Open AccessRe-emergence of a rare syndrome: A case of mauriac syndrome
Author(s) -
Manish Gutch,
Rajeev Philip,
Sonal Saran,
Rajiv Tyagi,
Keshav Kumar Gupta
Publication year - 2013
Publication title -
indian journal of endocrinology and metabolism
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.456
H-Index - 28
eISSN - 2230-9500
pISSN - 2230-8210
DOI - 10.4103/2230-8210.119611
Subject(s) - cushingoid , medicine , short stature , dexamethasone , endocrinology , pediatrics , diabetes mellitus , insulin , bolus (digestion)
Mauriac syndrome is a rare syndrome associated with type 1 diabetes (T1DM) in children presenting with growth retardation, hepatomegaly, and cushingoid features. Recently, there has been re-emergence of this syndrome, especially with the use of premix insulin. A 15-year old type 1 diabetic boy, who was on premix insulin with erratic blood glucose, was referred to us for evaluation of short stature. He had significant short stature, hepatomegaly, and cushingoid features. His growth hormone (GH) stimulation was normal, and so was the overnight dexamethasone suppression test, based on which the diagnosis of Mauriac syndrome was reported. He was made to switch over to basal bolus regime, and was advised to follow-up for 6 months. He had reduction in hepatomegaly and a height gain of 3 cms.