scholarly journals The Effect of a Fat-Restricted Diet in Four Patients with Familial Chylomicronemia Syndrome: A Long-Term Follow-Up Study

Children ◽  
2021 ◽  
Vol 8 (11) ◽  
pp. 1078
Author(s):  
Alexandra Thajer ◽  
Gabriele Skacel ◽  
Charlotte de Gier ◽  
Susanne Greber-Platzer
Keyword(s):  
Acute Pancreatitis ◽  
Dietary Fat ◽  
Pediatric Patients ◽  
Caloric Intake ◽  
Restricted Diet ◽  
Traffic Light ◽  
Low Fat Diet ◽  
Daily Caloric Intake ◽  
Light Table

(1) Background: Familial chylomicronemia syndrome (FCS) is a very rare autosomal recessive disorder characterized by severely elevated triglycerides and clinical symptoms in early childhood mainly presenting with abdominal pain, acute pancreatitis and hepatosplenomegaly. Primary treatment is a lifelong very strict low-fat diet, which might be challenging in pediatric patients. So far, data about children with FCS are rare. The aim of this study was to show the familial chylomicronemia syndrome traffic light table for pediatric patients and to assess the dietary fat intake and impact on triglycerides in children with FCS. (2) Methods: We performed a retrospective analysis in four children (50% male) affected by FCS from the Department of Pediatrics and Adolescent Medicine, Medical University of Vienna between January 2002 and September 2020. (3) Results: The four patients presented with classical FCS symptoms and showed baseline triglycerides (TG) exceeding 30,000 mg/dL in two patients, 10,000 mg/dL and 2400 mg/dL in one patient each. After diagnosis, fat percentage of total daily caloric intake was decreased and resulted immediately in triglyceride reduction. In all patients, FCS was genetically confirmed by mutations in genes encoding lipoprotein lipase. Acute pancreatitis and hepatosplenomegaly disappeared under the fat-restricted diet. A FCS traffic light table was developed as a dietary tool for affected families. (4) Conclusions: A restriction of dietary fat between 10% to 26% of the total daily caloric intake was feasible and effective in the long-term treatment of genetically confirmed FCS in children and could reduce the risk for acute pancreatitis. The dietary tool, the pediatric FCS traffic light table and the age-appropriate portion sizes for patients between 1 to 18 years, supports children and their parents to achieve and adhere to the lifelong strict low-fat diet.

scholarly journals Management of severe hypertriglyceridemia due to lipoprotein lipase deficiency in children

2019 ◽  
Vol 2019 ◽  
Author(s):  
Sarah W Y Poon ◽  
Karen K Y Leung ◽  
Joanna Y L Tung
Keyword(s):  
Acute Pancreatitis ◽  
Lipoprotein Lipase ◽  
Normal Growth ◽  
List Type ◽  
Low Fat Diet ◽  
Intravenous Insulin ◽  
Lipoprotein Lipase Deficiency ◽  
Severe Hypertriglyceridemia

Summary Severe hypertriglyceridemia is an endocrine emergency and is associated with acute pancreatitis and hyperviscosity syndrome. We describe an infant with lipoprotein lipase deficiency with severe hypertriglyceridemia who presented with acute pancreatitis. She was managed acutely with fasting and intravenous insulin infusion, followed by low-fat diet with no pharmacological agent. Subsequent follow-up until the age of 5 years showed satisfactory lipid profile and she has normal growth and development. Learning points: Hypertriglyceridemia-induced acute pancreatitis has significant morbidity and mortality, and prompt treatment is imperative. When no secondary causes are readily identified, genetic evaluation should be pursued in hypertriglyceridemia in children. Intravenous insulin is a safe and effective acute treatment for hypertriglyceridemia in children, even in infants. Long-term management with dietary modifications alone could be effective for primary hypertriglyceridemia due to lipoprotein lipase deficiency, at least in early childhood phase.

scholarly journals Effect of a long-term high-protein diet on survival, obesity development, and gut microbiota in mice

2016 ◽  
Vol 310 (11) ◽  
pp. E886-E899 ◽  
Author(s):  
Pia Kiilerich ◽  
Lene Secher Myrmel ◽  
Even Fjære ◽  
Qin Hao ◽  
Floor Hugenholtz ◽  
...  
Keyword(s):  
Gene Expression ◽  
Adipose Tissue ◽  
Gut Microbiota ◽  
Dietary Fat ◽  
High Fat Diet ◽  
High Fat ◽  
Low Fat ◽  
Low Fat Diet ◽  
Low Protein

Female C57BL/6J mice were fed a regular low-fat diet or high-fat diets combined with either high or low protein-to-sucrose ratios during their entire lifespan to examine the long-term effects on obesity development, gut microbiota, and survival. Intake of a high-fat diet with a low protein/sucrose ratio precipitated obesity and reduced survival relative to mice fed a low-fat diet. By contrast, intake of a high-fat diet with a high protein/sucrose ratio attenuated lifelong weight gain and adipose tissue expansion, and survival was not significantly altered relative to low-fat-fed mice. Our findings support the notion that reduced survival in response to high-fat/high-sucrose feeding is linked to obesity development. Digital gene expression analyses, further validated by qPCR, demonstrated that the protein/sucrose ratio modulated global gene expression over time in liver and adipose tissue, affecting pathways related to metabolism and inflammation. Analysis of fecal bacterial DNA using the Mouse Intestinal Tract Chip revealed significant changes in the composition of the gut microbiota in relation to host age and dietary fat content, but not the protein/sucrose ratio. Accordingly, dietary fat rather than the protein/sucrose ratio or adiposity is a major driver shaping the gut microbiota, whereas the effect of a high-fat diet on survival is dependent on the protein/sucrose ratio.

Dietary fat stimulates endogenous enkephalin and dynorphin in the paraventricular nucleus: role of circulating triglycerides

2007 ◽  
Vol 292 (2) ◽  
pp. E561-E570 ◽  
Author(s):  
G.-Q. Chang ◽  
O. Karatayev ◽  
R. Ahsan ◽  
V. Gaysinskaya ◽  
Z. Marwil ◽  
...  
Keyword(s):  
Paraventricular Nucleus ◽  
Dietary Fat ◽  
Opioid Peptides ◽  
High Fat Diet ◽  
Caloric Intake ◽  
High Fat ◽  
Caloric Density ◽  
Low Fat Diet ◽  
Diet Intake

The opioid peptides enkephalin (ENK) and dynorphin (DYN), when injected into the hypothalamus, are known to stimulate feeding behavior and preferentially increase the ingestion of a high-fat diet. Studies of another peptide, galanin (GAL), with similar effects on feeding demonstrate that a high-fat diet, in turn, can stimulate the expression of this peptide in the hypothalamus. The present study tested different diets and variable periods of high- vs. low-fat diet consumption to determine whether the opioid peptides respond in a similar manner as GAL. In six experiments, the effects of dietary fat on ENK and DYN were examined in three hypothalamic areas: the paraventricular nucleus (PVN), perifornical hypothalamus (PFH), and arcuate nucleus (ARC). The results demonstrated that the ingestion of a high-fat diet increases gene expression and peptide levels of both ENK and DYN in the hypothalamus. The strongest and most consistent effect is seen in the PVN. In this nucleus, ENK and DYN are increased by 50–100% after 1 wk, 1 day, 60 min, and even 15 min of high-fat diet consumption. While showing some effect in the PFH, these peptides in the ARC are considerably less responsive, exhibiting no change in response to the briefer periods of diet intake. This effect of dietary fat on PVN opioids can be observed with diets equal in caloric density and palatability and without a change in caloric intake, body weight, fat pad weight, or levels of insulin or leptin. The data reveal a strong and consistent association between these peptides and a rise in circulating levels of triglycerides, supporting a role for these lipids in the fat-induced stimulation of opioid peptides in the PVN, similar to GAL.

scholarly journals The role of dietary fat in obesity-induced insulin resistance

2016 ◽  
Vol 311 (6) ◽  
pp. E989-E997 ◽  
Author(s):  
Denise E. Lackey ◽  
Raul G. Lazaro ◽  
Pingping Li ◽  
Andrew Johnson ◽  
Angelina Hernandez-Carretero ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Dietary Fat ◽  
High Fat Diet ◽  
Caloric Intake ◽  
High Fat ◽  
Low Fat ◽  
Feeding Method ◽  
Low Fat Diet ◽  
Obesity And Diabetes

Consumption of excess calories results in obesity and insulin resistance and has been intensively studied in mice and humans. The objective of this study was to determine the specific contribution of dietary fat rather than total caloric intake to the development of obesity-associated insulin resistance. We used an intragastric feeding method to overfeed excess calories from a low-fat diet (and an isocalorically matched high-fat diet) through a surgically implanted gastric feeding tube to generate obesity in wild-type mice followed by hyperinsulinemic-euglycemic clamp studies to assess the development of insulin resistance. We show that overfeeding a low-fat diet results in levels of obesity similar to high-fat diet feeding in mice. However, despite a similar body weight, obese high-fat diet-fed mice are more insulin resistant than mice fed an isocaloric low-fat diet. Therefore, increased proportion of calories from dietary fat further potentiates insulin resistance in the obese state. Furthermore, crossover diet studies revealed that reduction in dietary fat composition improves glucose tolerance in obesity. In the context of the current obesity and diabetes epidemic, it is particularly important to fully understand the role of dietary macronutrients in the potentiation and amelioration of disease.

scholarly journals Transection of Gustatory Nerves Differentially Affects Dietary Fat Intake in Obesity-Prone and Obesity-Resistant Rats

2020 ◽  
Vol 45 (7) ◽  
pp. 541-548
Author(s):  
Allyson Schreiber ◽  
Hugh Douglas Braymer ◽  
Stefany D Primeaux
Keyword(s):  
Dietary Fat ◽  
Caloric Intake ◽  
Afferent Input ◽  
Fat Intake ◽  
Fungiform Papillae ◽  
Dietary Fat Intake ◽  
Low Fat Diet ◽  
Current Prevalence ◽  
Prevalence Of Obesity

Abstract The current prevalence of obesity has been linked to the consumption of highly palatable foods and may be mediated by a dysregulated or hyposensitive orosensory perception of dietary fat, thereby contributing to the susceptibility to develop obesity. The goal of the current study was to investigate the role of lingual taste input in obesity-prone (OP, Osborne-Mendel) and obesity-resistant (OR, S5B/Pl) rats on the consumption of a high-fat diet (HFD). Density of fungiform papillae was assessed as a marker of general orosensory input. To determine if orosensory afferent input mediates dietary fat intake, surgical transection of the chorda tympani and glossopharyngeal nerves (GLX/CTX) was performed in OP and OR rats and HFD caloric intake and body weight were measured. Fungiform papillae density was lower in OP rats, compared with OR rats. GLX/CTX decreased orosensory input in both OP and OR rats, as measured by an increase in the intake of a bitter, quinine solution. Consumption of low-fat diet was not altered by GLX/CTX in OP and OR rats; however, GLX/CTX decreased HFD intake in OR, without altering HFD intake in OP rats. Overall, these data suggest that inhibition of orosensory input in OP rats do not decrease fat intake, thereby supporting that idea that hyposensitive and/or dysregulated orosensory perception of highly palatable foods contribute to the susceptibility to develop obesity.

Hyperphagia and Weight Loss during Fluoxetine Treatment

1994 ◽  
Vol 28 (12) ◽  
pp. 1350-1352 ◽  
Author(s):  
Christopher G. Fichtner ◽  
Bennett G. Braun
Keyword(s):  
Weight Loss ◽  
Dietary Fat ◽  
Caloric Intake ◽  
Weight Changes ◽  
Fluoxetine Treatment ◽  
Case Summary ◽  
Daily Caloric Intake ◽  
Fat Consumption ◽  
Modest Weight Loss ◽  
Treatment Of Depression

OBJECTIVE: To report the unusual coincidence of weight loss with increased appetite and food intake in a patient treated for depression on two separate occasions with fluoxetine. CASE SUMMARY: A 27-year-old woman experienced a modest weight loss during treatment for depression with fluoxetine. The weight loss was associated with a reported increase in daily caloric intake and consumption of a greater proportion of dietary fat than usual for the patient. The same patient was treated again with fluoxetine more than a year later and again experienced weight loss associated with an increase in appetite, caloric intake, and dietary fat consumption. DISCUSSION: Fluoxetine is a selective serotonin reuptake inhibitor that often is associated with a modest weight loss when used for the treatment of depression, although it also has been reported to have the opposite effects of weight gain and hyperphagia in some patients. The effects on weight usually are assumed to be the result of primary effects on appetite, but the discrepancy between the appetite and weight changes in this case challenges the applicability of that assumption in all cases. CONCLUSIONS: The effects of fluoxetine on appetite and weight may be mediated by partially distinct mechanisms and might conceivably involve a direct metabolic effect in some patients.

Therapeutic Plasma Exchange Application in Children Requires Individual Decision

2020 ◽  
Author(s):  
Gürkan Atay ◽  
Demet Demirkol
Keyword(s):  
Plasma Exchange ◽  
Vascular Access ◽  
Pediatric Patients ◽  
Demyelinating Disease ◽  
Pediatric Intensive Care ◽  
Therapeutic Plasma Exchange ◽  
Transplantation Rejection ◽  
Access Problems ◽  
Uremic Syndrome

AbstractTherapeutic plasma exchange (TPE) is a treatment administered with the aim of removing a pathogenic material or compound causing morbidity in a variety of neurologic, hematologic, renal, and autoimmune diseases. In this study, we aimed to assess the indications, efficacy, reliability, complications, and treatment response of pediatric patients for TPE. This retrospective study analyzed data from 39 patients aged from 0 to 18 years who underwent a total of 172 TPE sessions from January 2015 to April 2018 in a tertiary pediatric intensive care unit. Indications for TPE were, in order of frequency, macrophage activation syndrome (28.2%, n = 11), renal transplantation rejection (15.4%, n = 6), liver failure (15.4%, n = 6), Guillain–Barre's syndrome (15%, n = 6), hemolytic uremic syndrome (7.7%, n = 3), acute demyelinating disease (7.7%, n = 3), septic shock (5.1%, n = 2), and intoxication (5.1%, n = 2). No patient had any adverse event related to the TPE during the procedure. The TPE session was ended prematurely in one patient due to insufficient vascular access and lack of blood flow (2.6%). In the long term, thrombosis due to the indwelling central catheter occurred (5.1%, n = 2). TPE appears to be an effective first-stage or supplementary treatment in a variety of diseases, may be safely used in pediatric patients, and there are significant findings that its area of use will increase. In experienced hands and when assessed carefully, it appears that the rate of adverse reactions and vascular access problems may be low enough to be negligible.

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