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Neonatal Ketosis

Epilepsy, Children, And The Ketogenic Diet

Epilepsy, Children, And The Ketogenic Diet

Some parents of children with epilepsy are skeptical of the ketogenic diet when they first hear about it. A diet that can control epilepsy and stop seizures without any medication? It almost sounds like a scam. But the ketogenic diet is real and legitimate. It works very well in many people. The catch is that it's extremely demanding and difficult to follow. In fact, it is so difficult to follow that most doctors recommend it only for people who haven't been able to control their seizures with medicine. The ketogenic diet is an extremely high-fat diet that requires a child to eat four times as many fat calories as calories from protein or carbohydrates. A meal might include a small portion of chicken, a little bit of fruit, and a lot of fat, typically butter or cream. Frankly, it's a difficult diet to swallow. Your child may start the diet in the hospital, so nurses and doctors can observe the first few days. Your child will probably need to go without any food for 36 to 48 hours before beginning the diet. After that, food is gradually increased over a few days. This diet does not provide all the vitamins a body needs, so your child will probably have to take sugar-free vitamin supplements. No one knows why the ketogenic diet -- which was developed in the 1920's -- works so well. But we do know something about how it affects the body. By reducing the number of carbohydrates a person eats, the body is forced to burn fat for energy, a process called ketosis. This ketosis is the same process that kicks in when someone is fasting -- on purpose or because of starvation. Fasting has been a traditional seizure treatment for centuries. How ketosis helps with epilepsy isn't known, but it does. Researchers at Johns Hopkins University studied 150 children with epilepsy in one impo Continue reading >>

Ketosis

Ketosis

Not to be confused with Ketoacidosis. Ketosis is a metabolic state in which some of the body's energy supply comes from ketone bodies in the blood, in contrast to a state of glycolysis in which blood glucose provides energy. Ketosis is a result of metabolizing fat to provide energy. Ketosis is a nutritional process characterised by serum concentrations of ketone bodies over 0.5 mM, with low and stable levels of insulin and blood glucose.[1][2] It is almost always generalized with hyperketonemia, that is, an elevated level of ketone bodies in the blood throughout the body. Ketone bodies are formed by ketogenesis when liver glycogen stores are depleted (or from metabolising medium-chain triglycerides[3]). The main ketone bodies used for energy are acetoacetate and β-hydroxybutyrate,[4] and the levels of ketone bodies are regulated mainly by insulin and glucagon.[5] Most cells in the body can use both glucose and ketone bodies for fuel, and during ketosis, free fatty acids and glucose synthesis (gluconeogenesis) fuel the remainder. Longer-term ketosis may result from fasting or staying on a low-carbohydrate diet (ketogenic diet), and deliberately induced ketosis serves as a medical intervention for various conditions, such as intractable epilepsy, and the various types of diabetes.[6] In glycolysis, higher levels of insulin promote storage of body fat and block release of fat from adipose tissues, while in ketosis, fat reserves are readily released and consumed.[5][7] For this reason, ketosis is sometimes referred to as the body's "fat burning" mode.[8] Ketosis and ketoacidosis are similar, but ketoacidosis is an acute life-threatening state requiring prompt medical intervention while ketosis can be physiological. However, there are situations (such as treatment-resistant Continue reading >>

Pitfalls Of Ketogenic Diet In A Neonate

Pitfalls Of Ketogenic Diet In A Neonate

Despite the exponential development of pharmacological agents, ketogenic diet is increasingly used in infants in a number of indications ranging from seizure disorder1 to selected metabolic disorders. We report specific difficulties we encountered treating a neonate with De Vivo disease with a ketogenic diet. This condition is characterized by deficiency of the GLUT1 transporter, which facilitates glucose diffusion through the blood-brain barrier.2 Patients present with seizures starting in the first months of life, developmental delay, persistent hypoglycorrhachia, and improve on a ketogenic diet. Our patient presented unusually early, at 13 days of age, with … Forste et al1 conclude that increases in breastfeeding among black women would narrow the gap between black and white infant mortality. They have incorrectly identified their outcome variable and reached conclusions not supported by their research. This is not a study of infant mortality (deaths in the first year of life). By excluding infant deaths in the first month of life, they have studied something akin to postneonatal mortality (deaths after day 27). High black infant mortality is largely caused by the increased incidence of preterm delivery in black women (compared with white women). Most infant mortality among black preterm babies occurs in the first 27 days. In addition, the authors have provided no … Continue reading >>

Dietary Therapies For Epilepsy Clinic

Dietary Therapies For Epilepsy Clinic

The Dietary Therapies for Epilepsy Clinic is dedicated to answering your questions about dietary treatment plans that prevent or reduce seizures. Donate to support Dietary Therapies for Epilepsy Clinic and other lifesaving efforts At the Childrens National Dietary Therapies for Epilepsy Clinic, our team works with you and your child to determine if specialized diets will help lessen or eliminate seizures that are difficult to control.Additionally, these therapies can be used to treat some metabolic conditions. The ketogenic diet for epilepsy is a high-fat, low-carbohydrate dietary program. It is used when a childs seizures cannot be controlled with medication or if he or she continues to experience intolerable side effects. In fact, in at least 38% of patients, the ketogenic diet and modified Atkins diet have decreased seizures by more than half 1. In some cases, the diet has eliminated seizures in patients all together. At Children's National, our patients and families have access to a multidisciplinary team who assists with adjusting toand maintaining a lifestyle ona ketogenic diet. Dietitians with specific expertise in ketogenic diets will work with you, helping to explain nutritional information and planning a daily diet with specific amounts of fats, protein and carbohydrates. The diet is very specialized and must be carried out under the care, supervision and guidance of trained medical specialists, so the expert knowledge of our dietitians is very important, as is their teamwork with our neurologists. The modified Atkins diet is also used for children who may have seizures or specific metabolic conditions. In contrast to the ketogenic diet, the MAD has a different ratio of grams of fat to grams of non-fat (protein and carbohydrates). This diet allows higher carb Continue reading >>

A Gestational Ketogenic Diet Alters Maternal Metabolic Status As Well As Offspring Physiological Growth And Brain Structure In The Neonatal Mouse

A Gestational Ketogenic Diet Alters Maternal Metabolic Status As Well As Offspring Physiological Growth And Brain Structure In The Neonatal Mouse

Abstract Background The use of the ketogenic diet (KD) among women of child-bearing age has been increasing, leading to increased interest in identifying the diet’s suitability during gestation. To date, no studies have thoroughly investigated the effect of a gestational KD on offspring growth. Since ketones have been reported to play a role in cerebral lipid and myelin synthesis, it is particularly important to investigate the diet’s impact on brain anatomy of the offspring. To fill this knowledge gap we imaged CD-1 mouse neonates whose mothers were fed either a standard diet (SD) or a KD prior to and during gestation. Images were collected at postnatal (P) 11.5 and 21.5 using Magnetic Resonance Imaging (MRI). Maternal metabolic status was also tracked during lactation, by following their body weight, blood glucose, ketone, cholesterol, and triglyceride concentrations. The KD dams exhibit a significant reduction in maternal fertility and litter size, as well as a high risk of developing fatal ketoacidosis by mid-lactation. To increase survival of the KD dams and offspring, fostering of P2.5 pups (from both KD and SD litters) by SD-foster dams was carried out. This resulted in stabilization of blood ketones of the KD dams, and aversion of the fatal ketoacidosis. We also note a slower and smaller weight loss for the KD compared with the SD dams. The average fostered KD pup exhibits retarded growth by P21.5 compared with the average fostered SD pup. An anatomical comparison of their brains further revealed significant structural differences at P11.5, and particularly at P21.5. The KD brain shows a relative bilateral decrease in the cortex, fimbria, hippocampus, corpus callosum and lateral ventricle, but a relative volumetric enlargement of the hypothalamus and medulla Continue reading >>

Ketogenic Diet Myth #1: Are Babies In Ketosis? Findings & Implications

Ketogenic Diet Myth #1: Are Babies In Ketosis? Findings & Implications

Ketogenic Diet Myth #1: Are Babies in Ketosis? Findings & Implications Ive heard several times now about this idea that babies are in ketosis, and in other words are keto-adapted. This idea is then used to support the ketogenic diet in human adults. Lets break this idea down here and examine its veracity. Its important to define our terms. Ive heard the saying babies are in ketosis rather than infants are in ketosis or infants less than 6 months old are in ketosis. What age group are we talking about? Infants are rapidly growing little people. Thus it become problematic already to assume that just because we have been in ketosis for a period of time during our breastfeeding careers we may be able to continue it as our bodies grow in every way and direction. So were really talking about infants, and more specifically, the typical age that breastfeeding occurs. Since this is pretty variable I cant specify an exact age, but Im guessing 12 months or less. It would also be interesting to discuss how the energy needs of infants may change as they develop physically. I would expect that as they learn to crawl, walk, and run, their bodies might start to prefer glucose. Adults who go on ketogenic diets typically go through an adaptation phase for the first two weeks of the diet. This adaptation period usually brings some discomfort, GI symptoms like constipation, and fatigue (which is often corrected with additional electrolytes). After this period, many people feel better and it is believed they have adapted to ketosis. Infants however are in a state of mild-ketosis. Their brains use ketones more so than the adult brain. From this paper (which has a very strong point of view and suggests that brains prefer ketones, which is up for debate): Postnatally, the brains dependence on Continue reading >>

Use Of The Ketogenic Diet In The Neonatal Intensive Care Unit-safety And Tolerability

Use Of The Ketogenic Diet In The Neonatal Intensive Care Unit-safety And Tolerability

Ketogenic diets are high in fat, low in carbohydrates, and contain an adequate amount of protein. In addition to the classic ketogenic diet, three alternative types of ketogenic diet therapies (KDTs) have emerged. In addition to clarifying the indications for early treatment using KDTs, ongoing research over the past decades has led to the recognition of their contraindications and adverse effects. Recent studies focusing on the targeted therapeutic range of KDTs are expected to elucidate the precise mechanisms by which they alleviate certain epilepsy syndromes and other disorders. In this review, we discuss recent advances in KDTs, focusing on six issues: the selection of a specific KDT; the use of KDTs for febrile infection-related epilepsy syndrome and super-refractory status epilepticus; the use of KDTs for infants with refractory epilepsy; links between the gut-brain axis and KDTs; triheptanoin; and the use of KDTs for disorders other than pediatric epilepsy. the Practice Committee of the Child Neurology Society The Charlie Foundation Matthew's Friends Ketogenic dietary therapies (KDT) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDT were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, preKDT counseling and evaluation, diet choice and attributes, implementation, supplementation, followup, side events, and KDT discontinuation. It has been helpful in outlining a stateoftheart protocol, standardizing KDT for multicenter clinical trials, and identifying areas of controversy and uncertainty for future research. Now one decade later, the organize Continue reading >>

Ketosis – Key To Human Babies’ Big Brains?

Ketosis – Key To Human Babies’ Big Brains?

Prof Noakes is on trial for ‘advising’ a mom to wean her baby onto low carb, high fat foods. Could babies’ innate ketosis – a state more often associated with low-carb, high-fat diets – be an arrow in Prof Noakes’ defence’s quiver? By Tamzyn Murphy Campbell BSc, BSc Med(Hons) Human Nutrition and Dietetics, RD Did you know that human newborns and exclusively breastfed babies are in ketosis? 1 I am a dietitian, with two years of intensive postgraduate training in nutrition, and I didn’t realise this until just over a month ago. The fact that human babies are naturally in ketosis is an inconvenient truth because it implies that ketosis (which also occurs when fasting or eating a low-carbohydrate, high-fat diet) is not only a natural metabolic state for human infants, but that it’s probably beneficial too. Nature seldom does something without a reason, so it’s likely that ketosis may confer some kind of evolutionary advantage to human infants. Research suggests that it may be one of the main factors behind the development of the large human brain. 2 “Nature seldom does something without a reason, so it’s likely that ketosis may confer some kind of evolutionary advantage to human infants. Research suggests that it may be one of the main factors behind the development of the large human brain. ” A word on ketones and ketosis Ketosis is a metabolic state where your body uses fat as fuel in preference to carbohydrates – as occurs when fasting or eating a low-carbohydrate, high-fat diet. The body makes ketones from fat, when dietary glucose (from carbohydrates and sugar) is low. Ketones can be used as fuel to produce the energy your body and brain needs to function. The human brain only has two options for fuel: glucose or ketones. The other body orga Continue reading >>

Ketogenic Diet

Ketogenic Diet

For certain pediatric epilepsies like Doose syndrome or myoclonia-astatic epilepsy, dietary treatment of medically uncontrolled seizures can be the first line of treatment. The Children’s Hospital of Michigan Division of Pediatric Neurology offers a variety of dietary treatment options for pediatric epilepsy. Each diet type is carefully selected in consultation with the patient’s family and physician and instruction and monitoring is provided by the registered dietitian. The “Keto Team” at the Children’s Hospital of Michigan includes the Pediatric Neurologist, Nurse Practitioner, and Registered Dietitian. The “classic” Ketogenic diet is a high-fat, low-carbohydrate diet that has been used to treat medically uncontrolled seizures for over 100 years. The team at the Children’s Hospital of Michigan has been using this diet to help children for about 20 years. The classic Ketogenic diet is the strictest version of the diet, requiring food to be weighed on a gram scale. The child is encouraged to eat all of the food provided at a meal or snack and nothing else. This diet is also the most researched, with hundred’s of scientific studies demonstrating seizure reduction. On average, 1/3 of patients see a >90% reduction in seizures, 1/3 see a 50-90% reduction, and 1/3 see <50%. The diet has been shown to be potentially effective with any types of seizure. Other dietary treatment options available at Children’s Hospital of Michigan include: the Modified Atkins Diet (MAD), which monitors the amount of carbohydrates per day and encourages fat intake, and the Low Glycemic Index diet (LGI), which limits carbohydrates to those low in glycemic index foods. If you think you are interested in a classic Ketogenic, MAD, or LGI diet for your child, please discuss this wi Continue reading >>

Weekly Q & A – Babies. Ketosis. And Misinformation.

Weekly Q & A – Babies. Ketosis. And Misinformation.

Ok guys, for those of you who were following the blog last summer, you’ve probably wondered where the heck I’ve been the last few months. Don’t worry! I’m still around – I’ve just been really busy providing face-to-face care for folks, and I’ve had less time to write and answer questions online. That being said, I have received some questions in recent weeks, and I want to get back to posting regularly. Do YOU have a nutrition question you’d like answered? If so, please send your question(s) to [email protected] Don’t worry – if you’d prefer your name not be published along with your question, simply let me know, and I’ll keep your identity secret. Now, let's check out this week's question . . . Today we're talkin' about infant nutrition. Can we just take a minute, though, to talk about how baby mugshots were a thing in the '80s? The bow scotch-taped to my bald head really makes the picture. #stylin #beforephotoshop This week’s Q & A format is going to be a little different. It’s really more of a myth-busting post. The myth we're busting is all about the infant diet and ketosis. In the last month, I’ve run into multiple people (in several different online nutrition forums, and also a few in person) who have been told at one point or another that infants, from birth, are in ketosis. The long and short of it is this: Myth: Babies are in ketosis and eat keto throughout infancy, until they transition to a solid-food diet. Fact: Babes are NOT in ketosis and are not eating keto during infancy if they are consuming breastmilk or standard infant formula. The only time that babies are maintained in ketosis is during a medically-supervised ketogenic feeding intervention, with special, prescription formula. The first time I ran into this m Continue reading >>

Use Of The Ketogenic Diet In The Neonatal Intensive Care Unit—safety And Tolerability

Use Of The Ketogenic Diet In The Neonatal Intensive Care Unit—safety And Tolerability

Summary Drug-resistant epilepsy poses a challenge in neonatal patients, especially those in the neonatal intensive care unit (NICU), who have various secondary comorbidities. We present results of four children with a history of drug-resistant epilepsy for whom a ketogenic diet was initiated and used in the NICU. A nonfasting induction into ketosis over 1–2 weeks was utilized, with gradual increases in the ketogenic ratio every 2–3 days. Data were collected retrospectively from a database, which included medical history, daily progress notes, relevant laboratory data, and imaging and diagnostic information. The ketogenic diet was well tolerated in all cases. The most common side effects observed were constipation, hypoglycemia, and weight loss. Serum β-hydroxybutyrate levels demonstrated improved reliability as a marker of ketosis when compared to urine ketones in this population. Perceived benefits to the infants included improved seizure control, increased alertness, and decreased need for invasive respiratory support. These cases demonstrate that the use of the ketogenic diet for treatment of neonatal encephalopathy and refractory epilepsy can be undertaken safely in the NICU and is well tolerated by carefully screened neonates and infants. Continue reading >>

Neonatal Iem

Neonatal Iem

Neonatal IEM Inborn errors of metabolism (IEM) are an important cause of acute illness in newborns. Presentation may mimic common neonatal conditions such as sepsis. Prompt detection requires a high index of suspicion and the early measurement of biochemical markers such as blood ammonia. Diagnosis is important not only for treatment but also for genetic counselling. In Neonatal IEM – pregnancy and delivery are generally uneventful. The newborn baby with IEM is normal for few hours or first day to 3-4 days after which the disorder presents due to intake of dietary protein or sugar (Lactose, Fructose). Neonates with IEMs are often misdiagnosed as Sepsis or other disorders. Sepsis often accompanies IEM but may confine the diagnosis. Neonates have limited responses to illness and prominent ones are poor feeding, lethargy, coma, failure to thrive, seizures, acidosis or ketosis. A high index of suspicion and follow-up diagnosis is necessary when the following symptoms are present - Parental consanguinity (marriages in relation). Positive Family History Symptoms onset a few hours to days after feeding (poor feeding, lethargy, seizures, vomiting, diarrhea, coma, hypotonia) Ketosis, hypoglycemia, acidosis Unusual odor in the urine Jaundice Dysmorphic features Newborn Screening Newborn Screening or NBS as it is commonly called, is the process of screening newborn babies for metabolic disorders, genetic diseases, blood diseases etc. Newborn Screening refers to tests carried out on a newborn baby's blood to detect Inborn Errors of Metabolism (IEMs). These tests cover a wide variety of diseases and help in preventing future issues. Early detection may, in some cases, even suppress a disorder entirely. Screening of newborns is most effective during the neonatal period, which is wh Continue reading >>

Is The Ketogenic Diet Natural For Humans?

Is The Ketogenic Diet Natural For Humans?

Is the Ketogenic Diet Natural for Humans? As discussed in the first article of this series, ketosis is a metabolic state in which the brain switches to using ketone bodies derived from the breakdown of fat as its primary energy source, instead of glucose. This way body protein, which would otherwise be broken down and converted into glucose through the process of gluconeogenesis, are spared. Ketosis is an adaptive state that allowed our ancestors to survive temporary food shortages. When food was not available at all, or the only food available was extremely low in energy (such as leaves and grasses), their bodies could start to break down their body fat reserves after a couple of days. Ketone bodies were generated as a result allowing them to sustain their brains and preserve their muscle and other vital proteins. Our human ancestors did not consume high-fat, low-carbohydrate diets and therefore would not have been in diet-induced ketosis. Note the emphasis on ketosis as a temporary adaptive state. Our ancestors could not be in fasting-induced ketosis permanently because they would eventually exhaust their fat reserves (which were presumably far more limited than ours, owing to the simple fact that they ate less and moved more than we do). They would then progress from fasting to starving, and subsequently die. Yet ketogenic diet proponents advocate that we should aim to keep ourselves in a permanent state of ketosis, something that is completely foreign to human experience and to the experience of all other animals, for that matter. No animal on earth lives permanently in ketosis. Omnivorous animals such as bears and dogs, and obligate carnivores such as cats the ultimate low-carbers use gluconeogenesis to transform amino acids from protein into glucose [1] . This al Continue reading >>

Ketogenic Diet, A Potentially Valuable Therapeutic Option For The Management Of Refractory Epilepsy In Classical Neonatal Nonketotic Hyperglycinemia: A Case Report

Ketogenic Diet, A Potentially Valuable Therapeutic Option For The Management Of Refractory Epilepsy In Classical Neonatal Nonketotic Hyperglycinemia: A Case Report

Nonketotic hyperglycinemia (NKH) is a devastating inborn error of glycine metabolism caused by deficient activity of the glycine cleavage enzyme. Classically, patients present with lethargy, hypotonia, myoclonic jerks, transient respiratory depression in the first week of life and often progress to death. Surviving infants have profound psychomotor retardation, refractory epilepsy and poor quality of life. Currently, no effective therapeutic avenues exist for severe NKH. Ketogenic diet (KD) has been trialled only in a small group of patients with neonatal NKH and early myoclonic encephalopathy, in whom significant improvements in seizure control were reported. We describe an infant with classical neonatal NKH who presented on the third day of life with hypotonia, poor feeding, respiratory insufficiency resulting in ventilatory support and seizures with burst-suppression pattern on electroencephalogram (EEG). KD initiated at age 6 months for intractable seizures, lead to a dramatic decrease in seizure frequency, EEG improvements, normalisation of plasma glycine levels, reduced spasticity and improved quality of life. KD may be a valuable treatment modality for refractory seizure control in classical NKH. Continue reading >>

Ketogenic Diet

Ketogenic Diet

Dr. Thio is an Assistant Professor of Neurology at Washington University School of Medicine and an epileptologist in the Pediatric Epilepsy Center at St. Louis Children’s Hospital. Introduction The ketogenic diet is only one of several methods available for treating epilepsy. Other treatment options include antiepileptic drugs, epilepsy surgery, and the vagal nerve stimulator. If your child’s epilepsy is treated, most neurologists will try using an antiepileptic drug first. Generally, the ketogenic diet will not be the first choice, though exceptions exist. The ketogenic diet is a high fat, low carbohydrate, and low protein diet designed to increase the body’s dependence on fat rather than glucose for energy. It mimics the fasting state, and can treat disorders of the brain. The two disorders most commonly treated with the ketogenic diet are epilepsy and certain inborn errors of metabolism involving glucose utilization. This paper will briefly review the history, physiology, efficacy, mechanism of action, indications, contraindications, deficiencies, adverse effects, and some of the practical aspects of initiating and maintaining the ketogenic diet as related to epilepsy. History The ketogenic diet has its origin in the observation that fasting reduces seizures. This observation is old since Hippocrates used fasting to treat seizures, and the Bible mentions fasting as a treatment for seizures. In 1921, Wilder proposed a high fat, low carbohydrate diet as a means of mimicking fasting, which is not practical for extended periods. After phenobarbital and phenytoin became available, interest in the diet declined. However, interest has increased recently because 20-30% of epileptic children have seizures that are resistant to antiepileptic drugs. The introduction of se Continue reading >>

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