scholarly journals Idiopathic Hypertrophic Pachymeningitis: Does Earlier Treatment Improve Outcome?

Children ◽  
2020 ◽  
Vol 8 (1) ◽  
pp. 11
Author(s):  
Emilia Rizzo ◽  
Ailsa Elizabeth Ritchie ◽  
Vinay Shivamurthy ◽  
Ata Siddiqui ◽  
Ming Lim

Background/goal: Hypertrophic pachymeningitis is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. This condition has largely been reported in adults, but there are very few reports in children. Methods: We describe a 14-year-old boy with idiopathic hypertrophic pachymeningitis, who presented with deteriorating vision on a background of severe headache. We evaluated pediatric cases of hypertrophic pachymeningitis and compared treatments and their relation to outcomes. Results: There are only eleven pediatric cases of hypertrophic pachymeningitis reported in the literature. In the patients treated with steroids either at presentation or subsequent relapses, a good response was reported. In the cases with delayed initiation of steroid treatment, this was often related to an incomplete recovery. In our patient, this delay may have contributed to his poor visual outcome. Conclusions: Early initiation of steroid treatment in children with idiopathic hypertrophic pachymeningitis may improve outcomes.

1997 ◽  
Vol 33 (5) ◽  
pp. 461-468 ◽  
Author(s):  
PR Hess ◽  
RK Sellon

Syndromes of steroid-responsive meningitis have been described in the dog and typically are characterized by neutrophilic pleocytosis and an elevated protein concentration of the cerebrospinal fluid. In a minority of cases, histopathology has demonstrated suppurative leptomeningeal (i.e., arachnoid and pia) inflammation. A case of compressive, cervical, pyogranulomatous inflammation of undetermined cause affecting the dura mater (i.e., pachymeningitis), accompanied by fever and hyperpathia, is presented. The pachymeningitis ultimately regressed with long-term immunosuppressive therapy. This case shares features with hypertrophic spinal pachymeningitis of humans, an uncommon, frequently idiopathic, chronic inflammatory disorder causing dural hypertrophy, radiculopathy, and spinal cord compression.


2010 ◽  
Vol 2010 ◽  
pp. 1-9 ◽  
Author(s):  
Saveria Pastore ◽  
Liudmila Korkina

The skin is permanently exposed to physical, chemical, and biological aggression by the environment. In addition, acute and chronic inflammatory events taking place in the skin are accompanied by abnormal release of pro-oxidative mediators. In this paper, we will briefly overview the homeostatic systems active in the skin to maintain the redox balance and also to counteract abnormal oxidative stress. We will concentrate on the evidence that a local and/or systemic redox dysregulation accompanies the chronic inflammatory disorder events associated to psoriasis, contact dermatitis, and atopic dermatitis. We will also discuss the fact that several well-established treatments for the therapy of chronic inflammatory skin disorders are based on the application of strong physical or chemical oxidants onto the skin, indicating that, in selected conditions, a further increase of the oxidative imbalance may lead to a beneficial outcome.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 540.3-540
Author(s):  
A. Munir ◽  
C. Sheehy

Background:Corneal melt is a rare inflammatory disease of the peripheral cornea; it may lead to perforation of the globe and visual failure. Corneal melt can be a manifestation of systemic vasculitis in patients with RA and other conditions, such as cancer. Without early and aggressive treatment it may be associated with a poor visual outcome and a high mortality. It has been reported in patients with stable RA.Objectives:A case report in a patient with long standing but well controlled Rheumatoid Arthritis (RA) and metastatic disease.Methods:A 75 year old male with a background of sero positive Rheumatoid Arthritis for more than 10 years presented to the Eye Casualty with a two week history of a painful left red eye. His other medical history was significant for Stage IIB poorly differentiated cancer of the left lower lobe. Left lower lobectomy with a patch of diaphragm resected. Intratumoural lymphovascular invasion noted. He completed Adjuvant Carboplatin/Vinorelbine chemotherapy September, 2017. He had DVT proximal left leg 22ndof September, 2017. Follow up CT in 2018 demonstrated a right renal upper pole lesion for which he was awaiting biopsy with?metastatic lung disease vs primary renal carcinoma. His RA was well controlled on Methotrexate 10mg weekly. He had been treated by the ophthalmology team for left marginal Keratitis for the prior 2 months with steroid eye drops without significant improvement. On presentation to ED, he described sharp eye pain, waking him from the sleep, associated with watery discharge and photophobia. Examination showed corneal melt in left eye involving 25% of inferior portion of the cornea and spastic entropion with injecting eye lashes. He had no active joints and there were no other signs of vasculitis. CRP was 4.1. He had a negative ANA and ANCA; viral swabs were negative. He was admitted under the medical team. Intravenous Methyl Prednisolone was started. The patient felt better after 5 days of Methyl Prednisolone. Left temporary tarsorrhaphy was done by Ophthalmology. Cyclophosphamide was initiated after discussion with Oncologist pending the result of the renal biopsy. Patient was discharged after 5 days of admission in the hospitalResults:The renal biopsy was positive for metastatic Squamous cell carcinoma of lung. Cyclophosphamide was withdrawn and he was started on Carboplatin/Gemcitabine. The corneal melt improved with complete resolution of his visual symptoms.Conclusion:In this case, although the history of RA was felt by the ophthalmology team to be the most likely association with the corneal melt, we would argue the oncological diagnoses were likely the driving force behind the presentation.References:[1]Sule A, Balakrishnan C, Gaitonde S, Mittal G, Pathan E, Gokhale NS, et al. Rheumatoid corneal melt. Rheumatology (Oxford)2002;41:705–6.[2]S. Yano, K. Kondo, M. Yamaguchi et al., “Distribution and function of EGFR in human tissue and the effect of EGFR tyrosine kinase inhibition,” Anticancer Research, vol. 23, no. 5, pp. 3639–3650, 2003.Disclosure of Interests:None declared


2021 ◽  
pp. 116-123
Author(s):  
Roald Vissing-Uhre ◽  
Alastair Hansen ◽  
Susanne Frevert ◽  
Ditte Hansen

Kimura disease (KD) is a chronic, inflammatory disorder with slowly developing subcutaneous tumor-like swellings, often occurring in the head and neck region. KD is diagnosed based on histology, elevated levels of immunoglobulin type E, and increased peripheral eosinophil granulocytes. KD may coexist with glomerular renal diseases, and this case report is based on a patient with KD-associated membranous nephropathy. Patients with membranous nephropathy without KD have demonstrated responsiveness to treatment with monoclonal anti-CD20 antibodies. This case report is the first to investigate the effect of rituximab treatment in a patient with KD-associated membranous nephropathy. A 30-year-old Italian man living in Denmark was diagnosed with Kimura’s disease based on subcutaneous nodules with eosinophil angiolymphoid hyperplasia. The patient was admitted to the hospital due to nephrotic syndrome. Serology showed eosinophil granulocytosis and negative PLA2-receptor antibody. Renal biopsy showed membranous nephropathy, and the patient was treated with systemic methylprednisolone followed by cyclosporin and then cyclophosphamide with only partial remission. Ultimately, treatment with intravenous rituximab was initiated, which resulted in overall remission and no nephrotic relapses at 30 months of follow-up. Thus, intravenous rituximab effectively decreased proteinuria and prevented nephrotic relapses in a patient with treatment-refractory membranous nephropathy due to KD.


2021 ◽  
pp. 112067212110006
Author(s):  
Xin Liu ◽  
Lufei Wang ◽  
Fengjuan Yang ◽  
Jia’nan Xie ◽  
Jinsong Zhao ◽  
...  

Purpose: To describe surgical management and establish visual outcomes of open globe injury (OGI) in pediatric patients requiring vitrectomy. Methods: Forty-eight eyes of 48 pediatric patients underwent vitrectomy for OGI with secondary vitreoretinal complications in the eye center of Jilin University were included. Characteristics of patients, details of ocular examination and operation, presenting and final visual acuity were recorded. Results: Presenting visual acuity less than 20/400 was found in 44 eyes (91.7%), which included no light perception (NLP) in four eyes. At last visit, there was no eyes with visual acuity of NLP, and 19 eyes (39.6%) had a vision recovery to 20/400 or better. Mechanisms of injury, intraocular contents prolapse, presence of hyphema, intraocular foreign body, vitreous hemorrhage, retinal detachment, and total time from injury to PPV > 2 weeks were significant predictors of visual prognosis. Logistic regression analysis showed that hyphema was a significant predictive factor for poor visual outcome. Conclusion: Visual acuity was improved in most of the patients with OGI in this study. Hyphema is an important presenting ocular sign in estimating the post-vitrectomy visual outcome for OGI in children. Proper timing of vitrectomy is suggested, and in this study patients may benefit more with early vitrectomy as less proliferative vitreoretinopathy (PVR) was found together with a better visual acuity.


2021 ◽  
Vol 13 ◽  
pp. 1759720X2110069
Author(s):  
Yanpan Gao ◽  
Yanyu Chen ◽  
Lun Wang ◽  
Chen Li ◽  
Wei Ge

Objective: Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic inflammatory disorder and the underlying pathogenesis is unclear. In this study, 88 SAPHO patients and 118 healthy controls were recruited to investigate the role of serum-derived extracellular vesicles (SEVs) in SAPHO syndrome. Methods: Quantitative proteomics was applied for SEVs proteome identification, and ELISA and Western blotting was performed to verify the results of mass spectrum data. In vitro osteoclastogenesis and osteogenesis assay was used to confirm the effects of SEVs on bone metabolism. Results: Tandem mass tagging-based quantitative proteomic analysis of SAPHO SEVs revealed differential expressed proteins involved in bone metabolism. Of these, serum amyloid A-1 (SAA1) and C-reactive protein (CRP) were upregulated. Higher SAA1 levels in SAPHO patients were confirmed by ELISA. In addition, SAA1 levels were positively correlated with CRP, an inflammatory marker related to the condition of patients. In vitro celluler studies confirmed that SAPHO SEVs inhibited osteoclastogenesis in patients mainly in the active phase of the disease. Further analysis demonstrated that Nucleolin was upregulated in osteoclasts of active-phase patients under SAPHO SEVs stimulation. Conclusion: In this study, we identified SAA1 as an additional inflammation marker that can potentially assist the diagnosis of SAPHO syndrome, and speculated that Nucleolin is a key regulator of osteoclastogenesis in active-phase patients.


2005 ◽  
Vol 219 (6) ◽  
pp. 366-372 ◽  
Author(s):  
Chang-Sue Yang ◽  
Der-Chong Tsai ◽  
Fenq-Lih Lee ◽  
Wen-Ming Hsu

2011 ◽  
Vol 26 (S2) ◽  
pp. 229-229
Author(s):  
F. Maner ◽  
Ö. Şahmelikoğlu ◽  
Ö. Hısım ◽  
H. Özhan ◽  
H. Sarıahmetoğlu ◽  
...  

IntroductionBehcet's Syndrome is a chronic inflammatory disorder of unknown etiology, characterized by aphthous lesions and recurrent ulceration of the mouth, genitals and uveitis.ObjectivesThe central nervous system is involved in about 20% of cases.AimsOnly few reports deal with affective symptoms associated with Behcet's syndrome.MethodsWe report a case of a 43 year old male with Neuro-Behcet's Syndrome that presents with a psychotic manic attack. He developed Behcet's Syndrome at the age of 23, with recurrent uveitis and aphthous lesions in the mouth, painful ulcers in the genitalia and erythema nodosum. HLA-B 5 was positive.ResultsHe was treated with azothioprine 150 mg/day for 13 years and prednole 100 mg/day during uveitis attacts for a week. At the age of 37 a sudden occurrence of right hemiparesia due to cerebrovascular accidence salicylic acid 100mg/day, siclosporine 150 mg/day, piracetame 1600mg/day were administered. He presented to psychiatry clinic in manic episode with euphoric mood, psychomotor agitation, talkativeness, decreased need for sleep, excessive buying and he had an unrealistical thought that he was a player of a famous soccer team. He was diagnosed as bipolar I disorder, according to DSM-IV. This was the patient's first admission and the symptoms which were continuing for 6 years exaggerated during uveitis attacks.Psychiatric examination releaved that increaced psychomotor activity, hypomaniac affect, amount and affect speed of speech affect, increased associations, grandiose delusions.ConclusionThere are a few reports dealing with bipolar disorder as an entity related to Behcet's syndrome.


2021 ◽  
Vol 19 (1) ◽  
pp. 17-20
Author(s):  
Raghu K C ◽  
◽  
Nagesh R ◽  
Viswash G K ◽  
◽  
...  

Background: Ankylosing spondylytes is a chronic inflammatory disorder characterized by inflammation in spines and spinal arthritis with a complex polygenic aetiology. The disease is more common in young males and risk factors include both genetic and environmental. Anesthesia management for ankylosing spondylitis is a challenge due to management of difficult airway, respiratory and cardiovascular complications, as well as the medications for disease and pain control. Both airway management and neuraxial access may prove to be difficult. Awake fibreoptic intubation is the safest option (²) in these patients with a potentially difficult airway as it allows continuous neurological monitoring while achieving a difficult airway. Methods: This is a Prospective Randomized Double-Blind Study conducted in Sri Sathya Sai Institute of Higher Medical Sciences; Total 70 Patients (Group A – 35, Group A – 35). All the subjects included after informed consent, blood samples and urine samples are collected from the all the subjects. Hb, RBCs, WBCs and Platelets was measured by laboratory standard methods. Along with Chest X- ray and ECG-for patients over 40 years of age. Results: This study was evaluated that in ankylosing spondylitis cases most of the physicians prefer to give general anaesthesia because to prevent trauma to the spinal cord but in these cases spine and surrounding tissues also it will involve at that time for maintain airway to the patient is challenge to the physicians by using fibreoptic intubation is good way to approach and maintain airway to the ankylosing patients. Conclusion: In this study suggest that in ankylosing spondylitis patients during surgery in place of tracheal intubation fibreoptic intubation is the best way to maintain airway to the patients and also we can prevent spinal cord damage.


Author(s):  
David Nugraha ◽  
Natasya Ariesta Selyardi Putri ◽  
Visuddho Visuddho ◽  
Citrawati Dyah Kencono Wungu

Inflammatory bowel disease (IBD), which consists of Crohn's disease (CD) and ulcerative colitis (UC), is a chronic inflammatory disorder of the intestine. The etiology is heterogeneous and multifactorial, including genetic susceptibility, immune-mediated tissue damage, and changes of lumen microenvironment, especially short-chain fatty acid (SCFA) producing bacteria. Several studies reported a decrease in SCFA concentration in both CD and UC. In fact, SCFAs has important roles in accelerating disease remission. This systematic review aimed to evaluate the changes in SCFA concentration, the composition of SCFA-producing bacteria, and SCFA metabolism in IBD. A literature search was conducted via PubMed, Scopus, and CENTRAL by selecting studies according to inclusion and exclusion criteria. The quality and risk of bias assessment were performed using the Newcastle-Ottawa Scale (NOS). Overall, 160 UC and 127 CD patients from 5 studies were reviewed. The SCFA concentration was significantly reduced (p <0.05) in both PC and UC. Moreover, there was a decrease in major SCFA-producing bacteria. Clostridium coccoides were significantly decreased in the feces of active UC (p = 0.015) and CD (p = 0.04). Clostridium leptum was decreased on intestinal mucosal biopsy of active CD and UC (p <0.0001). Faecalibacterium prausnitzii were decreased in active CD faeces (p <0.0001) and UC (p = 0.0001). Butyrate oxidation rate was also reported to decrease in UC compared to control (p<0.0001). In conclusion, the ability of major SCFA-producing bacterial production in IBD was diminished, which implies a decreased protective and anti-inflammatory effect of SCFA that altered its metabolism.


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