A Manual of Essential Pediatrics-Meharban Singh

A Manual of Essential Pediatrics, Second Edition, the revised and updated edition provides essential state-of-the-art information on childcare right from birth to adolescence. The book serves as a practical guide to pediatricians for the diagnosis and treatment of common disorders and diseases of neonates, children, and adolescents. The author has used his experience of over 50 years to cover core pediatric topics such as growth and development, behavior and developmental disorders, common day-to-day illnesses, immunizations, and nutrition in a simple and succinct manner.

1. Physical growth and mental development of children de-
pend on the interaction between nature (genetic potential
and constitution) and nurture (nutrition, health care, and
a safe and stimulating environment). Children must re-
ceive a well-balanced diet and optimal nutrition to enable
them to achieve their full genetic potential.
Over the years, the defi ciency of macronutrients (car-
bohydrates, fats, and proteins) has decreased with re-
duced incidence of severe protein–energy malnutrition
(PEM) and kwashiorkor because of greater food availabil-
ity, awareness, and improvement in socioeconomic status.
Even severe defi ciency states of single micronutrients (vi-
tamins and minerals) producing scurvy (vitamin C defi -
ciency), rickets (vitamin D defi ciency), beri-beri (vitamin
B
1
defi ciency), pellagra (nicotinic acid defi ciency), and
keratomalacia (vitamin A defi ciency, VAD) have become
rare. However, there is still a widespread prevalence of
diseases of public health importance caused by defi cien-
cies of single micronutrients such as iron-defi ciency ane-
mia, iodine-defi ciency disorders, zinc defi ciency diseases,
and milder forms of VAD. It is also being increasingly re-
alized that there is a widespread prevalence of subclini-
cal or biochemical defi ciencies of certain micronutrients,
which is being referred to as hidden hunger. Children with
defi ciencies of micronutrients are more vulnerable to de-
velop a variety of common day-to-day infections. Infec-
tive illnesses are known to further aggravate nutritional
defi ciencies by causing loss of appetite, tissue catabolism,
enhanced utilization of nutrients, and increased losses of
micronutrients. Acute infections adversely aff ect the nu-
tritional status and make an individual more vulnerable
to contract infection, thus setting up a vicious cycle of un-
dernutrition and recurrent infections.
9.1 Children Are at an Increased Risk of
Developing Nutritional Disorders
Children are more prone to develop nutritional disorders
compared with adults. Nutritional disorders are most
prevalent during fetal life, preschool years, and adoles-
cence. The increased vulnerability to develop nutritional
disorders in children is accounted for by the following fac-
tors.
1. Nutrition during fetal life and early infancy is en-
tirely transmaternal. The general neglect of the girl
children (future mothers) particularly their health
care, nutrition, and education is an important cause
of poor nutrition and overall poor health of children
in India.
2. Children who are dependent on their parents and care takers to look after their nutritional and health
needs. When parents are ignorant or socially disad- vantaged, their children are likely to be undernour- ished and suboptimal in their physical growth and mental development.
3. Children have high energy and nutrient requirements because of their rapid growth velocity and high level
of physical activity. For example, the caloric needs of newborns and infants (120 kcal/kg/d) are about three times that of adults (40 kcal/kg/d).
4. High incidence of low birth weight newborns (< 2.5 kg at birth), delayed weaning, denial of food during ill-
ness, short duration or lack of breastfeeding, and em- phasis on fruit juices and watery soups rather than wholesome weaning foods further compromise their nutritional status.
5. Children have high incidence of gastrointestinal and respiratory infections and a vicious cycle of interac-
tion between undernutrition and infections makes matters worse.
9.2 Epidemic of Hidden Hunger
Over the years, there has been a signifi cant change in the
clinical profi le of nutrition-related disorders in develop-
ing countries. Because of control of fl orid or severe cas-
es of malnutrition, child survival has improved, but the quality of life and human resource development have not improved (Figure 9.1 ). With progressive elimination of
Nutritional Disorders9
FOCUS
HIDDEN HUNGER
Stunting
Impaired coordination, cognition, and
learning capabilities
Biochemical and subclinical
Reduced vigor, lack of enthusiasm, laziness, behavior,
and psychological changes
Beri-beri
Scurvy
Pellagra
Kwashiorkor
Iodine-deficiency disorders
Protein energy malnutrition
Anemia
-
Deaths
Figure 9.1 Pyramid of nutritional disorders.
978-0-xxxx_Theime_Meh_Ch09.indd 90 10/06/13 5:34 PM

2. 9 Nutritional Disorders91
protein–energy defi cits in the diets, availability of micro-
nutrients is getting greater importance for the mainte-
nance of optimal health and well-being of children.
In clinical practice, nutritional defi ciencies are usu-
ally multiple and they occur in various combinations.
Although most cases of micronutrient defi ciencies are
subclinical, there is a growing concern that they do aff ect
growth and development and may adversely aff ect immu-
nocompetence. The lowered immunity leads to increased
risk of catching day-to-day infections, which further ag-
gravates undernutrition and growth faltering.
Gaps in Indian Diets
Country-wide surveys conducted by the National Nutri-
tion Monitoring Bureau (NNMB) of India have shown that
diets of children are more defi cient in micronutrients
than in energy or proteins. About 80 to 90% of children
consume less than 30% of recommended daily allowance
(RDA) of green leafy vegetables. Iron consumption is in-
adequate in 90% of children. About two-thirds of adoles-
cents consume less than 70% of RDA of vitamin A and ri-
bofl avin. Vitamin D defi ciency has assumed public health
importance. It is caused by overcrowding, limited expo-
sure to sunlight, pollution, and smoggy conditions, over-
clothing, dark skin, and the use of sunscreens. Excessive
use of broad-spectrum antibiotics is associated with defi -
ciency of vitamin B complex. The situation is further ag-
gravated because most urban children are fussy or choosy
in their dietary habits. There is a trend for excessive con-
sumption of fast foods that are loaded with trans fats and
are defi cient in micronutrients. In a recent study con-
ducted by the National Institute of Nutrition, Hyderabad,
on apparently healthy school going children belonging to
middle-income families, it was found that more than 50%
of children had subclinical or biochemical defi ciencies of
micronutrients.
9.3 Isolated Micronutrient Defi ciencies
In clinical practice, isolated micronutrient defi ciencies are
rare, and more often multiple micronutrient defi ciencies
occur mostly in subclinical form. Nevertheless, they are an
important cause of suboptimal physical growth and men-
tal development, impaired learning capabilities, reduced
vigor, lack of enthusiasm, laziness, behavior and psycho-
logical disorders, and so on. The well-recognized clinical
syndromes of vitamin C defi ciency (scurvy), thiamine de-
fi ciency (beri-beri), and nicotinic acid defi ciency (pellagra)
are rarely seen. Iron-defi ciency disorders and iodine defi -
ciency that are of public health importance are discussed
in Chapters 39 and 43, respectively. Vitamin A defi ciency,
which is widespread and a leading cause of preventable
blindness, and vitamin D defi ciency, which produces rickets,
a disease peculiar to children, are discussed in detail in
this chapter.
Vitamin A Defi ciency
Vitamin A defi ciency is the leading cause of preventable
blindness in children in developing countries. Globally, it
is estimated that 140 to 250 million children younger than
5 years of age are aff ected by VAD. Vitamin A is also known
as anti-infective vitamin because of its role in maintaining
integrity of epithelial tissues and cell-mediated immune
defenses. Beta-carotene has antioxidant properties and is
a scavenger of oxygen-free radicals and protects various
organs from oxidant damage. Retinol is necessary for the
integrity and regeneration of rhodopsin or visual purple.
Vitamin A is also essential for the integrity of germinal
epithelium of gonads and reproductive organs.
Sources
Vitamin A from the diet is ingested as carotene from plant
sources and as retinol from animal sources. Among diff er-
ent carotene pigments, β-carotene (provitamin A) yields
the highest amount of retinol. Absorbed vitamin A is
stored in the liver as retinyl palmitate. Zinc is required for
mobilization of retinyl palmitate to free retinol, which is
transported in blood, bound to a retinol-binding protein.
Rich sources of preformed vitamin A or retinol are cod
liver oil, shark liver oil, and liver. Other good sources of
retinol include butter, clarifi ed butter (ghee), and egg yolk.
Provitamin A carotenoids are present in good amount in
carrots, pumpkins, green leafy and yellow vegetables, ripe
mangoes, and papaya. Red palm oil is an extremely good
source of carotene. Breast milk is a good source of vitamin A
especially if the mother has received supple ments during
pregnancy and lactation. The RDAs of vitamin A are 300 to
400 ?g in infants, 400 to 600 ?g in children, and 750 ? g in
adolescents (1.0 i.u. of vitamin A is equivalent to 0.3 ?g of
retinol, 0.6 ?g of β-carotene, and 1.2 ? g of other carotenes).
Clinical Features
Subclinical defi ciency of vitamin A is associated with in-
creased incidence and severity of respiratory, urinary
tract, and gastrointestinal infections. There is increased
risk of mortality due to measles and pneumonia.
Eff ects on Eyes
Defective or slow dark adaptation leading to night blindness
is the most characteristic early feature of VAD. Prolonged
defi ciency of vitamin A leads to xerophthalmia with its con-
sequences including blindness. It is usually associated with
various grades of PEM. There is loss of normal luster and
moist appearance of palpebral conjunctiva that becomes
dry and wrinkled. Bitot spots appear as chalky-gray nod-
ules on the temporal side of corneoscleral junction. The
cornea becomes soft (keratomalacia) and may ulcerate. Su-
peradded infection may cause perforation of cornea leading
to opacifi cation and blindness. Fundus examination may
show pale yellow spots near the retinal blood vessels. The
World Health Organization (WHO) has proposed a detailed
classifi cation of ophthalmological manifestations of VAD
Book 1.indb 91 07/06/13 8:58 PM

3. A Manual of Essential Pediatrics92
(Table 9.1). Keratomalacia is usually irreversible and results
in scar formation followed by phthisis bulbi (shrunken eye).
During advanced ophthalmic manifestations, serum retinol
level is usually less than 12 ?g/dL.
Other Manifestations
Skin over the arms and legs may develop scaly dermatitis
and toad-like appearance (phrynoderma), although this
manifestation may be a feature of essential fatty acid defi -
ciency. Alterations in the mucosa of renal pelvis and urinary
bladder may predispose to the development of renal and
vesical calculi. Atrophy of germinal epithelium of gonads
and uterus may interfere with reproductive functions.
Treatment
Children with xerophthalmia should be treated promptly
before it progresses to keratomalacia and corneal ulcer-
ation. Oral vitamin A is administered in a dose of 50,000
i.u. to infants younger than 6 months, 100,000 i.u. to in-
fants aged between 6 and 12 months, and 200,000 i.u. to
infants older than 1 year. The same dose is repeated after
24 hours and 4 weeks later. When oral intake is compro-
mised because of vomiting or diarrhea, parenteral water-
soluble vitamin A is administered in three-fourths dose in
infants younger than 6 months and one-half dose in in-
fants older than 6 months. Oil-based preparations are not
recommended for the treatment of complicated cases of
xerophthalmia.
Local treatment of the eye includes instillation of anti-
biotic ophthalmic drops or ointment three times a day to
prevent secondary infection when corneal ulcer is pres-
ent. Atropine eye ointment should be applied once a day
to keep the pupils dilated. Eye should be kept padded gen-
tly to protect the cornea from exposure and dehydration.
Padding also reduces pain and promotes epithelial healing.
Prevention
Nutrition education is important to promote consump-
tion of vitamin A-rich foods. The vitamin A content of
breast milk can be enhanced by administration of vitamin
A 200,000 i.u. to the nursing mother during 4 to 8 weeks
after delivery, that is, at the time of fi rst visit to the pe-
diatrician for vaccination of the child. Routine administra-
tion of vitamin A 100,000 i.u. with measles vaccine at 9
months followed by four more doses of 200,000 i.u. at 18,
24, 30, and 36 months of age are recommended. Children
with PEM and postmeasles bronchopneumonia should
be administered two doses of vitamin A (100,000 i.u. < 1
year and 200,000 i.u. > 1 year) at an interval of 24 hours.
Children with chronic and persistent diarrhea can be given
two doses of vitamin A at an interval of 1 month.
Vitamin D Defi ciency (Rickets)
Apart from bones, vitamin D is essential for a large number
of physiological processes to maintain optimal health. Sub-
clinical vitamin D defi ciency is now recognized as a pan-
demic, aff ecting more than half of the world’s population.
Rickets is a disease of growing bones and occurs in chil-
dren with vitamin D defi ciency. It primarily aff ects bones,
skeletal muscles, and sometimes the nervous system. It oc-
curs because of defi ciency of vitamin D
3
(cholecalciferol),
which is a naturally occurring steroid. It can be formed in
the skin by irradiation of 7-dehydrocholesterol with ultra-
violet B light in the wavelengths of 280 to 305 nm. It is also
known as “sunshine vitamin.” Exposure to sunlight for 20
minutes every day is suα& cient for the body to synthesize
enough vitamin D to meet daily requirements. The endog-
enous production or availability of vitamin D
3
is reduced
in the absence of sunlight and in dark-skinned individuals.
The dietary sources of vitamin D
3
include fortifi ed foods
(milk and milk products, vegetable oil, breads, and juices),
fi sh, cod liver oil, and egg yolk. Ultraviolet B irradiation of
ergosterol in plants and yeast produces vitamin D
2
(ergo-
calciferol), which is also a potent antirachitic substance but
is not available commercially as a supplement.
Vitamin D Metabolism
Vitamin D
3
(cholecalciferol) is converted in the liver to
biologically inactive but stable 25(OH)D (calcidiol) by he-
patic microsomal 25-hydroxylase. This metabolite circu-
lates in the plasma with a transport protein and is used as
the marker of vitamin D status. It is further hydroxylated
in the kidneys to calcitriol or 1,25-dihydroxyvitamin D
(1,25(OH)
2
D) by the action of mitochondrial 1-α-hydrox-
ylase (Figure 9.2 ). The physiological eff ects of 1,25(OH)
2
D
include (i) increased calcium absorption from the gastro-
intestinal tract, (ii) improved reabsorption of calcium from
renal tubules, and (iii) calcium deposition in the bones.
Like vitamin D
3
, vitamin D
2
is further metabolized in the
liver and kidneys to form its active metabolite. Apart from
vitamin D, calcium absorption is enhanced by the acid-
ic pH in the gut, dietary lactose, and when calcium and
phosphate are consumed in a ratio of 2:1.
Biochemical Consequences of Vitamin D Defi ciency
Vitamin D defi ciency leads to a decrease in the serum cal-
cium level, which stimulates the release of parathyroid
hormone (PTH). Parathyroid hormone tries to maintain
Table 9.1 The World Health Organization classifi cation
of xerophthalmia due to vitamin A defi ciency
Primary signs Secondary signs
XIA Conjunctival xerosis XN Night blindness
XIB Bitot spots XF Fundus changes
X2 Corneal xerosis XS Corneal scarring
X3A Corneal ulceration
(< 1/3 of cornea)
X3B Corneal ulceration
(> 1/3 of cornea)
Book 1.indb 92 07/06/13 8:58 PM

4. 9 Nutritional Disorders93
serum calcium level by (i) mobilizing calcium from bones,
(ii) promoting synthesis of 1,25(OH)
2
D in the kidneys
which in turn increases absorption of calcium from intes-
tines, and (iii) reducing excretion of calcium by the kidneys.
Parathyroid hormone reduces renal tubular reabsorption
of phosphates leading to phosphaturia. These homeostatic
changes tend to maintain serum calcium level while the
serum phosphate level falls. This leads to low calcium ×
phosphorus product, which is a characteristic feature of
active rickets. In extreme cases, even serum calcium level
may fall when compensatory mechanisms fail. Serum alka-
line phosphatase level increases because of increase in the
osteoclastic activity in the bones.
Rickets may occur in exclusively breast-fed babies be-
cause breast milk is a poor source of vitamin D (0.5 to 10 i.u./
dL), and there is a high prevalence of vitamin D defi ciency
during pregnancy and lactation. Vitamin D defi ciency may
occur in infants fed with cow’s milk or unfortifi ed formula
feeds. Rickets may also occur because of gastrointestinal
malabsorption and hepatic and renal disorders. Phytates in
the dietary cereals may bind calcium (magnesium and iron
as well) in the gut to form phytin, thus compromising calci-
um absorption. Anticonvulsant therapy with phenytoin and
phenobarbitone may interfere with metabolism of vitamin
D. Glucocorticoids appear to have eff ects that are antagonis-
tic to vitamin D for calcium absorption. The risk factors for
development of vitamin D defi ciency are listed in the Box.
Risk factors for development of vitamin D defi ciency
Female gender
Insuffi cient sun exposure, skin pigmentation, over-
crowding, overclothing, atmospheric pollution, use of
sunscreens, and genetic factors
Lack of vitamin D supplementation
Poor socioeconomic status
Lack of intake of vitamin D fortifi ed foods, fi sh, cod
liver oil, and eggs
Diseases
Obesity, infl ammatory bowel disease, cystic fi brosis,
celiac disease, chronic renal failure, and epilepsy
Medications
Corticosteroids, anticonvulsants, and
rifampin
Clinical Features
Early Rickets
Congenital rickets may occur if the mother has subclini-
cal vitamin D defi ciency with compensatory hyperpara-
thyroidism. The characteristic sign of congenital rickets is
craniotabes (softening of skull bones). When the occipital
bone is pressed fi rmly, it gets depressed and gives a feel
like a table tennis ball. Tetany is common if the infant is
fed with unmodifi ed cow’s milk (because of high phos-
phate load). Preterm babies are prone to develop osteo-
penia of prematurity or rickets. This occurs because of di-
etary phosphate defi ciency and impaired hydroxylation of
vitamin D in the liver.
Classical Rickets
The classical vitamin D-defi ciency rickets manifest most
commonly in children between the age of 6 months and 2
years. Rickets is common in chubby children who are ac-
tively growing. Children with PEM may manifest rickets
during recovery when they are actively growing. The clini-
cal features are produced by enlargement of costochondral
junctions or ends of long bones, osteoporosis, softening of
bones, and skeletal deformities.
Skull is square-shaped and shows bossing or prom-
inence of frontal and parietal bones because of
subperiosteal deposition of osteoid. When bossing is
marked, the head looks large and square and may give
7-Dehydrocholesterol
HO
Cholecalciferol
(Vitamin )D
3
Skin
Sunlight
(UV B radiation)
HO
25-Hydroxycholecalciferol
Liver
(25-Hydroxyvitamin D )
3
HO
Kidneys
OH
1,25-Dihydroxycholecalciferol
(1,25-Dihydroxyvitamin )D
3
HO
OH
Active form
of vitamin D
OH
25-Hydroxylase
1 -Hydro-
xylase
a
Figure 9.2 Vitamin D metabolism.
Book 1.indb 93 07/06/13 8:58 PM

5. A Manual of Essential Pediatrics94
an appearance of “hot-cross-bun.” Anterior fontanel
is large and its closure is delayed beyond 18 months.
Dentition may be delayed.
Chest shows “rachitic rosary” with “beading of ribs”
because of enlargement of costochondral junctions.
There is visible and palpable swelling of costochon-
dral junctions on both sides of sternum, giving an ap-
pearance of a rosary of beads. Sternum may project
forward (pigeon breast). There may be depressions or
sulci on both sides of lower chest at the level of at-
tachment of diaphragm (Harrison groove) because of
softening of ribs. A similar appearance of lower chest
(without costochondral beading) may be seen in chil-
dren with recurrent respiratory infections, bronchial
asthma, and congenital heart disease.
Long bones show enlargement of ends, softening,
and deformities. There is enlargement and swelling
of wrists (Figure 9.3 ) and ankles. The shafts of long
bones of lower limbs may develop various curvatures
and deformities leading to genu varum (bowed legs),
genu valgum (knock knees), and coxa vara (widening
or abduction of hips).
Spinal deformities, such as dorsolumbar lordosis, ky-
phosis, or scoliosis, may occur because of laxity of
spinal ligaments.
Stature may be grossly aff ected by spinal and lower
limb deformities. Pelvic outlet may be narrowed
down by forward displacement of the sacral promon-
tory and deformities of pelvic bones leading to di& -
culties in child birth later in life of a female.
Non-skeletal manifestations of rickets include sweat-
ing muscular hypotonia, hair loss, protuberant abdo-
men or pot belly, and recurrent respiratory infections.
Iron-defi ciency anemia is also commonly associated.
Tetany may occur rarely.
Laboratory Investigations
Serum calcium is usually normal (9 to 11 mg/dL), phos-
phate is usually reduced (< 4 mg/dL), and alkaline phos-
phatase is elevated (> 500 i.u./dL). The normal plasma cal-
cium despite diminished intestinal absorption of calcium
(because of vitamin D defi ciency) is explained on the ba-
sis of compensatory increased parathyroid activity, which
mobilizes calcium from the bones and also causes hypo-
phosphatemia because of phosphaturia. A plasma calcium
phosphorus product (mg/dL) above 40 excludes vitamin
D-defi ciency rickets, whereas a value below 30 indicates
active rickets. Serum phosphate level is normal in vitamin
D-dependent rickets and metaphyseal dysostosis, where-
as it is elevated in renal osteodystrophy. Elevated alkaline
phosphatase level is a sensitive indicator of active rickets but
may be elevated in primary hyperparathyroidism, obstruc-
tive jaundice, fractures, metastatic bone disease, and the
“battered baby” syndrome. Plasma 25(OH)D level is usually
low while PTH level is increased. Plasma 25(OH)D level of
less than 20 ng/mL (50 nmol/L) is suggestive of vitamin D
defi ciency, whereas a level between 21 and 29 ng/mL sug-
gests vitamin D insu& ciency. However, these tests are not
routinely required for the diagnosis of rickets. Skiagram
of wrists or knees shows characteristic radiological fi nd-
ings. The normally smooth and convex ends of bones be-
come splayed out with metaphyseal fraying and “cupping”
(Figure 9.4). The distance between the epiphysis and
Figure 9.3 Widened wrist with pigeon-shaped chest in a 3-year-
old boy with vitamin D defi ciency rickets.
Figure 9.4 Note the typical cupping and rarefaction of metaph-
yseal ends of both the radius and ulna because of active rickets.
Book 1.indb 94 07/06/13 8:58 PM

6. 9 Nutritional Disorders95
diaphysis is increased because the metaphysis consists
largely of non-opaque osteoid tissue. The periosteum may
be raised because of laying down of osteoid tissue while
shafts of bones may become decalcifi ed and curved. Dur-
ing healing following administration of vitamin D, a line
of preparatory calcifi cation appears near the diaphysis fol-
lowed by appearance of calcifi cation in the irregular oste-
oid at the frayed ends of bones.
Diff erential Diagnosis
Congenital rickets may be confused with hypophospha-
temic rickets, which is characterized by defective calcifi ca-
tion of the membranous bones of skull, wide sutures, cu-
taneous dimples, failure to thrive, hypercalcemia, and low
alkaline phosphatase. Some healthy toddlers may show
physiological bowing of the legs. The ends of bones may be
broad in those with osteochondrodystrophy, but they have
no other clinical or biochemical features of rickets. Rickets
may occur because of malabsorption (celiac disease, fi bro-
cystic disease of the pancreas), hepatic (cholestatic disor-
ders) and renal (renal tubular acidosis, Fanconi syndrome,
renal osteodystrophy) causes. These should be excluded
on the basis of history, physical examination, and labora-
tory investigations.
Treatment
Vitamin D
3
is administered in a weekly oral dose of 60,000
i.u. (1 mg calciferol = 40,000 i.u.) for 10 weeks. Alternative-
ly 600,000 i.u. of vitamin D can be given orally or through
deep intramuscular injection as a single dose. Radiological
healing (zone of calcifi cation) and biochemical improve-
ment are seen within 3 to 4 weeks. The above dose may
be repeated if therapeutic response is incomplete. Main-
tenance doses of vitamin D
3
400 units (10 ?g) per day are
continued after the process of healing has started. The in-
fant should be given adequate amount of calcium in the
diet (600 mg calcium per day or 600 mL of milk). Calcium
supplements are not needed once serum 25(OH)D level is
normalized. In children with rickets because of renal or he-
patic causes, it is preferable to use calcitriol or 1,25(OH)
2
D
(0.05 ?g/kg/d). Weight-bearing on the legs may be restrict-
ed till complete radiological healing has occurred. Skeletal
deformities usually disappear over a period of time and
osteotomy is rarely required. Children should be encour-
aged to play outdoor for adequate exposure to sunlight. If
there is any underlying gastrointestinal, hepatic (cholesta-
sis), or renal (renal tubular acidosis, Fanconi disease, and
renal osteodystrophy) disease, it should be diagnosed and
appropriately managed. When two mega doses of vitamin
D (600,000 i.u. each dose) fail to elicit any biochemical or
radiological response, the child should be investigated for
refractory rickets (Figure 9.5).
Renal Osteodystrophy
Chronic renal dysfunction is an important cause of refrac-
tory rickets. There may be evidences of underlying renal
disease such as developmental malformations, recurrent
Low or normal
Blood pH
Low Normal
Normal PTH and
normal calcium
Hypophosphatemic
rickets
Renal tubular
acidosis
Serum PTH, calcium
High
Chronic kidney disease
High PTH and
low calcium
Vitamin D-dependent
rickets
Refractory rickets
Serum phosphate
Figure 9.5 Algorithm for the diagnosis of vitamin D refractory
rickets. PTH, parathyroid hormone.
pyelonephritis, or chronic glomerulonephritis. There is
failure to thrive, growth retardation, and skeletal abnor-
malities such as widened ends of long bones, genu val-
gum, and chest deformities (Figure 9.6). The characteristic
biochemical abnormalities include elevated blood levels of
creatinine and phosphate. The condition is treated by re-
nal replacement therapy, restricting intake of phosphate,
and providing supplements of calcium and active vitamin
D analogs (calcitriol).
Primary Hypophosphatemia
(Vitamin D-Resistant Rickets)
It is the most common non-nutritional form of rickets
that aff ects male children. The condition is inherited as
X-linked dominant with variable penetrance. The meta-
bolic defects include failure of reabsorption of phosphate
from proximal tubules and lack of conversion of 25(OH)
D to 1,25(OH)
2
D in the kidneys. Children present the
condition at an early age with short stature, waddling
gait, and marked skeletal abnormalities such as bowed
legs or genu valgum and coxa vara. Teeth may have ab-
normal enamel with increased risk of pulp deformities
and dental infections. The level of serum calcium is nor-
mal or low, phosphorus is reduced, and alkaline phos-
phatase is elevated. There is no aminoaciduria, glycos-
uria, and bicarbonaturia. The condition is treated with
oral phosphate (sodium phosphate 0.5 to 4.0 g/d in four
to six divided doses) along with vitamin D (alfacalcidol
0.02 mg/kg daily).
978-0-xxxx_Theime_Meh_Ch09.indd 95 10/06/13 5:54 PM

7. A Manual of Essential Pediatrics96
Vitamin D-Dependent Rickets
Vitamin D-dependent type 1 rickets is an autosomal-re-
cessive disorder that manifests around 3 to 6 months of
age. There is defi ciency of 1-α-hydroxylase in the kidneys
leading to low levels of 1,25(OH)
2
D. Serum calcium and
phosphate levels are low but PTH level is high. Serum
25(OH)D level is normal, whereas 1,25(OH)
2
D is extreme-
ly low. The condition is treated with high doses of vitamin
D
2
(200,000 to 1,000,000 i.u./d), or preferably calcitriol
(1,25(OH)
2
D) 0.25 to 2.0 μg/d along with supplements of
calcium.
Patients who fail to respond to massive doses of vita-
min D
2
are labeled to have vitamin D-dependent rickets
type 2. Alopecia is seen in 50 to 70% of patients. The defect
lies in end-organ resistance of vitamin D nuclear receptors
to 1,25(OH)
2
D. The blood levels of 1,25(OH)
2
D are grossly
elevated. There is no known treatment for this rare dis-
order. High doses of vitamin D
2
or calcitriol (2 μg/d) and
calcium 1000 to 3000 mg/d can be tried.
Non-Skeletal Health Benefi ts of Vitamin D
Calciotropic eff ects of vitamin D on intestines, bones, and
kidneys have been well known since ages. Recent evidence
suggests that vitamin D has a benefi cial eff ect on a large
Figure 9.6 Renal osteodystrophy in a 10-year-old girl. She had
laboratory evidences of azotemia and grossly elevated serum
phosphate level.
number of body tissues and metabolic processes to main-
tain sound health. Vitamin D receptors have been iden-
tifi ed in more than 30 body tissues including intestines,
liver, kidneys, heart, lungs, brain, muscles, skin, pancreas,
and various immune cells. Moreover, apart from kidneys,
enzyme CYP27B1 (which helps in conversion of 25(OH)D
into biologically active 1,25(OH)
2
D) has been found in var-
ious tissues of the body. The non-skeletal autocrine pleio-
tropic eff ects of vitamin D are independent of regulation
of serum calcium, phosphorus, and PTH levels and are not
controlled by the feedback-endocrine loop mechanism.
There is evidence to suggest that vitamin D exerts benefi -
cial eff ects on a large number of body organs to maintain
optimal health and well-being. Vitamin D is credited with
potent antioxidant eff ect to prevent free radical damage
and include cellular diff erentiation to protect against au-
toimmune diseases and carcinogenesis. The long-term
health benefi ts of vitamin D are listed in the following Box.
Non-skeletal health benefi ts of vitamin D
αAutoimmune diseases
→Reduced risk of type 1 diabetes mellitus, rheuma-
toid
arthritis, bronchial asthma, multiple sclerosis,
systemic lupus erythematosus, infl ammatory bowel
disease, and psoriasis.
αMalignant disorders
→Decreased risk of malignant disorders such as colo-
rectal cancer, breast cancer, prostate cancer,
and leukemia.
αHeart disease
→Reduced risk of hypertension and coronary artery
disease
αBetter control of type 2 diabetes mellitus and neuro-
pathic pains
αNeuropsychological functioning
→Better cognition, reduced risk of depression and
aff ective disorders
αInfections
→Reduced risk of periodontal diseases, respiratory
tract infections, and tuberculosis.
9.4 Protein–Energy Malnutrition
Protein–energy malnutrition occurs either because of di-
etary inadequacy or chronic systemic disease and recur-
rent infections. The National Family Health Survey-3 esti-
mates that 8 million children younger than 5 years of age
in India are suff ering from severe acute malnutrition. The
child is considered underweight when his/her weight is
less than 2SD from the median weight-for-age of National
Center for Health Statistics (NCHS) reference standards.
Book 1.indb 96 07/06/13 8:58 PM

8. 9 Nutritional Disorders97
Stunting and wasting may occur because of inadequate
intake of calories and proteins or because of an underly-
ing systemic disorder. However, with progressive elimi-
nation of protein–energy defi cits in the diet, it is being
increasingly recognized that defi ciency of micronutrients
(vitamins and trace minerals) is an important cause of
growth failure and recurrent infections. The extreme
forms of nutritional disorders known as marasmus and
kwashiorkor are relatively uncommon these days.
Marasmus
It is common in children younger than 1 year and occurs
primarily due to lack of calories. There is marked wasting
of muscles and subcutaneous tissues resulting in emacia-
tion and stunting. The loose folds of skin are seen over the
Stunting is diagnosed when height-for-age of the child is
below 2SD of the expected median height-for-age of refer-
ence population. By this criterion, around 50% of children
younger than 5 years of age are stunted in India. Wasting
is defi ned as weight-for-height of the child as less than
2SD of the expected median weight-for-height of refer-
ence standards. When PEM is acute in onset, body weight
is aff ected more than height. In chronic cases of PEM, both
body weight and height are aff ected. In severe malnutri-
tion, even brain growth may be aff ected with reduced
head circumference. According to the Indian Academy of
Pediatrics, PEM is graded as shown in the Box.
The Gomez classifi cation of malnutrition based on
weight-for-age criteria is shown in Table 9.2.
Causes Protein–energy malnutrition may be primary
due to lack of food and interplay of infections and dietary
intake or it may be secondary as a result of underlying sys-
temic disease such as chronic kidney, liver, heart disease,
or HIV-infection. The immediate or precipitating causes of
PEM include inadequate intake of food and repeated infec-
tions including diarrhea and pneumonia or it may follow
an attack of measles or pertussis, and may get complicated
by superadded tuberculosis. The root causes of PEM are
political and they include socioeconomic disparity, poor
housing, overcrowding, lack of environmental sanitation,
unsafe drinking water, lack of health awareness and nu-
trition education, gender inequality, and poor status of
women in some societies.
Table 9.2 Gomez classifi cation of malnutrition
Weight-for-age
a
(%) Grade of malnutrition
>80 Normal
71–80 Grade I
61–70 Grade II
51–60 Grade III
<50 Grade IV
a
50th percentile of NCHS reference data.
Figure 9.7 A 3-year-old boy with marasmus. Note wasted
extremities, poor muscle mass, loss of subcutaneous fat (skin
hangs in folds over buttocks and thighs), and visible bony
prominences.
IAP Grading for protein–energy malnutrition
Grade I
Body weight between 71 and 80% of the expected
weight-for-age
Grade II
Body weight between 61 and 70% of the expected
weight-for- age
Grade III
Body weight between 51 and 60% of the expected
weight-for-age
Grade IV
Body weight up to 50% or less of the expected weight
for age
Book 1.indb 97 07/06/13 8:58 PM

9. A Manual of Essential Pediatrics98
buttocks and inner side of thighs (“baggy pant” appear-
ance). The buccal pad of fat is the last to disappear lead-
ing to wrinkling of face (monkey facies). The skin is thin,
loose, dry, inelastic, and wrinkled (Figure 9.7). The abdo-
men is distended because of wasting and hypotonia of
muscles of abdominal wall. There is loss of subcutaneous
fat and muscles, the bony points including ribs stand out
prominently. The baby appears alert, may be irritable but
has good appetite unless complicated by intercurrent in-
fection. When a child with marasmus develops edema due
to intercurrent infection and aggravation of protein defi -
ciency, the condition is known as marasmic kwashiorkor.
Kwashiorkor
It is a severe form of dietary protein defi ciency when a
child is weaned from breastfeeding with starchy gruels for
example, following the birth of a sibling who is breast-fed.
Kwashiorkor in Ga language (tribal language) of West Africa
means “the disease of the displaced child.” Kwashiorkor has
become rare because of better socioeconomic status and
availability of food. The classical features include growth
retardation, psychomotor changes, generalized edema, skin
changes (fl aky paint or mosaic dermatosis because of ex-
posed raw skin areas with pigmentation and hypopigmen-
tation), hair changes (thin, dry, brown, and brittle hair; fl ag
sign because of alternate bands of normal and light colored
hair), enlargement of the liver due to fatty infi ltration and
mental changes (lethargy, listlessness, and apathy). There
is abdominal distension because of hypotonia and hepato-
megaly. The child looks miserable and has loss of appetite.
Stunting is present but body weight may be normal because
of generalized edema (Figure 9.8). There may be associated
defi ciency of micronutrients, especially iron, folic acid, zinc,
vitamin A, and vitamin B complex.
Complications
Children with severe PEM are prone to develop acute in-
tercurrent infections, oral thrush, septicemia, and tuber-
culosis. They are unable to maintain body temperature
and may develop severe hypothermia, hypoglycemia, and
electrolyte disturbances (especially hypokalemia). It is
di& cult to assess the severity of dehydration in malnour-
ished children. There may be associated defi ciencies of
other micronutrients, especially nutritional anemia. Rick- ets is uncommon in malnourished children because rick- ets is a disease of growing bones.
Management
Nutritional rehabilitation is a slow process and demands health education to the family. It must be explained that PEM
is not a disease and the child is suffering from lack of food. The
following principles of management should be followed:
1. Keep the child warm and protected against nosoco- mial infections and provide tender loving care with compassion.
2. Identify intercurrent infections and treat them ap- propriately with parenteral antibiotics.
3. Identify associated metabolic disturbances, such as dehydration, hypoglycemia, and dyselectrolytemia, and treat them appropriately through intravenous route. In children with persistent diarrhea, lactose in- tolerance should be ruled out. Potassium supplements are required in all malnourished children.
4. Provide increasing quantities of well-balanced liquid and semisolid food depending on the appetite and acceptability of the child. When the child refuses to accept orally because of severe anorexia, nasogastric feeding is recommended. The calculation for the cal- ories and protein requirements is based on the cur- rent weight of the child. Start with 80 kcal/kg/d of en- ergy and 0.7 g/kg of protein. Increase the food intake gradually, depending on the tolerance of feeds to 150 kcal/kg/d with 2 to 3 g proteins/kg/d over the next 7 to 10 days. Milk-based diet fortifi ed with vegetable
or coconut oil with additional protein through sup- plements or egg is suitable. Availability and accept- ability of ready-to-use therapeutic food (RUTF) has simplifi ed the management of malnutrition. There
is a need to indigenously produce RUTF in partner- ship with industry and food technology institutes. In children with lactose intolerance, lactose-free milk is recommended. Supplements of micronutrients in- cluding potassium, magnesium, vitamin A, vitamin K, vitamin B complex, and zinc should be provided. Iron should preferably be given through parenteral route. When the hemoglobin drops to below 6 g/dL, packed cells transfusion or whole blood can be given slowly after administration of a diuretic.
5. Nutrition education and health education must be provided to the family to prevent the recurrence of malnutrition. The child should be shifted to a home- based diet before discharge.
9.5 Obesity
Overnutrition and obesity are being increasingly recog- nized in children belonging to a' uent families. More
than one-fourth of adolescents attending private schools
Figure 9.8 A 1½-year-old girl with kwashiorkor. Note apathy,
growth retardation, generalized edema, sparse hair, and crazy-
pavement dermatosis over legs. There was hepatomegaly due to
fatty infi ltration of liver.
Book 1.indb 98 07/06/13 8:58 PM

10. 9 Nutritional Disorders99
are obese. Several school going adolescents prefer to take
calorie-dense unhealthy and ill-balanced snacks and junk
foods such as soft drinks, burgers, pizzas, and desserts,
and so on. A weight-for-age of greater than 2SD and 3SD
of NCHS or WHO reference standards is suggestive of over-
weight and obesity, respectively. According to WHO, body
mass index (BMI) (weight in kg/[height in meters]
2
) greater
than 85th and 95th percentile of BMI for age and sex refer-
ence standard are more reliable criteria for the diagnosis
of overweight and obesity, respectively. Body mass index
takes into account body mass or weight in relation to stat-
ure but does not provide any information on the composi-
tion or distribution of body fat. Body volume index (BVI) is
now considered a more reliable parameter of obesity, but it
demands the availability of a special three-dimensional full
body scanner to accurately assess both the quantum as well
as the distribution of fat in the body. Body volume index
provides computer-based data on BMI, waist circumfer-
ences and waist-to-hip ratio. Obese children are also likely
to be tall for their age if obesity is because of exogenous or
constitutional causes and overeating. The bone age is also
advanced. It is important to remember that obese adoles-
cents are likely to become obese adults with all the atten-
dant health consequences such as hypertension, metabolic
syndrome X, type 2 diabetes mellitus, and coronary artery
disease. Obesity because of hormonal imbalance or endog-
enous causes is uncommon. It occurs at a younger age and
is usually associated with additional manifestations such as
hypertension, short stature, and mental retardation.
Exogenous Obesity
Exogenous obesity occurs because of an imbalance between
energy or food intake and energy expended by physical ac-
tivity. Intake of excessively fatty, energy-dense junk food
and munching of snacks in between the meals are impor-
tant risk factors. Sedentary lifestyle, lack of participation in
outdoor games, munching of snacks while viewing televi-
sion, and playing video games for prolonged periods of time
further aggravate the situation because the food consumed
is not utilized and the extra calories are laid down in the
fat depots such as abdomen, buttocks, and limbs. The mod-
ern style of fasting or missing meals followed by periods of
bingeing is an important cause of obesity. The risk of obe-
sity is high if there is family predisposition to develop obe-
sity. There is some evidence that constitutional predisposi-
tion may occur because of the presence of an “ob” gene that
regulates the production of a protein called leptin which
controls metabolic rate and appetite. Overweight children
become lazy and inactive, thus establishing a vicious cycle
of overeating–inactivity–obesity. There is an increasing in-
cidence of hypertension, metabolic syndrome X, and type 2
diabetes mellitus among obese adolescents. Other compli-
cations of obesity include obstructive sleep apnea, dyslip-
idemia, gall stones, slipped capital femoral epiphysis, and
osteoarthritis.
Endogenous Obesity
Endogenous or pathological obesity is rare and account
for less than 1% of cases and occurs because of genetic or
endocrinal causes (see Box on the next page). The clinical
features that are suggestive of endogenous obesity include
normal or voracious appetite, central obesity with “buf-
falo-hump” (accumulation of fat over face, upper back,
and supraclavicular regions), dysmorphism, acne, hyper-
tension, and short stature. There may be hypogonadism
in boys; hirsutism and menstrual abnormalities in girls.
Central nervous system manifestations include excessive
sleepiness, evidences of raised intracranial pressure, vi-
sual fi eld defects, retinal degeneration, and mental retar-
dation. Pathological obesity is easy to diagnose and can be
diff erentiated from constitutional obesity on clinical ex-
amination (Table 9.3).
Table 9.3 Salient diff erences between constitutional and pathological obesity
Feature Constitutional or familial obesity Pathological or endocrinal obesity
Family history May be present Usually absent
Eating behavior and
lifestyle
Excessive or fast and faulty eating habits,
sedentary and lazy lifestyle
Eating normal or voracious, activity is aff ected after the
onset of obesity
Distribution of fat Generalized Central obesity with “buff alo hump” with greater deposi-
tion of fat over the face and upper back
Height and bone age Usually increased with advanced bone age Usually short with retarded bone age
Blood pressure Usually normal May be raised
Endocrinal eff ects Nil Acne, hirsutism, amenorrhea or menstrual irregularity,
metabolic syndrome X or type 2 diabetes mellitus
Hypogonadism None but penis may be embedded in the
pubic pad of fat
May be associated with several syndromes such as Prader-
Willi syndrome, Klinefelter syndrome, Alström syndrome,
Laurence-Moon-Biedl syndrome, growth hormone defi -
ciency, hypothalamic disorders
CNS features None Excessive sleepiness, hydrocephalus with visual fi eld de-
fects (craniopharyngioma, pituitary tumor), papilledema
or retinal degeneration, and mental retardation
Abbreviation: CNS, central nervous system.
Book 1.indb 99 07/06/13 8:58 PM

11. A Manual of Essential Pediatrics100
Causes for endogenous obesity
Genetic causes
Prader-Willi syndrome Infantile obesity, marked hypotonia, are fl exia, short stature, and hypogonadotropic hypogonadism
(Figure 9.9). Respiratory distress (Pickwickian syndrome) may develop because of deposition of fat in the interstitial tissue of
lungs.
Laurence-Moon-Biedl syndrome Obesity, polydactyly or syndactyly, retinitis pigmentosa, renal anomalies, hypogonadism,
and mental retardation.
Hormonal causes
Hypothyroidism
Hypogonadism (Klinefelter syndrome)
Polycystic ovary disease Excessive weight and obesity during adolescence; hirsutism; acne; pigmentation over axillae,
groins, and back of neck (acanthosis nigricans); delayed menarche; and type 2 diabetes mellitus.
Cushing syndrome Centripetal obesity, “buff alo-hump,” hypertension, violaceous striae on abdomen, hirsutism, short stat-
ure, and diminished glucose tolerance. The condition may occur because of carcinoma of adrenal cortex (Figure 9.10).
Pseudohypoparathyroidism Moon-shaped facies, depressed nasal bridge, stocky build, skeletal abnormalities (short meta-
carpals and metatarsals with index fi nger being longer than middle fi nger, curved radius, cubitus and genu valgum, coxa vara,
exostosis), calcifi cation of basal ganglia, cataracts, mental retardation and tetany with hypocalcemia, hyperphosphatemia,
and elevated parathyroid hormone levels.
Fröhlich syndrome Obesity, excessive appetite, short stature, sexual infantilism, and visual abnormalities due to tumor in
the hypothalamus.
Figure 9.9 A 1½-year-old boy with obesity, hypogonadism, and
mental retardation due to Prader-Willi syndrome.
Figure 9.10 Cushing syndrome in a 2-year-old girl caused by
adrenocortical carcinoma.
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12. 9 Nutritional Disorders101
Commonly Asked Questions
Investigations
When pathological obesity is suspected on clinical
grounds, investigations are done to rule out genetic and
endocrinal causes. Eyes should be examined by an oph-
thalmologist for fi eld of vision and any visual abnormali-
ties. Thyroid profi le (triiodothyronine, thyroxine, and thy-
roid stimulating hormone), insulin resistance (fasting glu-
cose), and electrolytes (sodium, potassium, calcium, phos-
phorus, and alkaline phosphatase) should be checked.
Elevation of serum sodium level and hypokalemia are
suggestive of hypercorticism. Bone age should be assessed
by taking skiagrams of the left wrist and elbow. Estima-
tion of morning and evening levels of cortisol with loss of
diurnal rhythm is a reliable marker of hypercorticism. In
adrenocorticotropin hormone (ACTH)-dependent Cushing
syndrome, magnetic resonance imaging (MRI) scan of the
hypothalamic-pituitary region is advised. When the ACTH
level is low, MRI scan of adrenal gland is done to rule out
adrenal adenoma and carcinoma.
Management
1. The whole family should be advised to change to a
healthier lifestyle. It is impossible to change the rou-
tine and habits of the child unless everybody in the
family cooperates and participates.
2. Healthy food habits should be practiced by taking
plenty of green leafy vegetables, salads, and seasonal
fruits. Food should be prepared in minimal oil or fat.
Energy-dense foods such as soft drinks, fruit juices,
junk food, crisps and French fries, fried items (such as
poories, samosas, kachoris, mathri, and namkeen), des-
serts, dry fruits, chocolates, and so on should be re-
stricted. Double-toned or fat-free milk should be used
for drinking and for making curd, cheese, and other
milk products. Before eating meals, salads and water
can be taken to fi ll the stomach. Food should be eaten
slowly and not in a hurry so that satiety is achieved
with lower intake of food. Missing of meals, “dieting,”
and “bingeing” are strongly condemned. The use of
drugs to reduce appetite or surgical treatment of obe-
sity by bariatric procedures is not recommended in
children.
3. Children should be encouraged to take part in out-
door activities and sports such as running, jogging,
skipping, cycling, and swimming. They should be en-
couraged to play badminton, tennis, football, or crick-
et depending on the interest. Aerobic exercises and
dancing with music are extremely useful and health-
friendly activities. Television viewing, playing video-
games, and Internet surfi ng should be restricted to a
maximum of 1 hour and no snacks should be allowed
while watching television.
4. The goal for weight reduction should be realistic,
slow, and sustainable. Motivation and willpower are
required to adhere to the weight reduction program.
It is reasonable to target a weight reduction of ap-
proximately 0.5 to 1 kg every 2 to 4 weeks.
5. The role of drugs is limited for treatment of obesity.
Metformin is useful in children with insulin resistance
(metabolic syndrome X and polycystic ovarian dis-
ease) and provides added benefi t of weight reduction.
There is doubtful utility of drugs such as orlistat (gas-
tric lipase inhibitor), sibutramine (neurotransmitter
modulator), leptin (leptin defi ciency), and octreotide
(hypothalamic obesity) in children with obesity.
6. Treatment of endogenous obesity is symptomatic and
depends on the underlying cause. Hormonal replace-
ment (thyroxin, human growth hormone, and sex
hormones) or surgical excision of an hormone-pro-
ducing tumor is likely to be curative.
Further Reading
What is alfacalcidol?
Alfacalcidol is 1-α -hydroxycholecalciferol, the synthetic
derivative of vitamin D
3
. It is a prodrug that is rapidly hy-
droxylated in the liver directly to calcitriol (1,25(OH)
2
D
3
).
It is useful for the treatment of hypoparathyroidism, re-
nal rickets, vitamin D-resistant rickets, and osteomala-
cia. It is given in an oral dose of 0.05 ?g/kg/d in children
Holick MF, Chen TC. Vitamin D de fi ciency: a world-
wide problem with health consequences. Am J Clin Nutr
2008;87(4):S1080–S1086.
Misra M, Pacaud D, Petryk A, Collett-Solberg PF, Kappy M.
Drug and Therapeutics Committee of the Lawson Wilkins
Pediatric Endocrine Society. Vitamin D defi ciency in chil-
dren and its management: review of current knowledge and
recommendations. Pediatrics 2008;122(2):398–417.
Rathi N, Rathi A. Vitamin D and child health in the 21st cen-
tury. Indian Pediatr 2011;48(8):619–625.
Sivakumar B, Nair KM, Sreeramulu D, et al. Eff ect of micronu-
trient supplement on health and nutritional status of school
children: biochemical status. Nutrition 2006;22:S15–S25.
Stechschulte SA, Kirsner RS, Federman DG. Vitamin D: bone
and beyond, rationale and recommendations for supple-
mentation. Am J Med 2009;122(9):793–802.
Book 1.indb 101 07/06/13 8:58 PM

13. A Manual of Essential Pediatrics102
weighing less than 20 kg and 1.0 ?g/d in older children
and adults.
Can active rickets be associated with normal or
low level of alkaline phosphatase?
Elevated alkaline phosphatase is a characteristic biochemi-
cal feature of active rickets. However, rickets may occur in
association with normal or even low levels of alkaline phos-
phatase in congenital hypophosphatasia, severe protein defi -
ciency, and zinc defi ciency.
What are the conditions with raised level of
alkaline phosphatase?
Elevated level of serum alkaline phosphatase is a character-
istic biochemical fi nding in all types of rickets except con-
genital hypophosphatasia. Other conditions with elevated
serum alkaline phosphatase include primary hyperpara-
thyroidism, obstructive or cholestatic jaundice, fractures,
metastases in the bones, and battered baby syndrome.
What are the clinical diff erences in costochondral
beading due to rickets and scurvy?
Rickets and scurvy both produce costochondral beading,
but it can be easily diff erentiated on clinical examination.
In rickets, there is smooth, rounded, and globular enlarge-
ment of costochondral junctions. In contrast, in scurvy the
beading is sharp like a bayonet due to posterior displace-
ment of sternum and the attached cartilage components
of ribs at the costochondral junction. Moreover, other clin-
ical features of rickets and scurvy are distinctive and the
two conditions can be readily diff erentiated.
What are the hazards of excessive intake of vitamins?
Micronutrients in optimal amounts are essential to
maintain optimum health and well-being. Excess of ev-
erything is bad and vitamins are no exception. Excessive
intake of water-soluble vitamins (vitamin B complex
and vitamin C) is not associated with any adverse eff ects
because they are readily excreted in the urine and not
stored in the body. However, there is recent epidemio-
logical evidence to suggest that routine intake of mega
doses of multivitamins by healthy adults for a prolonged
duration may increase the risk of adverse health conse-
quences.
Excessive intake of fat-soluble vitamins, especially vita-
mins A and D, may lead to symptoms of hypervitaminosis
because they are stored in the body. Acute manifestations
of hypervitaminosis A include signs of raised intracranial
tension due to pseudotumor cerebri such as headache,
vomiting, dizziness, bulging anterior fontanel, and pap-
illedema. The symptoms resolve gradually without any
sequelae on stoppage of vitamin A. Chronic intoxication
with vitamin A may cause anorexia, irritability, dry itchy
skin, failure to thrive, painful extremities with hyperosto-
sis of shafts of long bones and clavicles (Caff ey disease),
sparse hair, hepatosplenomegaly, hypoplastic anemia, and
benign intracranial hypertension. Excessive intake of ca-
rotenoids does not cause any symptoms of toxicity except
yellow discoloration of skin (not the sclera) which is re-
versible.
Hypervitaminosis D leads to toxic symptoms as a con-
sequence of hypercalcemia. The toxic manifestations in-
clude loss of appetite, irritability, failure to thrive, poly-
uria, polydipsia, hypotonia, constipation, cardiac arrhyth-
mias, and metastatic calcifi cation in the soft tissues. Cal-
ciuria may lead to nephrocalcinosis and kidney damage.
Serum calcium, phosphate, and 25(OH)D levels are grossly
elevated (> 150 ng/mL). Intake of calcium and vitamin D
including sun exposure should be reduced. Prednisolone 1
to 2 mg/kg/d can be given to reduce absorption of calcium
by blocking the action of 1,25(OH)
2
D. Normal saline and
loop diuretics can be given to increase calcium loss in the
urine. Sodium versenate, phosphate binders, bisphospho-
nates, and calcitonin can be used to chelate calcium from
the body. There are no reports of toxic manifestations due
to excessive intake of vitamins E and K.
Which vitamins are synthesized in the body?
Most vitamins are provided exogenously through nutra-
ceuticals and dietary sources. Vitamin D
3
is produced in
the skin by photoirradiation of 7-dehydrocholesterol to
cholecalciferol. Almost 90% of the requirements of the
body can be met through endogenous production of vita-
min D
3
if there is adequate exposure of skin to sunlight, es-
pecially when the skin is not too dark. Vitamin K
l
and most
B complex vitamins are produced in the gut by intestinal
microfl ora. Excessive or prolonged use of broad-spectrum
antibiotics may sterilize the gut by killing the “friendly
bacteria” leading to defi ciency of vitamin K and B com-
plex. It is standard practice to give supplements of vitamin
B complex and vitamin K whenever broad-spectrum an-
tibiotics are taken for a prolonged duration. Endogenous
production of vitamin D, vitamin K, and vitamin B com-
plex cannot meet the entire physiological requirements
and the balance RDAs are met through dietary sources or
nutritional supplements.
Should micronutrients be taken from dietary
sources and food-based supplements (nutraceuti-
cals) or by intake of medicinal supplements (tab-
lets, syrup, and drops)?
In clinical practice, supplements of micronutrients are
usually advised through pharmaceutical formulations
because of convenience, ease of administration, and cost-
eff ectiveness. However, when a cocktail of micronutrients
is taken from medicinal sources, there are several interac-
tions between various micronutrients that can adversely
aff ect their bioavailability.
In general, bioavailability of micronutrients from foods
and food-based supplements (nutrient-fortifi ed foods,
sprinklers, and health drinks) is better with less risk of
Book 1.indb 102 07/06/13 8:58 PM

14. 9 Nutritional Disorders103
internutrient interactions and interferences. Food-based
supplements are more natural and physiological com-
pared with intake of medicinal formulations.
Nevertheless, it is important to remember that nutri-
tional supplements should not be used as a substitute for
a healthy well-balanced diet. Moreover, a balanced nutri-
tious diet not only provides macronutrients but is also
loaded with health benefi ts of micronutrients, phytonu-
trients, antioxidants, and fi ber.
What are the essential components of an eff ective
and comprehensive nutrition program?
There are at least fi ve components or strategies to improve
nutritional status of children at the national level.
1. Special focus on health care, nutrition (life-cycle ap-
proach), and education of the girl children.
2. Promotion of exclusive breastfeeding for 6 months
and continuation of breastfeeding for at least 1 year
or even longer and ensuring proper nutrition of the
nursing mother to improve the quantity and quality
of breast milk.
3. Provision of complementary foods of adequate pro-
teins, calories, and micronutrient density starting at
the age of 6 months.
4. Fortifi cation of food products of universal consump-
tion such as common salt and wheat fl our with nu-
trients of public health relevance such as vitamin A,
iodine, iron, and zinc.
5. Dissemination of practical knowledge and informa-
tion about child nutrition through anganwadis, lay
press, magazines, and television.
Book 1.indb 103 07/06/13 8:58 PM