Kawasaki disease (KD) is an acute febrile vasculitis of an unknown cause. It affects children <5 year of age, even if cases over 5 years old have been also reported. It is the commonest cause of acquired heart diseases in children which may lead to serious morbidity and mortality. The complications and mortality increase when the diagnosis is delayed. One of the main reasons leading to delayed diagnosis and consequent delayed treatment is the unusual presentation of KD. Its unusual manifestations have been increasingly reported to jeopardize the timely diagnosis and proper treatment. As there is not yet available blood test to diagnose it, low threshold should be taken into account for considering KD, when the clinical criteria are not typical. KD with renal manifestations is infrequently described. We present and discuss a case of an unusual presentation of KD presenting as bloody diarrhea and acute renal failure.

The most common causes of umbilical discharge in infancy are infection and umbilical granuloma that may be treated by antibiotics and topical application of silver nitrate subsequently. If the umbilical discharge persists or if there is any abnormal discoloration around the umbilicus, it is important to investigate for underlying congenital abnormality that may be cured by surgical intervention. Unusual presentation of omphalomesenteric duct cyst has been reported in literature. We report, for the first time as far as our search is concerned, a case of a 16-month-old infant who presented initially with persistent umbilical discharge and finally with bruising around the umbilicus in keeping with Cullen’s sign. A diagnosis of omphalomesenteric duct cyst containing pancreatic tissue was made on histopathological examination. This case emphasizes that, a persistent umbilical discharge and or discoloration around the umbilicus should be further investigated and an omphalomesenteric duct cyst can present as Cullen’s sign.

Causes of facial rashes and erythema in infants are many but rarely only happen during feeding times which are commonly and sometimes wrongly attributed to food allergy. There is a rare condition called Auriculotemporal nerve syndrome that is characterized by recurrent episodes of gustatory facial flushing and sweating along the cutaneous distribution of Auriculotemporal nerve: the so-called Frey syndrome. This condition is most frequently observed in adults usually after parotid surgery. It is rare in children and is mostly attributed to forceps assisted delivery. It can also be misinterpreted as food allergy. Here we report a case of an infant with Frey syndrome without any history of perinatal trauma, which was considered initially as food allergy and highlights the importance of distinguishing it from food allergy.

Tuberculosis remains an important public health problem, especially in developing countries. Mycobacterium Tuberculosis mainly affects the lungs but extra-pulmonary Tuberculosis is not uncommon. However, renal involvement is very rare particularly in paediatric age group. This article is reporting a case of 14 years old Emirati boy who presented with history of fever, mild cough and night sweats after admission. The patient was treated as a case of fever of unknown origin, most likely secondary to community acquired pneumonia. On further investigations, he was found to have pulmonary tuberculosis with miliary pattern of the both lungs and also renal tuberculosis as the urine culture grew Acid Fast Bacilli (AFB) (Mycobacterium Tuberculosis). The patient responded very well to the Anti-tuberculosis treatment and became symptom free.

Children are developing individuals with countless factors affecting their growth and development, from genetics to parenting, schools, environments, nutrition, good habits of sleep and health. They are very sensitive individuals to sudden changes in routine. Schools represent life for them and a place of not only learning, but also social interaction and sensory neurodevelopment. Disasters and pandemics such as the existing situation of COVID-19 are a major cause of trauma affecting their education, play, mental health, physical health, vaccinations, sleep, and development. The governments' strategies particularly home confinement and school closure to prevent the COVID-19 pandemic spread added unavoidable stress and psychosocial impacts on both adults and children. Trauma of this kind may endure for a long time with negative consequences that may manifest later on, even up to adult life. We conducted a cross sectional parental survey, to assess the impacts of home confinement on children in the cosmopolitan city of Dubai, UAE. Children included were from 3 years until 16 years old who were in schools or pre-school placements before COVID-19 started. Total number of children included in the survey was 658 of which 327 were boys and 331 girls. We found that the impact of the home confinement on children was significant and directly affected their quality of life (QoL) that may extend beyond the lockdown for longtime. This study will help relevant authorities and organizations to understand the negative impacts brought by the COVID-19 confinement on children and to adopt appropriate strategies to help children and their parents tackle these impacts and get them back to normal life and school again. This study also paved the way for future studies in the identification and management of children's behavior, attention, education, and other factors that play active roles in QoL and normal development. Moreover, this study may help in embracing early preventative and management plans by schools and authorities in future similar pandemics, infections, disasters or school outbreaks. We also discuss strategies for school reopening and flexibility when an outbreak happens again in a school or community.

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Despite the reduction in incidence of Group A Streptococcal (GAS) infections in the last few decades its global burden on mortality and morbidity remained significantly high and represent major concerns in many developing countries. These morbidities and mortalities can be serious in immunocompromised individuals. It has been shown for the last two decades that vitamin D plays an important role in the immune system modulation and its deficiency were associated with recurrent and sever infections. Only few literatures were written about the vitamin D deficiency and GAS infections and very scarce about its complications. One of the rarely reported complications of GAS infections is post-streptococcal systemic vasculitis. The association of this complications and the vitamin D deficiency was not reported before. We report for the first time a 5 years old boy presented with sever post-scarlet fever vasculitis with sever vitamin D deficiency, only responded to monoclonal antibody infusion. The presentation was complicated by more than one complications of GAS infection, hence difficult to diagnose initially and found to have sever vitamin D deficiency which we concluded is the causative factor for these complications. On correction of vitamin D level the deranged immunological and other factors causing the vasculitis were corrected subsequent to vitamin D correction. It is therefore recommended to check vitamin D concentration in cases of sever GAS especially in association with its complications such as the systemic vasculitis.

Background:Study of pre-clinical autoimmunity can reveal immune changes that initiate or prevent clinical disease. We previously reported an At Risk cohort of ANA positive individuals referred to secondary care, of whom 16% progressed to SLE or Sjogren’s syndrome during follow up[1]. Recently, we also reported that even ANA positive individuals who do not develop clinical autoimmunity, have defective pDC function and non-haematopoietic interferon-production[2].Objectives:To describe the immunophenotype of ANA-positive progressors and non-progressors compared to healthy controls.Methods:We conducted a prospective observational longitudinal study of At-Risk individuals in Leeds (n=150) over 3 years with annual follow up. Those who met classification criteria for autoimmune connective tissue disease (AI-CTD) during follow up were termed progressors and others; non-progressors. Among the non-progressors, those with no clinical diagnostic criteria at any time point were called absolute non-progressors (ANP).At baseline we analysed two IFN-stimulated gene expression scores previously described[3] and flow cytometry performed for major circulating subsets. Association between progression group and biomarkers were tested using independent samples t-test.Results:3 year follow up data were available in 148/150 patients. Outcomes were: progressors: 30/148 (20%) [SLE=25; pSS=5] and non-progressors: 118/148 (80%). Within the non-progressors, 69/148 (47%) were ANP.A detailed analysis of baseline biomarkers is included in Table 1. Despite not developing clinically significant disease, non-progressors had significant alterations in several biomarkers. This was also evident in the ANP sub-group.Table 1.BiomarkerHealthy ControlsMean(95% CI)Non-progressorsMean(95% CI)Difference from healthy controlspAbsolute non-progressorsMean(95% CI)Difference from healthy controls pProgressorsMean (95% CI)Difference from healthy controls pIFN score A-6.27 (-6.88, -5.65)-5.22 (-5.64, -4.80)0.016-5.11 (-5.91, -4.32)0.120-4.14 (-5.11, -3.18)0.001IFN score B-5.43 (-6.00, -4.85)-5.30 (-5.62, -4.98)0.193-4.96 (-5.45, -4.47)0.949-4.27 (-4.90, -3.65)0.136% Mono-cytes14.80 (10.50, 19.11)15.46 (12.96, 17.97)0.18714.94 (11.42, 18.47)0.73810.36 (7.42, 13.30)0.227% CD4 T cells62.90 (57.75, 68.05)64.89 (60.86, 68.92)0.00168.72 (62.97, 74.47)0.26760.93 (53.64, 68.21)0.001% CD8 T cells32.18 (27.47, 36.90)28.60 (25.12, 32.09)0.13525.02 (20.28, 29.77)0.04431.51 (25.63, 37.40)0.875% Naïve B cells65.59 (56.56, 74.61)73.74 (70.02, 77.46)0.00176.86 (72.21, 81.51)0.12868.86 60.62, 75.84)0.173% Memory B cells29.56 (20.42, 38.72)19.72 (15.84, 23.61)<0.00023.13 (16.65, 29.61)0.05425.57 (17.18, 33.96)0.163% Plasma-blasts1.12 (0.81, 1.43)0.91 (0.58, 1.24)0.9641.01 (0.44, 1.58)0.4570.81 (0.53, 1.10)0.112% NK cells13.65 (9.65, 17.65)12.30 (8.90, 15.71)0.48012.35 (6.65, 18.05)0.78111.90 (8.65, 15.14)0.195Conclusion:Clinically benign ANA positivity is a complex immune state with many features seen in active SLE, including pDC exhaustion, keratinocyte interferon production and strong skin interferon score, increased blood interferon score, and disturbance of B cell subsets. These findings suggest that other factors are necessary for clinical disease, or that regulatory mechanisms stabilise autoimmunity. Further work in this cohort will address these questions.References:[1]Md Yusof et al. Ann Rheum Dis 2018[2]El-Sherbiny et al. Sci Rep 2018[3]Psarras et al. Nat Commun. 2020Disclosure of Interests:Sabih-Ul Hassan: None declared, Zoe Wigston: None declared, Agata Burska: None declared, Md Yuzaiful Md Yusof: None declared, Edward Vital Grant/research support from: Honoraria and research grant support from Roche, GSK and AstraZeneca.