Category: Adrenal Disorders

Monitor: 24

24 - USE OF PREDNISONE TO ACHIEVE PREGNANCY IN NONCLASSIC CONGENITAL ADRENAL HYPERPLASIA

Saturday, Apr 27
11:00 AM – 11:30 AM

Objective :

Congenital adrenal hyperplasias (CAHs) are autosomal recessive disorders, with the majority of cases resulting from mutation in the CYP21A2 gene, leading to 21-hydroxylase deficiency, and defective conversion of 17-hydroxyprogesterone (17-OHP) to 11-deoxycortisol. Nonclassic CAH (NCCAH) is a less severe form that can present in adult females with signs of androgen excess. Pregnancy is rare as elevated progesterone levels can affect the endometrial receptivity and contribute to delayed implantation that result on miscarriage.


Methods :  


Results :

This is the case of a 28-year-old female that presented to endocrinology clinics for evaluation of irregular menses and nipple discharge. Patient had a history of infertility; physical examination remarkable for galactorrhea, acne, hirsutism, and no evidence of ambiguous genitalia. Work up revealed an elevated prolactin level at 164 (nl 3-30ng/ml), normal thyroid function tests, and negative pregnancy test. Brain MRI identified pituitary macroadenoma measuring 1.3x1.8x1.3cm. In view of elevated prolactin levels, macroprolactinoma diagnosed and cabergoline therapy started. Subsequent normalization of prolactin was achieved, with a decrease in prolactinoma size on follow up imaging. Elevated testosterone level at 1.0 (nl 0.1-0.75ng/ml) was also discovered which raised concern for concomitant androgen disorder. Steroid work-up showed elevated baseline 17-OHP at 1,296 (nl 15-290ng/dL), testosterones at 0.81 (nl 0.1-0.75ng/ml) and androstenedione levels at 565 (nl 28-230ng/dL) with no electrolytes disturbances, and normal cortisol levels. Adrenal CT showed no adrenal lesions. A diagnosis of NCCAH was made; dexamethasone and oral contraceptives (OCPs) therapy started. On follow-up visit fertility was desired, so dexamethasone and OCPs were discontinued, and prednisone 5mg daily started. Genotyping offered, but the patient was unable to pay for the test. Three months later, the patient was pregnant with 10 weeks of gestational age. Therapy has been continued with no adverse effect reported.


Discussion :  


Conclusion :

Pregnancy in NCCAH is challenging. Very few cases of successful pregnancies have been reported using prednisolone, but little data exist with prednisone use. Long-term use of dexamethasone is not recommended, because it crosses the placenta and has been associated with detrimental impact in intellectual development. Prednisone is metabolized by placental 11-beta-hydroxysteroid dehydrogenase, which is believed to protect the fetus. Since prednisone benefits to prevent early pregnancy loss or to improve pregnancy outcome in NCCAH have not been clearly described, this case may provide us with more information for future guidance and research.

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Jinetsy I. Rivera-Ortiz

Endocrinology Fellow
Univeristy of Puerto Rico- Medical Science Campus
Vega Alta, Puerto Rico

I am first year Endocrinology fellow in-training from Puerto Rico. I did my Internal Medicine specialty in the University of Puerto Rico-Medical Science Campus, and I'm currently Internal Medicine Board Certified. My interest are General Endocrinology, Thyroid Disease, Diabetes, Adrenal Disease and Bone.

Milliette Alvarado-Santiago

Attending Physician
Univeristy of Puerto Rico- Medical Science Campus

Dr. Milliette Alvarado Santiago is an Endocrinology Specialist in Rio Piedras, Puerto Rico. She graduated with honors from University Of Puerto Rico School Of Medicine in 2010. Having more than 8 years of diverse experiences, especially in ENDOCRINOLOGY, INTERNAL MEDICINE, Dr. Milliette Alvarado Santiago has dedicated her early career to the academic field, being full time assistant professor at the Endocrinology Fellowship at the University of Puerto Rico.

Sheila Mercado-Mendez

Endocrinology Fellow
Univeristy of Puerto Rico- Medical Science Campus

Dr. Sheila Mercado Mendez, MD is an internal medicine specialist in San Juan, PR. Currently, second year endocrinology fellow in training at University of Puerto Rico-Medical Science Campus.

Margarita Ramirez-Vick

Attending Physician
University of Puerto Rico - Medical Science Campus

Dr. Margarita Ramirez-Vick is an endocrinology specialist in San Juan, PR and has been practicing for 29 years. She graduated from University of Puerto Rico School of Medicine in 1989 and received her medical degree from University of Puerto Rico School of Medicine. She specializes in endocrinology, diabetes & metabolism.

Loida A. Gonzalez-Rodriguez

Assistant Professor
University of Puerto Rico School of Medicine - Endocrinology, Diabetes and Metabolism Division
San Juan, Puerto Rico

Loida A. Gonzalez-Rodriguez, MD is an Endocrinology, Diabetes & Metabolism specialist in San Juan, Puerto Rico. She graduated from University of Puerto Rico - School of Medicine in 2008, completed her specialty in Internal Medicine in 2011 and her sub-specialty in Endocrinology in 2014 at the University Hospital of Puerto Rico. Since then she has been Assistant Professor at the Endocrinology, Diabetes and Metabolism Division of the University of Puerto Rico. Dr. Gonzalez-Rodriguez also participates in different research projects in the area of endocrinology.

Jinetsy I. Rivera-Ortiz

Endocrinology Fellow
Univeristy of Puerto Rico- Medical Science Campus
Vega Alta, Puerto Rico

I am first year Endocrinology fellow in-training from Puerto Rico. I did my Internal Medicine specialty in the University of Puerto Rico-Medical Science Campus, and I'm currently Internal Medicine Board Certified. My interest are General Endocrinology, Thyroid Disease, Diabetes, Adrenal Disease and Bone.