Ketogenic Diet Therapy for Epilepsy Associated With Aicardi Syndrome.

INTRODUCTION: Aicardi syndrome is a rare neurodevelopmental disorder associated with epilepsy in females. Ketogenic diet therapy represents a possible nonpharmacologic treatment in Aicardi syndrome patients. METHODS: All patients with Aicardi syndrome seen at Johns Hopkins Hospital (Baltimore, MD) and Johns Hopkins All Children's Hospital (St Petersburg, FL) treated with ketogenic diet therapy since 1994 were evaluated retrospectively. RESULTS: Fifteen patients, ages 4 months to 34 years, were identified. Ten (67%) patients experienced a ≥50% seizure reduction after 3 months, with 3 (20%) having a ≥90% reduction. Only 1 patient was seizure-free for a short period of time. The number of drugs tried prior to ketogenic diet therapy initiation was correlated with ≥50% seizure reduction at 3 months, 5.8 vs 2.6 in responders versus nonresponders (P = .01). In addition, the mean number of drugs actively received also correlated, 3.0 vs 1.2, P = .005. Ketogenic diet therapy was slightly more successful in those without infantile spasms, 78% vs 50%, P = .33. CONCLUSION: Ketogenic diet therapy was helpful in Aicardi syndrome, although seizure freedom was rare. It was especially helpful for those who were more drug-resistant and did not have infantile spasms at ketogenic diet therapy onset.

Enteral Nutrition Improves Vital Signs in Children With Bronchiolitis on Noninvasive Ventilation.

OBJECTIVES: In children hospitalized for bronchiolitis, enteral nutrition (EN) practices during noninvasive ventilation (NIV) vary widely. We sought to assess the potential impact of EN by observing changes in physiometric indices (heart rate [HR] and respiratory rate [RR]) before and after EN initiation. METHODS: We performed a retrospective cohort study in children <2 years of age hospitalized for bronchiolitis receiving NIV from 2017 to 2019 in a quaternary ICU. The primary outcome was patient HR and RR before and after EN initiation. Descriptive data included demographics, anthropometrics, comorbidities, NIV parameters, EN characteristics, and general hospital outcomes. Analyses included paired comparative and descriptive statistics. RESULTS: Of the 124 children studied, 85 (69%) were permitted EN at a median of 12 (interquartile range [IQR]: 7 to 29) hours. The route was oral (76.5%), nasogastric (15.3%), or postpyloric (8.2%) and was predominantly started during high-flow nasal cannula (71%) at flow rates of 1 (IQR: 0.7 to 1.4) L/kg per minute. After EN initiation, reductions in the median RR (percentage change: -11 [IQR: -23 to 3]; P < .01) and HR (percentage change: -5 [IQR: -12 to 1]; P < .01) were noted. Those permitted EN were younger (5 [IQR: 2 to 11] vs 11 [IQR: 3 to 17] months; P < .01) and more likely to have bronchopulmonary dysplasia (19% vs 5%; P = .04). Malnutrition rates, comorbidities, admission timing, flow rates, length of stay, and NIV duration did not differ for those provided or not provided EN. No aspiration events were observed. CONCLUSIONS: Reductions between pre- and postprandial RR after EN initiation among children hospitalized for bronchiolitis on NIV were observed without clinically significant aspiration. These findings support existing data that suggest that EN is safe during NIV and may lessen distress in some patients.

Ketogenic diet therapy provision in the COVID-19 pandemic: Dual-center experience and recommendations.

The current coronavirus-19 pandemic has changed dramatically how neurologists care for children and adults with epilepsy. Stay-at-home orders and resistance to hospitalizations by patients have led epileptologists to engage in telemedicine and reevaluate how to provide elective services. Ketogenic diet therapy is often started in the hospital, with families educated in hospital-based classes, but this is difficult to do in this current pandemic. At our two academic centers, both our pediatric and adult epilepsy diet centers have had to quickly consider alternative methods to both start and maintain ketogenic diet therapy. This paper provides several examples of how ketogenic diet therapy can be provided to patients in unique ways, along with recommendations from other experts and patients, learned over the past few months.

Transcutaneous carbon dioxide measurements in fruits, vegetables and humans: A prospective observational study.

BACKGROUND: Transcutaneous carbon dioxide measurement (TcCO2) is frequently used as a surrogate for arterial blood gas sampling in adults and children with critical illness. Data from noninvasive TcCO2 monitoring assists with clinical decisions regarding mechanical ventilation settings, estimation of metabolic consumption and determination of adequate end-organ tissue perfusion. OBJECTIVES: To report TcCO2 values obtained from various fruits, vegetables and elite critical care medicine specialists. DESIGN: Prospective, observational, nonblinded cohort study. SETTINGS: Single-centre, tertiary paediatric referral centre and organic farmers' market. PARTICIPANTS: Vegetables and fruits included 10 samples of each of the following: red delicious apple (Malus domestica), manzano banana (Musa sapientum), key lime (Citrus aurantiifolia), miniature sweet bell pepper (Capsicum annuum), sweet potato (Ipomoea batatas) and avocado (Persea americana). Ten human controls were studied including a paediatric intensivist, a paediatric inpatient hospital physician, four paediatric resident physicians and four paediatric critical care nurses. INTERVENTIONS: None. MAIN OUTCOME MEASURES: TcCO2 values for each species and device response times. RESULTS: TcCO2 readings were measurable in all study species except the sweet potato. Mean ±â€ŠSD values of TcCO2 for human controls [4.34 ±â€Š0.37 kPa (32.6 ±â€Š2.8 mmHg)] were greater than apples [3.09 ±â€Š0.19 kPa (23.2 ±â€Š1.4 mmHg), P < 0.01], bananas [2.73 ±â€Š0.28 kPa (20.5 ±â€Š2.1 mmHg), P < 0.01] and limes [2.76 ±â€Š0.52 kPa (20.7 ±â€Š3.9 mmHg), P < 0.01] but no different to those of avocados [4.29 ±â€Š0.44 kPa (32.2 ±â€Š3.3 mmHg), P = 0.77] and bell peppers [4.19 ±â€Š1.13 kPa (31.4 ±â€Š8.5 mmHg), P = 0.68]. Transcutaneous response times did not differ between research cohorts and human controls. CONCLUSION: We found nonroot, nontuberous vegetables to have TcCO2 values similar to that of healthy, human controls. Fruits yield TcCO2 readings, but substantially lower than human controls.

How often is antiseizure drug-free ketogenic diet therapy achieved?

The ketogenic diet (KD) is often started not only for seizure reduction but also to potentially wean antiseizure drugs (ASDs) in children with epilepsy. Although there have been several publications regarding ASD reduction on the KD, it is unknown how often complete medication withdrawal occurs. We reviewed the charts of all children started on the KD at Johns Hopkins Hospital and Johns Hopkins All Children's Hospital from 1/11 to 4/18. Children were defined as achieving drug-free diet (DFD) status if they started the KD on at least 1 ASD and achieved a period of time where they were on the KD alone. Over the time period, 232 children were evaluated; DFD status occurred in 43 (18.5%), of which 32 (13.8% of the full cohort) remained off ASDs for the remainder of their KD treatment course. Eleven children restarted ASD after a mean of 7 months. Children achieving DFD therapy were more likely to be younger, have fewer ASDs at KD onset, have Glut1 deficiency or epilepsy with myoclonic-atonic seizures, but were less likely to have Lennox-Gastaut syndrome or a gastrostomy tube.

A Proof-of-Principle, Case-Control Study to Compensate for Potential Carbohydrates in Liquid Antiseizure Drugs in Children on the Ketogenic Diet.

INTRODUCTION: Since its creation, patients on ketogenic diet are told to avoid liquid medications due to theoretical concerns of "hidden" carbohydrates. However, switching from liquid to tablet formulations can be problematic, especially for infants and young children. We theorized that increasing the daily ketogenic ratio might compensate for liquid antiseizure drug carbohydrates. METHODS: Two tables were created (for 3:1 and 4:1 ketogenic ratios), with variables including daily volume of antiseizure drugs and calories. Cases were those who had their ratio increased and liquid medications continued. Children already on tablet formulations, emergency situations, were primarily those included as controls. RESULTS: From May 2016 through August 2018, 59 children (33 cases and 26 controls) ages 0.3-14 years were started on the classic ketogenic diet. Compensated antiseizure drugs most commonly included levetiracetam, clobazam, and valproate (mean volume 16 mL/d (range: 3-62 mL/d)). Adjusted ratios for younger children and infants on a 3:1 diet ranged from 3.1 to 3.5:1 and older children on a 4:1 diet from 4.2 to 4.7:1. There was no difference between cases and controls in achieving large ketosis (76% vs 77%), weight gain (1.4 vs 1.2 kg), 1 month >50% seizure reduction (52% vs 50%), or >90% seizure reduction (30% vs 35%). Four (12%) cases had zero or small urinary ketosis, which improved when antiseizure drugs were switched to tablets or discontinued. CONCLUSIONS: This proof-of-principle study demonstrates feasibility of compensating for carbohydrates in liquid medications by increases in the daily ketogenic ratio. This ratio adjustment protocol may help ease an already complex adjustment to dietary therapy.

Use of dietary therapies amongst patients with GLUT1 deficiency syndrome.

PURPOSE: GLUT-1 deficiency syndrome (GLUT1DS) is a neurologic disorder manifesting as epilepsy, abnormal movements, and cognitive delay. The currently accepted treatment of choice is the classic 4:1 ratio ketogenic diet. METHODS: A 2-page survey was distributed to all attendees of a family-centered conference for GLUT1DS in July 2015. The surveys were completed by parents, collected anonymously, and information analyzed in a database. RESULTS: Surveys were received from 92 families, of which 90 (98%) had been treated with dietary therapies. Diets used were extremely varied: 59 were treated with the classic ketogenic diet (KD), 29 with the Modified Atkins Diet (MAD), 4 with the Medium-chain Triglyceride (MCT) Diet and 2 with the low glycemic index treatment. The mean diet duration was 5.5 years (range: 1 month-20 years). Of those with seizures, 95% of the children had >50% seizure reduction and 80% had >90% seizure reduction. Children who were seizure-free were currently younger on average (8.2 vs. 11.6 years, p=0.01) and slightly younger at GLUT1DS diagnosis (3.8 vs. 5.3 years, p=0.05). There was an equal percentage of children seizure-free receiving the KD/MCT Diets compared to the MAD/Low Glycemic Index Treatment (74% vs. 63%, p=0.30). The majority (64%) were not receiving anticonvulsants. CONCLUSION: This represents the largest series of KD experience in children with GLUT1DS. Nearly all patients surveyed were on dietary therapies for long durations with reported excellent seizure control, often without anticonvulsant drugs. Several different ketogenic diets were utilized with similar efficacy. Early diagnosis and treatment were correlated with success.