnoea (OSA) are prevalent respiratory diseases in China and impose significant burdens on the healthcare system. Moreover, the co-occurrence of COPD and...

A Rare Case of Renal Crisis in Systemic Sclerosis–Systemic Lupus Eritematosus Overlap Syndrome: A Case Report and Literature Review

A Rare Case of Renal Crisis in Systemic Sclerosis–Systemic Lupus Eritematosus Overlap Syndrome: A Case Report and Literature Review

A280 HEPATOBILIARY DISEASE IN INDIVIDUALS LIVING WITH FRAGILE X SYNDROME: A CASE SERIES

Abstract Background Fragile X syndrome is a genetic disorder known to cause intellectual disability through decreased or absent FMR protein (FMRP). Basic science studies have identified potential associations with hepatobiliary inflammation. Clinical reports of liver disease in Fragile X syndrome are however infrequent. Aims We describe 3 individuals living with Fragile X syndrome and chronic liver disease. Methods A case series and literature review were performed. Results The 1st person is a woman with concurrent Fragile X and autosomal recessive polycystic kidney disease, initially seen by Hepatology at 18 y.o. with evidence of hepatic fibrosis on abdominal ultrasound with normal liver enzymes (Table 1). She subsequently developed liver cirrhosis and portal hypertension, as well as biliary duct dilatation seen on MRI. Her overall presentation was thought to be in keeping with Caroli syndrome and congenital hepatic fibrosis, and she is currently pending genetic screening for hepatorenal ciliopathy. The 2nd person is a woman with Fragile X syndrome and ulcerative colitis, who initially presented to Hepatology at 24 y.o. with mixed liver enzyme abnormalities. Lab tests over time revealed immunology suggestive of autoimmune hepatitis (AIH) (Table 1). Subsequent investigations demonstrated primary sclerosing cholangitis (PSC) with cirrhosis. Her disease is currently managed as AIH/PSC overlap syndrome with ursodeoxycholic acid (UDCA), tacrolimus, mycophenolate, and budesonide. The 3rd person is a man with Fragile X syndrome who initially presented to Hepatology at 17 y.o. with acute hepatitis with AIH immunology (Table 1). Liver biopsy at the time demonstrated findings suggestive of early PSC as well as possible AIH. He continued to have progressive cirrhosis as well as overt PSC on subsequent abdominal imaging and is currently managed as AIH/PSC overlap syndrome with UDCA, azathioprine, and budesonide. A review of literature revealed that asides from known intellectual, behavioral, and physical effects of Fragile X syndrome, there are minimal reports of other associated medical complications including liver problems or autoimmune conditions including IBD. There are however reports of autoimmunity in those with altered FMR1 (Fragile X Mental Retardation 1) gene mutations. Conclusions These 3 cases of liver disease in Fragile X highlight young age of presentation for chronic liver disease with both biliary and immune-mediated characteristics, along with progression to cirrhosis. Additional review for liver manifestations in people living with Fragile X would help understand if this association is incidental or causal. Investigations of individuals with Fragile X syndrome and liver disease Funding Agencies None

Whole-Body MRI at Initial Presentation of Chronic Recurrent Multifocal Osteomyelitis, Juvenile Idiopathic Arthritis, Their Overlapping Syndrome, and Non-Specific Arthropathy.

(1) Background: Whole-body magnetic resonance imaging (WB-MRI) is central to defining total inflammatory burden in juveniles with arthritis. Our aim was to determine and compare the initial distribution of lesions in the WB-MRI in patients with chronic recurrent multifocal osteomyelitis (CRMO), juvenile idiopathic arthritis (JIA), their overlapping syndrome (OS), and with Non-specific Arthropathy (NA). (2) Methods: This retrospective single center study was performed on an Avanto 1.5-T MRI scanner with a dedicated multichannel surface coil system. A total of 173 pediatric patients were included with the following final diagnoses: CRMO (15.0%), JIA (29.5%), OS (4.6%), and NA (50.9%). (3) Results: Bone marrow edema (BME) was the most common abnormality, being seen in 100% patients with CRMO, 88% with OS, 55% with JIA, and 11% with NA. The bones of the lower extremities were the most affected in all compared entities. Effusion was seen in 62.5% children with OS, and in 52.9% with JIA, and in CRMO and NA, the exudate was sporadic. Enthesitis was found in 7.8% of patients with JIA and 3.8% with CRMO, and myositis was seen in 12.5% of patients with OS and in 3.9% with JIA. (4) Conclusions: The most frequent indication for WB-MRI in our center was JIA. The most common pathology in all rheumatic entities was BME, followed by effusion mainly seen in in OS and JIA. Enthesitis and myositis were less common; no case was observed in NA.

Pathogenesis and A Practical Guide to the Management of Steven-Johnson Syndrome &amp; Toxic Epidermal Necrolysis

Steven Johnson Syndrome (SJS) and Toxic Epidermal necrolysis are rare dermatological emergencies that are associated with a high degree of morbidity and mortality [1]. They are considered to be severe blistering conditions that portray an image of diffuse epidermal necrolysis in association with sloughing of the skin. SJS and TEN are effectively the same disease with the main difference being the percentage of body surface area involved. It is widely accepted that SJS belongs to the below 10% body surface area category and TEN to the more than 30% category. A Steven Johnson syndrome and Toxic Epidermal Necrolysis overlap exists when the body surface area affected is between 10 percent and 30 percent [2]. The causes of both conditions within this spectrum are most likely drug related, however certain infections can also trigger SJS/TEN but this is less likely. In a substantial number of cases the cause is idiopathic [3]. The main grading system is the SCORTEN criteria, which can also provide more information regarding prognosis and mortality [4]. Management of this condition is highly controversial with no clear guidelines but there is a wide agreement that patients should be managed in a burns unit with intravenous fluids and protection from secondary infections. Stopping all medications and investigating for the cause is important unless there is clear benefit from any single medication.