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P082 Appendicular syndrome revealing Kawasaki-like syndrome: a case report

Abstract Background With the wide spread of the current SARS-CoV-2, It was found that about 2% of children was affected according to several studies, However, a small number of children with Covid-19 develop a significant systemic inflammatory response similar to Kawasaki disease, a new disease entity called multisystem inflammatory syndrome. Methods A 12-year-old child, without a notable pathological history, who presented to the emergency, during the SARS-CoV-2 pandemic, for management of a pseudo-appendicular syndrome. Our patient was initially assessed by the surgical team due to his query acute abdomen. The pain had been evolving for 3 days associated with several episodes of bilious vomiting in a context of fever at 38.5°. Abdominal examination noted abdominal tenderness and defence. Extradigestive signs were not reported, The Lab Testing objectified a CRP at 235, elevated white blood cells at 18 180, an abdominal ultrasound was requested returning without particularities. Faced with the persistence of bilious vomiting, surgical exploration was indicated objectifying a catarrhal appendix. A pediatric opinion was requested, the clinical examination shows conscious child who presents infra cervical lymphadenopathy with a fever at 38 associated with an erythematous skin rash on the back and aseptic conjunctivitis. The Lab Testing objectified an important inflammatory syndrome, a acute kidney and heart failure a Covid 19 serology was requested with positive IGG, négative IGM, PCR covid test was negative, given the unavailability of In immunoglobulins, the treatment was based on corticosteroid bolus then relay by oral corticosteroid associated with an anti-inflammatory treatment, gastric protection by proton pump inhibitors, treatment of heart and acute kidney failure. The evolution was marked by clinical and biological improvement Discussion Coronavirus 2 (SARS-CoV-2) infection among children and adolescents, is mainly responsible for mild respiratory symptoms, in contrast to the severe forms reported in adults [7]. A systemic inflammatory syndrome mimicking kDa, temporally associated with infection with SARS-CoV-2 (Kawa-COVID-19) has recently been described as a serious illness sometimes requiring intensive care (44%). The median age is older (> 5 years), the frequency and severity of myocarditis are very different from classic kDa, abdominal pain and/or diarrhea were more frequently (81%) reported than in classic kDa, heart failure, pneumonia, neurological and renal impairment, associated with elevated CRP, hyperferritinemia are more common in Kawasaki-Like syndrome [10]. Some investigations must be systematically realized urgently to diagnose potentially fatal complications. These include testing for myocarditis, patients should benefit from careful monitoring and treatment with IV Ig 2 g/kg should be administered rapidly and seems to be effective in the majority of cases, associated anti-inflammatory therapy, such as steroids is necessary Conclusion Pediatricians should be aware of these atypical presentations of COVID-19 infection for early diagnosis.

any organ system and cause significant morbidity and mortality. The disease onset occurs before the age of 18, however the peak age of onset is between 12 and 14 years and it affects 15-20% of all SLE patients. Patients with disease onset before 5 years of age are very uncommon and may be referred to as early-onset SLE. They commonly present with fever and general malaise, rash and arthritis. In addition, cases of jSLE, tend to have more severe organ manifestations and greater damage at the time of diagnosis as well as a higher incidence of renal, cardiovascular and neuropsychiatric involvement. Compared with adult-onset SLE, it is more aggressive, with higher medication burden including corticosteroids and other immunosuppressive drugs.

Method and results
This case report reviews the journey of a 5-year-old boy, who developed recurrent skin rashes, fever and generalised lymphadenopathy in his second year of life. After multiple hospital admissions and various investigations at different facilities spanning a year, he was admitted to the paediatric emergency unit at a tertiary hospital on account of an acute infection. On presentation, he was pyrexial. There were hypo-and hyperpigmented lesions extending over his scalp, face and upper arms as well as vasculitic lesions in his palms and soles; lip ulcers, sparse brown hair and multiple, non-tender lymph nodes in the cervical, supraclavicular and axillary regions. Respiratory examination revealed a right lobar pneumonia. Heart rate was 112 bpm and heart sounds were normal, however an echocardiogram showed a moderate pericardial effusion with no signs of tamponade. His abdomen was distended with a non-tender hepatomegaly. There was no synovitis. He was lethargic, but had no focal neurological signs. Laboratory results were as follows: normocytic anaemia (8.5 g/dl); ESR (120 mm/h); deranged transaminases; albumin (32 g/dl); Creatinine (12umol/l); urinalysis was normal, positive antinuclear antibody (1:320); positive double-stranded DNA (1:40); positive anti-smith, anti-Ro and anti-RNP antibodies; low C3, C4. A lymph node biopsy showed reactive changes only, with no evidence of haematological malignancy. The diagnosis of jSLE was confirmed at age 3yrs. The acute illness due to pneumonia was treated with antibiotics. Prednisolone 10 mg, Hydroxychloroquine (HCQ) 80 mg daily, Azathioprine 25 mg daily, calcium supplementation and gastric protection were added to manage the jSLE. He made an initial recovery and was managed in the outpatient clinic for months. However, his illness flared up again around age 4 years when he developed generalized seizures, facial and pedal oedema, low urine output, proteinuria 3þ, low albumin (25 g/dl), creatinine (252umol/l) and high urine albumin-creatinine ratio (278.62 mg/mmol) reflecting new renal and CNS involvement. A renal biopsy was not available. He received pulse iv methylprednisolone 250 mg daily for 3 days followed by monthly iv cyclophosphamide therapy 500 mg (NIH Regime) which was discontinued after the 5th cycle due to haemorrhagic cystitis. He eventually continued treatment with mycophenolate mofetil 125 mg daily, HCQ 80 mg daily, Prednisolone 10 mg daily, Nifedipine and Enalapril and a year later, has low activity from the cutaneous, renal and CNS manifestations. There is renal damage (creatinine 180 mmol/l) and hypertension, as well as treatment complications from prednisolone causing bilateral cataracts. Conclusion Early diagnosis of jSLE is still a challenge and a high index of suspicion is required especially in the very young. Severe presentation and aggressive clinical course is associated with tissue damage, as in the case presented. Management is complex and we were challenged by the choice and availability of treatment suitable for the severity of his disease, bearing in mind that damage can occur early especially in a male child. In addition, it is important to consider his young age and long-term exposure to drug toxicity and complications.

Background
With the wide spread of the current SARS-CoV-2, It was found that about 2% of children was affected according to several studies, However, a small number of children with Covid-19 develop a significant systemic inflammatory response similar to Kawasaki disease, a new disease entity called multisystem inflammatory syndrome.

Methods
A 12-year-old child, without a notable pathological history, who presented to the emergency, during the SARS-CoV-2 pandemic, for management of a pseudo-appendicular syndrome. Our patient was initially assessed by the surgical team due to his query acute abdomen. The pain had been evolving for 3 days associated with several episodes of bilious vomiting in a context of fever at 38.58. Abdominal examination noted abdominal tenderness and defence. Extradigestive signs were not reported, The Lab Testing objectified a CRP at 235, elevated white blood cells at 18 180, an abdominal ultrasound was requested returning without particularities. Faced with the persistence of bilious vomiting, surgical exploration was indicated objectifying a catarrhal appendix. A pediatric opinion was requested, the clinical examination shows conscious child who presents infra cervical lymphadenopathy with a fever at 38 associated with an erythematous skin rash on the back and aseptic conjunctivitis. The Lab Testing objectified an important inflammatory syndrome, a acute kidney and heart failure a Covid 19 serology was requested with positive IGG, né gative IGM, PCR covid test was negative, given the unavailability of In immunoglobulins, the treatment was based on corticosteroid bolus then relay by oral corticosteroid associated with an anti-inflammatory treatment, gastric protection by proton pump inhibitors, treatment of heart and acute kidney failure. The evolution was marked by clinical and biological improvement Discussion Coronavirus 2 (SARS-CoV-2) infection among children and adolescents, is mainly responsible for mild respiratory symptoms, in contrast to the severe forms reported in adults [7]. A systemic inflammatory syndrome mimicking kDa, temporally associated with infection with SARS-CoV-2 (Kawa-COVID-19) has recently been described as a serious illness sometimes requiring intensive care (44%). The median age is older (> 5 years), the frequency and severity of myocarditis are very different from classic kDa, abdominal pain and/or diarrhea were more frequently (81%) reported than in classic kDa, heart failure, pneumonia, neurological and renal impairment, associated with elevated CRP, hyperferritinemia are more common in Kawasaki-Like syndrome [10]. Some investigations must be systematically realized urgently to diagnose potentially fatal complications. These include testing for myocarditis, patients should benefit from careful monitoring and treatment with IV Ig 2 g/kg should be administered rapidly and seems to be effective in the majority of cases, associated antiinflammatory therapy, such as steroids is necessary Conclusion Pediatricians should be aware of these atypical presentations of COVID-19 infection for early diagnosis. Background Gourgerot-Sjö gren syndrome (GSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to dryness of the mucous membranes. It may also involve other organs and organ systems. GSS can be primary (pGSS) or secondary due to other autoimmune diseases. pSGS mainly affects women over 40 years old, but can occur at any age. GSS is rare in children and is often secondary to other diseases. We report the case of a 12-year-old girl with primary Gougerot-Sjö gren Syndrome Methods and results A 12-year-old girl from Batna (Algeria) presented to her pediatrician in 2016 for a rash with arthralgia. She had no relevant past medical history and is the child of a non-consanguineous marriage. Clinical examination found a well-nourished child with a purpuric rash on her lower limbs. She complained of arthralgia without clinical signs of arthritis. The child also complained of a sensation of a foreign body and burning in both eyes. The eye exam was normal except for a positive Schirmer's test. There were no signs of parotitis. Laboratory tests showed an erythrocyte sedimentation rate (ESR) of 84 mm the first h and a negative C-reactive protein (CRP). She was also Leukopenic. Serological testing resulted in a positive Anti SS-A and Anti SS-B. The abdominal pelvic ultrasound was normal. The patient was diagnosed with primary Gougerot-Sjö gren Syndrome. Artificial tears and hydroxychloroquine were started as initial therapy. One year later we added an immunosuppressant (Methotrexate) due to the persistence of clinical signs.

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