Ready for a deep dive into a real-life pediatric ICU situation? Today, Dr. Pradip Kamat, Dr. Monica Gray, and Dr. Rahul Damania will walk you through the case of a seven-year-old girl with Hemoglobin SC (HbSC) disease, who presents with abdominal swelling, pneumonia, low oxygen, and pain.
In this episode, our team unpacks the spleen’s anatomy and its crucial role in immunity, then zooms in on how sickle cell disease can throw a wrench in splenic function. You’ll hear how they approach the diagnosis and management of acute splenic sequestration crisis, sharing clinical pearls along the way. Plus, they’ll break down why quick recognition is so important and discuss strategies for both immediate and long-term care in pediatric sickle cell patients. Don’t miss these practical insights from the frontlines of pediatric critical care!
Show Highlights:
- Case study of a seven-year-old girl with hemoglobin SC disease
- Presentation of symptoms: abdominal distension, pneumonia, hypoxia, and body pain
- Discussion of acute splenic sequestration crisis as a complication of sickle cell disease
- Anatomy and physiology of the spleen
- The role of the spleen in sickle cell disease and how sickled cells affect splenic function
- Acute splenic sequestration crisis, including clinical features and laboratory evaluations
- Management strategies for acute splenic sequestration crisis in the ICU
- Importance of blood transfusions and supportive care in treatment
- Prophylactic measures to prevent recurrence of splenic sequestration
- Educational emphasis on recognizing clinical signs and the need for timely intervention
References:
- Fuhrman & Zimmerman - Textbook of Pediatric Critical Care Chapter 88. Hemoglobinopathies. Baender, MA, Marsh Anne. Pages: 1457-1470
- Rogers' textbook of pediatric intensive care: Hematologic Emergencies. McCory MC, Bhar S, and Blaine E. Pages 2003-2005
- Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014 Jul;166(2):165-76. doi: 10.1111/bjh 12950. Epub 2014 May 26. PMID: 24862308.
- Waleed S, Aldabsa M, Gouher S. Splenic Sequestration Induced by Parvovirus B19: A Case Report. Cureus. 2024 May 23;16(5):e60937. doi: 10.7759/cureus. 60937. PMID: 38915956; PMCID: PMC11195323.
- Solanki DL, Kletter GG, Castro O. Acute splenic sequestration crises in adults with sickle cell disease. Am J Med. 1986 May;80(5):985-90. doi: 10.1016/0002-9343(86)90649-2. PMID: 3706382.
- Karna B, Jha SK, Al Zaabi E. Hemoglobin C Disease. [Updated 2023 May 29]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK559043/
