A CASE OF POLYGLANDULAR SYNDROME

Author: Sibi Thirunavukkarasu

Question
A 14 yrs and 4 months old boy , a known case of type 1 diabetes mellitus got admitted in our hospital for hypoglycemia. His height was 130 cms , weight - 37 cms, BMI-21.89 , us, ls ratio was normal, father`s height - 166cms and mother`s height - 148 cms. No obvious malformations, fundus examination was normal. Dental age corresponds to chronological age. IQ is normal. Sexual maturity corresponded to TANNER 2. Bone age delayed, corresponds to less than 11 years. He is a second born sibling of the four. His elder sister is suffering from type 1 diabetes mellitus and his younger brother is suffering from type 1 diabetes, hypothyroidism. His mother is also a known case of primary hypothyroidism.

INVESTIGATIONS:
Fasting serum c peptide levels - Less than 0.1 ng, dl.
T3 – 21.65 ng , dl ` T4 – 1.2 microgram , dl ` TSH – 163.00 mIU , ml
FSH – 4.45 Miu, ml
LH – 0.10 mIU , ml
PRL – 23.42 ng , ml
TESTOSTERONE – 0.01 ng , ml
8.00AM CORTISOL – 13.44 microgram , dl
PTH – 4.6 pg , ml

KINDLY OPINE REGARDING THE DIAGNOSIS AND FURTHER MANAGEMENT OF THIS PATIENT

Answer Discussion :

Sabri Sakrana

congenital polyglandular deficiency

15 years ago

Ashraf Soliman

A case of polyendocrine failure syndrome that need to be investigated for adrenal function, calcium homeostasis and antibodies for different endocrine glands {thyroid, adrenal} and GH-IGF-I axis.

15 years ago

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A CASE OF POLYGLANDULAR SYNDROME

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