Association of HMGB1, S100A12 and IL-17A Expression with Immunoglobulin-Resistant in Kawasaki Disease and the Comparisons Between Different Adjunctive Therapeutic Approaches

Objective: The DAMPs such as HMGB1, S100A12 and IL-17A have been reported to predict poor response to IVIG. The aim of this study was to analyze the role of HMGB1,S100A12 and IL-17A in the detection of inflammation in KD patients with IVIG-resistant, and to investigate the value of different adjunctive therapy.Method: This study enrolled 126 patients diagnosed with KD, as well as age-matched 16 febrile control subjects. The demographic or clinical data, laboratory parameter and blood sample were collected. Various laboratory parameters as predictive factors for IVIG-resistant were calculated. And the serum levels of IL-17A and mRNA expression levels of HMGB1 and S100A12 were tested in all patients. For patients with acute KD in IVIG-resistant, we studied the levels of laboratory variables when using of IVIG retreatment, methylprednisolone, infliximab for children patients. Result: The variance of laboratory parameters between the febrile control group and KD group were analyzed. Regarding laboratory parameters, KD individuals were found to have lower levels of L%, PA, CD4+, CD8+ and higher levels of WBC, N%, CRP, ESR, NT-proBNP, ALT, CD4+/CD8+ (P<0.05 or P<0.01). For KD group, the 53 IVIG-resistant patients had significantly higher levels of blood S100A12, HMGB1, serum IL-17A levels And N%, CRP, NT-pro BNP, TBIL, ALT, AST and lower levels of L%, PLT (P<0.05 or P<0.01) in comparison to the IVIG-responsive patients. For patients with acute KD in IVIG-resistant, after initial IVIG-treatment, the adjunctive therapy of IVIG, methyl prednisolone or infliximab were used, the inflammatory symptoms and laboratory inflammatory markers were improved when treated with those drugs. Conclusion: IVIG-resistant was associated with higher levels of HMGB1, S100A12, IL-17A, CRP, NT-pro BNP, TBIL, ALT, AST and lower levels of L%, PLT before IVIG, especially when combined, were useful predictors for IVIG-resistant in KD. In addition, the adjunctive therapy of methylprednisolone and infliximab showed more effective in relief clinical symptoms than IVIG retreatment.

Pulse Methylprednisolone with Oral Prednisolone versus Adrenocorticotropic Hormone in Children with West Syndrome: a Randomized Controlled Trial

Background and Purpose: West syndrome is an epileptic encephalopathy of infancy. According to guidelines, adrenocorticotrophic hormone (ACTH) is probably effective for the short-term management of infantile spasm, but there is little uniformity in treatment due to variable response. This study has been done to evaluate the efficacy of pulse methylprednisolone as compared to ACTH in children with West syndrome.Methods: Children between 3 months to 24 months with the diagnosis of West syndrome were included and ACTH and pulse methyl prednisolone followed by oral prednisolone were given after randomization. Total duration of treatment was 6 weeks in both groups.Results: Total 87 children were enrolled; 12 patients lost in follow up. Finally, 43 received ACTH and 32 received pulse methylprednisolone. In pulse methylprednisolone group, 28.13% showed 50-80% response, 28.13% showed 80-99% response and 21.87% patients showed 100% response. In ACTH group, 41.86% showed 50-80% response, 25.58% showed 80-99% response and only 3 (6.97%) patients showed 100% response. Methylprednisolone treatment regimen did not cause significant or persistent adverse effects.Conclusions: Pulse methylprednisolone followed by oral prednisolone for 6 weeks is as effective as ACTH. Thus, methylprednisolone therapy can be an important alternative to ACTH.

A Rare Case of Dengue Fever with Abducens Nerve Palsy

Dengue fever is one of the most common vector borne disease which is a viral infection transmitted by aedes mosquito. Most common in the tropical countries. Neurological manifestations are not commonly seen in dengue, it can present as encephalitis, encephalopathy, neuromuscular disorders and neuro-ocular disorders. Cranial mononeuropathy is a very exceptional manifestation. A 48-year-old Indian male was diagnosed with dengue, complicated with isolated unilateral sixth cranial nerve palsy. The patient was managed conservatively. Patient made a fast and full ocular recovery following treatment with methyl prednisolone. Hence, dengue can present with a cranial nerve palsy and the recovery process can be hasten with the use of corticosteroids.

COVID-19 MIS-C ( MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN) LIKE PRESENTATION WITH RARE ASSOCIATION OF SCRUB TYPHUS : A CASE SERIES.

Introduction: Recently there is a surge of post COVID-19 MIS-C cases worldwide. Its diagnosis becomes challenging when a case presented with MIS-C like feature associated with scrub typhus infection. Method: Our cases presented with high fever, neurological and gasrtointestinal symptoms, rash and non-purulant conjunctivitis . All the cases have high inammatory markers like ESR,CRP,LDH,ferritin,IL6 with elevated D-dimer and CPK-MB in case 1 and 2. Echocardiographic abnormality was present in rst two cases and SARS-CoV-2 Ab was positive in all . Due to their MIS-C like presentation we treated with methyl prednisolone , IVIG, enoxaparin and oral aspirin but fever did not responded .Then after getting the Scrub IgM Ab positive and discovering eschar in third case we started doxycycline and the patients were discharged in stable condition. Conclusion: Tropical infections like scrub typhus may be associated with a post COVID-19 MIS-C like presentation.

A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes

Ocular involvement in cases of bullous pemphigoid is rare and when present, the signs are usually subtle and in the form of fine tarsal scarring and dry eye disease. The current report aims to describe the clinical features and management protocols in a series of cases with aggressive ocular manifestations at presentation. All cases of bullous pemphigoid seen between 2017 and 2020 were included in this retrospective case series. Data regarding the clinical features, treatment administered, and outcomes was collected. Five cases (n=10 eyes) of bullous pemphigoid disease with ocular involvement were included. All eyes had significant cicatricial conjunctival changes in the form of symblephara, inferior forniceal shortening, and tarsal conjunctival scarring. Conjunctival granulomas were present in 3/10 eyes. Corneal involvement in the form of punctate keratitis was present in all eyes while 4/10 eyes had an epithelial defect as well. The management of these cases involved topical therapy with corticosteroids and lubricants (n=10 eyes) while pulse doses of intravenous methyl prednisolone were administered in 5/5 cases. Pulse intravenous cyclophosphamide was supplemented in 2/5 cases. Adequate control of the disease was noted in 3/5 cases while one case had a recalcitrant form of the disease and developed a dermalised ocular surface in both eyes. The last patient was lost to follow up during the course of therapy. Bullous pemphigoid can present with an aggressive form of cicatrizing conjunctivitis similar to other variants of autoimmune blistering disorders and must be considered as a differential in cases presenting with ocular cicatricial disease. Long-term intensive immunosuppression is required for the management of these cases to preserve the visual function and the integrity of the globe.

Clinical profile and outcome of multisystem inflammatory syndrome in children in a tertiary care centre of North Kerala: a prospective study

Multisystem inflammatory syndrome in children (MIS-C) is a newly emerged disease following the outbreak of COVID-19. The nomenclature is given by WHO. Similar condition is named as paediatric inflammatory multisystem syndrome-temporally associated with SARS-CoV2 virus (PIMS-TS), by RCPCH. To study the clinical profile and outcome of MIS-C in 2 months to 18 years old. The 25 children, admitted in PICU and pediatrics ward were included in the study. The 79% required PICU admission and two required mechanical ventilation. One child expired. Children presented with conjunctival congestion (53%), diarrhoea (47%), cervical lymphadenopathy (37%), shock (31.5%), vomiting (36.8%), rashes (36.8%), mucosal erythema (36.8%), cough (10.5%) and headache (10.5%). Inflammatory markers were elevated in all the children. On initial echocardiography, 4 children (21%) had LV dysfunction with LVEF <50%. Six children (31.5%) were treated with IVIG and IV methylprednisolone, changed to oral prednisolone after 3 days and continued for 2 weeks. Three children (18%) were treated with IVIG and oral prednisolone. Two children (12%) were treated with IVIG alone. Eight children (47%) were treated with methylprednisolone alone. The median duration of hospitalisation was 5 days. 18 children (94.7%) were discharged to home within one week of admission. One 8 months old baby presented with shock, succumbed to death in spite of giving IV methyl prednisolone and IVIG. In our study we observed that early diagnosis and treatment with IVIG or steroids offers good outcome for the condition. Of the 19 children studied, 17 children were discharged within one week.

Comparison of intralesional corticosteroid injection with and without thumb Spica cast for de-Quervain tenosynovitis.

Objective: To compare efficacy of methyl prednisolone acetate injection with and without thumb spica cast for the treatment of de-Quervain tenosynovitis. Study Design: Randomized Controlled Trial. Setting: Orthopedic Unit, Allied Hospital, Faisalabad. Period: April 2016 to September 2016. Material & Methods: In each group 41 patients were included with non-probability consecutive sampling. Results: Eighty two patients were enrolled in the study. Out of 82 patients in the study, 3 (3.7%) were males and 79 (96.3%) were females. There were 10 (12.20%) pateints with age >40 years, and 36 (43.90%) patient in each of age group 21-30 and 31-40 years. The proportions of cured persons between two groups i.e., treated with corticosteroid injection and those treated with combination of injection and thumb Spica splint were found same with p-value 0.19 for Z=1.31. The efficacy was independent of treatment methods with χ2=1.10 (p-value = 0.30). The same independence pattern was also observed in different age groups. Age range was between 18 and 70 years. Statistical analysis was performed using chi-square test. Six weeks following treatment, 29 (35.37%) patients from group A and 34 (41.46%) patients from group B showed relief of pain, swelling and tenderness and a negative Finkelstein test (p-value 0.295). post stratification of gender showed no significant association (p-value 0.388 and 0.328 in groups A and B respectively). Conclusion: It has been concluded that use of corticosteroid injection alone is sufficient to treat de-Quervain syndrome as compared to the use of thumb Spica splint with corticosteroid injection. It is therefore recommended that in patients suffering from de-Quervain syndrome, corticosteroid injection may be the choice of treatment.

Efficacy of Methylprednisolone With DexamethasoneiIn patients With Severe Covid Pneumonia

Aim: Efficacy of Methylprednisolone with Dexamethasone in patients with severe COVID pneumonia in term of clinical and biochemical improvement. Methodology: Cross Sectional analytical study conducted among COVID-19 patients admitted in HDU of Sir Sadiq Hospital, Bahawalpur from May, 2021 to June 2021 after taking approval from institutional ethical committee. 82 patients were included in this study after taking informed consent. Patients were divided into two groups A and B i.e. I/V methyl prednisolone and I/V dexamethasone respectively. Each group was containing 41 patients. Primary outcome was measured in terms of decrease or increase in oxygen demand leading to discharge or shifting to Intensive Care Unit. CRP levels were also measured to assess biochemical improvement. Results: 31 (75.6%) patients from group A and 22(53.7%) from group B were improved and discharged and difference was statistically significant (p<0.05). Number of patients shifted to ICU were 10(24.4%) and 19(46.3%) from A and B groups respectively. Conclusion: In terms of clinical and biochemical response methylprednisolone outperforms dexamethasone. Keywords: Methylprednisolone, Dexamethasone, COVID pneumonia,

Autoimmune epilepsy due to N-methyl-d-aspartate receptor antibodies in a child: a case report

Introduction Seizures of autoimmune etiology may occur independent of or predate syndromes of encephalitis. We report a child with “pure” autoimmune epilepsy followed up for 7 years to highlight long-term effects of this epilepsy and the importance of early initiation and appropriate escalation of immunosuppression to achieve a good long-term outcome. Case presentation A previously healthy 5-year-old Sri Lankan boy presented with acute, frequent, brief focal seizures of temporal-lobe semiology without clinical and investigatory findings suggestive of central nervous system infection, tumor, structural abnormality, or metabolic causes. His epilepsy showed poor response to increasing doses and combinations of antiseizure medications. Further investigations detected N-methyl-d-aspartate receptor antibodies in serum, but not cerebrospinal fluid. Treatment with intravenous methyl prednisolone and maintenance on mycophenolate resulted in a rapid reduction, with seizure freedom achieved within 5–6 weeks. He relapsed when immunotherapy and anti seizure medications were reduced after seizure freedom for 24 months. This, and subsequent relapses, showed poor response to modification of anti-seizure medications, but treatment with immunotherapy (methyl prednisolone and rituximab) achieved complete seizure freedom. At 7-years of follow-up, he remains free of seizure for over 3 years, and has average academic performance and satisfactory quality of life. Conclusions Autoimmune epilepsy is a recognized independent entity. Diagnostic criteria have been suggested for its early recognition and confirmation of diagnosis. Early diagnosis and initiation of immunosuppression, with prompt escalation of treatment when necessary, remains key to good patient outcome.