Insidious Onset Of Symptoms Research Articles

N-methylbenzothiazolium based theranostics as fluorescence imaging and photo-oxidation agents for Amyloid-β

Alzheimer's disease is a progressive neurodegenerative disorder with an insidious onset of symptoms. At present, there are no treatments that can effectively stop or reverse the disease from progressing. The development of effective methods for early diagnosis and treatment of Alzheimer's disease is therefore still urgently needed. In this article, we designed a family of Q-series probes (Q16∼Q21) that bind to Aβ aggregates by introducing different electron donors to bind to benzothiazole salts electron acceptors with different substituents to achieve imaging and photooxidation of aggregated Aβ proteins. The structure of the six probes was composed of N, N-dimethylaminophenyl and triphenylamine (donor groups), benzothiazole salts (acceptor groups), and π-conjugated double bonds. Among them, the probe Q17 with triphenylamine as the electron donor had a good singlet oxygen production efficiency, which can effectively carry out photooxidation after combining with Aβ aggregates, effectively reducing the degree of aggregation, and the cytotoxicity of Aβ aggregates was significantly reduced after bright light oxidation through cell experiments, which has great potentials in the treatment of Aβ photooxidation. On the other hand, probes Q16 and Q18 with N, N-dimethylaminophenyl as donors had excellent imaging abilities, good affinities for Aβ (Kd-Q16 = 26.95 nM, Kd-Q18 = 21.56 nM), can effectively image Aβ plaques in AD mouse brain slices and monitor the change of cell viscosity, and have a good application prospect in fluorescence imaging.

OA18 Takayasu arteritis mimicking pulmonary embolism: a case series

Abstract Background/Aims Takayasu arteritis (TA) is a rare autoimmune large vessel vasculitis with estimated incidence of 0.7-1.5 per million per annum in Europe. Conversely, pulmonary embolism (PE) is common with incidence estimates of 115 per 100,000 per year. Whilst TA is rare, it can have serious sequelae, including pulmonary vasculitis (PV), resulting in stenoses, aneurysm formation and pulmonary hypertension (PAH). Those with PV may have higher rates of PAH, with estimates around 42%. Early diagnosis ensures early treatment/immunosuppression to minimise arterial injury. Given the rarity of TA with PV, it can be misinterpreted on imaging as a PE. This study aimed to review initial imaging features of TA patients with confirmed PV to identify those who had been incorrectly diagnosed as having a PE. Subsequently, features to differentiate PE and PV were assessed. Methods We performed a retrospective analysis of all 158 patients in the Imperial College TA Cohort to identify those with PV. Records and imaging from these patients were reviewed including initial pulmonary vascular imaging. Results 9 patients with PV were identified, of which 3 had initially been misdiagnosed as having a PE, thereby delaying TA treatment and possibly resulting in accelerated disease course. Table 1 summarises initial presenting features. All patients received anticoagulation for PE; however, their symptoms did not improve. Details of subsequent imaging are included in the table. They each experienced a delay in diagnosis of 7 years, 3 years, and 6 months respectively. Conclusion Whilst TA is rare, it is an important diagnosis and not to be missed. Where clinical features are not fitting with a diagnosis of PE, or symptoms are persisting despite management, consider further investigation. Early diagnosis of TA can aid management to try and prevent long term sequelae. Important criteria that should raise suspicion and may suggest TA, some of which were seen in our patient cohort, could include: more insidious onset of symptoms and persistent symptoms despite treatment; normal saturations on observations; normal D-dimer results and a raised ESR/CRP. In the event of clinical doubt for PE, further investigations (ESR) and imaging (MR pulmonary angiogram) should be considered. Disclosure S. Kostich: None. S. Lee: None. S. Babar: None. T.A. Youngstein: None. J. Mason: None. A. Porter: None.

Based on the prognosis model of immunogenes, the prognosis model was constructed to predict the invasion of immune genes and immune cells related to primary liver cancer and its experimental validation

BackgroundPrimary liver cancer (PLC) is a prevalent malignancy of the digestive system characterized by insidious symptom onset and a generally poor prognosis. Recent studies have highlighted a significant correlation between the initiation and prognosis of liver cancer and the immune function of PLC patients. PurposeRevealing the expression of PLC-related immune genes and the characteristics of immune cell infiltration provides assistance for the analysis of clinical pathological parameters and prognosis of PLC patients. MethodsPLC-related differentially expressed genes (DEGs) with a median absolute deviation (MAD > 0.5) were identified from TCGA and GEO databases. These DEGs were intersected with immune-related genes (IRGs) from the ImmPort database to obtain PLC-related IRGs. The method of constructing a prognostic model through immune-related gene pairs (IRGPs) is used to obtain IRGPs and conduct the selection of central immune genes. The central immune genes obtained from the selection of IRGPs are validated in PLC. Subsequently, the relative proportions of 22 types of immune cells in different immune risk groups are evaluated, and the differential characteristics of PLC-related immune cells are verified through animal experiments. ResultsThrough database screening and the construction of an IRGP prognosis model, 84 pairs of IRGPs (P < 0.001) were ultimately obtained. Analysis of these 84 IRGPs revealed 11 central immune genes related to PLC, showing differential expression in liver cancer tissues compared to normal liver tissues. Results from the CiberSort platform indicate differential expression of immune cells such as naive B cells, macrophages, and neutrophils in different immune risk groups. Animal experiments demonstrated altered immune cell proportions in H22 tumor-bearing mice, validating findings from peripheral blood and spleen homogenate analyses. ConclusionOur study successfully predicted and validated PLC-related IRGs and immune cells, suggesting their potential as prognostic indicators and therapeutic targets for PLC.

A Continuous Red Eye: Case Report of Indirect Carotid Cavernous Fistula

Abstract&#x0D; Introduction : Carotid cavernous fistula (CCF) is a type of arteriovenous shunt in the cavernous sinus. Indirect CCF symptoms are not typical that can outwit us. Ocular manifestations occur because of venous and arterial stasis around the eye and orbit. The aim of this report is to increase awareness of the existence of other causes if there is no improvement after treatment.&#x0D; Case Illustration : A 59-year-old male presented with prolong conjunctival hyperemia on both eyes for two months that been diagnosed and treated as conjunctivitis. The ophthalmologic examination showed dilated episcleral vessels on both eyes with no evidence of infection and inflammation, this became a suspicion about the existence of a suppression from retro orbital region. The magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) brain examination were performed anda right CCF was diagnosed. The patient was referred to interventional neurology, digital subtraction angiography (DSA) examination showed a fistula in the right cavernous sinus and embolization was conducted.&#x0D; Discussion : The patient categorized as spontaneous, low flow, indirect CCF Barrow type D. On this type of CCF conjunctival injection is often the most prominent feature, and patients are commonly treated for other conditions, such as conjunctivitis, before a correct diagnosis is made.&#x0D; Conclusion : Carotid cavernous fistula can be characterized by the insidious onset of symptoms, diagnosis is based on the angiographic examination. When a sign suggestive of orbital or cavernous sinus pathology is present, a more thorough examination is crucial. Early diagnosis and appropriate management are essential to avoid vision and life threatening complications.

Clinical and Prognostic Implications of Cardiopulmonary Exercise Stress Echocardiography in Asymptomatic Degenerative Mitral Regurgitation

The current guidelines recommend intervention in severe degenerative mitral regurgitation (MR) in symptomatic patients or asymptomatic patients with left ventricular dilatation or dysfunction. The insidious onset of symptoms may mean that patients do not report their symptoms. The role of systematic exercise testing for symptoms in MR is not clearly defined. A total of 97 patients with moderate to severe asymptomatic MR underwent exercise echocardiography combined with cardiopulmonary exercise testing. The predictors of exercise-induced dyspnea, symptom-free survival, and mitral valve intervention were identified. A total of 18 patients (19%) developed limiting dyspnea on exercise. Spontaneous symptom-free survival at 24months was significantly higher in those without exercise-induced symptoms than those with exercise-induced symptoms, p <0.0001. The only independent predictors of spontaneous symptoms at 2years were effective regurgitant orifice area (odds ratio 27.45, 95% confidence interval [CI] 1.43 to 528.40, p=0.03) and exercise-induced symptoms (odds ratio 11.56, 95% CI 1.71 to 78.09, p=0.01). The only independent predictor of surgery was indexed left ventricular systolic volumes (odds ratio 1.17, 95% CI 1.04 to 1.30, p=0.006). Where only the patients who underwent surgery due to symptoms were included, the only independent predictor was exercise-induced symptoms (odds ratio 13.94, 95% CI 1.39 to 140.27, p=0.025). In conclusion, in patients with primary asymptomatic degenerative MR, 1/5 develop revealed symptoms during exercise. This predicts a subsequent development of spontaneous symptoms and mitral valve intervention due to symptoms.

Clinical and prognostic implications of cardiopulmonary exercise stress echocardiography in asymptomatic degenerative mitral regurgitation

Abstract Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): The Saudi Arabian Cultural Bureau. Background Current guidelines recommend intervention in severe degenerative mitral regurgitation (MR) in symptomatic patients or asymptomatic patients with left ventricular dilatation or dysfunction. The insidious onset of symptoms may mean patients do not report symptoms. The role of systematic exercise testing for symptoms in MR is not clearly defined. Methods 97 patients with moderate to severe, asymptomatic MR underwent exercise echocardiography combined with cardiopulmonary exercise testing. Predictors of exercise-induced dyspnoea, symptom-free survival and mitral valve intervention were identified. Results 18 (19%) patients developed limiting dyspnoea on exercise. Spontaneous symptom free survival at 24 months was significantly higher in those without exercise-induced symptoms compared to those with exercise-induced symptoms, p &amp;lt;0.0001. The only independent predictors of spontaneous symptoms at 2 years were effective regurgitant orifice area (odds ratio 27.45 (95% CI 1.43 – 528.40), p = 0.03) and exercise-induced symptoms (odds ratio 11.56 (95% CI 1.71 – 78.09), p = 0.01). The only independent predictor of surgery was indexed left ventricular systolic volumes (Odds ratio 1.17 (95% CI 1.04 – 1.30), p = 0.006). Where only patients undergoing surgery due to symptoms were included, the only independent predictor was exercise-induced symptoms (Odds ratio 13.94 (95% CI 1.39 – 140.27), p = 0.025). Conclusion In patients with primary asymptomatic degenerative MR, one fifth develop symptoms on exercise. This predicts subsequent development of spontaneous symptoms and mitral valve intervention due to symptoms.

Primary synovial chondromatosis of the hip joint (PrSC of the hip): A retrospective cohort analysis and review of the literature

IntroductionPrimary synovial osteochondromatosis (SOCM) or Synovial chondromatosis (SC) of the hip is a benign metaplastic condition of the synovium that is rare and may present with a spectrum of clinical features and radiological findings. Patients and methodsA retrospective search using the keyword ‘Synovial chondromatosis’ (SC) of the hip was performed at a tertiary care orthopaedic referral centre in the UK and a hospital in India. The radiology images were collected from our Picture Archiving and Communication System (PACS) and Radiology Information System (RIS), over 15 years. The patient's data was collated with Electronic Patient Records (EPR), RIS, and correlated with histo-pathology laboratory records where available. The demographic details of the patients, their clinical symptoms, imaging details, and management outcomes were collected. ResultsWe found 15 cases, with a mean age of 36.53 years (range: 14–50 years). There were 9 male and 6 female patients. The follow-up ranged from 1 year to 6 years. Predominantly unilateral presentation with insidious onset of symptoms was found. A spectrum of radiological Imaging was undertaken. Management strategies included supervised observation, arthroscopic or open synovectomy, and hip arthroplasty. No malignant transformation was found in the analysed cohort. ConclusionPrimary ‘Synovial chondromatosis’ of the hip had a male preponderance in our cohort, presenting with a range of clinical features. Radiologically, Magnetic Resonance Imaging (MRI) was the commonest modality of cross-sectional imaging utilised and crucial for the diagnosis, evaluating underlying articular involvement including guiding appropriate patient management presenting with Primary ‘Synovial chondromatosis’ of the hip.

109. The impact of patient characteristics on outcomes of surgically managed vertebral osteomyelitis in the United States: insights from a national database study

<h3>BACKGROUND CONTEXT</h3> Vertebral osteomyelitis (VO) is a potentially devastating spinal infection associated with a significant morbidity burden and a mortality rate. The incidence of VO is increasing and its diagnosis is often delayed due to its characteristic non-specific, insidious onset of symptoms (recalcitrant back pain). Nonsurgical management consisting of a 6-week course of antibiotics with optional bracing remains first line treatment for VO; however, surgical intervention is often required. At present, data are limited on the epidemiology of surgical management in VO in the United States as previous reports are based on older studies with small sample sizes, mostly from Europe or Asia Pacific. Furthermore, no study to date has evaluated the impact of patient characteristics on surgical outcomes and complications on a large scale. <h3>PURPOSE</h3> To analyze the characteristics of patients who have VO requiring surgical intervention. <h3>STUDY DESIGN/SETTING</h3> National database study. <h3>PATIENT SAMPLE</h3> This study included 228,044 patients with vertebral osteomyelitis from 1998 to 2013 (44,401 surgically managed). <h3>OUTCOME MEASURES</h3> (1) Patient demographics: age, sex, race, Deyo Index (DI - a measure of comorbidity), type of comorbid pathology; (2) hospitalization data: length of stay (LOS), hospitalization charges, insurance provider; (3) surgical procedure type and utilization; (4) complication and mortality rate. <h3>METHODS</h3> A review of the National Inpatient Sample (NIS) database was performed for all patients admitted in the United States between 1998 to 2013 with a diagnosis of VO. Patients were then sub-stratified into those who underwent surgical intervention vs those treated with nonsurgical management. Patient demographics, hospitalization data, surgical procedure type and utilization, and complication and mortality rate were compared between groups. Differences between patient demographics for the surgical and nonsurgical groups were assessed using independent sample t-tests, linear regressions, and ANOVA. <h3>RESULTS</h3> A total of 228,044 patients were estimated to be admitted in the United States for VO from 1998 to 2013, of which 44,401 patients underwent surgical intervention. The incidence of surgical intervention for patients who had VO significantly increased from 0.6 per 100,000 U.S. persons in 1998 to 1.1 per 100,000 US persons in 2013. Compared to the nonsurgically managed cohort, surgically managed VO patients were significantly p <0.001) younger (56 surgical vs 60 non-surgical), had a lower mean DI (0.88 vs 1.33) and lower hospital mortality rate, but experienced a significantly (p <0.001) longer length of hospitalization (9.01 vs 9.98 days) and higher total hospitalization charges. Regardless of surgical approach, surgery of the thoracic spine carried the highest complication and mortality rate (49% and 3.4%, respectively). Anterior approach to the cervical spine was associated with the lowest rate of any complication (10.2%). Surgical management of VO of the lumbar spine had the lowest mortality rate for all approaches: anterior (0.3%), posterior (0.2%), or combined (0.1%). Congestive heart failure and liver disease most significantly predicted mortality (OR: 6.1 for CHF and OR: 2.9 liver disease) and renal disease most significantly predicted increased total hospital costs (OR: 7.4). The mean inflation adjusted total hospital costs increased from $20,355 per patient in 2001 to $39,991 in 2013. <h3>CONCLUSIONS</h3> VO is an understudied condition with a steady increase in its incidence in the US. It appears that the rate and the need for invasive procedures to salvage this condition is also increasing. This study identified certain patient demographics, comorbidities, and surgical approaches associated with higher complications, mortality rate and overall costs. <h3>FDA DEVICE/DRUG STATUS</h3> This abstract does not discuss or include any applicable devices or drugs.

Case Report of Bone Metastases from Esophageal Cancer

Esophageal cancer is the eighth most common cancer worldwide. The most common sites of distant metastasis of esophageal cancer are the liver, distant lymph nodes, and the lungs. Metastasis to the bone is uncommon and the prognosis is poor. The authors describe a case of a 54-year old-man whose presentation of back pain with neuropathy and hoarseness of his voice led to the diagnosis of esophageal cancer with metastasis to his spine. Due to the patient's poor prognosis, the patient was referred to oncology for radiation and chemotherapy in addition to pain management. This unique case presents the insidious onset of symptoms associated with spinal metastasis.

Type VII Tibial Tubercle Fractures are Different from Osgood Schlatter Syndrome and Tibial Tubercle Physeal Fractures

Introduction:Children and adolescents are prone to both overuse and acute injuries at the tibial tubercle. The most common overuse injury is Osgood Schlatter Syndrome (OSS,) and the most common acute severe injury is tibial tubercle avulsion fracture (TTF). Here we present evidence for a Type VII, all-epiphyseal, tibial tubercle fracture (VIITF) that is different from OSS and TTF.Methods:This IRB–approved study involved patients that were treated and registered at a tertiary care pediatric hospital from 2014 to 2020. We compared the clinical information, the radiographs, the treatments, and the outcomes of 15 patients with VIITF to 19 patients with OSS and to 14 patients with TTF.Results:All VIITF patients had a sudden onset of pain associated with a specific sports-related injury, and all had lateral radiographs showing a chip-like ossific fragment displaced from the secondary center but with no involvement of the physis. All of the OSS patients had an insidious onset of symptoms with no specific injury, and none had radiographs showing a chip-like fragment. All the TTF patients had a sudden onset of pain after an acute injury. Eleven of the 15 VIITF patients (73%) have formed retro-patellar tendon ossicles, and 7 of those patients (46%) have undergone surgery. All but one of the OSS patients have resolved, and all with non-operative management. All the TTF patients have resolved, and all except those with Ogden type IA fractures, had undergone urgent surgery.Conclusions:We accepted our hypothesis. VIITF is different from OSS and TTF and can be differentiated on the basis of clinical and radiographic data.Clinical Relevance:Most patients with OSS resolve with non-operative management, and TTF patients usually require urgent surgery. Children with VIITF do not need urgent surgery but do need to be followed because some can form painful retro-patellar tendon ossicles, have focal anterior knee pain, and may need surgery to resolve their symptoms. Level of Evidence: Level III: Case Control Study.

Guided Growth for Tibial Recurvatum.

Aim and objectiveSagittal guided growth of the distal anterior femur has been shown to be effective for the correction of fixed knee flexion deformity that is encountered in clinical practice. The opposite deformity, namely genu recurvatum, is comparatively uncommon in children. The most common aetiology is post-traumatic. Acute correction by means of osteotomy has significant associated risks. Our objective was to determine whether a posterior 8-plate would suffice in correcting tibial recurvatum and obviate the need for an osteotomy.Materials and methodsWe included a total of five deformities, three boys (one bilateral) and one girl, managed by means of tethering of the posterior proximal tibial physis with a tension band plate. Standard radiographs obtained preoperatively and at follow-up included a standing anteroposterior (AP) of the legs noting limb lengths and the mechanical axis. We also obtained standing lateral views of each knee in maximal extension to measure and compare the posterior proximal tibial angle (PPTA).ResultsThe same-day surgery was well tolerated and there were no surgical or post-operative complications. The preoperative PPTA ranged from 106° to 117° and averaged 84° at follow-up. Correction occurred in an average of 20 months (range of 18–24 months). The patient with bilateral recurvatum due to Hurler's syndrome developed unilateral recurrent recurvatum culminating in percutaneous reinsertion of the metaphyseal screw. For each patient, knee hyperextension and associated pseudo-laxity resolved and limb lengths remained equal at follow-up.ConclusionChildren with progressive genu recurvatum typically present with an insidious onset of symptoms. Guided growth of the posterior proximal tibia is a safe and effective means of correcting the deformity; osteotomy was avoided in this series.Level of evidenceIII – retrospective case series – no controls.How to cite this articleStevens P, Stephens A, Rothberg D. Guided Growth for Tibial Recurvatum. Strategies Trauma Limb Reconstr 2021;16(3):172–175.

Successful surgical repair of inguinal hernia causing positional obstructive uropathy: A rare cause of renal graft dysfunction - Case report

Bladder herniation with obstructive uropathy is a relatively rare occurrence, however, urinary bladder herniation along with ureteroneocystostomy and leading to positional obstructive uropathy of the graft is an unusual presentation. This is a unique case of a 38-year-old male who presented with inguinal hernia with raised serum creatinine who had undergone renal transplant 10 years back in 2011. Magnetic resonance urogram revealed herniation of urinary bladder and hydroureteronephrosis of transplant kidney. Patient is managed by surgical exploration through inguinal incision and reduction of ureteroneocystostomy herniation after ureterolysis and hernioplasty was performed. The cause of obstructive uropathy was herniation of the bladder along with ureteroneocystostomy, and the patient has an insidious onset of symptoms, immediate exploration, and repair of hernia done without any need for percutaneous nephrostomy. Importance of this case report is that sliding herniation of the bladder with ureteroneocystostomy should be considered a diagnosis and investigated if the patient presents with positional obstructive uropathy with inguinal hernia, leading to early identification and salvaging the graft function.

Neuromyelitis Optica spectrum disorders in the elderly

Introduction. Neuromyelitis Optica is a severe autoimmune inflammatory demyelinating disease that affects mainly the spinal cord and optic nerves with a young female predilection. It can present with a severe neurological presentation from the beginning with a poor prognosis. However, it is rare to see it in elderly subjects with aggressive and insidious onset of symptoms. Objective. Our cases highlight the varied clinical presentations of NMOSD and we believe that the diagnosis of NMOSD should be considered in elderly patients in the appropriate clinical setting despite the presence of unconventional symptoms.

BIRD FANCIER'S DISEASE: A CASE OF HYPERSENSITIVITY PNEUMONITIS

TOPIC: Occupational and Environmental Lung Diseases TYPE: Medical Student/Resident Case Reports INTRODUCTION: The immunologic lung disease through inhalation and sensitization of an aerosolized antigen is termed hypersensitivity pneumonitis (HP). Here we describe a case of HP from exposure to avian antigen. CASE PRESENTATION: A 42 y/o F presented with progressively worsening dyspnea at rest & exertion, pleuritic chest pain, dry cough for past 2 months. She denied sick contacts or recent travel. On exam BP: 122/76mmHg, HR 107bpm, RR 30bpm, SpO2 82% on room air. Physical exam, significant for b/l crackles. Labs: WBC 16.33 k/mm3, ABG PaO2 of 45mmHg. CXR showed b/l airspace opacities & CT-thorax showed b/l diffuse GGO. ECHO showed LVEF 55% with normal global systolic function with mildly elevated PASP. Treatment was initiated with hi-flo oxygen and her SpO2 improved to 97%. QuantiFERON Gold, urine legionella, Mycoplasma IgM, and respiratory multiplex viral panel were negative. Cardiac CT revealed patent coronaries. Spirometry revealed severe restrictive ventilatory defect. Further questioning revealed that patient got parakeets a few months prior to onset of symptoms. She was then started on methylprednisolone for possible HP. Bronchoscopy with BAL and transbronchial biopsies showed no bacterial/fungal/viral growth. Cytology was unremarkable and biopsies revealed mild inflammatory changes with lymphocytes and aggregates of intralaveolar foamy macrophages. The CD4:CD8 ratio was 0.89. Patient clinically improved with steroid therapy and gradually weaned from supplemental oxygen. She was discharged on oral prednisone with close follow up where she continued to improve after removal of her parakeets DISCUSSION: HP an immune-mediated response from repeated inhalation, sensitization, & hyperresponsiveness to various organic particles which can be mammalian or avian proteins, fungi, bacterial, or chemicals. Based on duration of exposure & symptoms onset, HP can be divided into acute, subacute, or chronic. Acute HP presents as flu-like illness, subacute HP has insidious onset of symptoms, dyspnea on exertion or reduced exercise capacity. Physical exam reveals inspiratory crackles. Imaging usually demonstrates GGO, mosaic attenuation and centrilobular nodules. BAL reveals lymphocytic predominant cells with CD8 alveolitis. Histopathology usually demonstrates cellular bronchiolitis with predominant lymphocytic or plasmocytic infiltrates. It can also present as interstitial mononuclear infiltrates or loosely-formed granulomas. Treatment for HP is avoidance of antigen exposure. Role of steroids in treating HP is not well known, however general practice is slow tapering steroid regimen for about 3-6 months duration. Prognosis is usually good with complete recovery in most patients; a few may progress to chronic fibrosing HP. CONCLUSIONS: A high level of suspicion should be present in diagnosing HP with a thorough history and prompt management must be initiated to prevent progression of disease. REFERENCE #1: Selman M, Pardo A, King TE Jr. Hypersensitivity pneumonitis: insights in diagnosis and pathobiology. Is J Respir Crit Care Med. 2012; 186:314-24. REFERENCE #2: Costabel U, Bonella F, Guzman J. Chronic hypersensitivity pneumonitis. Clin Chest Med. 012; 33:15163. REFERENCE #3: Camarena A, Juárez A, Mejía M, Estrada A, Carrillo G, Falfán R, Zuñiga J, Navarro C, Granados J, Selman M. Major histocompatibility complex and tumor necrosis factor-alpha polymorphisms in pigeon breeder's disease. Am J Respir Crit Care Med. 2001;163:1528-33. DISCLOSURES: no disclosure on file for Jagadish Akella; No relevant relationships by Alex Diaz, source=Web Response no disclosure on file for Iqbal Javed; No relevant relationships by Raghavendra Sanivarapu, source=Web Response

Could New Generations of Sensors Reshape the Management of Parkinson’s Disease?

Parkinson's disease (PD) is a chronic neurologic disease that has a great impact on the patient’s quality of life. The natural course of the disease is characterized by an insidious onset of symptoms, such as rest tremor, shuffling gait, bradykinesia, followed by improvement with the initiation of dopaminergic therapy. However, this “honeymoon period” gradually comes to an end with the emergence of motor fluctuations and dyskinesia. PD patients need long-term treatments and monitoring throughout the day; however, clinical examinations in hospitals are often not sufficient for optimal management of the disease. Technology-based devices are a new comprehensive assessment method of PD patient’s symptoms that are easy to use and give unbiased measurements. This review article provides an exhaustive overview of motor complications of advanced PD and new approaches to the management of the disease using sensors.

Very late onset neuromyelitis optica spectrum disorders.

Neuromyelitis optica spectrum disorder (NMOSD) often presents in the elderly with an insidious onset of symptoms and aggressive progression. There have been anecdotal cases of very late onset (VLO)-NMOSD, but case series reports are rare. The aim of this retrospective study was to clarify the clinical features of VLO-NMOSD. According to the age at onset, we classified patients with NMOSD into three subgroups: ≤49years, early onset NMOSD (EO-NMOSD); 50-69years, late onset NMOSD (LO-NMOSD); and ≥70years, VLO-NMOSD. We evaluated the clinical characteristics, magnetic resonance imaging (MRI) findings, laboratory data, and immunotherapies of the groups. Overall, 12 men and 64 women with a median (interquartile range) age at onset and duration of disease of 42.0 (29.0-55.8) years and 70.0 (16.3-143.0)months, respectively, were included. Eight (11%) patients had VLO-NMOSD, 22 (29%) had LO-NMOSD, and 46 (61%) had EO-NMOSD. Patients with EO-NMOSD had a significantly longer interval between episodes as well as time between the first symptom and diagnosis of NMOSD than did those with VLO-NMOSD and LO-NMOSD (p=0.046). Optic neuritis and nerve lesions on MRI were significantly less frequent in patients with VLO-NMOSD than in those with LO-NMOSD and EO-NMOSD (p=0.002 and p=0.028, respectively). In contrast, patients with VLO-NMOSD had higher nadir Expanded Disability Status Scale and Nurick scale scores and a significantly longer spinal lesion length than did those with LO-NMOSD and EO-NMOSD (p=0.029, p=0.049, and p=0.032, respectively). Patients with VLO-NMOSD tend to develop severe myelitis with long cord lesions but not optic neuritis.

Delayed Tension Pneumocephalus Following Intraoperative Cerebrospinal Fluid Leakage Repair

Tension pneumocephalus is a rare complication of endoscopic sinus surgery (ESS) that may lead to rapid neurologic deterioration. Symptoms typically develop within the first postoperative week after enough air has entered the intracranial cavity to create pressure on the dura. Furthermore, there may be a rapid onset of symptoms after a positive pressure event. However, there may be a more insidious onset of symptoms in the absence of a positive pressure event. We report a rare case of delayed tension pneumocephalus following an intraoperative cerebrospinal fluid leak repair. We discuss here the mechanisms of this complication and the diagnosis and treatment strategies of tension pneumocephalus after an ESS.

Clinical Analysis of Hospitalized Children with Primary Immune Thrombocytopenia

To investigate the factors affecting the chronicity of childhood primary immune thrombo-cytopenia (ITP) and compare the efficiency of different first-line treatment regimens. Children with ITP hospitalized in our hospital from September 2013 to October 2018 were retrospectively analyzed. Three hundred and one children (150 males and 151 females) were included in this study, with a median age of 8 (0.17-17) years old, and 110 (36.5%), 92 (30.6%), and 99 (32.9%) cases were grouped into newly diagnosed, persistent, and chronic ITP, respectively. The median of follow-up was 41.92 (1.07-74.03) months. At the end of the follow-up (October 2019), among the 202 newly diagnosed/persistent ITP children, 79 cases (59 newly diagnosed and 20 persistent ITP) achieved remission within 1 year after initial diagnosis, with a remission rate of 39.3%; 122 cases (50 newly diagnosed and 72 persistent ITP) developed chronic disease, with a chronicity rate of 60.7%; one case underwent splenectomy. In 99 cases with chronic ITP, 5 cases underwent splenectomy. Multivariable logistic regression analysis showed that, the insidious onset of symptoms (OR=3.754, 95%CI: 1.882-7.488, P=0.000) increased the risk of chronicity, while the positive antibody to anti-platelet membrane glycoprotein (OR=0.446, 95%CI: 0.224-0.888, P=0.021) might reduce the risk of chronicity. And no difference was found by the analysis of subtype of anti-platelet membrane glycoprotein (P=0.305). The efficacy of the first-line treatment of intravenous immunoglobulin (IVIG) alone or combined with steroid was better than that of steroid alone (P=0.028, 0.028), however, the efficiency was not significantly different between IVIG alone and combined with steroid (P=0.086). Insidious onset of symptoms in pediatric ITP increases the risk of chronicity, while the positive titer of anti-platelet membrane glycoprotein may reduce the risk. In the first-line treatment for the newly diagnosed/persistent children. The efficacy of IVIG alone or combined with steroid is better than that of steroid alone.

Pulmonary langerhans cell histiocytosis: Can it originate from chest trauma?

Langerhans cell histiocytosis (LCH) is a rare medical condition which is defined by abnormal accumulation of a heterogeneous population of Langerhans cells that form nodules in certain tissues such as the skin, bone, and lungs. When the lungs are involved, the disease is called pulmonary LCH (PLCH). It is well established that PLCH is frequently observed in young adult smokers with equal gender distribution. Despite the clear role of smoking in PLCH pathogenesis, being a smoker rarely induces the disease, suggesting that host-related factors, inhaled antigens, or other stress-related factors may contribute to the pathogenesis. Here, we reported two cases of PLCH which both presented after chest trauma. Based on the findings from both the cases, it is concluded that the overstimulated inflammatory response in the posttraumatic lungs might be the responsible etiology resulting in the LCH if the lungs are already damaged and more sensitive due to smoking. We suggest that the differential diagnosis of PLCH should be especially considered in young adults with sudden or insidious onset of symptoms following chest trauma.

Genotypic and phenotypic characteristics of Korean children with childhood-onset Leber's hereditary optic neuropathy.

We sought to identify the phenotypic and genotypic characteristics of Korean children with genetically confirmed Leber's hereditary optic neuropathy (LHON). The medical records of 64 genetically confirmed LHON patients were reviewed. Seventeen patients aged 13years or younger with optic atrophy with positive mitochondrial DNA (mtDNA) mutations were considered to demonstrate childhood-onset LHON. The non-childhood-onset group included 47 patients with genetically confirmed LHON who experienced disease onset later than 13years of age. The type of mtDNA mutation, visual acuity (VA), color vision, fundus photography, retinal nerve fiber layer (RNFL) thickness, and visual field were investigated. Sequence analysis of the mitochondrial genome revealed five different kinds of LHON-associated mtDNA mutations among our childhood-onset patients, including m.11778G>A (58.8%), m.3496G>T (11.8%), m.3497C>T (5.9%), m.11696G>A (5.9%), and m.14502T>C (5.9%). The mean final best-corrected VA in the childhood-onset group was better than that in the non-childhood-onset group with the value of logMAR 0.29 (0.09-0.75) vs. 0.55 (0.27-1.29) (expressed as median (interquartile range); p = 0.05). Spontaneous visual recovery was observed in 35.3% of the childhood-onset group but in only 12.8% of the non-childhood-onset group (p = 0.04). Eight patients (47.1%) showed interocular asymmetry of the disease, with two presenting true unilateral involvement of the optic nerve and the other six patients demonstrating unilateral subclinical manifestations with bilateral optic atrophy. Involvement of secondary mitochondrial mutations was confirmed in patients with childhood-onset LHON. Characteristic clinical features of childhood-onset LHON included a higher proportion of subacute or insidious onset of symptoms, better VA, higher spontaneous recovery, and asymmetrical ocular involvement.