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Dr. Paul Thornton and Dr. Blake Palmer lead a lively round-table discussion on reaching clinical excellence through exceptional interdisciplinary care for patients and families.
Dr. Paul ThorntonDr. Blake PalmerCortney Wolfe-Christensen PhDEmily Haddad LCSW
Related InformationCongenital Adrenal Hyperplasia (CAH)Treating Congenital Adrenal HyperplasiaCook Children's Endocrinology DepartmentCook Children's Pediatric Urology Department
Transcript
00:00:02
Host: Hello and welcome to Cook Children's Doc Talk. Today we're talking about congenital adrenal hyperplasia. Cook Children's is designated by the Cares Foundation as a Center of Excellence for Congenital Adrenal Hyperplasia. Joining us are members of the team, Dr. Paul Thornton, Medical Director, Endocrine and Diabetes Program, Dr. Blake Palmer, Medical Director Pediatric Urology and Surgical Director Kidney Transplantation. Dr Cortney Wolfe Christiansen, clinical child psychologist, and Emily Haddad, a licensed clinical therapist and social worker. Welcome to all of you. 
00:00:39
All: Thanks for being here. Thank you for having us, happy to be here.
00:00:43
Host: Congenital adrenal hyperplasia or CAH, is one of the most common inherited metabolic disorders. We know that there are two forms of CAH, classic and non classic. Non classic is a mild form of CAH and fairly asymptomatic, while classic CAH  is very severe and often life threatening. From birth, the classic form of CAH affects approximately one in 15,000 babies born each year. While we can touch briefly on the non classic form, I'd really like to focus on the classic form of CAH and the unique challenges faced when caring for children with this disease. Dr Thorton, as the medical director of this program can you take us through an overview of CAH?
00:01:25
Dr. Thornton: Yes, congenital adrenal hyperplasia CAH as we like to abbreviate it, is a condition in which there is an enzyme deficiency in the adrenal glands and as a result we get a deficiency in some of the most critical hormones in the body for example cortisol with in excess of the sex steriods, particularly the androgens being produced. And why this is very important is that cortisol is essential for life and in times of critical illness our bodies make more cortisol in order to help us survive and children with CAH are unable to do this and hence they can have life threatening adrenal crises which can be manifested as hypotension and hypoglycemia and can be critically dangerous.
So with classical congenital adrenal hyperplasia there are 2 forms. there are those who are missing the cortisol and that would be called simple virilizing, and then 75 percent of the kids are missing both cortisol and aldosterone which is called salt wasting CAH, and that is by far the most dangerous of the 2 conditions.
00:02:31
Host: Dr Thorton, your specialty is endocr...

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