Dr Faraz Farishta: a Rare Case of Hyperinsulinemic Hypoglycemia (Hirata’s Disease (IAH))

CASE OVERVIEW

Insulin Autoimmune Syndrome (IAS)/ Hirata’s Disease (IAH) is a rare cause of hypoglycemia. Such case of 70-year old female was presented by Prof. Dr. Faraz Farishta, Director FS Endocrine & Diabetes Center. She had a history of diabetes, hypertension and was on Metformin 500 mg OD, Losartan 50 mg OD. She had a BMI of 34 kg/m2, PR 76/min, BP 140/70mmHg. She was acanthosis nigricans positive. Patient complaint of episodes of aggression, rage and irrelevant talk from last 2 months that starts after 6-8 hours of meal. Her HGT during episodes was approximately 27.36 mg/dl and symptoms resolves after glucose ingestion.

DIAGNOSIS

Her diagnostic reports reveled normal CBC, LFT, S. Creatinine – 1.0 mg/dl, Sodium -133 mEq/L, Potassium -5 mEq/L, Chlorine – 93 mEq/L, HbA1c – 6.4 %, T3 -62.94 ng/dL, T4 – 11.64mcg/dL, TSH-1.523micIU/mL, S. Cortisol-14mcg/dl. Mixed meal test after 6 hours revealed RBS of 46 mg/dl, S. Insulin- >1000 mcU/mL, S. C peptide- 40.0ng/mL. She was Anti Insulin Antibody negative. She was diagnosed with Insulin Autoimmune Syndrome/ Hirata’s Disease (IAH).

TREATMENT

Tab. Acarbose 25mg TID was started to delay the glucose absorption from GIT. Tab. Prednisolone 60mg OD (steroid) was introduced. Her diet was modified to high protein diet with small frequent meals and she was advised to avoid simple carbohydrates.

RESULTS

She adhered to treatment with steroid and Alpha Glucosidase Inhibitor. Dietary modification was the mainstay of therapy. Her episodes have come down gradually and by end of 2 weeks she was totally asymptomatic.