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How We Approach: Pediatric Congenital Chylous Effusions and Ascites
  • +3
  • Taizo Nakano,
  • Yoav Dori,
  • Lindsey Gumer,
  • Deborah Liptzin,
  • Lauren Hill,
  • Ann Kulungowski
Taizo Nakano
Children's Hospital Colorado
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Yoav Dori
Children's Hospital of Philadelphia
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Lindsey Gumer
Children's Hospital Colorado
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Deborah Liptzin
Children's Hospital Colorado
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Lauren Hill
Children's Hospital Colorado
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Ann Kulungowski
Children's Hospital Colorado
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Abstract

Congenital lymphatic leak may develop in patients with maldeveloped lymphatics and result in life-threatening fluid and electrolyte imbalance, protein deficiency and immunodeficiency. Rapid diagnosis and therapy are necessary to prevent these complications; however, the field lacks clinical trials to support standardized diagnostic treatment guidelines. We present our current multidisciplinary approach to the diagnosis and management of congenital lymphatic leak including chylous pleural effusion and ascites. Depending on the rate of lymphatic leak, therapy can range from observation with nutritional modifications to surgical and interventional procedures aimed to reduce lymphatic drainage. Modalities to image central and peripheral lymphatics have advanced considerably. Genetic variants and subsequent targets that drive lymphatic maldevelopment have expanded the repertoire of possible pharmacotherapeutic options.

Peer review status:UNDER REVIEW

23 Apr 2021Submitted to Pediatric Blood & Cancer
23 Apr 2021Submission Checks Completed
23 Apr 2021Assigned to Editor
26 Apr 2021Reviewer(s) Assigned
01 May 2021Review(s) Completed, Editorial Evaluation Pending
01 May 2021Editorial Decision: Revise Major
05 Jun 2021Submission Checks Completed
05 Jun 2021Assigned to Editor
05 Jun 20211st Revision Received
21 Jun 2021Reviewer(s) Assigned