Presentación inusual de infección por SARS-CoV-2 simulando púrpura de Schönlein Henoch (vasculitis por inmunoglobulina A)

La vasculitis por inmunoglobulina A, anteriormente llamada púrpura Schönlein Henoch (VIgA/PSH), es la vasculitis sistémica más frecuente en la infancia. El desencadenante más común es una infección previa del tracto respiratorio superior. Se caracteriza por púrpura palpable no trombocitopénica con artralgias y/o artritis, afectación gastrointestinal y compromiso renal. SARS-CoV-2 es un virus ARN que causa la enfermedad COVID-19. Afecta frecuentemente el sistema respiratorio con presentaciones que varían desde una rinitis hasta condiciones severas como síndrome de distress respiratorio, shock séptico o síndrome de in amación multisistémica (multi-system in ammation syndrome, MIS). Se describe el caso de un niño de 5 años de edad con clínica de VIgA/PSH como forma inicial de presentación y diagnóstico posterior de infección por SARS-CoV-2, derivado al hospital de mayor complejidad, con encefalopatía hipertensiva que presentó evolución favorable y restitución completa del cuadro clínico

Relationship between red blood cell transfusion requirements and severity of renal disease during the acute stage of hemolytic uremic syndrome

We performed a retrospective evaluation of patients with diarrhea-associated hemolytic uremic syndrome (D + HUS) with the aims of: (1) determining the rate of red blood cell (RBC) transfusions; (2) establishing the relationship between need for RBC transfusion and severity of renal involvement; (3) determining whether precise measurements of lactic dehydrogenase (LDH) levels can predict the rate of hemolysis and severity of renal disease. A total of 288 patients with D + HUS were retrospectively divided into three groups based on dialysis treatment: group 1, no dialysis treatment (144 patients); group 2, dialysis for 1–10 days (67 patients); group 3, dialysis for ≥11 days (77 patients). Of the patients in groups 1, 2 and 3, 73.6, 86.5 and 83.1 %, respectively, required at least one RBC transfusion. The number of RBC transfusions in groups 1, 2 and 3 was 163, 107 and 162, respectively. Comparison of the groups revealed that the number of RBC transfusions was significantly higher in patients in groups 2 and 3 than in those in group 1 (p = 0.0001). Most RBC transfusions (94.2 %) occurred during the first 2 weeks of the disease. The median peak LDH level was 2091 U/l in 32 patients with no RBC transfusion (group A), 3900 U/l in 73 patients with one transfusion (group B) and 6378 U/l in 62 patients with two or more transfusions (group C). Patients who received two or more RBC transfusions had a significantly higher median peak LDH level than those who did not receive RBC transfusions or received only one transfusion. This difference was also observed between patients who received only one RBC transfusion and those who did not receive any transfusions (p  10 days of dialysis (group 3) had the highest LDH levels, followed by patients with 1–10 days of dialysis (group 2) and then by patients with no dialysis requirements (group 1) (p < 0.00001). The rate of RBC transfusion was higher in patients with the most severe renal injury, and most were performed during the first 2 weeks of the disease. Patients with stable LDH levels seemed to require fewer RBC transfusions. Median peak LDH levels were significantly higher in the group of patients with the most severe renal disease.Facultad de Ciencias Veterinaria

Association of TNFSF15 polymorphism with irritable bowel syndrome

Background Irritable bowel syndrome (IBS) is the most common gastrointestinal disorder, affecting more than 10% of the general population worldwide. Although a genetic component is suspected, unambiguous susceptibility genes have so far not been identified. This study tested the hypothesis that genes contributing to epithelial barrier integrity, control of mucosal immune responses and interactions with bacteria in the gut are associated with IBS. Methods Single nucleotide polymorphisms (SNPs) corresponding to top signals of association with Crohn's disease at 30 known susceptibility loci were tested for their effect on IBS risk in 1992 individuals from two independent case-control cohorts from Sweden and the USA. Association tests included a conservative Bonferroni correction for multiple comparisons, and were also performed on specific subgroups of patients characterised by constipation (IBS-C), diarrhoea (IBS-D) or alternating constipation and diarrhoea (IBS-A). Results The Crohn's disease risk allele rs4263839 G in the TNFSF15 gene was significantly associated with an increased risk of both IBS (p=2.2 x 10(-5); OR 1.37) and more pronouncedly, IBS-C (p=8.7 x 10(-7); OR 1.79) in the entire sample. Similar associations and risk effects of the same magnitude were observed in the two cohorts analysed separately. A correlation between rs4263839 genotype and TNFSF15 mRNA expression was detected both in peripheral blood and in rectal mucosal biopsies from healthy individuals (combined p=0.0033). Conclusions TNFSF15 is a susceptibility gene for IBS and IBS constipation. As TL1A, the protein encoded by TNFSF15, contributes to the modulation of inflammatory responses, the results support a role of immune activation in IBS

Corrigendum to “A multicenter retrospective study of calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene variants.”

DOI of original article: https://doi.org/10.1016/j.kint.2023.02.022 The authors regret to report that Hee Gyung Kang, Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea, inadvertently was not included in the list of authors of the published article. The collaborator was removed from the Acknowledgments. This has been corrected in the article online. The authors would like to apologize for any inconvenience caused.</p