Predominant Myofibrillar Pathology with Preserved Sarcolemmal Aquaporin 4 Immunoreactivity in a Patient with Neuromyelitis Optica-Associated HyperCKemia

A 49-year-old man developed recurrent myalgia and hyperCKemia during acute attacks of neuromyelitis optica. Muscle biopsy was performed, and the pathological findings were analyzed. Predominant myofibrillar pathology was observed, which constitutes a unique finding that has not been reported before. This case result shows that neuromyelitis optica-associated hyperCKemia can produce variable pathologic phenotypes. Further studies are needed to elucidate the relationship between myofibril destruction and aquaporin 4 autoimmunity.

A Short on Ubrogepant

Migraine is a mysterious disorder characterized by pulsating head ache, which is actually characterized to one side and comes in attacks which will be lasting for about 3-48 hours and can be associated with nausea,vomiting,sensitivity to sound,flashes of light,vertigoand diarrhoea [1]. Most of the drugs which are in current use for actue migraine like triptans, treats the disorder symptomatically. A novel group of drugs has been in research for the migraine which treats the disorder pathologically. Calcitonin gene – related peptide (CGRP) has a major role in the pathophysiology of the disorder and hence CGRP receptor antagonist, known as Gepants are in the research process [2]. Gepants are being studied for the efficacy of treating acute migraine [2]. This article will be a review article about the drug – Ubrogepant, which is approved for treatment of migraine with acute attacks in adults [3].

Characteristics of older adult hospitalized patients with bronchial asthma: a retrospective study

Background Bronchial asthma is a chronic inflammation of the airways. Older adult patients with bronchial asthma are defined as patients older than 65 and with a previous or current clear diagnosis of asthma. The purpose of this study was to determine the characteristics of older adult hospitalized patients with bronchial asthma. Methods We retrospectively analyzed the data from patients with bronchial asthma admitted to the General Hospital of the Northern Theater Command from September 2018 to January 2020. We divided them into the older adult (≥ 65 years) and the younger (< 65 years) groups. We compared the clinical and epidemiological characteristics of the two groups. Results There were 181 inpatients with bronchial asthma, including 41 older adult patients, accounting for 22.7%. There were significant differences in age, sex, smoking, duration of disease, age at diagnosis of asthma, hospital stays, hospitalization costs, number of acute attacks 1 year before admission, number of hospitalizations in our hospital one year before admission, asthma control test score, forced expiratory volume in 1 s (FEV1), FEV1/FVC, the severity of acute attacks, comorbidities, and inhaled corticosteroid dose between the two groups. There were many older adult patients with asthma (mostly late-onset asthma). The hospitalization costs were high. Most patients had many comorbidities, poor asthma control, severe attack, and heavy economic burdens. Conclusion Attention should be focused on achieving asthma control in older adult patients to improve their quality of life and reduce their economic burdens.

Melkersson–Rosenthal syndrome in the context of sarcoidosis: a case report

Background Melkersson–Rosenthal syndrome is a rare disease characterized by the triad of recurrent orofacial swelling with facial paralysis and fissured dorsal tongue. Histologically, noncaseating granulomatous inflammation occurs that confirms the diagnosis. Overlaps between granulomatous diseases such as sarcoidosis and Crohn’s disease are described. Systemic corticosteroid therapy is the treatment of choice for acute attacks. Case presentation We here present a case of a 59-year-old White woman suffering from Melkersson–Rosenthal syndrome with a past history of sarcoidosis on therapy with leflunomide in combination with low-dose tacrolimus successfully treated with the anti-leprosy drug clofazimine after failure of systemic steroid therapy. Conclusions We propose clofazimine as an alternative treatment in steroid-refractory cases.

Bradykinin receptors in GtoPdb v.2021.3

Bradykinin (or kinin) receptors (nomenclature as agreed by the NC-IUPHAR subcommittee on Bradykinin (kinin) Receptors [91]) are activated by the endogenous peptides bradykinin (BK), [des-Arg9]bradykinin, Lys-BK (kallidin), [des-Arg10]kallidin, [Phospho-Ser6]-Bradykinin, T-kinin (Ile-Ser-BK), [Hyp3]bradykinin and Lys-[Hyp3]-bradykinin. Variation in pharmacology and activity of B1 and B2 receptor antagonists at species orthologs has been documented. icatibant (Hoe140, Firazir) is approved in North America and Europe for the treatment of acute attacks of hereditary angioedema.

Etiological Aspects And Preventive Measures Against Chronic Tonsillitis In Children

Tonsillitis is a common and frequent disease in childhood, divided into acute tonsillitis and chronic tonsillitis. Acute tonsillitis is caused by pathogens invading the tonsils. Symptoms: fever, cough, sore throat, fever in severe cases, difficulty swallowing, and examination reveals the tonsils are hyperemic, swollen and festering. The tonsils have acute attacks more than 4 times a year, which can be diagnosed as chronic tonsillitis, mostly caused by pathogens in the fossa of the tonsils. On examination, hypertrophy, hyperemia or secretion of the tonsils and enlargement of the submandibular lymph nodes can be seen

Difference in the Source of Anti-AQP4-IgG and Anti-MOG-IgG Antibodies in CSF in Patients With Neuromyelitis Optica Spectrum Disorder

ObjectiveTo elucidate the differences in the source and in the level of intrathecal synthesis between anti-aquaporin-4 antibodies (AQP4-IgG) and anti-myelin oligodendrocyte glycoprotein antibodies (MOG-IgG).MethodsThirty-eight patients with MOG-IgG-associated disease and 36 with AQP4-IgG-positive neuromyelitis optica spectrum disorders (NMOSD) were studied for the antibody titers in the sera and cerebrospinal fluids (CSF) simultaneously collected in the acute attacks. The quotients between CSF and serum levels of albumin, total IgG, and each disease-specific antibody were calculated. Intrathecal production level in each disease-specific antibody was evaluated by calculating antibody index from these quotients.ResultsEleven of the 38 patients with MOG-IgG were positive for the antibody only in the CSF, while no patient with AQP4-IgG showed CSF-restricted AQP4-IgG. Blood-brain barrier compromise as shown by raised albumin quotients was seen in 75.0% of MOG-IgG-positive cases and 43.8% of AQP4-IgG-positive cases. Moreover, MOG-IgG quotients were more than 10 times higher than AQP4-IgG quotients (effect size r = 0.659, p < 0.0001). Elevated antibody index (>4.0) was confirmed in 12 of 21 with MOG-IgG, whereas it was seen only in one of 16 with AQP4-IgG (φ = 0.528, p < 0.0001). The CSF MOG-IgG titers (rho = +0.519, p = 0.001) and antibody indexes for MOG-IgG (rho = +0.472, p = 0.036) correlated with the CSF cell counts but not with clinical disability.ConclusionsIntrathecal production of MOG-IgG may occur more frequently than that of AQP4-IgG. This finding implies the different properties of B-cell trafficking and antibody production between MOG-IgG-associated disease and AQP4-IgG-positive NMOSD.

Patient and caregiver experiences of living with acute hepatic porphyria in the UK: a mixed-methods study

Background This study used quantitative and qualitative research methods to analyze how acute hepatic porphyria (AHP) affects patients with varying annualized porphyria attack rates. The overall impact of AHP on patients and caregivers, including their quality of life, was explored. The nature and treatment of acute attacks, experiences of long-term heme arginate treatment and access to other appropriate treatment, and the extent of and treatment for chronic symptoms were also investigated within this study. Methods Patient and caregiver data were collected via an online survey of members of the British Porphyria Association, followed by an optional 1-h telephone interview. Results Thirty-eight patients and 10 caregivers responded to the survey. Of those, 10 patients and three caregivers completed follow-up interviews. Overall, 19 patients (50%) had experienced an acute attack within the previous 2 years, and the severity and types of symptoms experienced during or between acute attacks varied considerably. There were no clear definitions among patients for ‘mild’ or ‘severe’ attacks. Treatments and treatment settings used to manage attacks also varied. Following unsatisfactory care experiences at hospitals, some patients reported avoiding further hospital services for later attacks. Therefore, using settings of care as a measure of attack severity should be avoided. Ninety-four percent of patients also experienced chronic symptoms, which were as varied as acute attacks. Pain was the predominant chronic symptom and was managed with opioids in severe cases. Regardless of AAR, porphyria heavily impacted the daily lives of patients and caregivers. Although patients experiencing frequent attacks generally endured a greater impact on their daily life, patients with less frequent attacks also experienced impacts on all domains (social, leisure activities, relationship with family, relationships, psychological wellbeing, finances, employment, and study). Caregivers were most affected in the finance, relationships with family, and employment domains, and just over half of the caregivers reported a moderate impact on their psychological wellbeing. Conclusions/implications The burden of illness with AHP is high across all patients, regardless of frequency of attacks, and AHP negatively affects patients and caregivers alike.