Background Insulin deficiency secondary to pancreatic beta cell dysfunction ' worsening with age has been reported in Turner syndrome (TS) ' is usually more commonly associated to treatment with GH or sex hormones. Autoimmune endocrinopathy in TS seems to be limited mostly to autoimmune thyroiditis, celiac disease, inflammatory bowel disease ' juvenile rheumatoid arthritis. Type1 diabetes has been rarely reported.

Case Report(s) 13 10/12 yr old girl with severe growth retardation was admitted to hospital with complaint of polyuria ' polydipsia for the last 2 weeks. Three days before hospitalization abdominal pain ' vomiting were started. Her investigations revealed blood sugar 588 mg/dl, PH 6.8, HCO3 3 meq/L, blood ketones 3 positives, HbA1c 12.8, C- peptide very low, anti- GAD ' anti – ICA antibodies were positives. Diagnosis of diabetic ketoacidosis was made ' started management protocol. After stabilization, further evaluation of the patient showed height 127 cm (- 5 SD), weight 32 kg (- 2.8), webbed neck, cubitus valgus, shielded chest, tanner stage 1 ' birth marks of hypopigmented streaks ' patches all over the body appeared to be hypomelanosis of Ito which has an association with chromosomal abnormalities. LH ' FSH elevated, estradiol < 5 pg/ml, bon age 11 4/12 yrs, normal TFT ' celiac screen, negative anti –TPO ' anti – Tg antibodies, normal renal ultrasound ' echo study. Karyotype revealed 45 XO. Diagnosis of Turner syndrome was made. Six months later, the patient started on GH therapy with gradual increment of the dose ' the insulin requirement increased accordingly. After one year, the patient gained 7.4 cm in height. At age of 15 4/12 yrs, conjugated estrogen started at a dose of 0.3 mg every other day.

Conclusion(s) Association of TS to autoimmunity is however widely known. Therefore, it is proposed that all patients with TS should be investigated for diabetes ' the clinician must keep in mind the risks of metabolic complications when GH therapy applied in the presence of diabetes, but also aware that the treatment will improve their final height.