a case of sheehan's syndrome

INTERESTING CASE OF ANAEMIA

S.DHANRAJ PG M2DR.SUNDARAMURTHY’S UNIT

• 40 YRS OLD MRS.SARASU CAME WITH C/O on 4/4/13

Decreased urine output – 15 days -- gradual -- 300ml/day

Abdominal pain -- 6 months -- pricking,more towards epigastric

region,not radiating or referred,not assoc. with any other symptoms

Fever -- on and off,10 days -- low grade intermittent, not assoc. with

chills,rigor,rash,bleeding tendency

• Leg swelling -- 15 days -- bilateral,upto kneeNo h/o abdominal distentionNo h/o constipationNo h/o chest pain/ breathlesnessNo h/o jaundiceh/o hair fall +h/o easy fatigueability+h/o loss of apetiteNo h/o contactNo h/o traumaNo h/o loc, no h/o drug intake

• PAST HISTORY Not a known DM/SHT/CAD No previous admissions

• MENSTRUAL HISTORY amenorrhea for 18 yrs

EXAMINATIONDrowsyTemperature (98.4 F )dehydrated,Severe pallor,facial puffinessDry coarse skin,sparse hair in scalpHoarse voiceb/l pedal edema+ no clubbing,no palpable lymphadenopathy

CVS – s1s2+RS -- B/L normal breath soundsP/A– epigastric tenderness+ -- no organomegalyCNS – drowsy ,responding to oral commands -- Examination: able to move all four limbs -- delayed DTRVITALS : BP-90/60mmHg; PR- 60/min

• She was initially suspected as a case of ? Chronic kidney disease with encephalopathy and fever under evaluation

• INVESTIGATIONS• CBC – HB 7.6 SR. ELECTROLYTES TC 4500 Na-129 DC N70L27E3 K-3.7 ESR 15/35 CL-98 PCV 24 HCO3-23 MCV 83 URINE MCH 26 ALB-NIL MCHC 31 SUG-NIL RBC 2.8LAC DEP-1-3PUS CELLS PLATLETS 1L• RFT- SUGAR 203 UREA 12 CREATININE 0.8

• USG– N STUDY

• CXR- PA – N STUDY

• ECG -- WNL

• ECHO -- N LV FUNCTION

• FEVER PROFILE AND BLOOD CULTURE-- NEGATIVE

• CLINICALLY , suspected MYXEDEMA PRECOMA

TFT SENT and

started on ELTROXIN 100mcg WITH steroids

TFT results– FT3 - 0.37pg/ml(2.3 to 4.2pg) FT4 – 0.41ng/dl(0.6 to 2ng) TSH – 2.572miu/ml(1 to 5miu/ml)

ELTROXIN dose stepped up

INVESTIGATIONS• 5/4/2013Urine spot PCR– 23/13p.smear– microcytic hypochromic anaemia Wbc—N Platelets– adequate No immature cells/haemoparasite

• Reticulocyte count – 1.5% (0.5-1.5%)• • Se LDH 190 U/L (135-220)

• Coomb’s test: negative

• Serum iron: 48 (50 to 150mcg/dl)

• TIBC: 380 (300-360 mcg/dl)

• Serum ferritin : 28 (30 to100mcg/dl)

• 7/4/13 patient started showing improvementFURTHER INVESTIGATIONS With TFT results (low t3, t4, normal TSH) a pituitary cause of hypothyroid was suspected

andPROCEEDED FOR workup for endocrine

insufficiency

• PROLACTIN – 0.6ng/ml (4.7 to 23.3 ng)

• LH – 0.99miu/ml ( Follicular phase:2.4-12.6 Ovulation phase:14-95.6 Luteal phase: 1-11.4 mIU/ml)

• FSH – 3.14 miu/ml ( Follicular phase: 3.5-12.5 Ovulation phase: 4.7-21.5 Luteal phase: 1.7-7.7 mIU/ml)

• CORTISOL – 0.42mcg/dl ( 6.2 – 19.4 mcg/dl)

• ACTH – 3.07pg/ml (7.2 – 63.3 pg/ml)

• Patient was continued with Levothyroxine and steroids

• Patient improved dramatically

ON PROBING

She gave • h/o post partum haemorrhage at 3rd delivery• Lactation failure after 3rd delivery• Amenorrhea for 18 years

• PROCEEDED WITH MRI BRAIN

FINAL DIAGNOSIS

• EMPTY SELLA SYNDROME probably due to POSTPARTUM PITUITARY NECROSIS (SHEEHAN’S SYNDROME)

INVESTIGATIONS NOT DONE

• Stimulation and provocative tests

• Posterior pituitary hormones assay

• Autoimmune work up----primary hypophysitis (lymphocytic, granulomatous)• Secondary hypophysitis—(infections,

sarcoidosis, takayasu)• Haematological malignancy

SHEEHAN’S SYNDROME

- Postpartum hypopituitarism caused by necrosis of the pituitary gland- It is usually the result of severe

hypotension or shock caused by massive hemorrhage during or after  delivery- Usually presents as anterior pituitary

hormone deficiency- Evolves slowly and is diagnosed late

 - most common cause of

hypopituitarism in developing countries

-  prevalence to be about 3% for women above 20 years of age

-  average time between the previous obstetric event and diagnosis of SS was 6 to 13 years

MECHANISM

•  Ischemic pituitary necrosis due to severe postpartum hemorrhage • Vasospasm, thrombosis and

vascular compression of the hypophyseal arteries • Autoimmunity

PRESENTATION

SYMPTOMS• CORTICOTROPIN DEFICIENCY can cause

weakness, fatigue, hypoglycemia, or dizziness. • GONADOTROPIN DEFICIENCY will often cause

amenorrhea, oligomenorrhea, hot flashes, or decreased libido.

• GROWTH HORMONE DEFICIENCY causes many vague symptoms including fatigue, decreased quality of life, and decreased muscle mass.

• Difficulty breast-feeding or an inability to breast-feed• HYPOTHYROIDISM• Some women live for years with pituitary insufficiency,

can present as ADRENAL CRISIS triggered by extreme physical stressors, such as severe infection or surgery.

TREATMENT

Hormone replacement• Hydrocortisone or prednisolone is replaced first

because thyroxine therapy can exacerbate glucocorticoid deficiency and theoretically induce an adrenal crisis.

• thyroxine replacement • gonadotropin replacement • Replacement of growth hormone is necessary in

children with hypopituitarism but is controversial in adults.

REPLACEMENT• ACTH -- Hydrocortisone Cortisone acetate Prednisone• TSH – L-Thyroxine• FSH/LH -- Females Conjugated estrogen (0.65–1.25 mg qd for 25 days) Progesterone (5–10 mg qd) on days 16–25 For fertility: Menopausal gonadotropins, human CG

Males Testosterone enanthate

• GH -- Adults: Somatotropin (0.1–1.25 mg SC qd) Children: Somatotropin [0.02–0.05 (mg/kg per day)]

• VASOPRESSIN

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