Rickets in Denmark Prevalence of nutritional and hereditary rickets among children living in
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Transcript of Rickets
Introduction
Functions
Osteoblast
Bone Forming CellSecrete CollagenRelease ALP
Hydrolyses Phosphoryl Etanolamine
Liberates P++ Ca & P Products
( Howland Formula )Mineralization( Ossification )
Osteoclast
Bone Resorping CellRich in Acid PhosphataseMembraneouse Growth
Transverse Diameter of Bone
Cartilaginous Growth
Length of BoneZPC Formation Dependent On
Howland Formula In BLood
PTH
Bone(+) Bone Resorption
Kidney
Ca Reabsorption P Reabsorption
(+) 1 Hydroxylation of Vit.D
Intestine Ca Absorption
Serum Ca Serum P
Calcitonin Serum Ca
SteroidsCatabolic Effect on Bone
Anti-vitamin D Ca Absorption
Vitamin D
Daily NeedsFull Term400 - 800 IU/day
Pre Term800 - 1000 IU/day
Source
Endogenous7 Dehydrocholesterol in Skin
UVR Colcalciferol
( Vit. D3 )
Animal- Fish Liver Oil- Egg Yolk
D3
Plant- Irradiated Green Plants- Vegetable Oils
D2
NB.Cow Milk & Breast Milkare Poor Sources
Metabolism
HepaticActivation
25 Hydroxylase
25-Hydroxy cholecalciferol
RenalActivation
1 Hydroxylase
1,25-Dihydroxy cholecalciferol
- Low Ca , P- PTH
24 Hydroxylase
24,25-Dihydroxy cholecalciferol
- High Ca , P & Steroid
Action
Bone
Low Physiological DoseMineralisation
Indirectly
High Toxic DoseDemineralisation
Directly
Kidney Ca & P Reabsorption
Intestine Ca & P Absorption
Vitamin D Cycle
1- Introduction.mmap - 10/07/2012 - Mohammed Safian
14
Vit. D Def. Rickets
Causes
Rachitogenic Diet
Cow Milk - Vit. D- Ca/P ratio = 2/1
Prolonged BF e`/o Vit. D Supp.
Excess Phytates
Vit. C Gut acidity
Beverages Gut alkalinity
Inadequate Sun Exposure ?
- Glass Windows- Clowdy Weather- Polution- Infants if Overwrapped
Dark Skin
Symp.
M/C 6-24 mths.
Autonomic- Irretability- Anorexia- Sweating
Delayed Motor Milestones eg: Crowling & Walking
Delayed Teeth EruptionDeformities
Fractures - Asymptomatic- Green Stick Fracture
Recurrent Chest Infections
Tetany - Late , Rare- Most Dangerous
Sign
Skeletal
Skull
- Frontal & Parietal Bossing- Box Shaped Skull ( Caput Quadratum )- OFC- Delayed Closur Of A.F- Delayed Teeth Eruption- Cranio Tabes ( Only in 1st. year )
Thorax- Rickety Rosary- Harison`s Sulcus- Longitudinal Groove- Pigeon Chest
Limbs- Broadning- Marfan Sign- Genue Varum ( Bow Legs )- Genue Valgum ( Knok Knees )
Vertebral Columns
KyphosisSmoothRondedCorrectable
Lumber LordosisScoliosis
Pelvis- Inlet Contraction- Outlet Contraction
Non-Skeletal
Muscular Hypotonia
- Delayed Motor Milestones- Abdominal Distension- Spine Deformities- Visceroptosis- Acrobatic Rickets
Abdomen
Pot Belly Protruded Abd.
Palpable Liver & Spleen
Ptosis
Enlagment- RES Hyperplasia- Von Jack's Anemia- Hepatic Rickets- Cystinosis
Nurological
EarlyHypotonia
LateTetany- Manifist
- Latent
Infections Susceptibility to Infections
Pseudoleukemia Infantum
Von Jack's Anemia
anemia ass.e` HSM & GLN
CBC- Anemia- TLC ( 25000/mm )
due toEffect Of Vit. D DeficiencyOn Hematopoietic System
Invst.
Serum Ca
Normal , Low NormalN: 9 -11 mg %
Hypocalcemia ( Tetany ) if
- Failure of P.Th. Gland- Vit. D Dose e` No Ca- Exhaustion of Ca Bone Stores- Chest Infection Alkalosis
Serum P1.5 - 3 mg %N: 4.5 - 6.5 mg %earliest to return normal e` ttt
Serum ALP
N: 145 - 500 IU/LLeast to Return Normal
ALP + Rickets
- PEM- Cong.Hypophosphatasia- Zinc depletion
tttVit. D
Oral2000 - 6000 IU/day
Till Recovery
IMStoss Therapy =600.000 IU/dose
Repeated after 2 wks.adequate Ca , Ph.
2- Vit. D Def. Rickets.mmap - 10/07/2012 - Mohammed Safian
15
Renal rickets ( A )
Glomerular
Causes
Phosphate Retention 2ry Hyperparathyroidism Bone Resorption Osteitis Fibrosa Cystica
Hydroxylation of Vit. D
Metabolic Acidosis Solubilization of Ca Salts in Bone
Cl.P
- Rickets- Renal Disease- Growth Failure- Dwarfism- Tetany is Rare dt. Acidosis
Invst.Lab. PTH , P , ALP The Only Rickets e` P
X-Ray- Epiphyseal Changes of Rickets- Osteitis Fibrosa Cystica
ttt
- Renal Transplantation ( of Choice )- Active Vit D ( 1.25 (OH)2 D3- Ca- Low P Diet & P Chelators ( Ca Carbonat )- ttt of Acidosis ( NaHCO3 )
Renal Osteodystrophy Chronic renal failure
Tubular
Path.- Defect In PCT- Renal Absorption OF Phosphate- Activation Of Vit. D
invst.
- P is Low in Blood & v.High in urine- Normal urinary CAMP- Normal PTH- High ALP- Ca is low or normal
ttt- Oral P Supp.- Ca- Active Vit. D ( 1.25 (OH)2 D3
Primary Familial Hypophosphatemic Rickets ( XLD )
Fanconi $
Def.- Defect in PCT- Absorption of : P, AA , Glucose , HCO3 , Na , K
Causes
- Idiopathic AD or AR- Lowe $- IEM: Cystinosis ( Lignac $ )- Lead & Hg poisoning- Outdated Tetracyclins
Renal rickets .mmap - 10/07/2012 - Mohammed Safian
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Renal rickets ( B )
Fanconi $
Def.- Defect in PCT- Absorption of : P, AA , Glucose , HCO3 , Na , K
Causes
- Idiopathic AD or AR- Lowe $- IEM: Cystinosis ( Lignac $ )- Lead & Hg poisoning- Outdated Tetracyclins
Cystinosis
Def.- AR Disorder- Accumulation of Cystien Crystals- dt efflux from cells
Cl.P
Renal - Fanconi $- CRF
RES - HSM- G. Lumphadenopathy
Cornea Photophopia
Melanocytes - Blond Hair- Fair Skin
Bone Marrow Pancytopenia
Invst. Mesure Cystine Contentin WBCs & Fibroblast
ttt Cystien ChelatorCysteamin
Renal rickets ( B ).mmap - 12/07/2012 - Mohammed Safian
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Tetany
Def. Excitability Of Central & Peripheral Neurons
dt. Ionizable Ca, Mg or Alkalosis
Hypocalcemic
PTH
Transient Neonatal HypocalcemiaEarly 1st. 72 hrsLate 5 -10 days
Hypoplasia ( DiGeorge )ThyroidectomyAutoimmuneFamilialInfiltration ( Fe - Cu )
RicktsVit. D Def.
Vit. D e'/o Ca Supp. Of Bone Stores
Failure Of Para Th. GlandVDDR Type I & II
Steatorreah & Sever Liver Dis.
Hypomagnesimic
Nutritional InsufficiencyTPN e'/o Sufficient MgChr. DiarrheaTransfusion e' Citrsted BloodNephrotoxic & Diuretic Medications
Alkalotic
Respiratory Alkalosis Hyperventilation
Metabolic Alkalosis Vomiting Alkali Therapy
Cl/P
Latent Tetany
Chvostik SignTapping Facial n.
Deviation Of Angle Of Mouth
Troussau SignIsch. Of Forearm For 3 min.
Carpal Spasm
Pernoeal SignTapping Pernoeal n.
Dorsiflextion & Eversion Of Foot
Erb Test< 5 mA
Muscle Contraction Q-T Interval
- Ca 7- 9 mg % ,or- Mg 1.2 - 1.5 mg %
Manifest Tetany
Carpopeadal SpasmPainful
Laryngismus StridulousCroup & Cyanosis
ConvulsionsGeneralized
Numbnes & ParathesiaHand & Feet
- Ca < 7 mg % ,or- Mg < 1.2 mg %
Invst.
- Ca ( N: 9-11 mg % )
- Mg ( N: 1.5 - 1.8 mg % )
- PH ( N: 7.35 - 7.45 )
ttt
Ca Gluconate 10%- 1-2 ml/kg- IV over 10 min.- e' Monitoring of HR
Mg Sulphate 50%- 0.2 ml/kg- Deeply IM
Psychogenic HyperventilationRebreathing into Bag
CO2
NB
if Convulsing
ABC
Anti Convulsion
Diazipam
if Laryngeal SpasmO2
Tracheostomy if Needed
Tetany.mmap - 16/06/2012 - Mohammed Safian
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Hypervitaminosis D
Def.> 20.000 IU Vit. D for 1- 3 mths.
ttt
Stop Vit. D intake Ca in Diet
ChelatorsAl OHNa Versenat ( EDTA )
Cortisone in Sever Cases
Calcitonin New Line of ttt.
CL/P
Kidney
- Polyuria , Polydepsia- Dehydration- Nephrocalcinosis- Renal Stons- RF
GIT
- Anorexia- Constipation- Vomiting- Pain ( acute pancreatitis )
CVS- HTN- Calcivication of Aortic Valve
CNS- Confusion- Irretability
BloodAnemia
SystemicMetastatic Calcification
Vit K
- Oxidizing agent Hemolysis- ( Jaundice, Kernecterus)
Vit A
- Nasea Vomiting- ICP ( Pseudo tumor cerebri )- Bulge of fontanelles, Papilledema
Hypervitaminosis D.mmap - 12/07/2012 - Mohammed Safian
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