mauriac syndrome is a rare syndrome associated with type 1 diabetes ( t1 dm ) in children .
the incidence of this syndrome had decreased significantly with introduction of long - acting insulin and better control of blood sugar .
recently , there has been re - emergence of this syndrome , especially with the use of premix insulin .
a 15-year - old type 1 diabetic boy was referred to us for evaluation of short stature .
he was diagnosed to have t1 dm , following an episode of diabetic ketoacidosis ( dka ) 8 years back , and was started on premix ( 30/70 ) insulin .
examination showed that he was significantly short for his age , height 131 cms ( less than 3 percentile ) , weight 28 kg ( less than 3 percentile ) and body mass index of 16.31 .
he had abdominal fat deposition [ figures 1 and 2 ] and liver was palpable clinically 5 cms below costal margin [ figure 3 ] with no splenomegaly or free - fluid .
short stature , abdominal obesity short stature , abdominal obesity investigations showed haemoglobin of 13.5 gm / dl .
ultrasound abdomen showed liver enlargement 15.5 cms with increased echotexture , with normal spleen and portal vein and no free - fluid . on evaluation for diabetic status
, he had an hba1c of 10.3% , urine microalbumin 70 g / gm of creatinine , and fundus showing background diabetic retinopathy .
he had a bone age of 10.2 years , ( tanner whitehouse 2 ) thyroid - stimulating hormone ( tsh ) of 2.4 miu / ml and t4 of 8.8 microgm / dl .
his growth hormone ( gh ) stimulation test and overnight dexa methasone suppression test were normal ( after blood glucose control and testosterone priming ) . based on the clinical history and investigations , the final diagnosis of mauriac syndrome was made and the patient was advised tight control of sugars .
he was switched over to basal bolus regime , with glargine in the night and three doses of short - acting insulin before meals .
mauriac in 1930 , described growth failure and maturational delay with hepatomegaly and abdominal distension in children with t1 dm , who were treated with short - acting insulin .
hepatomegaly was commonly observed in children in the earlier periods of diabetic treatment , when only short - acting insulin was available and aglycosuria was the objective of treatment .
it was noticed that the hepatomegaly regressed when the children were given the newly introduced protamine zinc insulin , providing better sugar control . in the late 1930s
joslin clinic reported a case series of 60 youngsters with hepatomegaly , growth failure , delayed sexual maturation , and severe uncontrollable diabetes .
equal incidence is reported in males and females , with most of the cases occurring during adolescence . with better control of sugar ,
the incidence of this syndrome has reduced rapidly and in the current era this is a very rare syndrome .
two different forms of mauriac syndrome have been described , based on the presence or absence of obesity . in first form , as classically described , treatment with regular insulin alone there is associated cushingoid obesity and documented - wide fluctuation between hyperglycemia and hypoglycemia , suggestive of a pattern of over - and under - insulinization , with secondary hyperadrenalism .
periods of over - insulinization appear to be essential for the development of obesity , and for the induction of hyperadrenalism .
recently , mauriac syndrome has been reported in patients who are not obese and are without a history of alternating hypoglycemia and ketoacidosis .
this occurs in patients who have been given regular , under the dose insulin . 
 
inadequate glucose to the tissues , decreased insulin - like growth factor-1 and gh levels , hypercortisolism , resistant or defective hormone receptor action contribute to stunted growth and delay in puberty .
the cause of hepatomegaly is thought to be due to the deposition of glycogen in the liver , and similar subcutaneous deposition gives rise to the round moon like facies . 
 
growth failure , delayed puberty and hepatomegaly in mauriac 's syndrome improves with glycemic control .
aggressive glycemic control has been associated with worsening of retinopathy , which should be monitored . 

mauriac syndrome is a rare manifestation of poorly treated t1 dm . with aggressive glycemic control ,
 mauriac syndrome is a rare syndrome associated with type 1 diabetes ( t1 dm ) 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 . 