Mauriac syndrome, also known as Malnutrition-associated diabetes mellitus, is a rare complication of poorly controlled diabetes mellitus in children. This condition was first described by the French physician François Mauriac in the 1930s.
Mauriac syndrome is characterized by stunted growth, delayed puberty, and a distinctive body habitus, which includes abdominal distension, thin extremities, and a moon-shaped face. These physical changes are a result of malnutrition, which occurs because insulin therapy in poorly controlled diabetes can lead to decreased appetite and increased breakdown of fats and proteins in the body.
Symptoms
The symptoms of Mauriac syndrome, also known as malnutrition-associated diabetes mellitus, may include:
Stunted growth
Children with Mauriac syndrome often have delayed growth and are smaller than their peers.
Delayed puberty
Puberty may be delayed or absent in children with Mauriac syndrome.
Moon-shaped face
Children with Mauriac syndrome may have a round, moon-shaped face due to excess fat deposits.
Thin extremities
The arms and legs may appear thin compared to the torso due to the breakdown of fats and proteins in the body.
Abdominal distension
The abdomen may appear swollen or distended due to the buildup of fluid in the abdominal cavity.
Poor appetite
Children with Mauriac syndrome may have a decreased appetite due to high blood sugar levels.
Fatigue
High blood sugar levels can also lead to fatigue and lethargy
Causes
Mauriac syndrome, also known as malnutrition-associated diabetes mellitus, is caused by prolonged poorly controlled diabetes in children. When blood sugar levels are not well-managed, it can lead to a range of complications, including malnutrition.
The underlying mechanism of Mauriac syndrome involves insulin resistance, which is a common feature of poorly controlled diabetes. Insulin resistance makes it difficult for cells to use glucose for energy, leading to high levels of glucose in the bloodstream. This, in turn, can cause decreased appetite, increased fat breakdown, and muscle wasting, which can contribute to malnutrition.
Additionally, high blood sugar levels can also lead to the accumulation of fluid in the abdomen, a condition known as ascites. Ascites can cause abdominal distension and further contribute to the malnourished appearance seen in children with Mauriac syndrome.
Diagnosis
The diagnosis of Mauriac syndrome, also known as malnutrition-associated diabetes mellitus, typically involves a combination of clinical evaluation, blood tests, and imaging studies.
Clinical evaluation
A doctor may perform a physical exam to assess the child's growth and development, look for signs of malnutrition, such as thin extremities, abdominal distension, and a moon-shaped face, and assess the child's pubertal development.
Blood tests
Blood tests may be done to evaluate blood sugar levels and other markers of glycemic control, such as glycated hemoglobin (HbA1c) and fasting blood glucose levels. Blood tests may also be done to assess for malnutrition, such as albumin and prealbumin levels.
Imaging studies
Imaging studies, such as an abdominal ultrasound or CT scan, may be done to evaluate for the presence of ascites, which can contribute to abdominal distension.
Edema
Mauriac syndrome, also known as malnutrition-associated diabetes mellitus, can lead to the development of edema in some cases. The accumulation of fluid in the abdominal cavity, known as ascites, is a common complication of poorly controlled diabetes and can cause abdominal distension and edema in the legs and feet.
The underlying mechanism of edema in Mauriac syndrome is multifactorial. Prolonged high blood sugar levels can lead to damage to the blood vessels, making it difficult for fluid to be transported efficiently from the tissues to the blood vessels. Additionally, protein loss due to malnutrition can also contribute to edema, as proteins help to maintain the balance of fluid in the tissues.
Treatment
The treatment of Mauriac syndrome, also known as malnutrition-associated diabetes mellitus, typically involves a multifaceted approach that includes optimizing glycemic control, nutritional support, and treatment of associated complications.
Glycemic control
The cornerstone of treatment for Mauriac syndrome is achieving and maintaining good glycemic control. This may involve intensive insulin therapy, including the use of multiple daily injections or insulin pump therapy, to regulate blood sugar levels.
Nutritional support
Children with Mauriac syndrome are often malnourished and require nutritional support to improve their overall health and well-being. This may include a balanced diet rich in protein, carbohydrates, and healthy fats, as well as vitamin and mineral supplementation.
Treatment of complications
Complications associated with Mauriac syndrome, such as edema and delayed growth and development, may require additional treatment. This may include the use of diuretics to reduce fluid buildup, growth hormone therapy to improve growth, and surgery to address complications such as ascites.
