Infantile rickets for pedo

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    Infantile rickets for pedo Infantile rickets for pedo Presentation Transcript

    • INFANTILE RICKETSDr Hussein AbdeldayemProfessor of pediatrics
    • • Gross motor development in the first 18 months of age• Causes of delayed sitting in 10 months age baby• Infantile rickets i- daily Vitamin D requirment: 1000 u ii- craniotapes is the earliest bone change iii- delayed walking due to hypotonia and lax ligaments iv- serum phosphate is low due to compensatory hyper PTH
    • VITAMIN DNot really a vitamin● Prohormone produced photochemically in the skin● Closely related to classical steroids
    • Vitamin D sources1- sun exposure2- diet: egg yolk, fish liver oil3- supplemented food with vitamin D
    • Solar ultraviolet B radiation7-dehydrocholesterol → previtamin D3 25 hydroxylation 1 hydroxylation
    • Vitamin D Deficiency• Decreases ca absorptin from intestine• Increases PTH: 1- increases ca reabsorption from kidney 2- increases 1-25 (OH)2 vitamin D production 3- increases phosphate excretion in urine
    • • Solar ultraviolet B radiation (wavelength, 290 to 315 nm) penetrates the skin and converts 7-dehydrocholesterol to previtamin D3, which is rapidly converted to vitamin D3. Because any excess previtamin D3 or vitamin D3 is destroyed by sunlight, excessive exposure to sunlight does not cause vitamin D3 intoxication.
    • Cause of Vitamin D Deficiency• Vitamin D Intake Deficiency, malabsorption, relative deficiency• Impaired activation: - Impaired 25 OH Vitamin D production - Impaired 1,25 OH2 Vitamin D production• Defective Vitamin D receptor
    • Rickets• Rickets is Failure of mineralization of growing bone and cartilage leading to softening and weakening of the bones (of children).• It is caused by lack of vitamin D, calcium or phosphate in your diet.
    • Intake deficiency • No sun exposure • No diet with vitamin D ( exclusive milk ) • Relative as PMT, twinUV-B is shorter thanUV-A and is prone toscatter before 10 AM and after 3 PM ●
    • Intake deficiency• No sun exposure• No diet with vitamin D ( exclusive breast milk )• Relative as PMT, twin In a vitamin D sufficient mother 15-50 IU/L breast milk Assuming intake of 750 ml/day = 11-38 IU/ day vitamin D
    • Fortified formula• Formulas contain 40 to 100 IU vitamin D per 100 kcal● Assuming 750 ml/day this is 200-500 IU/day
    • Defective Absorptiona- rachitogenic diet:• Unmodified cow milk ( high in Phosphate P)• High CHO food: low in Vit D, calcium Ca and P• Cereals : high in phytate so decreases ca absorptionb- malabsorptionc- chronic diarrhea
    • 2- Defective activationa- liver diseases ( defective 25 hydroxylation)b- kidney diseases ( defective 1 hydroxylation) :• Chronic renal diseases (renal osteodytrophy)• Defective 1 alpha hydroxylase enzyme (Vi D dependent rickets type I)• Tubular acidosisc- Anticonvulsant (phenobarbitone, phenytoin): increased degradation of Vit D
    • End Organ Resistance • End-organ resistance to one alpha Vit D (Vit D dependent type II)It is characterized by florid rickets, alopecia, hypocalcemiaand resistance to therapy with high dosages of vitamin D3.It results from autosomal recessive mutations of the vitamin D receptor gene
    • Congenital Rickets• newborn with congenital rickets: SGA, PMT• Risk factors: 1-Low maternal nutrition of Vi D during pregnancy 2- Low sun exposure during pregnancy 3- Closely spaced pregnancy. Symptoms: hypocalcemia as convulsions, generalized decreased of bone ossification plus other features of early rickets)
    • CP
    • Rickets.mp4
    • Order of Pathological changes inrickets• First: increase AP• Then: radiological findings• Finally : clinical
    • Early Rickets• Irritability• Insomnia• Sweat head and body• Craniotapes• Chest rosaries• Harrison sulcus• +
    • DD craniotapes• DD: rickets, hydrocephalus, osteogenesis imperfecta, syphilis, newborn especially premature round suture lines
    • Rachitic rosaries DD• Infantile rickets ( early ),• Scurvy,• chondrodystrophy)
    • Head C/P• Large head• large and delayed closure AF• Head bosses ( box shaped skull)• craniotapes (early age )
    • DD head bosses• Rickets• Scurvy• Congenital syphilis• Hurler syndrome• Achondroplesia
    • Teeth• Delayed teeth eruption• Decayed teeth
    • Extremities• Broadening ( wrist. Ankle, knee )• Marfan sign• Deformity• Green stick fracture
    • Extremities• Broadening ( wrist. Ankle, knee )
    • Extremeties• Deformities Rickets2.mp4
    • Spine and Pelvis• Sitting kyphosis• Standing lordosis• Deformed pelvisShort stature:Spine + pelvis + deformed LL bone
    • Chest• Rosaries ± longitudinal groove• Harrison sulcus• Pigeon chest
    • abdomen• Protruded abdomen• Palpable liver ( ptosed liver)• Palpable spleen
    • Muscle and ligaments• Generalized hypotonia• Acrobatic baby• Delayed motor milestones ( delayed sitting, delayed crawling, delayed walking)
    • others• Repeated infection• Anemia• Malnutrition
    • Which is consistent with vitamin D deficiency rickets? CALCIUM PHOS ALK PHOS• Normal Normal Low• Low Low Low• Low Increased Increased• Low Normal Normal• Normal Low Increased
    • Investigation• Blood: ca, phosphate, alkaline phoshatase• Blood 25-hydroxyvitamin D levels below 20 ng per milliliter.• X- ray
    • X-ray of a 20 month old boy with rickets • Notice the bow shape of the legs.
    • X Ray Findings• 1- broadening• 2- cupping• 3- fraying• 4- widening of space between carpal or metatarsal bones and ulna or radius end (or tibia)• 5- decreases bone density• 6- Raised periosteal• 7- greenstick fracture• 8- deformity as bow legs• 9- chest rosaries
    • X Ray Findings• 1- broadening• 2- cupping• 3- fraying• 4- widening of space between carpal or metatarsal bones and ulna or radius end (or tibia)• 5- decreases bone density• 6- Raised periosteal• 7- greenstick fracture• 8- deformity as bow legs• 9- chest rosaries
    • Healing Rickets• i- X ray needs 2-3 weeks to appear• ii- X ray: line of preparatory calcification ( healing line)• iii- X ray start to increase bone calcification in the shaft and below the healing line
    • Rickets tt• oral daily Vit D3: 2000-6000 IU for 2-4 wks (till healing by X ray) then usual daily need dose of 400 IU, or• shock therapy: single IM ( or oral) large dose of Vit D3 : 600.000 IU, once healing appears by X ray starts oral usual daily need, or• daily one alpha (calcitriol) drops till healing then usual daily need : especially Vit D3 dependent rickets I or II N B.,• 1- in premature : gives Vit D plus Ca after 2 weeks of birth• 2- in malabsorption : oral (10.000 IU /d) or IM• 3- Vit D dependent type I : one alpha oral or Vit D3 oral 200.000- 1million IU/d
    • Quiz• Mother came to ER midnight with her baby. Noisy breathing with delayed closure of AF , broad wrist and delayed crawling. Diagnosis?
    • • tetany : Rickets CPT repeated infections: specially • 1-latent tetany : (Ca =7-9 mg/dl) chest infections ( pneumonia, • chovstoke sign, atelectasis) due to • • rosaries Trousseau sign, • rib softening • Erb’s ( or peroneal ) sign • general immune defect 2- manifest tetany: (Ca < 7 mg/dl) • carpopedal spasm • generalized convulsion • stridor ( night emergency)• skeletal deformity: • short stature • limb deformity as bow legs or knock knees • Deformed pelvis
    • Tetany ttemergency : • IV Ca 10 ml of 10% solution, over 10 minutes • For convulsion: IV diazepam ( 0.3 mg/kg) • For stridor: O2 inhalation• maintenance; oral Ca gluconate ( 500 mg/kg/day)• treat the cause: oral vitamin D for rickets
    • Prevention• sun exposure (UV) of both the mother and the baby: • direct sun ( not behind glass) • clear sky • around 12.00 am (noon) with few clothes• Vit D daily after 2 weeks of birth: 1000 IU/d for • premature • twin and • infants of mother with osteomalacia• 4th mo age, add 400IU/d oral Vit D of breast fed infants
    • Breast supplementation• Vitamin D• LCPUFA• Zinc• ? probiotic
    • Vitamin D ToxicityCP• irritability• anorexia• constipation with hard stool pellets• polyuria• polydypsia• hypotonia
    • • Complication: nephrocalcinosis and renal failure, dehydration,• Investigation: 1- blood Ca: > 12 mg/dl 2- U/S: Nephrocalcinosis 3-Plain X ray: Nephrocalcinosis, generalized osteoporosis, metastatic calcification •
    • Vitamin D Toxicitytreatment• stop intake of Vit D and Ca• hydration for dehydration• give rachitogenic diet: poor in milk + high CHO and cereals• prednisone 2mg/kg/d till normal Ca• alkanization: oral Aluminium hydroxide
    • AAP RecommendationREVISED 2008 At least Daily 400 IU of Vitamin D is needed
    • Nonskeletal Actions of Vitamin D{ 1-25 (HO)2 D }1- genes responsible for the regulation of cellular proliferation, differentiation, apoptosis, and angiogenesis ≠ cancer2- immunomodulator3- reduces risk of autoimmune disease as DM type 14- of importance in tt : schizophrenia, depression, wheezy chest, CV diseases ( heart disease, BP)
    • Vitamin D and autoimmune disease• DM type I• Hashimoto thyroiditis• Graves disease• Addison’s disease
    • Vitamin D and Brain• Fetal deprivation of vitamin D3 could be associated with adverse neuropsychiatric outcomes
    • Parathyroid Gland• Parathyroid hormone increases the metabolism of 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D, which further exacerbates the vitamin D deficiency. Parathyroid hormone also causes phosphaturia, resulting in a low-normal or low serum phosphorus level• Compensatory hypersecretion of PTH is abolished by hypomagnesemia
    • Actions of PTH Ca PO4 1. 2. 3. 25 OH Vit D 1α hydroxylase Gut 1,25 (OH)2 Vit D NET EFFECT
    • Choose correct answer A. Vitamin D deficiency rickets B. Renal osteodystrophy (renal rickets) C. Both D. Neither1. Increased phosphate level B2. Increased PTH level C3. Increased creatinine level B