ABSTRACT
Since the beginning of the severe SARS-CoV-2 pandemic, an increasing number of countries reported cases of a systemic hyperinflammatory condition defined as multi-system inflammatory syndrome in children (MIS-C). The clinical features of MIS-C can be an overlap of Kawasaki Disease (KD), Toxic Shock Syndrome (TSS), Macrophage Activation Syndrome (MAS), or have often an acute abdominal presentation. Intravenous immunoglobulin (IVIG) is recommended as first line therapy in KD. Recent evidence suggests intravenous immunoglobulins (IVIG) resistance in some cases of SARS-CoV-2 related MIS-C, thereby questioning the benefit of immunomodulators such as IL-1 or IL-6 blocking agents. We report on a cohort of 6 Swiss children with SARS-CoV2 related MIS-C presenting with clinical features compatible with Incomplete KD and Toxic Shock Syndrome associated to a cytokine storm. Serum cytokine profile investigations showed increased IL1RA levels (8 to 22-fold) in 5 of the 6 patients (one patient had not been tested), whereas, IL-6 serum levels were increased only in the 3 patients of the 6 who were tested. With exception of one patient who had only benefited by Anakinra, all patients received at least one dose of IVIG. One patient has only received Anakinra with favorable evolution, and three patients had also a steroid treatment. In addition to all this anti-inflammatory medication two patients have also received one dose of anti-IL6. In conclusion, our case series reports on clinical and laboratory findings of most of Swiss cases with MIS-C and suggests the use of Anakinra as an alternative to steroids in these children, most of whom presented with high IL-1RA levels.
ABSTRACT
OBJECTIVES: The cause of orthostatic proteinuria is not clear but may often relate to obstruction of the left renal vein in the fork between the aorta and the superior mesenteric artery (=renal nutcracker). However, reports dealing with proteinuria only marginally refer to this possible cause of orthostatic proteinuria. We analysed the corresponding literature. RESULTS: Five reports addressed the frequency of renal nutcracker in 229 subjects with orthostatic proteinuria. Their age ranged between 5.2 and 17 years (female-to-male ratio: 0.96:1.00). Imaging studies demonstrated renal nutcracker in 156 (68%) subjects. Renal nutcracker was also demonstrated in 9 anecdotal reports for a total of 53 subjects with postural proteinuria. Very recently, 13 Italian subjects with orthostatic proteinuria associated with renal nutcracker were reassessed 6 years after the initial diagnosis: in nine subjects, both orthostatic proteinuria and renal nutcracker had disappeared; in three, both orthostatic proteinuria and renal nutcracker had persisted; and in one, orthostatic proteinuria had persisted unassociated with renal nutcracker. CONCLUSIONS: These data provide substantial support for renal nutcracker as a common cause of orthostatic proteinuria.
