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BACKGROUND: Multisystemic inflammatory syndrome in children (MIS-C) is a novel disease that is associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2). MIS-C usually affects children older than 5 years of age and adolescents, with a median of 8-years and an interquartile range of 3 to 11 years. A multisystemic inflammatory disease has been described in neonates and named MIS-N (multisystemic inflammatory syndrome in Neonates). We report three cases of Mexican newborns with MIS-N presenting with multiorgan compromise and a positive anti-SARS-CoV-2 IgG who developed Kawasaki disease (KD)-like cardiac features and discuss the current dilemma regarding diagnosis and treatment in these patients.

Rowell syndrome (RS) is a rare disease characterized by the association of systemic lupus erythematosus (SLE) or cutaneous lupus with lesions similar to erythema multiforme and the presence of autoantibodies including ANA, SSA, SSB, or rheumatoid factor. Due to the low incidence of this disease, the epidemiology of RS is not clear. So far there are 95 cases reported in the literature; of these, only seven cases are pediatric patients. Macrophage activation syndrome (MAS) is an increasingly recognized complication of SLE, although its true prevalence in childhood is still unknown. We describe a unique pediatric patient with RS who developed MAS.

Background Pediatric-onset Systemic Erythematosus Lupus (pSLE) is known to be more aggressive than adult-onset SLE. The disease is extremely rare under the age of 5 years and with severe course. Another big difference is the association of pSLE with monogenic primary immune deficiencies (PID). Some PIDs have been associated with SLE or lupus-like manifestations: deficiencies of the classical complement pathway, female carriers of X-linked chronic granulomatous disease, IgA deficiency (present in 5% of pSLE). It is important to consider PIDs in patients with severe manifestations in pSLE, in our country there are no studies of this association. Objectives To describe the clinical manifestations, laboratory features, characteristics and therapeutics of patients with diagnosis of SLE before 6 years of age at National Institute of Pediatrics in Mexico City Methods This is a retrospective study with the review of the patient’s records with diagnosis of SLE before 6 years old, from the Immunology department between January 2005 and December 2016 at National Institute of Pediatrics in Mexico City. Results Between 2005-2016 there were 295 newly patients diagnosed with pediatric Systemic Lupus Erythematosus (pSLE) at our hospital, from these patients 2.7% (8) were diagnosed with pSLE before 6 years of age. According to this, 7 (87.5%) were female. The youngest patient was 26 months at moment of diagnosis. Every patient had at least 4 criteria of the ACR classification for SLE. Only 2 (25%) had malar rash at diagnosis, and 1 (12.5%) presented discoid rash. Seventy-five percent of patients presented glomerulonephritis at diagnosis and also 75% had an Hematological disorder. In terms of serology, 37.5% had positive native DNA antibody, 37.5% antibody to Sm protein and 37.5% antiphospholipid antibodies. One hundred percent of patients had presence of antinuclear antibody (ANA) at moment of diagnosis. Conclusion During 2005-2016 we found 295 newly patients with diagnosis of pediatric Systemic Erythematosus Lupus (pSLE), from these, eight patients (2.7%) were diagnosed before 6 years of age. Seventy-five percent presented recurrent infections and 50% with severe infections, and one patient died due to septic shock. Systemic erythematosus lupus with an early onset has a more severe phenotype and has been associated with monogenic primary immune deficiency, presenting with severe infections and higher mortality. In our country, we do not have genetic testing to help us determine the endless monogenic defects associated with early-onset SLE, being necessary to determine etiology and specific treatment as needed. We recommended that in every patient with severe pSLE who develops life-threatening, infections or due to unusual germs, associated with hypocomplementemia and/or consanguinity, to perform an approach to rule out any primary immune deficiency. References [1] Levy, D. Kamphuis Systemic Lupus Erythematosus in Children and Adolescents. Pediatr Clin North Am. 2012April; 59(2): 345–364 [2] F.Rivas-Larrauri, M.A.Yamazaki-Nakashimada. Lupus eritematoso sistemico: ?es una sola enfermedad?Reumatol Clin. 2016;12(5):274–281 [3] Carneiro M, Understanding Systemic Lupus Erythematosus Physiopathology in the Light of Primary Immunodeficiencies J Clin Immunol (2008) 28 (Suppl 1):S34–S41 [4] G. Tsokos, K. Sullivan, New insights into de immunopathogenesis of systemic lupus erythematosus. Nature Reviews (2016) 10.1038 Disclosure of Interests None declared

Morphoea, also known as localized scleroderma, is a disorder characterized by excessive collagen deposition leading to thickening of the dermis and/or subcutaneous tissues. Intravenous IgG therapy has induced improvement in some fibrotic conditions. The primary indication for subcutaneous IgG (SCIG) is in primary immunodeficiency disorders as replacement therapy; however, recently there has been considerable interest in SCIG as an immunomodulatory agent. We report an 11-year-old girl with deep morphoea who was successfully treated with SCIG.

Chronic granulomatous disease (CGD) is an infrequent inherited disorder characterized by recurrent infections and abnormal granuloma formation. Patients with CGD have an exuberant inflammatory response and an increased risk of developing autoimmunity. We present the case of a 1-year-old boy with CGD who developed several of the characteristic clinical features of Kawasaki Disease. His illness responded to intravenous immunoglobulin, aspirin, and corticosteroids.

We present the case of a four-year-old boy with a history of repeated upper respiratory tract infections and pyoderma. He presented fever, seizures, inability to talk, loss of swallowing, fine tremor in the upper extremities; positive bilateral Babinski reflex and quadriparesis. The diagnosis of Bruton's disease and generalized microporidiosis was based on immunologic analysis, smear tests with chromotrope R2 stain and indirect immunofluorescense with monoclonal 3B6 antibody for Encephalitozoon species in samples of spinal fluid, bronchial and paranasal sinus aspirates and stool, which were all positive. The patient was treated with albendazol during 72 days; he left the hospital in a good condition, walking, talking and able to swallow. His laboratory test controls were negative; he is followed up in the outpatient department.

In a retrospective study the authors analyzed the clinical records of 199 children ages one month to 16 years hospitalized, with the diagnosis of intestinal ascariasis, in the Instituto Nacional de Pediatria of Mexico from 1984 to 1999. The purpose of the study was to evaluate the use of anthelmintics drugs as a risk factor of intestinal obstruction by A. lumbricoides. Two groups were made for the study: Group A (n = 66) of children who presented intestinal obstruction, Group B (n = 133) children with no complications. A comparative analysis of clinical data of both groups was made by means of chi square with Yates correction and a stratified analysis by means of chi square. Possible confusing elements were overcrowding, age and the use of antiparasitic drugs. The calculus of risk factors for intestinal obstruction by A. lumbricoides was done by means of contingency tables of 2 x 2 and odds ratio with an IC of 95%. The significant risk factors were included in a model of logistics regression with an impact variable consting in the presence or absence of intestinal obstruction in order to establish a multivariate model of predictive risk at level of significance of p0.05. Twenty-seven patients (40.90%) in group A (n = 66) were given anthelmintics medications prior to the intestinal obstruction: mebendazol, 14 (51-85%); two, albedazol (7.4%); eight, a non-specified anthelmintic (29.6%). In addition, an anthelmintic medication without a specified time of ingestion: two with mebendazol and one with piperazine (11.3%). In the case of mebendazol, the drug most frequently associated with intestinal obstruction, seven patients received it on the same day of the obstruction; five patients received it between one and seven days prior to the obstruction; two received it seven days prior to the complication. In the control group, only 7% had taken the anthelmintic one to seven days before the diagnosis of uncomplicated intestinal ascariasis diagnosis was made. With the step by step (Backward) logistic regression conditioned by the treatment variable with an anthelmintic, an X2 = 38.15 gl, p0.000 was obtained for which reason it was considered by A. lumbricoides. Of the probable risk factors analyzed in this study, the only one capable of influencing and predicting the presentation of intestinal obstruction by A. lumbricoides in children, was the prior anthelmintic treatment particularly with mebendazol.