Endocrine Abstracts

A female patient was first referred to the Endocrinology department due to primary amenorrhea at age 37. She mentioned trans-sphenoidal surgery at age 15 due to a craniopharyngioma and the indication to be supplemented with hydrocortisone (HC) and DDAVP, that she adhered to until age 20, when she abandoned all appointments. Apart from that, she mentioned normal pubertal development and said she was generally healthy. She denied any episodes compatible with Addisonian crises. From the interview it was clear she was polyureic (≥2 mictions every night), depressed, asthenic. Her complains were unspecific muscle-joint aches, tiredness, lack of drive, easy crying. Observation revealed short stature (142 cm; target 159 cm), obesity (84 kg) of android distribution, peripheral chronic edema of the limbs and face, puffy hands (indicis ring size of 24.5 mm), hypokinetic movements, BP 94/66 mmhg, HR 64 bpm, unpalpable thyroid, external female genitalia. Breast tissue was soft and no gland bud could be palpable. She was extremely reluctant to start HC because of the fear of weight gain, but after a comprehensive explanation, finally agreed on making blood tests the next day and start HC immediately after. Laboratory: glucose 76 (80–100) mg/dl, Na 137 (135–124) mmol/l, K 4.35 (3.5–4.5) mmol/l, PRL 24 (10–28) ng/ml, TSH 10 mIU/L, fT4l 6 (10–24) pg/ml, fT3l 2.76 (8–12) pg/ml, FSH and LH <1 mIU/ml, E2<5 pg/ml, GH 0.14 ng/ml, IGF1<40 ng/ml, ACTH 5.4 (10–52) pg/ml, cortisol 0.6 (5–25) ug/dl, 24 h urinary volume 6300 ml, urine density 615 mOsm. The Pelvic-US revealed an infantile uterus of 60×15×18 mm (L×AP×T) without visible endometrium or ovaries and the breast-US confirmed the presence of adipose tissue. Bone health was compromised with a lumbar and femoral Z score of −3.2 and −2 respectively on dual-energy x-ray absorptiometry. The thyroid-scan showed a normal gland with 19 cc of volume. After 1 week, under HC substitution, there was a dramatic improvement of vigour, activity and well-being. She seemed euthymic. Nocturia was controlled with DDAVP at night. Levothyroxine was then started and after 2 weeks a estradiol patch was initiated together with calcium and vitamin D. TD estradiol was prescribed as for puberty induction regimens but up-titrated every 4 months. After one year she lost 7 kg, has a gynoid fat distribution, significantly less peripheral edemas (indicis of 23 mm), shows a positive attitude towards life, has no complaints and adheres daily to treatment.