the Prevalence of Familial Partial Lipodystrophy in patients with extreme insulin-resistant type 2 Diabetes

Familial partial lipodystrophy (FPLD) is an inherited disease that is
characterized by an abnormal distribution of subcutaneous fat. As a result
these patients have metabolic abnormalities including; insulin resistance with
type 2 diabetes, acanthosis nigricans, dyslipidemia predominantly consisting of
hypertriglyceridemia (associated with the onset of pancreatitis), liver
steatosis and hypertension. Women are usually hirsute, often associated with
the presence of polycystic ovarian syndrome (PCOS). The majority of these
symptoms may to some extent also be present in patients with the metabolic
syndrome. Since the prevalence of metabolic syndrome by far outweighs that of
lipodystrophy, the diagnosis of this rare disorder may often be discarded as
being metabolic syndrome.

Since the first description of FPLD it is becoming increasingly evident that
lipodystrophy is often misdiagnosed as diabetes with extreme insulin
resistance. With this project, we want to find out the prevalence of
lipodystrophy in patients with severe insulin resistance.

Those patients with an extreme insuline resistance will be selected from three
different hospitals for further analysis. During a one time visit, the
anthropometric measurements with a skinfold caliper will be performed in a
standardized fashion. The normal distribution of fat in general population is
already available in the literature. These skinfold measurements will be
compared to the general population.
We will also measure Intima Media Thickness (IMT) to assess the cardiovascular
burden in these patients.

There are no risks involved in this project. If the patient accepts to enter
the study, he/she will be invited for a one time visit for the following
assessments:

Taking history
Physical examination and anthropometric measurements
Vein puncture for drawing blood
IMT measurements