Congenital adrenal hyperplasia with right adrenal adenoma in a 24 year-old black African lady
Jean-Claude Njabou Katte1,&, Eliane Ngassam2, Martine Etoa2, Eugene Sobngwi2,3
1Department of Public Health, Faculty of Medicine and Biomedical Sciences, University of Yaoundé 1, Yaoundé, Cameroon, 2National Obesity Center, Diabetes and Metabolic Diseases Unit, Yaoundé Central Hospital, Yaoundé, Cameroon, 3Department of Internal Medicine and Specialties, Faculty of Medicine and Biomedical Sciences, University of Yaoundé 1, Yaoundé, Cameroon
Jean-Claude Njabou Katte, Department of Public Health, Faculty of Medicine and Biomedical Sciences, University of Yaoundé 1, Yaoundé, Cameroon
Congenital Adrenal Hyperplasia (CAH) is an endocrine disorder comprising of a group of inherited autosomal recessive anomalies that cause a deficiency in an adrenal enzyme, resulting in altered cortisol and aldosterone secretion. We present this case in view of its complexity in making an accurate diagnosis, its rarity, low level of suspicion and hospital diagnosis, stigmatization and self-denial that usually accompanies the pathology.
this was a single case review of a young Cameroonian lady at the out-patient department of the National Obesity Centre.
a 24 year-old single lady who presented to the out-patient department of the National Obesity Centre of the Yaoundé Central Hospital on account of amenorrhea. She has never been sick or admitted before and consulted about 4 years prior to this present consultation for the same reasons. On examination, she is well-looking but with an abnormal facial hair distribution. Blood pressure of 114/79 mmHg, pulse rate of 80 bpm and weight 80 kg. She also presents with hirsutism, Tanner 2 for the mammary glands, Tanner 5 for the pubic hair distribution, clitoral hypertrophy with a total diameter of 2 cm. Investigations showed: Hormone profile showed: estradiol 215.66 pg/mL, follicule stimulating hormone 2.81 mUI/mL, progesterone 6.88 ng/mL, 17 alpha hydroxyl-progesterone 180.7 ng/mL, Testosterone 1.69 ng/mL. Total leukocyte count 7 150/uL (differential count – neutrophils: 48%, eosinophils: 3%, basophils: 0%, lymphocytes: 47%, monocytes: 2%), hemoglobin 13.9 g/dL, platelets s225 000/ul, fasting blood glucose 80 mg/dL, natremia 146.4 mmol/L, kalemia 3.3 mmol/L, chloremia 112.3 mmol/L, calcemia 98 mg/L, magnesemia 26mg/L, total bilirubin 13.9 mg/L, conjugated bilirubin 5.36 mg/L, non-conjugated bilirubin 8.54 mg/L, aspartate-amino transferase 16 IU/L, alanine-amino transferase 6 IU/L. Abdominal ultrasound showed a hypotrophic uterus measuring 43 x 27 x 23 (mm) with the endometrium not visible, dystrophic ovaries measuring 41 x 26 x 27 (mm) right and 39 x 21 x 23 (mm) left. Abdominal CT Scan with injection of contrast medium revealed a bilateral adrenal hypertrophy with a particular central necrosis on the right adrenal gland suggestive of an adenoma. The rest of the abdominal organs were morphologically normal. She was treated with hydrocortisone, cyproterone acetate and estradiol valerate.
Congenital Adrenal Hyperplasia should be considered in females who present with signs of virilization and infertility in our setting.