markers of insulin resistance in a case of ......markers of insulin resistance in a case of...
Post on 04-Mar-2020
3 Views
Preview:
TRANSCRIPT
Rev. Med. Chir. Soc. Med. Nat., Iaşi – 2013 – vol. 117, no. 2
INTERNAL MEDICINE - PEDIATRICS CASE REPORTS
404
MARKERS OF INSULIN RESISTANCE IN A CASE OF LAUNOIS-
BENSAUDE SYNDROME
Doina-Clementina Cojocaru1,2
, Corina Dima Cozma1,3
, Paraschiva Postolache1,2
University of Medicine and Pharmacy “Grigore T. Popa” – Iasi
Faculty of Medicine
1. Ist Medical Department
Rehabilitation Clinic Hospital Iasi
2. Pulmonary Rehabilitation Clinic
3. Cardiovascular Rehabilitation Clinic
MARKERS OF INSULIN RESISTANCE IN A CASE OF LAUNOIS-BENSAUDE SYN-
DROME (Abstract): Launois-Bensaude syndrome (benign symmetric lipoma-tosis) is a rare
disease characterized by symmetric fat deposits localized in the cervical region, shoulders
and proximal parts of upper and lower limbs. We present the case of a 63-year-old male who
presented the typical location of fatty masses and a history of chronic alcoholism associated
with elements defining the metabolic syndrome. The biological profile indicated high -
atherogenic mixed dyslipidemia, high basal insulinemia (30 μU/ml), and multiple markers of
insulin resistance (Reaven index, lipid accumulation product, homeostatic model, insulin
sensitivity index, and modified glycemic curve following oral glucose load). The particular i-
ty of the presented case is the discordance between the severity of metabolic disturbances
and their clinical expression, raising the question whether this patient's cardiometabolic risk
is increased or rather lowered by the association of benign symmetric lipomatosis. Kewords:
LAUNOIS-BENSAUDE SYNDROME, INSULIN RESISTANCE.
Launois-Bensaude syndrome (benign
symmetric lipomatosis, Madelung’s disease)
is a rare disease of unknown prevalence,
characterized by symmetric fat deposits
prevalently localized around the cervical
region, in the shoulders and proximal part of
the arms and legs. The name of this disease
is chronologically disputed by Sir Benjamin
Brodie (1846), the first who described this
deformity, followed by Otto W. Madelung
(1888), a German surgeon, his name being
particularly related to the disease epony-
mous with prevalently cervical location
("Madelung’s collar or neck”); in 1898,
Raoul Bensaude and Pierre-Emile Launois
published the first series of 75 cases and
provided a full description of the character-
istic features of the disease, adopting the
term symmetric lipomatosis (1,2).
Middle-aged (30-60 years) males are
most commonly affected, developing the
characteristic distribution of body fat after
at least 10 years of abusive ethanol con-
sumption. The etiopathogenesis of benign
symmetric lipomatosis is not fully known,
the hypertrophy of malfunctioning brown
fat and the decreased activity of mitochon-
drial respiratory enzymes that may result in
lipolytic pathway depression being incrim-
inated. In all these processes, alcohol is a
Markers of insulin resistance in a case of Launois-Bensaude syndrome
405
possible co-factor. The only effective
treatment is surgery, through lipectomy
and/or liposuction of fat masses (2, 3).
CASE PRESENTATION
A 63-year-ols male from a rural area
presented to the Pulmonary Rehabilitation
Clinic of the Iasi Rehabilitation Clinic
Hospital complaining of dyspnea, mMRC
(modified Medical Research Council)
breathlessness scale grade 2, intermittent
productive cough and mild fatigability.
The patient presented to our clinic for
entering a respiratory rehabilitation pro-
gram having a previous diagnosis of mod-
erate chronic obstructive pulmonary di-
sease (COPD), the symptoms of this chron-
ic lung disease being well controlled with
inhaled tiotropium18 µg/day. He also pre-
sented grade 2 essential hypertension,
blood pressure being optimally controlled
with amlodipine 10 mg daily.
What drew our attention during physical
examination were the pseudotumoral sub-
cutaneous masses: relatively symmetrical,
located mainly in the proximal parts of the
upper and lower limbs, with a pseudohy-
pertrophic appearance, and under the chin,
behind the ear, and in the nuchal region,
resembling a Madelung’s collar (fig. 1).
Fig. 1. Relatively symmetric fat deposits in the upper half of the trunk and upper limbs
On palpation, multiple mobile and pain-
less small abdominal lipomas, like the
masses in the upper body, were detected
(fig. 2). Family history revealed that two
patient’s brothers present a similar appear-
ance; from personal history we found out
that the fat masses with a typical location
for Launois-Bensaude syndrome developed
progressively over the last 20 years. Surgi-
cal removal of one mass located behind the
right ear that became painful confirmed its
benign nature, lipoma, but resulted in local
recurrence.
The patient was a smoker (35 pack-
years) and had a history of chronic ethanol
consumption (70 international units/week,
abstinent for about 6 months); clinically,
stigmata of chronic alcoholism persisted,
represented by retraction of palmar apo-
neurosis (Dupuytren’s contracture), bilat-
Doina-Clementina Cojocaru et al.
406
eral parotid gland hypertrophy, telangiec-
tasias on the nose and cheekbone, but
laboratory tests confirmed abstinence,
serum transaminases, gamma-glutamyltrans-
peptidase and mean corpuscular volume
being normal.
Fig. 2. Abdominal lipomas (left) and under the chin (right)
Patient’s anthropometric data showed a
body mass index of 30.14 kg/m2, corre-
sponding to grade I obesity, and a patholog-
ical waist circumference of 115 cm, meas-
ured in standing position halfway between
the costal margin and iliac crest line.
The metabolic profile revealed mixed
dyslipidemia at high atherogenic risk (total
cholesterol = 6 mmol/l, HDL-cholesterol = 0.9
mmol/l, triglycerides = 1.64 mmol/l, LDL-
cholesterol = 4.32 mmol/l), normal glucose
levels (4.93 mmol/l, and high basal insuline-
mia (30 μU/ml), indicator of insulin resistance.
Given the presence of basal hyper-
insulinemia, we calculated the markers of
insulin resistance derived from previous
biological and anthropometric measure-
ments (tab. I).
Also a 3-hour 75-gram oral glucose tol-
erance test was performed (fig. 3).
TABLE I
Markers of insulin resistance
MARKERS OF INSULIN
RESISTANCE
RESULTS COMMENTS
TG/HDL ratio (Reaven index) 4.17 Reaven index ≥ 3,5 indicates insulin re-
sistance (4)
LAP (lipid accumulation product) 82 cm. mmol/l
Lipid accumulation product correlates well
with insulin resistance and metabolic syn-
drome (5)
HOMA-IR (homeostatic model
assessment of insulin resistance) 5.33
Values ≥ 2.6 indicate insulin resistance sta-
tus; ≥ 4 it is considered prediabetic status (6)
QUICKI( quantitative insulin
sensitivity check index) 0.29
Low values (< 0.45) suggest insulin re-
sistance ; < 0.3 in diabetics (7)
Markers of insulin resistance in a case of Launois-Bensaude syndrome
407
Fig. 3. Evolution of glycemia over the 3-hour 75-g oral glucose load
DISCUSSION
The patient presented a rare disease,
benign symmetric lipomatosis, associated
with metabolic syndrome, defined in this
case by three factors: hypertension, ab-
dominal obesity and low HDL-cholesterol,
serum triglycerides being at the limit of
diagnostic significance and basal glycemia
levels within normal range (8). Simultane-
ous determination of basal insulinemia and
calculation of some markers of insulin
resistance (tab. I) revealed frankly patho-
logical values, allowing the assertion of a
hyperinsulinemic status associated with
insulin resistance.
Remarkable is the discordance between
the severity of metabolic disturbances and
their clinical expression: the associated
pathological conditions (hypertension,
COPD) were easily controlled therapeuti-
cally and glucose metabolism disturbances
were not very obvious. Thus, basal glyce-
mia and the values of oral glucose toler-
ance test (fig. 3) were normal at fasting or
at 2 hours, but prolonged monitoring for
three hours revealed a late hypoglycemic
dip, result of an abnormal overproduction
of insulin in response to glucose load.
The particularity of this case is related
to whether the patient's cardiometabolic
risk, consequence of the accumulation of
risk factors - male, smoking status, hyper-
tension, chronic alcohol consumption, obe-
sity - is increased or rather lowered by the
association of Launois-Bensaude syn-
drome, having in view the mild clinical
course despite the severity of underlying
metabolic changes?
Review of literature data on Launois-
Bensaude syndrome reveals a quite wide
phenotypic and clinical variability in this
category of patients, ranging from a type of
"metabolically innocent" obesity with in-
creased insulin sensitivity by decreased
visceral adiposity ascertained by computer
tomography (9), to a metabolic picture
dominated by insulin resistance, particular-
ly in those patients associating cervical
lipomatose deposits and an obstructive
sleep apnea syndrome, in its turn generat-
ing insulin resistance (10).
The presented case seems to be halfway
between these two extremes, but the lack of
large studies and validation of any of the
models does not allow drawing an indis-
putable conclusion.
Doina-Clementina Cojocaru et al.
408
REFERENCES
1. Verna G, Kefalas N, Boriani F, Carlucci S, Choc I, Bocchiotti MA.Launois-Bensaude syndrome: an
unusual localization of obesity disease.Obes Surg 2008; 1: 1313-1317.
2. Sokolov M, Mendes D, Ophir D.Madelung’s disease. IMAJ 2010; 12: 253-254.
3. Trandafir D, Gogălniceanu D, Trandafir V, Popescu E, Costan VV, Vicol C.Sindromul Launois-
Bensaude și rolul chirurgiei cervico-faciale.Jurnalul de Chirurgie 2009; 5(4): 359-364.
4. Gianninni C, Santoro N, Caprio S et al.The triglyceride-to-HDL cholesterol ratio. Association with
insulin resistance in obese youths of different ethnic back-grounds.Diabetes Care 2011; 34(8): 1869-
1874.
5. Xia C, Li R, Zhang S, Gong L, Ren W, Wang Z, Li Q. Lipid accumulation product is a powerful
index for recognizing insulin resistance in non-diabetic individuals.Eur J Clin Nutr 2012; 66(9):
1035-1038.
6. Ascaso JF, Pardo S, Real JT, Lorente R , Priego A, Carmena R. Diagnosing insulinresistance by
simple quantitative methods in subjects with normal glucose metabolism. Diabetes Care 2003;
26(12): 3320-3325.
7. Katz A, Nambi SS, Mather K et al.Quantitative insulin sensitivity check index: a simple, accurate
method for assessing insulin sensitivity in humans.J Clin Endocrinol Metab 2000; 85(7): 2402-2410.
8. Alberti KG, Eckel RH, Grundy SM et al.Harmonizing the metabolic syndrome. A joint interim state-
ment of the International Diabetes Federation Task Force on Epidemiology and Prevention; National
Heart, Lung, and Blood Institute; American Heart Association. Circulation 2009; 120: 1640-1645.
9. Haap M, Siewecke C, Thamer C, Machann J, Schick F et al.Multiple symmetric lipomatosis: a para-
digm of metabolically innocent obesity?Diabetes Care 2004: 27: 794-795.
10. Harsch IA, Bergmann T, Koebnick C et al.Adiponectin, resistin and subclinical inflammation - the
metabolic burden in Launois-Bensaude Syndrome, a rare form of obesity.J Physiol Pharmacol 2007;
58(suppl.1): 65-76.
PELTOPHORUM AFRICANUM: ANTIMICROBIAL POTENTIAL AND HEPATOTOXICITY
The antimicrobial activity and hepatotoxicity of Peltophorum africanum were evaluated in a
study by Okeleye et al. Agar well and macrodilution methods were used for antimicrobial
activity testing, as well as CellTiter-Blue cell viability assay to assess the toxicity on human
liver cells. The results showed both bactericidal and bacteriostatic or fungistatic activity of
the extract. The researchers observed good activity against Plesiomonas shigelloides. De-
spite its antimicrobial activity, the extract was toxic to human liver cell lines. Lethal dose at
50 revealed 82.64 ± 1.40 degree of toxicity at 24 hrs, while 95 percentile of cell death dose
activity varied between log 3.12 and 4.59. (Okeleye BI, Mkwetshana NT, Ndip RN. Evalua-
tion of the Antibacterial and Antifungal Potential of Peltophorum africanum: Toxicological
Effect on Human Chang Liver Cell Line. ScientificWorldJournal 2013; 2013: 878735. doi:
10.1155/2013/878735).
Teodora Vremera
NOUTĂȚI NEWS
top related