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Rickets
1.
2. Clinical condition in which there is inadequate
mineralisation of growing bone.
Primarily due to Vit D deficiecy or a disturbance in its
metabolism.
Rickets – children
Osteomalacia bones of adults.
3.
4.
5. VIT.D DEFICIENCY decrease absorption of ca. & p from
the gut decrease ca. level in blood
increase PTH
mobilization of ca. & po4 from bones and
decrease tubular reabsorption of p in kidneys
normal serum ca. & low serum po4
decrease ca. available for bones
ca. & po4 will be far below 40
failure of calcification around the mature cart. cells
and osteoblasts in the ostoeid tissue.
6. Normal bone growth
THERE ARE 4 ZONES :
1.ZONE OF RESTING CARTILAGE : 1 layer
2.ZONE OF PROLIFERATING CART. : 6 layers
3.zone OF PROVISSIONAL CALCIFICATION "epiphyseal line " :
the cart. cells in this layer become mature, they containe alkaline phosphatase
release the phosphate in the matrix which already contains ca. & po4 in
solution increase production of ca. & po4 once the production exceeds
40 precipitation of ca.phosphate in the matrix around the cartilage cells
death of the cells.
4.ZONE OF BONE FORMATION :
The layer of prov. calc. is invaded by capillaries and osteoblast which deposit a
layer of organic bone matrix "osteoid tis.“ rapidly meniralized and the
calcified cartilage ultimitly replaced by bone.
7.
8. PATHOLOGY
1. The mature cartilage cells will not die and the
proliferating zone will be formed of many layers and
invades the adjacent zone of of provis. calc.- irrIegularity
of epiphseal line.
2. The prov. calc. zone and newly formed ost. tis. will fail to
calcify or will calcified irregularly.
wide irregular frayed zone of non rigid tis. "
RACHITIC METAPHSIS " is produced.
3. In the shaft the preformed bone is replaced by
uncalcified ost.
soft rarified cortical bone
bone deformities & green stick fractures.
9. CAUSES
Vitamin D deficiency
Lack of sunlight exposure
Dietry lack of meat & dairy products
Malsbsorption
Failure of 1,25 vit D synthesis
Chronic renal failure
Hyperphosphataemia & kidney damage
Vit D dependent rickets ( type 1) - AR
Inactivating mutation in 1,25,hydroxylase enzyme.
10. Vitamin D receptor defects
Vit D dependent rickets type2 (AR)
Mutation in vit D receptors
Defects in phosphate metabolism
Primary hypophosphataemic rickets(XLH)
renal phosphate wasting.
Fanconi syndromes
Proximal renal tubular acidosis.
Hypophosphatasia
Mutation of bone specific ALP
11. NUTRITIONAL RICKETS
VIT D DEFICIENCY
3-18 MONTHS
LACK OF SUNLIGHT EXPOSURE & NO
DIETARY SUPPLEMENTATION
PROLONGED BREAST FEEDING
CALCIUM DEFICIENT DIET
BOTH
VEGETARIANS WHO AVOID DAIRY
PRODUCTS
13. X LINKED HYPOPHOSPHATEMIA
MOST COMMON INHERITED ETIOLOGY
X LINKED DOMONANT DISORDER
DEFECT IN PHEX GENE(REGULATE TRANSPORT OF
RENAL PHOSPHATES)
RENALPHOSPHATE WASTING
HYPOPHOSPHATEMIA
RICKETS
SHORT STATURE
DENTAL ABSCESSES
14. McCUNE-ALBRIGHT SYNDROME
HYPOPHOSPHATEMIC RICKETS
CAFÉ AU LAIT SPOTS
PRECOCIOUS PUBERTY
FIBROUS DYSPLASIA OF MULTIPLE LONG
BONES
CONSTITUTIONAL ACTIVATION OF C-AMP-PKA
SIGNALING PATHWAY DUE TO GENETIC DEFECTS
IN G SIGNALING PROTEINS
15. 1 ALPHA HYDROXYLASE
DEFICIENCY
VITAMIN D DEPENDENT RICKETS
<24WKS OF AGE
WEAKNESS
PNEMONIA
SEIZURES
BONEPAIN
BONE CHANGES OF RICKETS
16. END ORGAN INSENSITIVITY
3 TO 30 FOLD HIGHER THAN NORMAL VALUE OF
1,25(OH)2VITD3
NEAR TOTAL LOSS OF HAIR FROM HEAD & BODY
17. FANCONIS SYNDROME(RENAL
TUBULAR ABNORMALITY)
FAILURE OF RENAL TUBULAR ABSORPTION OF MANY
MOLECULES SMALLER THAN 50Da
KIDNEYS LOSE CALCIUM,PHOSPHATE,MAGNESIUM,
BICARBONATE,SODIUM,POTASSIUM,GLUCOSE, URIC
ACID AND SMALL AMINOACIDS
SHORT WITH RICKETS & DELAYED BONE AGE
HYPOPHOSPHATEMIA,METABOLIC ACIDOSIS,RENAL
OSTEODYSTROPHY(DECREASED CALCIUM AND
PHOSPHATE REABSORPTION)
18. HYPOPHOSPHATASIA
. ALP DEFICIENCY
. 1 PER 1 LAKH POPULATION
AUTOSOMAL RECESSIVE CONDITION
MUTATION IN ALP GENE IN CHROMOSOME 1
ABNORMAL MINERALISATION OF BONE
PATHOLOGICAL FRACTURES
LOSS OF TEETH
FAILURE TO THRIVE,RAISED INTRACRANIAL
PRESSURE & CRANIOSYNOSTOSIS
19. PERINATAL LETHAL FORM
CHILDHOOD FORM PRESENTS WITH RICKETS AT
2 OR 3 Yrs WITH REMISSION IN ADOLESCENCE
ADULT FORM-MILD OSTEOMALACIA WITH
PATHOLOGICAL FRACTURES
20. RENAL OSTEODYSTROPHY
BONY CHANGES ACCOMPANYING ESRD
RF HYPERPHOSPHATEMIA
HYPOCALCEMIA SECONDARY
HYPERPARATHYROIDISM
SUBPERIOSTEAL EROSIONS & BROWN TUMORS
RICKETS
VARUS OR VALGUS DEFORMITY AT THE KNEE OR
ANKLES
21. CLINICAL FEATURES OF RICKETS
Delayed milestones
Irritable child
Lethargy & hypotonia
Stunted growth
22. Rickets: signs
Skull
Craniotabes
Frontal and parietal bossing,
flat occiput
Anterior fontanelle is large with delay in closure.
27. “POT BELLY “ due to
hypotonia of abdominal muscles & intestine.
Downward displacement of the liver & spleen.
28. Investigations
X-RAYS
The X-RAYS of the wrists is best for early diagnosis
1.The classic triad of rickets :
Broadening
Cupping (concave)
Fraying ( irregular)
2.Increase distance between the distal ends of radius &
ulna and the metacarpal bones .
29. 3.Demeniralization of the shaft “ hypodensity”
4.Fractures & deformities may be present
LOOSER’S ZONE pseudo # occuring at the
site of stress.
30. SUBPERIOSTEAL EROSIONS & BROWN TUMOR IN
RENALOSTEODYSTROPHY
SUBPERIOSTEAL EROSIONS-LATERAL ASPECT OF
DISTAL RADIUS & ULNA & MEDIAL ASPECT OF
PROXIMAL TIBIA.
31. Biochemical findings
NUTRITIONAL RICKETS
S . Ca - low / normal
S . Posphorus – low
S . ALP - High
PTH – Raised
25(OH)D - low
32. VDRR is suspected when rachitic patients fail to
respond to Vit D & calcium.
1,25(OH)2D raised 3-30 FOLD
34. 1 ALPHA HYDROXYLASE DEFICIENCY
LOW CALCIUM AND PHOSPHORUS
HIGH ALP & PTH
NORMAL 25-HYDROXY VITAMIN D3
MARKEDLY DECREASED LEVEL OF 1,25(OH)2VITD3
35. Treatment of rickets
NUTRITIONAL RICKETS
Adequate exposure to sunlight.
Supplementation of vit D in diet.
Therapeutic doses of vit D 200-600 units/day or
As single IM inj of 600000 IU induces rapid
healing.
If line of healing ( sclerosis on the metaphyseal
side of growth plate) is not seen after 3-4 weeks ,
same dose is repeated.