Table 5.
Dietary treatment, food preferences/aversions and growth parameters of all study participants.
| Subject | Presentation | Growth at Presentation (Height-for-Age z-Scores) |
Last Growth (Height-for-Age z-Scores) |
Food Aversions/Preferences | Current Prescribed Dietary Management | Median Intake from Dietary Assessments | Oral Emergency Feed for Illness Management |
|---|---|---|---|---|---|---|---|
| 1 (Family 1) |
Family screening | −1.11 | −1.52 | Likes custard. | Normal diet. | CHO: 42% Protein: 18% Fat: 40% |
Not prescribed. |
| 2 (Family 1) |
Family screening | −2.29 | −2.35 | Avoids: sweets, puddings, fruit juice, cakes, biscuits. Prefers: meat, fish, cheese, eggs, soya, nuts. |
Low sugar, high protein, high fat diet. | CHO: 28% Protein: 20% Fat: 53% |
Low sugar soya milk. |
| 3 (Family 1) |
FTTDCD | −2.96 | −2.62 | Avoids: sweets, puddings, fruit juice, cakes and biscuits. Prefers: meat, fish, cheese, eggs, soya, nuts. |
Low sugar, high protein, high fat diet + MCT oil. One oral feed in the middle of the night. | CHO: 30% Protein: 19% Fat: 51% |
Low sugar soya milk + MCT emulsion + soya protein powder. |
| 4 | NICCD | −0.98 | −2.31 | Initial self- restriction of sweets but now eats them (with symptoms). Prefers high protein foods. |
Normal diet (but feeds 4 hourly day and night). |
CHO: 44% Protein: 18% Fat: 43% |
Not prescribed. |
| 5 (Family 2) |
Family screening | 1.85 | −1.01 | Dislikes sweets. | Normal diet (but feeds 4 hourly day and night). |
NA | Not prescribed. |
| 6 (Family 2) |
FTTDCD | −0.03 | 1.36 | Dislikes sweets. Likes meat. | Low sugar, high protein, high fat diet (MCT). | NA | Liquid chocolate yoghurt. |
| 7 | NICCD | −2.91 | 0.84 | Dislikes CHO foods, e.g., bread, rice, potatoes, sweets. Prefers high protein foods, e.g., bacon, eggs, nuts. |
Low sugar, high protein high fat diet (LCT/MCT). CHO limited to 30g/day. | CHO: 18% Protein: 27% Fat: 55% |
Soya protein powder + LCT/MCT emulsions + water. |
| 8 | NICCD | −1.08 | 1.94 | None described. | Normal diet. | CHO: 47% Protein: 19% Fat: 33% |
Not prescribed. |
| 9 | NICCD | −0.57 | −0.60 | Avoids sugar and likes cheese, fish, potatoes. | Normal diet. | CHO: 42% Protein: 23% Fat: 35% |
Soya milk. |
| 10 | FTTDCD | −1.05 | −0.58 | Avoids sugar. | Normal diet. | CHO: 45% Protein: 19% Fat: 36% |
Not prescribed. |
| 11 | NICCD | −0.43 | −2.01 | Eats sugary foods. Dislikes cheese. | Low sugar, high protein, high fat diet. Casein hydrolysed formula with MCT. | CHO: 40% Protein: 11% Fat: 49% |
Hydrolysed formula (55% MCT fat). |
| 12 | NICCD | −2.54 | −4.51 | Eats sweet puddings. | Casein hydrolysed formula with MCT. | CHO: 43% Protein: 12% Fat: 45% |
Hydrolysed formula (55% MCT fat). |
| 13 | FTTDCD | −0.19 | −0.44 | Does not eat cake or biscuits; rarely eats rice, pasta and bread. Prefers high protein foods (yoghurt, milk, meat, eggs). | Low sugar, high protein/ high fat diet. | CHO: 47% Protein: 19% Fat: 38% |
Not prescribed. |
| 14 (Family 3) |
FTTDCD | 0.69 | 0.91 | Dislikes sweets. Avoids CHO and prefers high protein foods (meat/cheese/milk). | Normal diet. | CHO: 28% Protein: 26% Fat: 46% |
Not prescribed. |
| 15 (Family 3) |
Family screening | −0.16 | −0.08 | Dislikes sweets. Avoids vegetables and fruits. Craves high protein foods, particularly yoghurt. Likes cream. | Low sugar, high protein, high fat diet. | CHO: 32% Protein: 20% Fat: 48% |
Full fat milk. |
| 16 | NICCD | −1.04 | 0.02 | Dislikes sweets. Avoids high CHO containing foods. Prefers high protein foods (egg, meat, cheese). |
Normal diet. | CHO: 43% Protein: 17% Fat: 37% |
Full fat milk. |
| 17 | Family screening | −2.71 | −1.70 | Avoids sugary foods. Likes yoghurt. | Lactose free diet (soya milk). | CHO: 41% Protein: 15% Fat: 47% |
Soya milk. |
| 18 | NICCD | −0.26 | −0.22 | Self-selects a high protein and high fat diet. Dislikes sweets and fizzy drinks. | Low sugar, high protein, high fat diet. | CHO: 42% Protein: 19% Fat: 38% |
Full fat milk, nuts. |
| 19 (Family 4) |
Family screening | −0.89 | −0.40 | Likes CHO containing foods and sweets. | Normal diet. | CHO: 44% Protein: 16% Fat: 38% |
Not prescribed. |
| 20 (Family 4) |
FTTDCD | −1.25 | −0.53 | Dislikes CHO containing foods and fruit. Likes meat. | Normal diet. | CHO: 42% Protein: 15% Fat: 43% |
Not prescribed. |
| 21 | FTTDCD | −1.41 | −1.74 | Dislikes fruit and vegetables. Prefers chicken. | Normal diet. | CHO: 46% Protein: 17% Fat: 35% |
Not prescribed. |
| 22 | NICCD | −2.69 | −0.46 | Dislikes vegetables. Prefers cheese. | Low sugar, high protein, high fat diet. | CHO: 40% Protein: 17% Fat: 41% |
Not prescribed. |
| 23 | Family screening | −3.47 | −1.04 | Likes high protein foods. | Low sugar, high protein, high fat diet. | CHO: 43% Protein: 14% Fat: 43% |
Not prescribed. |
| 24 | NICCD | −2.97 | −1.29 | Dislikes rice, potatoes and pasta but likes chicken and fish. | Low sugar, high protein, high fat diet. | NA | Full fat milk. |
| 25 | NICCD | 0.22 | −0.43 | Likes meat. | Low sugar, high protein, high fat diet. | NA | Full fat milk. |
| 26 (Family 5) |
Family screening | −2.29 | −2.12 | Avoids many CHO containing food, fruit and vegetables. Prefers high protein foods. | Normal diet. | CHO: 41% Protein: 22% Fat: 36% |
Full fat milk. |
| 27 (Family 5) |
Family screening | −0.64 | −0.15 | No food aversions. Likes dairy products. | Normal diet. | CHO: 44% Protein: 20% Fat: 36% |
Full fat milk. |
| 28 (Family 5) |
NICCD | −0.82 | −0.39 | No food aversions. Likes chicken. | Normal diet. | NA | Full fat milk. |
| 29 (Family 5) |
Family screening | −0.99 | −1.38 | Likes chicken and cheese. Does not like dairy products. | Normal diet. | CHO: 49% Protein: 13% Fat: 38% |
Full fat milk. |
| 30 (Family 5) |
Family screening | −1.43 | −0.91 | No preferences. | Normal diet. | CHO: 48% Protein: 20% Fat: 32% |
Full fat milk. |
| 31 (Family 5) |
Family screening | −0.89 | −0.89 | Dislikes sweet foods. Poor tolerance to dairy foods. | Normal diet. | CHO: 31% Protein: 23% Fat: 42% |
Full fat milk. |
| 32 | FTTDCD | −0.57 | −0.96 | Dislikes sugary foods. Prefers savoury foods. | Normal diet. | CHO: 37% Protein: 23% Fat: 38% |
Soya milk, nuts. |
Abbreviations: MCT—medium chain triglyceride; LCT—long chain triglyceride; CHO—Carbohydrates; NICCD—neonatal intrahepatic cholestasis; FTTDCD—failure to thrive and dyslipidaemia caused by citrin deficiency; NA—Not available.