Children healthcare with specifics of nutrition and maintenance of health
BruceMwaenga
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184 slides
Sep 20, 2024
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About This Presentation
About paediatric care
Size: 12.15 MB
Language: en
Added: Sep 20, 2024
Slides: 184 pages
Slide Content
Growth and physical
development
Professor
Ledyaev
Mikhail Yakovlevich
Age periods of childhood
•Preparatory period
•Intrauterine (prenatal) life
•Extrauterine (postnatal) life
Preparatory period
•Genetic forming period
•Forming of somatic & reproduction
parents’ health
•Time before conception
Intrauterine (prenatal) life
•Embryo (first 75 days)
•Fetus (from 76 day to delivery)
Extrauterine (postnatal) life
•Neonatal infant –first month after birth
•Infant –1-12 mo
•1-3 years baby
•Preschool child –3-6 years
•Early school years –7-11 years
•Teenager (adolescence) –12-18
years
•Neonatal period
•early -from the moment of birth to 7 days
•late -from 7 to 28 days of life
•adaptationofthenewborntoextrauterinelife
occurs;
•theformationofconditionedreflexesbegins,
activephysicalandneuropsychic
developmentoccurs
•Breast age (28 days -1 year)
•the adaptation of the child to
extrauterine life ends;
•high metabolic rate with a
predominance of anabolic
processes;
•intensive physical, neuropsychic
and intellectual development
•Preschool period (3 -7 years)
•decrease in the rate of physical
development;
•maturation of organs and systems
reaches a high degree;
•CPD is improved, individual interests
are formed, gender differences in
behavior (period of imitation of adults)
•Early school years 7 -11 years old
•maturation continues with clear
sexual demorphism;
•the acquired skills are stabilized;
•Puberty 12 -18 years old
•intense puberty;
•significant acceleration of physical
development, secondary sexual
characteristics appear;
•ends the formation of character and
personality
•One of the major challenges facing
pediatricians is that the range of
body sizes (and weights) that they
face in pediatrics is much greater
than in adult medicine
•Childhood is the period of greatest
growth, development and
maturation of the various organ
systems in the body. Years of
training and experience (above
and beyond basic medical
training) goes into recognizing the
difference between normal
variants and what is actually
pathological
•Growth is a normal
process of increase
in size;
•Physical development is
a normal process of
growth and differentiation
(progressive change in
function and/or
morphology).
•Growth and physical
development are
multifaceted processes
involving genetic,
nutritional, and
environmental (physical and
psychological) factors
EVALUATION OF THE PHYSICAL GROWTH
The physical growth consist on:
•morphological & functional conditions
due to the biological age;
•increasing of weight & height in
progress
.
EVALUATION OF THE PHYSICAL GROWTH
•The basic regulative factors of
fetal growth are placenta’s blood
flow (perfusion), growth peptides &
hormones, secreted by placenta
(somatomammotropin), insulin.
EVALUATION OF THE PHYSICAL GROWTH
•
After birth –the first 5 yr
thyroid hormones works
(thyroxin), & than also in
adolescence (stimulate
osteogenesis
).
EVALUATION OF THE PHYSICAL GROWTH
•Somatotropin, secreted by hypophysis, has
anabolic effect, can aid bone, muscles &
internal organ’s growth, stimulate
hondrogenesis; maximum works from
3 to 11 yr.
•Androgens works in adolescence –
increased muscles volume, hondroplastic
growth of bones; besides, aid to close the
growth zones of bones, to stop the growth.
The main principles of growth are:
•decreasing of growth rate due to
the age (or growth rate is
inversely to age).
•absence of equability in growth
velocity during childhood. It’s
called “growth jump”, first from 4
yr to 8 yr, second at
adolescence.
The main principles of growth are:
•cranio-caudal gradient of growth
velocity –distal parts of human
body have increased growth
velocity in dependence to
proximal parts, as a result –the
equation body’s proportions
during the childhood.
•The extremities grow at a
faster rate than the trunk,
leading to a gradual change in
relative proportions. The
crown-to-pubis/pubis-to-heel
ratio is 1.7 at birth, 1.5 at 1 yr,
1.2 at 5 yr, and 1 at 12 yr.
•Height at 5 yr is about double birth
length.
1,7
1,0
1,0
1,0
The crown-to-pubis/pubis-to-heel ratio
The main principles of growth are:
•sex dependence of growth rates –
weight & height rates are more high
in male than in female.
•asymmetry of growth –in right
handed person right leg & arm are
more longer, twins organs sizes
(lungs, kidneys, gonads) are differ.
Growth determinant factors are:
•genetic –more than 100 genes determine
the growth rate
•nutrition status –protein deficit caused
decreasing of height,
•physical activity (excess of physical
exercises may caused the interruption of
height),
•emotional conditions of child,
•acute & chronic diseases.
EVALUATION OF height
Two distinct patterns
of growth
1.From birth to about age 2 yr
2.From about 2 yr to the onset
of puberty
Velocity of linear growth
(height) in boys and girls
•Typically, the infantincreases in
length about 30% by age5 mo and
>50% by age 1 yr
•Linear growth continues to slowly
decrease until thepuberty
Height and age equivalents in boys and girls.
Velocity of linear growth (height) in
boys and girls in cm/yr
EVALUATION OF weight
Age3 мес.
weight–6 кg
height–60 cm
50 perc.
50 perc.
Percentiles of height and weight for boys 0-36 months
Weight
•The infant doubles his birth
weight by 5-6 mo of age
•The infant triples his birth
weight by 1 yr, and almost
quadruples it by 2 yr.
•Between ages 2 and 5 yr, the
annual increments are fairly
similar. Subsequently, yearly
increments increase slowly
until the onset of puberty
Calculation of weight gain
Approximate
(g) Monthly
Weight Gain
Approximate
Daily Weight
Gain
Age
600
800
800
30 g0-3 m
750
700
650
20 g4-6 m
600
550
500
15g7-9 m
450
400
350
12 g
10-12 m
Nutritional
insufficiency
•Istage –10-20%
•I Istage–20-30%
•I I Istage–>30 %
Normal
Body mass index
W (kg)
BMI=----------------
H (m)²
Obesity
BMI-for-age weight status
categories
Percentile RangeWeight Status
Category
Less than the 5th
percentile
Underweight
5th percentile to less
than the 90th percentile
Healthy weight
90th to less than the
95th percentile
At risk of
overweight
Equal to or greater than
the 95th percentile
Overweight
1. Congenital pathologic short
stature
•These infants are born small and growth
gradually tapers off throughout infancy.
•These babies are born with intrauterine
growth retardation (IUGR), and are small
for gestational age (SGA).
•Examples are chromosomal
abnormalities,
TORCH infections(
TORCHES)
,
teratogens, extreme
prematurity, maternal smoking, etc.
TORCHES infections
•The infectious agents are:
•T–Toxoplasmosis
•O–Other agents, such as Coxsackie
virus, Listeriaand human parvovirus
•R–Rubella
•C–Cytomegalovirus/ Chlamydia
•HE–HErpes simplex virus/ HEpatitis B/
HHV4, i.e. Epstein-Barr virus/ HHV3,
i.e.varicella-zoster virus/ HIV
•S–Syphilis
2. Familial short stature
•Infant and parents are small
•Growth runs parallel to and
just below the normal curves
incorrect
correct
•A handy method to remember about
the occiput-frontal circumference
•The occiput-frontal circumference is:
•Increases 2 cm/month for the first 3
months
•Increases 1 cm/month for the next 3
months
•Increases 0,5 cm/month for the next
6 months
•А4 month old female was born at 39-40
weeks gestation by normal spontaneous
vaginal delivery without any
complications.
•At birth, her weight was 3856 g, length
53 cm, head circumference 34 cm, and
chest circumference 35.5 cm
•Exam: VS are normal. Weight 7.4 kg
(95%tile), length 64 cm (90%tile), head
circumference 43 cm (90%tile), and
chest circumference 41 cm.
http://who.int/growthref/tools/en/
95
90
Organ systems
•The lymphoid,
reproductive, and central
nervous systems do not
follow the general pattern
of growth occurring with
height and weight.
Organ systems
•The lymphoid system grows fairly
constantly and rapidly throughout
childhood, reaching a peak just
before puberty. The mass of
lymphoid tissue subsequently
recedes so that an adult has
approximately 50% that of the
preadolescent
Organ systems
•The reproductive system,
except for a brief time in the
immediate postnatal period,
shows little physical
development until later
childhood and puberty.
Organ systems
•The CNS grows almost
exclusively during the early years
of life. At birth, the brain is 25% of
adult size. By age 1 yr, the brain
has completed half its postnatal
growth and is 75% of adult size.
By age 3 yr, it reaches 80% of
adult size, and by age 7 yr, it is
90%.
•
Organ systems
•Functional development of organs,
independent of organ size, occurs
primarily during the early growth
period with the obvious exception of
the reproduction system. The most
notable changes occur in renal,
immune and CNS functions.
Organ systems
•At birth, renal function is generally
reduced. However, renal acidifying
and concentrating abilities are
functionally similar to those of adults.
By age 1 yr, urea clearance, and
maximum tubular clearances have
reached adult levels.
Organ systems
•CNS functional changes occur largely
and most rapidly during the first 4 to 5
yr of life and are best demonstrated in
the psychomotor and intellectual
development of the child.
Body composition:
•At birth, body fat is about 12% of
body weight. Its proportion
increases rapidly to 25% at 6 mo
and then somewhat more slowly
to 30% at 1 yr, accounting for the
chubby appearance of the 1-yr-
old infant.
Body composition:
•Subsequently, a slow fall occurs until
age 5 to 6 yr, when body fat
approximates that of the newborn.
Then there is again a slow rise until
the onset of puberty. After puberty,
the rise generally continues in girls,
while in boys there tends to be a
slight fall in body fat.
Body composition:
•Body water measured as a
percentage of body weight is 75% at
birth, dropping to 60% at 1 yr
•This change is fundamentally due to
a decrease in ECF from 45% to 28%
of body weight. ICF stays relatively
constant.
Body composition:
•After age 1 yr, there is a slow
and somewhat variable fall in
ECF and rise in ICF to adult
levels of about 16% and 47%,
respectively.
Tanner stages of
female breast development
Tanner stages of female
pubic hair growth
Tanner stages of male
genital development
Children's nutrition
(Breastfeeding, mixed,
artificial feeding)
Prof. M.J.Ledyaev
General Recommendations
for Pregnancy
•No ketogenic diet,
i.e.
–No low
carbohydrate diet,
–No very low kCal
diets
General Recommendations for
Pregnancy
•No sodium restriction just because
of pregnancy
•Prenatal supplements (prescribed)
•No aspirin, alcohol or other drugs
unless prescribed
Nutrients Needed
•Energy = fuel for the body
–Usually 300 additional kcal per day
in 2nd & 3rd trimesters
–Food source: Any of the food
groups
Nutrients Needed
•Protein = building material for
growth and repair of body tissue
–Recommendation: +10 g per day
–Food source: milk, cheese &
yogurt; meat, poultry & fish
Nutrient Needs
•Carbohydrate = for energy
–50% of kCal = 1000 kcal (250 g)
–Food source: bread, starchy
vegetables, fruit, grains
Nutrients Needed
•Folate and Vitamin B
12
= rapid
cell proliferation
–Supplement: 400 g/day
–Food source: green leafy
vegetables, legumes, liver, orange
juice
Nutrients Needed
•Minerals = building the skeleton
–Calcium: 1200 mg/day
–Phosphorus: 1200 mg/day
–Magnesium: 320 mg/day
Nutrients Needed
•Fluoride --mineralization of the
baby’s teeth
–1.5-4.0 mg•Zinc --required for DNA and RNA
synthesis
–15 mg/day
•Iron
–30 mg/day
Breastfeeding Around the World
The Importance of Breastfeeding in
the Developing World
1.Clean water.
2.Milk or formula.
3.Money to buy the milk or
formula.
4.A way to safely store the milk or
formula.
5.Containers for the milk or
formula.
6.Effective cleaning methods for
the containers.
Breastfeeding Around the World:
Economics
Cost as % OF
GNP
Cost Of Breast milk
Substitutes For 1 Year
Country
3%$778USA
19%$120China
257%$900Haiti
GNP: gross national product
Present Breastfeeding
Recommendations
The World Health Organization
recommends that:
•all infants should be breast-fed
up to two years of age with the
addition of appropriate solid
foods at six months of age.
The American Academy of
Pediatrics Recommends:
•Continued breast feeding for up
to at least twelve months of age
and thereafter as long as
mutually desired with the addition
of supplemental foods at four to
six months of age
Ten Steps to Successful
Breastfeeding
(WHO, 1989)
1. Have a written breastfeeding policy
that is routinely communicated to all
health care staff.
2. Train all health care staff in skills
necessary to implement this policy.
3. Inform all pregnant women about
the benefits and management of
breastfeeding.
Ten Steps to Successful
Breastfeeding
(WHO, 1989)
4. Help mothers initiate
breastfeeding within a half-hour
of birth.
5. Show mothers how to
breastfeed and how to maintain
lactation even if they should be
separated from their infants.
Stimulation of a lactation
Ten Steps to Successful
Breastfeeding
(WHO, 1989)
6. Give newborn infants no food or
drink other than breast milk unless
medically indicated.
7. Practice rooming in: Allow mothers
and infants to remain together 24
hours a day.
Practice rooming in
8. Encourage breastfeeding on demand.
Ten Steps to Successful
Breastfeeding
(WHO, 1989)
9. Give no artificial teats or pacifiers,
also called dummies or soothers to
breastfeeding infants.
10. Foster the establishment of
breastfeeding support groups and
refer mothers to them on discharge
from the hospital or clinic.
Baby Friendly Hospitals
•A baby friendly hospitalis one
that meets all the requirements of
the «Ten Steps to Successful
Breastfeeding» as designated by
WHO. The baby friendly hospital
initiative was launched in 1992,
and is now operating in 134
countries.
Baby Friendly Hospitals
Today there were more than 15,000
hospitals around the world that
had been certified as baby
friendly, in Russia –200 and in
Volgograd region –89.
Statistics on Breastfeeding
Around the World
Percentage of infants (aged 0-4
months) exclusively breastfed
Region
34%Africa
46%South Asia
78%Volgograd
44%World
Breast development
•A woman's breasts grow during
puberty in response to
hormones: prolactin, estrogen,
progesterone, cortisol, insulin,
thyroid hormones and growth
hormone
Tanner stages of
female breast development
Breast development
•During pregnancy the release of
estrogen and progesterone from the
placenta and prolactin from the
adenohypophysis causes the breast
development.
•Breasts increase in size due to an
increase in lobules and alveoli.
Breast development
•Both male and female infants may
have palpable breast tissue at birth.
•5%of infants had galactorrhea
•The galactorrhea was most likely to
be present in the first two weeks of
life
•By two to three months of age the
breast tissue regresses.
Hormones involved in breast
development and breastfeeding
•Prolactin
is produced by the
adenohypophysis.
•Oxytocin
is produced by the
neurohypophysis.
•insulin, cortisol, thyroid hormone,
parathyroid hormone, parathyroid
hormone-related protein, and human
growth hormone.
Milk production
•Milk productionis initiated in the
breasts in the post-partum period
due to prolactin production and
decreased estrogen and
progesterone after delivery of the
placenta
Milk production
•By day 3 or 4 post-partum,
stimulation of the breast by
suckling is required to continue
milk production.
•During continued lactation, milk
production is based on infant
demand
Milk Composition
•Colostrum
is a thick,
yellowish milk that is secreted
by a woman's breast in the first
several days after delivery. It
has increased concentration of
calcium, potassium, proteins,
fat-soluble vitamins, minerals
and antibodies.
Colostrum
•The volume is approximately
100 cm
3
in a 24-hour period.
Due to its high concentration
of antibodies, this milk is
particularly valuable for
infants in preventing
infection.
Milk Composition
•Transitional milk
is secreted
between about four days and ten
days postpartum. It is
intermediate in composition in
between colostrum and mature
milk. The volume increases
during this time.
Mature Milk Components
Mature Milk Components
1.
Energy
(750 kcal / liter)
2.
Lipids
(38 g / liter) -The main
lipids found in human breast milk are
the triacyl-glycerols, phospholipids,
and fatty acids including essential fatty
acids.
Maternal diet does not affect the amount
of fat in milk but does affect the types
of fat. Cholesterol is present in breast
milk.
Mature Milk Components
3.
Whey
(7.4 g / liter) -protein -
the whey proteins are located in
the clear liquid left behind when
clotted milk stands. The largest
components are alpha-
lactalbumen, lactoferrin,
lyzozyme, albumen and
immunoglobulins.
Mature Milk Components
4.
Casein
(2.5 g / liter) -protein -
Casein or curds are proteins with
low solubility which complex with
calcium. These are present in
breast milk in much lower
concentration than in cow's milk.
Mature Milk Components
5. Nonprotein Nitrogenis used in amino acid
synthesis and includes the nitrogen in urea,
creatine, creatinine, uric acid and ammonia.
Peptides, such as epidermal growth factor,
somatomedin -C and insulin are also present
in this fraction.
Nucleotides such as cytidine monophosphate are
derived from nucleic acids and play an
important role in the immune system and
protein synthesis.
Mature Milk Components
6.
Lactose(70 g / liter) carbohydrate -
Lactose is the major carbohydrate
in breast milk. It is composed of
galactose and glucose. Lactose
concentration in breast milk
increases over the duration of
breastfeeding.
Mature Milk Components (minerals):
•Sodium, potassium, calcium
and magnesiumare the major
cations in human milk
•Ironis necessary for
hemoglobin formation.
Mature Milk Components (minerals):
•Zincis found in human milk and is
necessary for enzyme production
and activation
•Copper, selenium, chromium,
manganese, molybdemunand
nickelare present in small amounts
in breast milk.
Mature Milk Components (Vitamins
)
•Vitamin Kis a fat-soluble vitamin
necessary for blood coagulation
•Vitamin Dis a fat soluble vitamin
necessary for bone development.
Infant
Formulas
Cow's milk Human
breast milk
Ingredients
1,5-2,03,3-3,50,9-1,3protein g/dL
60/4020/8070/30The
whey:casein
ratio
4,4-6,03,2-3,53,9-5,7Lipid g/dL
YesNoYesEssential
fatty acids
LactoseLactoseLactosecarbohydrate
Supplements for breastfed babies
•1.All infants should receive
a Vitamin K supplement in
the immediate postpartum
period.
Supplements for breastfed babies
•2.Supplementation with
Vitamin D is recommended
particularly for infants of women
with a dark complexion in areas
where sunshine is limited, or for
women who take little milk in
their diet.
Supplements for breastfed babies
•3.
Women who are
breastfeeding should
continue their prenatal
vitamins for the vitamin D,
calcium and iron that they
supply.
Supplements for breastfed babies
•4.Once infants reach six
months of age supplemental
foods should be added.
These should include foods
rich in iron.
Breastfeeding: The Advantages
Antibodies passed from a nursing mother
to her baby can help lower the
occurrence of many conditions,
including:
•ear infections
•diarrhea
•respiratory infections
•meningitis
Breastfeeding: The Advantages
Breastfeeding is particularly beneficial for
premature babies and may also protect
children against:
•allergies
•asthma
•diabetes
•obesity
•sudden infant death syndrome (SIDS)
Breastfeeding: The Advantages
Obesity prevention
Recent studies indicate that breastfeeding might
help prevent childhood and adult obesity
Smarter babies
Recent studies suggest that children who were
exclusively breastfed for 6 months have IQs 5
to 10 points higher than children who were
formula fed.
Reasons not to Breastfeed
•1.
Maternal death,
•2.Maternal HIV infection (in
some situations),
•3.Infectious tuberculosis in
the developed world
•4.Maternal use of illegal
drugs.
The Breastfeeding Couple:
Positioning of Infants
•theside lying position
•thefootball position
•the cradle position
•cross cradle position
The cradle position
Thefootball position
Some signs of thrush for mothers(1):
1.Red nipples or areola.
2.Itchy nipples, peeling skin.
3.Sore nipples that don’t respond to
basic treatment.
4.Nipples that remain sore
throughout the feeding (for no
apparent reason)
Some signs of thrush for mothers(2):
5.Burning or shooting pain in the
breast during or after a feeding.
6.Repeated breast infections.
7.Cracked nipples that do not
heal.
8.History of repeated vaginal
yeast infections.
Some signs of thrush for babies:
1.Baby has white spots in mouth or
white tongue.
2.Diaper rash.
3.Baby is gassy and cranky.
4.Baby repeatedly pulls off the
breast.
5.Inside of baby’s lips have a
mother-of-pearl look.
Some signs of thrush for babies:
Treatment thrush for mom(1)
1.Often, nystatin is prescribed in a
cream form.
2.After each breast-feeding, rinse
your nipples with a solution of
one tablespoon vinegar in one
cup of water. Allow your nipples
to air-dry.
Treatment thrush for mom (2):
3.Apply a small amount of the
nystatin to thenipples, areola, and
any part ofthebreast that comes
in contact withbaby’s lips.
4.Sunbathe thebreasts about 10
minutes twice daily.
Treatment thrush for mom(3):
5.It is best not to use breast
pads.
6.If you are pumping your milk,
feed it toyour baby the same
day.
7.Boil items that come into
contact withthe breast for 20
minutes every day.
Treatment thrush for mom(4):
8.Use chlorine bleach to
wash bras, clothnursing
pads and baby’s diapers.
9.Wash your hands
thoroughly beforeand
after nursing.
The foods which cause yeast to grow:
•Sugar (including artificial sweeteners)
•Yeast breads
•Fermented foods (wine, beer, vinegar)
•Milk, dairy products
•Peanuts, peanut butter
•Grapes, melons, dried fruits
•Mushrooms
The supplements which prevent the
growth of yeast:
•B-complex vitamins
•Garlic (especially raw if you
can)
Treatment thrush for baby
1.Often, nystatin is prescribed in
an oralsuspension.
2.Apply the medicine with afinger.
Treatment thrush for baby
1.Boil things used in your baby’s
mouthfor 20 minutes each day.
2.Wash your hands thoroughly
beforeand after putting medicine
in baby’s mouth.
Mastitis
•A blocked duct
•Generalized
tenderness of
the breast
•Swelling
•Redness
•A fever
•Aches
Breastfeeding & Drugs:
1.Is the medication transferred into
breast milk?
2.If the medication gets into breast
milk, what is the effect on the
infant?
3.Is the medicine routinely given to
infants of this age?
Breastfeeding & Drugs:
1.Is the medication absorbed
when given orally?
2.Can the infant excrete the
medication?
Breastfeeding & Drugs: Illicit drugs
1.All these drugs can cross into breast
milk in varying amounts and be
transferred to the infant.
2.Mothers who use these drugs may
impair their own ability to care for
their infants
Breastfeeding & Drugs: Illicit drugs
1.There is little data on the effect
of some of these drugs on the
infant.
There are indications for the use of
infant formula
1.As a supplement or substitute for
breast milk when a mother cannot or
chooses not to breast-feed
2.Infants whose mothers are infected
with organisms known to be
transmittable by human milk
(e.g., HIV)
3.Infants whose mothers are undergoing
chemotherapy
There are indications for the use of
infant formula
4.Infants whose mothers are
receiving medication or drugs
that are excreted into human
milk
5.Infants who are unable to tolerate
human milk because of metabolic
disorders (e.g., galactosemia).
Formula Feeding: The Advantages
•Breastfeeding is considered the best
nutritional option for babies by the
major medical organizations, but not
every mother chooses -or is able -to
breastfeed. Commercially prepared
infant formulas are a nutritious
alternative to breast milk and even
contain iron.
Formula Feeding
•Time and frequency of feedings.
Because formula digests slower than
breast milk, formula-fed babies
usually need to eat less often than do
breastfed babies.
Formula Feeding
•Expense.
Formula can be costly. Powdered formula
is the least expensive, followed by
concentrated, with ready-to-feed
being the most expensive. And
specialty formulas (i.e., soy and
hypoallergenic) cost more -sometimes
far more -than the basic formulas.
During the first year of life, the cost of
basic formula can run about $1,500.
Formula Feeding
•For a healthy, full-term infant, cow's milk
formula would be the first choice.
•The only indication that I see for soy is for
babies with lactose intolerance.
•Lactoseis the main carbohydrate in milk.
Infants who don't have enough enzyme lactase
to digest may suffer from abdominal pain,
diarrhea, gas, bloating, or cramps. There is no
lactose in soy formula.
Complementary foods
•Complementary food is the stage of a
child's transition from exclusively breast
milk to food from the family table
•Complementary foods are designed to
compensate for the child's increasing need
for energy and nutrients, especially iron
•Complementary food allows the child to
develop cognitive and motor life skills
Complementary foods
•The introduction of a new type of food
should start with one product, gradually
moving to a mixture of two, and then
several products of this group.
Professor M.Y.Ledyaev
Natural History of Bone
Childhood New
Bone Added
Faster than Old
Bone Removed
Peak Bone Mass
Bone Resorption
Exceeds Bone
Formation
Rapid Loss
Menopause 3-6
yrs
From: Kemper, Pediatric Exercise
Science 2000;12:198-216.
M
F
Rickets
Rickets has been
known since
ancient times,
but described in
detail in the mid-
17th century in
England.
Francis Glisson
1597-1677
Definition
The word "rickets" in Greek means
"spine". The disease often manifests
curvature of the spine (the so-called
"rachitic hump")
Rickets
Rickets
What is Rickets ?
Disease of the growing child
Impaired mineralisation of the growth plate & osteoid
Low serum phosphate is fundamental to
pathogenesis of rickets
Н е у д ает с я о т о б р аз и т ь р и с у н ок.Н е у д ает с я о т о б р аз и т ь р и с у н ок.
Normal Growth Plate
Rachitic Growth Plate
Apoptosis of
Hypertrophic
Chondrocytes
caused by
PHOSPHATE ions
HYPOPHOSPHATEMI
A
No Apoptosis of
Hypertrophic
Chondrocytes
1.
Vitamin D Deficiency Rickets
2.
Calcium Deficiency Rickets
3.
Vitamin D Dependent Rickets type I &
type II
4.
X-Linked Hypophosphataemic Rickets
What is Rickets?
Rickets
Vitamin D Related Rickets
-
Vitamin D Deficiency
-Impaired Hepatic 25-hydroxylation
-Impaired Renal 1α-hydroxylation of 25(OH)D
-End organ resistance to 1,25(OH)
2
D
Rickets due to Dietary Calcium Deficiency
Calcipaenic Rickets Phosphopaenic Rickets
Hypophosphataemic Rickets
-
X-linked Dominant (PHEX gene mutation)
-Autosomal Dominant (FGF23 mutation)
-Autosomal Recessive Type 1 (DMP1mutation)
-Autosomal Recessive Type 2 (ENPP1mutation)
-With Hypercalciuria (SLC34A3 gene mutation)
-Associated with:
(a) McCune-Albright syndrome
(b) Tumour induced osteomalacia
(c) Linear nevus sebaceous syndrome
-
Raised PTH
Renal Phosphate Wastage
Hypophosphatemia
Impaired Apoptosis of Terminally Differentiated Chondrocytes in the Growth Plate
Curr Osteoporos Rep. 2011;9(4):291-9
Vitamin D: Who’s Who?
Vitamin D2 = ergocalciferol
Vitamin D3 = cholecalciferol
25(OH)D3 = calcidiol
•
Relatively inactive, very stable
•
Reflects vitamin D status, low in vitamin D
deficiency, longer half-life than other metabolites
•
The one to measure
1,25(OH)D3 = calcitriol
•
‘active’ metabolite, highest affinity + activity at
nuclear VDR, short half-life
•
Concentrations 1000-fold < 25(OH)D
Vitamin D Metabolism
Sunlight and Vitamin D
Melanin: absorbs UVB radiation + competes
with 7-DHC for photons in skin of darkly
pigmented individuals
SPF8: reduces vitamin D
3
production by
97.5%
Latitude: Skin unable to produce any vitamin
D3 at all in Boston: Nov-February (JCEM
1988;67:373-378)
Individuals in extremelatitudes(northern or
southern) may require supplementation
(JCEM 1999;84:1839-1843; J Bone Miner
Res 1993;20:99-108)
FUNCTIONS OF VITAMIN D
Intestine:
Increases calcium binding protein
Active transport in the jejunal cells
Phosphorus ions absorption through
specific phosphate carrier
Alkaline phosphatase (AP) synthesis
ATP-ase sensibility to calcium ions
Liver
25-(OH)D3
Kidney
1,25-(OH)
2
D3
Vitamin D
Dietary
intake
Skin
intestines
PTH
Bone Kidney
UV
Vitamin D Metabolism
Ca
++
blood
Ca
++
Ca
++
2
Calcitonin
Liver
25-(OH)D3
Kidney
1,25-(OH)
2
D3
Vitamin D
Dietary
intake
Skin
intestines
PTH
Bone Kidne
y
UV
Vitamin D Metabolism
Ca
++
blood
Ca
++
Ca
++
2
PTH
Prevalence in Children with Chronic Disease
Inflammatory bowel disease
•
Pediatrics
2006;118(5):1950
Cystic fibrosis
•
Am J Respir Crit Care
Med 1998;157:1892;
Osteoporos Int.
2006;17(5):783-90
Seizure disorders
•
Anticonvulsants,
ketogenic diet
•
Epilepsia
2007;48(1):66-71;
Epilepsy Behav
2004;5 Supp 2:S30
Anorexia nervosa
•
More compliant with
calcium + vitamin D;
lowprevalence
•
Low body fat; more
bioavailable?
Rickets
Emotional lability
Regurgitation
Weakness
Excessive sweating
Alopecia back of the
head
Rickets
Rickets
Hypotonia
Frog stomach
Constipation
Rickets
Rickets
Cranial deformation
Chest deformation
Kyphosis
Deformations of
extremities
Violation of teething
Neurodevelopmental
disability
Rickets
Rickets
Rickets
Rickets
Biomarkers for Vitamin D Sufficiency
25(OH)D
PTH
Bone mineral density (BMD)
Fracture + falls
Intestinal calcium absorption
Blood pressure
Dental health
Insulin sensitivity
Immune function
Respiratory disease, wheezing, TB
Work-up for Vitamin D Insufficiency
Serum 25(OH)D
PTH
Calcium
Magnesium
Phosphorus
Alkaline phosphatase (total)
Urine calcium/creatinine ratio
Start with spot sample
If abnormal, 24-hour sample
Biochemical Changes in Vitamin D Deficiency
Early vitamin D deficiency:
25(OH)D ↓ Ca Normal
PTH ↑P↓
1,25(OH)
2
D ↑ALP↑
Severe vitamin D deficiency:
25(OH)D ↓ ↓ Ca ↓
PTH ↑ ↑ P↓ ↓
1,25-(OH)
2
D ↓ ALP↑↑
Occasionally PTH resistance:Ca ↓, P ↑, 25(OH)D ↓↓,
PTH ↑↑ & 1,25-(OH)
2
D ↓↓
Archives of Disease in Childhood. 2009; 94:932-937
Vitamin D concentration in the
serum (
25(ОН)D
3
1,2
)
•
Norm>30 ng/ml(>75 nmol/l)
Insufficiency20-30 ng/ml(50-75 nmol/l)
Deficiency<20 ng/ml(
<
50nmol/l)
1
Bischoff-FerrariHA,GiovannucciE,WillettWC,etal.Estimationofoptimalserumconcentrationsof25-
hydroxyvitaminDformultiplehealthoutcomes.Am.J.Clin.Nutr.2006;84:18–28.
2
HolickMF.HighprevalenceofvitaminDinadequacyandimplicationsforhealth.MayoClin.Proc.2006(b);
81(3):353–373.
Prevalence of Vitamin D Deficiency
among Healthy Adolescents in Boston
(n=307)
Higher prevalence
•
Winter vs summer
•
Black vs white adolescents
Vitamin D insufficiency
(25OHD <20 ng/mL)
-42%
Gordon et al., Arch Ped Adol Med 2004
Subclinical Vitamin D Deficiency in
Healthy Infants and Toddlers
12% healthy 8-24 month old’s (<20 ng/mL)
40% suboptimal (< 30 ng/mL)
Did not vary by season or race/ethnicity
Significant predictors
•
Breastfeeding without
supplementation
•
Lack of milk consumption
Demineralization (33%) on x-rays
How much is enough?
Guidelines for Vitamin D Intake
Safe upper
limit**
RDA
(recommended
daily allowance)
Age
1000 -1500
IU
400 IU0 -1 yr
2500 IU600 IU1 –3 yr
4000 IU600 IU4 -70 yr
Institute of Medicine 2010
Drugs of vitamin D
Cholecalciferol(Vigantol,
Aquadetrim)
Ergocalciferol(Ergocalciferol)
Armas, L. A. G. et al. J Clin Endocrinol Metab 2004;89:5387-5391
Time course of the rise in serum 25(OH)D after a single oral dose of 50,000 IU of either
cholecalciferol (vitamin D3) or ergocalciferol (vitamin D2) to two groups of 10 normal men
each
Vitamin D
3
or Vitamin D
2
?
Vitamin D Dependent
Rickets (VDDR)
Type I & Type II
VDDR Type I
Ca 2.02 mmol/l
P 0.59 mmol/l(1.1 –2.0)
ALP 3636 IU/l(100 -733)
PTH 1087 pg/ml(10 -60)
25(OH)D 31 ng/ml
1,25(OH)
2
D < 10 pg/ml (20
-50)
16 month old child with severe Rickets
Known inactivating
mutations in
the CYP27B1 gene
Vitamin D Dependent Rickets Type I & Type II
VDDR Type I
Physiological doses of calcitriol (1,25(OH)2D) or
alphacalcidiol
VDDR Type II
Pharmacological doses of calcitriol or
alphacalcidiol (e.g. 3-6 mcg/day)
+
Oral calcium –2 to 3 grams/day
Long-term treatment calcium infusions
(especially patientswith alopecia)
Drugs of vitamin D
Calcitriol(Osteotril, Rocaltrol)
Alfacalcidol(Alfadol, Oxidevitum,
Etalpha)
Treatment of Vitamin D Deficiency
Vitamin D2 or D3: 2000-5000 IU/D or 50,000
IU once weekly
•
provide calcium supps to prevent
“hungry bone”
Malabsorption
•
Larger doses of vitamin D: 10000-
25000 IU/d
Anticonvulsant therapy-vitamin D -800 -
2000 IU/d
Treatment of Vitamin D Deficiency
Impaired production of vitamin D:
calcitriol
•
Liver disease: 25(OH)D or
1,25(OH)
2
D
•
1-hydroxylase deficiency:
1,25(OH)
2
D
Hereditary 1,25(OH)
2
D resistant
rickets-large doses of vitamin D –
treatment is not very effective
How Much is Too Much?
Vitamin D Intoxication
Intoxication: Case series of 8 children with high
vitamin D levels (731 +/-434 nmol/L)
Symptoms hypercalcemia or hypercalciuria
All 8 drank milk from same local dairy
Milk at local dairy had vitamin D concentration
ranging from undetectable to 245840 IU/L
Intoxication only seen at total daily doses of
10000 IU or greater
Jacobus et al. NEJM 1992
Definition of “osteoporosis” in children
No WHO definitions in children and teens
Concern for low bone mass
•
BMD Z-score by DXA <-2.0 SD
•
Slightly low if Z-score between -1.0
and -2.0
“Diagnosis of osteoporosis in children and
adolescents should NOT be made on the
basis of BMD alone.”
Int’l Soc Clinical Densitometry 2007
When should you order DXA scans?
Patients with multiple fractures
Pathologic (atraumatic fractures)
Diseases associated with skeletal
deficiency states
Hypothalamic amenorrhea: after 6 months
of amenorrhea
Be suspicious of low BMD if strong family
history
Repeat scans only annually (except as part
of research protocol)