Leonard Pierre Mauriac

Leonard Pierre Mauriac (1882-1963) was a physician in Bordeaux who is remembered for his eponymous syndrome, the triad of dwarfism, hepatomegaly and Cushingoid like obesity in children with diabetes. At first Mauriac thought this was a side effect of insulin but by 1940 it was agreed that it only occurred in children whose control was very poor.

The concept of hepatomegaly in young diabetic patients seems to have been introduced to audiences in the United States by an article in the Journal of the American Medical Association in 1936. The author, Per Hanssen of the Steno Memorial Hospital, Copenhagen, reported 12 patients, 10 under the age of 15, with hepatomegaly over a 2½ year period. In five the liver extended below the umbilicus. In all cases, while in hospital, it was found that on their usual twice daily unmodified insulin, the children had gross swings of blood sugar and all were changed to protamine insulinate. In all cases the liver shrank although in the grossest cases it took more than three months. [1]Many writers attributed the hepatomegaly to fat but it was later shown to be due to glycogen. Many clinicians were struck by the similarity of the clinical picture to that in glycogen storage disease (Von Gierke’s) although children with Mauriac’s syndrome had a normal glycaemic response to adrenaline. The moon face in many children with the syndrome was attributed to adrenal stimulation from the unstable blood sugars. The syndrome became very much less common after 1940 which was attributed to the use of long acting insulins although a relaxation in standards of control may have contributed.

Pierre Mauriac began as a bacteriologist and his thesis was on a modification of the Wasserman test for syphilis. He served throughout World War 1 and was awarded the croix de guerre and legion d’honneur. In 1930 he left the chair of bacteriology and experimental medicine and became professor of clinical medicine. He studied many aspects of diabetes including insulin resistance and the nervous complications.

He was also a writer, medical historian and mayor of Bordeaux. His brother Francois won the Nobel prize for literature in 1952.

References

  1. ^ Hanssen P. Enlargement of the liver in diabetes mellitus. J Am Med Assoc 1936; 106: 915-6.

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