CARPENTER SYNDROME - A RARE CASE REPORT

G.Ravi Kiran, Dr.V. Chandrashekar, Dr. Bikshapathi Rao

Department of General Medicine, MGM Hospital
Kakatiya Medical College, Warangal, Telangana

Case Report
A 28 Year old female patient presented with C/C of
Irregular Menstrual Cycles from 2 years,
easy fatigability, Dizziness from 2 months,
she is a known diabetic since 8 years (on human mixtard 30/70 16U/14U),
known epileptic (on carbamazepine since 3 years),
O/E patient is pale, afebrile, PR-114/m. Regular , BP- 90/50 mm hg (supine position), BP - 50/34 mm hg
(Standing Position) & there is Hyperpigmentation of Hands, feet, Axillae, Face
CNS & other Systemic Examination was Normal

INVESTIGATIONS
GRBS (at presentation) was 211mg/dl &
Haemogram: Hb: 7.9 gm % , Microcytic Hypochromic Anaemia, TLC: 5400/mm3, Platelets: 2.1lakh/mm3,
ESR: 15mm/1st Hour,
1st day FBS: 115 mg/dl, PLBS: 192 mg/dl, HbA1c: 5.4% ,
CXR, USG abdomen: Normal Study ,
RFT & LFT: Within Normal Range,
USG neck shows diffuse thyromegaly, with Free T3:2.0 pg/ml, Free T4:0.444 ng/dl, TSH:11.4IU/ml,
FNAC of thyroid: Lymphocytic Thyroiditis & Anti TPO Ab (Immuno ligand Assay) : 260 IU/l
Anti GAD Ab (Chemi luminescent Assay): 189 IU/ml
Serum Electrolytes (Before Treatment) Consistently Na+ (mean 128.1meq/l) & K+ (Mean 5.95meq/l) with
Cl- (Mean 93.6 meq/l) &
early morning Serum Cortisol: 3.9 g/dl
Serum ACTH level: 277pg/ml
High Dose (250 ug) Cosyntopin test:
Serum Cortisol (Baseline) 3.9 g/dl ,
(After 30 min): 6.8 g/dl
(after 60 min): 7.6 g/dl),
Upper GI endoscopy: Normal Study,
Stool for Occult Blood: -ve &
Monteux test: -ve (<5mm induration)
CECT Brain Shows Calcified Granulomas in
frontal Lobe

Discussion

This triple Endocrine Insufficiency is part of PAS & is Historically labelled as Carpenter Syndrome (part of PAS
II). Polyglandular autoimmune syndromes (PAS) are rare endocrinopathies characterized by coexistence of at
least 2 glandular autoimmune diseases. Types I,II,III,IV are distinguished, Thus, In Cases with 2 or more
endocrinopathies possibility of PAS should be Considered, evaluated & treated Accordingly