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Pallister-Killian Syndrome - PKS · PDF filePallister-Killian Syndrome: Medical Nutrition...
Transcript of Pallister-Killian Syndrome - PKS · PDF filePallister-Killian Syndrome: Medical Nutrition...
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Pallister-Killian Syndrome:
Medical Nutrition Therapy (MNT), Nutrition Interventions
Kevan Mellendick, MS, RD
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Best Practices in PKS
• Registered Dietitian (RD) should remain part of
interdisciplinary team
• continuous updates throughout lifecycle
• continuous adjustment based on symptoms of PKS
• medical nutrition therapy to improve outcomes
• continuous adjustments based on unexpected
modalities of PKS, changes in growth pattern over
time
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Nutrition Throughout the
Lifecycle
• Infants- adjust formula/
tube feed for kcal,
protein, micronutrient
needs
• Calcium
• Phosphorus
• Iron
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Nutrition Throughout the
Lifecycle
• Toddlers- introduce new
foods individually
• increase range of
textures
• identify potential
allergies
• promote motor
development
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Nutrition Throughout the
Lifecycle
• Adolescence- balanced diet
with variety
• promote individual choice in
foods
• promote variety for
adequate vitamin/mineral
intake
• promote balance for
appropriate calorie intake
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Nutrition Throughout the
Lifecycle • Adulthood- sustain
positive eating practices
established in
adolescence
• adopt increases in
individual control of diet
where possible
• promote simple cooking
techniques where
possible
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Symptoms of PKS Potentially
Affecting Nutrition
• Developmental Delay
• Walking Difficulties/ Limited Movement/ Brachymelia
• Hypotonia
• Osteopenia, Joint Contractures
• Gastroesophageal Reflux
• Cleft Palate, Delayed Dental Eruption
• Edema
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MNT: Developmental Delay
• RD, parents to work with
physical and/or
occupational therapist
• improve coordination of
sucking, swallowing,
chewing, breathing
• sucking may be
weakened, leading to
fatigue
• Environmental changes-
• regular eating schedule
• 3 meals with set times
• planned snacks
• special meal order at
school may be
necessary
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MNT: Walking DIfficulties, Limited
Movement, Brachymelia
• Decreased activity level
decreases calorie needs
• parent and RD must
coordinate to identify
appropriate calorie
intake
• may require planning
more low fat meals and
snacks, reducing sugar
• Smaller limbs
• height not a good
predictor of calorie
needs
• peripheral muscle mass
likely less than average
• adjust calorie needs
accordingly
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MNT: Hypotonia
• low muscle mass
• high fat mass
• may slow physical motor
development
• decrease activity level
• adjust calorie needs
accordingly
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MNT: Osteopenia, Joint
Contractures • To prevent or delay osteoporosis-
• 175 mg/ 100 calories/ day Calcium
• 91.5 mg/ 100 calories/ day Phosphorus
• add human milk fortifier when breastfeeding
• formula adequate
• consider supplementation in toddler, adolescent
years
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MNT: Gastroesophageal
Reflux
• avoid feeding shortly
before sleep
• avoid large, high fat meals
• thicken feedings for infant
formula, enteral feedings
• low fat diet is often
beneficial
• liquid diet may be
beneficial
• avoiding heavily spiced
foods may be beneficial
• decrease intake of acidic
foods and carbonated
beverages
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MNT: Cleft Palate, Delayed
Dental Eruption
• Surgical repair of cleft
palate normally at 9
months
• tube feed
• concentrate formula
• therapeutic bottle and
nipple designs
• Delayed dental eruption
does not indicate
delaying solid foods
• important for mandible
muscle, motor skill
development
• softer textures, more
chewing
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MNT: Edema
• Swelling can be an issue
• Sodium and water
restriction may be
necessary
• adjust calorie needs to
account for water weight
in edematous tissues
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MNT: Seizures,
Epileptic Events
• Vitamin D supplementation
recommended
• Avoid folic acid
supplementation
• Separate enteral feedings
from medication
administration
• Consider ketogenic diet
• low carbohydrate
• high fat
• 4:1 (fat: carb/protein)
calorie ratio
• high MCT- more
nonketogenic foods OK
• MVI, Calcium supplement
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MNT: Other Complications
• Deal with unique symptoms as they arise
• Endocrinologist may be important-
• thyroid
• Routine evaluation/ reevaluation by interdisciplinary
team
• new or previously unknown symptoms arise routinely
with PKS
• possible postnatal overgrowth syndrome
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Case Study in Temporary
Overgrowth Syndrome
• 30 month old child with moderately severe to severe
symptoms of PKS
• Fed enterally (G button) with monthly F/U with RD
• Very fast growth despite very low calorie intake
• over 100 calories below estimated resting energy
expenditure
• sudden onset, temporary period of overgrowth
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Postnatal Overgrowth
• Be aware of this possibility
• F/U regularly with RD
• monitor growth closely in early childhood
• postnatal overgowth recently noted in Croatian case*
• 21 month old girl
• possible timeframe of 20 months to 36 months?
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Summary
• Work with RD as part of interdisciplinary team
• Evaluate MNT for each symptom, combination of
symptoms
• Continually reevaluate
• Promote new foods, more independence with age as
appropriate
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Thank You for Your Time
• Please feel free to
• ask questions
• comment
• express your concerns
• contact me for my references
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Kevan Mellendick, MS, RD [email protected]
410-627-3247
Please feel free to contact me;
I will be available to speak in private after the
presentation as well