The rare syndrome imposes a limit of 10% of daily calories;  learn about SQF

The rare syndrome imposes a limit of 10% of daily calories; learn about SQF


Familial chylomicronemia syndrome (FFS) significantly increases triglyceride levels, requiring a restrictive diet

We all know it’s important to avoid ultra-processed foods high in sodium, sugar and fat. But what to do when even healthy foods shouldn’t be part of the diet? This is the case for those who have the Familial chylomicronemia syndrome (FFS)a rare disease that limits the daily amount of calories in food.

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Patients affected by this syndrome have a defect in the gene responsible for the production of the enzyme lipoprotein lipase (LPL), which has the role of metabolizing triglycerides. As a result, the rates increase, causing abdominal pain, pancreatitis, changes in the retinal vessels and skin lesions, called eruptive xanthomas, for example.

To minimize these complications, patients must follow a very restricted diet throughout their lives, typically no more than 10% of total daily calories. This is because even the foods suggested for the diet of those seeking a more balanced and healthy life, despite being rich in nutrients and minerals, may have a high fat content. Therefore, they are contraindicated for individuals suffering from SQF.

“Consequently, once the diagnosis is confirmed, it is important that the patient is followed by a nutritionist who will provide adequate and personalized dietary advice”, explains Ana Maria Pita Lottenberg, PhD and Master in Food Sciences at the Faculty of Pharmaceutical Sciences of the University of Sao Paulo (USP).

Familial chylomicronemia syndrome (FFS)

FQS is a rare, autosomal recessive disorder, meaning the affected person must inherit the altered genes from both parents to develop it. Therefore, if both the father and mother carry the gene, each child of the couple has a 25% chance of having the disease. The disease is estimated to affect between one and two people in 1 million worldwide.1,6

The disease is characterized by a defect in the gene responsible for the production of the enzyme lipoprotein lipase (LPL), or by a defect in other genes associated with the function of LPL, which has the role of metabolizing triglycerides. As a result, there is a buildup of chylomicrons, which are fat particles that transport triglycerides through the blood system.

This excess significantly increases triglyceride levels, causing abdominal pain, fatigue, memory impairment, attacks of pancreatitis, changes in the retinal vessels and skin lesions, called eruptive xanthomas.

Banned Foods for Those Suffering from Familial Chylomicronemia Syndrome (FFS)

Due to the restrictions, patients must replace a number of foods, as listed below. Watch:[arelacionadoabaixoConfira:[arelacionadoabaixoConfira:

  • Red meat: Red meat is high in fat and has a high content of saturated fatty acids, which further increases triglycerides in the blood. An alternative is the consumption of chicken (skinless), fish and eggs;
  • Whole or partially skimmed milk, cheeses, yogurt and derivatives: Milk, yogurt and curd can only be consumed in fully skimmed form. As for cheese, almost all types are very high in fat and, like red meat, are also rich in saturated fatty acids. One option could be to consume skimmed ricotta.
  • Oil, olive oil and butter: avoid adding fat to recipes. Cook with water, garlic, onion and spices. Leave your daily portion of fat reserved for those naturally present in certain foods that will be part of your eating plan, such as protein sources. In the case of butter you can replace it, for example, with skimmed ricotta.
  • Coconut, avocado and oily fruits: Although they are rich in nutrients, vitamins and minerals, these fruits represent one of the main dietary sources of vegetable fats. Therefore, they should be avoided by SQF holders. Other fruits can be consumed in the quantities recommended by the nutritionist.

Recipes for those with SQF

With this dietary restriction in mind, a group of specialized nutritionists, with the support of PTC Therapeutics, developed the “Discovering New Flavors” cookbook, specifically for CFS sufferers. The aim is to offer practical, regional dishes, low in fat and easy to prepare even for those who do not have much experience in the kitchen.

It contains recipes with foods allowed in the diet of these people, such as fruit, green leaves, colorful vegetables, beans, peas and lentils, non-fatty white fish, herbs and spices, for example. Click on the link to download the book for free which contains more than 130 recipes.

Source: Terra

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