The catastrophic antiphospholipid syndrome in children

Go, Ellen J.L.; O’Neil, Kathleen M.

doi:10.1097/bor.0000000000000426

Cited by 25 publications

(22 citation statements)

References 48 publications

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“…[22][23][24] Additionally, the severe thrombocytopenia and schistocytosis observed in Case 3 are very rare or absent in CAPS but frequent in HUS. [22][23][24][25] We also noted the use of plasmapheresis with 5% albumin in Case 3, based on the possibility of eliminating anti-T IgM antibodies and bacterial neuraminidase, 26 with good results in the clinical and hemodynamic state of the patient. Nonetheless, some authors do not recommend this therapy, as it can increase pre-existing secondary hemolysis in the presence of anti-T IgM class antibodies in the plasma and result in a reaction with the T antigen exposed in patients with Sp-HUS.…”

Section: Discussionmentioning

confidence: 91%

“…Although the differential diagnosis with other entities, such as catastrophic antiphospholipid syndrome (CAPS), was difficult in this case due to the lack of additional studies, such as lupus anticoagulant and anticardiolipin antibodies, during hospital stay, our patient had no thrombosis documented in the main organs involved in CAPS (kidney, heart, brain, liver). [22][23][24] The multi-system thrombosis in CAPS leads to high morbidity and mortality. [22][23][24] Additionally, the severe thrombocytopenia and schistocytosis observed in Case 3 are very rare or absent in CAPS but frequent in HUS.…”

Section: Discussionmentioning

confidence: 99%

“…[22][23][24] The multi-system thrombosis in CAPS leads to high morbidity and mortality. [22][23][24] Additionally, the severe thrombocytopenia and schistocytosis observed in Case 3 are very rare or absent in CAPS but frequent in HUS. [22][23][24][25] We also noted the use of plasmapheresis with 5% albumin in Case 3, based on the possibility of eliminating anti-T IgM antibodies and bacterial neuraminidase, 26 with good results in the clinical and hemodynamic state of the patient.…”

Section: Discussionmentioning

confidence: 99%

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Hemolytic Uremic Syndrome Associated With Streptococcus Pneumoniae in Pediatrics: A Case Series

Guerra¹,

Rodríguez²,

Camacho

et al. 2020

Rev. paul. pediatr.

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Objective: To describe a case series of four (4) patients with hemolytic uremic syndrome due to Streptococcus pneumoniae in a level four complexity institution in the city of Bogotá, D.C., Colombia. Cases description: We describe cases of four patients who presented respiratory symptoms and fever. All four patients were in regular conditions on hospital admission, after which they required intensive care and ventilatory support. Upon admission, three cases showed evidence of pleuropulmonary complication. Penicillin-sensitive Streptococcus pneumoniae was isolated in all cases. All patients presented anemia, severe thrombocytopenia, schistocytes on peripheral blood smear, and hyperazotemia. They required blood transfusion and renal replacement therapy during their hospitalization. The patients were diagnosed with hemolytic uremic syndrome due to S. pneumoniae. Three of the four patients had a progressive recovery of the renal function and were discharged after an average of 36 days of hospital stay. The remaining patient had two amputations in the extremities due to thrombotic vascular complications and was discharged after 99 days of hospital stay, requiring hemodialysis every other day. Comments: Hemolytic uremic syndrome due to Streptococcus pneumoniae is a rare but severe complication of invasive pneumococcal disease. Complicated pneumonia is the main condition associated with this entity. It is noteworthy the short period in which these cases were presented, considering the low annual incidence of the disease.

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Hemolytic Uremic Syndrome Associated With Streptococcus Pneumoniae in Pediatrics: A Case Series

Guerra¹,

Rodríguez²,

Camacho

et al. 2020

Rev. paul. pediatr.

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“…CAPS is a life-threatening presentation of APS, typically characterized by systemic inflammation and rapid (within 1 week) development of widespread microvascular thrombosis in multiple organ systems (3 or more), similar to other thrombotic microangiopathies [2,5,48,49]. Preliminary classification criteria defining "definite" and "probable" CAPS were established and validated in adults and children [50,51].…”

Section: Catastrophic Apsmentioning

confidence: 99%

Pediatric Antiphospholipid Syndrome: from Pathogenesis to Clinical Management

et al. 2021

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Purpose of Review Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome (APS). Recent Findings aPL are traditionally regarded as the main pathogenic players in APS, inducing thrombosis via the interaction with fluid-phase and cellular components of coagulation. Recent APS research has focused on the role of β2 glycoprotein I, which bridges innate immunity and coagulation. In pediatric populations, aPL should be screened in appropriate clinical settings, such as thrombosis, multiple-organ dysfunction, or concomitant systemic autoimmune diseases. Children positive for aPL tests often present non-thrombotic non-criteria manifestations or asymptomatic aPL positivity. In utero aPL exposure has been suggested to result in developmental disabilities, warranting long-term follow-up. Summary The knowledge of the multifaceted nature of pediatric APS should be implemented to reduce the risk of underdiagnosing/undertreating this condition. Hopefully, recent pathogenic insights will open new windows of opportunity in the management of pediatric APS.

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“…Kidney is the most commonly affected organ, followed by the lung, the central nervous system, the heart, and the skin. The skin involvement presents as livedo reticularis, skin ulcers, cutaneous necrosis, gangrene, digital ischemia, and purpura . Among laboratory investigations, thrombocytopenia was found to be one of the main hallmarks of pediatric CAPS as noted in 70.5% of patients in CAPS registry …”

mentioning

confidence: 99%