Vitamin use in children should be done only after knowing the RDA and Toxic upper limit of dosing. Many a times some uncommon presentations of Vitamin deficiency go unnoticed. The main purpose of this presentation is to promote rational use of vitamin and shed some myths and false claims regarding vitamins.

1. OFTEN OUT OF MIND BUT NOT OUT OF SIGHT:
VITAMINS IN PEDIATRIC DISEASES
Dr. Shailesh Mehta
MD Pediatrics
Senior Pediatric Consultant, Chandigarh

2. Vitamin Bhel – Suggest its dose for a 2yr child

3. Deficiency of this vitamin is rare except in
malabsorptive syndromes or in preterms
• Its deficiency in premature infants causes
thrombocytosis, edema, and hemolysis( 2nd
month), potentially causing anemia.
• Children with prolonged deficiency present
after 1 yr age with cerebellar disease,
posterior column dysfunction, and retinal
disease.

4. Vitamin E –RDA Adult 15mg/day( 33 I.U.)
Kids 7mg/day (15 I.U.) Toxic UL=RDA X 60
• Rare presentation-Ataxia with isolated vitamin E deficiency (AVED)- AR –mutation in TTPA gene.
• Needed in Abetalipoprotenemia, Cystic fibrosis ( 1 I.U./Kg /day)
• In neonates, the dose of vitamin E is 25-50 units/day for 1 wk

5. Vitamin E as an anti - oxidant
• Some evidence supports its role in Non
alcoholic fatty liver disease
No evidence proven role in-
• Age related macular degeneration
• Prevention of Cardiovascular diseases,
• Retinopathy of prematurity
• Cancers.
• Tardive dyskinesia, dementia, Alzheimers

6. What would you suspect?
A 6-week-old exclusively breast fed infant
presented to a village primary health centre
with
• a 1-day history of poor breastfeeding,
• increased crying
• pallor
• a “full and hard” anterior fontanelle

7. Vitamin K RDA 2 to 2.5 mg/d for infants and
30 to 75 mg/d for 1- to 18-year-olds Vitamin K - cofactor for
synthesis of gamma-carboxylated proteins ,clotting factors such
as factors II (prothrombin) ,VII, IX, X and activation of protein C
and S

8. The village doctor suspects VKDB aka HDN and gives
Vit K- 1mg I.M. and refers
• After 48 hrs, child the neuroimaging shows
ICH but Prothrombin time is normal ( short
half life)
• PIVKA 2 can be of major value in retrospective
diagnosis of VKDB even weeks after the event
Vitamin K 0.5mg -1 mg I.M. recommended
universally at birth
Vitamin K used for reversal of effects of
coumarin like anticoagulants
Its role in bone mineralization in osteoporosis
is conflicting.

9. Guess the vitamin

10. Pellagra – 4 Ds- Treated on 50-300 mg/day of niacin
Niacin (RDA)
6 to 12 mg/day in children

11. Niacin dose for Hyperlipidemias
1000 to 3000 mg/day

12. Role of -----in Promyelocytic leukemia

13. Role of -----in Promyelocytic leukemia

14. Retinol, Retinal, Retinoic acid
Vision,
epithelial
differentiation
, antioxidant
Night
blindness,
Xeropthalmia,
Bitot’s spots,
Follicular
hyperkeratosis
Used in Pro
Myelocytic
Leukemia

15. High dose Vit A used

16. High dose Vit A x 2days then one
more dose after 2 weeks

17. Topical and systemic retinoids used

18. Topical derivatives of
Vitamin A (Retinoids) and D (Calcipotriol)

19. India’s Universal Mega-dose Vitamin A Supplement
Program: Time to Bid Goodbye!
Dr Piyush Gupta Indian Pediatr 2021;58: 311-312
RDA
0-1 Yrs 400- 500 mcg RAE UL= 600 mcg
1-3 Yrs 300 mcg UL= 600 mcg
3-8 Yrs 400 mcg UL= 900 mcg
8-18 Yrs 600- 900 mcg UL= 2800 mcg
1 mcg RAE= 3 I.U.
CRUX – We should keep Daily Vit A dose between 1500- 2500 I.U. to be
safe in children
In measles x 2 days
6 months to 1 yr = 1 lakh I.U. ( 30000 mcg RAE)
1 Yr onwards = 2 lakh I.U. ( 60000 mcg RAE)

20. Other uses if Vitamin A
• Some evidence exists to show its role in
Bronchopulmonary dysplasia in preterms.
No conclusive evidence for its role in-
• ROP
• Atherosclerosis
• NEC

21. RDA Adults 2 mcg/day, Kids 1 mcg/day
No toxic upper limit
This Vitamin good for Blood and Brain

22. Exclusively breast fed 8 months old with globally
delayed milestones, anemia and tremors

23. Exclusively breast fed 8 months old with globally
delayed milestones, anemia and tremors

25. B12- THE BLOOD AND BRAIN VIT
(Function is one carbon transfer)

27. NO TOXIC UL FOR B12
RDA-0.9 mcg per day in Infants and toddlers
1.2 – 1.8 mcg /day for children 4yrs -13 yrs
2.4 mcg/ day thereafter
• S.B12 levels may not always correlate with tissue B12
levels.
• Blood B12 level < 200 pg/ml is considered deficiency
• Low B12 levels with high MMA is specific for B12
deficiency
Deficency leads to megaloblastic anemia and neurological
symptoms (subacute combined degeneration )
Infantile Tremor Syndrome-Treated on daily 1000mcg B12
injections for 1 week

28. FOLIC ACID

29. Folic acid
RDA of FOLIC ACID- CHILDREN 150 mcg/day – 400mcg/day
Toxic UL 1000 mcg/day

30. Folic acid
Used in TPN, malabsorptive states and during increased
demands in hemolytic anemias
No evidence to support its role in preventing cancers
although its active form “Folinic acid” used with
chemotherapeutic agents , to prevent deficiency.
Low Serum Folate detectable within 2 weeks of deficient
diet
High Homocysteine levels may be seen in B12 and Folate
deficiency both
If Homocysteine levels are raised and MMA is normal, it
is indicative Folate deficiency

31. Vitamin as a lifesaver in an acute condition !

32. Methylene blue can cause worsening of hemolysis if used to treat
Methemoglobinemia in patients with G6PD deficiency
In such cases electron donors like Ascorbic acid ( 500mg to
1000 mg) may be used in high doses along with exchange
transfusions

33. RDA ( for children) Vit C= 15-45 mg/day
• Collagen synthesis ( Used to treat Scurvy- 100-200
mg/day for 3 months)
• Conversion of dopamine to norepinephrine and
tryptophan to serotonin
• Conversion of cholesterol to steroid hormones and bile
acids
• Biosynthesis of carnitine
• Used to treat methemoglobinemia in G6PD deficiency
, where using methylene blue may be detrimental.
VITAMIN C is a important electron donor (antioxidant
and a reducing agent)

34. THIS IS HOW WE DO IT

35. THIS IS HOW ITS DONE

36. Vitamin D –Recommended for all children-RDA-
400 I.U. till 1 year , After 1 yr age,600 I.U. till adolescence
Upper limit 1,000 I.U./day for infants 0 to 12 months old and
2,000 IU/ day for older children upto 10 yrs age
• Deficiency can be treated over 6 weeks by
• Daily dose of 2000 I.U.
• Or Weekly 60000 I.U.
Rickets can be treated by- Stoss Regime
100000 I.U. to 600000 I.U. over 1- 5 DAYS
Daily dose for 90 days
Daily 2000 I.U. for age <1 yr
Daily 3000 I.U.- 6000 I.U. for age >1yr
Endocrine Society Practice Guidelines and Consensus
J Clin Endocrinol Metab, February 2016, 101(2):394–415
J Clin Endocrinol Metab, July 2011, 96(7):1911–1930

37. AUTOCRINE PARACRINE ROLE OF VIT D

38. Which vitamin deficiency ?

39. A syndrome of megaloblastic anemia, diabetes
mellitus, and sensorineural deafness.

40. Patients aged 7–20 years
Distal weakness,
contractures,
atrophy, legs affected
more than arms
T2 bilateral
caudate
and putamen
hyperintense
lesions

41. Determination of the SLC25A19 sequence should be considered
in patients with bilateral striatal necrosis and progressive
polyneuropathy.
Ann Neurol 2009;66:419–424
Thiamine is absorbed in the intestine by a high-affinity thiamine transporter encoded by
the SLC19A2 gene.
Within the cell, thiamine is converted to TPP by the cytoplasmic enzyme TPP-kinase and
is transported to the mitochondria by a dedicated transporter, SLC25A19, in exchange
for thiamine monophosphate

42. Thiamine metabolism dysfunction syndrome type 2
Gerards M et al. Brain. 2013 Mar;136(Pt 3):882-90. Epub 2013 Feb 18.
Screening for mutation in the thiamine transporter SLC19A3 in patients with Leigh
syndrome, who might benefit from high doses of thiamine and/or biotin.

43. Thiamine-responsive megaloblastic anemia –
Mutations in the SLC19A2gene, which encodes a thiamine transporter.
Ribose sugar is a building block for nucleic acid synthesis.
Key enzyme for synthesis of Ribose requires thiamine

44. THIAMINE – Vitamin B 1
(RDA)
0.5 to 0.9 mg/day for children
• In the TCA cycle, serves as a catalyst in the conversion of pyruvate to
acetyl CoA
• Transketolation of the pentose phosphate pathway
• Synthesis of glutamate and γ-aminobutyric acid as well as myelin
sheath maintenance .
• Promote cholinergic and serotonergic nerve conduction and synaptic
axonal transmission
• Its deficiency caused Beriberi , Wernicks- Korsakows encephalopathy
• Children with cardiac failure, convulsions, or coma should be given
10 mg of Thiamine intramuscularly (IM) or intravenously (IV) daily for
the 1st wk. This treatment should then be followed by 3-5 mg/day of
thiamine orally (PO) for at least 6 wk.
• TRMA,Infantile Beriberi and other dependency states require
higher dosages (100-200 mg/day).

45. Vitamin as a life saver!
• A seven-month-old male Caucasian child presented to
APC PGI with a history of
-a peculiar sweat sock breath odour for four days
-fever for two days and
-an altered sensorium for the past one day.
On examination, acidic breathing and hypotonia
• Laboratory investigations revealed a high anion gap
metabolic acidosis and hypoglycaemia
Urine biochemistry revealed an excretion of
glutaric, propionic and methyl malanoic acids
MRI was done for altered sensorium

46. Axial T2WI MR image shows
bilateral symmetrical
hyperintensities in both
globus pallidi (black arrows)
and increased
perisylvian and fronto-
temporal extra axial
cerebrospinal fluid
spaces (white arrows).

47. Axial T1WI MR image shows mild hypointensity (arrows) noted
in the globus pallidus on both sides.
Diffusion-weighted MR image shows restricted diffusion
in the bilateral globus pallidi (arrows)

48. Successfully treated by a vitamin!
HINT -Treated by Vitamin which is used for
cheilitis, stomatitis , anorexia nervosa and even
migraine!
HINT –This Vitamin reverses lactic acidosis by
Stavudine and Zidovudine
HINT-This vitamin is abundant in milk, cheese,
eggs, liver, leafy vegetables, whole grains, whey

49. Riboflavin – Vitamin B2
R.D.A 0.5 to 0.9 mg/day in children
Riboflavin and carnitine may be the first-line treatment for
MADD. (Multiple acylcoenzyme A (acyl-CoA) dehydrogenase
deficiency also known as Glutaric acidemia type 2 [GA2]
Flavoproteins (FAD, FMN) are catalysts in a number of
mitochondrial oxidative and reductive reactions and
function as electron transporters
MRI FINDINGS OF GLUTARIC ACIDURIA TYPE 2
Mumtaz HA, Gupta V, Singh P, Marwaha RK, Khandelwal N
Singapore Med J 2010; 51(4): e69-e71

50. Which vitamins are used in this
condition?
Developmental delay, Marfanoid appearance,
osteoporosis, ocular abnormalities,
thromboembolic disease, and severe
premature atherosclerosis.

51. Which vitamins are used in this
condition?
Developmental delay, Marfanoid appearance,
osteoporosis, ocular abnormalities,
thromboembolic disease, and severe
premature atherosclerosis.
Vitamin B6, B12, Folic acid

52. Guess the vitamin
• Helps in transamination of amino acids, a key step in
gluconeogenesis.
• Decarboxylation of amino acids, a key reaction in
the conversion of tryptophan to niacin,
• Heme synthesis,
• Sphingolipid biosynthesis
• Neurotransmitter synthesis,
• Immune function
• Steroid hormone modulation.
It is also a key enzyme cofactor in the
• transsulfuration pathway by which homocysteine is
converted into cystathionine and its subsequent
conversion to cysteine

53. Vitamin B6 Pyridoxine
RDA 0.5 to 1mg/day in children
• Pyridoxine-dependent epilepsy (PDE) due to
antiquitin (ATQ) deficiency and the related disorder,
pyridoxamine 5'-phosphate oxidase (PNPO) deficiency,
are rare but treatable genetic causes of medically
refractory neonatal seizures.
• Marginal Deficiency-manifested as nonspecific
stomatitis, glossitis, cheilosis, irritability, confusion,
depression, and possibly peripheral neuropathy
• VitaminB6 Dependant states – B6 responsive anemia,
xanthurenic aciduria, cystathioninuria, and
homocystinuria
• Routine 10mg pyridoxine to children on ATT ( NTEP)
• For pyridoxine-dependent children, daily doses of
2-10 mg IM or 10-100 mg PO may be necessary

54. Think of vitamins which have role in seizures
You are evaluating a 12- month-old boy for his
• Hypotonia
• Alopecia,
• Seizures.
His Lab shows
- ketoacidosis,
- hyperammonemia
-elevated urine organic acid levels

55. Guess this Vitamin
It is an essential cofactor for several
carboxylase enzyme complexes
• gluconeogenesis,
• fatty acid synthesis,
• catabolism of amino acids
• Gene expression.

56. BIOTIN-(No RDA)Adequate Intake (A.I.)is
8 to 12 mcg/day for children
• Biotin-responsive multiple carboxylase deficiency -
autosomal recessive
• Ataxia
• Ketoacidosis
• Dermatitis
• Seizures, myoclonus, and nystagmus .
The infantile form can be caused by a variety of
mutations in the holocarboxylase synthetase gene.
In children with late onset (usually juvenile) disease,
the defect appears to lie in biotinidase.

57. Take home message
• Campaign use of Vitamin A –
Time to say goodbye.
• Universal supplementation of Vitamin K and
Vitamin D still in place
• Targeted use of Vitamins preferred rather
than a blanket approach
• In the conventional OTC preparations
( VITAMIN BHEL), we should focus on Toxic
Upper Limit of vitamins ( esp Vit A)