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Endocrinology NRIMC HYPOPITUITARISM Dr Srikanth M.D., D.M. Associate Professor Dept. of Endocrinology NRIAS Dr Sirisha M.D. Senior Resident Dept. of Endocrinology NRIAS Endocrinology Hypopituitarism NRIMC • Underdiagnosed entity • Diagnosis Requires – – – – High index of suspicion Low threshold for investigations Availability of all dynamic endocrine investigations Endocrine expertise while carrying out investigations • Not so rare Endocrinology NRIMC A 26 year old male patient, a product of consangenous parentage presented with Growth Retardation Failure to develop secondary sexual characters normally . Decreased frequency of nocturnal erections Reduced exercise capacity Endocrinology NRIMC No H/o head injury No H/o Anosmia / Visual abnormalities No evidence of Chronic illness / Viral infection No H/o any drug abuse Endocrinology • General Examination: . NRIMC PR – 88/min BP – 110/70 mm Hg without any postural variation Weight – 42 Kg Height – 152 Cm • US – 65 cm • LS _ 87 cm • Arm span – 158 cms Endocrinology • Genital Examination: . NRIMC Pubic Hair – P2 SPL – 10cm Testes Volume – Prepubertal No facial hair & hair in other androgen dependent areas Axillary hair – present but minimal Other systemic examination is normal Anosmia –ve, CNS - Normal Endocrinology Bone age NRIMC • Bone age - 13 years – Delayed – Chronological age – 26 years – Epiphyses – still unfused Endocrinology NRIMC Thyroid function tests Parameter Result Units Normal range T3 0.92 ng/ml 0.8-1.81 FT3 2.38 pg/ml 0.92-11.25 T4 3.3 gm/dl 4.0 -12.6 FT4 0.29 ng/dl 0.89-1.76 TSH 11.34 IU/ml 0.35 -5.50 Comment Central Hypothyroidism / Primary hypothyroidism Endocrinology NRIMC Gonadal axis evaluation Parameter Result Units Comment FSH 0.60 mIU/ml Low LH 0.29 U/L Low Testosterone 0.02 ng/ml Low Impression Central Hypogonadism Endocrinology Growth Axis NRIMC • IGF 1 – Active form of GH – < 25 ng/ml – Normal range ( 116-358 ng/ml) Endocrinology NRIMC Parameter Adrenal axis evaluation Result 27 • ACTH Basal Cortisol 2.0 ACTH Stim cortisol Units 27Pg/ml pg/ml g/dl Comment Normal Normal 4.3- 22.4 Low Not done • Basal cortisol 2.0 mcg/dl Low Central Hypocortisolism ! Impression Endocrinology NRIMC Endocrinology NRIMC • Empty sella appearance Endocrinology NRIMC Endocrinology • A 42 year old Female Patient presented with . NRIMC Recurrent episodes of facial puffiness of 10 years duration Dryness of skin associated with scaling & itching Amenorrhoea of 10 years duration Loss of pubic hair & axillary hair Weakness, fatiguability, depression & impaired memory Previous H/o PPH and Lactation failure Endocrinology • General Examination . • Periorbital puffiness • Skin – dry, coarse & scaly • No axillary and pubic hair • Breast atrophy • PR – 90/min • BP – 100/70 mm Hg without postural variation NRIMC • Other Systemic examination is Normal Endocrinology NRIMC Endocrinology NRIMC • • • • Hemogram Hb 10.6 gm% WBC count – 4200 Platelets – 2.1 lakh DC – normal • Mild normocytic normocytic anemia Endocrinology NRIMCParameter • • • • • • • • • • Creatinine Bilirubin SGOT SGPT SAP : Total protein Albumin Globulin Na K Biochemistry Result : : : : 87 : : : : : 1.1 0.4 115 46 U/L 7.9 4.0 3.9 126 2.6 mg% ( mg% U/L U/L gm/dl gm/dl gm/dl meq/l meq/l Normal range Endocrinology NRIMC Thyroid function tests Parameter Result Units Normal range T3 < 0.01 ng/ml 0.8-1.81 FT3 1.03 pg/ml 0.92-11.25 T4 < 0.01 gm/dl 4.0 -12.6 FT4 0.11 ng/dl 0.89-1.76 TSH 2.38 IU/ml 0.35 -5.50 Comment Central Hypothyroidism Endocrinology NRIMC Gonadal axis evaluation Parameter Result Units Comment FSH 1.98 mIU/ml Low LH < 0.07 U/L Low Estradiol 28.30 pg/ml Low Impression Central Hypogonadism Endocrinology NRIMC Adrenal axis evaluation Parameter Result Units Comment ACTH 10 Pg/ml Low Basal Cortisol 2.0 g/dl 4.3- 22.4 Low Stimulated cortisol- 0 min 1.2 g/dl > 20 g/dl Low Stimulated cortisol- 45 min 12.8 g/dl > 20 g/dl Low Stimulated cortisol- 60 min 10.4 g/dl > 20 g/dl Low Stimulated cortisol- 90 min 8.0 g/dl > 20 g/dl Low Stimulation test was done by insulin tolerance test (0.15 U /Kg) Impression Central Hypocortisolism Endocrinology NRIMC Growth Hormone axis evaluation Parameter Result Units Comment Basal GH < 0.5 ng/ml Low Stimulated GH - 0 min < 0.5 ng/ml > 10 ng/ml Low Stimulated GH - 45 min < 0.5 ng/ml > 10 ng/ml Low Stimulated GH - 60 min < 0.5 ng/ml > 10 ng/ml Low Stimulated GH - 90 min < 0.5 ng/ml > 10 ng/ml Low Stimulated GH - 120 min < 0.5 Ng/ml > 10 ng/ml Low IGF -1 Stimulation test was done by insulin tolerance test (0.15 U /Kg) Peak GH response of > 10 ng/ml is considered normal response Impression Adult Growth Hormone deficiency Endocrinology Radiological evaluation NRIMC MRI of pituitary is suggestive of partial empty sella Minimal pituitary tissue is present at floor of sella Endocrinology Hypothyroidism NRIMC 1. 2. 3. 4. 5. 6. 7. Puffiness Lethargy Coma Weakness Infertility Cramps Can not tolerate LT4 Hypopituitarism Hypogonadism 1. Pubertal delay 2. Infertility 3. Amenorrhea * Primary * Secondary 4. Loss of libido 5. Breast atrophy 6. Osteoporosis Hypocortisolism GH deficiency Low Prolactin 1. 2. 3. 4. 5. Children 1.Growth failure 2.Shortstature Lactational failure Weakness Weight loss Lethargy Hypotension Postural hypotension 6. Giddiness 7. Vomitings 8. Diarrhea 9. Pain abdomen 10. Refractory shock 11. Hyponatremia Adults 1.Adult growth hormone deficiency No symptoms in male Endocrinology NRIMC Causes of Hypopituitarism • Heriditory Pituitary Hormone deficiency ( Pit 1, Prop 1, LHX 3, HESX1) • Acquired Pituitary hormone deficiency – Infections – Infiltrative rare • Primary Hypophysitis ( Lymphocytic, Granulomatous, Xanthomatous) • Secondary Hypophysitis ( Histiocytosis X, sarcoidosis ) – Neoplastic • Pituitary Tumor and parasellar tumors • Craniopharyngioma etc • Post Pituitary surgery – Trauma • Radiation, surgical resection, brain trauma – Vascular • Apoplexy, pregnancy related ( post partum pituitary necrosis), Hypotension Endocrinology NRIMC Summary • High index of suspicion is required • Low threshold of investigations • Seek for endocrine consultation where ever there is a doubt • Treatment is difficult – Life threatening hypocortisolemia / hypothyroid coma can occur – Multiple pituitary hormone replacement is needed in physiological manner – Stress advise regarding the steroid replacement is mandatory – Iatrogenic cushings is possible – Constant reinforcement is needed for good complaince – Fertility needs special protocols both in males and females