MAURIAC SYNDROME

Dear friends , Today let's have a look at Mauriac syndrome, a very rare complication of poorly controlled Type 1DM . There are around 4 to 5 rare cases of Mauriac syndrome in the literature reported as case studies. Very recently we diagnosed a case of Mauriac syndrome in our Sepuri Diabetes Centre, so we are going to present it to either BMJ or NEJM and now the boy is improved a lot by achieving good glycemic control. Every parameter of Mauriac syndrome is recorded in our case . with regards, Dr Sepuri Tirumala Devi

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Dear Madam. I am more in touch with Dear Dr.Mohanji...So I was lacking vary valuable posts of yours. Well, i have read about your case handeling of Mauriac Syndrome...The rarest to diagnosed.... Before I proceed let me clear that I am bit Orthodox academician..Ask more to Mohanji.. In Commonwealth and European countries..... to label "Majuriac Syndrome ".. Following are necessarily to be ticked... * Hepatomegaly..Due to intra hepatic glucogen deposits. * Growth/Puberty delay. * Elevated Transminas &S.Lipids. * Cushingoid features. * Nephropathy & Microvascular complications like Retinopathy.. * Puberty delay... I had a line to approach .. * Endocrinology../Pediatrics / Nutritions / Psychology & Psychiatric.. You both have performed wonderful task.. But where am I confused is... ABSENCE OF RATINOPATHY..!!??? I like Dr.Prasad approach.he seems to be more similar to my nature.. Convey my regards to my dear Mohanji.. I wish all the success in your professional Carrier.. Pranam.
Thanks a lot dear Gurujee for your kind comments. Except Retinopathy, everything else was present in this case. When Ophthalmologists said no Retinopathy, we also got little bit confused. You are the best academician in the platform of Curofy. Bheeshmacharya. Regards & thanks, Dr Sepuri Krishna Mohan Dr Sepuri Tirumala Devi.
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Really a good catch.!.congrats- Madam and Sir. U could have released some more data that could have made the diagnosis still impressive. 1) an u/s report cofirming ' extreme hepatomegaly. 2) Ht/ wt percentiles to substantiate ' grouth retardation' 3) age to substantiate delayed puberty. 4) Duration of inadequately treated hyperglycemia - as the syndrome occurs after prolonged poor control of hyperglycemia. 5 BMI- TO SUPORT OBESITY. 6) FULL PIC TO SUGGEST CUSHINGOID FEATURES. 7) BLOOD SUGAR LEVELS AT THE START OF TREATMENT. 8) FAMILY HISTORY. FOR PUBLICATION PURPOSE- 9 )LIVER BIOPSY. 10) GENE DEFECT ANALYSIS. WITHOUT 9 AND 10 THE DATA FORTIFIED WITH POINTS 1 TO 8 STILL MAKES A GOOD CASE REPORT. IF DONE IT WOULD BE A FEATHER IN CAP OF " SEPURIS ". CONGRACTS AND WISHING WIDER APPRECIATION BY WAY OF PUBLICATION ...I remain ( that should have been done b 4 !, better late than never !) Regards...
Thanks again dear GURUJEE. We will get back to you with all the data. GURUJEE is always GURUJEE.
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Fundus shows diabetic retinopathy with papilledema, signifying raised ICT what about left eye. What is the vision
Vision is perfect & Fundus showed no evidence of Retinopathy, seen by senior most Ophthalmologist sir. Thanks for your valuable feedback. We r/o Retinopathy & vision is perfect sir.
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We both are very happy to diagnose a very rare case in our Sepuri Diabetes center.
Thanks for the informative post....@Dr. Sepuri Tirumala Devi
Most welcome dear Rajan
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I have similar case of 18 yes old male with uncontrolled dm1
Of, that's fine, Try to investigate methodically and establish the diagnosis of Mauriac Syndrome.
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Congratulations for your achievement Convey Madam also
Thanks again dear Raghavendra Rao, So nice of you dear.
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Congratulations ma'am and dear sir,,, very useful
Thanks again dear Raj
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Good explanation. Happy to see such cases.
Thanks again dear Gaurav
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CONGRATULATIONS TO BOTH OF YOU
Thanks again dear Umesh
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