a case of sheehan's syndrome
TRANSCRIPT
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INTERESTING CASE OF ANAEMIA
S.DHANRAJ PG M2DR.SUNDARAMURTHY’S UNIT
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• 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
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• 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
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• PAST HISTORY Not a known DM/SHT/CAD No previous admissions
• MENSTRUAL HISTORY amenorrhea for 18 yrs
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EXAMINATIONDrowsyTemperature (98.4 F )dehydrated,Severe pallor,facial puffinessDry coarse skin,sparse hair in scalpHoarse voiceb/l pedal edema+ no clubbing,no palpable lymphadenopathy
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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
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• She was initially suspected as a case of ? Chronic kidney disease with encephalopathy and fever under evaluation
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• 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
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• USG– N STUDY
• CXR- PA – N STUDY
• ECG -- WNL
• ECHO -- N LV FUNCTION
• FEVER PROFILE AND BLOOD CULTURE-- NEGATIVE
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• CLINICALLY , suspected MYXEDEMA PRECOMA
TFT SENT and
started on ELTROXIN 100mcg WITH steroids
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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
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INVESTIGATIONS• 5/4/2013Urine spot PCR– 23/13p.smear– microcytic hypochromic anaemia Wbc—N Platelets– adequate No immature cells/haemoparasite
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• 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)
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• 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
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• 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)
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• Patient was continued with Levothyroxine and steroids
• Patient improved dramatically
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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
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FINAL DIAGNOSIS
• EMPTY SELLA SYNDROME probably due to POSTPARTUM PITUITARY NECROSIS (SHEEHAN’S SYNDROME)
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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
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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
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- 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
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MECHANISM
• Ischemic pituitary necrosis due to severe postpartum hemorrhage • Vasospasm, thrombosis and
vascular compression of the hypophyseal arteries • Autoimmunity
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PRESENTATION
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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.
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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.
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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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THANK YOU