Potential of essential fatty acid deficiency with extremely low fat diet in lipoprotein lipase deficiency during pregnancy: A case report

BACKGROUND: Pregnancy in patients with lipoprotein lipase deficiency is associated with high risk of maternal pancreatitis and fetal death. A very low fat diet (< 10% of calories) is the primary treatment modality for the prevention of acute pancreatitis, a rare but potentially serious complication of severe hypertriglyceridemia. Since pregnancy can exacerbate hypertriglyceridemia in the genetic absence of lipoprotein lipase, a further reduction of dietary fat intake to < 1–2% of total caloric intake may be required during the pregnancy, along with the administration of a fibrate. It is uncertain if essential fatty acid deficiency will develop in the mother and fetus with this extremely low fat diet, or whether fibrates will cross the placenta and concentrate in the fetus. CASE PRESENTATION: A 23 year-old gravida 1 woman with primary lipoprotein lipase deficiency was seen at 7 weeks of gestation in the Lipid Clinic for management of severe hypertriglyceridemia that had worsened with pregnancy. While on her habitual fat intake of 10% of total calories, her pregnancy resulted in an exacerbation of the hypertriglyceridemia, which prompted further restriction of fat intake to < 2% of total calories, as well as administration of gemfibrozil at a lower than average dose. The level of gemfibrozil, as the active metabolite, in the venous and arterial fetal cord blood was within the expected therapeutic range for adults. The clinical signs and a biomarker of essential fatty acid deficiency, namely the ratio of 20:3 [n-9] to 20:4 [n-6] fatty acids, were closely monitored throughout her pregnancy. Despite her extremely low fat diet, the levels of essential fatty acids measured in the mother and in the fetal blood immediately postpartum were normal. Normal essential fatty acid levels may have been achieved by the topical application of sunflower oil. CONCLUSIONS: An extremely low fat diet in combination with topical sunflower oil and gemfibrozil administration was safely implemented in pregnancy associated with the severe hypertriglyceridemia of lipoprotein lipase deficiency

THE VARIATION OF CARNITINE CONTENT IN HUMAN BLOOD-CELLS DURING DISEASE - A STUDY IN BACTERIAL-INFECTION AND INFLAMMATORY BOWEL-DISEASE

Carnitine in erythrocytes and leucocytes represents a small but essential part of the cellular carnitine pool. It was the objective of this study to document the changes of blood cell camitine concentrations in disease entities with an enhanced cellular metabolism during acute and chronic inflammation. The plasma, erythrocyte, lymphocyte, granulocyte and thrombocyte camitine concentrations were determined in 23 patients (11.0 +/- 8.8 years) with bacterial infections and nine patients (17.5 +/- 2.4 years) with Crohn disease and compared to 20 healthy controls (27.0 +/- 10.6 years). In patients with bacterial infections the granulocyte camitine concentrations (126.4 +/- 73.5 nmoles/10(6) cells) were higher (P <0.001) than in controls (37.9 +/- 22.8 nmoles/10(6) eels). In patients with Crohn disease the lymphocyte carnitine concentrations (169.4 +/- 108.2 nmoles/10(6) cells) were increased (P < 0.001) when compared to controls (48.1 +/- 18.3 nmoles/10(6) cells). The plasma carnitine concentrations were decreased (P < 0.05) in both patient groups, whereas they were increased (P < 0.05) in the patients' erythrocytes. The carnitine concentrations in thrombocytes did not differ significantly within the individual groups

Improved N-retention during L-carnitine-supplemented total parenteral nutrition

The influence of intravenously administered L-carnitine on lipid- and nitrogen-metabolism was studied during total parenteral nutrition of piglets (mean weight 4077 g; n = 9). The infusion protocol was divided into three isocaloric and isonitrogenous 48-hr periods. Amino acids (3 g/kg day) were administered throughout all three periods: 140 cal/kg/day were given as nonprotein calories, consisting only of glucose during period 1; during periods 2 and 3, an amount of glucose calorically equivalent to 4 g fat/kg/day was substituted with a lipid emulsion, and L-carnitine (1.5 mg/kg/day) was added in period 3. Key parameters of fat- and nitrogen-metabolism were determined during the entire regime. Indirect calorimetry was performed and the respiratory quotient calculated during all three periods. The results demonstrate a more effective lipolysis and oxidation of fatty acids during L-carnitine supplementation. These changes produce an increased energy gain from exogenously administered fat and a distinct improvement in nitrogen balance