International Consensus Statement on Rhinology and Allergy: Rhinosinusitis

Background: The 5 years since the publication of the first International Consensus Statement on Allergy and Rhinology: Rhinosinusitis (ICAR‐RS) has witnessed foundational progress in our understanding and treatment of rhinologic disease. These advances are reflected within the more than 40 new topics covered within the ICAR‐RS‐2021 as well as updates to the original 140 topics. This executive summary consolidates the evidence‐based findings of the document. Methods: ICAR‐RS presents over 180 topics in the forms of evidence‐based reviews with recommendations (EBRRs), evidence‐based reviews, and literature reviews. The highest grade structured recommendations of the EBRR sections are summarized in this executive summary. Results: ICAR‐RS‐2021 covers 22 topics regarding the medical management of RS, which are grade A/B and are presented in the executive summary. Additionally, 4 topics regarding the surgical management of RS are grade A/B and are presented in the executive summary. Finally, a comprehensive evidence‐based management algorithm is provided. Conclusion: This ICAR‐RS‐2021 executive summary provides a compilation of the evidence‐based recommendations for medical and surgical treatment of the most common forms of RS

Dego's disease in a female child with Anti-Scl-70 antibody positivity

Degos' disease (DD) also known as malignant atrophic papulosis is considered to be a disorder of abnormal coagulation with obliterative arteritis. Its association has been described with connective tissue disorders, human immunodeficiency virus infection (HIV) and wegener's granulomatosis. Gastrointestinal, neurological, ocular, cardiovascular, and pulmonary involvement can also occur in DD. Benign version of DD with only cutaneous manifestations has been described. We are presenting a case of 7-year-old female with multiple irregular-shaped crusted tender lesions with porcelain white scars of healed lesions mainly over the trunk and upper limb and few lesions involving face and legs for the last 6 months. There was a history of occasional headache. Other systemic examination by experts was within normal limit. On further investigation, antinuclear antibody, and anti-Scl-70 antibody were positive with histopathology suggestive of DD. The patient had reduced peak expiratory flaw rate (PEFR). The patient was given oral prednisolone without much improvement. With oral aspirin for a month, the patient had mild improvement. The patient is presently under follow-up for future systemic involvement and response to therapy. Anticipated mortality in DD is 50%–60%. Earlier diagnosis and proper management at such a younger age might benefit the patient; however, they need to be followed up for complications. DD is very rare and very few cases have been reported in children. Ours is unique with anti-Scl-70 antibody positivity

Could Length and Reduced Diameter of Aberrant Renal Artery be Attributed to Significant Reno-vascular Hypertension in the Absence of Stenosis?

Hypertension depends upon various factors such as resistance, pressure difference viscocity, caliber and length of a vessel according to Hagen–Poiseuille law. Length of a vessel is one of the important causes of hypertension of reno–vascular origin in adolescence in the aberrant renal arteries. An adolescent girl with bilateral uveitis and hypertension was evaluated for the cause of hypertension. Serum rennin and aldosterone levels favoured hypertension of renovascular origin. Ultrasonography and C.T. scan angiography of the abdomen revealed multiple aberrant renal arteries without any stenosis. In the absence of renal artery stenosis length and caliber of the aberrant arteries are the parameters which contribute significantly to renovascular hypertension

The Inflammatory Relationship Between Hepatitis C Virus With Coronary and Carotid Atherosclerosis.

Hepatitis C virus (HCV), a global health concern, has been linked to various hepatic and extrahepatic deleterious manifestations. Several observational studies have either supported the increased likelihood of coronary and carotid atherosclerosis after infection with HCV or refuted it. To date, there has been no clear consensus to support either train of thought, as randomized, controlled clinical trials have not been completed. In this review, we first discuss articles that support the notion that HCV infection leads to increased plaque formation due to systemic inflammation and then focus on articles that refute this idea. From the literature, we do know that both inflammatory and lipid processes play a role in plaque formation, and thus both components are important in the successful treatment of atherosclerosis. Based on our review of the literature, we do believe that HCV-infected individuals are at an increased risk for more severe coronary artery disease than their healthy counterparts. Although there is no irrefutable evidence that links HCV infection with plaque formation and/or rupture, cardioprotective measures should be taken to reduce poor health outcomes, especially in those individuals who are already at risk of coronary disease

The Inflammatory Relationship between Hepatitis C Virus with Coronary and Carotid Atherosclerosis

Hepatitis C virus (HCV), a global health concern, has been linked to various hepatic and extrahepatic deleterious manifestations. Several observational studies have either supported the increased likelihood of coronary and carotid atherosclerosis after infection with HCV or refuted it. To date, there has been no clear consensus to support either train of thought, as randomized, controlled clinical trials have not been completed. In this review, we first discuss articles that support the notion that HCV infection leads to increased plaque formation due to systemic inflammation and then focus on articles that refute this idea. From the literature, we do know that both inflammatory and lipid processes play a role in plaque formation, and thus both components are important in the successful treatment of atherosclerosis. Based on our review of the literature, we do believe that HCV-infected individuals are at an increased risk for more severe coronary artery disease than their healthy counterparts. Although there is no irrefutable evidence that links HCV infection with plaque formation and/or rupture, cardioprotective measures should be taken to reduce poor health outcomes, especially in those individuals who are already at risk of coronary disease

Unusual presentations and associations of hyper IgE Syndrome: Retrospective analysis of ten cases at tertiary care institute – With review of indian published reports

Background: Job syndrome also known as hyper IgE syndrome (HIES) is primary immunodeficiency syndrome. Autosomal dominant variant caused by mutation in signal transducer and activator of transcription-3 gene is characterized by recurrent staphylococcal skin infections, sinopulmonary infections, eczema, recurrent bone fractures, and coarse facial features. Autosomal recessive (AR) variant is because of mutation in DOCK8 gene which lacks the skeletal and dental involvement but manifest with severe viral infection and develop neurological complications. Aims: This study aims to evaluate variable presentations and associations of job syndrome. Methods: Analysis of ten cases, presented at our department between October 2015 and September 2016, with suspected HIES was done retrospectively. All cases were studied for their presentations and associations and were investigated accordingly for the same. Score for HIES was counted as per National Institutes of Health (NIH) scoring system. Indian published reports were found by internet search engine and were reviewed for unusual presentations. Results: NIH scoring was indeterminate in two patients and was highly likely for autosomal dominant-HIES in five patients. Three patients were diagnosed as AR-HIES. Two of our patients had multiple endocrinopathy, one had pyoderma gangrenosum and two patients had autoimmune disorders. Limitations: Genetic analysis was not done because of nonavailability of testing in our state and poor financial conditions of patients. It is a retrospective study. Conclusion: Our patients had unusual association in the form of multiple endocrinopathy, pyoderma gangrenosum, dermatomyositis, and all were diagnosed very much late in life. Hence, a vigilant eye for suspicion at a younger age might benefit patients

Efficacy and safety of propranolol on the proliferative phase of infantile hemangioma: A hospital-based prospective study

Background: Propranolol may be more effective and safer than previously established therapies, and it may be used as a first-line therapy for infantile hemangioma (IH). Propranolol is thought to inhibit the growth of blood vessels by decreasing vascular endothelial growth factor. Aims and Objectives: The aim of this study is to study the efficacy and safety of propranolol in IH and for standardization of dose in tablet form. Materials and Methods: A total of 23 patients with 30 IH s were recruited in the study prospectively, after ruling out any contraindications for oral propranolol and obtaining consent from parents. Patients with 7 kg weight were given 5 mg thrice daily. Patients were evaluated according to visual analog scale and ultrasonographically on day 0, day 30, day 60, and day 90. Results: Out of a total of 30, 25 (83.33%) were superficial, 3 (10%) were deep, and 2 (6.66%) were mixed hemangioma. All patients with superficial hemangiomas showed a change in the color of the lesion and arrest of growth within the 1st month of therapy and a gradual decrease in size was noticed in 23 (92%, n = 25) patients during the study period except in 2 (8%). Complete clearance was noticed in 68% of patients of superficial hemangioma at the end of the study period. Out of 25 patients, a total of 5 (20%) patients had ulceration at the time of presentation which started to heal within 15 days of therapy. Five (16.66%, n = 30) patients with deep and mixed variety showed arrest of growth but no decrease in size. No side effects were seen except temporary coldness of extremity in 1 (3.33%) patient. Conclusion: Propranolol is safe and effective for treatment of proliferative phase of superficial hemangiomas with very less side effects compared to oral steroids and other recommended therapies, and unavailability of syrup can be overcome by giving tablet in fixed dosage