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Neonatal Ketosis

Use Of The Ketogenic Diet In Neonates And Infants

Use Of The Ketogenic Diet In Neonates And Infants

Abstract The ketogenic diet (KD) is still viewed as virtually last-line therapy in childhood epilepsies, and specifically as unsafe and difficult to initiate and maintain in neonates and infants. Information is presented to show that the KD is safe and efficacious in this population, and should be carefully studied to determine its real usefulness as first-line or early therapy (one or fewer anticonvulsants) in the catastrophic epilepsies of infancy. Continue reading >>

Ketogenic Diet … For Neonates?

Ketogenic Diet … For Neonates?

The last few years have seen a trend towards considering the ketogenic diet for the “extremes” of age. Some research points to safety and benefits for elderly people with epilepsy (and possibly dementia), and other studies suggest that infants may in fact do better than older children. Using the diet in people outside the typical school-aged childhood age range has changed our way of thinking about this therapy. Study of Neonatal Infants on Ketogenic Diet Adding to this trend, an interesting article was just published in January 2017 in the journal Epilepsia from Lindsey Thompson RD, the ketogenic dietitian at Children’s Mercy Comprehensive Epilepsy Center in Kansas City, and her excellent team. Entitled “Use of the ketogenic diet in the neonatal intensive care unit – safety and tolerability,” this was a short case series of four infants with very severe epilepsy who had failed to respond to 3 or 4 drugs to control their seizures in the ICU. Their ages ranged from 6 to 10 weeks when the ketogenic diet was started, which is amongst the youngest ever reported. All were started gradually without a fast over a 1- to 2-week period, which is a bit slower than typically done for older children. What happened? All four achieved ketosis with side effects of constipation in two and hypoglycemia in just one (interestingly, the oldest infant). Three of four had subjective improvement in seizures, including two infants who had periods of seizure freedom. However, the EEG improved in only one child, fitting with research suggesting the EEG does not always correlate with clinical improvement. Even more remarkably, all four are still on the ketogenic diet at this time, including three children with over 2 years of continued dietary treatment. Study Authors Answer Questions 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 >>

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 >>

Babies In Ketosis

Babies In Ketosis

This post topic was inspired by the following article: Ketosis - key to human babies’ big brains? It is hosted on Tim Noakes' website and written by one of his associates in nutritional information misdirection, Tamzyn Murphy Campbell, RD. I'm going to address this misdirection and the disturbing parts of this article vis a vis Campbell in a future BabyGate Files, but for now I want to discuss the role of ketones in metabolism. In doing so, I'd also like to explain my somewhat cryptic recent post on heating my kitchen. (I've C&P'd that entire post to the end further down in this one, so if you don't wish to go to another page, you can just scroll down to The Kitchen Heating Analogy). I'm going to structure this post a little differently than most and get to my point, then provide the back up information. Let's see how this goes. The major source (6 of 12 numbered citations, 1 of 6 unique sources) for her article is: Survival of the fattest: fat babies were the key to evolution of the large human brain (2003) Stephen C. Cunnane, Michael A. Crawford (I'll call this C&C) In this paper, they make the following points: Human babies have higher body fat than other mammals and this may be as a source of ketones in early infancy. Infants have slightly elevated ketones (mild ketonemia) regardless of feeding status which differs from adults who only have elevated ketones when food is restricted (no details here). Glucose is the primary fuel for the human brain Ketones are an alternate source of fuel when glucose is less available Ketones "appear to be" an essential fuel for the midterm fetus, and may provide as much as 30% of the developing brain's needs Ketones are both an energy source and carbon source for fat and cholesterol synthesis in the brain. Campbell relates these so Continue reading >>

Neonatal Ketosis Is Not Rare: Experience Of Neonatal Screening Using Gas Chromatography-mass Spectrometry.

Neonatal Ketosis Is Not Rare: Experience Of Neonatal Screening Using Gas Chromatography-mass Spectrometry.

Abstract The causes and effects of transient neonatal ketosis, discovered during a pilot study of screening for abnormalities in neonatal metabolism using gas chromatography-mass spectrometry, were investigated. Of the 21,342 neonates that were screened, 47 had significant ketosis. The organic acid profile accompanying ketosis in the urine of neonates followed the pattern of ketotic dicarboxylic aciduria in approximately half of the cases. Ketosis was more often found in neonates nourished by breast feeding (33 out of 47). Over half of the neonates showing ketosis (28 out of 47) were asymptomatic. When normal neonates and neonates testing positive for ketosis were compared, no statistically significant correlations were found with regard to birth mass, gestational period, or gender. However, neonates with ketosis tended to have low mass gain rates in the 5 days from birth and a statistically significant difference was found in this regard in comparison to normal neonates (P<0.0001). From the above results, development of ketosis in neonates was found to be possible even in normal subjects. Most ketosis in neonates was also found to depend largely on nourishment after birth. Existence of an asymptomatic ketosis category was also suggested. 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 >>

Babies Thrive Under A Ketogenic Metabolism

Babies Thrive Under A Ketogenic Metabolism

Some people, even some scientists who study ketogenic metabolism, have the idea that ketogenesis is somehow abnormal, or exceptional; an adaptation for emergencies only. We disagree. One reason we think a ketogenic metabolism is normal and desirable, is that human newborns are in ketosis. Despite the moderate sugar content of human breast milk, breastfeeding is particularly ketogenic. This period of development is crucial, and there is extensive brain growth during it. Although the composition of breast milk can be affected by diet [1], it is reasonable to assume that breast milk has always been ketogenic, and this is not an effect of modernisation. When the brain is in its period of highest growth, and when the source of food is likely to be close to what it evolved to be for that period, ketones are used to fuel that growth. If nothing else, this suggests that learning is well supported by a ketogenic metabolism. It is also consistent with the ability of ketogenic diets to treat a variety of seemingly unrelated brain disorders and brain trauma. Newborn infants are in ketosis. This is their normal state. Breastfeeding is particularly ketogenic (compared to formula feeding). Breastfeeding longer (up to a point) is associated with better health outcomes. This suggests the hypothesis that weaning onto a ketogenic diet would be healthier than weaning onto a high-carb diet. (Mark-up ours) Human babies are in ketosis Soon after birth, human babies are in ketosis, and remain so while breastfeeding [2]. They use ketones and fats for energy and for brain growth. When this has been studied, in the first couple of hours after birth, babies aren't immediately in ketosis. There is a short delay [3]. During that brief period before ketogenesis starts, lactate (confusingly not to do Continue reading >>

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 >>

Abstract

Abstract

Ketone Body Transport in the Human Neonate and Infant P. F. Bougneres, C. Lemmel, P. Ferre, and D. M. Bier U 188 Institut National de la Sante et de la Recherche Medicale, Pediatric Endocrinology, Hbpital Saint Vincent de Paul, 7S014 Paris; Centre de la Nutrition du Centre National de la Recherche Scientifique, Meudon-Bellevue; Metabolism Division, Washington University School of Medicine, St. Louis, Missouri 63110 Abstract Using a continuous intravenous infusion of D-(-)-3-hy- droxyl4,4,4_2H31butyrate tracer, we measured total ketone body transport in 12 infants: six newborns, four 1-6-mo-olds, one di- abetic, and one hyperinsulinemic infant. Ketone body inflow-out- flow transport (flux) averaged 17.3±1.A Mmol kg-' min' in the neonates, a value not different from that of 20.6±0.9 !mol kg-' min' measured in the older infants. This rate was accelerated to 32.2 jAmol kg-' min' in the diabetic and slowed to 5.0 gsmol kg-' min-' in the hyperinsulinemic child. As in the adult, ketone turnover was directly proportional to free fatty acid and ketone body concentrations, while ketone clearance declined as the cir- culatory content of ketone bodies increased. Compared with the adult, however, ketone body turnover rates of 12.8-21.9 jsmol kg-' min' in newborns fasted for <8 h, and rates of 17.9-26.0 gsmol kg-' min-' in older infants fasted for <10 h, were in a range found in adults only after several days of total fasting. If the bulk of transported ketone body fuels are oxidized in the infant as they are in the adult, ketone bodies could account for as much as 25% of the neonate's basal energy requirements in the first several days of life. These studies demonstrate active ketogenesis and quanti- tatively important ketone body fuel transport in the human infant. Furthermor 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 >>

Ketosis In An Evolutionary Context

Ketosis In An Evolutionary Context

Humans are unique in their remarkable ability to enter ketosis. They’re also situated near the top of the food chain. Coincidence? During starvation, humans rapidly enter ketosis; they do this better than king penguins, and bears don’t do it at all. Starvation ketosis Humans maintain a high level of functionality during starvation. We can still hunt & plan; some would even argue it’s a more finely tuned state, cognitively. And that’s important, because if we became progressively weaker and slower, chances of acquiring food would rapidly decline. Perhaps this is why fasting bears just sleep most of the time: no ketones = no bueno..? Animals with a low brain/carcass weight ratio (ie, small brain) don’t need it. Babies and children have a higher brain/carcass weight ratio, so they develop ketosis more rapidly than adults. Is this a harmful process? No, more likely an evolutionary adaptation which supports the brain. The brain of newborn babies consumes a huge amount of total daily energy, and nearly half comes from ketones. A week or so later, even after the carbohydrate content of breast milk increases, they still don’t get “kicked out of ketosis” (Bourneres et al., 1986). If this were a harmful state, why would Nature have done this? …and all those anecdotes, like babies learn at incredibly rapid rates… coincidence? Maybe they’re myths. Maybe not. Ketosis in the animal kingdom Imagine a hibernating bear: huge adipose tissue but small brain fuel requirement relative to body size and total energy expenditure. No ketosis, because brain accounts for less than 5% of total metabolism. In adult humans, this is around 19-23%, and babies are much higher (eg, Cahill and Veech, 2003 & Hayes et al., 2012). For the rest of this article and more, head over to Pat Continue reading >>

Interventions For Ketosis During Labour

Interventions For Ketosis During Labour

Physical stress compounded by reduced food intake during labour can lead to raised levels of ketones in the blood and urine (ketosis). Ketone bodies transport fat-derived energy from the liver to other organs to provide an alternative source of energy. They also cross the placenta and the effect of ketosis on mother and baby is not clear. It is not clear whether ketosis during labour is a normal physiological response, or if women with ketosis in labour require intervention (such as intravenous and oral fluids). This uncertainty has resulted in differences in opinion and practice. Adverse effects of ketosis for the mother include increased likelihood of augmentation of labour, forceps-assisted delivery and postpartum blood loss. Yet intravenous therapies can have adverse effects, either by interfering with glucose and insulin levels for the mother and infant (causing neonatal hypoglycaemia) or lowering sodium level, for example. Other reported adverse effects include headache, nausea, maternal fluid overload, slowing of labour and difficulty in establishment of breastfeeding, as well as local pain and discomfort and interference with the woman's freedom of movement in labour. The newborn may have acidic blood and increased lactate levels. This review found no information on which to base practice in the treatment of women with ketosis during labour. The authors looked for studies comparing oral intake or intravenous fluids with no intervention (defined as no oral intake, ice chips only or oral intake on demand) and pregnancy outcomes. The only six studies identified focussed on maternal biochemical measures during or shortly after labour and could not be included in the review. The studies were conducted in the late 1970s to mid-1980s. Future trials should examine the u Continue reading >>

Neonatal Ketosis

Neonatal Ketosis

Adam, P. A., Räihä, N., Rahiala, E. L., & Kekomäki, M. (1975). Oxidation of glucose and D-B-OH-butyrate by the early human fetal brain. Acta paediatrica Scandinavica, 64(1), 17–24. Retrieved from The isolated brains of 12 previable human fetuses obtained at 12 to 21 weeks’ gestation, were perfused through the interval carotid artery with glucose (3 mM) and/or DL-B-OH-butyrate (DL-BOHB), 4.5 MM, plus tracer quantities of either glucose-6-14C (G6-14C) or beta-OH-butyrate-3-14C (BOHB3-14C). Oxidative metabolism was demonstrated by serial collection of gaseous 14CO2 from the closed perfusion system, and from the recirculating medium. Glucose and BOHB were utilized at physiological rates as indicated (mean plus or minus SEM): G6-14C at 0.10 plus or minus 0.01 mumoles/min g brain (n equal 7) or 17.5 plus or minus 1.9 mumoles/min kg fetus; and BOHB3-14C at 0.16 plus or minus 0.05 mumoles/min g (n equal to 5) or 27.3 plus or minus 7.4 mumoles/min kg. Based on fetal weight, glucose metabolism by brain apparently accounted for about 1/3 of basal glucose utilization in the fetus. On a molar basis BOHB3-14C was taken up at 1.47 times the rate of G6-14C. Both BOHB3-14C and G6 14C were converted to 14CO2. The rate of BOHB3-14C conversion to 14CO2 was equal to its rate of consumption, and exceeded the conversion of glucose to CO2 because 45% of the G6-14C was incorporated into lactate-14C. Accordingly, both substrates support oxidative metabolism by brain; and BOHB is a major potential alternate fuel which can replace glucose early in human development. Bon, C., Raudrant, D., Golfier, F., Poloce, F., Champion, F., Pichot, J., & Revol, A. (n.d.). [Feto-maternal metabolism in human normal pregnancies: study of 73 cases]. Annales de biologie clinique, 65(6), 609–19. Retrieved fr 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 >>

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