In this episode of "PICU Doc on Call," Dr. Pradip Kamat and Dr. Rahul Damania dive into a fascinating case of a 9-month-old infant who comes in with hypoglycemia and seizures. Together, they break down the basics of glucose metabolism, walk through the causes of hypoglycemia, and discuss the best diagnostic strategies and acute management steps. They put a special spotlight on using diazoxide for hyperinsulinemic hypoglycemia, discussing not only how it works but also its potential side effects. The conversation also discusses dietary interventions for metabolic disorders and highlights the importance of rapid diagnosis and personalized treatment.

Show Highlights:

1. Pediatric hypoglycemia and its implications in infants
2. Case study of a 9-month-old infant with hypoglycemia and seizures
3. Physiology of glucose metabolism and its regulation
4. Causes of hypoglycemia, categorized into primary and secondary etiologies
5. Diagnostic approaches for identifying the cause of hypoglycemia
6. Initial management strategies for acute hypoglycemia
7. Long-term treatment options based on underlying causes
8. Importance of timely diagnosis and intervention in the PICU setting
9. Pharmacologic management of hyperinsulinemic hypoglycemia, including the use of diazoxide
10. Multidisciplinary care and follow-up for pediatric patients with hypoglycemia

1. Fuhrman & Zimmerman - Textbook of Pediatric Critical Care Chapter 84 Alder M et al. Pediatric Sepsis. Pages 1293-1309
2. Honarmand K, Sirimaturos M, Hirshberg EL, Bircher NG, Agus MSD, Carpenter DL, Downs CR, Farrington EA, Freire AX, Grow A, Irving SY, Krinsley JS, Lanspa MJ, Long MT, Nagpal D, Preiser JC, Srinivasan V, Umpierrez GE, Jacobi J. Society of Critical Care Medicine Guidelines on Glycemic Control for Critically Ill Children and Adults 2024. Crit Care Med. 2024 Apr 1;52(4):e161-e181. doi: 10.1097/CCM.0000000000006174. Epub 2024 Jan 19. PMID: 38240484.
3. Rosenfeld E, Thornton PS. Hypoglycemia in Neonates, Infants, and Children. 2023 Aug 22. In: Feingold KR, Ahmed SF, Anawalt B, Blackman MR, Boyce A, Chrousos G, Corpas E, de Herder WW, Dhatariya K, Dungan K, Hofland J, Kalra S, Kaltsas G, Kapoor N, Koch C, Kopp P, Korbonits M, Kovacs CS, Kuohung W, Laferrère B, Levy M, McGee EA, McLachlan R, Muzumdar R, Purnell J, Rey R, Sahay R, Shah AS, Singer F, Sperling MA, Stratakis CA, Trence DL, Wilson DP, editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000–. PMID: 37665756.
4. Rayas MS, Salehi M. Non-Diabetic Hypoglycemia. 2024 Jan 27. In: Feingold KR, Ahmed SF, Anawalt B, Blackman MR, Boyce A, Chrousos G, Corpas E, de Herder WW, Dhatariya K, Dungan K, Hofland J, Kalra S, Kaltsas G, Kapoor N,...

Today, Dr. Monica Gray, Dr. Pradip Kamat, and Rahul Damania discuss a critical case involving a 10-year-old boy who developed post-intubation desaturation. Using the DOPE mnemonic (Displacement, Obstruction, Pneumothorax, Equipment failure), they systematically troubleshoot the emergency, highlighting the importance of teamwork, capnography, and manual ventilation. The team emphasizes structured approaches, simulation training, and essential bedside tools to ensure rapid, effective management of acute deterioration in intubated children, turning a life-threatening crisis into a controlled, solvable situation.

Show Highlights:

• Clinical case discussion of a ten-year-old boy with post-intubation desaturation in the pediatric ICU
• Use of the "DOPE" mnemonic (Displacement, Obstruction, Pneumothorax, Equipment failure) for troubleshooting
• Systematic approaches in emergency situations in pediatric critical care
• Assessment and management of sudden desaturation in intubated patients
• Evaluation of potential causes of desaturation, including tube displacement and obstruction
• Role of equipment failure in acute deterioration and strategies to address it
• Significance of continuous capnography and manual ventilation techniques
• Prevention strategies for unplanned extubation in pediatric ICU settings
• Emphasis on teamwork, communication, and simulation training in crisis management
• Review of literature insights related to hypoxemia and equipment issues in pediatric intubation

References:

• Topjian AA, et al. Part 4: Pediatric Basic and Advanced Life Support—2020 AHA PALS Guidelines. Circulation. 2020.Foundational pediatric resuscitation guidance endorsing early switch to manual ventilation and structured troubleshooting for the deteriorating intubated child.
• Cook TM, et al. Major complications of airway management in the UK: NAP4. British Journal of Anaesthesia. 2011.Seminal audit highlighting ICU/ED airway failures and the critical role of waveform capnography in preventing unrecognized esophageal intubation.
• Volpicelli G, et al. International evidence-based recommendations for point-of-care lung ultrasound. Intensive Care Medicine. 2012. High-impact consensus placing lung ultrasound at the bedside to rapidly diagnose pneumothorax during post-intubation deterioration.
• Prekker ME, et al. Video vs direct laryngoscopy for ED intubation—randomized trial. New England Journal of Medicine. 2023.NEJM RCT showing higher first-pass success with video laryngoscopy—relevant to preventing displacement/misplacement drivers of desaturation.
• Chrimes N, et al. Preventing unrecognised oesophageal intubation: consensus guideline. Anaesthesia. 2022.Modern, practice-changing guidance: sustained waveform capnography is the mainstay to exclude esophageal placement and avert catastrophic hypoxemia.

Welcome to "PICU Doc on Call," the podcast where the world of pediatric critical care comes alive! Today, Dr. Monica Gray, Dr. Pradip Kamat, and Rahul Damania delve into a fascinating case involving a 16-year-old male presenting with headache, photophobia, anemia, and cerebral venous thrombosis. After some detective work, the diagnosis? Paroxysmal nocturnal hemoglobinuria, or PNH.

Join us as we break down the pathogenesis and clinical features of PNH, walk through the diagnostic workup, and discuss management strategies, especially the game-changing role of complement inhibitors like Eculizumab. We’ll also review this patient’s clinical journey, highlighting the key pearls for recognizing and treating PNH in the pediatric intensive care unit.

So, tune in to hear more!

Show Highlights:

• Clinical case presentation of a 16-year-old male with symptoms including headache, photophobia, and anemia
• Diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) and its clinical significance
• Pathogenesis of PNH, including the role of the PIGA gene mutation and GPI-anchored proteins
• Clinical features and complications associated with PNH, such as thrombosis and hemolysis
• Diagnostic workup for PNH, including laboratory tests and flow cytometry
• Management strategies for PNH, focusing on complement inhibitors like Eculizumab
• Importance of supportive care in the PICU for patients with PNH
• Discussion of emerging therapies and advancements in PNH treatment
• Patient outcome and clinical course following treatment for PNH
• Key takeaways regarding the diagnosis and management of PNH in pediatric intensive care

References:

• Fuhrman & Zimmerman - Textbook of Pediatric Critical Care.
• Reference 1: Brodsky RA. Paroxysmal nocturnal hemoglobinuria. Blood. 2014 Oct 30;124(18):2804-11.
• Reference 2 Waheed A, Shammo J, Dingli D. Paroxysmal nocturnal hemoglobinuria: Review of the patient experience and treatment landscape. Blood Rev. 2024 Mar;64:101158.
• Reference 3: Kokoris S, Polyviou A, Evangelidis P, Grouzi E, Valsami S, Tragiannidis K, Gialeraki A, Tsakiris DA, Gavriilaki E. Thrombosis in Paroxysmal Nocturnal Hemoglobinuria (PNH): From Pathogenesis to Treatment. Int. J. Mol. Sci. 2024 Nov 11;25(22):12104.