scholarly journals Effective Early Treatment of AChR Antibody-Positive Myasthenia Gravis with Rituximab; the Experience from a Neuroimmunology Clinic in a Developing Country

2021 ◽  
Vol 13 ◽  
pp. 117957352110160
Author(s):  
Thomas Mathew ◽  
Kurian Thomas ◽  
Saji K John ◽  
Shruthi Venkatesh ◽  
Raghunandan Nadig ◽  
...  
Keyword(s):  
Observational Study ◽  
Myasthenia Gravis ◽  
Treatment Option ◽  
Early Treatment ◽  
Early Stage ◽  
Tertiary Care ◽  
Response To Treatment ◽  
Myasthenic Crisis ◽  
Achr Antibody ◽  
Significant Difference

Background: Rituximab is reserved for treating refractory myasthenia gravis (MG) patients. Here we report our experience with rituximab in AChR antibody positive generalized MG (gMG) and impending myasthenic crisis (IMC). Methods: This retrospective, observational study, conducted at a tertiary care, neuroimmunology clinic, analyzed the data of patients with AChR antibody positive gMG, treated with rituximab between 1st January 2016 and 30th October 2018. Results: Eleven patients with AChR antibody positive gMG received rituximab. Mean age of the cohort was 50.54 ± 18.71 years with 9 males. Seven out of 11 patients received rituximab in the early stage (<2 years from onset) and had good response to treatment. Four of the 5 patients with IMC improved with rituximab alone. In the 10 patients who regularly followed up, there was a significant difference between the QMG scores at baseline and at 1, 2, 6, 12, and 18 months ( P < .0001). Conclusion: Rituximab appears to be a potentially effective early treatment option for AChR antibody positive generalized MG and impending myasthenic crisis.

Adherence to the BCLC guidelines and impact on overall survival.

2014 ◽  
Vol 32 (3_suppl) ◽  
pp. 345-345 ◽  
Author(s):  
Jesna Mathew ◽  
Sasha Slipak ◽  
Anil Kotru ◽  
Joseph Blansfield ◽  
Nicole Woll ◽  
...  
Keyword(s):  
Overall Survival ◽  
Survival Benefit ◽  
Cox Regression ◽  
Early Stage ◽  
Univariate Analysis ◽  
Tertiary Care ◽  
Treatment Recommendations ◽  
Rank Test ◽  
P Value ◽  
Significant Difference

345 Background: Multiple studies exist that validate the prognostic value of the Barcelona Clinic Liver Cancer (BCLC) staging. However, none have established a survival benefit to the treatment recommendations. The aim of this study was to evaluate the adherence to the BCLC guidelines at a rural tertiary care center, and to determine the effect of following the treatment recommendations on overall survival. Methods: A retrospective chart review was conducted for 97 patients newly diagnosed with hepatocellular carcinoma (HCC) from 2000 to 2012. The treatment choice was compared with the BCLC guidelines and percentage adherence calculated. Overall survival was estimated using the Kaplan-Meier method and the log rank test was used to test the difference between the two groups. Cox regression tests were used to determine independent effects of stage, treatment aggressiveness, and guideline adherence on survival. A p-value <0.05 was considered statistically significant. Results: Of 97 patients, 75% (n=73) were male. Median overall survival was 12.9 months. In 59.8% (n=58) of the patients, treatment was adherent to stage specific guidelines proposed by the BCLC classification. There was no significant difference in overall survival between the adherent and non-adherent groups (11.2 vs 14.1 months, p<0.98). However on stage specific survival analysis, we noted a significant survival benefit for adherence to the guidelines for early stage HCC (27.9 vs 14.1 months, p<0.05), but a decrease in survival for adherence in the end stage (20 days vs 9.3 months, p<0.01). On univariate analysis, more aggressive treatment was associated with increased survival (hazard ratio [HR], 0.4; 95% confidence interval [CI], 0.22 to 0.87; p = 0.018). Multivariate analysis revealed that adherence did not independently affect survival when stage and aggressiveness of treatment were included in the model (HR, 1.3; 95% CI, 0.76 to 2.2, p = 0.34). Conclusions: Although the BCLC guidelines serve as a practical guide to the management of patients with HCC, they are not universally practiced. These results indicate that survival of patients with hepatocellular cancer is determined by stage and aggressiveness of treatment, not adherence to BCLC guidelines.

Expecting the unexpected.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e14066-e14066
Author(s):  
Blessie Elizabeth Nelson ◽  
Adrian Gerard Murphy ◽  
Jacquelyn W. Zimmerman
Keyword(s):  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
De Novo ◽  
Early Recognition ◽  
Compound Muscle Action Potential ◽  
Care Center ◽  
Tertiary Care ◽  
Sudden Onset ◽  
Shoulder Abduction ◽  
Myasthenic Crisis

e14066 Background: Myasthenia gravis is an autoimmune neuro-muscular disorder traditionally seen in bi-modal distribution in young women or older men. Discovery of immunotherapy has brought hope in survival outcomes for patients with malignant melanoma, lung, renal and head/neck cancers but it also opens Pandora’s box of immune-related toxicities for which early recognition and appropriate clinical management are paramount. Here we describe a case of immunotherapy induced myasthenia gravis de novo. Methods: A 77-year-old man with HPV+ stage IVA squamous cell carcinoma of the tongue presented with sudden onset orthopnea and dyspnea on exertion for the past day. One week ago, he received his second cycle of nivolumab as part of his neoadjuvant therapy. He was seen at an outside hospital and was found to be acute hypercapnic respiratory failure and placed on BiPAP. He was started on antibiotics for community-acquired pneumonia with levofloxacin and doxycycline and transferred to a tertiary care center for further management. On further evaluation, he endorsed diplopia, blurry vision, fluctuating muscle weakness that is worse at the end of the day, change in voice and proximal muscle weakness. His exam was consistent with bilateral ptosis, weak hip flexion and shoulder abduction, positive sniff test and poor vital capacity and negative inspiratory force values suggestive of impending respiratory and diaphragmatic failure secondary to myasthenic crisis. He was admitted to the ICU and placed on BiPAP and frequent NIF and VC monitoring. He was started on pyridostigmine but showed no clinical improvement on day 1 and hence was initiated on plasmapheresis from day 2 for a total of 10 days. Results: Investigations showed positivity of Ach-R modulating and binding and blocking antibodies with negative voltage gated calcium channel antibodies. EMG revealed decrement of the compound muscle action potential in the repetitive stimulation test indicative of myasthenia gravis. He responded well to the above treatment and underwent successful left partial glossectomy and weaned off mechanical ventilation and has been cancer free so far. He is doing well on maintenance prednisone and pyridostigmine. Conclusions: There is a significant 30.4% MG-specific-related mortality due to immunotherapy alone which this case demonstrates the importance of vigilance and early detection for effective treatment and management. It highlights need for the oncological world and our colleagues in various other disciplines of healthcare to identify and mitigate the effects of immunotherapy which has become our hope in beating cancer.

Maternal and neonatal outcomes among pregnant women with myasthenia gravis

2020 ◽  
Vol 48 (8) ◽  
pp. 793-798
Author(s):  
Laura Nicholls-Dempsey ◽  
Nicholas Czuzoj-Shulman ◽  
Haim Arie Abenhaim
Keyword(s):  
Respiratory Failure ◽  
Myasthenia Gravis ◽  
Care Center ◽  
Tertiary Care ◽  
The United States ◽  
Neonatal Outcomes ◽  
Chronic Hypertension ◽  
Significant Difference ◽  
Maternal And Neonatal Outcomes ◽  
In Pregnancy

AbstractObjectivesMyasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction marked by weakness and fatiguability of skeletal muscle. MG has an unpredictable course in pregnancy. Our purpose was to evaluate the effect of MG on maternal and neonatal outcomes.MethodsUsing the United States’ Healthcare Cost and Utilization Project Nationwide Inpatient Sample from 2005 to 2015, we conducted a retrospective cohort study consisting of women who delivered during that period. Multivariate logistic regression models, adjusted for baseline maternal demographics and comorbidities, were used to compare maternal and neonatal outcomes among pregnancies in women with and without MG.ResultsDuring the study period, 974 deliveries were to women diagnosed with MG. Women with MG were more likely to be older, African American, obese, have Medicare insurance and be discharged from an urban teaching hospital. Women with MG were also more likely to have chronic hypertension, pre-gestational diabetes, hypothyroidism, and chronic steroid use. Women with MG were at greater risk for acute respiratory failure (OR 13.7, 95% CI 8.9–21.2) and increased length of hospital stay (OR 2.5, 95% CI 1.9–3.3). No significant difference was observed in the risk of preterm premature rupture of membranes, caesarean section or instrumental vaginal delivery. Neonates of women with MG were more likely to be premature (OR 1.4, 95% CI 1.2–1.8).ConclusionsMG in pregnancy is a high-risk condition associated with greater risk of maternal respiratory failure and preterm birth. Management in a tertiary care center with obstetrical, neurological, anesthesia and neonatology collaboration is recommended.

scholarly journals Effect of metformin in treating polycystic ovarian syndrome among women attending a tertiary care setting -A prospective observational study

2021 ◽  
Vol 8 (2) ◽  
pp. 188-193
Author(s):  
Suresh Chandra Mondal ◽  
Pinaki Sarkar
Keyword(s):  
Observational Study ◽  
Prospective Observational Study ◽  
Polycystic Ovarian Syndrome ◽  
Care Setting ◽  
Statistical Significance ◽  
Tertiary Care ◽  
Reproductive Age ◽  
Significant Difference ◽  
Tertiary Care Setting ◽  
Ovarian Syndrome

To determine the efficacy of metformin on clinical profile and metabolic disorders in women with polycystic ovarian syndrome. Polycystic ovarian syndrome, is a heterogeneous endocrinological disorder affecting women of reproductive age and metformin was introduced to influence the pathogenesis.: A prospective observational study was conducted on 100 women with polycystic ovarian syndrome, attending department of gynaecology in a tertiary care setting and were divided into two group of 50 each. Metformin was used in 50 patients for one year. Rest 50 patients were advised about diet control and exercise. The parameters like body mass index (BMI), weight, hormonal imbalance, ovulation, and menstrual changes were analysed in both groups. Descriptive statistics for mean, SD were carried and chi-square test was used to test statistical significance using coGuide version V.1.0 and p value set at &#60; 0.05.: The mean age was 25±10 years. Both the groups were comparable with respect to BMI and weight. There was a significant difference between the groups with respect to fasting insulin, which reduced to 7.44±1.12 from 11.97±2mIU/ml, testosterone levels reduced to 0.59±0.1 from 0.76±0.3 mIU/ml, luteinizing hormone levels reduced to 8.55±2 from 20.01±7.3 mIU/ml, and LH:FSH ratio reduced to 1.11±0.1 from 2.31±0.2 mIU/ml. In the metformin group, 22 (44%) participants were anovulation before treatment, and ovulation started in 15 (30%) after treatment which was not seen other group.Clinical, hormonal, and biochemical changes in polycystic ovarian syndrome may effectively be treated by metformin.

scholarly journals Can endometrial volume assessment predict the endometrial receptivity on the day of hCG trigger in patients of fresh IVF cycles: a prospective observational study

2018 ◽  
Vol 7 (4) ◽  
pp. 1523
Author(s):  
Neeta Singh ◽  
Anshu Yadav ◽  
Perumal Vanamail ◽  
Sunesh Kumar ◽  
Kallol K. Roy ◽  
...  
Keyword(s):  
Observational Study ◽  
Pregnant Women ◽  
Prospective Observational Study ◽  
Tertiary Care ◽  
Three Dimensional ◽  
Endometrial Receptivity ◽  
Operating Characteristics ◽  
Significant Difference ◽  
Endometrial Volume ◽  
Hcg Trigger

Background: Objective of present study was to evaluate the role of three dimensional (3D) endometrial volume measurement on the day of hCG trigger in predicting the endometrial receptivity. The present study is a prospective observational study conducted at assisted reproductive centre of a tertiary care hospital.Methods: Endometrial volume was evaluated by three-dimensional ultrasound in 90 patients undergoing first cycle of IVF on hCG trigger day and was correlated with endometrial receptivity.Results: Out of 90 patients studied 12 patients achieved pregnancy. A significant difference was found in mean endometrial volume on hCG trigger day among pregnant (5.33±2.14 cm3) women compared to non-pregnant women (4.17±1.72cm3). Using Receiver operating characteristics (ROC) analysis the cutoff value for endometrial volume on hCG trigger day was 3.50 cm3 corresponding to sensitivity 75% and specificity 37.2%. Conclusions: The endometrial volume on hCG trigger day was significantly higher in pregnant women as compared to non-pregnant.

scholarly journals Gastrointestinal Disease in Patients with Common Variable Immunodeficiency: A Retrospective Observational Study

2019 ◽  
Vol 3 (4) ◽  
pp. 162-168 ◽  
Author(s):  
Rishad Khan ◽  
Mohamad Habbal ◽  
Michael A Scaffidi ◽  
Abbas A Bukhari ◽  
Amir Rumman ◽  
...  
Keyword(s):  
Observational Study ◽  
Common Variable Immunodeficiency ◽  
Age Of Onset ◽  
Gastrointestinal Disease ◽  
Tertiary Care ◽  
Retrospective Observational Study ◽  
Response To Treatment ◽  
Tertiary Care Centre ◽  
Gi Symptoms ◽  
Common Variable

Abstract Background Gastrointestinal (GI) symptoms are common among patients with common variable immunodeficiency disorder (CVID) yet remain poorly understood. Aims The aim of this study was to characterize the demographic, clinical, endoscopic and histologic features of patients with CVID and GI symptoms. Methods We conducted a retrospective observational study of all patients with CVID at a large Canadian tertiary care centre between January 2000 and May 2018. Results We included 95 patients with CVID. The mean age of patients at the time of CVID diagnosis was 38.2(±16.0). Fifty-three (56%) patients were female. Sixty-four (67%) patients had GI symptoms, with a mean age of onset for GI symptoms of 43.4(±15.1) years. The most common symptoms were bowel movement changes (n = 55 [58%]) and abdominal pain (n = 44 [46%]). Patients with GI symptoms were more likely to have anemia (n = 23 [36%] versus n = 3 [10%], P = 0.0129), iron deficiency (n = 16 [25%] versus n = 2 [7%], P = 0.0481), and have received GI antibiotics (n = 37 [58%] versus n = 0, P &lt; 0.0001) and proton pump inhibitors for reflux (n = 24 [38%] versus n = 3 [10%], P = 0.0067). The most common GI infections were Giardia lamblia (n = 14 [15%]) and Clostridium difficile (n = 4 [4%]). Forty-three (45%) patients with GI symptoms underwent colonoscopy, esophagogastroduodenoscopy or both. The most common findings were inflammation, nodular lymphoid hyperplasia, reduced plasma cells and increased intraepithelial lymphocytes. Conclusions This is the largest study on CVID patients in a North American setting. The majority of patients experienced GI symptoms. Future studies should study response to treatment for GI disease among patients with CVID.

scholarly journals Subclinical rickets among children visiting a tertiary care hospital

2018 ◽  
Vol 5 (4) ◽  
pp. 1555
Author(s):  
Lakshmi Lakkappa ◽  
Roy Ninan Abraham ◽  
Chirsti D. Savio
Keyword(s):  
Vitamin D ◽  
Alkaline Phosphatase ◽  
Early Stage ◽  
Tertiary Care ◽  
Care Hospital ◽  
Age Group ◽  
Care Centre ◽  
Tertiary Care Centre ◽  
Significant Difference ◽  
Group 6

Background: Subclinical rickets is the early stage of rickets which can be defined as a state where there are no clinically appreciable changes of rickets but is characterized by biochemical changes which include the following Low or normal calcium, Low phosphorus, raised alkaline phosphatase and Low Vitamin D or raised Parathyroid hormone (PTH) levels. Radiological changes are often subtle to detect in sub clinical rickets. The objective of the present study was to estimate the prevalence of subclinical rickets in the age group 6 weeks to 15 years in tertiary care centre and to study their clinical profile.Methods: Hospital based Cross sectional study was conducted among 156 children in the age group 6 weeks to 15 years over a period of 1 year at a tertiary care centre in South Bangalore. Evaluation and diagnosis of Subclinical rickets was done using various biochemical parameters such as Calcium, phosphorus and alkaline phosphatase and Wrist X ray.Results: Overall Prevalence of Subclinical Rickets among the study group was 57 (36.5%). Sub clinical rickets was more common among Females (41.4%). Significant difference was observed in time spent for outdoor activities among children with (7.18±3.2 hrs) and without (9.04±4.4 hrs) subclinical rickets. Vitamin D supplemented group had lower prevalence of Subclinical rickets. 26% of them had Wrist Changes for sub clinical rickets. Significant decrease in Serum calcium and phosphorus was observed between subjects with and without subclinical rickets.Conclusions: Prevalence of Subclinical Rickets in children aged between 6weeks to 15 years is significantly high. Optimal exposure to sunlight and Vitamin D supplementation can bridge the deficiency gap.

Criteria positive and criteria negative anaphylaxis, with a focus on undifferentiated somatoform idiopathic anaphylaxis: A review and case series

2020 ◽  
Vol 41 (6) ◽  
pp. 436-441 ◽  
Author(s):  
Daniel A. Rosloff ◽  
Kunal Patel ◽  
Paul J. Feustel ◽  
Jocelyn Celestin
Keyword(s):  
Diagnostic Criteria ◽  
Statistical Significance ◽  
Case Series ◽  
Poor Response ◽  
Response To Treatment ◽  
Fisher Exact Test ◽  
Subjective Symptoms ◽  
Significant Difference ◽  
Physical Findings ◽  
Idiopathic Anaphylaxis

Background: Undifferentiated somatoform (US) idiopathic anaphylaxis (IA) is considered a psychogenic disorder characterized by a lack of observable physical findings and poor response to treatment. Although failure to diagnose true anaphylaxis can have disastrous consequences, identification of US-IA is crucial to limit unnecessary expenses and use of health care resources. Objective: To better define the presentation and understand the potential relationship between US-IA and underlying psychiatric comorbidities. Methods: We retrospectively reviewed 110 visits by 107 patients to our institution for evaluation and management of anaphylaxis over a 1-year period. The patients were classified as having either criteria positive (CP) or criteria negative (CN) anaphylaxis based on whether they met Second National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network Symposium criteria for the clinical diagnosis of anaphylaxis. Patient characteristics, including objective and subjective signs and symptoms, and the presence of psychiatric diagnoses were collected and analyzed. Statistical significance was assessed by using the Fisher exact test. A literature review of US-IA and other psychogenic forms of anaphylaxis was performed. Results: Patients with CP anaphylaxis were more likely to present with hypotension, wheezing, urticaria, and vomiting than were patients with CN anaphylaxis. The patients with CN anaphylaxis were more likely to present with subjective symptoms of sensory throat tightness or swelling compared with patients with CP anaphylaxis. No significant difference was detected in the prevalence of psychiatric conditions between the two groups. Conclusion: Patients who met previously established diagnostic criteria for anaphylaxis were more likely to present with objective physical findings than those who did not meet criteria for true anaphylaxis. CN patients who presented for treatment of anaphylaxis were more likely to present with subjective symptoms. Formal diagnostic criteria should be used by clinicians when evaluating patients with suspected anaphylaxis.

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