scholarly journals A Toddler with Juvenile Ocular Myasthenia Gravis: Clinical Experience

2021 ◽  
Vol 8 (3) ◽  
pp. 412-415
Author(s):  
Alifiani Hikmah Putranti ◽  
Rr.Kartika Dwi Septieningtyas

Background:Myasthenia gravis is an extremely rare  autoimmune disorder affecting the neuromuscular junction. The incidence rate is 0.9-2.0 cases per 1 million children per year.Ocular myasthenia gravispresents as ptosis with extraocular motility restriction and is prone to be misdiagnosed as third nerve palsy and is difficult to diagnose in very young children. Case: A girl aged 2 years 6 months with clinical features with bilateral ptosis and was diagnosed as juvenile ocular myastenia gravis  based on history, physical examination and other diagnostic proceduressuch as chest X-raywithin normal limit and no thymoma, the ice test showed positive result, electromyography (EMG) showed decrement response >10%,progstigmin test showed positive result,  andserum acetylcholine receptor antibody levels was 0.43 nmol/L (reference range : positive as >0.40 nmol/L). Conclusion:Juvenile ocular myastenia gravis diagnostics can be established using simple examinations such as ice tests,prostigmin test to sophisticated examinations as systemic acetylcholinesterase antibodies. Management begins with a first-line drug, pyridostigmine, that is safe and effective. Disease monitoring and looking for etiology are very important for successful treatment.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 904.1-904
Author(s):  
P. Vandormael ◽  
A. Pues ◽  
E. Sleurs ◽  
P. Verschueren ◽  
V. Somers

Background:Rheumatoid arthritis (RA) is an autoimmune disorder that is characterized by chronic inflammation of the joint synovium and presence of autoantibodies in most patients. For RA, many treatments are currently available but each treatment will only induce disease remission in a subset of patients. Moreover, finding out which patients respond well to first-line therapy with classical synthetic disease modifying anti-rheumatic drugs (csDMARDs), still largely depends on trial and error.Objectives:In this study, we aim to find novel RA autoantibody biomarkers that predict therapy response to csDMARDs before the initiation of treatment.Methods:In the CareRA trial, a Flemish multicenter study of different treatment regimes, serum samples were collected from RA patients that did or did not show disease remission (DAS28(CRP)<2.6) in response to csDMARDs, combined with a step down glucocorticoid treatment. In our study, baseline samples, collected before the start of treatment, were used to determine predictive antibody reactivity. A cDNA phage display library, representing the antigens from RA synovial tissue, was constructed and screened for antibody reactivity in baseline serum samples of RA patients that failed to reach remission at week 16. Using enzyme-linked immunosorbent assays (ELISA), antibody reactivity against the identified antigens was initially determined in pooled baseline serum samples of RA patients that did (n=50) or did not (n=40) reach disease remission at week 16. Antigenic targets that showed increased antibody reactivity in pools from patients that did not reach disease remission, were further validated in individual serum samples of 69 RA patients that did not reach DAS28(CRP) remission at week 16, and 122 RA patients that did.Results:Screening and validation of antibody reactivity resulted in 41 novel antigens. The retrieved antigenic sequences correspond to (parts of) known proteins and to randomly formed peptides. A panel of 3 of these peptide antigens could be composed, whose baseline antibody reactivity correlated with lack of therapy response at week 16. Presence of antibodies against at least one of these 3 antigens was significantly higher in individual samples of RA patients that did not reach DAS28(CRP) remission (43 vs. 29%, p=0.041), or that failed to reach ACR 70 (42 vs. 26%, p=0.029) response criteria at week 16, compared to RA patients that did reach these respective criteria. In addition, RA patients which were positive for this antibody panel at baseline, also showed less DAS(CRP) remission at week 4 and week 8.Conclusion:We have identified a set of 3 antibody biomarkers that can predict failure of early disease remission after first-line RA therapy, which might contribute to personalized medicine decisions.Disclosure of Interests:Patrick Vandormael: None declared, Astrid Pues: None declared, Ellen Sleurs: None declared, Patrick Verschueren Grant/research support from: Pfizer unrestricted chair of early RA research, Speakers bureau: various companies, Veerle Somers Grant/research support from: Research grant from Pfizer and BMS


2021 ◽  
Vol 14 (4) ◽  
pp. 361
Author(s):  
Sarentha Chetty ◽  
Tom Armstrong ◽  
Shalu Sharma Kharkwal ◽  
William C. Drewe ◽  
Cristina I. De Matteis ◽  
...  

The emergence of multidrug-resistant (MDR) and extensively drug-resistant (XDR) tuberculosis (TB) has reinforced the need for the development of new anti-TB drugs. The first line drug isoniazid inhibits InhA. This is a prodrug requiring activation by the enzyme KatG. Mutations in KatG have largely contributed to clinical isoniazid resistance. We aimed to design new ‘direct’ InhA inhibitors that obviate the need for activation by KatG, circumventing pre-existing resistance. In silico molecular modelling was used as part of a rational structure-based drug-design approach involving inspection of protein crystal structures of InhA:inhibitor complexes, including the broad spectrum antibiotic triclosan (TCS). One crystal structure exhibited the unusual presence of two triclosan molecules within the Mycobacterium tuberculosis InhA binding site. This became the basis of a strategy for the synthesis of novel inhibitors. A series of new, flexible ligands were designed and synthesised, expanding on the triclosan structure. Low Minimum Inhibitory Concentrations (MICs) were obtained for benzylphenyl compounds (12, 43 and 44) and di-triclosan derivative (39), against Mycobacterium bovis BCG although these may also be inhibiting other enzymes. The ether linked di-triclosan derivative (38) displayed excellent in vitro isolated enzyme inhibition results comparable with triclosan, but at a higher MIC (125 µg mL−1). These compounds offer good opportunities as leads for further optimisation.


Author(s):  
Madalena Feio ◽  

Palliative sedation does not have a universally accepted definition. It is used as a measure of last resort for the control of refractory symptoms in the last days of life. The ethical principles invoked in its use are those of double effect and proportionality. Its prevalence varies according to the place of care, type of study and country. The most frequent indications for its use are the control of dyspnea, delirium and pain. The recommended first line drug is midazolam. The studies performed do not diminish the survival of the patient. It is important that fami­ly support is maintained throughout the process. Several scientific societies and medical associations have defined guidelines that regulate their implementation.


Author(s):  
Riyadh S. Almalki ◽  
Hala Eweis ◽  
Fatemah Kamal ◽  
Dina Kutbi

Methotrexate (MTX) is the most widely used drug in cancer chemotherapy and is considered to be the first-line drug for the treatment of a number of rheumatic and non-rheumatic disorders. The pulmonary toxicity, hepatotoxicity of MTX are two of its major side effects. Other toxicities such as endocrinological toxicity, GI toxicity, cutaneous toxicity, hematological toxicity, fatal malfunction or loss, and malignancy can also occur, but at a significantly lower rate of prevalence. This review aims to provide a comprehensive understanding of the molecular mechanisms of methotrexate toxic effects and Lastly, we discussed the management of this toxicity.


2016 ◽  
Vol 23 (02) ◽  
pp. 152-155
Author(s):  
Dr. Shazia Abbass ◽  
Dr. Ejaz Ahmed ◽  
Dr. Iram Shabbir
Keyword(s):  

Author(s):  
Pragati Dwivedi

Hypothyroidism is one of the fastest rising health issues in India, with the prevalence rate of 10.95% ~1 in 10 adults. Thus, it is of great need to deal with this ever-emerging disease. In Ayurveda, Hypothyroidism is often taken as Rasa - pradoshaja vyadhi and treated accordingly but the outcome is not as expected always. So, there is a need to find out other conditions which shows similar signs and symptoms as hypothyroidism and that can be considered in differential diagnosis. In Modern medicine the causes of Hypothyroidism are differentiated in deficiency, insufficiency of hormone, inefficiency of gland, autoimmune disorder, Cancer etc. In our study we will refer it with Kaphavruta udana vayu vikriti with the help of classical textual references which will be helpful in the treatment precisely. Aims & objective – 1. To evaluate co-relation between hypothyroidism with kaphavruta udana vayu vikruti 2. To evaluate action of erand sneh in kaphavruta udana vayu vikruti Methodology – All classical texts were referred like Bruhtrayi & Laghutrayi and others to evaluate the clinical correlation of hypothyroidism and kaphavruta udana vayu vikruti. Whether line of treatment mentioned in kaphavruta udana vayu vikruti shows the positive result in hypothyroidism. Conclusion –It was observed that the signs and symptoms of hypothyroidism were very much similar with kapha vruta udana vayu vikruti. Hypothyroidism can successes fully be referred as Kaphavruta udana yavu vikruti. According to textual references we can utilize Eranda Sneha Nasya & Paan in the management of hypothyroidism. Thus, it has significant role in reducing the signs and Symptoms of Hypothyroidism because of its Vata - kaphhara, srotovishodhan, Anulomana, vrushya actions. Further studies and clinical trials are essential to evaluate the efficacy of erand sneha and correlation between hypothyroidism & kaphavruta udana vayu vikriti.


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