DM type 1 vs 1.5 vs 2 ?

A 35 year old male, diagnosed as diabetes 3 years ago. He has oculocutaneous albinism with history of easy bruising and gum bleeding and he was tested throroughly with normal blood coagulation profile and normal platelet function test, ?hermansky pudlak syndrome. On examination he is very thin, weighs 52 kg and BMI of 21.13. He is alright except for decreased visual acuity 6/36 in both eyes and congential horizontal nystagmus. He has two more siblings with oculocutaneous albinism and history of distant consanguinity is there. He has father who is a known diabetic. He started losing weight and was initially on doctor's advice he started life style modification, with exercise and MNT. His current fasting blood glucose remained around 180 mg/dl the report I am attaching here has HbA1c of 9.5. I sent his C peptide level to rule out any possibility of LADA. His fasting c peptide level has been reported as 0.85 ng/ml. I am attaching relevant reports here. His main concern with which he has presented is now is diabetes and I need to confirm if the screening result from fasting c peptide points towards possibility of LADA. And how to start treatment , with insulin or OHA.


Dear Dr Majaz Ahmed, Thank you for tagging me. Again a wonderful and rare case with a nice presentation. Hermansky puldak syndrome is a rare autosomal recessive disorder. Actually there is no association between this syndrome and increased incidence of Diabetes PerSay. But there are rare ,very few case reports in the literature regarding association of Diabetes associated with HPS. There is a family history of Diabetes in this particular case which points towards Type 2 DM but the age ,loss of weight, high HbA1C ,high FPG, ( you never mentioned about 2 hours PG) point towards LADA but to establish a case of LADA you inevitably have to prove the presence of GAD 65 antibodies as a protocol and you can order serum insulin and C peptide for corroboration of the diagnosis. So seeing the normal C peptide in this case neither we can conclude nor exclude LADA. But based on the clinical picture it is definitely a case of insulin requiring DM until we get the positive GAD 65 antibodies. Hence i would prefer Insulin to OHA s in this case, preferably a biphasic premixed Insulin which would be more appropriate and convenient to this patient. Proper counseling is required regarding Diabetes and it's complications. Usually life expectancy of HPS is short . Keep me posted regarding the GAD 65 antibodies and serum insulin. Continue LSM and MNT. Regards, Dr Sepuri Tirumala Devi

Respected mam, Thank you very much again. Mam, 2 hour plasma glucose was 289 mg/dl, I missed adding this in the case. I will order GAD 65 antibodies and serum insulin tomorrow itself and I will inform you about it as soon as I get the results. I have counselled him and explained about the complications. Continuing MNT and LSM and will be starting biphasic insulin from tomorrow after I have sent the tests of serum insulin and GAD antibody. Sincerely Dr Majaz Ahmad

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Dear Mazad, Based on the GAD 65 Antibodies, C Peptide, Fasting and post lunch Insulin this case falls under the Category of T2DM only. T1DM or 1.5 DM are ruled out in this Case. But the patient requires Insulin Therapy for some time f/b OHAs. Regards and thanks, Dr Sepuri Krishna Mohan.

Brilliant explanation mam...

Thank you Dr Rahul Mohtra

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