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Background: Acute bronchiolitis is the most common respiratory tract infection in young children. Despite the high prevalence of acute bronchiolitis, no consensus exists on the management. Studies have shown that except oxygen therapy, no other treatment found to be effective. Hence, the present study was conducted to find out the efficacy of nebulised 3% saline versus is 0.9% saline for the treatment of acute bronchiolitis.Methods: A prospective randomized controlled study of 150 children between the age group of 2 months to 24 months with signs and symptoms of Acute Bronchiolitis admitted to Indira Gandhi Institute of Child Health, Bangalore from January 2016 to December 2016 formed the study group, they were randomized into 2 groups, one received 3% saline nebulization and the other received 0.9% saline.Results: A total of 150 children were enrolled in the study, 75 children (group A) received 0.9% saline and 75 children (group B) received 3% saline. At 24 hours, the mean clinical severity score for group A was 2.49±1.03 and group B was 2.16±0.49 (P=0.013). The duration of hospital stay was shorter (1-3 days) in 3% saline with a mean of 2.35 days and was longer (3-5 days) in 0.9% saline with mean value of 4.04 days which was statistically significant (p <0.001).Conclusions: 3% saline nebulization can be used as an effective treatment for acute bronchiolitis. It significantly reduced the clinical severity score and length of hospital stay compared to 0.9% normal saline.

Introduction/Background Relapsing polychondritis (RP) is a rare systemic autoimmune disease of unknown etiology, characterized by recurrent inflammation of cartilaginous and connective tissue. The disease includes chondritis of ears, nose, and larynx, evolving by recurrent flares, and may lead to floppy ears, saddle nose, and laryngotracheal stenosis. Very few cases have been reported in paediatrics with the most common presentation being auricular chondritis. Here, we report a patient who presented with acute airway obstruction requiring tracheostomy. Description/Method A 16-year-old boy from Uganda, moved to UK 3 years ago and was referred to our department with Subglottic/tracheal stenosis of unknown aetiology. Initially it started as shortness of breath and then he developed voice hoarseness and stridor. CT neck showed severe laryngeal oedema and stenosis. He underwent an awake tracheostomy and pan endoscopy in theatre. He was noted to have severe laryngeal oedema and stenosis. Aetiology of lesion was unclear. He also reported significant weight loss. Examinations at local hospitals had not identified the cause of the subglottic stenosis and he had not responded to antibiotic therapy. At this stage, he was referred to our hospital for a Rheumatological assessment. His condition evolved and he developed transient redness of his eyes and pain over his bilateral ears. On examination he had tender pinna with sparing of the lobule, a saddle nose with a transverse crease on his nose, and tenderness over the sternal region. Laboratory findings showed increased inflammatory markers. Serology tests for antinuclear antibody and autoantibodies were all negative. Chest computed tomography was normal. Biopsy specimens from Hypo pharyngeal and right subglottic region showed non-specific chronic inflammation and haemorrhage. PET CT was performed which showed increased uptake affecting the outer ears bilaterally and suggestion of low- grade uptake associated with the costal cartilages and more apparent xiphisternal uptake, suggestive of RP. The patient fulfilled the McAdam-Damiani-Levine criteria for the diagnosis of RP, according to the presence of 3 of McAdam’s signs (auricular chondritis, ocular inflammation, and respiratory chondritis). He was started on IV Methylprednisolone 10mg/kg/day for 3 days followed by oral steroids 40mg and tapered slowly. He was also started on Mycophenolate Mofetil 600mg/m2 twice daily, which led to an improvement in his symptoms. The auricular pain and pain over sternal region disappeared. No flare up to date. Discussion/Results Paediatric-onset RP is rare and occurs in 5%–10% of the reported cases. In almost all patients the presenting symptom is auricular chondritis. Arthralgia, with or without arthritis is the second commonest presenting symptom. Of note, neither of these symptoms were presenting feature in our patient. Airway involvement while uncommon at presentation, occurs in approximately 50% of patients with RP. Airway manifestations are the commonest cause of morbidity and mortality in RP. This patient was initially managed as asthma and was given bronchodilators with no clinical improvement. He then developed noisy breathing and dyspnoea which led to him being referred to a tertiary respiratory team. This is not unusual for the reported cases of RP that have initially been managed as asthma. The histological findings of chronic inflammation in the sub-glottis prompted a referral to Rheumatology. In RP (unlike asthma), inhaled corticosteroids and bronchodilators are ineffective, spirometry demonstrates upper airway obstruction, CT imaging can reveal thickening and stenosis of the airways, and bronchoscopy can show inflammation, narrowing or collapse of the airways. Currently there is no standard treatment for RP. No clinical trials have been carried out due to its rarity. Therefore, current medical therapy for RP is largely empirical and established on case reports. Despite this, RP generally responds to steroids and other immunosuppressive agents. Corticosteroids are first-line therapy. They reduce severity and frequency of relapses. A low maintenance dose of steroid is used to prevent relapse and the dose is increased during flare ups. However, there is no evidence that corticosteroids alter the natural progression of RP. Steroid-sparing agents such as cyclophosphamide, methotrexate, Mycophenolate Mofetil, azathioprine and cyclosporine have been used and have shown benefit in the isolated reported cases. To date there is no evidence that one is more effective at preventing exacerbations than another. Key learning points/Conclusion RP is a rare condition characterized by recurrent inflammation and destruction of cartilaginous structures. RP can present with various clinical findings which can lead to a delay in diagnosis and management The diagnostic criteria includes: McAdam et al.: (1) Recurrent chondritis of both auricles, (2) Nonerosive inflammatory polyarthritis, (3) Chondritis of nasal cartilages, (4) Inflammation of ocular structures, (5) Chondritis of respiratory tract, (6) Cochlear and/or vestibular damage  (Requirement—three out of six criteria) Damiani and Levine: (1) Three out of six McAdam et al.'s criteria (2) One out of six McAdam et al.'s criteria and a positive histologic confirmation (3) Two out of six McAdam et al.'s criteria and response to corticosteroid or dapsone (requirement—any of these) Asthma can mimic RP when the airways are involved. Airway involvement in RP requires prompt corticosteroids/immunosuppression and if severe may lead to stenosis requiring emergency tracheostomy. The treatment should be tailored according to individual patient's organ involvement and complications, although no guidelines exist to date. Awareness of early airway involvement in RP is essential for prompt diagnosis and allows treatment to prevent life-threatening airway collapse. It is essential to examine the auricle, nose and joints in patients presenting with atypical asthma not responding to conventional therapy.

Background: Juvenile dermatomyositis (JDM) is one of the commonest forms of inflammatory myositis in childhood. Objective: The objective study was to study the clinical characteristics and course of JDM patients. Material and Methods: Retrospective analysis of the charts of 25 JDM patients admitted to two hospitals in Bangalore from March 2011 to July 2017. Results: The mean age at onset of disease was 7.74 ± 3.74 years. The male to female ratio was 1.5:1. All patients had skin rashes typical of JDM and 24/25 had demonstrable muscle weakness. Six patients were either lost to follow-up or died. Of the remaining 19 patients, 11 (57.9%) had a monocyclic course, 5 (26.3%) patients had a chronic continuous course, and 3 (15.8%) patients had a polycyclic course. Conclusions: JDM though rare should always be considered in the differential diagnosis in any child with skin rash and muscle pains and weakness. When diagnosed early and treated appropriately, sustained remission without medications is possible in a good proportion of patients.

Introduction/Background Auto inflammatory bone disorders are characterized by chronic non-infectious osteomyelitis and inflammation-induced bone resorption. It results from aberrant activation of components of the innate immune system. The auto inflammatory bone disorder includes chronic non-bacterial osteomyelitis (CNO) and its severe form chronic recurrent multifocal osteomyelitis (CRMO); synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome; Majeed syndrome; deficiency of interleukin-1 receptor antagonist (DIRA) and cherubism. The most common among this is CRMO/CNO. The disease can affect any part of the body but the metaphyseal region of the long bones, clavicle, and vertebra are the most commonly affected site. Description/Method A 9 year old girl presented with five month history of swelling over right periocular and temporal region, associated with skin discoloration and difficulty in opening her eyes. She also reported headache more in the right frontal region. She had on and off exacerbations of skin discoloration and headaches. There was no history of fevers, weight loss, night sweats, skin lesions or arthritic symptoms. She did not have a personal or family history of inflammatory or autoimmune disorders. She is under ophthalmology elsewhere since the age of 3 months with a right divergent squint and history of right squint surgery at 6 years. Physical examination revealed swelling involving right infraorbital and temporal region, which was soft and not tender to touch with associated erythema in the same site. There were no signs of meningism or altered mental status. Musculoskeletal examination was unremarkable. Her blood test results showed mild increase in platelets (476 * 109), CRP 8mg/L, ESR (18mm/hr). IgG4 titres were mildly raised (1.16g/L). Other immunologic markers including rheumatoid factor, human leukocyte antigen B27, antinuclear antibody and anti-neutrophil cytoplasmic antibody were normal. Histopathological examination of bone and soft tissue of her right orbital rim showed low grade non-specific chronic inflammation with extensive appositional remodelling of bone suggestive of CRMO/SAPHO. No evidence of malignancy/infection. Later whole body MRI was performed to identify the extent of disease. MRI whole body was performed which showed multiple areas of bone marrow oedema and periostitis within the appendicular and axial skeleton, bilateral gluteal enthesitis and sacroilitis. There are some features that are atypical for CRMO (asymmetrical involvement, transphyseal involvement including epiphyseal involvement of the left humerus). Also noted to have right-sided periorbital oedema, oedema of the right masticator muscles and temporalis. Further planned to do dedicated MRI of left humerus with contrast. Discussion/Results It can be difficult to consider what the problem is when a health care provider is presented with a group of seemingly disparate signs and symptoms with a history and time course that do not match classic (or commonly atypical) disease presentations. Common things happen commonly, so when symptoms occur without the usual co-occurring symptoms, unusual symptoms or time courses, it can be challenging. The current patient presented with periocular swelling with headache and skin discolouration. The differentials can be Langerhans cell histiocytosis, IgG4-related disease, osteoblastoma, and osteosarcoma. There were no hepatosplenomegaly, lymphadenopathy, also the mass was soft to palpate, and hence the above differentials were unlikely. Patient was initially evaluated at tertiary oncology centre and malignancy ruled out. The histopathology slides were reviewed and reported as mentioned above. In view of no skin manifestations, CRMO was the likely diagnosis considered. Hence MRI whole body performed. Barrani et al reported a similar type of unusual case of a 12-year-old girl with CRMO arising with recurrent episodes of left supraorbital headache, followed by the appearance of a periorbital dyschromia. Magnetic resonance imaging (MRI) of the skull and orbits revealed an important subacute inflammatory process. However, a few months later, the child presented a painful swelling of the left clavicle; the histological examination of the related biopsy allowed us to establish the diagnosis of CRMO. The follow up with our patient is quite a short duration and she might evolve later in the disease course giving more clarity! Key learning points/Conclusion In evaluating a diagnostic dilemma, starting the laboratory evaluation helps to begin the process of trying to find an answer and can also help the families as “something is being done.” Chronic recurrent multifocal osteomyelitis can present with unifocal lesions, atypical locations or absence of recurrence. A high level of suspicion is paramount to avoid unnecessary biopsies and repeated antibiotic regimens. Families will often be quite concerned and support for them is also necessary and close communication and follow up is appropriate.