





Management



Malnutrition














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Malnutrition leading to growth failure occurs in as many as 60% of infants with chronic cholestasis. The causes are multifactorial. To prevent malnutrition, caloric intake should approximate 125% of that recommended for age. Occasionally, this may require nocturnal NG feedings to supplement the daytime intake. Also helpful are caloric supplements with carbohydrate or medium chain triglycerides (MCTs) - see table below. Parenteral nutrition via a CVC may be necessary in selected cases.

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Increasing the calorie content of formulas
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Add less water
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3 oz H2O + 3 oz concentrate = 20 cal/oz
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2 oz H2O + 3 oz concentrate = 24 cal/oz
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1 oz H2O + 3 oz concentrate = 30 cal/oz
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Mix with term formula
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2 oz preterm formula (24 cal/oz) + 1 oz standard concentrate = 30 cal/oz
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Add modulars
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MCT or corn oil = 8 cal/cc
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Polycose = 2 cal/cc
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Duocal (MCT + Polycose) = 16 cal/tsp
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HMF 2 packets/50cc = 24 cal/oz
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2-3 gm/kg/day of protein are recommended and may be supplied by protein hydrolysate formulas, although this is not always necessary. MCTs are readily absorbed by the intestinal mucosa, making them an ideal source of fat calories in patients with cholestasis. Since they do not contain essential fatty acids, they are best supplied in MCT-containing formulas (Alimentum, Pregestimil Lipil, Elecare).

However MCTs will not correct the fat malabsorption, so treatment of fat-soluble vitamin deficiencies will still be necessary. This can be accomplished either by providing 2-4 times the RDA of these vitamins (A, D, E and K) or by giving water-soluble preparations.

Finally, attention must be paid to several minerals. Even with aggressive treatment of vitamin deficiency, most children with chronic cholestasis have osteopenia and many have pathologic fractures. So additional calcium (1200 mg/day) and magnesium (8-16 mg/day) supplementation is required. Zinc deficiency is common in cholestasis and additional zinc should be supplied. Iron deficiency is also common - early due to inadequate dietary intake, later due to increased GI losses.

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