HYPERTRIGLYCERIDEMIA INDUCED PANCREATITIS WITH TYPE 1 DIABETES

MELLITUS

Back ground

Hypertriglyceridemia is defined as fasting plasma triglyceride (TG) level above an age and
gender –adjusted 95th percentile. Monogenic hypertriglyceridemia includes genetic defects in
triglyceride metabolism, whereas polygenic hypertriglyceridemia is often due to interplay
between genetic, hormonal and environmental factors including uncontrolled diabetes, obesity,
metabolic syndrome and medications in the pediatric age group. Severe hypertriglyceridemia,
defined as triglyceride concentration of greater than 1000mg/dl, can present with pancreatitis in
the first decade of life Hyper triglyceridemic pancreatitis is often correlated with higher severity
and elevated complication rate, thus appropriate treatment and prevention of disease recurrence
is essential. At present no approved treatment guidelines are available, although different
modalities such as insulin, omega 3 fatty acids, heparin, and fibric acids have been given
successfully. Our case highlights the use of insulin as an adjunct to lower the TG, followed by
medium-chain triglyceride (MCT)- based diet, and subsequently, strict dietary fat restrictions as
long-term management in a young infant with familial chylomicronemia syndrome (FCS).

Case report

We report a case of 5-year-oldnon obese female child born out of non consanguineous marriage
with uneventful birth and postnatal history with family history (paternal and maternal
grandparents) of hypertriglyceridemia diagnosed at 60years of age. Child has undergone
polypectomy (recto sigmoid polyps) 7 months back. Prior to polypectomy as per routine
screening necessary blood investigations were sent, of which lipid profile was suggestive of
elevated serum triglycerides (3825 mg/dl), total cholesterol (200.6 mg/dl) decreased HDL
(10mg/dl), high LDL/HDL ratio (6.3) normal complete blood count, renal function tests and
serum electrolytes. Ultrasound of the abdomen and pelvis was normal. Regular blood sugars
monitoring showed high fasting and random blood sugars for which she was started on
subcutaneous insulin, oral fibrates along with dietary advises as required. Ophthalmic
examination showed Lipemia retinitis

Figure 1 Lipemia retinalis

After 7 months child was brought to our hospital with complaints of abdominal pain in right
upper quadrant associated with bilious vomiting. At the time of admission child was euthermic,
normotensive, maintaining saturation at room air with normal growth and development. The
abdominal examination was significant for tenderness in epigastric and right hypochondriac
region, any other abnormality was not detected in cardiovascular, respiratory and neurological
system. Physical examination was unremarkable otherwise and there were no skin lesions or
abnormal body fat distribution. Laboratory parameters showed hypertriglyceridemia,
hypercholesteremia, hyperglycemia, elevated amylase and lipase levels. Contrast enhanced CT
showed acute necrotizing pancreatitis. Sepsis screen was positive. During venesection, milky
serum was noted.

Figure 2 Milky serum

Investigations

TG levels were found to be 3390mg/dl with the total cholesterol level at 919mg/dl. Serum lipase
was elevated at 142.1 IU/L (normal 13-60 IU/L), while amylase level at 426 IU/L (normal < 96
IU/L). C Reactive protein level at 1.75 mg/dl (normal 0.02-0.1mg/dl). Total leukocyte count of
11.55/mm3 (normal 4-10/mm3) with neutrophil predominance. Blood glucose levels were
175mg/dl (normal-70-140mg/dl). Corrected Calcium being 8.4mg/dl and normal serum
electrolytes, renal function tests. Chest x ray and abdominal x ray was normal. Abdominal
computed tomography showed diffuse edematous pancreas with extensive peripancreatic and left
paracolic necrotic collection suggestive of acute necrotizing pancreatitis (Modified CTSI- 6),
moderate ascites and minimal left pleural effusion. 2D echocardiography was normal.

Treatment

She was kept fasted and started on intravenous dextrose normal saline. Intravenous insulin
infusion was then started at 0.05unit/kg/h around 24h after admission. TG levels started trending
down from 447mg/dl to 259mg/dl with insulin and fasting alone. Feeding was resumed after
3days of fasting with low-fat, low calorie, high protein, high fiber diet and omega 3 fatty acid
supplementation.

During the hospital stay abdominal pain, triglyceride levels and blood sugar levels were
improved and was discharged from hospital with triglyceride level of 259mg/dl.
Discussion

As in our patient, affected individuals typically present with lipemic plasma during blood
sampling, lipemic retinitis and abdominal pain suggestive of pancreatitis. Other physical findings
may include eruptive xanthomas, hepatospleenomegaly, but it may not be present in all cases and
could be non-specific. Management of hypertriglyceridemia in children is challenging as there is
lack of established guideline.

Acute management

Fasting

As chylomicrons produced in the small intestine are dependent on dietary fat for formation,
fasting results in drastic decline in TG levels and allows gradual clearance. Adequate hydration
should be maintained with intravenous fluid. No fat diet, such as glucose water, can be
introduced when TG level reaches <1000mg/dl.

For further treatment of HTIP plasmapheresis, subcutaneous heparin were reported but still
considered as experimental treatment options.

Insulin

Insulin is a potent activator of LPL, accelerates TG degradation into fatty acid and glycerol and
facilitates storage in adipocytes. Case report on insulin use in patients with FCS, in particular due
to LPL deficiency, is limited. Our case demonstrated sustained decline in TG level. The initiation
of insulin infusion was based on the balance between the potential risks and benefits.
Concomitant dextrose infusion, along the same intravenous line as insulin infusion, is important
to maintain euglycemia Blood glucose should be monitored closely during insulin infusion. In
our case, we started insulin infusion 0.05units/kg/h in view of her young age. Together with
fasting, a rapid decline in TG level was observed. It is thus an effective, simple and safe adjunct
treatment strategy for pediatric patients with severe hypertriglyceridemia.

Long-term management

Acute pancreatitis, which can be consequent to severe TG of >1000mg/dl, is the most worrisome
complication of hypertriglyceridemia in children. Long-term management should therefore aim
to keep TG concentration less than 500mg/dL, as post-prandial TG concentration can increase
exponentially.
Diet

The best long-term management in hypertriglyceridemia in FCS is using low-fat diet that
restricts fat intake to 10–15% of the total caloric consumption.

Pharmacological treatment

At present there is no FDA approved pharmacological treatment for this condition. Fibrates are
mainly effective in those with residual LPL function. Niacin as TG lowering agent has not been
evaluated specifically.

Conclusion

Severe hypertriglyceridemia is an endocrine emergency associated with acute pancreatitis.

Fasting and insulin seems to be non invasive, safe treatment options in acute presentations. As
discussed strict dietary management remains the long term treatment.

Consent

The authors declare that they have provided informed consent from the described patient for case
report to be published.

Conflict of Interest

The authors declare that there is no conflicts of interests regarding the publication of this paper.

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