Vnitr Lek 2006, 52(3):275-279

Glibenclamide site of in insuline: a new chance for MODY 3 type diabetes patients: case report

L. Brunerová1,*, L. Trešlová1, Š. Průhová2, J. Vosáhlo2, J. Brož1, J. Lebl2, M. Anděl1
1 Diabetologické centrum II. interní kliniky 3. lékařské fakulty UK a FN Královské Vinohrady, Praha, přednosta prof. MUDr. Michal Anděl, CSc.
2 Diabetologické centrum Kliniky dětí a dorostu 3. lékařské fakulty UK a FN Královské Vinohrady, Praha, přednosta prof. MUDr. Jan Lebl, CSc.

MODY 3 belongs to monogenic forms of diabetes mellitus and is caused by monoallelic mutation in gene for transcription factor HNF-1α, essential for regulation of β-cell function. Clinical presentation of MODY 3 is similar to that of type 1 diabetes. Although MODY 3 patients are not threatened by ketoacidosis, tight metabolic control is important for prevention of chronic diabetic complications. In the sibbling pair diabetes was manifested by osmotic symptoms resulting from hyperglycaemia at the age of 18 years (brother) resp. 15 years (sister) and both of them started being treated with intensified insulin treatment. Metabolic control of the brother was very tight with HbA1c 3.3 % but frequent hypoglycaemias occured. On the contrary metabolic control of the sister was very poor due to her non-compliance (HbA1c 10.9 %, IFCC). Molecular-genetic testing proved HNF-1α gene mutation (Arg200Gly). In accordance with the references treatment with sulphonylurea derivate glibenclamide was initiated [at the doses 1.25 (brother) resp. 7.5 (sister) mg/day] and insulin treatment was discontinued. The treatment change led to better quality of life and metabolic control in both the patients and suprisingly to the lower frequency of the hypoglycaemias in the brother (HbA1c decreased from 3.3 % to 2.8 % in three months in the brother resp. from 10.9 % to 10.0 % in two months in the sister). Molecular-genetic testing enables the change of treatment leading to better quality of life and metabolic control, although its longterm safety and efficacy will have to be confirmed.

Keywords: MODY 3 type diabetes; therapy; metabolic control; glibenclamide; quality of life

Received: October 11, 2005; Accepted: January 10, 2006; Published: March 1, 2006  Show citation

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Brunerová L, Trešlová L, Průhová Š, Vosáhlo J, Brož J, Lebl J, Anděl M. Glibenclamide site of in insuline: a new chance for MODY 3 type diabetes patients: case report. Vnitr Lek. 2006;52(3):275-279.
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