NRIMC

Endocrinology

HYPOPITUITARISMHYPOPITUITARISM

Dr Srikanth M.D., D.M.Dr Srikanth M.D., D.M.Associate ProfessorAssociate Professor

Dept. of EndocrinologyDept. of EndocrinologyNRIASNRIAS

Dr Sirisha M.D.Dr Sirisha M.D.Senior ResidentSenior Resident

Dept. of EndocrinologyDept. of EndocrinologyNRIASNRIAS

NRIMC

Endocrinology

HypopituitarismHypopituitarism

• Underdiagnosed entityUnderdiagnosed entity

• Diagnosis Requires Diagnosis Requires – High index of suspicionHigh index of suspicion

– Low threshold for investigationsLow threshold for investigations

– Availability of all dynamic endocrine investigations Availability of all dynamic endocrine investigations

– Endocrine expertise while carrying out investigationsEndocrine expertise while carrying out investigations

• Not so rareNot so rare

NRIMC

Endocrinology

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A 26 year old male patient, a product ofA 26 year old male patient, a product of consangenous parentage presented with consangenous parentage presented with

Growth RetardationGrowth Retardation

Failure to develop secondary sexual charactersFailure to develop secondary sexual characters normallynormally

Decreased frequency of nocturnal erectionsDecreased frequency of nocturnal erections

Reduced exercise capacityReduced exercise capacity

NRIMC

Endocrinology

No H/o head injuryNo H/o head injury

No H/o Anosmia / Visual abnormalitiesNo H/o Anosmia / Visual abnormalities

No evidence of Chronic illness / Viral infectionNo evidence of Chronic illness / Viral infection

No H/o any drug abuseNo H/o any drug abuse

NRIMC

Endocrinology

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• General ExaminationGeneral Examination::

PR – 88/minPR – 88/min

BP – 110/70 mm Hg without any postural variationBP – 110/70 mm Hg without any postural variation

WeightWeight – 42 Kg – 42 Kg HeightHeight – 152 Cm – 152 Cm• US – US – 65 cm 65 cm• LS LS _ _ 87 cm 87 cm• Arm spanArm span – 158 cms – 158 cms

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Endocrinology

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• Genital ExaminationGenital Examination::

Pubic Hair – P2Pubic Hair – P2

SPL – 10cmSPL – 10cm

Testes Volume – PrepubertalTestes Volume – Prepubertal

No facial hair & hair in other androgen dependent areas No facial hair & hair in other androgen dependent areas

Axillary hair – present but minimalAxillary hair – present but minimal Other systemic examination is normalOther systemic examination is normalAnosmia –ve, CNS - NormalAnosmia –ve, CNS - Normal

NRIMC

Endocrinology

Bone ageBone age

• Bone age -Bone age - 13 years13 years– DelayedDelayed– Chronological age – 26 yearsChronological age – 26 years– Epiphyses – still unfusedEpiphyses – still unfused

NRIMC

Endocrinology

Thyroid function testsThyroid function testsParameter 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

NRIMC

Endocrinology

Gonadal axis evaluationGonadal 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

NRIMC

Endocrinology

Growth AxisGrowth Axis

• IGF 1 IGF 1 – Active form of GHActive form of GH– < 25 ng/ml< 25 ng/ml– Normal range ( 116-358 ng/ml)Normal range ( 116-358 ng/ml)

NRIMC

Endocrinology

Adrenal axis evaluationAdrenal axis evaluation

• ACTH ACTH 27 pg/ml27 pg/ml NormalNormal

• Basal cortisolBasal cortisol 2.0 mcg/dl2.0 mcg/dl LowLow

Parameter Result Units Comment

ACTH 27 Pg/ml Normal

Basal Cortisol 2.0 g/dl 4.3- 22.4 Low

Stim cortisol Not done

Impression Central Hypocortisolism !

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Endocrinology

NRIMC

Endocrinology

• Empty sella appearanceEmpty sella appearance

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Endocrinology

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Endocrinology

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• A 42 year old Female Patient presented withA 42 year old Female Patient presented with

Recurrent episodes of facial puffiness of 10 years durationRecurrent episodes of facial puffiness of 10 years duration

Dryness of skin associated with scaling & itchingDryness of skin associated with scaling & itching

Amenorrhoea of 10 years durationAmenorrhoea of 10 years duration

Loss of pubic hair & axillary hairLoss of pubic hair & axillary hair

Weakness, fatiguability, depression & impaired memoryWeakness, fatiguability, depression & impaired memory

Previous H/o PPH and Lactation failurePrevious H/o PPH and Lactation failure

NRIMC

Endocrinology

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• General ExaminationGeneral Examination

• Periorbital puffinessPeriorbital puffiness

• Skin – dry, coarse & scalySkin – dry, coarse & scaly

• No axillary and pubic hairNo axillary and pubic hair

• Breast atrophyBreast atrophy

• PR – 90/minPR – 90/min

• BP – 100/70 mm Hg without postural variationBP – 100/70 mm Hg without postural variation

• Other Systemic examination is NormalOther Systemic examination is Normal

NRIMC

Endocrinology

NRIMC

Endocrinology

HemogramHemogram

• Hb 10.6 gm%Hb 10.6 gm%

• WBC count – 4200WBC count – 4200

• Platelets – 2.1 lakhPlatelets – 2.1 lakh

• DC – normalDC – normal

• Mild normocytic normocytic anemiaMild normocytic normocytic anemia

NRIMC

Endocrinology

BiochemistryBiochemistry

• CreatinineCreatinine :: 1.1 1.1 mg% (mg% (• BilirubinBilirubin :: 0.40.4 mg%mg%• SGOTSGOT :: 115115 U/LU/L• SGPTSGPT :: 4646 U/LU/L• SAPSAP :: 8787 U/LU/L• Total proteinTotal protein :: 7.97.9 gm/dlgm/dl• AlbuminAlbumin :: 4.04.0 gm/dlgm/dl• GlobulinGlobulin :: 3.93.9 gm/dlgm/dl• NaNa :: 126126 meq/lmeq/l• KK :: 2.62.6 meq/lmeq/l

Parameter Result Normal range

NRIMC

Endocrinology

Thyroid function testsThyroid 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

NRIMC

Endocrinology

Gonadal axis evaluationGonadal 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

NRIMC

Endocrinology

Adrenal axis evaluationAdrenal axis evaluationParameter 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

NRIMC

Endocrinology

Growth Hormone axis evaluationGrowth Hormone axis evaluationParameter Result Units Comment

IGF -1

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

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

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Endocrinology

Radiological evaluation

MRI of pituitary is suggestive of partial empty sella

Minimal pituitary tissue is present at floor of sella

NRIMC

EndocrinologyHypopituitarism

Hypothyroidism Hypogonadism Hypocortisolism GH deficiency Low Prolactin

1. Puffiness 2. Lethargy3. Coma4. Weakness5. Infertility6. Cramps7. Can not

tolerate LT4

1. Pubertal delay2. Infertility3. Amenorrhea

* Primary * Secondary4. Loss of libido5. Breast

atrophy6. Osteoporosis

1. Weakness2. Weight loss3. Lethargy4. Hypotension5. Postural

hypotension6. Giddiness7. Vomitings8. Diarrhea9. Pain

abdomen10. Refractory

shock11. Hyponatremia

Children1.Growth failure2.Shortstature

Adults

1.Adult growth hormone deficiency

Lactational failure

No symptoms in male

NRIMC

Endocrinology

Causes of Hypopituitarism

• Heriditory Pituitary Hormone deficiency ( Pit 1, Prop 1, LHX 3, HESX1)

• Acquired Pituitary hormone deficiency– Infections - rare– Infiltrative

• 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

NRIMC

Endocrinology

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