Background: Acute intermittent porphyria (AIP) is characterized by attacks of abdominal pain and neuropsychiatric symptoms. This disease is more frequently and more severely affecting women. Hem deficiency in the liver of AIP patients stimulates an increase in ALA-synthase which triggers an escalating metabolic chain reaction, leading to an increase in the porphyrin content. Treatment of AIP is carbohydrate-rich diet and by treating the attacks with intravenous infusions of glucose or hem. This presents difficulty in treating patients with diabetes mellitus. SGLT2 inhibitors drugs are a class of medications that inhibit reabsorption of glucose in the kidney and therefore lower blood sugar.
Method: A case report
Result: We present a 60 years old female diagnosed with porphyry seven years ago, which presented with acute abdominal pain and positive porphyrin in urine. She was treated with carbohydrate-rich diet and was symptom free for three years. She complained polydipsia and polyuria, her blood sugar lever were over 11 mmol/L in her profile and HbA1c 6.5%. Diagnosis of diabetes mellitus was made and she started using metformin. For the last year her abdominal pain attacks are more often, Other reasons for chronic abdominal pain were examined. Her ultrasound of abdomen, MR enterography and MR colonography were normal. Antibodies for celiac disease and calprotectin were in referent range. As we dont have hem treatment in our country and its used for only attacks she started using carbohydrate rich diet which caused bad regulation of her diabetes (HbA1c 7.5…8.3%). We decided to start treatment of diabetes mellitus with SGLT2 inhibitors. Blood sugar profiles values range from 5.1 to 6.4, and her HbA1c on the last control was 6.1, as far as her abdominal pain attacks, frequency of attacks reduces almost double and intensity of pain is lower.
Conclusion: Treating porphyria patients with diabetes is a two-edged sword, while carbohydrate help a lot patient with porphyria, they cause bad regulation of diabetes. With using SGLT2 inhibitors and their mechanism it is possible to keep both diseases under control.
18 - 21 May 2019
European Society of Endocrinology