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///2018 Abstract Details
2018 Abstract Details2019-08-02T15:57:01-05:00

A parturient with Clarkson's disease and preeclampsia with severe features: a complex combination

Abstract Number: F3C-1
Abstract Type: Case Report/Case Series

Hilary Sheridan MD1 ; Molly Cason MD2; Gina Hendren MD3

Introduction: Clarkson’s disease, also known as Idiopathic Systemic Capillary Leak Syndrome (SCLS), is a rare disease characterized by recurrent episodes of extreme capillary leakage leading to hypovolemic shock (1). We present a parturient with a six-year history of recurrent Clarkson’s episodes who developed preeclampsia with severe features.

Case Presentation: A 31-year-old G2P0 presented at 24 weeks gestation with symptoms of tachycardia, malaise, dyspnea, and back pain concerning for a recurrent capillary leak crisis. She was treated with intravenous immunoglobulin (IVIG), high-dose steroids and careful fluid resuscitation. Recovery was complicated by pulmonary edema requiring intensive care. In addition to signs of a SCLS crisis including extremity and facial swelling, hypoalbuminemia and diffuse pain, her blood pressure was elevated which is not typical of SCLS. A 24-hour urine protein collection showed significant proteinuria (>2g). Preeclampsia was not initially diagnosed due to the concurrence of a capillary leak crisis and uncertainty of whether SCLS could also cause proteinuria. Prior to delivery, she was admitted twice more for SCLS crisis. At 33 weeks and 6 days gestation, she developed headache, abdominal pain, and severely elevated blood pressure. Labor was induced for severe preeclampsia, and she had a spontaneous vaginal delivery under epidural anesthesia with an uneventful postpartum course.

Discussion: There are no published cases in the literature describing a parturient with Clarkson’s disease who also develops preeclampsia. This case demonstrates the complex overlap between the two conditions. Clarkson’s disease is characterized by episodes of varying intensity of hypovolemic shock and generalized edema. Common presenting symptoms include a prodromal flu-like illness, dyspnea, abdominal pain and edema. Specific laboratory findings suggesting SCLS include low albumin, hemoconcentration, and leukocytosis (1). The exact pathogenesis behind the disease is not well understood; however increased levels of cytokines, leukotrienes and vascular endothelial growth factor have been identified as possible culprits (2). While edema, dyspnea, abdominal pain and headache are seen in both preeclampsia and Clarkson’s disease, the additional diagnosis of preeclampsia was made based on persistent hypertension and proteinuria. This patient’s Clarkson’s disease remained quiescent during the peripartum period, but nonetheless highlights a unique comorbidity of a high risk obstetric patient.

References:

1. Eo TS, Chun KJ, Hong SJ, et al. Clinical presentation, management, and prognostic factors of idiopathic systemic capillary leak syndrome: A systematic review. J Allergy Clin Immunol Pract 2017 doi: 10.1016/j.jaip.2017.07.021 [Epub ahead of print]

2. Kapoor P, Greipp PT, Schaefer EW, et al. Idiopathic systemic capillary leak syndrome (Clarkson's disease): the Mayo clinic experience. Mayo Clin Proc 2010;85(10): 905-912.

SOAP 2018