A FAMILIAL HYPERLIPIDEMIA TYPE 1 CASE PRESENTING
WİTH ACUTE PANCREATITIS WITH A GOOD RESPOND TO CARNITINE TREATMENT
Urganci N1 ,
Akyildiz B1 , Aydin A2 , Hatipoglu S1
1Department of Pediatrics ,
Sisli Etfal Training and Research Hospital, Istanbul, Turkey
2Department of Pediatrics ,
Cerrahpasa Faculty of Medicine , Istanbul, Turkey
In recent years,
literature that contaıns the affects of carnitine in hyperlipidemias are
observed. Because of this, the importance of carnitine in the treatment of
hyperlipidemia tip I which is one of the pancreatitis couses has been
reviewed.
A 2 months old male baby
was admitted to our hospital with the symptoms of iritability, vomiting,
fever. He had tachypnea, tachycardia, and on examination he had abdominal
distension , his bowel sounds were diminished, he had hepatomegaly of 4
centimeter palpable liver. He had no splenomegaly. His laboratory findings
were amilase 372 IU/L, lipase 241 UI/L nA: 127 mmol/L. His blood specimens
had lipemic milky plasma and there wasn¡¯t performed any surgical
intervention. After 12 hours of starvation, another blood sample was taken
and left in the refrigatör under +4 0C for one night. In the
upper portion of the plasma a part like a creme was seen. The lipid
electroforesis showed HDL: 16 mg/dL, LDL: 161 mg/dL, VLDL: 1480 mg/dL.
Oral seeding was stopped,
total parateral nutrition and 150 mg/kg/day carnitine was started. On the
fourth day the clinical symptomalogy were better. The laboratory findings
were amilase: 55 mg/dL, trigliserid: 332 mg/dL, cholesterol: 287 mg/dL,
VLDL: 86 mg/dL and child began to feed orally. The patient continued to
breast feeding and 100 mg/kg/day carnitine. The trigliserid levels were
1000 mg/dL and lower than that, in the patient except the infections time.
Our patient is 1,5 year old now and he hasn¡¯t any problem.Finally in our
country where parents may be relatives, in this cases presenting with
abdominal cramps and vomiting, hipertrigliseridemia and LPL defects should
be considered in the diagnosis of panereatitis, and it shouldn¡¯t be
forgetten that the treatment with carnitine will decline the levels of
trigliserid and improve the clinical progress.